Humans ; Consensus ; Congenital Hyperinsulinism

Congenital Hyperinsulinism ; diagnosis ; therapy ; Humans

Recently, the incidence of thyroid nodule has been rising due to the usage of ultrasonography in health check-up. Also, modern society is faced with increasing rates of obesity by sedentary life style and high caloric intake. Obesity is closely related to metabolic syndrome and insulin resistance. Insulin resistance leads to hyperinsulinemia, increased insulin-like growth factor 1 and alteration of adipocytokine level. These factors not only have a role in metabolic regulation, but they also have mitogenic effect in cell proliferation. Epidemiologic and clinical evidence about the association between obesity and thyroid nodule are reviewed in this section.
Cell Proliferation ; Energy Intake ; Hyperinsulinism ; Incidence ; Insulin Resistance ; Life Style ; Metabolic Syndrome X ; Obesity* ; Thyroid Gland* ; Thyroid Nodule* ; Ultrasonography

Nesidioblastosis, also known as persistent hyperinsulinemic hypoglycemia of infancy(PHHI) or familial hyperinsulinsm, is the most common cause of recurrent severe hypoglycemia in infancy. It is an autosomal recessive disorder characterized by irregular insulin secretion leading to inappropriately raised plasma insulin concentration compared to blood glucose levels. Recently, mutations in the sulfonylurea receptor(SUR) have been described in association with PHHI. The mainstay of medical treatment is glucose infusion and diazoxide or long acting somatostatin. If medical treatment fails in preventing hypoglycemia, near total pancreatectomy is recommended. We report one case of nesidioblastosis cured by near total pancreatectomy with brief review of literatures.
Blood Glucose ; Congenital Hyperinsulinism* ; Diazoxide ; Glucose ; Humans ; Hypoglycemia ; Infant, Newborn* ; Insulin ; Nesidioblastosis* ; Pancreatectomy ; Plasma ; Somatostatin

Nesidioblastosis, persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is a disorder characterized by diffuse pancreatic islet cell hyperplasia arising from the ductal epithelium. Patients usually present during the neonatal or infantile period with apnea, hypotonia, poor feeding, lethargy, or seizure. Despite of greater awareness, one in three has some degree of mental retardation by the time the diagnosis is made. The diagnosis is established by demonstrating high plasma insulin concentration during an episode of hypoglycemia. This hypoglycemia is initially managed medically, but these medical treatment modalities are failed in more than half of nesidioblastosis. Patient who failed to respond to optimal medical treatment should be referred for surgery early, if permanent neurologic damage is to be avoided. The surgical procedure of choice is near total pancreatectomy (95~98% resection). We herein discuss the anesthetic management of a patient with nesidioblastosis who presented for near total pancreatectomy.
Apnea ; Congenital Hyperinsulinism ; Diagnosis ; Epithelium ; Humans ; Hyperinsulinism ; Hyperplasia ; Hypoglycemia ; Infant* ; Insulin ; Intellectual Disability ; Islets of Langerhans ; Lethargy ; Metabolism ; Muscle Hypotonia ; Nesidioblastosis* ; Pancreatectomy ; Plasma ; Seizures

Congenital Hyperinsulinism ; diagnosis ; Humans ; Infant, Newborn ; Male

Nesidioblastosis is a term that describes multifocal hyperplasia of all panereatic cell components and is characterized primarily by their disorganization and proliferation throughout the entire panaeas. Adult onset nesidioblastosis is an extremely rare entity associated with hypersecretion of insulin. The authors have recently experieneed a case of nesidioblastosis in an adult. A 41-year old man was admitted due to interrnittenr hypoglycemic symptoms, that had been relieved by carbohydrate ingestion. Hyperinsulinemic hypoglycemia was documented during prolonged fast. Under the presumptive diagnosis of insulinoma, abdominal CT, celiac angiogram and percutaneous transhepatic portal venous sampling were done but we could not find any definitive mass. Eight-five percent of the panacas was removed. Pathologic examination of the resected pancreas revealed irregularly sized islets and scattering of small endocrine cell clusters throughout the acinar tissue and ductuloinsular complex.
Adult ; Cellular Structures ; Congenital Hyperinsulinism ; Diagnosis ; Eating ; Endocrine Cells ; Humans ; Hyperplasia ; Hypoglycemia ; Insulin ; Insulinoma ; Nesidioblastosis ; Pancreas ; Tomography, X-Ray Computed

Congenital hyperinsulinism is the most frequent cause of severe, persistent hypoglycemia in infancy and childhood. It is caused by an inappropriate insulin secretion from the pancreatic beta-cells secondary to various genetic disorders. Recognition of this entity becomes important due to the fact that hypoglycemia is very severe and frequent and that it may lead to severe neurological damage in the infant manifesting as mental or psychomotor retardation or even a life-threatening events if not recognized and treated effectively in time. Hypoglycemias can be detected by seizures, fainting, or any other neurological symptoms in the neonatal period or later, usually within the first two years of life. Hypoglycemias must be rapidly and intensively treated to prevent severe and irreversible brain damages. Next, a treatment to prevent the recurrence of hypoglycemia must be set, which may include frequent and glucose-enriched feeding, diazoxide and octreotide. We report a case of congenital hyperinsulinemia in a 2 months old infant presenting as atonic seizure which has been treated with diazoxide.
Brain ; Congenital Hyperinsulinism* ; Diazoxide ; Humans ; Hyperinsulinism ; Hypoglycemia ; Infant* ; Insulin ; Octreotide ; Recurrence ; Seizures* ; Syncope

Congenital hyperinsulinism (CHI), the most important cause of hyperglycemia in early infancy, is a heterogenous disease characterized by dysregulation of insulin secretion. Mutations in five proteins have been associated with CHI: sulfonyl urea receptor 1; Kir 6.2; glucokinase; glutamate dehydrogenase and mitochondrial enzyme short-chain 3-hydroxyacyl-coenzyme A dehydrogenase. Early recognition of hypoglycemia, diagnosis of CHI and appropriate management of the hypoglycemia are of the utmost importance to prevent neurologic damage. We report a case of persistent hyperinsulinemic hypoglycemia in 8-month-old male infant. This patient has no mutation in previously mentioned genes. Treatment with diazoxide was successful without any severe side effects in this patient.
Congenital Hyperinsulinism* ; Diagnosis ; Diazoxide* ; Glucokinase ; Glutamate Dehydrogenase ; Humans ; Hyperglycemia ; Hyperinsulinism ; Hypoglycemia ; Infant ; Insulin ; Male ; Oxidoreductases ; Urea

Among all serious diseases globally, diabetes (type 1 and type 2) still poses a major challenge to the world population. Several target proteins have been identified, and the etiology causing diabetes has been reasonably well studied. But, there is still a gap in deciding on the choice of a drug, especially when the target is mutated. Mutations in the KCNJ11 gene, encoding the kir6.2 channel, are reported to be associated with congenital hyperinsulinism, having a major impact in causing type 1 diabetes, and due to the lack of its 3D structure, an attempt has been made to predict the structure of kir6.2, applying fold recognition methods. The current work is intended to investigate the affinity of four phytochemicals namely, curcumin (Curcuma longa), genistein (Genista tinctoria), piperine (Piper nigrum), and pterostilbene (Vitis vinifera) in a normal as well as in a mutant kir6.2 model by adopting a molecular docking methodology. The phytochemicals were docked in both wild and mutated kir6.2 models in two rounds: blind docking followed by ATP-binding pocket-specific docking. From the binding pockets, the common interacting amino acid residues participating strongly within the binding pocket were identified and compared. From the study, we conclude that these phytochemicals have strong affinity in both the normal and mutant kir6.2 model. This work would be helpful for further study of the phytochemicals above for the treatment of type 1 diabetes by targeting the kir6.2 channel.
Congenital Hyperinsulinism ; Curcumin ; Diabetes Mellitus ; Genistein ; Molecular Docking Simulation ; Phytochemicals*