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Surgery in active acromegaly with prognathism and a lateral open bite.

The case of a 33-year-old Caucasian with acromegaly is presented. The maxillo-facial deformity with the rare combination of unilateral open bite is demonstrated. The patient underwent surgical treatment of the pituitary gland three times. In the last operative procedure the sella was found to be empty, but despite this the growth hormone level appeared to be too high. Nevertheless, it was decided to correct the mandibular deformity. Risks during and after mandibular osteotomy are discussed, and the results are shown.

Acromegaly↗

Osteon cross-sectional size in the iliac crest: variation in normals and patients with osteoporosis, hyperparathyroidism, acromegaly, hypothyroidism and treated epilepsia.

Cross-sectional osteon size was measured in undecalcified stained sections of iliac crest bone specimens from normal individuals (n = 68) and from patients with spinal osteoporosis (n = 27), primary hyperparathyroidism (n = 23), epilepsia (receiving chronic anti-convulsant therapy) (n = 11), acromegaly (n = 18), and hypothyroidism (n = 12). In each individual the shortest osteon diameter (D) and the corresponding Haversian canal diameter (d) were measured in a minimum of 20 completed secondary osteons by means of a micrometer eyepiece. Among normal males the areas of bone resorbed and formed increased with age (p less than 0.01), owing to an increase in the thickness of bone resorbed (p less than 0.01) and an unchanged thickness of bone formed. Among the females, both the areas of bone resorbed and formed decreased with age (p less than 0.05), owing to a reduction in the thickness of bone resorbed (p less than 0.05) as well as formed (p less than 0.001). Resorbed and formed areas were reduced in the epileptic (p less than 0.01) and acromegalic (p less than 0.01) groups but increased in the hypothyroid group (p less than 0.01) compared to sex- and age-matched controls. Neither the osteoporotic nor the hyperparathyroid group showed any alterations in osteon size. The Haversian canal diameter was slightly increased in the epileptic group but normal in the other patient groups. The observed changes reflect variations in the amount of work performed by osteoclasts and osteoblasts during bone remodelling and may be explained by variations in cellular activity and bone turn-over rates.

Acromegaly↗

[Surgical treatment of acromegaly by the trans-sphenoidal approach. II. Hormonal results].

Growth hormone determinations were done in the serum of 45 patients with acromegaly. In 3 cases the basal GH levels was normal, in the remaining ones it was raised. Of the functional tests those with insulin, glucose and L-Dopa were done. The tests with glucose and L-Dopa loading were found to be more useful diagnostically than the insulin test. After surgical treatment the GH level returned to its normal value in 10 cases, but in most cases it fell to at least one half of the initial value. In 5 cases with more pronounced expansion of the pituitary tumour the GH value was not significantly changed.

Acromegaly↗

Cardiovascular pathology in acromegaly and some effects of the 90 yttrium implant in the hypophysis.

Examination of the cardiovascular apparatus (cv) of 25 acromegalic patients revealed an increased incidence of cardiovascular pathology as against normal individuals. Acromegalic patients with arterial hypertension (AH) show a twice higher incidence of ischemic cardiopathy, cardiomegaly with obvious or latent cardiac failure, arrhythmias, which frequently lead to death. The uncertain pathogeny of cardiomyopathy implied: hypoxia due to heart hypertrophy (over 500 g and increased cross-section of the fibre to over 26/mu), adrenergic deficiency resulting from increased protein synthesis and decrease in thyrosine, a precursor of myocardic cathecolamines. The increased incidence of coronary pathology is favoured in acromegalic patients by diabetes, hyperlipoproteinemia, low endogenous heparin, increased ureic acid and platelet adhesivity. Hypophysectomy by hypophysolysis (20 cases) brings relief and cures cardiovascular pathology, reduces AH, corrects the coronary risk factors, cures hypophyseal diabetes. The existence of AH and cardiovascular pathology is an indication for surgical treatment in evolutive acromegaly.

Acromegaly↗

The dyslipemic syndrome in acromegaly.

The clinico-hormonal course of acromegaly and the presence of the hyperlipemic syndrome were studied in relation to age, sex, associated hypothyroidism, diabetes, obesity and cardiovascular and atherosclerotic complications in 43 acromegalic patients (18 males and 25 females) of which 6 had received no treatment and 37 had been submitted 2 - 13 years to conventional roentgentherapy (31 cases), 90Y (5 cases) and hypophysectomy (1 case). Hyperlipemia (HLP), present in 24 acromegalic patients (55.8% of the cases) unrelated to age, was more frequent in women (64% as against 44% in males) and correlated with the clinico-hormonal evolution (GH greater than 20 mg) (60% of the cases), and associated hypothyroidism (79%), obesity (42%) and diabetes (25%). Of the hyperlipemic acromegalic patients, 62% had cardiovascular and atherosclerotic complications. The results of the study point to the need for a hypoglucidic, hypolipidic and associated treatment for hypothyroidism, diabetes and obesity in the prophylaxis of acromegalic atherosclerosis.

Acromegaly↗

The acromegaly syndrome. Relation between clinical features, growth hormone values and radiological characteristics of the pituitary tumours.

One hundred and fifty-five acromegalics, 76 males (mean age 44 +/- 1.3 years) and 79 females (45 +/- 1.4 years) were studied. The frequency of clinical features were: acral enlargement 100 per cent, hyperhidrosis 65 per cent, headache 55 per cent, paraesthesiae 49 per cent, cardiac problems 34 per cent, hypertension 32 per cent, diabetes mellitus (clinical and chemical) 27 per cent, and visual field defects 6 per cent. Signs and symptoms, and particularly headache, did not show any relation with the size or shape of pituitary tumours, nor with growth hormone (GH) values, age, sex or weight. Mean of GH values at 60, 90 and 120 minutes during a GTT averaged 135 mIU/l, range 8-1833. Diabetes mellitus was more frequent and severe in patients with higher GH values, occurring in 32 per cent of patients with mean GH values greater than or equal to 50 mIU/l and only in 16 per cent of the rest (p less than 0.05). GH values correlated positively with size of tumours. Forty-nine per cent of patients presented with entirely intrasellar tumours, 27 per cent with suprasellar extensions and 23 per cent with partially empty sellae. The mean GH (238 mIU/l) of patients with suprasellar extensions was significantly higher (p less than 0.004) than those of the others. Younger acromegalics showed a tendency to larger tumours. Early treatment of acromegaly, particularly in young patients and those presenting with high GH values, is recommended.

Acromegaly↗

Additional antitumoral effects of bromocriptine and radiotherapy in patients with prolactinomas or acromegaly.

Thirteen patients with prolactin-secreting and/or growth hormone secreting pituitary tumours have been treated with bromocriptine in doses of about 10 mg/day for several months. Nine of these patients were previously submitted to external or interstitial radiotherapy and one case to pituitary microsurgery. Serum prolactin concentration in patients with prolactinomas was still very high within one year after pituitary irradiation or operation, i.e. 5,125.6 +/- 974 mU/l (mean +/- SEM). It has been reduced to normal level only during bromocriptine therapy, i.e. to 329.1 +/- 88mU/l (p less than 0.001), and increased thereafter, but remained to a significantly lower concentration than before bromocriptine treatment, i.e. 2,709.0 +/- 553 mU/l (p less than 0.05). Serial pneumonecephalotomography has demonstrated the reduction of tumour size afrer bromcriptine therapy to two prolactinomas with suprasellar extension. In patients with acromegaly the effects of bromocriptine were less evident. The antitumoral effects of bromocriptine on prolactin-secreting adenomas is independent and additional to the effects of pituitary radiotherapy or microsurgery, and has therapeuticical implications.

Acromegaly↗

Acromegaly, hyperparathyroidism, and pheochromocytoma in the same patient. A multiple endocrine disorder.

In the course of follow-up of a patient with primary hyperparathyroidism, signs and symptoms of acromegaly developed. The patient subsequently was found to have recurrent primary hyperparathyroidism and, later, pheochromocytoma was discovered. The patient seems to have an overlap of features found in the multiple endocrine neoplasia syndromes, type 1 and type 2 as previously classified.

Acromegaly↗

Rarity of diabetic retinopathy in patients with acromegaly.

We studied 44 acromegalic patients with the use of fluorescein angiography to detect the presence of diabetic retinopathy. We found elements of diabetic retinopathy in only one patient (2.2%). The average patient had a growth hormone concentration of 32 ng/mL (range, 1 to 275 ng/mL) for a duration of 10.5 years (range, three to 26 years). Forty-five percent (20) of our group had glucose intolerance and 14% (six) had elevated fasting blood glucose levels for an average duration of two years (range, one to four years). We conclude that diabetic retinopathy is an uncommon finding in patients with acromegaly and that fluorescein angiography does not reveal cases that were not detected by conventional funduscopic examination.

Acromegaly↗

Acromegaly with an adenomatous pharyngeal pituitary.

A patient with acromegaly was found to have a discrete adenoma of the pharyngeal pituitary lodged within the sphenoid sinus. Removal of this adenoma, believed to be complete, did not normalize the growth hormone secretion. At the time of surgery, inspection and biopsy of the pituitary gland failed to disclose the presence of any identifiable intrasellar adenoma; one year later, however, definite radiological changes indicated the presence of an intrasellar mass. The underlying pathophysiology in this patient may have been due to diffuse hyperplasia of hypersecreting nonsuppressible somatotrophs, with adenoma formation secondary to the hyperplasia.

Acromegaly↗

[Pre- and postoperative endocrinologic findings in patients with acromegaly in transsphenoidal surgery].

34 (69%) of 49 patients suffering from acromegaly had normal growth hormone levels after transnasal-transsphenoidal adenotomy. The operation impaired the other pituitary endocrine functions in a few cases only, these being improved in other cases. There were few operative complications (meningitis 3, significant peroperative bleeding 3, longer lasting diabetes insipidus 5). Transnasal-transsphenoidal selective adenomectomy with the aid of a dissecting microscope has a lower morbidity and mortality than the transfrontal approach. Its results are good in comparison to other therapies, especially if the postoperative complications and endocrine losses of function are considered.

Acromegaly↗

[Long term results of the surgical treatment of acromegaly. Longitudinal study of a series of 68 patients (author's transl)].

In order to evaluate treatment of acromegaly, a 5-year study was undertaken on 68 acromegalics, selected from a series of 155 cases, treated by surgery alone (31 cases) or by combined surgery and radiotherapy (37 cases). Immediate return to normal of STH levels in the post-operatoire period is rare (7%) and the clinical improvement observed in most instances seems to be explained by the relative fall in STH that is usually found. Blood volume appears to be the best metabolic parameter of STH levels. Rapid return to normal of STH levels in the post-operative period is a good pronostic sign, but the authors emphasize that STH secretion can vary in the same patient after surgery alone or after combined surgery and radiotherapy. The pituitary insufficiency observed in 35% of cases is more frequent after limited surgical excision followed by radiotherapy or after wide excision alone. In 70% of cases, patients with pituitary insufficiency retain raised STH levels.

Acromegaly↗

Treatment of acromegaly, Cushing disease and Nelson syndrome.

Since 1957 we have treated more than 429 patients who had pituitary neoplasms, most of which were hormone-secreting tumors. Long-term follow-up in the large group of patients treated for acromegaly shows a median survival of better than 16 years, with improvement over time. The short-term follow-up results in patients with Cushing* disease, Nelson syndrome and chromophobe adenoma are very encouraging. To compare these excellent results with those following surgical procedures, a large study of patients followed for a long period after the operations is needed.

Acromegaly↗

Medical management of acromegaly with bromocriptine. Effects of continuous treatment for over three years.

Eighty-seven patients with active acromegaly were treated with bromocriptine for periods of up to 42 months. On treatment, 82 of these patients noted a rapid alleviation of one or more symptoms of their disease. In 67 patients, the mean growth hormone level of four values taken through the day fell by 14 mu/L or more, or became undetectable. Eighty patients have been treated for over 12 months and this suppression was maintained. Improvement in abnormal glucose tolerance occurred in 25 out of 29 diabetics and tolerance became normal in 19. Bromocriptine is of great value in cases in which surgery or radiotherapy has not been completely effective or as interim treatment until the full effect of other therapy has taken place. However, the long-term effect of high doses of bromocriptine is not yet known.

Acromegaly↗

Treatment of acromegaly with octreotide: effectiveness and tolerability of its pulsatile administration by portable pump.

This study was undertaken to evaluate effectiveness and tolerability of octreotide administered in active acromegaly by pulsatile means and compare these data with intermittent three-times-a-day therapy. We studied 13 acromegalics with active disease. All patients received octreotide subcutaneously administered in a pulsatile way using a portable pump delivering 25 micrograms every 120 minutes and in an intermittent way (100 micrograms three times daily) (TID). From pretreatment values (56.5 +/- 13.4 micrograms/L) the 24-h integrated mean GH levels (IC-GH) were significantly reduced both during pulsatile and TID octreotide administration (P < 0.01). IC-GH was significantly lower during pulsatile therapy (17.0 +/- 5.2 micrograms/L) than during TID (22.0 +/- 11.5 micrograms/L; P < 0.05). Before octreotide, IGF-I levels were 669.8 +/- 85.7 micrograms/L; during octreotide therapy they were reduced in 12/13 patients (TID 340.2 +/- 41.5 micrograms/L, pulsatile 338.1 +/- 55.3 micrograms/L; P < 0.01). A correlation between IC-GH and IGF-I levels was observed only during TID administration of octreotide (R = 0.652; P < 0.05). The 24-hour GH pattern fluctuated widely before the start of octreotide therapy. During TID administration, GH levels tended to rise again before the following octreotide injection; this did not occur during pulsatile therapy. Side effects were fewer during pulsatile (15%) than TID (31%) octreotide administration (NS). Asymptomatic gallstones appeared in 1 patient. In conclusion subcutaneous pulsatile octreotide administration in acromegalic patients by means of a small portable pump seems able to produce, a steadier control of GH-IGF-I hypersecretion and fewer side effects than TID administration at same dosage.

Acromegaly↗

[The sleep apnea syndrome in patients with acromegaly].

The authors examined 19 nonselected patients with acromegaly (8 women and 11 men), incl. 16 where the disease was hormonally active, 3 had normal growth hormone values, 11 patients had a surgical operation of the pituitary. The examination was made by the MESAM 4 method (all-night recording of the heart rate, respiratory sounds, body position and arterial oxygen blood saturation). The sleep apnoea syndrome was diagnosed in 10 patients (52.6%). In patients where the sleep apnoea syndrome was not confirmed by MESAM 4, so-called intermittent snoring was found, an indirect sign of impaired ventilation during sleep.

Acromegaly↗

[The effects of somatostatin in acromegaly].

Somatostatine is the hypothalamic factor which inhibits the secretion of growth hormone. The administration of a synthetic form decreased growth hormone levels by 50 to 75% in 5 acromegalic patients. The action is rapid but the effect is not prolonged. Prolactin was reduced in only case with galactorrhea. Thyreostimulin, as well as gastrin, are unaffected. Plasma insulin levels, and to a lesser extent those of glucagon, are decreased by somatostatine which causes no variation in either cortisol or blood glucose. Somatostatine, by correcting the pathological secretion of hormone, opens the way to medical treatment of acromegaly.

Acromegaly↗

[The CD2, CD4 and CD8 mRNA expression of T lymphocytes in patients with acromegaly and hyperprolactinemia].

The CD2, CD4 and CD8 mRNA expression of human peripheral blood lymphocytes (HPBL) from 49 female patients with acromegaly and hyperprolactinemia and 15 healthy women were studied by dot blotting assay using corresponding CD2, CD4 and CD8 antisense RNA probes. It was found that CD2 mRNA expression increased in women with growth hormone over-expression and the ratio of CD4 and CD8 mRNA declined significantly in the women with prolactin over-expression. These suggested that the mRNA expression of these cluster differentiation antigens in peripheral T lymphocytes was affected by the pituitary hormones. The significance of these findings relevant to the study of the neuroendocrine and immune network was discussed.

Acromegaly↗