Journal of the IAP Karnataka State Branch

Karnataka Paediatric Journal

Vol. 25, No. 3, 4 July - Dec 2011

Karnataka Paediatric Journal Vol. 25, No. 3, 4 Quarterly ; July - December 2011

Journal of the IAP Karnataka State Branch CONTENTS

PAGE No.

1. Prevalence Of Hypertension And Prehypertension Among School Children 82-86 D NARAYANAPPA, HS RAJANI, KB MAHENDRAPPA, 2. Omental Infarct- A Rare Cause Acute Abdomen 87-88 DR.GURUPRASAD SHETTY,ABY DANY, SANJEEV RAI B,HABEEB KHAN,P.C. DAS 3. Klippel Feil Syndrome: A Case Report 89-90 DR.HARSHA M ,SHARAN GOWDA PATIL, SHRIKANT SW, GACHINMANI N 4. Arthrogryposis Multiplex Congenita 91-93 Dr.Niveditha R., Dr.Vikram Singhal, Dr.Murali Keshav, Dr.Rajesh Sm, Dr.Kiran Baliga 5. Prevalence Of Anemia, Morbidity And Examination Performance Among 94-98 Lower Primary School Children Of Davangere City DR.SARVAMANGALA.K .KOUJALGI. M.B MANJUNATH.T.P. 6. Para-Esophageal Hernia Presenting With Severe Anaemia and Pneumonia 99-101 DR.RAVICHANDRAG,FAHEEM M,RAGHAVENDRA B,DEVDAS A, HABEEBR, RESHAD M. 7. Profile Of Respiratory Distress Among NICU Admissions 102 DR BUDENSAB A H. SDM MEDICAL COLLEGE HOSPITAL DHARWAD 8. Multiple Splenic Abscesses Due To An Unsuspected Cause- Case Report 103-105 DR.NARAYANAPPA , DR.N.RASHMI , DR.ANIL KUMAR, DR.RAJANI.H.S ,DR.ANITHA.C 9. Vincristine Induced Unilateral Ptosis 106-108 DR. GURUPRASADA SHETTY, DR. K. SHREEDHARA AVABRATHA, DR B SANJEEV RAI , DR. SEEMA, DR ABY DANY AND DR. DINESH SHET 10. Familial Dandy Walker Variant. 109-111 DR. ROOPA.MANGSHETTY,DR. HOSGOUDA KIRAN, DR. SHRIKANTH.S.W. 11 Rett Syndrome - A Case Report 112-114 DR.R.SHANTI PRIYA, DR.RAJESH SM, DR.VIKRAM SINGHAL, DR.KIRAN BALIGA 12. A Single Center Observational Study Of Use Of Tacrolimus In Managing Nephrotic Syndrome And Review Of Literature 115-119 DR. SUDARSHAN SHETTY K. DR. KISHAN ALVA

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Vol. 25, No. 3, 4 July - Dec 2011

INDIAN ACADEMY OF PEDIATRICS Karnataka State Branch Society Reg No: EKM – S460-2006-2007

OFFICE BEARERS FOR THE YEAR 2011 President Dr. R.T. Patil

Secretary Dr. Ashok V. Badakali

Treasurer Dr. P. Subba Rao

President Elect Dr.Suresh Babu

Bagalkot Mob: 9845365795 Bagalkot Mob: 9880227403 Magalore Mob: 9845872653 Davangere Mob: 9844096775

Historian Joint Secretary Editor K.P.J. Dr. Santosh Soans Dr. Pavan Hegde Dr. B. Sanjeev Rai. Mangalore. Mob: 9343565558 Mangalore. Mob: 9845088116 Mangalore. Mob :9448133494 Executive Board Members Bagalkot : Dr. R. N. Vanaki Bangaluru : Dr. Basavaraj G. V. Belgaum : Dr. Shailesh Patil Bellary : Dr. Kailash Soni Bidar : Dr. Somashekhar Bhalke Bijapur : Dr. M. M. Patil Chikkamangalur : Dr. Sundaresh Chitradurga : Dr. Natraj Dakshina Kannada : Dr. Prasad Naik Davanagere : Dr. Naveen Nadig Dharwad : Dr. Sudhindra Gadag : Dr. Vijay Neelgund Hassan : Dr. Dinesh Kodagu : Dr. Krishnanand Haveri : Dr. Rajkumar Marol Kolar : Dr. J. Krishnappa Kollegal : Dr. Sridhar M. Koppal : Dr. Anand Kumar Mandya : Dr. Narendrababu Mysore : Dr. Shrinivas Murthy Raichur : Dr. Balasubramanya Shimoga : Dr. Deepak Chirdoni Tumkur : Dr. Shivaprakash Udupi : Dr. Shrikiran Uttar Kannada : Dr. Dinesh Hegde Central Council Executive Board Members Dr. Devraj Raichur. Mob : 9449864828 Dr. Karunakar B.P. Mob : 9845263322 Dr. Dinesh S.R. Mob : 9448006166 Zonal Coordinators Bangaluru : Dr. Subramanya N. K Dharwad : Dr. Vijay Kulkarni Gulbarga : Dr. Arundathi Patil Davanagere : Dr. Deepak Chirdoni Mysore : Dr. Narayanappa Ex – Officio’s Dr. K. Doddegowda Dr. S. R. Dinesh 81


Vol. 25, No. 3, 4 July - Dec 2011

PREVALENCE OF HYPERTENSION AND PREHYPERTENSION AMONG SCHOOL GOING CHILDREN *D Narayanappa, HS Rajani, KB Mahendrappa

at any age, in either sex is a contributor to all forms of cardiovascular diseases 4,5 . The prevalence of hypertension in children is reported to range from a high of 16.2% to a low of less than 1% 5-7. This diversity in prevalence of hypertension is due to the varying age groups taken for the study, different criteria adapted for defining Hypertension, basic difference between racial subgroups related to geographic, dietary and cultural factors1,5 .Studies on Hypertension in childhood have an important advantage that they may help in early detection, control and may possibly prevent the harmful sequalae of high blood pressure. The data on Hypertension and Pre hypertension in school going children (5-15 year) is scanty in India. The present study was conducted to evaluate the prevalence of Hypertension and Prehypertension in apparently healthy school children in Mysore city.

ABSTRACT OBJECTIVE: To estimate the prevalence of Hypertension and Prehypertension among school children in the age group of 5-15 years in Mysore city. DESIGN: Cross sectional study SETTINGS: Children aged 5-15 years from three schools in Mysore city were included. Study was done over a period of 1 year during 2006-2007. PARTICIPANTS: A total of 1217 children between 5-15 years. Of them 58.7% were males & remaining 41.3% were females. MAIN OUTCOME: Prevalence of Hypertension and Prehypertension among school children aged 5-15years RESULTS: The prevalence of Hypertension and Prehypertension was 4.8 % and 11 % respectively.

MATERIAL AND METHODS This school based cross sectional study was conducted on one Thousand two hundred and seventeen children in the age group of 5-15 years from 3 schools in Mysore city . The schools were selected by purposive sampling procedure keeping in view the operational feasibility. Information regarding age was obtained from the school records and was also rechecked from each student. The age was recorded in completed years. A pretested proforma was used to record age, socio economic status, dietary habits, family history, anthropometric measurement, body mass index(BMI), blood pressure measurements, detailed clinical examination etc. Family history of hypertension was taken as history of hypertension in parents or grandparents.

CONCLUSION: Efforts must be made to recognize Hypertension and Prehypertension in Childhood. Key words : children, , hypertension ,India , prevalence, prehypertension INTRODUCTION Increasing trend of Hypertension is a worldwide phenomenon1. In the era of rapid epidemiological transition, the burden of chronic diseases is overtaking the burden of infectious diseases in India 2,3. Hence Preventive strategies are essential to combat this epidemic. Hypertension is the most common, most potent universal contributor to cardiovascular mortality. Elevated blood pressure, labile or fixed, systolic or diastolic

* Department of Pediatrics JSS Medical College, JSS University; Mysore, India.

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The weight (kg) was taken by standardized digital weighting machine calibrated to 0.5kg accuracy. Height (to the nearest mm) and weight (to the nearest100 g) were measured using standard techniques. Anthropometric measurements were taken by medical graduates under supervision of co-investigators. Their techniques were corrected and retested until desired level of intra-observer and interobserver variability was reached (coefficient of variation for all measurements < 5%).

institutional ethical committee. A written informed

For diagnosing obesity, the BMI was calculated by the following formula weight in kg divided by height in m2. Agarwal charts of BMI for age and sex were used as reference standards8. Children with BMI above 95th percentile were considered as obese, those above 85th percentile and below the 95th percentile as overweight8.

RESULTS

Blood pressure measurements were taken using a mercury sphygmomanometer as per the recommendations of American Heart Association 9,10 . The measurements were taken in a quiet room in the sitting posture with the arm resting on the table. Efforts were made to eliminate the factors which may affect BP eg anxiety, crying, exercise etc. The average of 3 consecutive readings, taken at 5 mins interval was taken as the blood pressure of the child and compared to age, sex and height percentile standards given by the report of Fourth task force on hypertension control in children9 .

TABLE I : AGE AND SEX DISTRIBUTION OF STUDY POPULATION

Hypertension was diagnosed if the blood pressure either systolic, diastolic or both were equal or more than 95th percentile for age, sex and height percentile. Blood pressure either systolic, diastolic or both between 90 and 95th centile was taken as pre hypertension and Blood pressure either systolic, diastolic or both less than 90th centile was taken as normal9.

TABLE II: PREVALENCE OF PREHYPERTENSION AND HYPERTENSION IN STUDY POPULATION

consent was obtained from the head of the institutions and parents before data collection. Children on antihypertensive medications and known to have chronic heart, renal or hepatic disease were excluded from the study. Statistical analysis were done using EPIINFO software. Out of total 1217 children included in the study, 714 (58.7%) were males and 503 (41.3%) were female children. 728 children were in the age group of 5-10 years and remaining 489 were aged between 11-15 years

AGE(YEARS) SEX MALE 5-10 434 11-15 280 TOTAL 714(58)

FEMALE 294 209 503(42)

TOTAL 728(59.8) 489(40.2) 1217(100)

Numbers in parantheses indicate percentages

The prevalence of Hypertension in age group 5-15 year was 4.8 % and Pre hypertension was 11% respectively. The prevalence of Hypertension in children between 5-10 years was 4.1% and Pre hypertension was 4.8% . In 11-15 years age group it was 5.7% and 20.2% respectively.

Ethical clearance was obtained from 83

AGE

SYSTOLIC

DIASTOLIC

TOTAL

HT N 12

PREHTN HT N 26 18

PREHTN HTN

5-10

PREHT N 9

11-15

63

15

36

13

99

28

TOTAL

72

27

62

31

134(11)

58(4.8)

35

30


Vol. 25, No. 3, 4 July - Dec 2011

PREHTN: Prehypertension, HTN: Hypertension Numbers in parantheses indicate percentage Both systolic and Diastolic blood pressure showed an increasing trend with advance in age .

Increasing evidence indicates that essential hypertension begins to develop during the first two decades of life 10 . It is important to determine the prevalence of prehypertension and hypertension in children to identify the population at risk as early identification leads to early interventions thereby preventing later morbidity and mortality.

No statistically significant difference was observed between male and female children.

The prevalence of hypertension have been reported to have a wide range (1%16.2%) among various studies 5-7 .In the present study , the prevalence of Hypertension was 4.8% and Pre hypertension was 11% between 5-15 years.

Family history of hypertension was present in only 4 children . The prevalence of overweight and obesity between 5-15 years was 11.8% and 4.4% respectively. In children between 5-15 years with hypertension, 23 children ( 11.9%) were either obese or overweight . Prevalence of obesity was 20% and 7.9% in children between 5-10 years and 11-15 years with either Pre Hypertension or Hypertension.

The prevalence of Hypertension in the present study was high in the age group of 11-15 years. The elevation of blood pressure in adolescence has been noted by other studies also 1,5, although the exact reason for the same are not yet clear. Various Postulates put forward include biological maturation, body mass and hormonal changes 5.

In children who were either obese or overweight, prevalence of hypertension was 11.7%

In the present study, trend of increasing blood pressure with advance in age is similar to other studies 11.

TABLE III : PREVALENCE OF PREHYPERTENSION AND HYPERTENSION AMONG OVERWEIGHT AND OBESE CHILDREN Blood pressure

NORMAL

851

PREHYPER TENSION AND HYPERTEN SION 169

OVERWEIGHT AND OBESE

174

23(11.7)

197

TOTAL

1025

192

1217(100)

BMI

NOR MAL

The relationship between Hypertension and Obesity in childhood has been noted though less extensively evaluated. Hypertension in obese children may occur due to increased cardiac output, excessive sodium intake, steroid production and alteration in reception for various pressor substances 1.

TOTAL

1020

The prevalence of overweight and obesity in our study was 11% and 4.4%, respectively in concordance with other school based data in India, which demonstrated prevalence of obesity in the range of 5.6% to 24% among children and adolescents 12-15.

Numbers in parantheses indicate percentages DISCUSSION Hypertension is a major risk factor for cardiovascular and cerebrovascular diseases. 84


Vol. 25, No. 3, 4 July - Dec 2011

In our study Prevalence of Pre hypertension and Hypertension together in children either overweight or obesity was 11.7%. Gupta and Ahmad 6 have reported prevalence of sustained HTN in obese to be 20 times more as compared to control. Prevalence of Hypertension in study done by Bishav et al 1 showed 15.33% in overweight and 43.10% in obese group in urban population. Various other studies have reported the prevalence of obesity in asymptomatic hypertension as high as 50 65% 4,16 . In our study the prevalence of obesity was 20% and 7.9% in children between 5-10 years and 11-15 years with either Prehypertension or Hypertension. An interesting fact brought out in this study is a low prevalence of obesity among hypertensives and low prevalence of hypertension among obese and overweight children thereby warranting need of regular monitoring of blood pressure irrespective of their BMI status.

necessary in order to document persistent deviations, Further studies with larger samples are needed to confirm the epidemiological consistency of the observations made and for planning interventions. Efforts must be made to recognize hypertension and prehypertension in asymptomatic children. Early identification of at risk children and appropriate lifestyle modification would help in preventing hypertension and its related cardiovascular and cerebrovascular complications. ACKNOWLEDGEMENTS; The authors gratefully acknowledge the assistance provided by co investigators Dr. Sudarshan Murthy K.A, Professor and Head, Department of Medicine, JSS Medical college, Dr.Renuka, Associate Professor, Department of Community Medicine , Senior research fellows: Dr.Ravikumar, Dr.Purushottam.V, Dr.Purushottam.D.R and medical social workers : Smt Sharada ,Smt Prakruti and Ms Rekha and CFTRI for providing Annapoorna software for nutritional assessment.

A statistically significant correlation was not noted between Hypertension/ Prehypertension and family history in contrast to other studies5,6,17 .

Funding: ICMR References

The prevalence of Hypertension and Prehypertension in the present study was high which could be due to Point Hypertension in concordance with other studies6,7,11. This study was done as a part of tracking study on hypertension. In the Muscatine study enrolling 6622 children 18, 13 % had hypertension when first examined but on repeated measurements less than 1% had blood pressure in hypertension range and similar observations have been made by other researchers also6,16,19. Hence Serial determinations of blood pressure are

1. Mohan B, Kumar N, Aslam N, Rangbulla A, Kumb Karni S, Sood NK et

al.

Prevalence

of

sustained

hypertension and obesity in Urban and Rural school going children in Ludhiana. Indian Heart J 2004:56: 310-314. 2. Fall CHD, Barker DJP. The fetal origins of coronary heart disease and noninsulin dependent diabetes in India. Indian Pediatr; 1997:34:5-8. 85


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3. Vedavati S, Jayashree R, Rafi M. Prevalence of overweight and obesity in affluent adolescent Girls in Chennai in 1981 and 1998. Indian Pediatr 2003; 40: 332-336.

physical activity Indian Pediatr 2004; 41: 545-550. 13. Bhave S, Bavdekar A, Otiv M. IAP National Task Force for childhood prevention

4. Kannel WB. Role of blood pressure in cardiovascular disease The Framingham study. Angiology;1975; 26: 1-14.

of

adult

diseases:

Childhood Obesity. Indian Pediatr 2004; 41: 559-575. 14. Kapil U, Singh P, Pathak P, Dwivedi S,

5. Verma M. Chhatwal J, George SM. Obesity and Hypertension in children. Indian Pediatr. 1994; 31:1065-1069.

Bhasin S. Prevalence of obesity amongst affluent adolescent school children in Delhi. Indian Pediatr 2002:

6. Gupta AK, Ahmad AJ. Normal blood pressure and the evaluation of sustained Blood pressure deviation in childhood. Indian Pediatr. 1990:27:3342.

39: 449-552. 15. Ramachandran A, Snehalatha C, Vinitha R, Thayyil.M, Sathishkumar CK, Shubha C, et al. Prevalence of

7. Sachdev Y. Normal BP and HTN in Indian children: Indian Pediatr. 1984:21:41-48.

overweight in urban Indian adolescent

8. Khadlikar VV, Khadlikar AV, Choudhury P, Agarwal KN, Ugra D, Shah NK. IAP growth monitoring guidelines for children from birth to 18 years. Indian pediatr.2007; 44;187-196.

16. Anand NK. Tandon L. Prevalence of HTN in school going children. Indian

9. Bagga A, Jain R, Vijayakumar M, Kanitkar M , Ali U. Evaluation and management of hypertension. Indian pediatr.2007;44;103-121.

blood pressure level to school

school children. Diabetes Res Clin Pract 2002; 57: 185-190.

Pediatr 1996; 33: 377-381. 17.

Gupta AK. Influence of family history of morbid cardiovascular events on children. Indian Pediatr 1991; 28:131139.

18. Rames LK, Clarke WR, Connor WE,

10. The fourth report on the diagnosis, evaluation and treatment of high BP in children and adolescents. Pediatrics 2004:114(2):555-575.

Reiter MA, Lauer RM. Normal blood pressure and evaluation of sustained blood pressure elevation in childhood: the Muscatine study. Pediatrics,

11. Chadha SL, Tandon R., Shekhawat S, Gopinath N. An epidemiological study of blood pressure in school children (5-14 year) in Delhi. Indian Heart J 1999;51: 178-182.

1978:61:245-251. 19. Sorof JM, Lai D, Turner J, Poffenbarger T, Portman RJ. Overweight, ethnicity and the prevalence of hypertension in school aged children. Pediatrics

12. Greydanus DE, Bhave S. Obesity and adolescents; Time for increased

2004:113(3):475-482. 86


Vol. 25, No. 3, 4 July - Dec 2011

OMENTAL INFARCT- A RARE CAUSE ACUTE ABDOMEN *Dr.Guruprasad Shetty*,Aby Dany*, Sanjeev Rai B*, Habeeb Khan*, P.C. Das

Abstract: Omental infarction is a rare cause of acute abdominal pain in the paediatric population, being more common in adults. There were hardly 160 cases reported in the medical literature, in that more than 85% of reported cases in adults1,2. We are reporting a case of 16 year old boy presented with acute abdominal pain. Ultrasound abdomen showed hyperechoeic lesion in the right hypochondrium suggestive of omental infarct. Child was operated successfully and diagnosis was confirmed by histopathology.

On examination, child was on agony. Vital parameters were stable. Abdominal examination showed tenderness in the right hypochondrium with no hepatomegaly. Blood investigation showed normal haemoglobin, total leucocyte count and differential count. ESR (26mm/h) was elevated. Liver function and renal function tests were normal. An abdominal ultrasound (US) examination showed a hyperechoic, triangular shaped lesion located in the right hypochondrium, lying just posterior to the abdominal wall and in the vivinity of the ascending colon suggestive of omental infarct. Since there were no precipitating factors for it, a diagnosis of idiopathic segmental omental infarction was made. Child was operated by laprotomy and 5 × 7 cm infarcted omentum was excised. Diagnosis was confirmed by histopathological examination.

Keywords: omental infarction, acute abdomen, ultra sound Introduction Omental infarction (OI) is a rare cause of acute abdominal pain in the paediatric population this condition was first described in 1896 by Bush 2. Low incidence and non specific presentation contribute to OI being misdiagnosed for appendicitis, peptic ulcer disease, cholecystitis, and pancreatitis. Approximately, 0.1% of children undergo laparotomy for suspected appendicitis that is diagnosed later surgically as omental infarction 1 ,2. Here we are reporting a case of a 15 year old boy who presented with acute abdominal pain, diagnosed as primary idiopathic omental infarct and treated surgically.

Fig 1- US shoeing hyperechoeic lesion right hypochondriac region

Case report A 15 year-old male patient Consulted our hospital with 3 days history of persisting abdominal pain. The Pain was dull aching type which was non radiating, located mainly in the right upper quadrant.

Fig2- excised infracted segment of greater omentum measuring about 5× 7cm

*Dept of Pediatrics, Pediatric surgeon, Fr Muller Medical College, Mangalore

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Discussion

and efficient procedure, allowing complete view of the abdominal cavity and facilitating a correct diagnosis and surgical intervention. Same time surgery potentially hastens symptom resolution and enables patient discharge much sooner post operation. Symptoms can otherwise persist for an average of 13.5 days through conservative management. As yet, no comparative study demonstrates a significant difference in outcome following surgery and conservative management2, 3.

Omental infarction is rare in the paediatric population, there were hardly 160 cases reported in the medical literature with less than 15% of cases occurring in children. Omental infarction is reportedly more common in men, with a male to female ratio of 2:1 1,4 . Children with omental infarction typically present with sudden onset of right-sided abdominal pain and tenderness. Although the pathogenesis of omental infarction is still unknown, theories about the causes of omental infarction include anomalous arterial supply to the omentum, kinking of the veins associated with increased intra-abdominal pressure, or vascular congestion after large meals. Obesity is a well-known predisposing factor. Most of the paediatric series have reported omental infarction to occur in obese children1,2,3.

Conclusion Omental infarction is a rare in children and should be considered in the differential diagnosis of right sided abdominal pain. The diagnosis may be confirmed by US. In cases of doubt, a CT scan of the abdomen can help in the differential diagnosis. The characteristic imaging features of omental infarction are important in making the diagnosis preoperatively and in distinguishing omental infarction from acute appendicitis

In most patients segmental omental infarction is a self limiting, benign condition that may resolve spontaneously. The imaging features are characteristic and identical to those described in adults. Omental infarction is typically triangular and mostly involves the inferior aspect of the right side of the omentum. Ultrasound (US) examination is diagnostic and further confirmation can be done by computer tomography (CT) abdomen1,2.

References

There is, at present, no authoritative course of action for managing omental infarction. If accurate recognition of omental infarction is made with CT imaging without exploratory surgery, child can be managed conservatively. This approach utilises analgesics and anti-inflammatory medication with optimal fluid management in the first instance. With a doubtful preoperative diagnosis of acute right abdominal pain, laparoscopic exploration is a convenient 88

1.

Van Kerkhove et al, omental infarction in children JBRBTR, 2006, 89: 198-200

2.

Barai. K. P. et al, Diagnosis and management of idiopathic omental infarction: A case report, International Journal of Surgery Case Reports 2 (2011) 138 140

3.

C. L. Ho et al, Idiopathic Segmental Infarction of Right Sided Greater Omentum. Case report and review of the literature, Acta chir belg, 2004, 104, 459-461

4.

W. Lee et al, Omental infarction in children: imaging features with pathological correlation Singapore Med J 2005 46(7), 328-332


Vol. 25, No. 3, 4 July - Dec 2011

KLIPPEL FEIL SYNDROME: A CASE REPORT *Dr.Harsha M ,Sharan Gowda Patil, Shrikant SW, Gachinmani N. ABSTRACT:

The childs complete hemogram revealed microcytic hypochromic anemia. Neck x-ray AP and lateral showed congenital fusion of cervical vertebraes with spina bifida. Renal scan and Echo were normal. Karyotyping was normal. A diagnosis of Klippel-Feil syndrome was made.

A 4 month old girl born to nonconsanguineous parents presented with clinical features of short neck and reduced movements of neck. On examination she had low hair line. Neck x-ray revealed congenital fusion of cervical vertebraes with spina bifida. Hence a clinical diagnosis of Klippel-Feil syndrome was considered.

DISCUSSION: In this malformation sequence, the cervical vertebrae are usually fused, although hemi vertebrae and other defects may also be found. It may be a part of a serious problem in early neural tube development.

Key words: Short neck; reduced movement of neck, low hair line, fusion of vertebraes. Klippel-Feil syndrome is one of the rare causes of short neck and limitation of neck movement, first reported by Klippel and Feil in 1912 with a frequency of 1: 42,000 births and 65% of patients being female 1, 2 . We report a 4 month old girl with Klippel-Feil syndrome.

A strong association exists between mirror movements and cerviocomedullary neruoschisis3. The following defects have occurred in a non-random association in patients with Klippel-Feil sequence: deafness either conductive or neural is noted in as many as 30%4, ventricular septal defects 5, mental deficiency, cleft palate, rib defects Sprengel anomaly, posterior fossa dermoid cysts, scoliosis, renal abnormalities (unilateral absence of the kidney or hydronephrosis or both, ureteropelvic obstruction) and absent vagina 6 . The characteristic physical findings include a low hair line, short webbed neck, torticollis and decreased cervical motion.The characteristic x-ray neck (AP and Lat) flexion-extension shows congenital fusion of two or more vertebrae (failure of segmentation) 7.

CASE REPORT: A 4 month old girl child was brought to our OPD with complaints of short neck and reduced movements of neck since birth. On examination she had short neck with webbing, low posterior hair line, pilonidal dimple and tuft of hair at lumbosacral region. Anthropometric parameters were within normal limits, neck length was 3cm and total length of patient was 55cm. Developmental milestones (Neck holding, social smile, turning head towards sound) were within normal limits. Systemic examination was found to be normal. The patient did not have bowel and bladder disturbances or deafness.

Those with hypermobility of the upper cervical segment are at a high risk of

* Department of Pediatrics, M.R. Medical College, Gulbarga, Karnataka, India

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developing neurological impairment and should avoid violent activities. Individuals with hypermobility of the lower cervical segment are at increased risk for degenerative disk disease and should be treated symptomatically 8. MRI may be required if neurological symptoms are present. Surgery may be required to decompress the neural elements and / or stabilize segments of cervical spine.

REFERENCES:

Radiographs of cervical spine demonstrating congenital fusion, spina bifida and spinal instability

1.

Klippel M, Feil A: Un Cas d absence des vertebras cervicales, avec cage thoracique remontant jusqua la base du Crane (Cage thoracique cervicale). Monv In Conogr Salpet 25: 223, 1912.

2.

Clarke et al. Clarke RA, Catalan G, Diwan AD et al. Heterogencity in Klippel-Feil syndrome: A new classification. Pediatr Radiol 1998; 28: 967-974.

3.

Roya SA et al: Investigations into the association between cervicomedullary neuroschisis and mirror movements in patients with Klippel-Feil syndrome. AJNR 2002; 23: 724.

4.

Palant DJ, Carter BL: Klippel-Feil syndrome and deafness. AM J Dis Child 1972; 123: 218.

5.

Morrison SG, Perry LW, Scott LP III: Congenital brevicollis (Klippel-Feil syndrome) and cardiovascular anomalies. Am J Dis Child 1968; 115: 614.

6.

Hensinger et al. Hensinger RN, Lang JE, MacEwen GD. Klippel-Feil syndrome : A constellation of associated anamolies. J Bone Joint Surg AM 1974; 56: 1246-1253.

7.

David A S, Harish S H, John P D and Denis S D: Bone and Joint Disorders;The Neck; Klippel-Feil Syndrome. Nelson Text book of pediatrics: 18th edition;2823-24

8.

Pizzutillo et al. Pizzutillo PD, Woods M, Nicholson L et al. Risk factors in Klippel-Feil síndrome. Spine 1994; 19: 2110-2116.

Figure showing Short neck and Low hair line, tuft of hair and Pilonidal Dimple at lumbosacral region

Karyotyping showing normal study.

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ARTHROGRYPOSIS MULTIPLEX CONGENITA * Dr.Niveditha R., Dr.Vikram Singhal, Dr.Murali Keshav, Dr.Rajesh SM, Dr.Kiran Baliga ABSTRACT:

The clinical examination revealed Term small for gestation age baby with microcephaly, deep set eyes, long eye lashes, low set ears, long philtrum, retrognathia, widely spaced nipple, flexion contracture of all the four limbs. Baby has specific postureHip flexed, abducted and externally rotated flexion at the knees and bilateral equinox varus deformity. Extension and pronation at elbow and fingers overlapping each other

Arthrogryposis is a congenital disorder characterized by multiple joint contractures which are present at birth. We present the case of newborn with arthrogryposis involving bilateral hip, knee and ankle joints with Osteum secondum Atrial Septal Defect. There is a possibility of genetic counselling for some of the arthrogryphosis conditions which render the very precise diagnosis necessity. KEY WORDS: Contractures

Figure :

Arthrogryposis,

INTRODUCTION Arthrogryposis Multiplex Congenita also called as amyloplasia is characterized by curved or hooked joints. It is a nonprogressive, rare congenital disorder that is characterized by multiple joint contractures and can include muscle weakness and fibrosis, which limits movement 1. It is a heterogeneous group of disorders. There are about 150 entities2. We present a 5 day old baby who presented to us with features of arthrogryposis.

There was also a left sided inguinal hernia, bilateral crptochrodism, and Osteum secondum Atrial Septal Defect. Based on the above clinical features diagnosis of Arthrogryposis Multiplex Congenita was considered .Karyotyping was 46XY. Serum Creatinine Phospho Kinase, transaminase levels done to rule out muscular dystrophy were normal. Difficulty in initiation of feeds and also maintenance of feeds was there. Child was on Ryles tube feeding for 14days. Oral stimulation and physiotherapy was given and the gradually changed over to pallada feeds and then discharged on direct breast feeds

CASE REPORT: A 5 day old male child born out of a second degree consanguineous marriage to a G2P1L1 mother, delivered by normal vaginal delivery with the birth weight of 1.75 kgs was referred to our hospital in view of congenital anomalies. Mother also gives a history of decreased fetal movements comparing to the previous pregnancy. Antenatal scans were normal. No family history of congenital anomaly.

DISCUSSION: Arthrogryposis Multiplex Congenita (AMC) is a rare congenital diseases

* Department of Pediatrics, Kasturba Medical College, Mangalore, Manipal University.

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Vol. 25, No. 3, 4 July - Dec 2011

characterised by contractures of all 4 limbs. AMC is seen in 1 out of every 3,000 live births with no sex predominance. Amyoplasia, the most common form (43%) is characterized by fatty and fibrous tissue replacement of the limb muscles .Risk in subsequent pregnancy is about 5%. AMC is typically symmetrical and involves all four extremities with some variation seen .Some of the more common signs and symptoms are associated with the shoulder (internal rotation), elbow (extension and pronation), wrist (volar and ulnar), hand (fingers in fixed flexion and thumb-in-palm), hip (flexed, abducted and externally rotated, often dislocated), knee (flexion) and foot (clubfoot).Complications may include scoliosis, pulmonary hypoplasia, respiratory problems, growth retardation, midfacial hemangioma, facial and jaw variations, and abdominal hernias. Cognition and language are usually normal .Different etiologies, clinical courses, prognosis genetics, and pathologic processes has been put forward2.

are lacking. Deformities are usually symmetric, and severity increases distally, with hands and feet typically being the most deformed. Associated with joint dislocation, especially the hips and, occasionally, the knees. Atrophy may be present, and muscles or muscle groups may be absent2. Sensation is usually intact, although deep tendon reflexes may be diminished or absent. In 2/3 of patients all four limbs are affected equally and in 1/3 of patients lower-limb deformities predominate, and only on rare occasions do the upper extremities predominate. Deformities tend to be more severe and more rigid distally.4 Management is mainly conservative and consist of gentle stretching ,light weight splinting, casting, soft tissue relearns, muscle transfer and osteotomy Outcomes better if joint surgery is done early, before adaptive intraarticular changes occur.Osteotomies are usually performed closer to the completion of growth. Early motion, and avoidance of prolonged casting, may increase joint motion, improving function. Many children require long-term bracing or other assistive devices. Many will have feeding problem. As a whole a holistic team approach should be there5.

The principal cause of AMC is believed to be decreased fetal movements (akinesia) caused by maternal or fetal abnormalities. Fetal abnormalities (neurogenic, muscle, or connective tissue abnormalities, mechanical limitations to movement like Oligohydramnios .Maternal disorders (infection, drugs, trauma, other maternal illnesses).1 30% of the cases genetic in nature and autosommal recessive in inheritance. Life span is usually related to the disease severity and associated malformations. Nearly 20% of patients die in the 1st yr of life. Scoliosis may compromise respiratory function. By age 5 yrs 85% ambulatory with normal mental development3.

No prenatal diagnostic tools are available to test for the condition. Muscle biopsies, blood tests and general clinical findings rule out other disorders like congenital muscular dystrophy, Spinal muscular dystrophy, and Myasthenia gravis and provide evidence for AMC. Conclusion: Arthrogryposis is a rare congenital disorder. There is no cure for arthrogryposis, but early vigorous physical therapy can help stretch out the contracted joints and develop the weak muscles. The life span for

Physical Examination is best for establishing a diagnosis. The limbs are featureless and tubular. Normal skin creases 92


Vol. 25, No. 3, 4 July - Dec 2011

an individual with arthrogryposis is usually normal, but may be altered by heart defects or central nervous system problems. Genetic counseling plays an important role in management of Arthrogryposis Multiplex Congenita. Reference: 1)

2)

Smiths recognizable patterns of human malformation, Kenneth Lyons Jones 17, 2005,576-579.

3)

A new arthrogryposis syndrome with facial and limb anomalies. Am J Dis Child 1975, 129:120-122.

4)

Arthrogryposis multiplex congenital: etiology, genetics, classification, diagnostic approach, and general aspects. J Pediatr Orthop B 6 (3): 15966.

5)

Lovell and Winter, Pediatric Orthopedics chapter 36 Page No.765-795. Robert M. Bernstein, Arthrogryposis and Amyoplasia. JAAOS 10(6):417424, 2002

DISHA Free Diabetes Clinic For poor and needy children with diabetes SAMATVAM - Jnana Sanjeevini Medical Center, Bangalore, India “Climb high, climb far; your goal is the sky, your aim a star.”

Mission: To provide comprehensive diabetes care and counseling to poor and needy children with insulin dependent [type 1] diabetes. To inspire, motivate and help them achieve and maintain highest health, happiness, well being, productivity and prosperity. Benefits: FREE insulin FREE syringes FREE blood glucose meters and strips FREE laboratory / diagnostic tests Health education and counseling Psychosocial support Patient – parent support groups Residential health – recreation camps Adopt a child and Scholarships Facilitation of school and college education Employment placement Contact: 2, 1st A Cross, Marenahalli, J P Nagar, Phase 2, Bangalore 560078, India Phone: 080 26403040, 26493060, Email: [email protected]; samatvam@gmail,com, 24 hour diabetes helplines 9448350728, 9448356575 Attn: Ms Prathima Krishnamurthy, Mr Belavady Prasanna, Ms Uma Dayashankar, Ms Usha Rangaraj Web: dishastars.org; jsindia.org; samatvamdiabetes.com Directors: Dr SS Srikanta, Dr A Sharda, Dr Tejeswini Deepak 93


Vol. 25, No. 3, 4 July - Dec 2011

PREVALENCE OF ANEMIA, MORBIDITY AND EXAMINATION PERFORMANCE AMONG LOWER PRIMARY SCHOOL CHILDREN OF DAVANGERE CITY *Sarvamangala K Koujalgi M.B. Manjunath T.P. ABSTRACT

along with mid- day school meals.Health education to the parents and school teachers.

Anemia is estimated to affect one half of school age children in developing countries such as India. Anemia, not only causes physical ill health, but also is known to cause cognitive delays and poor achievement in academic examination. In this regard a study was taken up with the following objectives . To assess the prevalence and severity of anemia, and morbidity among anemic lower primary school children and To know exam performance in relation to anemia and morbidity. Cross sectional study on Lower Primary School children (6-11yrs) of Davangere city was carried out from December 2007 to March 2008. Results:1925 Lower Primary School children (6-10 years) were studied for anemia. Anemia prevalence was high i.e.58.6%, it was more in girls (64.7%) than in boys (52.4%) and significantly decreased with age in both sex. 25.1% were mildly anemia, 20.5% moderately anemic and (12.9%) severely anemia. Significant difference in morbidity rates, and exam performance between different grades of anemia were noted.Conclusion: In the light of results to reduce prevelence of anemia and its effect, the authors conclude the following recommendations for school health services. Testing for Hemoglobin levels to identify anemic school children at admission and once in six months.Periodic deworming and supply of paediatrics IFA tablets at school

INTRODUCTION Nutritional anemia is a major world wide public health problem.1 Iron deficiency is the most common cause of nutritional anemia in the world.2 Iron deficiency anemia is a major nutritional problem in India and is widely prevalent among special groups in the community such as under fives, women in reproductive age and elderly people. 3 These special groups are given much importance in the draft of the services under appropriate National Health Programmes. Nutritional anemia is also common in school children of 5-15 years where less importance is given, during the implementation of National Health Programmes which cover this age group for services. This fact has been very well highlighted in the surveys such as NFHS, ICMR etc. During school years a child has to develop physical, motor and mental abilities to cope up for adolescent and adult life. Iron Deficiency Anemia impairs these abilities which are required for the learning achievements for example examination performance. 4,5 Various nutritional deficiencies and parasitic infections in children can also cause IDA .6 Studies by various authors have been reported on prevalence of anemia and its consequences among school children.7,8

* Assistant Professor Community Medicine*, Professor, Dept of Paediatrics **, Professor Dept of Community medicine *** : J.J.M. Medical College, Davangere, Karnataka, India.

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Vol. 25, No. 3, 4 July - Dec 2011

To understand the phenomenon of anemia and its relation to learning achievements, morbidity patterns the current study titled Prevalence of Anemia, Morbidity and Examination performance among Lower Primary School children of Davangere city had been undertaken.

assess the prevalence and severity of anaemia. During pilot study school children who suffered from mental retardation, or any other serious systemic diseases were excluded from the current study. Strata II : Assessment of morbidity and collection of Annua l exam marks cards and class attendance of previous 3 months to assess academic performance of sampled anemic and normal children.

OBJECTIVES 1. To assess the prevalence and severity of anemia, and morbidity among anemic lower primary school children of Davangere city.

RESULTS AND DISCUSSION Population coverage :

2. To know exam performance in relation to anemia and morbidity.

1925 Lower Primary School children drawn from 10 schools of Davangere city were assessed for anemia.,morbidityand examination performance after compiling and tabulating the data.

MATERIALS AND METHOD The current study was undertaken in Davangere city of Karnataka state.,Total population of LPS children was 19,249, Stratified, Simple random sampling is used,

Table No.1: Distribution of lower primary school children by Age and Sex

Record based information was collected regarding age of the child, parents occupation, ducation and income. Morbidity of the children were assessed by oral questionnaires to the children regarding symptoms of illness and .morbidty rates were calculated per child according to anemic status. Height and weight measurement and Hb% estimation by Sahlis method. Hemoglobin level was used to assess the anemia and severity based on cut off values by WHO.1 Child was considered anaemic if Hb% < 12 gm% which is specific for age of child.Mild: Hb levels 10 g/dl but below the cut-off level.Moderate: 7 and 10 g/dl.Severe: < 7 g/dl. The collected information was compiled, tabulated and analysed for results by using SPSS software package

Age in Years

Boys

Girls

Total

No

%

No

%

No

%

6

175

42.8

233

57.1

408

21.2

7

190

48.5

201

51.4

391

20.3

8

205

53.8

176

46.1

381

19.8

9

204

51

196

49

400

20.8

10

194

56.2

151

43.7

345

17.9

Total

968

50.3

957

49.7

1925

100

Out of 1925 students studied 968 (50.3%) were boys, 957 (49.7%) were girls. Age of our students ranged from 6 to 10 yrs 408 (21.2%); 391 (20.3%); 381 (19.8%); 400 (20.8%); and 345 (17.9%) were of

The study was conducted in two stratas Strata I Cross sectional study to

6, 7, 8, 9 and 10 years respectively. 95


Vol. 25, No. 3, 4 July - Dec 2011

Prevalence of anemia :

It was observed there was a gradual increase in the morbidity rate from 4 per

Table No.2 : Prevalence of anemia : No. of children examined 1925

Anemic children

Prevalence

1128

58.6%

child in the mildly anemic to 6.6 among the moderately and to 9 per child among the severely anemic children. ANEMIA AND EXAMINATION

Out of 1925 children examined, 1128 were anemic at the time of study. Thus the prevalence of anemia was 58.6%. Studies conducted in different parts of India have shown prevalence ranging from 51.5-81.8%

(ACADEMIC) PERFORMANCE : Table No. 5: Distribution of Normal and Anemic Lower Primary School children according to their examination performance (clubbed grades)

9 10 11

Table No.3 Distribution of anemia among LPS children by age and sex Age in years

Girls No. of children examined

Anemic children No. %

Boys No. of children examined

Anemic children No. %

6 7 8 9 10 Total

233 201 176 196 151 957

211 145 110 90 64 620

175 190 205 204 194 968

155 130 115 70 38 508

90.5 72 62 46 42 64.7

Normal Anemic Total

A+ and A 673 (84.4%) 13 (3.2%) 686

B+, B, C, D 124 (15.5%) 287 (71.7%) 401

Total 797 400 1197

2= 627.31 df = 1 p < 0.001 HS

88 68 56 34 20 52.4

Key for grades as per BEO Guidelines :

A+ : 90 – 100 B+ : 60 – 74 C+ : 30 – 49 D : Detained

2= 136.802 df =4 p < 0.001 HS 2 = 223.002 df = 4 p < 0.001 HS

A : 75 – 89 B : 50 – 59 C : < 30

Table No 5 shows Effects of Anemia

By studying the prevalence of anemia by age and sex, it was found that

on exam performance among LPS children.

prevalence of anemia was higher in girls (64.7%) than in boys (52.4%) in all age

between Normal and Anemic children is

The difference in Exam performance

groups. The prevalence also decreased

statistically highly significant.

steadily with age in both sexes. The

Details of table No.5 (Normal and Anemic LPS children with their grades) :

decrease in the prevalence by age was significant both in boys (p value 0.001) and

A [A+, A] Normal 673 (84.4) Mild 11 (6.8) Moderate 2 (1.1)

girls (p value < 0.001 HS). Table No.4 Morbidity rate per child Status of Anemia

Rate / child

Mild Moderate Severe

4 6.6 9

Min No. of symptoms 3 6 8

Maximum No. symptoms 5 7 10

96

Severe

0 (0)

Total

686

B [B+, B] 115 (14.4) 109 (68) 67 (37.2)

C

D

6(0.75)

3 797 (0.37) 8 (5) 160 7 (3.8) 180

32 (20) 104 (57.7) 10 (16.6) 17 (28.3) 33 (55) 301 159 51

Total

60 1197


Vol. 25, No. 3, 4 July - Dec 2011

Associations with interpretations were calculated separately for the grades

of all children and iron and folic acid supplementation to anaemic children .Thereby it is observed that for the improvement of health status and examination performance which is one of the measures of cognitive development of Lower Primary School Children, the following recommendations can be considered to be implemented in school health services.

Obtained by LPS children in their exam performances as follows:

2 = 466.307

Grades A&B

df = 1

p < 0.001

2 = 63.62

Grades B&C

df = 1

p < 0.001 HS

Grades B &combined C + D

2 = 77.42 Grades C& D

df = 1 p < 0.001

Short term :

2 = 0.16042

df = 1

1) To distribute iron and folic acid tablets, (small) along with mid day school meal programmes to all anemic Lower Primary School Children.

p > 0.05 NS

Anemic children differed in their exam performance from the normal children.It was observed that to score higher grades from C to A+ heamoglobin levele should be normal We have not come across studies on anemia and exam performance among LPS children using similar parameters to compare our study. Various studies on anemia and cognitive development in school children have been reported. One such study by Batra J, Sood A has shown that IDA has negative effects on cognitive development and impairs learning process among children.12.

2) Immediate care to routine morbidities of Lower Primary School Children, for which school teachers must be trained . 3) Immediate referral of the needy morbid Lower Primary School Child to the pediatrician should be made by the school teachers. For this the pediatricians along with Urban MOH can be attached to LPS depending upon the availability of pediatricians and appropriate renumeration can be given if pediatrician is a private consultant.

DISCUSSION The present study has identified a high prevalence of anemia among lower primary school children. Anemia has increased morbidity among the children and has shown to affect negatively the academic performance in significant proportions.

Long term recommendation : 1) Knowledge regarding healthy life style and healthy food consumption including greeen leafy vegetables and other iron rich foods should be encouraged and inculcated to the school children through curriculum and teachers should also be trained.

Effective school health checkups for early diagnosis should be made compulsory and interventions in the form of health education to school authorities, parents and school children regarding anemia, nutrition and timely treatment by periodic deworming

2) Necessary measures to be taken to educate the public (mainly mothers and children) to build healthy future generation by creating awareness to combat anemia by 97


Vol. 25, No. 3, 4 July - Dec 2011

modifying the food at home for both anemic and normal children.

1996. 6. Stoltzfus J, Mchwaya H, James MT,

The study limitations were

Kerry J, Albonico M, Saviolirenzo. Epidemiology of Iron deficiency Anemia in Zanzibari school children.

1) Other nutrients such as Iodine which affects on morbidity, and exam performance were not being considered either clinically or biochemically.

The importance of hookworms. Am J Clin Nutr 1997;65:153-159.

2) Social factors such as private and extra tuitions, and special influences while evaluating the children for exam performance could not be analyzed due to lack of information.

7. Satyanarayana

K,

Pradhan

RD,

Ramnath T, Rao NP. Anemia and physical fitness of school children of rural Hyderabad. Indian Pediatr 1990;27:715-721.

3) The information collected in the current study was analyzed by methods of descriptive studies. Analytical studies such as case-control studies could have been established.

8. Agarwal DK, Upadhyay SK, Agarwal KN, Singh RD, Tripati AM. Anemia and mental functions in rural primary school children. Annals Tropical Pediatrics Dec 1989;4:194-198.

REFERENCES

9. Verma M, Chhatwal J, Kaur G.

1. Demaeyer EM. Preventing and Controlling Iron Deficiency Anemia

Prevalence of anemia among urban school children of Punjab. Indian

through Primary Health care. Geneva,

Pediatr 1998;35:1181-1186.

World Health Organization 1989.

10. Sethi V, Goindi G, Kapil V. Prevalence

2. Benoist B, Mc Lean E, Egli I, Cogswell

of anemia amongst primary school age

M. Worldwide prevalence of Anaemia1993-2005. WHO Global

children (6-11 yrs) in National capital territory of Delhi. Indian J Pediatr

Database on Anemia. World Health

2003;70(6):519.

Organization Geneva,2008;1-4.

11. Verma

3. Park K. Park Textbook of Preventive

A,

Rawal

VS.

Factors

influencing anaemia among girls of

and Social medicine. 20th edn. Jabalpur M/s Banarsidas Bhanot Publisher

school going age (6-18 yrs) from slums of Ahmedabad City. Indian J Comm

2009;556-557.

Med, Jan-March 2004;XXIX(1):25-26.

4. WHO Promoting Health through

12. Batra J, Sood A. Iron deficiency

Schools.Technical Report Series.870:

anemia.

Geneva;1997;68-69.

Effect

on

cognitive

development in children : A review.

5. Draper A. Child development and Iron

Indian J Clin Biochem 2005;20(2):119-

deficiency. The Oxford Brief. Reports of meeting in Oxford University. Sep

125. 98


Vol. 25, No. 3, 4 July - Dec 2011

PARA-ESOPHAGEAL HERNIA PRESENTING WITH SEVERE ANAEMIA AND PNEUMONIA *Ravichandra G, Faheem M*, Raghavendra B, Devdas A, Habeeb R, Reshad M. Abstract We report a one and half year old male child who presented with right lower lobe pneumonia with history of recurrent cough and expectoration. Blood investigation revealed severe anaemia. On further evaluation with ultra sonography of chest and computed tomography revealed sliding para-esophageal hernia, gastric volvulus and right lower lobe pneumonia. The anaemia was the result of Cameron lesions associated with diaphragmatic hernia and pneumonia was a result of recurrent aspiration.

History of similar complaints present in the past, on and off since one month of age for which he has taken symptomatic treatment from a local doctor. There was no history dyspnoea, vomiting, abdominal pain, constipation or bleeding from any site. The child was immunised properly up to 9months. The diet of the child was adequate; he weighed 9kgs with height of 75cms On admission the child was febrile with mild cough. On examination severe pallor was present, pulse and respiratory rate was normal. There was no icterus, clubbing or petechiae. On chest examination, percussion revealed decreased resonance in the right inframammary, right axillary and left inframammary areas. Auscultation revealed decreased breath sounds in these areas. Abdominal examination revealed no organomegaly. CVS and CNS examination were normal. Investigations revealed haemoglobin of 2.3 g/dl, PCV of 9.9%, total leukocyte count of 6,600/cu mm and platelet count of 2.3 lac/cu mm. Peripheral smear reported severe degree of microcytic hypochromic anaemia which was suggestive of iron-deficiency anaemia. Liver function and renal function tests were normal.

Introduction Congenital diaphragmatic hernia (CDH) is a common, life threatening congenital anomaly with an incidence ranging between 1 in 2000 and 1 in 5000 live birth [1]. Sliding para-esophageal hernia with gastric volvulus is rarely seen in children. Potential complication of the diaphragmatic hernia includes pneumonia and anaemia due to gastrointestinal bleeding [2]. Sliding para-esophageal hernia with gastric volvulus resulting in iron deficiency anaemia and associated chest infection is very rare in children. Here, we report a one and half year old boy who presented with this condition and was managed successfully.

Chest X-ray showed a nonhomogenous opacity in the right mid and lower zone, and left lower zone. Rest of the lung parenchyma appears normal. USG was

Case report A one and half year old child presented with history of fever which was intermittent and low grade with cough.

* Dept of Radiology,* Sr.Resident Dept of Paediatrics Yenepoya Medical College, Mangalore - Correspond to Ravichandra Dept of Radiology,Yenepoya Medical College,Mangalore 575018

99


Vol. 25, No. 3, 4 July - Dec 2011

performed to assess the consolidation and pleural effusion; it revealed consolidation and fluid filled cystic areas in the posterior costo-phrenic angle on the right side. Hence, CT was suggested. CT chest was done before and after giving IV contrast, in addition oral contrast was given to opacify the bowel loops which revealed Consolidation of the right lower lobe with air bronchogram with patchy consolidation at right middle lobe [Fig 1(a&b)] with minimal right pleural effusion Suggestive of pneumonic consolidation and Large sliding para-esophageal hernia with herniation of the distal stomach into the right lower thoracic cavity with stomach showing upside down configuration - Suggestive of gastric volvulus

Discussion Para-esophageal hernias constitute approximately five present of all diaphragmatic hernias with a female predominance (M: F 1:4) (3). Vast majority are believed to be acquired than truly congenital. They involve the greater curvature of the stomach or very rarely the whole of stomach ascends into a performed sac within the mediastinum. Most congenital para-esophageal hiatus hernia occurs sporadically but there are about 21 case reports of familial para-esophageal hernias (4). These patients may have associated lung hypoplasia. Patients with Marfan syndrome and connective tissue disorders has higher predisposition to develop PEHH (5) Patients may be asymptomatic or present with recurrent respiratory infections or vague GI symptoms resulting in misleading clinical and radiological assessment with frequent delay in diagnosis. Hence correct diagnosis requires high index of suspicion. 100

Congenital or acquired para-esophageal hernias are associated with potentially lethal complication like gastric volvulus, incarceration and perforation (6). Diaphragmatic hernia with gastric volvulus in children is a rare subtype with only few cases reported in literature. A review of the literature revealed one such study in which the authors described three children, all of whom had an acute presentation and had to be operated on an emergency basis (7). Late presentation of diaphragmatic hernia as anaemia has been described in adults (8).The cause of anaemia has been attributed to Cameron lesions which are linear gastric ulcers or erosions on the mucosal folds at the diaphragmatic impression in patients with a large hiatus hernia(9, 10). These gastric erosions can cause iron deficiency anaemia from chronic blood loss. Gastric volvulus may be idiopathic or secondary to various congenital or acquired conditions. Among the associated problems, diaphragmatic defects predominate (11). The presentation can be acute, chronic, acute-onchronic or intermittent in type. The clinical symptoms depend on the degree of rotation and obstruction. Severe epigastric pain and distension, violent unproductive retching and inability to pass a nasogastric tube comprise the classical triad of Burckhardt (7). Intermittent type of gastric volvulus may cause diverse gastrointestinal symptoms in children. Intermittent gastric volvulus causes symptoms intermittently. Routine investigations done in the asymptomatic period may not reveal any abnormality and hence diagnosis may be missed. Treatment of Cameron lesions is primarily medical and surgery is reserved for refractory cases and a few complicated


Vol. 25, No. 3, 4 July - Dec 2011

cases. Surgical treatment (fundoplication, laparoscopic or open) is recommended in patients with medically refractory disease, uncontrolled bleeding from the lesions and in patients in whom the hernia is complicated with volvulus, incarceration and perforation (2) . With growing use of laparoscopic surgery, patients benefit from a minimally invasive approach as several authors have reported favourable outcomes after performing laparoscopic diaphragmatic hernia repairs and gastropexy (12, 13). REFERENCES 1) M. R. Langham, D. W. Kays, D. J. Ledbetter, B. Frentzen, L. L. Sanford, and D. S. Richards, Congenital diaphragmatic hernia: epidemiology and outcome, Clinics in Perinatology, vol. 23, no. 4, pp. 671688, 1996. 2) Maganty K, Smith RL. Cameron lesions: Unusual cause of gastrointestinal bleeding and anaemia. Digestion 2008; 77: 214-217. 3) Anderson KD, Congenital diaphragmatic hernia. In Welch KJ, Randolph HG, Ravitch MM, Rowe MC, eds. Pediatric Surger, 4 th edn. Chicago: Year Book Medical Publishers; 1986; 599 4) Baglag SM, Noblett HR. Paraesophageal hernia in Chidren: familial occurrence and review literature. Pediatric Surgery International 1999; 15 : 85-87. 5) Parida SK Kriss VM, Hall BD. Hiatus/ para-esophageal hernias in neonatal Marfans Syndrome. Am J Med Genet, 1997 Oct 17;72(2) : 156-158 6) Jawad AJ, AL-Samaarai AI, AIMofada, AI-Howasi M, Hawass NE, 101

AI-Beruit Z, Congenital paraesophageal hiatal hernia in infancy. Pediatric Surgery International 1998; 13(2-3) : 91-94 7) Karande TP, Oak SN, Karmarkar SJ, Kulkarni BK, Deshmukh SS. Gastric volvulus in childhood. J Postgrad Med 1997; 43: 46-47. 8) Pauwelyn KA, Verhamme M. Large hiatus hernia and iron deficiency anaemia: clinico-endoscopical findings. Acta Clin Belg 2005; 60: 166172. 9) Cameron AJ, Higgins JA. Linear gastric erosion. A lesion associated with large diaphragmatic hernia and chronic blood loss anemia. Gastroenterology 1986; 91: 338-342. 10) Moskovitz M, Fadden R, Min T, Jansma D, Gavaler J. Large hiatal hernias, anemia, and linear gastric erosion: studies of etiology and medical therapy. Am J Gastroenterol 1992; 87: 622-626. 11) Singal AK, Vignesh KG , Mathai J. Acute gastric volvulus secondary to eventration of the diaphragm in a child. J Indian Assoc Pediatr Surg 2006; 11: 44-46. 12) Naim HJ, Smith R, Gorecki PJ. Emergent laparoscopic reduction of acute gastric volvulus with anterior gastropexy. Surg Laparosc Endosc Percutan Tech 2003; 13: 389-391 13) Casaccia M, Torelli P, Troilo BM, Savelli A, Valente U. Composite mesh repair of a large paraoesophageal hiatus hernia. J Laparoendosc Adv Surg Tech A 2006; 16: 381-385.


Vol. 25, No. 3, 4 July - Dec 2011

PROFILE OF RESPIRATORY DISTRESS AMONG NICU ADMISSIONS IN SDM MEDICAL COLLEGE HOSPITAL *Dr Budensab A H. Background: Respiratory distress is one of the commonest cause of admission to neonatal unit. The causes can be multiple and outcome variable. Objective: To analyze the causes, associated conditions, course during hospital stay and outcome of newborns with respiratory distress. Methods: This retrospective was conducted during August 2009 to July 2010. All the newborns that developed respiratory distress within 24 hours of life are included. A total of 220 newborn were admitted with RDS out of 856 admissions (25.7%). Results: 25.7 % admissions were due to RDS, Male female ratio 2.5:1 Incidence according to birth weight Birth w t 3500

Num ber 3 29 87 92 9

% 0.13 13.18 39.54 41.81 0.40

Preterm babies 85 (38.63%) and full term babies 135 (61.36%) Causes of admissions were due to Diagnosis Preterm with RDS Full term with TTNB Full term with MAS Birth asphyxia Pneumonia and sepsis others

Number 85 51 25 22 18 19

% 38.63 23.18 11.36 10 8.18 8.63

Other causes being congenital diaphragmatic hernia-1, jejuna atresia - 1, trachea-esophageal fistula - 1, congenital heart disease 1 and trisomy 2 21 babies were ventilated (9.54%) * SDM Medical college Hospital Dharwad

102

Among the ventilated babies commonest causes being preterm with RDS - 9 (42.85%) followed by full term with MAS - 4 (19.04%), birth asphyxia 4 (19.04%) pneumonia and sepsis 2 (9.52%) others 2 (9.52%). Duration of ventilation 48 h ou rs

8 babies (38,08 % ) 4 babies (19.04 % )

21 babies ventilated out of which 8 died (38.08%), the overall mortality being 8 out of 220 (3.63). Discussion: our study of incidence, profile of RDS and mortality is comparable with other studies. Ref wide below. Conclusion: RDS due to prematurity was the commonest cause of respiratory distress requiring admission and ventilation Abbreviations: RDS=respiratory distress syndrome, TTNB= transient tachypnea of newborn, MAS=meconium aspiration syndrome REFERENCES:

1. Kumar A, Bhat B V Epidemiology of respiratory distress of newborns. Indian journal of pediatrics 1996 ;63-93-98. 2. Ashish jaswal, Srinivas murki mukri, Pramod gaddam and Anupama reddy Early neonatal morbidities in late preterm infants Indian pediatrics 2011 48 -607-611 3. Socioclinical analysis of sick neonates in pediatric department of MKCG Hospital Brahmapur Pedocon 2007 by L Patnaik, RM Tripathy, DS Malini and S Sahu 4. Zulfiqar Ahmed Bhutta and Kamran Yusuf Journal of tropical pediatrics 199743 -143-148 5. Study of newborn of mothers having meconium stained liquor Pedicon 2007 by Pankti V modi, Rekha Bhavsar, Archana Shah and Pankit Modi.


Vol. 25, No. 3, 4 July - Dec 2011

MULTIPLE SPLENIC ABSCESSES DUE TO AN UNSUSPECTED CAUSE- CASE REPORT *Dr.Narayanappa , Dr.N.Rashmi , Dr.Anil Kumar, Dr.Rajani.H.S ,Dr.Anitha.C Abstract Enteric fever complicated by splenic abscess is a rare entity in Pediatric age group. It is usually a part of disseminated infection. There can be single or multiple abscesses. Single abscess most commonly responds to conservative management with appropriate antibiotics and ultrasound guided percutaneous drainage of the abscess. However, multiple abscesses require splenectomy. The child presented here had multiple splenic abscesses as a complication of enteric fever. There was no response to conservative management and hence he had to undergo splenectomy. So, in children with enteric fever, it is strong suspicion which helps in identifying complications early and treating them accordingly. Salvaging the spleen is not possible in case of multiple splenic abscesses.

remains the mainstay of treatment with a success rate of 86-94% (1,5,6) . It is often fatal if left untreated. A 13 year old boy presented to the Pediatric emergency ward with history of continuous high grade fever and diffuse intermittent pain abdomen of eight days duration and nausea and loose stools of three days duration. There was no history of any other associated symptoms or significant past history. He was immunized to date according to the National Immunization Programme. On examination, he was conscious, oriented, pale, dehydrated and in shock. He was resuscitated with 20 ml/kg of intravenous Normal Saline and oxygen supplementation by mask. His blood pressure improved and he did not require inotropic support. His general examination also revealed pallor, with no icterus or edema or significant lymphadenopathy. He was poorly built and nourished. There was no rash or bleeding tendency. Hess test was negative. His abdominal examination revealed diffuse tenderness with mild hepatosplenomegaly, Other systems were normal. His investigations revealed a hemoglobin percentage of 9.9g/dl, total WBC count of 13,200/cmm, with neutrophilic predominance and absent eosinophils. Platelet count was 1.77 lakh/cmm and ESR of 15mm/1st hour. Peripheral blood smear showed microcytic hypochromic anaemia. Urea, creatinine and serum electrolytes were all within normal limits. Liver enzymes were

Key words: Enteric fever, splenic abscess, splenectomy. Introduction: Splenic abscess is rare, with lesser than 500 cases reported in literature (1). Enteric fever as a cause of this condition is even rarer (2). Only about 38 cases are described in literature since 1977, with majority being solitary and a few of them, multiple abscesses. The clinical diagnosis of splenic abscess due to typhoid fever is difficult because it is rare, has an insidious onset and nonspecific clinical features (3,4). It therefore requires a very high index of suspicion. Ultrasonography and computed tomography (CT scan) help in achieving early diagnosis. Splenectomy *Department: Pediatrics,

*

Pediatric Surgery. JSS Medical College and Hospital, JSS University, Mysore.

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mildly elevated with an AST of 214 U/L and ALT of 85 U/L and normal bilirubin level. Blood culture was sterile on two consecutive samples. Urine culture was sterile. Widal test did not show any significant titres or any significant rise in titres on subsequent samples. Dengue serology and QBC and peripheral smear for malarial parasite were negative. Ultrasound abdomen, however revealed multiple splenic abscesses/splenic infarctions. Child was started on intravenous Ceftriaxone initially, and following ultrasound, intravenous Linezolid and Metronidazole were added. Further investigations to look for the cause of splenic abscesses were done. Brucella test was negative by slide agglutination, sickling test was negative, HIV ELISA was non-reactive, and 2D ECHO did not show any evidence of infective endocarditis. So, ultrasound guided aspiration of the splenic abscess was done, following CT scan of the abdomen (Figure 1). The aspirate grew nalidixic acid resistant Salmonella typhi which was sensitive to ceftriaxone, chloramphenicol, ampicillin and tetracycline. So, Ceftriaxone was continued for 14 days. However, the child remained febrile, and pain abdomen persisted. Repeat CT abdomen showed persistence of the splenic abscesses. Since there was no response to conservative management, splenectomy was planned for. Child underwent splenectomy, following which he became afebrile on the second post-operative day. The splenectomized specimen revealed areas of abscess and infarcts (Figure 2). Ceftriaxone was continued for seven more days and the patient remained afebrile through out the rest of his hospital stay. He was discharged in a stable condition. 104

Fig 1a

Fig 1a

Fig 1 (a & b) :- NECT shows right lower lobe consolidation with air bronchogram with patchy consolidation at right middle lobe

Fig 2 :- NECT shows cystic lesion with air fluid level and hyper-dense layering in the right lower hemi-thorax

Fig 3 :- Reformed Image - upside down configuration of stomach

Fig 4a

Fig 4b

Fig 4 (a & b) :- CECT with oral contrast shows contrast filled stomach in the right lower hemi-thorax

Discussion: Splenic abscesses can occur due to systemic bacteraemia originating in another site, such as


Vol. 25, No. 3, 4 July - Dec 2011

endocarditis and intravenous drug abuse, superinfection of a spleen damaged by ischaemia or infarction (as in haemoglobinopathies and sickle cell anaemia), penetrating or blunt trauma with superinfection of a subcapsular haematoma, or other diseases (malaria, leukaemia and splenic cyst), immunodeficiency due to acquired immunodeficiency syndrome (AIDS), chemotherapy or long term steroid therapy, or from conditions that predispose to immunosuppresion such as diabetes mellitus and alcoholism and extension from a contiguous focus such as pancreatic, subphrenic or perinephric abscess (4). Staphylococcus aureus, streptococci, and various aerobic, and anaerobic intestinal flora and, rarely, fungi are important causes of splenic abscess. However, nontyphoidal salmonella are more frequently reported than Salmonella typhi (4). Another rare cause especially in the developing countries is tuberculosis, in which the splenic abscess may be isolated or associated with a disseminated disease (6) . Reports show that in upto 20% of patients with splenic abscess, the pus is sterile while the blood culture isolates the organisms in 40-50% of cases (4). A negative pus or blood culture probably results from the use of antibiotics, as was seen in our patient. Diagnosis of splenic abscess requires a very high index of suspicion with the presenting symptoms being nonspecific. The triad of fever, left upper quadrant pain, and a tender mass (suggested by Sarr and Zudeima) as an important presenting complex is seen in only about one-third of all patients (1). Ultrasound abdomen helps in the preliminary diagnosis. CT scan is most useful in establishing the diagnosis and demonstrating the number and location of abscesses, besides showing any other concomitant disease or involvement of other 105

organs. MRI gives some clues in the diagnosis by defining the extent and internal structure of splenic abscess because of its greater tissue resolution (7) . Single unilocular abscesses may respond well to percutaneous drainage. Multiple or loculated abscesses may respond to antibiotics alone but splenectomy is the preferred treatment. In general, failure to respond to antibiotics with or without percutaneous drainage requires splenectomy (8) . This fact was evident in our patient. REFERENCES 1. Philips GS, Radosevich MD, Lipsett PA. Splenic abscess another look at an old disease. Arch Surg 1997; 132: 1331-1336. 2. Shin PJ, Choi H, Bae CW et al. Percutaneous drainage of splenic abscess in typhoid fever a case report. J Kor Med Sc 1995; 10: 44-7. 3. Ng KK, Lee TY, Wan YL, Tan CF, Lui KW, Cheung YC, et al. Splenic abscess: Diagnosis and management. Hepatogastroenterology 2002; 49:567-571. 4. Liang JT, Lee PH, Wang SM, Chang KJ. Splenic abscess: A diagnostic pitfall in the ED. Am J Emer Med 1995; 13: 337-43. 5. Smyrniotis V, Kehagias D, Voros D et al. Splenic abscess an old disease with new interest. Dig Surg 2000; 17: 354-7. 6. Anuradha S, Singh NP, Agarwal SK. Splenic abscess a diversity within. J Indian Aca Clin Med 2000; 1: 279-81. 7. Elsayes KM, Narra VR, Mukundan G, Lewis JS, Menias CO, Heiken JP. MRI Imaging of the spleen: Spectrum of abnormalities. Radiographics 2005; 25: 967-982. 8. Mehta SS, Gittes GK. Splenic Abscess. In: Ashcraft KW, Holcomb GW, Murphy JP eds. Pediatric Surgery. 4th edn. Philadelphia: Elsevier Saunders; 2005; 652-653.


Vol. 25, No. 3, 4 July - Dec 2011

VINCRISTINE INDUCED UNILATERAL PTOSIS *Dr. Guruprasada Shetty, Dr. K. Shreedhara Avabratha, Dr B Sanjeev Rai , Dr. Seema Dr Aby Dany and Dr. Dinesh Shet Abstract Vincristine is a vinca alkaloid used in combination with other agents in the treatment of solid tumors, lymphoma, and leukemia, as well as for idiopathic thrombocytopenic purpura and autoimmune haemolytic anemia. A dose-limiting complication of vinca alkaloids is neurotoxicity. Vincristine is the oldest and also the most neurotoxic agent in this group. Here we are reporting two cases with T-cell acute lymphoblastic leukemia, who developed unilateral palpebral ptosis 4 weeks after vincristine therapy was initiated. In both the cases, ptosis was treated conservatively without using any drug. Experience with these cases suggests conservative treatment, with periodic examination is sufficient, especially if ptosis is mild. Key words: vincristine, ptosis, neurotoxicity Introduction The vinca alkaloid antimicrotubule agents, which include vindesine, vinblastine, and vincristine, have been widely used as clinical anticancer agents for the treatment of leukemia, lymphomas, and some solid tumors, as well as for idiopathic thrombocytopenic purpura, and autoimmune hemolytic anemia. The neurotoxicity of vincristine is well recognised and is a limiting factor for its administration 1. Neuropathic symptoms frequently included paresthesias of lower limbs and as the disease progresses, impairment of proprioception and motor

nerve conduction become evident. The ocular system can be involved in vincristine neurotoxicity, with palpebral ptosis, bilateral ophthalmoplegia, diplopia, and bilateral seventh nerve palsy with lagophthalmos. Palpebral ptosis is almost always bilateral 1,2. We are reporting two cases of vincristine induced unilateral ptosis which is very rarely reported in literature. Case 1 A 3-year-old boy was diagnosed with T-cell acute lymphoblastic leukemia and was started on therapy according to MCP841 treatment protocol. His chemotherapy included prednisolone (60 mg/m2), Lasparaginase (6000 U/m2), vincristine (1.4 mg/m2) and daunorubicin (30 mg/m2) and methotrexate intrathecal therapy. On the 40th day of therapy, palpebral ptosis was noticed in his left eye. At this time, he had been administered a total of 4 doses of vincristine (5.6 mg/m2) and 10 doses of Lasparaginase (60,000 U/m2). There were no previous clinical symptoms of neuropathy and no positive history for inherited neuropathies. Neurologic examination revealed unilateral ptosis, with normal ophthalmic movements and normal pupillary and corneal reflexes. The remaining physical findings were normal. Ophthalmologic examination confirmed normal visual acuity and fundus. Cerebrospinal fluid was clear with normal pressure, glucose, and protein levels and no atypical cells. Neurophysiologic studies (electroencephalography, nerve conduction velocity) were carried out to evaluate the

*Department of Pediatrics and Medical Oncology* Father Muller medical College Mangalore.

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possibility of concomitant but subclinical peripheral neuropathy or encephalopathy induced by vincristine therapy. Both were normal. The ptosis was interpreted as mild neurotoxicity due to vincristine and treated conservatively. Child was examined regularly for worsening of symptoms and for evidence of other neurotoxic symptoms. The child improved slowly over next 4 weeks without any residual neurological deficit. Case 2 A 6-year-old boy was diagnosed with T-cell acute lymphoblastic leukemia and was started on chemotherapy according to MCP-841 treatment protocol. After 1 week of completion of induction phase, unilateral ptosis was noticed in his left eye (fig 1A). Neurologic examination revealed unilateral ptosis, with normal ophthalmic movements, normal pupillary and corneal reflexes, normal visual acuity and fundus. Cerebrospinal fluid was clear with normal pressure, glucose, and protein levels and no atypical cells. With experience from our 1st case, child was treated conservatively and kept under observation. Ptosis improved spontaneously after 4 weeks (fig 1B). Fig

1A

Discussion Vincristine is a vinca alkaloid used in combination with other agents in cancer chemotherapy. Pharmacokinetic studies indicate that the terminal half life of vincristine in humans is from 19 to 155 hours, so it must be used at intervals of 1 week or longer. The liver is the major excretory organ. Approximately 80% of an injected dose of vincristine appears in the faeces and 10-20% can be found in the urine. Vincristine is often chosen as part of polychemotherapy because of lack of significant bone marrow suppression2. The neurotoxicity of vincristine is well recognized and may be divided into four groups: peripheral neuropathy, autonomic neuropathy, encephalopathy, and cranial neuropathy. The severity of vinca alkaloid neurotoxicity is usually dosedependent. Recovery may take weeks or months, depending on the severity of the neuropathy, and residual minor abnormalities sometimes persist. These neuropathies are seen in most patients who have been taking the drug for more than 2 months. The pathogenesis of neuropathy is explained by the fact that vincristine causes structural changes in the microtubules of peripheral nerves and interferes with axoplasmic transport1-4. The most common neurotoxicity is dose-dependent peripheral neuropathy with early depression of the deep-tendon reflexes. Other recognized effects include paresthesia, gait disturbances, cranial nerve palsies and cerebral dysfunction in the more advanced cases.

1B

Figure 1A. Ptosis of the left eye in a 6-year-old boy after vincristine therapy. Figure 1B. Same child four weeks later ptosis resolved.

107

Cranial neuropathies are seen less often and usually develop as a late manifestation of generalized vincristineinduced neuropathies. However, an isolated cranial neuropathy, as in the present cases,


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may occasionally be the first sign of vincristine toxicity. Until now, seven cranial nerves (numbers II, III, V, VI, VII, VIII, and X) have been reported to be involved in vincristine associated neurotoxicity3,4. These palsies are self resolving and improvement was noted in most patients 2 to 6 weeks after discontinuation of vincristine with complete recovery within 4 months4. There are no entirely convincing reports of effective pharmacologic measures to prevent or to treat vincristine induced neuropathy, apart from a few case reports of cranial neuropathy treated with pyridoxine and pyridostigmine 2,5. It is considered that if chemotherapy can cure the patients cancer, chemotherapy-induced neurotoxicity might be tolerated without changing the effectivedose regimen. As our patients had only mild neurologic complication, the protocol was continued without modifications and drug was administered for ptosis. However the immediate next cycle did not involve vincristine. Bilateral ptosis is the most common feature of vincristine-induced cranial neuropathy. Lash et al3 reported a case of 2-year-old boy who developed unilateral ptosis with convergent strabismus during chemotherapy for ALL. In 2005, a case of vinblastine-induced unilateral ptosis was reported 6 , but the present finding of vincristine induced unilateral ptosis is very rare in the literature According to the best of our knowledge, there are only three case reports in English literature 2,5,7 and our case report probably is the 4th case report of vincristine induced unilateral ptosis without other clinical signs of cranial or peripheral neuropathy. 108

Conculsions Vincristine induced unilateral ptosis is predominantly benign and self limiting. Hence modification of chemotherapy protocol may not be necessary. REFERTENCES 1.

Akbayram S, Akgun C, Dogan M, Sayin R, Caksen H, Faik A et al. Use of Pyridoxine and Pyridostigmine in Children with Vincristine-Induced Neuropathy, Indian J Pediatr 2010; 77 (6) : 681-683

2.

Gursel O, Sari E, Altun D, Atay A, Akin R. Vincristine-Induced Unilateral Ptosis in a Child. Pediatr Neurol 2009;41:461-463.

3.

Lash SC, Williams CPR, Marsh CS, Hodgkins PR, MacKie EJ, Acute SixthNerve Palsy After Vincristine Therapy. Journal of AAPOS 2004;8(1):67-68

4.

Bay A, Yilmaz C, Yilmaz N, Oner AF. Vincristine induced cranial polyneuropathy. Indian J Pediatr 2006;73:531-3

5.

Dejan , Dragana B, Ivana P, Borivoje B, Marko P. Vincristine Induced Unilateral Ptosis J Pediatr Hematol Oncol 2009;31(6):463-4

6.

Parentin F, Liberali T, Perissutti P, Rabusin M. Unilateral palpebral ptosis associated with vinblastine therapy. Neuroophthalmology 2005;29:133-5

7.

Revannasiddaiah S, Bhattacharyya T. Vincristine-induced unilateral ptosis with serendipitous response to modafinil. BMJ Case Reports 2011; doi:10.1136/bcr.07.2010.3178


Vol. 25, No. 3, 4 July - Dec 2011

FAMILIAL DANDY WALKER VARIANT. *Dr. Roopa.Mangshetty,Dr. Hosgouda Kiran, Dr. Shrikanth.S.W. ABSTRACT:

On examination, Pulse- 80/min, BP100/60 mm of Hg , RR- 29 cycles/ min , Chest circumference- 47 cms, Height- 91 cms, Weight- 9 kg. Examination of Central nervous system revealed a conscious child, able to recognize parents. Motor system examination showed, no wasting and muscle girth was normal. There was hypotonia in all four limbs, with power of grade 3/ 5. There were no involuntary movements, and all sensory modalities were intact. Funduscopy was normal, convergent squint and head lag were present. There were no cerebellar signs. Signs of meningeal irritation were not present Other systems were normal. Based on clinical features such as developmental delay, mental retardation, microcephaly, prominent occiput, generalized hypotonia, prominent sutures, convergent squint, poor fine motor control a diagnosis of DANDY WALKER VARIANT was considered.

This report describes 2 cases with IV th ventricle cystic dilatation communicating with mega cistern magna with partial agenesis of cerebellar vermis with hypoplasia of both cerebellar hemispheres in two siblings from same family presenting with developmental delay, mental retardation, convergent squint, microcephaly, prominent occiput and generalized hypotonia. KEY WORDS: Cerebellar hypoplasia, developmental delay, mental retardation, microcephaly, prominent occiput. INTRODUCTION: Dandy Walker malformation is a rare abnormality of the CNS with a reported incidence of 1 in 25,000- 35,000 live births and a slight female predominance. It accounts for 1-4% of cases of antenatally detected hydrocephalus. Dandy- Walker variant forms part of the spectrum of Dandy Walker malformation. It is characterized by partial agenesis of the vermis and the cisterna magna.

Case-2 A sibling of case -1, two and a half year old child presented with inability to sit and stand, delayed mile stones & mental retardation, birth history was uneventful. On examination child had hypotonia with power 3/ 5 in all the limbs. Sibling of case 1.with delayed milestones, mental retardation, generalized hypotonia.

CASE REPORT: A three and a half year old child presented to us with a history of delayed motor and mental mile stones , and child was unable to sit and stand. This child was born to a non consanguineous couple, and birth history was uneventful. On examination she had microcephaly with a head circumference- 42 cms, prominent occiput, prominent sutures, closed anterior fontanelle and convergent squint.

INVESTIGATIONS: Creatinine phosphokinase-levels was normal

*Department of pediatrics M R Medical College Gulbarga-India

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b. Dandy- Walker variant- cystic posterior fossa mass with variable hypoplasia of the cerebellar vermis and no enlargement of the posterior fossa. c.Mega cisterna magna- enlarged cisterna magna with normal cerebellar vermis and IVth ventricle.(1) Computed tomography (CT) showing diffuse hypodensity of both cerebellar hemispheres with hypoplasia of cerebellar vermis,dilated IV th ventricle and mega cistern magna.

Magnetic resonance imaging (MRI) showing cystic dilation of IVth ventricle communicating with mega cisterna magna and partial agenesis of cerebellar vermis. Both cerebellar hemispheres are hypoplastic. MUSCLE BIOPSY- Many muscle fibers show presence of central nuclei and selective atrophy of the fibers, In addition there are widely scattered degenerating fibers. DISCUSSION Dandy- Walker complex is a rare congenital intracranial malformation that comprises a spectrum of abnormalities of the posterior fossa which are classified a.Dandy- Walker malformation- cystic dilation of the IV th ventricle, complete/ partial agenesis of the cerebellar vermis and an enlarged posterior fossa. 110

This order was first described by Dandy and Blackfan in 1914 and was designated as Dandy- Walker syndrome in 1954 by Benda, who also reported its familial occurrence.(1) The etiology of Dandy- Walker malformation is heterogenous and familial occurrence has been reported and a low recurrence risk in siblings (1-5%). A few cases resulting from autosomal recessive genes have been reported although in most patients the cause of Dandy- Walker malformation is not known (2-5). Dandy- Walker malformation is frequently associated with other intracranial anomalies such as agenesis of the corpus callosum, holoprosencephaly, occipital encephaloceles and ocular abnormalities. Extra cranial anomalies include polycystic kidney , cardiovascular defects, polydactyl and cleft palate (1) Increasing evidence apparently indicates that the cerebellum may be involved in higher cognitive function in addition to motor control co-ordination. Patient with cerebellar hypoplasia usually presents with developmental delay. Poor fine- motor difficulty, microcephaly, hypotonia, titubation, tremor, dysmetria, nystagmus, ataxia, oculomotor aprasia, synkinesis, attention deficit hyperactivity, and autisim. Cerebellum plays a role in cognitive, social, language development. (6)


Vol. 25, No. 3, 4 July - Dec 2011

Patient with Dandy- Walker malformation presents with developmental delay, enlarged head circumference or signs and symptoms of hydrocephalus. Hydrocephalus is present in approximately 90% of patients at the time of diagnosis.

5. Ulm B, Ulm MR, Deutinger J,

Hearing/ visual difficulties, subnormal intelligence (IQ < 83) is manifested in 41-47% patients (7-9),

Stuart H. Green , MD: clinical spectrum

Dandy- Walker variant forms part of the spectrum of Dandy- Walker malformation. It is characterized by partial agenesis of the vermis, resulting in communication between IVth ventricle and the cisterna magna.(10,11) This case has been reported because of its rarity and there is no signs and symptoms of hydrocephalus, macrocephaly, and presented with generalized hypotonia, with subnormal intelligence. REFERENCE: 1. Wg Cdr A Alam, Gp Capt B N Chander, Sqn Ldr M Bhatia. DandyWalker variant: Prenatal diagnosis by ultrasonography.MJAFI 2004;60;287-289. 2. Murray JC, Johson JA, Bird TD. Dandy- Walker malformation: etiology hetrogenity and empiric recurrence risks. Clin Genet. Oct 1985; 28(4); 272283 3. Bordarier C, Aicardi J. . DandyWalker syndrome and agenisis of cerebellar vermis; diagnostic problems and genetic counseling. Dev med child neurol. Apr 1990; 32(4): 285-294. 4. Cavalcanti DP, Salamao MA. DandyWalker malformation with postaxial polyddactyly: further evidence for autosomal recessive inheritance. Am J Med Genet. Jul 16 1999: 85(2) : 183184. 111

Bernaschek G. isolated Dandy- Walker malformation: prenatal ents 6. Evangeline Wasser, MD , Paul Danies, PhD , William P. White house, Md and associated with cerebellar hypoplasia. 7. Sasaki Adams D,Elbabaa SK, Jewells V,Carter L,Campbell,RitterAM.The Dandy Walker variant a case series of 24 Peadiatric patients and evaluation of associated anamolies. Incidence of Hydrocephalus

and

developmental outcomes.J Neurosurg Pediatrics Sept 2008 :2 (3): 194-9. 8. Kalidasan

V.Carroll

T.Allcutt

D.Fitzgerald RJ.The Dandiy-Walker Syndrome a 10-Year experience of its management and out come Eur J Pediate Surg Dec 1995:5 Suppl 1:16-8 9. Hadzagic Catiburic F,Maksic H, Uzicanin S, Helyic S, Zubcenic S, Merhemic

Z,etal.

Congenital

Malformations of the central neurous system : Clinical approach, Born J Basic Med Sci.Nov 2008:8 (4): 356-60 10. T LAVANYA M COHEN S V GANDHI T FARRELL E H WHITBY : A Case of a Dandy-Walkar

Varient : the

impoitance of a multidisciplinary team approach using complementary techniques

to

obtain

accurate

diagnostic information 11. Kliegman RM,.Behrman RE,.Jenson HB,.Stanton BF. Nelson Textbook of Pediatrics, Volume2; 18th ed. New Delhi, Elsevier, 2008


Vol. 25, No. 3, 4 July - Dec 2011

RETT SYNDROME - A CASE

REPORT

*Dr.R.Shanti priya, Dr.Rajesh SM, Dr.Vikram Singhal, Dr.Kiran Baliga ABSTRACT

CASE REPORT

Rett syndrome is a condition of unknown cause, so far reported only in girls, which has been differentiated on the basis of a characteristic onset, course, and pattern of symptomatology. Typically, apparently normal or near-normal early development is followed by partial or complete loss of acquired hand skills and of speech, together with deceleration in head growth, usually with an onset between 7 and 24 months of age. A case of 3 year old girl with Rett syndrome is presented here. This girl had normal development till the age of two and half years. However, gradually over the next few months, she developed features suggestive of Rett Syndrome.

A 3 year old female child born out of second degree consanguineous marriage to a primigravida mother at 28 weeks of gestation with birth weight of 1.3Kg presented to us with complaints of decreased communication with parents since last 4-5 months. This started after an episode of generalized tonic clonic seizures 5 months back. Child is on oral Phenobarbitone and no h/o further seizures after that. Child at present was able to stand with support and palmar grasp was present. Prior to the episode of GTCS, child was able to speak 2-3 words sentences with meaning which she had attained at around two and half years of age. Now the child was able to speak only some random irrelevant words.

Keywords: Rett syndrome, pervasive developmental disorder, neurodevelopmental disorder INTRODUCTION Rett syndrome is not actually a degenerative disease, but a disorder of early brain development1. It is characterized by normal development till 6 months to 48 months of age followed by gradual loss of purposeful hand movements and development of characteristic, stereotypical hand movements; loss of previously acquired speech; psychomotor retardation; ataxia; deceleration of head circumference and autistic symptoms 3. It occurs predominantly in girls. The frequency is 1/ 15,000-1/22,0001.We report a girl child who presented with regression of language milestones and a diagnosis of Rett syndrome was considered.

The clinical examination revealed continuous purposeless movements of both upper limbs. Weight and height were below the 3 rd centile and microcephaly was present. Child had convergent squint with hypertonia of both upper limbs and lower limbs with exaggerated deep tendon reflexes. Other systems were within normal limits. Ophthalmologic evaluation revealed right esotropia with normal fundus examination. Audiological evaluation was normal. MRI: There is mild reduction in cerebral and cerebellar volume with mildly prominent sulci and few deep gyrations. Bilateral lateral and third ventricles are mildly prominent with subtle undulated margins.

*Department of Pediatrics, Kasturba Medical College,Mangalore, Manipal University.

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DISCUSSION

Supportive criteria include breathing dysfunction, seizures, spasticity, scoliosis, and growth retardation. The diagnosis of RS is considered tentative until 2 to 5 years of age3.

Rett syndrome is a disorder of the nervous system that leads to developmental reversals, especially in the areas of expressive language and hand use3. Rett syndrome occurs almost exclusively in girls. Studies have linked many Rett syndrome cases to a defect in the methl-CpG-binding protein 2 (MeCP2) gene a transcription factor that binds to methylated CpG islands and silences transcription3. This gene is on the X chromosome. Females have two X chromosomes, so even when one has this significant defect, the other X chromosome is normal enough for the child to survive. Males born with this defective gene do not have a second X chromosome to make up for the problem 3 . Therefore, the defect usually results in miscarriage, stillbirth, or very early death2. Development may proceed normally until 6-18 months age, when regression of language and motor milestones and acquired microcephaly becomes apparent. An ataxic gait or fine tremor of hand movements is an early neurologic finding3.

The differential diagnosis includes other disorders associated with mental retardation, cerebral palsy, and seizure disorders. Symptoms may include apraxia, excessive salivation, intellectual disabilities, generalised tonic clonic seizures, loss of purposeful hand movements and severe language development problems3. The hallmark of Rett syndrome is repetitive hand-wringing movements and a loss of purposeful and spontaneous use of hands; these features may not appear until 2-3 years age1. Autistic behavior is a typical finding in all patients. After the initial period of neuroregression, the disease process appears to plateau, with persistence of the autistic behavior1. Four stages of Retts syndrome have been defined to help characterize the disorder and improve its recognition and diagnosis2. These stages may be described as follows:

Diagnostic criteria for Rett syndrome were developed by representatives of the International Rett Syndrome Association and the Center for Disease Control3.

- Stage I: Early onset (618 months of age) affects early development first with stagnation

Necessary criteria include normal prenatal and perinatal period; normal psychomotor development through the first 6 months of life; normal head circumference at birth, with subsequent deceleration of head growth; loss of purposeful hand skills; severely impaired expressive and receptive language; apparent severe mental retardation; and gait apraxia and truncal apraxia/ataxia3.

- Stage II: Regressive/rapid deterioration stage (14years) devastating cognitive and motor regression - Stage III: Relative stabilization/ plateau (210 years) partial recovery - Stage IV: Late motor impairment (after 10 years) cognitive stability with motor impairment 113


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Rett syndrome may be classified as atypical, classical (meets the diagnostic criteria) or provisional (some symptoms

REFERENCES 1.

appear between ages 1 and 3). 3 Rett

psychosis. In: Kliegman RM, Behrman

syndrome is classified as atypical if it

RE, Jenson HB, Stanton BF, eds. Nelson

begins early (soon after birth) or late

Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier;

(beyond 18 months of age, sometimes as late as 3 or 4 years old); if speech and hand skill problems are mild or if is appears in a boy (very rare).3

2007:chap 29. 2. World Health Organization. Retts syndrome. In: The ICD-10 classification

Treatment may include assistance

of mental and behavioural disorders: Clinical description and diagnostic

with feeding and diapering and physical therapy to help prevent the hands from contracting.

Medications

such

guidelines. New Delhi: Oxford University

as

Press;

carbamazepine may be used to treat seizures. Other medications or supplements that have been used or studied include Bromocriptine, Dextromethorphan, folate, L-

Shah PE, Dalton R, Boris NW. Pervasive developmental disorders and childhood

1992.www.ninds.nih.gov/

disorders/rett. 3. ADAM medical encyclopedia, NCBI (National centre for biotechnology information) U.S. National library of

carnitine and L-dopa3.

Medicine, Pubmed health.

The disease slowly progresses until the patient is a teenager. Then, symptoms

4.

syndrome - a neurodevelopmentaldisorder:

may improve. Developmental regression or

Report of two cases. Neurol India 2002; 50: 330-3.

delays vary. The average life expectancy of a girl with Rett syndrome may be mid-40s. Death is often related to seizure, aspiration

5. 6.

Mount RH, Charman T, Hastings RP, Reilly S, Cass H. Features of autism in

In conclusion, it is important for

Rett syndrome and severe mentalretardation. J Autism Dev Disord.

clinicians to be aware of this disorder because increased identification will help in

2003; 33: 435-42.

greater understanding of this disorder and proper guidance will help the patient and

Gupta V. Retts syndrome: A case report. Indian J Psychiatry 2001; 43: 81-4.

pneumonia, malnutrition, and accidents. CONCLUSION

Malhotra S, Kumar D, Gupta N. Retts

7.

Popper CW, Gammon GD, West SA,

family, and reduce the burden of care on the

Bailey CE. Disorders usually first

parents. Thus, it is suggested that all female

diagnosed in infancy, childhood or

children presenting with low intelligence and autistic symptoms should be suspected

adolescence.In: Hales RE, Yodofsky SC (eds). Textbook of clinical Psychiatry.

of having Rett syndrome until proved

4th ed. Washington, DC:American

otherwise.

Psychiatric Publishing; 2003:833-974. 114


Vol. 25, No. 3, 4 July - Dec 2011

NEPHROLOGY SECTION A SINGLE CENTER OBSERVATIONAL STUDY OF USE OF TACROLIMUS IN MANAGING NEPHROTIC SYNDROME AND REVIEW OF LITERATURE * Dr. Sudarshan Shetty K. Dr. Kishan Alva ABSTRACT: BACKGROUND: Nephrotic syndrome (NS) is a chronic kidney disease seen in childhood requiring immune suppressants. Tacrolimus (TAC) is a promising option in managing children with nephrotic syndrome with frequent relapses or steroid dependence. The present work is an observational data on 9 consecutive children with NS managed with TAC presenting to a single center. AIM: To evaluate the efficacy of TAC in maintaining remission in

INTRODUCTION: Nephrotic Syndrome (NS) is a chronic disease in children requiring long term follow up. Though the cornerstone of treatment has been oral steroids, due to its side effects, many steroid sparing drugs have been used in the management of NS (1, 2 ,3). NS is a disease which commonly relapses wherein a relapse is defined as significant proteinuria occurring after remission on 3 consecutive morning samples of urine. More than 3 episodes of

children with frequently relapsing NS.

relapses in a year or 2 or more relapses in

PATIENTS AND METHODS: The inclusion criteria were subjects who were steroid

NS. Steroid dependence is defined as NS

sensitive with frequent relapses of NS and

6 months is defined as frequently relapsing with relapses during steroid therapy or

not responding to cyclophosphamide. All

within 2 weeks of stopping steroids.

subjects with steroid resistance, severe renal dysfunction or chronic renal failure were

relatively safe but potent calcineurin

not included. RESULTS: All the subjects

Tacrolimus (TAC) has been emerging as a inhibitor in the management of NS. Data on

maintained remission for more than 6

the use of TAC in children in the management

months. None showed any toxicity to TAC. CONCLUSION: TAC is a well tolerated

of NS is sparse. Our observation presented here is an attempt to enrich this database.

alternative to cyclosporine A (CsA) in the

STUDY METHODOLOGY:

management of steroid sensitive NS.

This is a retrospective observational

KEY WORDS:

study of patients managed at a single center

Cyclophosphamide, cyclosporine,

in southern India with frequently relapsing

tacrolimus, minimal change disease, steroid

NS (n=9). The data collection was over a

resistant nephrotic syndrome, steroid

period of 36 months from January 2007 to

dependent nephrotic syndrome

December 2009.

* Department of Pediatrics, K.S.Hegde Medical Academy Deralakatte, Mangalore

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Vol. 25, No. 3, 4 July - Dec 2011

AIM: To evaluate the safety and efficacy of TAC in maintaining remission in children with frequently relapsing NS. PATIENTS AND METHODS: The inclusion criteria required the subjects to be steroid sensitive during the first episode. Subjects with frequent relapses or steroid dependence who did not respond to cyclophosphamide were included. All subjects with no response to steroid treatment, renal dysfunction or chronic renal failure were not included in the study. Among the study group 2 subjects had steroid dependence confirmed on two consecutive episodes. There were 5 males & 4 females in the study group. The age ranged from 3 years to 14 years (mean 7.5 years). All the subjects hailed from a similar geographic & cultural background. All the subjects had received oral cyclophosphamide over 8 12 weeks before the start of TAC therapy. Among the subjects 2 with steroid dependence had received CsA before starting TAC. CsA resistance was defined as inability to induce remission after 8 weeks of therapy. TAC was started only when cyclophosphamide and or CsA was unable to maintain remission. Before the start of TAC therapy all the subjects received daily steroids at a dose of 2 mg/kg to induce remission. TAC was started only after remission was documented with a urine albuminuria of 1+ or less by dipstick test. Complete blood counts, renal function tests and renal biopsy was done prior to the start of therapy. TAC was started at a dose of 0.15mg/kg along with alternate day steroids and TAC dose adjusted accordingly to attain target TAC trough levels of 5-10 ng/L. All TAC trough levels were measured by Microparticle Enzyme Immuno Assay (MEIA) method in 116

the same standardized laboratory. As an institutional policy oral steroids were tapered slowly by 0.5mg/kg every 4 weeks & stopped over 12-14 weeks after the start of TAC therapy. The subjects were instructed to take the medications at the same time in the morning & evenings which was documented in their medication diary. All the subjects were followed up after 2 weeks of starting therapy. Blood for TAC trough levels, hemoglobin, total leucocyte count, differential leucocyte counts, serum creatinine, blood urea, serum sodium, serum potassium, serum chlorides, serum amylase, fasting blood sugar, aspartate transaminase (AST), alanine transaminase (ALT) and urine for proteinuria, glycosuria & microscopic examination for hemeturia were analysed. Blood pressure was measured with a mercury sphygmomanometer, using an appropriate sized cuff in the right upper limb of the subject in sitting position. Blood investigations and TAC trough levels were repeated every 3 months. Minimum follow up period among the study group was 6 months with maximum period of follow up being 30 months. Written informed consent was taken from the parents of the subjects before administartion of TAC. They had no objections for the use of this data for research purposes. As an institutional policy we use TAC or CsA in the management of NS not responding to cyclophosphomide or NS with frequent relapses or NS with steroid dependence. As the present data is retrospective in nature ethical clearance was not required for the publication of the present data. RESULTS: Irrespective of the age, compliance and tolerability of TAC was excellent in all the subjects. Histological features of 5


Vol. 25, No. 3, 4 July - Dec 2011

subjects were suggestive of minimal change disease (MCD), 2 had focal segmental glomerulosclerosis (FSGS) and 2 had light microscopic features of MCD with IgM deposits on immunoflorescence study. Both the subjects with steroid dependence had histological features suggestive of FSGS. Irrespective of the histopathology, all the subjects were in remission for a minimum of 6 months after starting TAC therapy (range: 6 months to 30 months; mean: 15 months). All the subjects had normal renal function before the start of TAC therapy. One subject with the clinical presentation of frequently relapsing NS had a documented relapse secondary to upper respiratory infection after 8 months of starting TAC therapy. The subject went into remission after treatment

of the infection with antibiotics & stress dose of steroids that is prednisalone at 1mg/kg/day given for 5 days. TAC was continued during the relapse without dose correction. Follow up of all subjects were done once in 3 months if asymptomatic or were told to followup whenever there was edema or documented relapse. All the subjects maintained normal blood pressure during the study period and blood investigations were unremarkable. None of the subjects showed any evidence of toxicity to TAC requiring dosage adjustment or withdrawal. All the subjects were gaining weight satisfactorily and were physically active during the last followup.

Table 1: Subject profile of clinical presentation, histology & blood investigations S.Crd (mg/dL) Before TAC

S.Cr (mg/dL) Last follow up

TAC duration (months) Mean=13.3

MCDf -

0.8

1.0

30

06

MCD

IgM

0.5

0.6

17

07

FSGS

IgM

0.8

0.8

18

8/4

M FRNS/S DNSg F FRNS

04

MCD

-

0.4

0.6

6.5

7/0

F

FRNS

04

MCD

IgM

0.4

0.3

09

2.5

1/3

M FRNS

04

MCD

-

0.8

0.6

10

Sub 7

05

3/6

M FRNS

05

MCD

-

0.8

0.4

13

Sub 8

08

6/1

F

07

FSGS

-

0.7

0.8

7.5

Sub 9

05

3/2

F

04

MCD

-

0.6

0.8

8.5

n=9

Age (years) Mean: 7.5 years

Age at onset of NS (years/m onths)

S e x

Sub 1

12

7/2

Sub 2

07

Sub 3

No of relapses before TACa

Histo logy LMb

M FRNSe

10

5/1

M FRNS

09

6/0

Sub 4

10

Sub 5

9.5

Sub 6

Clinical Present ation

FRNS/S DNS FRNS

h

Histology IFc

Footnote: TACa - tacrolimus, LM b - light microscopy, IFc - immunoflorescence study, S.Crd - serum creatinine, FRNSe - frequently relapsing nephritic syndrome; MCD f - minimal change disease, SDNSg - steroid dependent nephritic syndrome, FSGSh - focal segmental glomerulosclerosis.

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Vol. 25, No. 3, 4 July - Dec 2011

DISCUSSION:

inhibitors like tacrolimus exert their effects

Nephrotic syndrome is a glomerular

by binding to proteins called immunophilins

disease characterized by protein loss in the urine which may damage the tubules. The

and B-cell lymphocytes resulting in potent

cornerstone of treatment has been oral

and affects growth and differentiation of Timmune suppression (4, 6). In our study

steroids, which needs to be given during

group 2 subjects who did not respond to

each episode of the disease. Frequent use of steroids during relapses carries many side

CsA went into remission after TAC therapy. But as the number of subjects is very small,

effects, especially growth which is a concern

it is premature to conclude that TAC is

in children with NS (1, 2). Many steroid

superior to CsA in inducing remission.

sparing drugs like levamisole, cyclophosphamide, cyclosporine A and

Though not seen in our study group there is

mycophenolate mofetil have been used in

with toxicities like diabetes, hypertension,

the past with varied response in the

nephrotoxicity and hepatotoxicity (2, 3).

management of frequently relapsing NS (3). Calcineurin inhibitors (cyclosporine,

Hence careful monitoring of the blood

tacrolimus) are capable of inducing

and renal function tests are mandatory.

remission in a significant proportion of

Monitoring of renal histopathology has been

patients. While most patients respond to CsA (4, 5), prolonged use carries the risk of

recommended in long term use of the drug.

cosmetic sideeffects and nephrotoxicity.

therapy was not done in our study group

Withdrawal is usually associated with

as none of the subjects had hypertension or

relapse. Tacrolimus (TAC) is more potent (4, 6) with lesser nephrotoxic potential compared to cyclosporine. Though TAC has been used in renal transplantation there have been few reports on the use of TAC in children with nephrotic syndrome (4, 6, 7). Most of the case series were small, heterogeneous and have included children with steroid resistant nephrotic syndrome (SRNS). There is data to suggest that TAC has better inhibition of the vascular permeability factor and differing effects on proteinuria in NS compared with cyclosporine (8, 9). TAC also has better cytokine suppression than cyclosporine, which may also influence the differing responses to therapy. Calcineurin 118

documented evidence that TAC is associated

trough levels, complete blood counts, liver

But renal biopsy after the initiation of TAC

showed any evidence of renal function deterioration on blood investigations. Recent data suggest that the toxicity were minimal when the drug was given in lower recommended doses to maintain trough levels between 5 to 10 ng/ml (2, 10) which was followed in our study group. Economically

TAC

is

in

par

with

cyclosporine A and has advantage over CsA in the form of lesser cosmetic side effects but has the disadvantage of not being available in liquid form for use in very young. The possibility of diabetes in children treated with TAC should caution the treating physician though it was not seen in our study group. The weakness of our study was the small number of subjects. Data on


Vol. 25, No. 3, 4 July - Dec 2011

follow up after discontinuation of TAC is

steroid dependent nephrotic syndrome.

presently not available as the subjects are

Clin Nephrol 2006; 65: 393-400

on therapy in our study group.

5. Sinha MD, MacLeod R, Emma Rigby et al. Treatment of severe steroid-

CONCLUSION: Tacrolimus is a relatively safe and potent alternative in managing children with

dependent nephrotic syndrome in children with tacrolimus. Nephrol Dial

NS especially children having frequent

Transplant 2006; 21: 184818541,54

relapses or resistant to drugs like

6. Sanjeev G, Narayan P, Raj KS et al.

cyclophosphamide or cyclosporin. The toxicity is very minimal when maintained in minimal doses & has the advantage of cosmetic superiority over cyclosporin. Larger study group and follow up would strengthen the conclusion.

resistant nephrotic syndrome in children. Nephrol Dial transplant 2008;23:910-3 7. Schweda F, Liebl R, Riegger GAJ et al. TAC treatment for steroid- and

REFERENCES:

cyclosporin-resistant minimal change

1. Bagga A, Afzal K, Agarwal I et al. Management of Steroid Sensitive Nephrotic

Tacrolimus: a new therapy for steroid-

Syndrome:

Revised

Guidelines. Indian society of Pediatric Nephrology.

Indian

Pediatr

2008;45:203-214

nephrotic syndrome. Nephrol Dial Transplant 1997; 12: 24332435 8. Budde K, Fritsche L, Neumayer HH. Differing proteinuria control with cyclosporine and TAC. Lancet 1997; 349: 330

2. Gulati A, Bagga A, Gulati S et al. Guidelines for management of steroid resistant nephrotic syndrome. Indian Pediatr 2009;46: 35-49

9. Loeffler K, Gowrishankar M, Yiu V. TAC therapy in pediatric patients with treatment resistant nephrotic syndrome. Pediatr Nephrol 2004; 19: 281287

3. Bagga A, Mantan M. Nephrotic syndrome in children. Indian J Med Res 2005; 122: 13-28

10. Jörg D, Katalin D, Christian P et al. Is tacrolimus for childhood steroid dependent nephrotic syndrome better

4. Westhoff TH, Schmidt S, Zidek W et al. Tacrolimus in steroid-resistant and

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than ciclosporin A? Nephrol Dial Transplant 2006; 21:1761-3.