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Page 1
Persistent hyperinsulinaemic hypoglycaemia.
Semin Neonatol. 2002 Feb;7(1):95-100. doi: 10.1053/siny.2001.0090.
Semin Neonatol. 2002.
PMID: 12069542
Review.
Only diazoxide-sensitive neonates should be orientated to transient hyperinsulinism or hyperinsulinism-hyperammonemia syndrome. Focal CI is characterized by a sporadic somatic islet-cell hyperplasia. ...
Only diazoxide-sensitive neonates should be orientated to transient hyperinsulinism or hyperinsulinism-hyperammonemia syndr …
Hyperinsulinism-hyperammonemia syndrome in two Peruvian children with refractory epilepsy.
De Los Santos-La Torre MA, Del Águila-Villar CM, Lu-de Lama LR, Nuñez-Almache O, Chávez-Tejada EM, Espinoza-Robles OA, Pinto-Ibárcena PM, Calagua-Quispe MR, Azabache-Tafur PM, Tucto-Manchego RM.
De Los Santos-La Torre MA, et al.
J Pediatr Endocrinol Metab. 2022 Dec 8;36(2):207-211. doi: 10.1515/jpem-2022-0490. Print 2023 Feb 23.
J Pediatr Endocrinol Metab. 2022.
PMID: 36476334
OBJECTIVES: Congenital hyperinsulinism (HI) is a heterogeneous clinical disorder with great variability in its clinical phenotype, and to date, pathogenic variants in 23 genes have been recognized. Hyperinsulinism-hyperammonemia syndrome (HI/HA) …
OBJECTIVES: Congenital hyperinsulinism (HI) is a heterogeneous clinical disorder with great variability in its clinical phenot …
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Protein-sensitive and fasting hypoglycemia in children with the hyperinsulinism/hyperammonemia syndrome.
Hsu BY, Kelly A, Thornton PS, Greenberg CR, Dilling LA, Stanley CA.
Hsu BY, et al.
J Pediatr. 2001 Mar;138(3):383-9. doi: 10.1067/mpd.2001.111818.
J Pediatr. 2001.
PMID: 11241047
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Familial hyperinsulinism-hyperammonemia syndrome in a family with seizures: case report.
de las Heras J, Garin I, de Nanclares GP, Aguayo A, Rica I, Castaño L, Vela A.
de las Heras J, et al.
J Pediatr Endocrinol Metab. 2010 Aug;23(8):827-30. doi: 10.1515/jpem.2010.132.
J Pediatr Endocrinol Metab. 2010.
PMID: 21073125
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Robust regulation of hepatic pericentral amination by glutamate dehydrogenase kinetics.
Bera S, Lamba S, Rashid M, Sharma AK, Medvinsky AB, Acquisti C, Chakraborty A, Li BL.
Bera S, et al.
Integr Biol (Camb). 2016 Nov 7;8(11):1126-1132. doi: 10.1039/c6ib00158k.
Integr Biol (Camb). 2016.
PMID: 27747338
Impaired glutamate dehydrogenase (GDH) sensitivity to its inhibitors causes excessive insulin secretion by pancreatic beta-cells and defective ammonia metabolism in the liver. These symptoms are commonly associated with hyperinsulinism/hyperammonemia syndrome …
Impaired glutamate dehydrogenase (GDH) sensitivity to its inhibitors causes excessive insulin secretion by pancreatic beta-cells and defecti …
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