[Management of acromegaly in 2003].
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Biomedical subjects
Publications and source records attributed to E Requeda.
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Osteoporosis is a common complication of Cushing's disease/syndrome. Fractures can be the presenting manifestation. We report two cases and review 28 others from the literature. Clinical, laboratory, and absorptiometry data are reviewed; the vertebrae and ribs were the most common fracture sites, and osteoporosis reversal after treatment varied with age, gender, and symptom duration. The pathophysiology, which remains controversial, is discussed. A search for Cushing's disease/syndrome should be part of the routine evaluation of osteoporosis in children and adults, particularly men.
Non-Invasive coronary investigations are positive in 12 to 52% (average 22%) of type II diabetics, and 11 to 30% (average 17%) of type i diabetics. These statistics vary according to bias of recruitment. Haemodynamic lesions are found at coronary angiography in 35 to 80% of patients who have at least one positive non-invasive investigation. Nine to 12% of diabetics have silent myocardial ischaemia (SMI) confirmed by coronary angiography, compared with 1.3 to 5.3% of non-diabetic controls paired for age and sex. The higher frequency of SMI in diabetics seems to be mostly due to the increased frequency of ischaemic heart disease in diabetics. The importance of cardiac autonomic neuropathy (CAN) in SMI is controversial. The risk factors associated with SMI are those usually associated with coronary artery disease: age, masculine gender, hypercholesterolaemia, hypertriglyceridaemia, hypertension, smoking, a family history of cardiovascular disease, insulin therapy (for type II diabetes), proteinuria, retinopathy, peripheral occlusive arterial disease.... The French recommendations for investigating SMI seem to be contradictory. A single risk score in a given patient could help codify the investigation of SMI in diabetics, but this type of score has not yet been validated.
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Thirteen patients with a mass in the adrenal gland area discovered at ultrasonography or computed tomography were studied. Hormone levels were normal in all but three patients with adrenal insufficiency. With the exception of three patients with metastatic tumours or adrenal lymphoma, all were operated upon on account of complications or for diagnostic purposes. The pre-operative diagnosis was confirmed by histology in 5 out of 10 cases (tuberculous abscess in 2, cysts in 2 and 1 haematoma in 1 case). In the remaining 5 cases the tentative diagnosis was erroneous: these patients had haematoma, neurofibroma, schwannoma, leiomyosarcoma and angiomyolipoma respectively. Thus, ultrasonography and computed tomography do not always differentiate between adrenal and extra-adrenal masses and between malignancy and non-malignancy; surgical excision therefore seems to be desirable in such cases.
The mean age of the 13 patients studied (9 women, 7 men) was 50.5 +/- 15.7 years. The disease was discovered on account of malaise (3 cases), behavioural disorders (4 cases), coma (3 cases), syncope (1 case) or right hemiparesis (1 case) or in the course of systematic examination (1 case). Eleven patients consulted for evaluation of hypoglycaemia and 2 for behavioural disorders. The history was characteristic, with malaise, loss of consciousness, severe neurological disorders (seizures, hemiparesis, hemiplegia or coma) and psychiatric disorders. These symptoms typically occurred in the morning before breakfast or between meals in 9 patients, and atypically at any point of time or after meals in 4 patients. Their hypoglycaemic nature was demonstrated by blood glucose determination in 11/13 cases and by response to ingestion of sugar in 12/13 cases. The mean period elapsed between the initial symptoms and the final diagnosis was 20.3 +/- 17.3 months. Inappropriate insulin secretion was elicited a.m. before breakfast, during Conn's diet or fasting test, or by calculating the blood insulin/glucose ratio or Turner's coefficient. Prior to surgery, the insulinoma was located by ultrasonography in 3/8 cases, by computerized tomography in 2/6 cases, by selective arteriography in 6/11 cases, and by phlebography with spleno-portal catheterization and staged sampling for insulin and C-peptide assays in 8/9 cases. Histological examination after surgery (11 cases) or necropsy (1 case) showed an adenoma without evidence of malignancy.
The 20 women investigated were 22 to 59 years old (average 35.9 +/- 2.3 years) when the diagnosis of Sheehan's syndrome was confirmed. Fourteen had had previous pregnancies, 6 were primiparas; average parity was 4. The patients were delivered in hospital, in France, in 14 cases, and abroad (Algeria, Portugal) at home in 6 cases. A history of severe haemorrhage during delivery was recorded in all but 2 cases. This was serious enough to warrant hysterectomy in 2 cases. Agalactia and amenorrhea were observed in all but 1 case in the immediate post-partum period. Transient polyuria and polydipsia occurred in 3 patients. In the months following birth, most women lead restricted lives; they were apathetic, asthenic and indifferent. The diagnosis was established under three different circumstances: in the majority (15/20) on clinical grounds, in 3 cases after acute adrenal failure, in 2 cases, fortuitously. Clinical examination showed signs of global anterior hypophyseal insufficiency involving thyroid, adrenal and gonad stimulating hormones in 14 cases; in 6 cases, the pituitary failure was dissociated. Corticotrophin, somatotropin and prolactin deficiencies were observed in all patients; gonadotrophin (17/20) and thyrotrophin (16/20) deficiencies were common. The diagnosis was confirmed less than 1 month (2 cases) to over 12 years (7 cases) after obstetric haemorrhage (average 6.9 +/- 1.9 years). No correlation was observed between the severity of the syndrome and this time interval. The short term outcome was favourable with return of menstruation and even pregnancy in one patient.(ABSTRACT TRUNCATED AT 250 WORDS)
Three cases of mucous cysts, or Rathke's cyst, of the pituitary gland are reported. The clinical and biological features were comparable to those of a non-secreting adenoma. Although extremely rare, these cysts must be recognized since their particular and, in theory, specific characteristics (wall made up of a ciliated epithelium with mucus cells and cavity filled with a mucoid substance) distinguish them from other intra or suprasellar pathological formations, especially craniopharyngiomas. The 62 cases reported in the literature suggest that they have a favourable prognosis.
In order to explain the discrepancy between the patency rate (80%) and the pregnancy rate (46%) in a series of vasovasostomies, attention was focused on a group of patients who became normospermic. The mean age at vasectomy, the duration of vasobstruction, and the parameters of semen analysis were not different for those couples who achieved a pregnancy (n = 8), compared with those couples without pregnancy (n = 7). In the group with pregnancy, six of the eight patients had low titers of serum agglutinins (absent to 1:32), and the fertilizing capacity of their spermatozoa was normal. None had immobilizing antibodies. In the group without pregnancy, six of the seven patients had elevated serum agglutinins (greater than 1:256), and four had agglutinating antibodies in their seminal plasma as well as serum immobilizing antibodies. The spermatozoa of seven patients failed to fertilize zona-free hamster ova. It is concluded that a loss of fertilizing ability of the spermatozoa due to sperm antibodies is an important cause of infertility in vasovasostomized men.
Scintigraphy was used in 66 patients with biochemically demonstrated hyperactivity of the adrenal cortex in order to determine the nature and site of the lesions. In cases of hypercortisolism, uptake was bilateral in 12 patients with Cushing's disease, unilateral in 7 patients with malignant or non-malignant tumours, and absent in 3 cases of large malignant tumours. In cases of hyperaldosteronism, scintigraphy performed during dexamethasone-induced ACTH suppression showed distinctly asymmetrical uptake in 13 patients with Conn's adenoma (confirmed by surgery as being on the good uptake side in 10 patients), symmetrical in 20 patients with biochemical findings indicating bilaterality, and intermediate in 9 patients. There was no false positive diagnosis of tumour. Scintigraphy appears to be of considerable value for locating adrenocortical lesions, especially small tumours.
Six women aged from 17 to 30 years (mean: 21 years) were referred on account disorders which had begun at puberty and had been present for 3 to 15 years. The reasons for consulting were hirsutism in 5 cases and sterility in one. The patients' height (1.61 to 1.70 m; mean: 1.64 m) and weight (54 to 70 kg; mean: 59 kg) were normal. Three women menstruated regularly and 3 had anovular spaniomenorrhaea. Hirsutism with enlargement of the clitoris (Prader's stage I) was apparent in all six cases. Three patients had permanent, though moderate hypertension. The biochemical changes essential to the diagnosis were as follows: 1) in 2 women with 21-hydroxalyse deficiency there was a rise in cortisol precursors (plasma 17-OH progesterone and its urinary metabolite, pregnanetriol). In 4 women with 11-hydroxylase deficiency urinary 17-OH corticosteroids were increased; two had high desoxycorticol levels. A rise in plasma desoxycortisol/cortisol ration under tetracosactrin stimulation is of considerable diagnostic value; 2) plasma androgens (testosterone, delta 4 androstenedione) and their urinary metabolites (17-ketosteroids) were increased; 3) all abnormalities disappeared when the adrenal function was suppressed. Under dexamethasone treatment hirsutism became stabilized or even regressed, blood pressure values rapidly returned to normal, menstrual disorders disappeared and ovular cycles were established. Three women became pregnant and delivered on term.