Objective:Because children with congenital hyperinsulinism(HI) caused by recessive loss of function mutations in the adenosine triphosphate (ATP)-dependent potassium channel(KATP-HI) are not leucine sensitive,we evalu...Objective:Because children with congenital hyperinsulinism(HI) caused by recessive loss of function mutations in the adenosine triphosphate (ATP)-dependent potassium channel(KATP-HI) are not leucine sensitive,we evaluated for protein induced hypoglycemia with oral protein tolerance tests. Study design:Blood glucose and insulin concentrations were measure devery 15 minutes for 3 hours after an oral protein loadin children with KATP-HI (n=11) and compared with those of children with glutamate dehydrogenase HI (n=12) and control subjects (n=12). Results:Similar to children with glutamate dehydrogenase HI,patients with KATP-HI displayed protein-induced hypoglycemia (10/11)with blood glucose concentrations declining by 17 to 69 mg/dL. In contrast,oral proteinhad little effect on blood glucose concentrations in control subjects.Conclusions:Protein induced hypoglycemia is a feature of KATP-HI,despite the absence of leucine sensitivity. The results indicate that amino acids can stimulate insulin secretionvia a glutamate dehydrogenase and KATP channel-independent pathway.展开更多
Congenital hyperinsulinism of infancy is a severe disease that leads to important brain damage.Two different forms of the disease have been identified by pathologists: a diffuse and a focal form.A specific genetic ano...Congenital hyperinsulinism of infancy is a severe disease that leads to important brain damage.Two different forms of the disease have been identified by pathologists: a diffuse and a focal form.A specific genetic anomaly identified in focal forms has never been described in diffuse ones.However, for most of authors, failure of medical treatment results in near-total pancreatectomy in all cases, which ends in diabetus.The aim of this retrospective study was to assess the results of elective partial pancreatectomy performed in 60 cases of focal form of hyperinsulinism over the last 18 years.Fifty-eight patients were cured with euglycemia at both fasting and hyperglycaemic tests without insulin-dependent diabetes mellitus.One patient is still in hypoglycaemia from unrecognized lesion; insulin-dependent diabetes mellitus occurred in one case nine years after surgery (a near-total pancreatectomy has been performed because of unknown focal form, in 1985).展开更多
文摘Objective:Because children with congenital hyperinsulinism(HI) caused by recessive loss of function mutations in the adenosine triphosphate (ATP)-dependent potassium channel(KATP-HI) are not leucine sensitive,we evaluated for protein induced hypoglycemia with oral protein tolerance tests. Study design:Blood glucose and insulin concentrations were measure devery 15 minutes for 3 hours after an oral protein loadin children with KATP-HI (n=11) and compared with those of children with glutamate dehydrogenase HI (n=12) and control subjects (n=12). Results:Similar to children with glutamate dehydrogenase HI,patients with KATP-HI displayed protein-induced hypoglycemia (10/11)with blood glucose concentrations declining by 17 to 69 mg/dL. In contrast,oral proteinhad little effect on blood glucose concentrations in control subjects.Conclusions:Protein induced hypoglycemia is a feature of KATP-HI,despite the absence of leucine sensitivity. The results indicate that amino acids can stimulate insulin secretionvia a glutamate dehydrogenase and KATP channel-independent pathway.
文摘Congenital hyperinsulinism of infancy is a severe disease that leads to important brain damage.Two different forms of the disease have been identified by pathologists: a diffuse and a focal form.A specific genetic anomaly identified in focal forms has never been described in diffuse ones.However, for most of authors, failure of medical treatment results in near-total pancreatectomy in all cases, which ends in diabetus.The aim of this retrospective study was to assess the results of elective partial pancreatectomy performed in 60 cases of focal form of hyperinsulinism over the last 18 years.Fifty-eight patients were cured with euglycemia at both fasting and hyperglycaemic tests without insulin-dependent diabetes mellitus.One patient is still in hypoglycaemia from unrecognized lesion; insulin-dependent diabetes mellitus occurred in one case nine years after surgery (a near-total pancreatectomy has been performed because of unknown focal form, in 1985).