Persistent Hyperinsulinemic Hypoglycemia of Infancy: Long-Term Outcome Following Subtotal Pancreatectomy

Mahachoklertwattana, Pat; Suprasongsin, Chittiwat; Teeraratkul, Sumate; Preeyasombat, Chawalit

doi:10.1515/jpem.2000.13.1.37

Cited by 12 publications

(8 citation statements)

References 28 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…However, technical difficulties in neonates often limit the usefulness of some of these techniques. It has also been suggested that those with later‐onset hyperinsulinsim requiring surgery may require lesser pancreatectomy 35 . Postoperatively, either hypoglycaemia or diabetes can develop transiently but, more importantly, there is a risk of permanent IDDM developing 30,36–38 .…”

Section: Discussionmentioning

confidence: 99%

Ten years’ experience of persistent hyperinsulinaemic hypoglycaemia of infancy

Ambler

et al. 2001

J Paediatrics Child Health

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 99%

Ten years’ experience of persistent hyperinsulinaemic hypoglycaemia of infancy

Ambler

et al. 2001

J Paediatrics Child Health

View full text Add to dashboard Cite

show abstract

“…Furthermore, repeated episodes of severe, prolonged, sublethal hypoglycemia can result in permanent neurological damage, including developmental delay, mental retardation, and/or focal CNS deficits. Complications of neonatal hyperinsulinism are found in up to 50% of survivors, and this incidence has changed little during the past 20 years, reflecting the major problems that are faced in managing and treating this condition (5,8,9,29,166,173,181,196,201,253,284,313).…”

Section: Early Complications Of Hyperinsulinism In Infancymentioning

confidence: 99%

Hyperinsulinism in Infancy: From Basic Science to Clinical Disease

Dunne

Cosgrove²,

Shepherd³

et al. 2004

Physiological Reviews

263

277

View full text Add to dashboard Cite

Dunne, Mark J., Karen E. Cosgrove, Ruth M. Shepherd, Albert Aynsley-Green, and Keith J. Lindley. Hyperinsulinism in Infancy: From Basic Science to Clinical Disease. Physiol Rev 84: 239–275, 2004; 10.1152/physrev.00022.2003.—Ion channelopathies have now been described in many well-characterized cell types including neurons, myocytes, epithelial cells, and endocrine cells. However, in only a few cases has the relationship between altered ion channel function, cell biology, and clinical disease been defined. Hyperinsulinism in infancy (HI) is a rare, potentially lethal condition of the newborn and early childhood. The causes of HI are varied and numerous, but in almost all cases they share a common target protein, the ATP-sensitive K+ channel. From gene defects in ion channel subunits to defects in β-cell metabolism and anaplerosis, this review describes the relationship between pathogenesis and clinical medicine. Until recently, HI was generally considered an orphan disease, but as parallel defects in ion channels, enzymes, and metabolic pathways also give rise to diabetes and impaired insulin release, the HI paradigm has wider implications for more common disorders of the endocrine pancreas and the molecular physiology of ion transport.

show abstract

“…Extent of pancreatectomy has been the point of intensive discussion over the last 20 years [23,58,64,69,83,110,120]. Whereas in focal disease a limited resection of the focal area with preservation of as much pancreas as possible is performed, extension of surgery for diuse disease up to complete pancreatectomy results in an increasing risk of developing diabetes mellitus [15,62,69].…”

Section: Treatmentmentioning

confidence: 99%

“…Whereas in focal disease a limited resection of the focal area with preservation of as much pancreas as possible is performed, extension of surgery for diuse disease up to complete pancreatectomy results in an increasing risk of developing diabetes mellitus [15,62,69]. A recent study reported the occurrence of diabetes in 67% of patients after a >95% pancreatectomy [15].…”

Section: Treatmentmentioning

confidence: 99%