[Nesidioblastosis as extracolonic manifestation associated with adenomatous familial polyposis]. / Nesidioblastosis como una manifestación extracolónica asociada a poliposis familiar adenomatosa.

OBJECTIVE: To describe the case of a patient with familial adenomatous polyposis (FAP) who developed organic hyperinsulinemia and hypoglycemia due to pancreatic nesidioblastosis. BACKGROUND: FAP can be considered as one of the familial cancer syndromes. The development of endocrine tumors in these patients is an infrequent event, and most of these tumors have been well-differentiated thyroid neoplasms. To our knowledge, the occurrence of organic hyperinsulinemia in the context of FAP has not been reported. METHODS: Organic hyperinsulinemia was demonstrated during a 72-h fast, measuring insulin and C-peptide by RIA at the time of hypoglycemia. Immunohistochemistry for insulin and other pancreatic hormones was performed on the surgical pancreatic specimen. RESULTS: The patient underwent a distal pancreatectomy with resolution of hypoglycemia. Histopathologically, the excised pancreas showed beta cell hyperplasia arising from the ductal epithelium. Such beta cells strongly immunostained for insulin, pancreatic polypeptide, and neuron-specific enolase. CONCLUSIONS: The development of endocrine tumors in an inherited cancer syndromes such as FAP is rare. Such an association may suggest shared mechanisms of tumorigenesis. The case herein reported should prompt us to consider organic hyperinsulinism as an extracolonic manifestation of FAP.

[Cushing syndrome. The diagnostic and therapeutic challenges]. / Síndrome de Cushing. Desafíos diagnósticos y terapéuticos.

Endogenous hypercortisolism is characteristically a condition that should be diagnosed and treated in tertiary care centers with the participation of several specialists. Cushing's syndrome represents the clinical expression of a prolonged exposure to glucocorticoids, independently of its origin. The term Cushing's disease refers to the hypercortisolism that results from the excessive secretion of corticotropin (ACTH) by a pituitary microadenoma. The mechanisms that give rise to the different forms of hypercortisolism are complex and its precise differential diagnosis is one of the major challenges in modern endocrinology. This review focuses on current aspects of the pathophysiology, differential diagnosis and treatment of Cushing's syndrome.

Differential effects of octreotide treatment and transsphenoidal surgery on growth hormone-binding protein levels in patients with acromegaly.

OBJECT: The high-affinity growth hormone-binding protein (GHBP) represents the extracellular portion of the growth hormone (GH) receptor, and its serum levels are a reflection of the tissue receptor status. Levels of GHBP are decreased in patients with active acromegaly, probably because of downregulation of GH receptors. However, there are no studies of patients with acromegaly in which the effects of medical (that is, administration of somatostatin analogs) and surgical therapy on GHBP levels have been compared. That is the task the authors set out to accomplish in this study. METHODS: The authors studied seven patients in whom acromegaly had been recently diagnosed. They examined these patients at baseline, 2 months after octreotide treatment (subcutaneous administration of 100 microg octreotide three times per day), and 1 month after transsphenoidal surgery. Growth hormone-binding activity was measured, as well as the following biochemical markers of the somatotropic axis: GH suppression induced by oral administration of glucose, insulin-like growth factor-I (IGF-I), and insulin-like growth factor-binding protein-3 (IGFBP3). Although octreotide treatment induced a decrease in the levels of GH, IGF-I, and IGFBP3, as well as an increase in the level of GHBP, these biochemical markers did not reach normal levels. On the other hand, after transsphenoidal surgery, GHBP levels became normal, particularly in those patients in whom serum GH could be suppressed to an undetectable level after glucose loading. CONCLUSIONS: The authors conclude that persistently low GHBP levels in patients with acromegaly are normalized by successful pituitary surgery and correlate well with disease activity.