Effects of an 8-week aerobic exercise training on saliva steroid hormones, physical capacity, and quality of life in diabetic obese men.

The purpose of the study was to assess the effects of aerobic exercise training on saliva steroid hormones [i. e., cortisol, dehydroepiandrosterone (DHEA), and testosterone], physical capacity, and quality of life in obese diabetic men. 8 abdominally obese type 2 diabetic men (59.5±1.7 years old, BMI=35.5±1.6 kg/m(2), waist circumference=119.4±3.3 cm) and 9 healthy men (57.4±1.5 years old, BMI=24.5±0.8 kg/m(2), waist circumference=92.3±1.9 cm) participated in the study. The obese diabetic men underwent 8 weeks of aerobic exercise training: twice a week 45 min sessions at 75% of peak heart rate and once a week 45 min session of intermittent exercise. Before and after training, steroid hormone concentrations were analyzed from saliva samples, physical capacity was assessed by the 6-minute walking test, and quality of life was estimated by a specific questionnaire for obese subjects. These data were compared with the data from the healthy untrained men. The basal saliva DHEA and testosterone concentrations, physical capacity, and quality of life scores of the obese diabetic men were significantly lower than those of the healthy men. Aerobic training induces a significant increase in the 6-min walking distance and improve the psychosocial impact dimension of quality of life, without modifying significantly any other parameter investigated. These data suggest that an 8-week aerobic exercise program improves physical capacity and quality of life in obese diabetic men, but was insufficient to correct the anthropometric and hormonal alterations observed in this population.

Serum and saliva adrenocortical hormones in obese diabetic men during submaximal exercise.

The aim of this study was to evaluate serum and saliva adrenocortical hormones and their relationships at rest and during submaximal exercise and recovery in 9 obese diabetic middle-aged men (BMI: 35.2 ± 1.6 kg/m (2)). Blood and saliva samples were taken at rest, every 10 min of a 30-min cycling exercise at 70% of maximal heart rate, and after 10 min of recovery in order to analyze cortisol, dehydroepiandrosterone sulfate (DHEA-S) and dehydroepiandrosterone (DHEA). Serum and saliva cortisol increased significantly during recovery (p<0.05), but no significant difference was observed between the rest, exercise, and recovery DHEA-S and DHEA concentrations. A strong correlation was found at rest between both serum and saliva cortisol (r=0.72, p<0.001) and DHEA-S and DHEA (r=0.93, p<0.001). Serum DHEA-S and saliva DHEA remained strongly correlated during and after the submaximal exercise (r=0.81, p<0.001), whereas a weaker but still significant relationship was observed between serum and saliva cortisol during and after the exercise (r=0.52, p<0.001). In conclusion, these results suggest that saliva adrenocortical hormones, and especially saliva DHEA, may offer a practical surrogate for serum concentrations during both rest and exercise in obese diabetic men.

Clinical characterization of familial isolated pituitary adenomas.

CONTEXT: Familial pituitary adenomas occur rarely in the absence of multiple endocrine neoplasia type 1 (MEN1) and Carney complex (CNC). OBJECTIVE: Our objective was to characterize the clinical and genealogical features of non-MEN1/CNC familial isolated pituitary adenomas (FIPA). DESIGN AND SETTING: We conducted a retrospective study of clinical and genealogical characteristics of FIPA cases and performed a comparison with a sporadic population at 22 university hospitals in Belgium, Italy, France, and The Netherlands. RESULTS: Sixty-four FIPA families including 138 affected individuals were identified [55 prolactinomas, 47 somatotropinomas, 28 nonsecreting adenomas (NS), and eight ACTH-secreting tumors]. Cases were MEN1/PRKAR1A-mutation negative. First-degree relationships predominated (75.6%) among affected individuals. A single tumor phenotype occurred in 30 families (homogeneous), and heterogeneous phenotypes occurred in 34 families. FIPA cases were younger at diagnosis than sporadic cases (P = 0.015); tumors were diagnosed earlier in the first vs. the second generation of multigenerational families. Macroadenomas were more frequent in heterogeneous vs. homogeneous FIPA families (P = 0.036). Prolactinomas from heterogeneous families were larger and had more frequent suprasellar extension (P = 0.004) than sporadic cases. Somatotropinomas occurred as isolated familial somatotropinoma cases and within heterogeneous FIPA families; isolated familial somatotropinoma cases represented 18% of FIPA cases and were younger at diagnosis than patients with sporadic somatotropinomas. Familial NS cases were younger at diagnosis (P = 0.03) and had more frequently invasive tumors (P = 0.024) than sporadic cases. CONCLUSIONS: Homogeneous and heterogeneous expression of prolactinomas, somatotropinomas, NS, and Cushing's disease can occur within families in the absence of MEN1/CNC. FIPA and sporadic cases have differing clinical characteristics. FIPA may represent a novel endocrine neoplasia classification that requires further genetic characterization.

[Duodenal somatostatinomas associated with von Recklinghausen's neurofibromatosis. Apropos of 2 cases]. / Somatostatinomes duodénaux et neurofibromatose de von Recklinghausen.

Somatostatinomas are rare neuroendocrine tumors; they are essentially located in the pancreas and in the duodenum. The association with a neurofibromatosis type I is especially observed when the tumor is located in the ampulla of Vater. These tumors are not associated with a "somatostatin syndrome", but often present with gastrointestinal bleeding, abdominal pain and obstructive jaundice. The diagnosis is confirmed by immunohistochemical studies. The aim of this study is to report 2 cases of metastazing duodenal periampullary somatostatinomas associated with von Recklinghausen's disease and to discuss the prognosis of these tumors. Future genetic research are necessary as point out the familial feature of this association in one of our cases.

[Paresthetic notalgia and multiple endocrine neoplasia type 2a (Sipple's syndrome): 3 cases]. / Notalgie paresthésique et néoplasie endocrinienne multiple de type IIA (syndrome de Sipple): 3 cas.

INTRODUCTION: Notalgia paresthetica is an isolated sensory mononeuropathy. Patients have a pruritus in the mid-upper back. Its association with multiple endocrine neoplasia type IIA has been reported in a few cases. We report three cases of this association. CASE REPORTS: Case n(o) 1: A 45 year-old woman had multiple endocrine neoplasia type IIA with a medullary thyroid carcinoma and a primary hyperparathyroidism; she had a mid-upper back pigmented lesion. Histological examination showed dermal melanosis and deposits of amyloid in the dermis. Case n(o) 2: A woman had a multiple endocrine neoplasia type IIA which was diagnosed at the age of 60; she had a surgical treatment for a pheochromocytoma, a medullary thyroid carcinoma, and a primary hyperparathyroidism; she had dermatological examination for a pruriginous lesion of the mid-upper back. Case n(o) 3: The daughter of the patient n(o) 2 had had a surgical cure for a medullary thyroid carcinoma and a pheochromocytoma at the age of 31; she had a papulous and pruriginous lesion in the left scapular area. Her daughter and her sister had a multiple endocrine neoplasia type IIA without notalgia paresthetica. DISCUSSION: Notalgia paresthetica is a benign cutaneous disorder which can be associated with multiple endocrine neoplasia type IIA. It can be considered that notalgia paresthetica is an early clinical marker of multiple endocrine neoplasia type IIA. Patients with a familial history of notalgia paresthetica or with an onset of notalgia paresthetica in childhood should be screened for multiple endocrine neoplasia type IIA. Patients with multiple endocrine neoplasia must also been screened for notalgia paresthetica because its finding is an argument for a familial form of multiple endocrine neoplasia type IIA. Dermatologists should be aware of this association.

[Microscopic thyroid papillary cancers presenting as cervical lymph node metastases]. / Métastases ganglionnaires cervicales révélatrices des micro-cancers papillaires de la thyroïde.

Microscopic papillary thyroid cancer (< 1 cm in diameter) is reputed to have an excellent prognosis. In 10 to 20% of cases, it presents in the form of lymph ode metastases. Immediately metastatic forms can be associated with unfavourable prognostic factors, such as multifocal tumours, extension to adjacent tissues, capsular effraction and development of distant metastases. We report 4 cases of microscopic papillary thyroid cancer presenting in the form of lymph node metastases. No primary thyroid lesion was palpable in any of these patients, but the subsequent course was complicated by ling metastases in one case. All patients underwent total thyroidectomy with lymph node dissection followed by adjuvant therapy with radioactive iodine and thyroxin inhibitory treatment. Lung metastases were observed in one case. The authors propose a therapeutic approach based on analysis of severity factors, which determine the recurrence rate.

Cytotoxic therapy with etoposide and cisplatin in advanced adrenocortical carcinoma.

Adrenocortical carcinoma (ACC) is a rare tumour with a poor prognosis. Cisplatin is the most widely tested cytotoxic agent in this disease. A total of 18 patients with advanced ACC were enrolled. Cytotoxic therapy consisted of etoposide (VP16) (100 mg m(-2) day(-1) on days 1-3) and cisplatin (100 mg m(-2) day(-1) on day 1) every 4 weeks. Mitotane treatment was maintained during chemotherapy in 14 patients. A complete response was observed in three cases and a partial response in three cases, giving an overall response rate of 33%. Tumour response was observed in three of the six patients with progressive disease during treatment with mitotane given at an effective dosage, as shown by serum levels >14 mg l(-1). Toxic effects were as expected and were non-life-threatening; no treatment interruption was required.

Germ-line mutation analysis in patients with multiple endocrine neoplasia type 1 and related disorders.

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant syndrome predisposing to tumors of the parathyroid, endocrine pancreas, anterior pituitary, adrenal glands, and diffuse neuroendocrine tissues. The MEN1 gene has been assigned, by linkage analysis and loss of heterozygosity, to chromosome 11q13 and recently has been identified by positional cloning. In this study, a total of 84 families and/or isolated patients with either MEN1 or MEN1-related inherited endocrine tumors were screened for MEN1 germ-line mutations, by heteroduplex and sequence analysis of the MEN1 gene-coding region and untranslated exon 1. Germ-line MEN1 alterations were identified in 47/54 (87%) MEN1 families, in 9/11 (82%) isolated MEN1 patients, and in only 6/19 (31.5%) atypical MEN1-related inherited cases. We characterized 52 distinct mutations in a total of 62 MEN1 germ-line alterations. Thirty-five of the 52 mutations were frameshifts and nonsense mutations predicted to encode for a truncated MEN1 protein. We identified eight missense mutations and five in-frame deletions over the entire coding sequence. Six mutations were observed more than once in familial MEN1. Haplotype analysis in families with identical mutations indicate that these occurrences reflected mainly independent mutational events. No MEN1 germ-line mutations were found in 7/54 (13%) MEN1 families, in 2/11 (18%) isolated MEN1 cases, in 13/19 (68. 5%) MEN1-related cases, and in a kindred with familial isolated hyperparathyroidism. Two hundred twenty gene carriers (167 affected and 53 unaffected) were identified. No evidence of genotype-phenotype correlation was found. Age-related penetrance was estimated to be >95% at age >30 years. Our results add to the diversity of MEN1 germ-line mutations and provide new tools in genetic screening of MEN1 and clinically related cases.

Iron overload in reticuloendothelial systems of pediatric oncology patients who have undergone transfusions: MR observations.

OBJECTIVE: Pediatric oncology patients who undergo intensive chemotherapy develop anemia caused by myeloid suppression that necessitates transfusions that, in turn, cause iron deposition in the reticuloendothelial system. We describe MR imaging of iron overload in pediatric patients who underwent such chemotherapy and who have solid and hematologic tumors. MATERIALS AND METHODS: The MR appearance of the liver, spleen, and bone marrow was evaluated in 13 children with both solid (n = 10) and hematologic (n = 3) malignant lesions using known criteria for the presence of iron deposition. Findings were correlated with transfusional history, chemotherapeutic regimens, and ferritin levels. RESULTS: MR imaging obtained after chemotherapy and transfusional therapy revealed signs of iron deposition in the liver and spleen, particularly on T2-weighted images. Bone marrow signal intensity varied among patients. Pancreatic signal intensity was normal. Ferritin levels were elevated in all patients. CONCLUSION: Reticuloendothelial system iron deposition present in follow-up MR imaging of pediatric solid and hematologic malignant lesions reflected the intensity of the chemotherapeutic regimen, the degree of myeloid suppression, and the resultant transfusional requirements. Such iron deposition appeared to have no effect on cardiac, liver, or pancreatic function.

[Mediastinal parathyroid adenomas on a 5th ectopic gland. 2 case reports]. / Les adénomes parathyroïdiens médiastinaux sur 5ème glande ectopique. Deux observations.

Mediastinal parathyroid adenoma located on the 5th ectopic gland is rare. We report here two new cases diagnosed by scintigraphy. In one case the adenoma was found to be located in the mediastinum prior to cervicotomy. The modern imaging methods capable of locating parathyroid adenomas are evaluated.

[Influence of dose and modalities of administration of BIM 23014 on growth hormone secretion in patients with acromegaly]. / Influence de la dose et des modalités d'administration du BIM 23014 sur la sécrétion d'hormone de croissance de l'acromégale.

Somatostatin analogs, with prolonged half-lives have been proposed for the treatment of acromegalics. The aim of the study was to evaluate the short term efficacy of different doses and modalities of administration of the new somatostatin analog, BIM 23014 (BIM), on GH secretion in acromegalics. Ten acromegalics, with evolutive disease, who previously had had transsphenoidal surgery (and pituitary radiotherapy in 8) were evaluated in a three step study. The first part included four patients who received in a random order either vehicle or 500, 1000 and 1500 micrograms BIM for a day as a continuous s.c. infusion using programmable pumps at one-week interval for 24 hours to measure plasma GH levels. The second part included six patients who received in a random order either vehicle or 1500 micrograms/24h BIM as 500 micrograms x 3 s.c. injections, 750 micrograms x 2 s.c. injections and a continuous s.c. infusion using programmable pumps at one-week interval. During each period of the study blood was sampled at 4 hour intervals for 24 hours in order to measure plasma GH and BIM levels by radioimmunoassays. The third part of the study included the same 6 patients as the second part, who received 30 mg IM of a long acting formulation of BIM. Blood was sampled before and thereafter on days 1, 3, 6, 9, 12, 15, 18 and 21 following the injection for measurement of plasma GH and BIM levels. In first group 500 micrograms BIM slightly decreased plasma GH levels.(ABSTRACT TRUNCATED AT 250 WORDS)

[Polymyositis, dermatomyositis and pregnancy: high-risk pregnancy. A further case report and review of the literature]. / Polymyosites, dermatomyosites et grossesse: grossesse à haut risque. Nouvelle observation et revue de la littérature.

The authors report a new case where polymyositis/dermatomyositis and pregnancy were associated. The polymyositis and dermatomyositis appeared in the last third of the pregnancy. A caesarean operation had to be performed for fetal distress after 37 weeks of amenorrhoea. An apparently dead small-for-dates baby was born. Progress, however, after resuscitation of the baby was good. At 20 months after delivery the polymyositis and dermatomyositis also were improved. Two and a half years later there was no recurrence. In the light of cases that have previously been reported in the literature, it has been established that on the one hand pregnancy may trigger off the condition and worsen the PM/DM and on the other hand the condition has a bad effect on the progress of the pregnancy. 50% of the babies are born dead or die in the week after delivery and 55% are born prematurely. We discuss the possibility that PM/DM is of viral origin. A confirmation of this is the presence of viral inclusion bodies on electron microscopy found in the muscle cells of the patients who have PM/DM. However, in the previous history of these patients an acute viral myositis in childhood is a troublesome factor. Pregnancy, because it involves alterations in the immune responses of the patients, may lead to a recrudescence of a "sleeping" virus and so be responsible for the appearance or aggravation of polymyositis and dermatomyositis.

[Adrenomyeloneuropathy. Endocrine, clinical and biological study of 4 cases]. / Adrénomyéloneuropathie. Etude endocrinienne, clinique et biologique de quatre observations.

Adrenoleukomyeloneuropathy (ALMN) is a rare neurological and endocrine disorder. It usually affects children, and consists of cortical disease and adrenal insufficiency (adrenoleukodystrophy: ALD). An adult form has recently been identified in which medullary disease is associated with an endocrine disorder (adrenomyeloneuropathy AMN). The authors describe the clinical, biological and physiopathological features of four cases of AMN.