Graves' disease in children.
Abstract Graves' disease is the most frequent cause of hyperthyroidism in children. It may occur at any time during childhood, but its frequency increases with age, peaking during adolescence. Antithyroid drugs (ATDs; methimazole or carbimazole) are usually recommended as the initial treatment and are generally well tolerated. The overall frequency of relapse is higher in children than in adults, with remission achieved in only 30% of children after a first course of treatment for about 2 years. More prolonged medical treatment may increase the remission rate to up to 50%. The identification of factors pre...
Source: Endocrine Development - September 20, 2014 Category: Endocrinology Authors: Léger J Tags: Endocr Dev Source Type: research

Thyroid nodules and differentiated thyroid cancer.
Abstract The incidence of thyroid nodules and thyroid cancer has increased over the last several decades. This change is influenced by several factors, to include the status of iodine sufficiency, access to the health care system and, perhaps most significantly, the increased use of radiological imaging. Thorough evaluation of a thyroid nodule includes review of the patient's medical and family history, followed by thyroid ultrasound and fine-needle aspiration biopsy. Similar to adults, the majority of nodules in children are benign; however, there is a 3- to 5-fold higher risk that a nodule found in a ped...
Source: Endocrine Development - September 20, 2014 Category: Endocrinology Authors: Bauer AJ Tags: Endocr Dev Source Type: research

Medullary thyroid carcinoma in children.
Abstract Medullary thyroid carcinoma (MTC) originates from thyroid parafollicular C cells, and it accounts for 5% of thyroid malignancies. MTC is sporadic in approximately 80% and hereditary in 20% of cases. When hereditary it can be associated with other benign endocrine neoplasias and/or typical nonendocrine diseases, thus configuring the multiple endocrine neoplasia syndromes (type 2, MEN2/familial MTC, FMTC). Sporadic MTC is usually diagnosed in adult life. Children with clinically evident MTC are belonging to MEN2 families, particularly MEN2A and MEN2B. Children belonging to families with FMTC are usu...
Source: Endocrine Development - September 20, 2014 Category: Endocrinology Authors: Viola D, Romei C, Elisei R Tags: Endocr Dev Source Type: research

Clinical case seminar in pediatric thyroid disease.
Abstract Pediatric thyroid diseases cover a large spectrum of congenital and acquired forms, ranging from congenital primary or central hypothyroidism, autoimmune thyroid disease, iodine deficiency, rare genetic defects of thyroid hormone action, metabolism and cell membrane transport to benign nodules and malignant tumors. The previous 15 papers of the textbook Paediatric Thyroidology gave a systematic overview of the current knowledge and guidelines on all these diseases. In this final paper, the authors collected a series of patient histories from their clinics illustrating frequently encountered clinic...
Source: Endocrine Development - September 20, 2014 Category: Endocrinology Authors: Szinnai G, Léger J, Bauer AJ, Pearce EN, Ramos HE, Canalli MH, Onigata K, Elisei R, Radetti G, Polak M, Van Vliet G, Deladoëy J Tags: Endocr Dev Source Type: research

Ghrelin discovery: a decade after.
Abstract Since its discovery 12 years ago, intensive research has been performed on ghrelin. The significance of ghrelin as a growth hormone-releasing hormone, appetite regulator, energy conservator and sympathetic nerve suppressor has now been well established. In this short essay, we summarize the history of the discovery of ghrelin. PMID: 23652386 [PubMed - in process] (Source: Endocrine Development)
Source: Endocrine Development - May 25, 2013 Category: Endocrinology Authors: Kojima M, Kangawa K Tags: Endocr Dev Source Type: research

The Ghrelin Receptors (GHS-R1a and GHS-R1b).
Abstract The growth hormone (GH) secretagogue receptor (GHS-R1a) is a G protein-coupled receptor (GPCR) expressed in the brain as well as other areas of the body. In the early 1990s, this receptor was expression cloned in MERCK laboratories by using a group of synthesized small molecules known to increase GH release in humans and other animals. Since its discovery, hundreds of studies have shown the importance of this receptor and its endogenous ligand, ghrelin, in metabolism, neurotransmission, and behavior. Even more relevant are the prospective benefits that will result from pharmacologic manipulation o...
Source: Endocrine Development - May 25, 2013 Category: Endocrinology Authors: Albarrán-Zeckler RG, Smith RG Tags: Endocr Dev Source Type: research

Discovery of ghrelin o-acyltransferase.
Abstract Ghrelin is a gut hormone with potent orexigenic and growth hormone release stimulatory effects, and is the first known endogenous ligand of the growth hormone secretagogue receptor. A notable feature of ghrelin is that it carries an acyl group, in most cases an octanoyl group, in the third serine. While it has been shown that the acylation is critical for the majority of ghrelin functions, the mechanisms of acylation of ghrelin remained poorly understood. In 2008, it was discovered that ghrelin O-acyltransferase (GOAT) is the enzyme responsible for acylating ghrelin. GOAT is highly conserved from ...
Source: Endocrine Development - May 25, 2013 Category: Endocrinology Authors: Mohan H, Unniappan S Tags: Endocr Dev Source Type: research

Genetics of the ghrelin system.
Abstract Ghrelin was originally identified as a growth hormone secretagogue, but later studies suggested that it plays an important role in the regulation of appetite and body weight. The implication of genetic variants of the ghrelin-ghrelin receptor axis in the determination of stature, appetite, body weight, glucose metabolism and eating disorders has been the focus of numerous small and larger studies. More recently, several studies have shown some involvement of ghrelin and growth hormone secretagogue receptor (GHSR) genetic variants in some cancers, or their role in the genetics of immune diseases or...
Source: Endocrine Development - May 25, 2013 Category: Endocrinology Authors: Gueorguiev M, Korbonits M Tags: Endocr Dev Source Type: research

Ghrelin and the gut.
Abstract Ghrelin is the endogenous ligand for the growth hormone secretagogue receptor (GHS-R) which was identified in the pituitary gland and is now named the ghrelin receptor. However, the peptide is most abundant in the stomach and ghrelin receptors are present in all major organ systems and tissues. Ghrelin forms with motilin, a new gut peptide family and the sequence similarities of peptides and receptors suggest they evolved by gene duplication. Nevertheless, no cross-reactivity exits between both peptides. Ghrelin shares with motilin motor effects in the gut, in particular gastric emptying and the i...
Source: Endocrine Development - May 25, 2013 Category: Endocrinology Authors: Peeters TL Tags: Endocr Dev Source Type: research

Ghrelin as a GH-Releasing Factor.
Abstract Growth hormone (GH) is secreted by the pituitary gland in a pulsatile manner. It is accepted that this pulsatility is primarily controlled by the hypothalamus, although this secretion can also be modulated by factors from GH-targeted organs, the pituitary and other regions of the central nervous systems, or by factors arriving from peripheral organs. In mammals, hypothalamic control of GH pulsatility is classically regulated by the interplay of two opposing hormones, stimulatory GHRH and inhibitory somatostatin (SS). Recognition of the gastric ghrelin peptide as the natural ligand for GH secretago...
Source: Endocrine Development - May 25, 2013 Category: Endocrinology Authors: Carreira MC, Crujeiras AB, Andrade S, Monteiro MP, Casanueva FF Tags: Endocr Dev Source Type: research

Other than growth hormone neuroendocrine actions of ghrelin.
Abstract Besides its growth hormone-releasing effect, ghrelin has been demonstrated to influence other hormonal systems, such as the hypothalamo-pituitary-adrenal axis, prolactin secretion, the thyroid axis as well as the gonadal axis. Ghrelin and its analogues stimulate the hypothalamo-pituitary-adrenal axis independent of the pituitary, via the hypothalamus, involving both corticotrophin-releasing hormone, arginine-vasopressin and neuropeptide Y stimulation. In adrenocortocotropic hormone (ACTH)-secreting tumors, the ghrelin receptor is pathologically expressed, thus accounting for especially high ACTH a...
Source: Endocrine Development - May 25, 2013 Category: Endocrinology Authors: Benso A, Calvi E, Gramaglia E, Olivetti I, Tomelini M, Ghigo E, Broglio F Tags: Endocr Dev Source Type: research

Ghrelin, the gonadal axis and the onset of puberty.
Abstract Puberty onset and reproduction are metabolically gated; conditions of energy insufficiency are associated to perturbations in the timing of puberty and subfertility. The neuroendocrine basis for this phenomenon relies on the regulatory actions of a diversity of nutritional and metabolic cues as well as hormones controlling energy homeostasis at different levels of the reproductive axis. Among these, experimental evidence from different species, including rodents and primates, has accumulated in recent years to strongly suggest that the gut hormone, ghrelin, which operates as signal of energy insuf...
Source: Endocrine Development - May 25, 2013 Category: Endocrinology Authors: Tena-Sempere M Tags: Endocr Dev Source Type: research

Ghrelin and the cardiovascular system.
Abstract Although ghrelin was initially associated with regulation of appetite, the cardiovascular system has also been recognized as a potentially important target for its effects. Moreover, experimental and a limited number of clinical studies suggest a potential role for ghrelin in the treatment of congestive heart failure. So far, reported cardiovascular effects of growth hormone secretagogues and/or ghrelin include lowering of peripheral resistance, either direct at the vascular level and/or by modulating sympathetic nervous activity. Other observed effects indicate possible improvement of contractili...
Source: Endocrine Development - May 25, 2013 Category: Endocrinology Authors: Isgaard J Tags: Endocr Dev Source Type: research

Ghrelin - a key pleiotropic hormone-regulating systemic energy metabolism.
Abstract The gastrointestinal peptide hormone ghrelin was discovered in 1999 as the endogenous ligand for the growth hormone secretagogue receptor (GHSR-1a). Since its discovery tremendous research efforts have been directed at unraveling ghrelin's mechanisms of action, revealing that ghrelin is a pleiotropic hormone implicated in myriad of molecular signaling mechanisms. Accordingly, ghrelin is the only known circulating peripheral hormone with the ability to promote a positive energy balance by stimulating food intake while decreasing energy expenditure and body fat utilization. Moreover, beyond its abil...
Source: Endocrine Development - May 25, 2013 Category: Endocrinology Authors: Müller TD, Tschöp MH Tags: Endocr Dev Source Type: research

Ghrelin, reward and motivation.
Abstract Almost all circulating gut peptides contribute to the control of food intake by signalling satiety. One important exception is ghrelin, the only orexigenic peptide hormone thus far described. Ghrelin secretion increases before meals and behavioural and electrophysiological evidence shows that ghrelin acts in the hypothalamus via homeostatic pathways to signal hunger and increase food intake and adiposity. These findings strongly suggest that ghrelin is a dynamically regulated peripheral hunger signal. However, ghrelin also interacts with the brain reward pathways to increase food intake, alter foo...
Source: Endocrine Development - May 25, 2013 Category: Endocrinology Authors: Menzies JR, Skibicka KP, Leng G, Dickson SL Tags: Endocr Dev Source Type: research