Intestinal malformations and Down's syndrome ... (1979-1986) of the different intestinal malformations ... duodenal and anorectal anomalies than jejunal and.
Journal of the Royal Society of Medicine Volume 80 January 1987
performed when the intracompartmental pressure exceeds 30-45 mmHg3 s. Because the displaced fascia was so easily visualized, we believe that CAT is suitable for d6monstration of the compartmental syndrome, even in the early stages. IVAN W JENSEN Aalborg Sygehus Syd Aalborg, Denmark A OLSEN References 1 Mubarak SJ, Hargens AR. Acute compartment syndromes. Surg Clin North Am 1983;63(June) 2 Matzen FA. Surgical pros and cons. Surg Gynecol Obstet 1979;149:74-5 3 Matzen FA m, Mayo KA, Sheridan GW, et al. Monitoring of intramuscular pressure. Surgery 1976;79:702-9 4 Rorabeck CH, Castle GSP, Hardie R, et al. Compartmental pressure measurements: An experimental investigation using the slit catheter. J Trauma 1981;21:446-9 5 Whitesides TE, Haney TC, Marimoto K, et al. Tissue pressure measurements as a determinant for the need of fasciotomy. Clin Orthop 1975;113:43-51
Cardiovascular risk in patients with treated familial hypercholesterolaemia and patients with severe hypertriglyceridaemia Sir, Dr Way and colleagues' important paper (July 1986 JRSM, p 391) suggests that hypertriglyceridaemia is an appreciable cardiovascular risk. Their study reinforces the findings of an equally elegant earlier study which reported a 'hypercoagulable' state in association with hypertriglyceridaemial. However, Way et al. did not assess the coagulation factors which were included in the earlier study1. This detail would have helped establish whether any association between hypertriglyceridaemia and ischaemic heart disease (IHD) is mainly mediated by concomitant unfavourable changes in coagulation factors. We are especially interested in this question because of the association of diabetes mellitus with hypertriglyceridaemia2 and raised levels of plasma coagulation factors including fibrinogen3. Furthermore, the study by Way et al. suggests that patients with hypertriglyceridaemia alone should be treated and there is now evidence that bezafibrate, which lowers both plasma cholesterol and triglyceride concentrations, also causes a significant fall in plasma fibrinogen concentrations4. This recent observation is clinically relevant because plasma fibrinogen is a powerful predictor of IHD1 3,5, a finding that is in agreement with the central role that this coagulation factor plays in coagulation, plasma viscosity and platelet function5. We are unaware if treatment of hypercholesterolaemia with cholestyramine also significantly reduces plasma fibrinogen. There is further evidence linking lipid metabolism with plasma fibrinogen levels since elevated plasma non-esterified fatty acid (NEFA) concentrations stimulate fibrinogen synthesis6. This observation could therefore account, at least in part, for the elevated plasma fibrinogen levels reported in diabetics since they tend to have elevated plasma NEFA levels2'7. Did the hypertriglyceridaemic patients described by Way et al. also have raised plasma NEFA levels? Unfortunately, although we have documented that supplementation with dihomogammalinolenic acid lowers plasma NEFA levels in diabetics, we did not establish whether plasma fibrinogen concentrations were also decreased.
Finally, previous studies investigating the incidence of IHD in association with hypertriglyceridaemia may not have shown a convincing link (as discussed by Way et al. and in reference 1) because coagulation factors are possibly not invariably raised in hypertriglyceridaemia. Other factors such as diet, race, stress, control of lipolysis, and severity of hypertriglyceridaemial may also be relevant. D P MIKHAILIDIS Diabetic Service and Metabolic Unit, Department ofChemical Pathology M A BARRADs and Human P DANDONA Royal Free Hospital and School Metaboliamn, ofMedicine, London NW3
References 1 Simpson HCR, Mann JI, Meade TW, Chakrabarti R, Stirling Y, Woolf L. Hypertriglyceridaemia and hypercoagulability. Lancet 1983;i:786-90 2 Stout RW, Bierman EL, Brunzell JD. Atherosclerosis and disorders of lipid metabolism in diabetes. In: Vallance-Owen J, ed. Diabetes, its physiological and biochemical basis. Lancaster: MTP Press, 1975:125-69 3 Fuller JH, Keen H, Jarrett RJ, et al. Haemostatic variables associated with diabetes and its complications. Br Med J1979;ii:964-6 4 Almer L-O, Kjellstrom T. The fibrinolytic system and coagulation during bezafibrate treatment of hypertriglyceridaemia. Atherosclerosis 1986;61:81-6 5 Mikhailidis DP, Barradas MA, Maris A, Jeremy JY, Dandona P. Fibrinogen mediated activation of platelet aggregation and thromboxane A2 release: pathological implications in vascular disease. J Clin Pathol 1985; 88:1166-71 6 Carlson TH, Wentland SH, Leonard BD, Ruder MA, Reeve EB. Effects of prostaglandin El, analogs, fatty acids and indomethacin on fibrinogen level. Am J Physiol 1978;235:H223-30 7 Mikhailidis DP, Kirtland SJ, Barradas MA, Mahadevia S, Dandona P. The effect ofdihomogammalinolenic acid on platelet aggregation and prostaglandin release, erythrocyte membrane fatty acids and serum lipids: evidence for defects in PGE1 synthesis and *A-desturase activity in insulin-dependent diabetics. Diabetes Res 1986;3:7-12
Intestinal malformations and Down's syndrome in Arabs Sir, We would like to report our 7-year-experience (1979-1986) of the different intestinal malformations seen in the first 600 consecutive Down's syndrome (DS) neonates and infants (mostly Arab) to be diagnosed clinically and cytogenetically by trypsin-G banding techniques in Kuwait. Seventeen patients (11 males, 6 females) had associated intestinal malformations (2.8%) and underwent operation. Seven cases (1.16%) had imperforate anus, 6 '(1%) duodenal obstruction and 4 (0.67%) Hirschprung's disease. Sixteen DS patients had regular trisomy 21. One case with DS phenotype died before karyotyping but her mother proved to be a balanced translocation carrier with 46XX,t(14q;21q) karyotype. In a previous study of congenital malformations in Kuwait1, no mention was made of any association between gut malformationls and DS. This study estimated separately the incidence of gut anomalies and DS to be 1.9/1000 and 1.1/1000 births respectively. Only 20 DS patients were included in this study. The association of different gastrointestinal malformations has been well known for some time.
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Differing high overall incidence figures were given from three large studies by Carter2, Hanhart3, and Knox and Ten Bensel' (725, 800 and 110 DS patients respectively). Comparison of the findings in the present Kuwaiti study with those of Knox and Ten Bensel4 from Minnesota has led us to make the following conclusions: (1) The present study showed a lower overall incidence of intestinal malformations (2.83%) compared with the Minnesota figures (9.1%). This significant difference between Arabs and non-Arabs seems to be due to different methodological approaches. (2) The present study supports the characteristic disease profile of intestinal malformations associated with DS proposed by the Minnesota study, with more duodenal and anorectal anomalies than jejunal and ileal malformations and Hirschprung's disease. A detailed analysis of our data is to be published elsewhere. S A AL-AWADI T I FARAG K K NAGUIB
Kuwait Medical Genetics Centre, and Paediatric Surgery Department Ibn Sina Hospital, Safat, Kuwait
A S TEEiI M Y EL-KHALIFA M J MARAFIE MAISSA
E-S H MAHrouz References 1 El-Alfi OS, Shaker YK, Shaath R, Salam TA. Congenital malformations on Kuwait. Journal of the Kuwaiti Medical Association 1968;2:99-109 2 Carter CO. A life table for Mongols with the causes of death. JMent Defic Res 1958;2:64 3 Hanhart E. 800 Folle Von Mongoloidismus (Down syndrome) in Konstitutioneller Betrachtung. Arch Julius Klaus-Stiftg 1960;35:Heft 1/2 4 Knox CE, Ten Bensel RW. Gastrointestinal malformations in Down's syndrome. Minn Med 1972;542-4
Anorectal ulceration due to abuse of dextropropoxyphene and paracetamol suppositories Sir, We read with interest the case report by Eigler and colleagues (July 1986 JRSM, p 424) of a female patient developing bilateral anorectal ulceration following abuse of ergotamine suppositories for migraine. Proctologists may be interested to know that there -is at least one other type of 'analgesic' suppository often responsible for severe anorectal lesions.
Prolonged use or abuse of dextropropoxyphene and paracetamol suppositories (marketed in France as DiantalvicR), taken for the relief of migraine, headache or other painful condition, often results in widespread anorectal ulceration (Figure 1). The latter can occasionally be complicated by stenosis. The addictive properties of dextropropoxyphene are well recognized'. Fifteen cases of anorectal lesions due to DiantalvicR suppositories have already been published in the French literature2. Of these, 13 patients were women: abdominoperineal resection for intractable stenosis was carried out in 2 patients. Lesions caused by DiantalvicR suppositories may heal after withdrawal of this addictive drug. Dextropropoxyphene contained in DiantalvicR suppositories, and not paracetamol, is thought to be responsible for the anorectal ulceration. ALBERT FENZY Groupe M6dical St R6mi, and Institut Jean Godinot WLADIMIR V BOGOMOLETZ Reims, France References 1 Reynolds EF, Prasad AB, eds. Martindale. The Extra Parmacopoiea. London: The Pharmaceutical Press, 1982;1006-8 2 Hemet J, Leroy A, Duprey F, Rocher V, M6tayer J, Ducastelle T. Stenose anorectale par suppositoires de dextropropoxyphbne et paracktamol (DiantalvicR). Gastroentbrol Clin Biol 1986;10:517-20
Infections caused by opportunistic mycobacteria Sir, We are grateful to Wain and his colleagues (September 1986 JRSM, p 559) for their comments on our review paper on opportunist mycobacterial infections (April JRSM, p 226) and for drawing attention to the danger of such infection following the implantation of porcine heart valve bioprostheses. Although overt porcine tuberculosis is now very rare, pigs are not infrequently infected, covertly or overtly, with Mycobacterium avium, other 'atypical' mycobacteria such as M. chelonei and also Nocardia spp. Adequate sterilization and screening procedures are therefore of the greatest importance. The addition of mycobactin - a lipid-soluble ironbinding agent - is essential for the primary isolation and subsequent cultivation of M. paratuberculosis, the causative agent for Johne's disease of cattle. It has, however, been shown that some strains of M. avium are also mycobactin-dependent, particularly on primary isolation from small inocula'I 3. For this reason we would recommend the addition of mycobactin to media used for screening porcine tissues for mycobacteria. J M GRANGE Cardiothoracic Institute, M D YATES
Fulham Road, London SW3 Public Health Laboratory, Dulwich Hospital, London SE22
References several months F
1 Matthews PRJ. The use of culture medium containing mycobactin for the isolation of acid-fast organisms for pig head lymph nodes. Res Vet &ci 1969;1O:104-6
2 Matthews PRJ, McDiarmid A, Collins P, Brown A. The dependence of some strains of Mycobacterium avium on mycobactin for initial and subsequent growth. J Med Microbiol 1977;11:53-7 3 lThoen CO, Himes EM, Jarnagin JL, Harmngton R. Comparison of four culture media for isolation of Mycobacterium avium complex from porcine tissues. J Clin Microbiol 1979;9:194-6
