Abstract
A 17 month old boy, the first child of unrelated parents, presented with polydipsia, lack of appetite and failure to thrive. Blood pressure was 150/100 mmHg. He had hypokalemia (2.6 mEq/1), suppressed renin activity (0.4 ng/ml/hr) and low plasma aldosterone level (2 ng/100 ml).
Urinary 11β-hydroxy-steroids were increased relative to 11 oxosteroids (THF: 110, THE 8 μg/day; ratio 13.8. Control ratio 0.4) indicating 11 β-hydroxysteroid-dehydrogenase deficiency. Both parents were normal. The hypertension and hypokalemia were unresponsive to spironolactone. Dexamethason treatment did not influence the hypokalemia and even increased blood pressure. Triamterene (3 × 25 mg) normalised serum potassium, but addition of furosemide (2 × 10 mg) was required for normalisation of blood pressure. This treatment resulted in catch up growth (from -3.6 SD to -1.8 SD for height).
This is the youngest patient known with this syndrome. Unlike the other cases (Ulick et all. J.C.E.M., 49: 757, 1979) he did not respond to triamterene alone. Also the growth retardation and catch up growth after treatment is documented for the first time.
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Otten, B., Monnens, L., Fiselier, T. et al. Hypertension, hypokalemia and retarded growth in a 17 month-old boy with 11β hydroxysteroid-dehydrogenase deficiency. Pediatr Res 15, 1558 (1981). https://doi.org/10.1203/00006450-198112000-00141
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DOI: https://doi.org/10.1203/00006450-198112000-00141