Objective: The aim was to provide guidelines for evaluation, treatment, and genetic testing for multiple endocrine neoplasia type 1 (MEN1). Participants: The group, which comprised 10 experts, including physicians, surgeons, and geneticists from international centers, received no corporate funding or remuneration. Process: Guidelines were developed by reviews of peer-reviewed publications; a draft was prepared, reviewed, and rigorously revised at several stages; and agreed-upon revisions were incorporated. Conclusions: MEN1 is an autosomal dominant disorder that is due to mutations in the tumor suppressor gene MEN1, which encodes a 610-amino acid protein, menin. Thus, the finding of MEN1 in a patient has important implications for family members because first-degree relatives have a 50% risk of developing the disease and can often be identified by MEN1 mutational analysis. MEN1 is characterized by the occurrence of parathyroid, pancreatic islet, and anterior pituitary tumors. Some patients may also develo...

/pdf/clinical-practice-guidelines-for-multiple-endocrine-iebfm32j0y.pdf

Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1)

This review focuses on the systemic complications of acromegaly. Mortality in this disease is increased mostly because of cardiovascular and respiratory diseases, although currently neoplastic complications have been questioned as a relevant cause of increased risk of death. Biventricular hypertrophy, occurring independently of hypertension and metabolic complications, is the most frequent cardiac complication. Diastolic and systolic dysfunction develops along with disease duration; and other cardiac disorders, such as arrhythmias, valve disease, hypertension, atherosclerosis, and endothelial dysfunction, are also common in acromegaly. Control of acromegaly by surgery or pharmacotherapy, especially somatostatin analogs, improves cardiovascular morbidity. Respiratory disorders, sleep apnea, and ventilatory dysfunction are also important contributors in increasing mortality and are advantageously benefitted by controlling GH and IGF-I hypersecretion. An increased risk of colonic polyps, which more frequently recur in patients not controlled after treatment, has been reported by several independent investigations, although malignancies in other organs have also been described, but less convincingly than at the gastrointestinal level. Finally, the most important cause of morbidity and functional disability of the disease is arthropathy, which can be reversed at an initial stage, but not if the disease is left untreated for several years.

/pdf/systemic-complications-of-acromegaly-epidemiology-48hlt1tw8j.pdf

Systemic complications of acromegaly: epidemiology, pathogenesis, and management.

Chronic sleep loss as a consequence of voluntary bedtime restriction is an endemic condition in modern society. Although sleep exerts marked modulatory effects on glucose metabolism, and molecular mechanisms for the interaction between sleeping and feeding have been documented, the potential impact of recurrent sleep curtailment on the risk for diabetes and obesity has only recently been investigated. In laboratory studies of healthy young adults submitted to recurrent partial sleep restriction, marked alterations in glucose metabolism including decreased glucose tolerance and insulin sensitivity have been demonstrated. The neuroendocrine regulation of appetite was also affected as the levels of the anorexigenic hormone leptin were decreased, whereas the levels of the orexigenic factor ghrelin were increased. Importantly, these neuroendocrine abnormalities were correlated with increased hunger and appetite, which may lead to overeating and weight gain. Consistent with these laboratory findings, a growing body of epidemiological evidence supports an association between short sleep duration and the risk for obesity and diabetes. Chronic sleep loss may also be the consequence of pathological conditions such as sleep-disordered breathing. In this increasingly prevalent syndrome, a feedforward cascade of negative events generated by sleep loss, sleep fragmentation, and hypoxia are likely to exacerbate the severity of metabolic disturbances. In conclusion, chronic sleep loss, behavioral or sleep disorder related, may represent a novel risk factor for weight gain, insulin resistance, and Type 2 diabetes.

http://sites.oxy.edu/clint/physio/article/sleeplossanovelriskfactorforinsulinresistance.pdf

Sleep loss: A novel risk factor for insulin resistance and Type 2 diabetes

Background: Previous guidelines for the management of thyroid nodules and cancers were geared toward adults. Compared with thyroid neoplasms in adults, however, those in the pediatric population ex...

/pdf/management-guidelines-for-children-with-thyroid-nodules-and-1aqe86ijrc.pdf

Management Guidelines for Children with Thyroid Nodules and Differentiated Thyroid Cancer

In evolutionary terms, GH and intracellular STAT 5 signaling is a very old regulatory system. Whereas insulin dominates periprandially, GH may be viewed as the primary anabolic hormone during stress and fasting. GH exerts anabolic effects directly and through stimulation of IGF-I, insulin, and free fatty acids (FFA). When subjects are well nourished, the GH-induced stimulation of IGF-I and insulin is important for anabolic storage and growth of lean body mass (LBM), adipose tissue, and glycogen reserves. During fasting and other catabolic states, GH predominantly stimulates the release and oxidation of FFA, which leads to decreased glucose and protein oxidation and preservation of LBM and glycogen stores. The most prominent metabolic effect of GH is a marked increase in lipolysis and FFA levels. In the basal state, the effects of GH on protein metabolism are modest and include increased protein synthesis and decreased breakdown at the whole body level and in muscle together with decreased amino acid degradation/oxidation and decreased hepatic urea formation. During fasting and stress, the effects of GH on protein metabolism become more pronounced; lack of GH during fasting increases protein loss and urea production rates by approximately 50%, with a similar increase in muscle protein breakdown. GH is a counterregulatory hormone that antagonizes the hepatic and peripheral effects of insulin on glucose metabolism via mechanisms involving the concomitant increase in FFA flux and uptake. This ability of GH to induce insulin resistance is significant for the defense against hypoglycemia, for the development of "stress" diabetes during fasting and inflammatory illness, and perhaps for the "Dawn" phenomenon (the increase in insulin requirements in the early morning hours). Adult patients with GH deficiency are insulin resistant-probably related to increased adiposity, reduced LBM, and impaired physical performance-which temporarily worsens when GH treatment is initiated. Conversely, despite increased LBM and decreased fat mass, patients with acromegaly are consistently insulin resistant and become more sensitive after appropriate treatment.

Effects of growth hormone on glucose, lipid, and protein metabolism in human subjects.

The alterations in metabolic parameters, circulating iodothyronines and serum TSH were studied during a 81 h period of insulin withdrawal in 6 young patients with juvenile type diabetes mellitus. Concomitant with the derangement of metabolic state a significant fall in serum T3 (in average 27%), serum free T3 (28%), and T4 (12%) was observed. Serum free T4 remained unchanged. Before and after the period of ketosis the normal diurnal pattern of high serum TSH at night and low levels during the daytime period was observed. During the period of ketosis the night level of serum TSH was significantly depressed (46 ± 9% lower at 23.00 h, p < 0.01) while no significant alterations occurred in daytime TSH. Both the variations in T3, reverse T3 (rT3) and night TSH were correlated to the increase in blood-3-hydroxybutyrate. The depression of the night surge in serum TSH may be of importance for the fall in circulating levels of active iodothyronines during the initial phase of illness, together with the well known inhibition of T4 deiodination to T3 in peripheral tissues occurring in acute illness.

Suppression of the night increase in serum TSH during development of ketosis in diabetic patients

Active acromegaly is characterized by inappropriate tissue growth, increased mortality, and perturbations of intermediary metabolism. It is, in general, not well described to which extent these disturbances are normalized after treatment of the disease. To further assess basal and insulin stimulated fuel metabolism in acromegaly six patients with monotropic GH excess were each studied approximately 1 month prior to and 2 months after successful selective pituitary adenomectomy and compared to a control population of seven subjects. The studies consisted of a 3-h basal postabsorptive period and a 2-h hyperinsulinaemic (0.4 mU/kg/min) euglycemic clamp and the methods employed included isotopical measurement of glucose turnover, indirect calorimetry, and the forearm technique. When compared to the control subjects the patients with acromegaly were preoperatively and in the basal state characterized by: 1) increased circulating concentrations of GH, insulin, and C-peptide (P less than 0.05); 2) increased plasma glucose (5.9 +/- 0.2 vs. 5.2 +/- 0.2 mmol/L), blood lactate (710 +/- 90 vs. 580 +/- 70 mumol/L), glucose turnover (2.34 +/- 0.12 vs. 1.93 +/- 0.12 mg/kg/min), and plasma lipid intermediates and a decreased forearm glucose uptake (0.06 +/- 0.02 vs. 0.19 +/- 0.04 mmol/L) (P less than 0.05); and 3) a 20% increase in energy expenditure, a 50% elevation of lipid oxidation rates, and a 130% elevation of nonoxidative glucose turnover (P less than 0.05). During the clamp the patients with active acromegaly were substantially resistant to the actions of insulin on both glucose and lipid metabolism. Following pituitary surgery all of these metabolic abnormalities were abolished. We conclude that active acromegaly is characterized by profound disturbances of not only glucose but also lipid metabolism, which in theory may precipitate the increased mortality in this disease. By showing that these abnormalities and the concomitant overall insulin resistance can be completely reversed our results may also have important implications for other insulin-resistant states and for the potential therapeutic use of GH.

Basal- and insulin-stimulated substrate metabolism in patients with active acromegaly before and after adenomectomy.

Under physiological circumstances growth hormone (GH) is secreted in bursts after the onset of sleep and a few hours postprandially. Because most relevant studies have employed constant or repeated infusion of high doses of GH, the possible metabolic effects of such bursts are largely unknown. We have studied seven healthy, male subjects for 7 h after an intravenous bolus of 1) 140 micrograms GH and 2) saline. When injected, serum GH rose to a peak of 21 +/- 3 micrograms/l 10 min after injection. GH caused 1) a rapid, sustained 55% decrease in forearm glucose uptake (P less than 0.05) followed by increases toward control values, 2) a delayed 5 mg/100 ml decrease in plasma glucose (P less than 0.05), and 3) significant 60-250% increases (P less than 0.05) in all measured lipid intermediates (nonesterified fatty acids, 3-hydroxybutyrate, and glycerol) 120-160 min after administration followed by decreases to below control values (P less than 0.05). GH did not influence circulating levels of insulin, C-peptide, glucagon, or insulin-like growth factor I (IGF-I), or isotopically determined glucose turnover. Physiological bursts of GH secretion appear to have acute insulin antagonistic effects with maximal effect on lipolysis after 2 h. These effects are reversed after 4 h. Therefore, GH could play a key role in regulation of diurnal rhythms of substrate levels and fuel utilization in humans.

Effects of a growth hormone pulse on total and forearm substrate fluxes in humans.

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Suppression of the night increase in serum TSH during development of ketosis in diabetic patients

Basal- and insulin-stimulated substrate metabolism in patients with active acromegaly before and after adenomectomy.

Effects of a growth hormone pulse on total and forearm substrate fluxes in humans.