Ashley Grossman

..MRI is used for characterising adrenal adenomas, problem solving in difficult cases and for detecting ACTH-secreting pituitary adenomas...

..It is essential to perform validated diagnostic procedures and manage such patients with clear objectives and based on well-established replacement programs...

..This article summarizes the discussion and recommendations derived from that session...

..She did not take tobacco, recreational drugs or alcohol. Upon physical examination she was grossly Cushingoid with florid clinical manifestations...

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..We hypothesized that the physiological IGF-I/GH negative feedback loop may be reset in somatotroph adenomas, specifically in terms of the level of expression of these receptors or mutations of the GH receptor (GH-R) in such tumours...

..These pathways share a common root in the tyrosine kinase receptor, and a change to these receptors or their relationship to membrane matrix-related proteins may be an early event in tumorigenesis...

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..Development of new somatostatin analogues and new drug combination therapies should further improve the clinical management of these patients...

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..We speculated that this same gene may be either mutated at this site, or overexpressed, in pituitary adenomas...

..To define the test characteristics of an enzyme immunoassay (EIA) for plasma-free metanephrines (metanephrine and normetanephrine) in the diagnosis of pheochromocytoma and paraganglioma...

..The management of paediatric CD patients after cure also presents challenges for optimizing growth, bone health, reproduction and body composition from childhood into and during adult life...

..CONCLUSIONS :Our data suggest that the antiproliferative action of rosiglitazone is probably not via PPAR-gamma...

..VHL-related tumours tend to be adrenal phaeochromocytomas, frequently bilateral and associated with a milder phenotype...

..These cases require a prolonged follow-up, review, and repetition of diagnostic tests and scans...

..The effect of GH replacement on thyroid function in hypopituitary patients has hitherto been studied in small groups of children and adults with conflicting results...

..A protocol for investigation of the child with suspected Cushing's syndrome is presented followed by principles of management...

..The management of pediatric CD patients after cure also presents challenges for optimizing growth, bone health, reproduction and body composition from childhood into and during adult life...

..Mutations have been identified in the aryl hydrocarbon receptor-interacting protein (AIP) gene in familial isolated pituitary adenomas (FIPA). It is not clear, however, how this molecular chaperone is involved in tumorigenesis...

..The aim of this study was to describe the clinical manifestations of subjects with SDH-B gene mutations...

..The management of paediatric CD patients after cure also presents challenges for optimizing growth, bone health, reproduction and body composition from childhood into and during adult life...

..This article will aim to provide an overall view of pediatric CS highlighting some of the differences between adult and pediatric CS...

..GH deficiency occurred in 86% patients. Long-term follow-up suggests some recovery of GH secretion and preservation of other anterior pituitary function...

..These data suggest that variations in the ghrelin gene contribute to obesity in children and may modulate glucose-induced insulin secretion...

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..Increasing data link ghrelin to the overall control of energy use and flow in situations where there is a limitation of energy sources and ghrelin appears to play a pivotal role in energy homeostasis...

..Familiarity with the range of imaging appearances of the adrenal glands in primary adrenal Cushing syndrome may help establish the underlying diagnosis...

..Our data suggest that increased p27 degradation through the ubiquitin-proteasome pathway could be regulated in pituitary tumors by changes in Skp2 expression, although other factors probably also play a role...

..These data suggest that ghrelin might have widespread physiological effects via different, partly unidentified, subtypes of the GHS-R in endocrine and non-endocrine tissues...

..04). This study demonstrates that joint analysis of several factors involved in apoptosis, proliferation and cell cycle regulation, but not AI alone, might provide additional prognostic information in NSCLC patients...

..Maintenance of the quality of life should be a priority, particularly because patients with disseminated disease may experience prolonged survival...

..Identification of GOAT expression in various tissues support the concept that in addition to the important endocrine effect of acylated ghrelin, the paracrine effects of locally synthetised and acylated ghrelin may be important...

..The most reliable indicator of malignancy in these tumors remains the presence of metastases. Treatment is essentially surgical, although the role of mitotane is one undergoing evaluation...

..Recently, a quantitative trait locus for stature was reported on chromosome 3p26 in patients with type 2 diabetes...

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..Protein expression analysis of pituitary samples disclosed both novel proteins and putative protein candidates for pituitary tumorigenesis, though validation using conventional techniques are necessary to confirm the protein array data...

..In the current study, we demonstrate the effect of high glucocorticoid levels on AMPK activity of human adipose tissue samples from patients with Cushing's syndrome...

..Recent studies have illustrated the interaction between hormones and AMPK, and highlighted AMPK as a potential target for the development of tissue-specific AMPK modulators in the treatment of obesity and the metabolic syndrome...

..More potent and broader-spectrum SST analogues are likely to play an increasing role in the treatment of tumours, where the MAP kinase pathway is overactivated...

..Relative mRNA expression was not decreased in any of the sporadic pituitary tumour samples. The present data thus do not suggest a major role for the PRKAR1A tumour suppressor gene in sporadic GH-secreting or other pituitary tumours...

..Awareness of the imaging characteristics will aid identification of the source of ACTH production and allow potentially curative surgical resection...

..CONCLUSIONS: Expression array analysis of pituitary adenomas using the Affymetrix GeneChip HG-U133A arrays appears to be a valid method of identifying genes that may be important in tumour pathogenesis...

..Few patients with macroadenomas were cured by surgery. CONCLUSIONS: Pituitary macroadenomas causing Cushing's disease have biochemical features largely distinct from patients harboring microadenomas but represent one end of a continuum...

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..Whether AMPK changes are one of the causes or the consequence of these pathological conditions remains a matter of debate, but AMPK clearly represents a major potential pharmacological target in the treatment of these conditions...

..Functional characterization of AIP changes is important to identify the functional impact of gene sequence variants...

..Preoperative localisation of insulinoma improves cure rate and reduces complications, but may be challenging...

..Ghrelin and its receptor play an important role in glucose metabolism and energy homeostasis, and therefore they are functional candidates for genes carrying susceptibility alleles for type 2 diabetes...

..There does not appear to be any necessity for retaining the high-dose dexamethasone suppression test in this diagnostic work-up...

..Our data suggest that metformin treatment could be effective in preventing the metabolic complications of chronic glucocorticoid excess...

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..In conclusion, female preponderance of CD in adult patients was not present in childhood. In patients 18 yr of age or younger, there was no difference in the severity of hypercortisolemia or ACTH at diagnosis between males and females...

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..However, ghrelin has also been shown to have anti-proliferative effects on breast, lung and thyroid cell lines. We therefore examined the effect of ghrelin on the rat pituitary cell line GH3...

..The hyperghrelinemia in PWS adults is therefore not solely explained by their reduced visceral adiposity and relative hypoinsulinemia. Its cause and consequences await further elucidation...

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..We review and assess the role of the beta-catenin signaling pathway in the pathogenesis of pituitary adenomas...

..The development of new SSA, new drug combination therapies and chimaeric molecules should further improve the clinical management of these patients, as should a more complete understanding of their mode of action...

..The biochemical results should be interpreted jointly with the clinical aspects and the radiology findings in a probabilistic matrix, and not as part of a uniform algorithm...

..Evidence regarding the accuracy of [(123)I] metaiodobenzylguanidine (MIBG) imaging for phaeochromocytoma localization is currently limited to small series...

..CONCLUSIONS: Although the sensitivity of the 72-h fast is high and still plays an important role in the diagnosis of an insulinoma, we suggest that a "normal" test result should be interpreted in the light of clinical symptoms...

..This study highlights the possibility of using retinoic acid as a novel therapy in the treatment of ACTH-secreting tumors in humans with Cushing's disease...

..Ongoing microarray studies may provide novel intracellular pathways of importance for proliferation/cell cycle control, and lead to the development of novel pharmacological agents...

..Adrenal imaging complements and assists the clinical and hormonal evaluation of adrenal tumours...

..We found the use of intraoperative neuronavigation to be an excellent aid in overcoming such anatomic difficulties...

..However, no effective drug that directly and reliably targets the adrenocorticotropin-secreting pituitary adenoma has yet been found...

..Our data demonstrate that AMPK not only links the orexigenic effects of endocannabinoids and ghrelin in the hypothalamus but also their effects on the metabolism of peripheral tissues...

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..We explored the mechanism of action of octreotide, RAD001, and their combination on cell proliferation and kinase activation in a neuro-endocrine tumour cell line (rat insulinoma cell line, INS1)...

..This is an important diagnosis to make as rapidly as possible to allow the early institution of effective therapy...

..Here, we review the current understanding of pathogenesis, clinical features, diagnostic, and differential diagnostic approaches. We provide diagnostic algorithms and recommendations for management...

..The cannabinoid receptor type 1 (CB1) antagonist rimonabant can block the orexigenic effect of ghrelin. In this study, we have elucidated the mechanism of the putative ghrelin-cannabinoid interaction...

..Inadequate methods to distinguish malignant from benign tumors and a lack of effective treatments for malignancy are important problems requiring further resolution...

..The management of paediatric CS patients after cure also presents challenges for optimizing growth, bone health, reproduction and body composition from childhood into and during adult life...

..These observations explain the beneficial role of macrophage migration inhibitory factor neutralization in models of inflammatory arthritis...

..CONCLUSION: The significantly lower frequency (P = 0.028) of germline SDH subunit mutations in familial PC only cases compared to those with familial PC and HNPGL suggests that further PC susceptibility gene(s) remain to be identified...