Current Status of Childhood Hyperinsulinemic Hypoglycemia in Turkey.

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Term Occurence Count Dictionary
diabetes mellitus 7 endocrinologydiseases
diazoxide 20 endocrinologydiseasesdrugs
hypoglycemia 11 endocrinologydiseases
type 2 diabetes mellitus 1 endocrinologydiseases

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Select Drug Character Offset Drug Term Instance
diazoxide 7706 spontaneously in a few weeks, in some patients, the duration of hyperinsulinism can be protracted, requiring diazoxide treatment ([10]). In those patients, the mechanism for hyperinsulinism is not clear. Güven et al ([32])
diazoxide 7904 Güven et al ([32]) reported a case series of CHI patients, and in 7 of 13 patients which responded to diazoxide therapy, treatment had been stopped between 15 days to 12 years. Ağladıoğlu et al ([15]) presented
diazoxide 8078 Ağladıoğlu et al ([15]) presented 17 HH cases in their series, eight of which had transient HH; diazoxide therapy had been ceased between 3 to 154 months. No etiological or molecular genetic studies were done
diazoxide 9958 sugar was 38 mg/dL and serum insulin level was found as 7.3 mIU/mL). The patient responded well to diazoxide treatment in a dose of 10 mg/kg/day. During follow-up, diazoxide dose was tapered gradually and ceased
diazoxide 10023 mIU/mL). The patient responded well to diazoxide treatment in a dose of 10 mg/kg/day. During follow-up, diazoxide dose was tapered gradually and ceased at 3 months of life. Now, he is 2.5 years of age and good in condition.
diazoxide 12275 review detected three patients with KCNJ11 mutation. All except one patient with this mutation were diazoxide -unresponsive and required pancreatectomy. One diazoxide-unresponsive patient with KCNJ11 gene mutation
diazoxide 12331 except one patient with this mutation were diazoxide-unresponsive and required pancreatectomy. One diazoxide -unresponsive patient with KCNJ11 gene mutation responded well to octreotide therapy ([32]).A female
diazoxide 14562 resulting from a mutation in the HADH gene have been reported, and all of them have responded well to diazoxide therapy ([6]).Because the consanguinity is high in the Turkish population, occurrence of recessively
diazoxide 14826 to be inevitable. It is convenient that sequencing of HADH gene be recommended in all patients with diazoxide -responsive HH.The method for detection of HADH gene mutation is also important which could be different
diazoxide 15682 intervention remains the mainstay of treatment in HH. The first-line drug management of persistent CHI is diazoxide therapy. Diazoxide binds to SUR1 component of KATP channels resulting in their opening. It is effective
diazoxide 16161 surgical intervention is usually required.Most of the Turkish patients with CHI were responsive to diazoxide treatment (100/141, 71%), while 18 of them had the transient form. Among patients with reported genetic
diazoxide 16310 them had the transient form. Among patients with reported genetic mutation analysis (n=115), 75 were diazoxide -responsive.All Turkish patients with HADH gene mutation were diazoxide-responsive, thus no surgical
diazoxide 16381 mutation analysis (n=115), 75 were diazoxide-responsive.All Turkish patients with HADH gene mutation were diazoxide -responsive, thus no surgical treatment was applied, as expected. Pancreatectomy was implemented in 28
diazoxide 16496 diazoxide-responsive, thus no surgical treatment was applied, as expected. Pancreatectomy was implemented in 28 of diazoxide -unresponsive CHI patients which accounts for 19.8% of all CHI patients. All of them, except one, had
diazoxide 18210 patients.Very recently, a new medical treatment option has emerged for diffuse CHI unresponsive to diazoxide and/or octreotide treatment. The mammalian target of rapamycin inhibitor sirolimus has been successfully
diazoxide 18338 treatment. The mammalian target of rapamycin inhibitor sirolimus has been successfully used in some diazoxide - and octreotide-unresponsive CHI patients. It reduces the pancreatic B-cell proliferation and inhibits
diazoxide 18912 neurological sequelae were encountered in almost one third of patients (34% and 29%) ([15],[24]). Especially diazoxide -unresponsive patients were under high risk for development of neurological sequelae.The most favorable
diazoxide 19313 ([6]).Early and aggressive treatment of patients with severe CHI is necessary to prevent brain damage, and diazoxide responsiveness gives an important clue for good prognosis and further treatment.In conclusion, CHI is
diazoxide 19805 mutations, and diabetes mellitus may develop later in life. Because long-term neurological damage is high in diazoxide -unresponsive patients, early and prompt intervention is needed in such patients. There is no clear information
diazoxide 20262 Externally peer-reviewed.Figure 1Distribution of patients according to mutation analysis results and diazoxide responsivenes
Select Disease Character Offset Disease Term Instance
diabetes mellitus 2421 congenital hyperinsulinism (CHI) usually occur in newborns with certain risk factors like maternal diabetes mellitus , intrauterine growth retardation, perinatal asphyxia. Some case with transient HH might have HNF4A gene,
diabetes mellitus 7231 HYPERINSULINEMIC HYPOGLYCEMIA OF PATIENTSHH can be transient. Among the causes for transient form of HH, maternal diabetes mellitus , intra-uterine growth retardation, perinatal asphyxia, erythroblastosis fetalis, maternal administration
diabetes mellitus 9229 they responded well to medical therapy. Both siblings have been diagnosed with autoantibody-negative diabetes mellitus during the prepubertal period following a remission of the HH in childhood. Their mother, maternal aunt,
diabetes mellitus 9460 maternal grandfather were also heterozygous for the same mutation. The mother was diagnosed with type 2 diabetes mellitus at the age of 28 years, and the maternal aunt and grandfather had a medical history of postprandial
diabetes mellitus 10533 it should be kept in mind that transient HH in infancy can be related to KATP channel mutations, and diabetes mellitus may develop later in life.GENETIC MUTATIONS OF PATIENTSMolecular studies showed that the congenital
diabetes mellitus 17490 insufficient in some patients.It has been reported that pancreatectomy is associated with a high incidence of diabetes mellitus and pancreatic exocrine insufficiency. For that reason, surgical treatment should be reserved for the
diabetes mellitus 19711 frequent among them. Transient form of HH in infancy could be caused by KATP channel mutations, and diabetes mellitus may develop later in life. Because long-term neurological damage is high in diazoxide-unresponsive patients,
hypoglycemia 463 a rare disease characterized by dysregulated insulin secretion from pancreatic β-cells. Recurrent hypoglycemia can lead to neurological insult and permanent brain injury. Recently, there are important advances in
hypoglycemia 710 mechanisms, histological characteristics, imaging, and surgical techniques of congenital hyperinsulinemic hypoglycemia that could reflect to improvement in the clinical care of infants with this disorder. In Turkey, there
hypoglycemia 1656 analyzed, and 56 of them had one of the mutation leading to hyperinsulinism.INTRODUCTIONHyperinsulinemic hypoglycemia (HH) consist of a group of heterogeneous disorders characterized by unregulated insulin secretion from
hypoglycemia 1858 secretion from pancreatic β-cells ([1]). It is the commonest cause of both persistent and transient hypoglycemia in neonates and infants ([2],[3],[4]). Because there can be severe brain damage related to hypoglycemia,
hypoglycemia 1962 hypoglycemia in neonates and infants ([2],[3],[4]). Because there can be severe brain damage related to hypoglycemia , it is vital to diagnose and treat patients with HH correctly ([1],[2],[3],[4]).The clinical presentation
hypoglycemia 5355 10, 2016. The search terms were “hyperinsulinism” or “CHI” or “nesidioblastosis” or “ hypoglycemia ” or “persistent hyperinsulinemic hypoglycemia of infancy” or “hyperinsulinemic hypoglycemia
hypoglycemia 5405 “hyperinsulinism” or “CHI” or “nesidioblastosis” or “hypoglycemia” or “persistent hyperinsulinemic hypoglycemia of infancy” or “hyperinsulinemic hypoglycemia of infancy” or “HI” or “hyperinsulinaemic”
hypoglycemia 5455 “hypoglycemia” or “persistent hyperinsulinemic hypoglycemia of infancy” or “hyperinsulinemic hypoglycemia of infancy” or “HI” or “hyperinsulinaemic” or “hyperinsulinemic” and “Turkey” or “Turkish”.
hypoglycemia 9631 grandfather had a medical history of postprandial hypoglycemic attacks; there was no family history of hypoglycemia in neonatal period.Another interesting patient that we diagnosed as transient HH was born after caesarian
hypoglycemia 15507 while one patient had GLUD1 gene mutation ([32]).TREATMENT MODALITIES OF TURKISH PATIENTSFor preventing hypoglycemia -related irreversible brain damage, aggressive and early intervention remains the mainstay of treatment
hypoglycemia 16048 ([10]). The second-line medical treatment is octreotide therapy. If octreotide fails to control the hypoglycemia , surgical intervention is usually required.Most of the Turkish patients with CHI were responsive to
type 2 diabetes mellitus 9453 and maternal grandfather were also heterozygous for the same mutation. The mother was diagnosed with type 2 diabetes mellitus at the age of 28 years, and the maternal aunt and grandfather had a medical history of postprandial

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