(Brown & Person 1962). ... Conn's Syndrome with Associated Pregnancy. B J Boucher Mvff MRCP .... the fingers, wrists, knees and ankles became stiff, painful ...
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Section ofEndocrinology
& Weingarten 1964) occurred in a patient with a strong family history of diabetes. It is usual for steroids to inhibit ketonuria after starvation (Brown & Person 1962).
Physical signs (1963): Blood pressure 180/120 to 240/135. She was generally weak and the fundi showed arteriovenous nipping.
REFERENCES Blereau R P & Weingarten C M (1964) New Engl. J. Med. 271, 836 Brown J & Person C (1962) Clinical Uses of Adrenal Steroids. New York Escovitz W E & Reingold I M (1961) Ann. intern. Med. 54, 1248 Fajans S S & Conn J W (1954) Diabetes 3,296 Peart W S, Porter K A, Robertson J I, Sandler M & Baldock E (1963) Lancet i, 239 Riggs B L & Sprague R G (1961) Arch. intern. Med. 108, 841
Conn's Syndrome with Associated Pregnancy B J Boucher Mvff MRCP and A Stuart Mason MD FRCP Mrs M R, aged 40 History: This patient presented in 1963 with a history of eight years' hypertension dating back to severe toxemia in her fourth pregnancy. She had developed thirst, polyuria and occasional weakness when thiazide diuretics were begun four years later. After four years on this therapy all drugs were stopped and it was the persistence of hypokaleemia despite potassium supplements that led Dr E Frankel to suggest that she might have primary aldosteronism. SODIUM
Investigations: Chest X-ray: heart slightly en-larged. Urine: few granular casts and pus cells. IVP and presacral air insufflation: normal right adrenal, largeleft adrenal; kidneys normal. Plasma sodium 144-160, potassium 1 7-2-3, bicarbonate 30-35 mEq/l. Total exchangeable potassium 1,861 mEq (26-6 mEq/kg body weight). ECG: nonspecific ST and T wave changes. Arterial blood: pH 7-48; arterial pCO2 40 mmHg; standard bicarbonate 29 mEq/l.; oxygen saturation 85%. Urine aldosterone 62 and 74 1ig in 24 h. Pregnancy test negative on admission.
Balance studies (see Fig 1) were carried out on 70 mEq of potassium and 100 mEq of sodium daily. Courses of spironolactone and metyrapone were given without significant effect on potassium balance or the plasma potassium. Potassium supplements were then given. Operation (April 1963): A left adrenal adenoma (4 x 3 x 3 cm) was excised and the left kidney was biopsied. Post-operative progress: The blood pressure fell to'l 10/70 by the fourth day and has now settled to
4
mEq/
-250-
OEP
|mEfq R11
MN
SODIUM 150 DIAY. OUTPUT 1500 RAW.L
0LINTAKE FOTASSIM
T
POTASSIUM150-
mEq
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3lOTAL Ex
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I6lmEq
POTASSIUM 2-
11
O
19633
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246 200mg 3 *& 1111
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APRIILt 3910 I I Il 11 1 1 1 I I I I 11 t1 4 1 7 ? YV VA 3* V7 V V P92 22 Fig 1 Sodium and potassium balance data showing the effect of the administration first ofspironolactone and then of metyrapone with dexamethasone. Potassium supplements were given alone for ten days before operation. Shaded areas represent urinary excretion and black areas facal excretion. (Spironolactone given as Aldactone A) 4
5
575
576 Proceedings ofthe Royal Society ofMedicine
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155/90 to 160/105. The plasma potassium rose to normal in ten days without supplements. Pregnancy was then diagnosed and terminated at eight weeks.
1963), so that this lack of response may well have been due to inadequate dosage for the activity of the tumour rather than to the associated pregnancy.
Pathology: The adrenal adenoma was histologically typical of Conn's syndrome with large, lipid-filled cells with much variation in nuclear size and some multinuclear cells. A renal biopsy showed patchy interstitial fibrosis. There was some vacuolation of proximal tubule cells and some arteriolar hyperplasia but minimal glomerular damage.
REFERENCES H6kfelt B (1962) In: Aldosterone. Netherlands Society for Endocrinology. Folia med. neer. Ad. 1, p 25 Relman A B (1963) Boerhaave Course. Hypertension. Leiden Venning E R4 & Dyrenfurth 1 (1956) J. clin. Endocrin. 16,426 Watanabe M, Meeker C I, Gray M J, Sims E A H & Solomon S (1963)J. clin. Invest. 42, 1619
Discussion This patient presented with symptoms of potassium depletion precipitated by diuretic therapy as often happens in primary aldosteronism (Relman 1963). The high urinary potassium in the presence of evidence of potassium depletion, a low plasma potassium and a raised plasma sodium were suggestive of aldosteronism due to a primary adrenal lesion. Fig 1 shows the failure ofthe plasma and urinary potassium to alter significantly when the action of aldosterone was antagonized with a spironolactone or its production blocked by metyrapone (with dexamethasone cover) in doses found to be effective in 13 cases by Hokfelt (1962). Although the patient was investigated and treated between the second and seventh weeks of pregnancy, significantly raised aldosterone excretion is not usually present until the third month of pregnancy (Venning & Dyrenfurth 1956). Raised aldosterone secretion rates are not usually found before the fifteenth week of pregnancy (Watanabe et al.
The following cases were also shown:
Hypertension and Pituitary Tumour with Pigmentation after Subtotal Adrenalectomy for Cushing's Syndrome Dr M Hartog (for Professor Russell Fraser and Dr R J Harrison)
o,p'-DDD1 in the Treatment of Adrenocortical Carcinoma Dr D A D Montgomery and Professor R B Welbourn (see Brit. med. J. 1965, i, 1356) Thyrotoxic Giantism Associated with Klinefelter's Syndrome Dr W Singer (for Dr Raymond Greene)
Amenorrhea Following Tuberculous Meningitis Dr Nigel Oakley (for Professor Russell Fraser) lo, p'-DDD: 2(2-chlorophenyl)-2-(4-chlorophenyl)-l, 1-dichlorethane
Meeting March 24 1965
Cases (Esophageal Polyp with Hypertrophic Osteoarthropathy K E W Melvin MRcP (for G F Joplin MRcp and Professor T Russell Fraser MD) P B, woman, aged 23 History: Ulcerative colitis developed in June 1960, with typical sigmoidoscopic and radiological changes. It has remained mild, with infrequent exacerbations.
Clubbing of the fingers was first apparent at the onset of the colitis, and progressed rapidly.
During the next two years there developed also considerable soft-tissue swelling about the wrists and ankles, together with marked pain and tenderness distally in the long bones. The joints of the fingers, wrists, knees and ankles became stiff, painful, and swollen, leading to severe incapacity. Radiographs revealed gross hypertrophic osteoarthropathy, with extensive subperiosteal new bone along the shafts of radii and ulnv, tibim and fibulk. In view of the mild nature of the ulcerative colitis, and the exceedingly rare occurrence of hypertrophic osteoarthropathy in this condition, it seemed unlikely that the two were associated. No other cause could be found at that
