Diabetes insipidus and blindness caused by a suprasellar tumor: Pieter Pauw's observations from the 16th century.

Tumors in the suprasellar region may cause both visual and endocrinologic symptoms. This association, well known to modern physicians, was established during the 19th century. However, we have identified a 16th-century autopsy report, written by the Dutch professor of anatomy Pieter Pauw (1564-1617), which describes an 18-year-old girl who developed marked polyuria and subsequently became totally blind from a cystic tumor compressing the optic chiasm. Based on prevailing theories on the nature of diabetes, Pauw attributed the disease to the kidneys. Undoubtedly, however, his lucid report is the earliest known account of diabetes insipidus caused by an arachnoid cyst, the Rathke cleft cyst, or craniopharyngioma in the region of the pouch of Rathke. The description also gives insights into the role of anatomic dissections in late 16th-century northern Europe.

Long-term health risk of primary hyperparathyroidism: the effect of surgery.

Primary hyperparathyroidism is a common disease but 90% of sufferers remain undetected. In elderly women its prevalence is about 1%. The disease can cause severe hypercalcaemia and lead to life-threatening symptoms. However, the majority of cases represent milder forms of hyperparathyroidism with minimal symptoms or no symptoms at all, and slight hypercalcaemia which does not progress during follow-up. Surgery is the only effective treatment of primary hyperparathyroidism and most of the symptoms respond favourably to surgery. Nevertheless, the role of surgery has been controversial in the treatment of mini-symptomatic patients. Recently it has become evident that primary hyperparathyroidism is associated with increased mortality due mainly to cardiovascular diseases, and it has been shown that surgical treatment is able to diminish or even eliminate this risk of premature death. The extra mortality is connected also to the mild and nonprogressive forms of the disease, and it seems to have a strong correlation with the duration of the disease. This aspect favours active treatment at an early stage of the disease. Conservative treatment can be considered only in old patients with mild and stable clinical course. Primary hyperparathyroidism is an important risk factor causing morbidity and mortality.

Hyponatremia after transsphenoidal surgery for pituitary tumors.

We studied the incidence of postoperative hyponatremia in 91 consecutive patients (44 males and 47 females; age, 45 yr; range, 12-76) operated on transsphenoidally for pituitary tumors. A postoperative serum sodium concentration less than 135 mmol/L (the lowest, 109 mmol/L) was observed in 32 (35%) patients. Hyponatremia occurred most commonly in patients operated on for Cushing's disease (11 of 18 patients; 61%). Hyponatremia was symptomatic in 18 (56%) of the patients. Neither the size nor the operability of the tumor or transient postoperative polyuria predicted the development of hyponatremia. Hyponatremia was first observed on the sixth or seventh postoperative day. The patients were treated with water restriction and by increasing the hydrocortisone replacement dose in the case of ACTH deficiency, and recovery took place, on the average, within 5 days. High urinary osmolality and plasma arginine vasopressin concentration during hyponatremia in a subgroup of study patients with these measurements indicated that inappropriate vasopressin secretion was involved in the pathogenesis of hyponatremia. In conclusion, postoperative hyponatremia after transsphenoidal surgery is common and may put the patients at increased risk of severe hyponatremic symptoms. Therefore, all patients should be screened for serum electrolytes for 1 week after transsphenoidal surgery.

Autoantibodies to cytochrome P450 enzymes P450scc, P450c17, and P450c21 in autoimmune polyglandular disease types I and II and in isolated Addison's disease.

Patients with idiopathic Addison's disease have autoantibodies reacting with adrenal cortex. If Addison's disease is associated with other endocrine immune diseases like autoimmune polyglandular diseases (APD) type I and type II, antibodies may recognize all steroid-producing cells. We showed previously that one antigen recognized by APD-I sera is the cytochrome P450c17 hydroxylase. We have now looked for antibodies to P450c17 and to two other key enzymes in the steroid biosynthetic pathway, the P450scc and P450c21, in a series of patients with isolated Addison's disease (8 patients) or with APD-I or APD-II (50 and 9 patients, respectively). The result of antienzyme antibodies were further correlated with the immunofluorescence pattern against adrenal gland, testis, ovary, and placenta, and with the clinical findings presented. In APD-I patients with Addison's disease and in APD-II patients, antibodies to at least one of the P450 enzymes were frequently found (positive findings in 81% and 78%, respectively). Such antibodies were less frequent in APD-I patients without Addison's disease (21%) and in the isolated Addison cases (25%). In APD-I, antibodies recognized as frequently P450c17 and P450scc, specific for all steroid-producing cells as the adrenal specific enzyme P450c21. In contrast, patients with APD-II or with the isolated Addison's disease reacted almost exclusively with P450c21. Immunofluorescence studies showed good correlation with the known fact that the zona glomerulosa of the adrenal cortex is devoid of the P450c17, that the Leydig cells of the testis and the theca interna cells of the ovary express P450c17 and P450scc, and that the placental trophoblasts express only P450scc. The presence of antibodies to P450scc or to at least one of the tested P450 enzymes correlated significantly to gonadal failure in the females but not in the males.