ABSTRACT. In 1985, a follow‐up investigation was performed of all patients with acromegaly (n = 166, 89 women and 77 men) seen over a 30‐year period (1955–1984) at our Endocrine Unit, referral centre for the western region of Sweden (population 1.5 million). At the end of 1984, the prevalence of the disease was 6.9 per 105 inhabitants, the average incidence per year was 3.3 per million people. Mean age at diagnosis was 46.3 years and at death (n=62) 64.1 years. About one‐third of the patients (50/153), in whom it had been possible to estimate the total duration of the disease, had survived for more than 25 years. Mortality was, however, increased compared to the normal population. The observed number of deaths from vascular and malignant disorders was 32 and 15, respectively, compared to the expected numbers 9.0 (p<0.001) and 5.6 (p<0.01).

We studied a man who sought medical attention at age 28 years because of infertility in both his first and second marriages. His sexual development appeared to have been normal, with normal puberty and virilization, and normal libido and sexual potency. At examination, his testicles were small and soft; otherwise he had a normal physical appearance. Evaluations revealed azoospermia, undetectable in serum before and after 100 microg of intravenously administered gonadotrophin releasing hormone, but moderately elevated lutropin concentration with a brisk rise after gonadotrophin releasing hormone. The alpha subunit concentration was normal before and after gonadotrophin releasing hormone; that of inhibin B was very low. Analysis of the follitropin beta gene, exon 3, revealed a Cys82 --> Arg mutation (TGT --> CGT). Judging from studies of the biosynthesis of the chorionic gonadotrophin beta subunit one may conclude that inability to form the first intramolecular disulphide bond in the follitropin beta subunit results in an abnormal tertiary structure during follitropin beta biosynthesis with extensive intracellular degradation of the products, inability to associate with the alpha subunit and defective glycosylation, as well as inability to form a biologically active hormone. This first male case of follitropin deficiency thus defines a new syndrome of male infertility.

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ABSTRACT. During the years 1956–1982, 64 pheochromocytoma patients were operated upon without mortality. Twenty‐eight patients had sustained hypertension and 29 paroxysmal hypertension only. In two patients high blood pressure was not related to pheochromocytoma and five subjects were normotensive. In two women the pheochromocytoma demonstrated malignancy by widespread metastases. Sixteen patients also had neuroectodermal manifestations other than pheochromocytoma. Preoperatively, heart disease was found in most of the hypertensive patients aged 50 years or more at operation, but was uncommon in the others. In these subjects, heart disease persisted after surgery. Young subjects with sustained hypertension were not less affected by preoperative cerebrovascular accidents than older subjects. After surgery, hypertension persisted in 12 patients, and was easily controlled by drug therapy in eight. Nine patients died 7 months‐18 years after surgery. In no case was the death directly associated with the pheochromocytoma disease. Three died from other neuroectodermal abnormalities. The 55 surviving patients have been followed up for a mean of 12 years after surgery. During the observation time the survival of the pheochromocytoma patients was similar to that of the normal population. At the end of the study, 44 out of the 55 surviving patients were free from symptoms.

SUMMARY Forty-four patients with Cushing's disease underwent bilateral adrenalectomy and 39 survivors were followed for, on an average, eight years and a half. There were three postoperative deaths and two other patients died within one year after operation. Thirty patients are now working but six remain invalidized. In four subjects cortisol secretion persisted in spite of "total" adrenalectomy but a clinical remission ultimately occurred. Five patients, who presented with an atypical clinical picture, failed to show any obvious changes in the distribution of fat or in the complexion. Body weight did not decrease after surgery in several patients and in many others body weight increased again after an initial loss of weight. Menstruations reappeared in all premenopausal women except two. Manifest diabetes, which was found in seven subjects, disappeared and in several other cases glucose tolerance improved. The blood pressure, which was high in most patients, normalized or decreased in all hypertensive patients but the blood pressure still remains elevated in 22 subjects. ECG-abnormalities disappeared in 11 of 17 subjects, who presented with ST-T changes and/or high amplitude R waves. The size of the heart decreased in most patients with cardiomegaly. A remarkable improvement in the radiological appearance of the vertebrae occurred in a 16-year-old boy but in the other patients there were no such changes. Mental disturbances, which were a main cause of preoperative disability, disappeared in most cases. Two patients, however, continue to be invalidized from mental disease, which developed during the course of the hypercorticism. One patient presented with a grossly evident pituitary tumour and in four other subjects a large pituitary tumour developed after adrenalectomy together with hyperpigmentation. Pituitary apoplexy occurred in one of these patients and another was operated upon because of visual field losses. It is concluded that the remission obtained after adrenalectomy for Cushing's disease increases the survival rate and makes a normal life possible in most patients. However, persistent hypertension and cardiovascular disease as well as the possibility of the development of a pituitary tumour make the late prognosis somewhat uncertain.