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A Case of Hyperinsulinism/hyperammonemia Syndrome

Sung JY, Hong SY, Shin CH, Yang SW

  • KMID: 1838802
  • J Korean Soc Pediatr Endocrinol.
  • 2005 Dec;10(2):236-240.
The hyperinsulinism/hyperammonemia (HI/HA) syndrome is a form of congenital hyperinsulinism. The children with HI/HA syndrome present recurrent symptomatic hypoglycemia and asymptomatic, persistent hyperammonemia, caused by mutations of the GLUD1 encoding...
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A Case of 2-Month-Old Infant with Persistent Hyperinsulinemic Hypoglycemia Presenting as Atonic Seizure

Kim JW, Kim DH, Kim SS

  • KMID: 1910339
  • Soonchunhyang Med Sci.
  • 2014 Jun;20(1):56-59.
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...
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A novel mutation of ABCC8 gene in a patient with diazoxide-unresponsive congenital hyperinsulinism

Park JS, Lee HJ, Park CH

Congenital hyperinsulinism (CHI) is a rare condition that can cause irreversible brain damage during the neonatal period owing to the associated hypoglycemia. Hypoglycemia in CHI occurs secondary to the dysregulation...
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A Case of Hyperinsulinism/Hyperammonemia (HI/HA) Syndrome due to a Mutation in the Glutamate Dehydrogenase Gene (GLUD1)

Jin HY, Choi JH, Kim GH, Ko JM, Yoo HW

  • KMID: 2322014
  • J Korean Soc Pediatr Endocrinol.
  • 2009 Dec;14(2):168-173.
Hyperinsulinism/Hyperammonemia (HI/HA) syndrome is a form of congenital hyperinsulinism (CHI) caused by a mutation in the GLUD1 gene. It is characterized by hyperinsulinemic hypoglycemia accompanying hyperammonemia. A 6-month-old male infant...
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Hyperinsulinemic Hypoglycemia After Roux-en-Y Gastric Bypass

Ha J, Park J

Bariatric surgery is one of the most efficient ways to achieve weight loss and reduce the complications associated with obesity. Severe hypoglycemia can occur after bariatric surgery, though it is...
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A Case of Persistent Hyperinsulinemic Hypoglycemia Treated with Diazoxide

Kim MS, Park EH, Kim SY, Lee DY

  • KMID: 1520749
  • J Korean Soc Pediatr Endocrinol.
  • 2007 Dec;12(2):164-167.
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...
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Congenital hyperinsulinism: current status and future perspectives

Yorifuji T

The diagnosis and treatment of congenital hyperinsulinism (CHI) have made a remarkable progress over the past 20 years and, currently, it is relatively rare to see patients who are left...
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Pancreatic islet-cell adenoma

Kim JH, Kim HS, Kim DH, Im HY, Park CI

  • KMID: 1690267
  • J Korean Pediatr Soc.
  • 1993 Jun;36(6):870-876.
Pancreatic islet cell adenoma is a benign tumor of pancreatic beta-cell and a rare cause of hyperinsulinemic hypoglycemia in children. The authors experienced a case of pancreatic islet cell adenoma(Insulinoma)in...
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A case of autoimmune insulin syndrome

Kim DJ, Kim NH, Seo JA, Kim SG, Choi KM, Baik SH, Choi DS

  • KMID: 2256511
  • Korean J Med.
  • 2006 Dec;71(6):683-687.
Autoimmune insulin syndrome is characterized by a combination of fasting hypoglycemia, high total serum insulin concentrations, and the presence of autoantibodies to the native human insulin in serum. A healthy...
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Anesthetic Management of an Infant with Nesidioblastosis: A case report

Kim DK, Bahk JH, Kim JS, Min SW

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...
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Serious Necrotizing Enterocolitis in a Neonate Treated with Octreotide for Congenital Chylothorax

Jo MJ, Kim DH, Kim HS

The somatostatin analog octreotide has recently been introduced in the treatment of infants with chylothorax. The safety profile of octreotide has not been fully evaluated. Octreotide reduces the splanchnic blood...
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Polycystic Ovary Syndrome in Adolescence

Hwang IT

  • KMID: 2321952
  • J Korean Soc Pediatr Endocrinol.
  • 2008 Jun;13(1):21-28.
Polycystic ovary syndrome (PCOS) is a syndrome of variable combinations of menstrual irregularity, hirsutism, and obesity. It is frequently diagnosed during adolescence and may be increasing in prevalence secondary to...
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Radiological diagnosis of islet cell tumor: Percutaneous transhepatic portal venous blood sampling

Yang I, Yoon Y, Ko YT, Lim JW, Lim JH

  • KMID: 2348023
  • J Korean Radiol Soc.
  • 1993 Aug;29(4):718-722.
Two patients with clinical and biochemical evidences of islet cell hyperfunction underwent percutaneous transhepatic portal and pancreatic vein catheterization and hormone assays. Local step-ups of insulin level in the protal...
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Anesthesia for an Insulinoma Case

Lee HS, Suh CH, An DA, Lim SH, Shin JS

  • KMID: 2357556
  • Korean J Anesthesiol.
  • 1979 Sep;12(3):280-284.
Hyperinsulinism caused by a functioning islet cell tumor of the pancreas is an uncommon but well established metabolic entity which can usually be diagnosed accurately. We treated a patient with...
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Congenital hyperinsulinism: diagnostic and management challenges in a developing country – case report

John CM, Agarwal P, Govindarajulu S, Sundaram S, Senniappan S

Management of congenital hyperinsulinemia of infancy (CHI) is challenging. A 4-month-old female infant with persistent hypoglycemia and elevated insulin levels was diagnosed with CHI. Gallium-68 DOTANOC positron emission tomography/computed tomography...
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A Case of Surgical Treatment of Nesidioblastosis in Infancy

Huh YS, Chai SC, Nah MC, Kim MJ

Nesidioblastosis in one of the causes of hyperinsulinemic hypoglysemia in infancy. The most important goal of treatment for persistent hypoglycemia is the prevention of permanent brain damage. The early surgical...
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A Case of Recurrent Spontaneous Hypoglycemia in a 73-year-old Woman with Congestive Heart Failure

Kim K, Kim WC, Lee EJ, Koh JS, Park JR, Hahm JR, Hwang JY

Hypoglycemia in adults without diabetes can be caused by drugs, critical illness, hormone deficiency, and hyperinsulinism. Spontaneous hypoglycemia in congestive heart failure has been reported rarely in adults. We report...
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Polycystic Ovary Syndrome in Korean Women: Clinical Characteristics and Diagnostic Criteria

Sung YA

Polycystic ovary syndrome (PCOS) is a common disorder of premenopausal women, affecting 4 to 8% of this population. Diagnosis of PCOS lays on a combination of clinical, biological and ultrasound...
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Images from ¹⁸F-DOPA Scan in Congenital Hyperinsulinism: Not Always a Clue for Diagnosis

Maines E, Giacomello L, D'Onofrio , Salgarello M, Gaudino R, Baggio L, Bordugo A

Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycaemia in childhood (Horm Res 70:65-72, 2008; J Clin Endocr Metab 93:869-875, 2008). ¹⁸⁻Fluoro-L-dihydroxy-phenylalanine (¹⁸F-DOPA) positron emission tomography (PET) can...
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A Case of Congenital Hypopituitarism with Anterior Pituitary Hypoplasia and Ectopic Posterior Pituitary Gland

Son YJ, Park HS, Shim KS, Choeh K, Han TI

  • KMID: 1829649
  • J Korean Pediatr Soc.
  • 1999 Dec;42(12):1746-1750.
The pituitary gland develops from two different parts of the brain. The anterior pituitary gland originates from the Rathke pouch and the posterior one from the infundibulum. Therefore, the pathologic...
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