| Ten years' experience of persistent hyperinsulinaemic hypoglycaemia of infancy. | |
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MedLine Citation:
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PMID: 11885714 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: To review the presentation, management and outcome of persistent hyperinsulinaemic hypoglycaemia of infancy seen at the Royal Alexandra Hospital for Children over a 10 year period. METHODOLOGY: A retrospective review of 20 subjects was performed. As well as laboratory data, data were collected on clinical presentation, medical and surgical management and developmental outcome. RESULTS: Twenty subjects (11 male) were identified with presentation at a median age of 1.5 months (range 0-10 months), with 10 (50%) presenting in the first week of life. Only 20% of patients were large for gestational age. Diagnosis was made on the basis of high glucose requirements and inappropriately high insulin levels at the time of hypoglycaemia. Eight (40%) responded well to diazoxide treatment alone, seven (35%) received diazoxide in combination with other short-term medical therapy initially and five (25%) required pancreatectomy (repeat surgery in three). Those who required surgery had a higher mean birth weight. Infants presenting in the first week of life were less likely to respond to diazoxide. At the time of last review, eight (40%) of those treated medically had ceased all treatment. Two of the five cases requiring pancreatectomy now require insulin treatment. Neurodevelopmental assessment was normal in 11 (55%), mild delay was found in six (30%) and moderate or severe delay was found in three (15%). CONCLUSIONS: Persistent hyperinsulinaemic hypoglycaemia of infancy remains a major diagnostic and management challenge. Early suspicion and recognition is critical with definitive investigation and medical therapy to avoid hypoglycaemia, with pancreatectomy in medically unresponsive cases. Normal neurodevelopmental outcome was found in only 55% of cases. |
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Authors:
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V J Tyrrell; G R Ambler; W H Yeow; C T Cowell; M Silink |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Journal of paediatrics and child health Volume: 37 ISSN: 1034-4810 ISO Abbreviation: J Paediatr Child Health Publication Date: 2001 Oct |
Date Detail:
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Created Date: 2002-03-11 Completed Date: 2002-07-18 Revised Date: 2007-09-24 |
Medline Journal Info:
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Nlm Unique ID: 9005421 Medline TA: J Paediatr Child Health Country: Australia |
Other Details:
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Languages: eng Pagination: 483-8 Citation Subset: IM |
Affiliation:
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Ray Williams Institute of Paediatric Endocrinology, Diabetes and Metabolism, Royal Alexandra Hospital for Children, Westmead, New South Wales, Australia. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Blood Glucose / metabolism Chi-Square Distribution Child Child Development Child, Preschool Diazoxide / therapeutic use* Female Follow-Up Studies Humans Hyperinsulinism / complications*, drug therapy*, surgery Hypoglycemia / complications*, drug therapy* Infant Infant, Newborn Insulin / blood Male Pancreatectomy Retrospective Studies Treatment Outcome |
| Chemical | |
Reg. No./Substance:
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0/Blood Glucose; 11061-68-0/Insulin; 364-98-7/Diazoxide |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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