1/48. Hypoglycaemia associated with the production of insulin-like growth factor ii and insulin-like growth factor binding protein 6 by a haemangiopericytoma.Non-islet-cell tumour-induced hypoglycaemia (NICTH) is, in most cases, attributable to tumour production of insulin-like growth factor ii (IGF-II). Tumour-derived IGF-II has a higher than normal molecular weight (big 'IGF-II') and an impaired ability to form the normal ternary 150 kD complex with IGF binding protein-3 (IGFBP-3) and the acid-labile subunit (ALS). Consequently, tumoral IGF-II circulates mainly in smaller binary complexes which have a higher bioavailability than the ternary complex. We had the opportunity to analyze IGFs and IGF-related factors in both pre- and post-operative blood, tumour tissue and tumour cyst fluid from a patient with a disseminated haemangiopericytoma and severe hypoglycaemia. In addition, the effect of serum and tumour cyst fluid on autophosphorylation of the insulin receptor was examined. Patient serum contained low levels of IGF-I, IGFBP-3 and ALS, while the concentrations of IGFBP-2 and IGFBP-6 were markedly elevated. The total level of circulating IGF-II was within the normal range, but Biogel P-60 gel filtration of patient serum revealed that 77% of the IGF-II was present in high molecular weight forms (normal: 10-15%), which decreased to 53% after partial removal of the tumour. Most of the IGF-II immunoreactivity in pre- and post-operative patient serum was associated with 50-60 kD complexes with only a minimal contribution (<10%) from the 150 kD complex. Tumour cyst fluid contained excessive amounts of both big IGF-II and IGFBP-6. Northern blot analysis of total mRNA isolated from the tumour demonstrated high expression of the IGF-II gene and abundant 1.1 kb IGFBP-6 transcript, while the genes encoding IGFBP-3, -4 and -5 were only weakly expressed and mRNA of IGFBP-1, -2 and IGF-I could not be detected. mRNAs for the IGF type II receptor could be easily demonstrated, whereas those for the insulin- and IGF type I receptor were hardly detectable. In contrast to patient serum tumour cyst fluid strongly stimulated the insulin receptor in vitro. The present study suggests an important role of the simultaneous production of IGF-II and IGFBP-6 in the pathophysiology of tumour-induced hypoglycaemia.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/48. gene expression and serum levels of insulin-like growth factors (IGFs) and IGF-binding proteins in a case of non-islet cell tumour hypoglycaemia.We describe a case of non-islet cell tumour hypoglycaemia (NICTH) associated with a renal cell carcinoma. serum insulin-like growth factors (IGFs) (including IGF-II E peptide), IGF-binding proteins (IGFBPs), insulin and c-peptide were measured before and after surgical removal of the tumour. IGFBPs were visualized by Western ligand blotting. Preoperatively 'big' IGF-II and IGFBP-2 levels were raised. IGF-I, IGFBP-1 and IGFBP-3 were low, while insulin, c-peptide and GH were undetectable. These changes were reversed by 2 days postoperatively. Protease assays showed little IGFBP-3 protease activity preoperatively. Preoperatively, neutral chromatography demonstrated most of the immunoassayable IGFBP-3 in a high molecular weight form with a small amount of IGF-II. Most of the IGF-II and big IGF-II eluted in lower molecular weight forms. Postoperative samples showed a shift in IGF-II which became increasingly associated with IGFBP-3 in both low and high molecular weight complexes. By Northern blotting, expression of all species of IGF-II mRNA in the tumour was 10-fold greater than in normal human liver. The tumour did not express IGFBP-1 or IGFBP-2. IGFBP-3 was expressed in small amounts, while the expression of IGFBP-4 was two-fold higher than in liver. In conclusion, we have confirmed high levels of big IGF-II and IGFBP-2 in NICTH, changes which are reversed postoperatively. The IGF-II is derived from the tumour which overexpresses these genes but IGFBP-2 probably arises from extratumour upregulation.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
3/48. Sequential hypoglycemia, hyperglycemia, and the carcinoid syndrome arising from a plurihormonal neuroendocrine neoplasm.OBJECTIVE: To describe the first patient with a neuroendocrine tumor secreting serotonin, insulin, and glucagon and having the corresponding clinical syndromes. methods: We present a detailed case report, including serial laboratory and clinical findings, in a man with type 2 diabetes, symptomatic hypoglycemia, and the carcinoid syndrome and in whom a plurihormonal metastatic neuroendocrine neoplasm was ultimately diagnosed. RESULTS: In a 58-year-old man with type 2 diabetes, which was treated effectively with a sulfonylurea for 7 years, episodes of hypoglycemia developed. After discontinuation of the orally administered agent, the hypoglycemic episodes initially resolved but then recurred and were associated with inappropriately increased plasma insulin concentrations. In addition, the patient had symptoms and biochemical evidence of the carcinoid syndrome. Computed tomography of the abdomen showed multiple hypodense lesions in the liver, and ultrasound-guided fine-needle aspiration of a liver mass was performed. Undifferentiated neuroendocrine tumor with hepatic metastatic involvement was diagnosed. After a hepatic artery embolization procedure, the hypoglycemia was alleviated, but hyperglycemia soon recurred, associated with inappropriately increased serum glucagon concentrations. The patient's course strongly suggested the presence of a plurihormonal tumor secreting serotonin, insulin, and glucagon. CONCLUSION: To our knowledge, this is the first reported case of a patient with a neuroendocrine neoplasm secreting serotonin, glucagon, and insulin, manifested by the carcinoid syndrome, hyperglycemia, and hypoglycemia.- - - - - - - - - - ranking = 1.2016101977391keywords = neoplasm (Clic here for more details about this article) |
4/48. Hyperinsulinemic hypoglycemia associated with possible hypopituitarism in a patient with acquired immunodeficiency syndrome.OBJECTIVE: To describe endocrine dysfunctions associated with human immunodeficiency virus (hiv) infection. methods: We present a case report, discuss the laboratory findings, and suggest potential contributing factors in this complex milieu. RESULTS: A 44-year-old man infected with hiv was admitted with loss of consciousness after a grand mal seizure and was found to have a prolonged course of hypoglycemia in conjunction with increased serum levels of insulin and C peptide. Iatrogenic hypoglycemia was excluded by laboratory evaluations. Hypothalamic-pituitary dysfunction was suspected because the serum growth hormone and cortisol concentrations during hypoglycemia were low. Radiologic evaluation of the brain revealed no structural abnormalities involving the hypothalamic-pituitary area. A normal cortisol response to adrenocorticotropic hormone stimulation (cosyntropin, 250 mg) was documented on two occasions. The cause of the severe hypoglycemic episode and the possible hypothalamic-pituitary-adrenal axis dysfunction in this patient is unknown. CONCLUSION: Despite the complexity of endocrine disorders in hiv-infected patients, the course of the disease in this patient underscores the possibility that some of the endocrinologic changes may be reversible.- - - - - - - - - - ranking = 0.4keywords = complex (Clic here for more details about this article) |
5/48. Solitary fibrous tumor of the liver.Solitary fibrous tumor of the liver is a rare neoplasm. So far, 21 cases have been described in the English literature. We reported an additional case. Our patient, a 75-year-old man, suffered from abdominal fullness and body weight loss of 6 kg over 6 months, and chills and fever for 2 months. hypoglycemia was noted at admission. Both abdominal sonography and CT showed a huge mass occupying the right lobe of liver. liver biopsy showed fibrous tumor. Right lobectomy was performed and the tumor was resected. Pathological examination showed spindle-shaped and fibroblast-like cells within the collagenous stroma. On immunohistochemical stains, these spindle tumor cells showed CD34 positive reactivity. The post-operative course was uneventful and there was no more hypoglycemia. The patient recovered smoothly, regained his body weight, and was alive without evidence of disease recurrence at the last follow-up visit in November, 2000.- - - - - - - - - - ranking = 0.20026836628985keywords = neoplasm (Clic here for more details about this article) |
6/48. Congenital hypopituitarism associated with neonatal hypoglycemia and microphallus: four cases secondary to hypothalamic hormone deficiencies.The association of hypoglycemia and microphallus in the male neonate is presumptive evidence of congenital hypopituitarism. This was observed in four male infants with normal birth weight and length, optic discs, and intelligence, and without gross central nervous system malformations. plasma and urinary cortisol values were low. Stimulation with metyrapone and insulin hypoglycemia failed to elicit a rise in plasma corticoids, but multiple doses of ACTH evoked a response. growth hormone responses to arginine, insulin, sleep, L-dopa, and glucagon were uniformly less than 2.5 ng/ml. In three patients, however, length remained within 2 SD of the mean until two years of age; in one, there was a sharp decrease in growth by three months. Two patients had low plasma TSH and thyroxine concentrations within the first month of life. In the other two patients, whose thyroxine levels were measurable, intravenous administration of thyrotropin-releasing factor evoked a normal rise in plasma TSH; serum thyroxine decreased into the hypothyroid range in one after GH therapy was initiated. plasma prolactin was normal in the first two patients receiving thyroxine replacement therapy. The other two patients had elevated baseline prolactin levels and had an augmented rise in plasma prolactin after administration of TRF. Human chorionic gonadotropin induced a 10- to 15-fold rise in plasma testosterone in the two patients tested. The changes in plasma FSH and LH after luteinizing hormone-releasing factor were either low or in the prepubertal range. In three patients, treated with testosterone enanthate intramuscularly, phallic growth occurred. In addition, all three had a transient increase in height but no acceleration of skeletal maturation. The data suggest a deficiency of hypothalamic hypophysiotropic hormones rather than a primary pituitary defect. Early recognition of this syndrome complex is critical for prompt treatment of the life-threatening cortisol deficiency. The diagnosis is more difficult in affected females because their external genitals are normal. The microphallus is a remediable manifestation of hypopituitarism.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
7/48. Drug-induced hypoglycemia presenting as acute mountain sickness, after mistaking acetohexamide for acetazolamide.Acute mountain sickness (AMS) can present with a wide variety of symptoms in unacclimatized persons who rapidly ascend to altitudes > 2500 m. The clinical manifestations of drug-induced hypoglycemia, including adrenergic and neurologic symptoms, have significant overlap with the AMS symptom complex. These similarities can lead to confusion in the diagnosis of hypoglycemia versus AMS, particularly for diabetics ascending to altitude. A case is described in which the oral hypoglycemic agent acetohexamide, instead of acetazolamide, was mistakenly self-administered for the prophylaxis and treatment of altitude illness. Improper self-medication by travelers in remote areas may be more common than is currently recognized.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
8/48. Severe hypoglycaemia associated with a giant solitary fibrous tumor of the pleura.solitary fibrous tumors (SFT) of the pleura are a rare neoplasm, with benign biological behaviour. Recurrences are rare, and no distant metastases are described in the literature. SFT can secrete hormone-like substances, responsible for paraneoplastic syndromes. The authors describe a case of severe hypoglycaemia due to insulin-like growth factor ii (IGF-2)'s secretion by a giant SFT of the pleura. Hypoglycaemia was controlled by the resection of the tumor. diagnosis and surgical management of these neoplasms are also discussed.- - - - - - - - - - ranking = 0.40053673257969keywords = neoplasm (Clic here for more details about this article) |
9/48. A deletion in the human QP-C gene causes a complex III deficiency resulting in hypoglycaemia and lactic acidosis.Mitochondrial respiratory chain complex III (ubiquinol-cytochrome c reductase) consists of 11 subunits, only one (cytochrome b) being encoded by the mitochondrial dna. Disorders of complex III are comparatively rare but are nevertheless present as a clinically heterogeneous group of diseases. To date, no mutation in any of the nuclear-encoded subunits has been described. We report here a deletion in the nuclear gene UQCRB encoding the human ubiquinone-binding protein of complex III (QP-C subunit or subunit VII) in a consanguineous family with an isolated complex III defect. In the proband, a homozygous 4-bp deletion was identified at nucleotides 338-341 of the cDNA predicting both a change in the last seven amino acids and an addition of a stretch of 14 amino acids at the C-terminal end of the protein. Both parents were found to be heterozygous for the deletion, which was absent from 55 controls. Low temperature (-196 degrees C) spectral studies performed on isolated mitochondria from cultured skin fibroblast of the proband showed a decreased cytochrome b content suggestive of a role for the QP-C subunit in the assembly or maintenance of complex III structure.- - - - - - - - - - ranking = 1.8keywords = complex (Clic here for more details about this article) |
10/48. reproduction of features of the glucagonoma syndrome with continuous intravenous glucagon infusion as therapy for tumor-induced hypoglycemia.OBJECTIVE: To describe the adverse effects of continuous intravenous infusion of glucagon as therapy for tumor-induced hypoglycemia and to correlate these treatment-related effects with symptoms of endogenous hyper-glucagonemia. methods: We reviewed three cases in which patients received continuous glucagon therapy for tumor-induced hypoglycemia and experienced adverse side effects to the treatment. We noted that these adverse events were consistent with changes that are described in the literature as symptoms of the glucagonoma syndrome. RESULTS: Continuous intravenous glucagon infusion has evolved as a reliable and efficacious modality for the treatment of tumor-induced hypoglycemia. We report the adverse events of venous thromboembolism, necrolytic migratory erythema, and angular cheilitis in conjunction with continuous intravenous glucagon treatment. These complications resemble symptoms that characterize the human model of hyperglucagonemia--the glucagonoma syndrome--which is associated with hyperglucagonemia and alpha-islet cell neoplasms of the pancreas. CONCLUSION: Symptoms that characterize the islet cell neoplasm-related glucagonoma syndrome may develop in patients receiving an infusion of exogenous glucagon. This observation lends support to the suggestion that glucagon may have a direct, causative role.- - - - - - - - - - ranking = 0.40053673257969keywords = neoplasm (Clic here for more details about this article) |
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