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Pancreatectomy for hyperinsulinism
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
The first successful pancreatectomy in a child with HI was performed in 1934 by Evarts Ambrose Graham, an American surgeon working in St Louis, Missouri, USA. Searching for what was thought to be an adenoma, a subtotal pancreatectomy was done and the patient's hypoglycemia resolved. The operation was done 20 years before the first description of the disease by Irvine McQuarrie in 1954, and was initially termed “syndrome of idiopathic hypoglycemia of infants.” In the 1970s the disease was named “nesidioblastosis,” a term abandoned in the 1990s after advances in molecular diagnosis showed that it results from a variety of genetic derangements that alter the regulatory mechanisms of insulin secretion and glucose homeostasis. The term “congenital hyperinsulinism” is the current term for the family of diseases characterized as a heterogeneous genetic disorder of insulin metabolism that results in severe persistent neonatal hypoglycemia, often associated with neurological complications.
Features of Lipid Metabolism in Diabetes Mellitus and Ischemic Heart Disease
Published in E.I. Sokolov, Obesity and Diabetes Mellitus, 2020
Major importance is attached to hereditary factors in the development of obesity (in particular, an increased amount of fat cells and a changed nature of the hypothalamic function is transmitted from generation to generation), and also to disturbance of processes of tissue metabolism. Clinical studies of obesity patients showed the presence of hypertrophy of fat cells, their inadequate metabolic adaptation with lowered oxidation in the Krebs cycle, a lower activity of the lipolytic enzymes, and an increased content of the atherogenic fractions of the blood. Biochemical investigation of the blood of obesity patients indicated an increased content of lactate, pyruvate, and betaglycerophosphatedehydrogenase. The clinical relation between obesity and the development of DM is of special importance. Most scientists consider that 60% of DM patients also suffer from obesity. Generally in about 50% of obesity patients, a disturbed tolerance to carbohydrates is determined. We must stress that the presence of hyperinsulinism and also disturbance of the secretion and deposition of insulin are noted in obesity patients.
Organ-specific autoimmune diseases
Published in Gabriel Virella, Medical Immunology, 2019
Gabriel Virella, George C. Tsokos
The clinical symptoms can be rather variable, depending on the biological properties of the predominant antibody population. Blocking antibodies to the insulin receptor cause hyperglycemia that does not respond to the administration of insulin (insulin-resistant diabetes). In contrast, insulin receptor antibodies with stimulating properties may induce the cellular metabolic effects usually triggered by insulin, albeit in an abnormal and unregulated fashion. The clinical picture is one of hyperinsulinism. The same patient may undergo cycles of predominance of hypo- and hyperinsulinism-like symptoms, mimicking an extremely brittle and difficult-to-control form of diabetes.
The association between insulin-like growth factor 1 levels within reference range and early postoperative remission rate in patients with Cushing’s disease
Published in Endocrine Research, 2021
Emre Gezer, Berrin Çetinarslan, Alev Selek, Zeynep Cantürk, Mehmet Sözen, Özlem Elen, Canan Baydemir, Burak Çabuk, Savaş Ceylan
Both long-term endogenous and exogenous hypercortisolism have been shown to have a suppressive effect on circulating GH.3,5,17,22,29–31 Similarly, the median (25th-75th) concentration of GH was 0.30 (0.12–0.83) ng/mL in our study. Furthermore, there was a significant difference in GH concentrations between the tertiles of IGF-1, with the lowest concentrations in T1 and the highest in T3 (p < .001). Potential mechanisms for this effect have been suggested to be desensitization of pituitary growth hormone releasing hormone (GHRH) receptors or the deterioration of hypothalamic GHRH secretion.5 Nevertheless, the pathophysiological mechanisms elevating IGF-1 in patients with CD, are still unclear. Hyperinsulinism induced by hypercortisolism is one possible mechanism, but Bang et al.19 found increased levels of IGF binding proteins (IGFBPs) in CD and two other studies reported the same outcome. It was hypothesized that increased IGFBPs caused a reduction IGF-1 bioactivity with subsequent enhanced sensitivity of tissue GH receptors leading to increased IGF-1.3,10 Considering all these as a seesaw-like mechanism, our results may indicate that in the majority of CD patients, there has been a positive elevation trend in IGF-1 concentration, possibly due to the increase in IGFBP and a decrease in IGF-1 bioactivity. On the other hand, the increased inhibition of GH due to severe hypercortisolism might dominate the other possible mechanisms, resulting in a final decrease in IGF-1 concentration.
Menopausal hyperinsulinism and hypertension – new approach
Published in Gynecological Endocrinology, 2020
Aleksandar Đogo, Milos Stojanovic, Miomira Ivovic, Milina Tancic Gajic, Ljiljana V. Marina, Goran Citlucanin, Milena Brkic, Srdjan Popovic, Svetlana Vujovic
In untreated hypoestrogenism, hyperinsulinism will progress to metabolic syndrome and diabetes. The study Study of Chen et al. [23] confirmed that the inflammation is the link between hyperinsulinism and hypertension. Inhibition of the NFkB inflammatory pathway significantly decreases blood pressure, vascular inflammation and improves endothelial function, and vascular insulin resistance. Insulin resistance also enhances the blood pressure response to sodium intake. Data from 1502 women 42–53 years old from the Korean Epidemiological study database have shown that development of hypertension was positively correlated with metabolic syndrome (HR 3.90, 95%, CI 2.51–6.07) and B < I (HR 1.09, 95%, Ci 1.03–1.16) [24]. It was clearly demonstrated in this study that E2/DRSP in otherwise healthy menopausal women with grade I hypertension and normal BMI significantly decreases HOMA index and insulin AUC improving insulin sensitivity and regulating blood pressure. Following up 8181 women, 56 years old, Rey Y et al. found that 13.2% had diabetes mellitus type 2 [25]. Mauvais Jarvis [26] has shown a preventive role of MHT on type 2 diabetes. We believe we should prevent hyperinsulinism and stop further metabolic changes leading to diabetes mellitus.
From pathogenesis to novel therapies in primary hyperoxaluria
Published in Expert Opinion on Orphan Drugs, 2019
Gill Rumsby, Sally-Anne Hulton
RNAi of GO and LDHA are both under active investigation in human trials. The anticipated difficulties with these treatments include mode of administration, off-target effects, long term effects and the wider consequences of inactivating a normal enzyme. GO inhibition, only really applicable to PH1, would not appear to have any additional sequelae as patients have been described with natural deficiency of this enzyme, in two cases in conjunction with another, unrelated, pathological disorder [82,83] and in one case as an incidental finding in an apparently normal individual [84]. These individuals have markedly elevated urine glycolate, more than twice that typically seen in PH1, but that in itself is not thought to be of consequence as it is relatively soluble. In one case, however, urine oxalate was also found to be elevated [83]. It is not yet clear whether this was a side effect of treatment for the congenital hyperinsulinism also present in this patient. Phase 1 clinical trials are underway with Lumasiran (ALN-GO1) (NCT02706886) [85]. Preliminary results show reduction of urine oxalate with corresponding increase of urinary glycolate [86].