[2 cases of familial multiple endocrine neoplasia syndrome].
Two cases of familial multiple endocrine neoplasia syndrome (MEN) are reported. Case 1. A 31-year-old woman began to have repeated attacks of coma in 1970. Blood examination showed fasting sugar as low as 2.05 mmol/L (37 mg/dl). Her disease was cured after the removal of two insulinomas in 1972. However, coma occurred again in 1982. Examination revealed the ratio of concentrations of serum insulin and sugar being more than 0.3. Another operation with removal of 19 insulinomas were performed in our hospital in 1985. After operation, the level of blood sugar returned to normal. Her father had suffered from Zollinger-Ellison Syndrome; the entire stomach and a part of pancreas were removed in 1967. So we examined her brother. His serum calcium level was 2.5-2.9 mmol/L (10.0-11.6 mg/dl) and plasma iPTH level 49.5-115.5 ng/L (normal value 21.4 +/- 7.7 ng/L n = 71). The high level of iPTH could not be suppressed by calcium load test. The total of two and a half of the remaining two parathyroid glands were removed. Pathology confirmed hyperplasia of parathyroid glands. The levels of serum calcium and iPTH returned to normal after operation. Case 2. A 25-year-old woman suffered from hypertension at the age of ten. Adrenal tumor with pheochromocytoma (2 x 2 x 1.5 cm3) was diagnosed and removed at that time. She complained of intermittent coma for 10 months and was thus admitted to our hospital in Nov. 1984. The level of blood sugar was 1.5-3.8 mmol/L (26-67 mg/dl).(ABSTRACT TRUNCATED AT 250 WORDS)
Zeng
Z P
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Meng
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Liu
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D007516:Adenoma, Islet Cell / Q000235:genetics*; D000310:Adrenal Gland Neoplasms / Q000235:genetics*; D000328:Adult; D005260:Female; D006801:Humans; D006961:Hyperparathyroidism / Q000235:genetics; D007340:Insulinoma / Q000235:genetics*; D009377:Multiple Endocrine Neoplasia / Q000235:genetics*; D010190:Pancreatic Neoplasms / Q000235:genetics*; D010673:Pheochromocytoma / Q000235:genetics*