- 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... -
- CITED
-
- Close
- SHARE
-
- Close
- 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... -
- CITED
-
- Close
- SHARE
-
- Close
- A novel mutation of ABCC8 gene in a patient with diazoxide-unresponsive congenital hyperinsulinism
-
Park JS, Lee HJ, Park CH
- KMID: 2362271
- Korean J Pediatr.
- 2016 Nov;59(Suppl 1):S116-S120.
- doi: 10.3345/kjp.2016.59.11.S116
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... -
- CITED
-
- Close
- SHARE
-
- Close
- Serious Necrotizing Enterocolitis in a Neonate Treated with Octreotide for Congenital Chylothorax
-
Jo MJ, Kim DH, Kim HS
- KMID: 2468548
- Perinatology.
- 2019 Dec;30(4):249-253.
- doi: 10.14734/PN.2019.30.4.249
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... -
- CITED
-
- Close
- SHARE
-
- Close
- 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... -
- CITED
-
- Close
- SHARE
-
- Close
- Congenital hyperinsulinism: current status and future perspectives
-
Yorifuji T
- KMID: 1803855
- Ann Pediatr Endocrinol Metab.
- 2014 Jun;19(2):57-68.
- doi: 10.6065/apem.2014.19.2.57
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... -
- CITED
-
- Close
- SHARE
-
- Close
- 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... -
- CITED
-
- Close
- SHARE
-
- Close
- Congenital hyperinsulinism: diagnostic and management challenges in a developing country – case report
-
John CM, Agarwal P, Govindarajulu S, Sundaram S, Senniappan S
- KMID: 2400785
- Ann Pediatr Endocrinol Metab.
- 2017 Dec;22(4):272-275.
- doi: 10.6065/apem.2017.22.4.272
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... -
- CITED
-
- Close
- SHARE
-
- Close
- Images from ¹â¸F-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
- KMID: 2403513
- Nucl Med Mol Imaging.
- 2017 Dec;51(4):362-363.
- doi: 10.1007/s13139-016-0434-9
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). ¹â¸â»Fluoro-L-dihydroxy-phenylalanine (¹â¸F-DOPA) positron emission tomography (PET) can... -
- CITED
-
- Close
- SHARE
-
- Close
- Anesthetic Management of an Infant with Nesidioblastosis: A case report
-
Kim DK, Bahk JH, Kim JS, Min SW
- KMID: 2237773
- Korean J Anesthesiol.
- 1997 Jun;32(6):1031-1035.
- doi: 10.4097/kjae.1997.32.6.1031
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... -
- CITED
-
- Close
- SHARE
-
- Close
- Tracer Accumulation in Relation to Venous Thrombus on ¹â¸F-DOPA PET/CT in a Case of Persistent Hyperinsulinemic Hypoglycemia of Infancy
-
Arora S, Damle NA, Passah A, Sharma R, Goyal H, Arunraj ST, Gupta P, Jana M
- KMID: 2448103
- Nucl Med Mol Imaging.
- 2019 Apr;53(2):148-151.
- doi: 10.1007/s13139-018-00568-5
¹â¸F-DOPA PET/CT is commonly done in patients with persistent hyperinsulinemic hypoglycemia of infancy (PHHI) to look for any focal lesion in the pancreas.We present the findings in a 20-day-old neonate... -
- CITED
-
- Close
- SHARE
-
- Close
- Elucidation of the Inhibitory Effect of Phytochemicals with Kir6.2 Wild-Type and Mutant Models Associated in Type-1 Diabetes through Molecular Docking Approach
-
Jagadeb M, Konkimalla VB, Rath SN, Das RP
- KMID: 2391509
- Genomics Inform.
- 2014 Dec;12(4):283-288.
- doi: 10.5808/GI.2014.12.4.283
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... -
- CITED
-
- Close
- SHARE
-
- Close
- Nesidioblastosis in Neonate with Persistent Hyperinsulinemic Hypoglycemia
-
Whang IT, Kim HS, Han HS
- KMID: 2193882
- J Korean Soc Pediatr Endocrinol.
- 1998 Nov;3(2):231-236.
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... -
- CITED
-
- Close
- SHARE
-
- Close
- Diffuse Nesidioblastosis of the Pancreasin Adult with Persistent Hyperinsulinemic Hypoglycemia
-
Lee SH, Hyoung KY, Seo GS, Shin BJ, Cho CG, Yang KS, Chae KM, Yun KJ
- KMID: 2461629
- J Korean Soc Endocrinol.
- 1996 Jun;11(2):247-253.
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... -
- CITED
-
- Close
- SHARE
-
- Close
- Medical Therapy in Children with Persistent Hyperinsulinemic Hypoglycemia of Infancy(PHHI)
-
Kim HJ, Kim MJ, Shin HS, Kim JS, Uhm MR, Ko SH, Lee SH, Jin DK
- KMID: 2207677
- J Korean Pediatr Soc.
- 2000 Feb;43(2):253-259.
PURPOSE: Persistent hyperinsulinemic hypoglycemia of infancy(PHHI), which is characterised by inappropriate insulin secretion in spite of hypoglycemia, needs urgent treatment to prevent cerebral hypoglycemic damage. Although pancreatectomy is the treatment... -
- CITED
-
- Close
- SHARE
-
- Close
- Clinical Outcome after Pancreatectomy in Patients with Persistent Hyperinsulinemic Hypoglycemia of Infancy
-
Jung MH, Hwang JS, Shin CH, Yang SW, Chi JG
- KMID: 2001523
- J Korean Soc Pediatr Endocrinol.
- 2000 Dec;5(2):171-181.
PURPOSE: The purpose of this study was to describe the clinical outcome after pancreatcetmy and its relationship with pathological appearances and clinical features in patients with persistent hyperinsulinemic hypoglycemia of... -
- CITED
-
- Close
- SHARE
-
- Close
