Case report: Persistent hyperinsulinemic hypoglycemia of infancy associated with congenital neuroblastoma: A case report

The authors report a rare case of persistent hyperinsulinemic hypoglycemia of infancy (PHHI) with congenital neuroblastoma without feature(s) of Beckwith-Wiedemann syndrome. A term newborn with a birth weight of 3,900g developed hypoglycemia one hour after birth and required up to 20 mg/kg/min of in...

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Bibliographic Details
Main Authors: Dejkhamron P., Unachak K., Thanarattanakorn P., Charoenkwan P., Tantiprabha W., Chotinaruemol S., Chaiwun MD B.
Format: Article
Language:English; Thai
Published: 2014
Online Access:http://www.scopus.com/inward/record.url?eid=2-s2.0-77954481356&partnerID=40&md5=e0cc198cbec089b0c6c1ae0428f93039
http://cmuir.cmu.ac.th/handle/6653943832/3659
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Institution: Chiang Mai University
Language: English; Thai
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Summary:The authors report a rare case of persistent hyperinsulinemic hypoglycemia of infancy (PHHI) with congenital neuroblastoma without feature(s) of Beckwith-Wiedemann syndrome. A term newborn with a birth weight of 3,900g developed hypoglycemia one hour after birth and required up to 20 mg/kg/min of intravenous glucose infusion to maintain euglycemia. Investigations during the critical period revealed an inappropriately high insulin level. An abdominal CT scan revealed a normal pancreas, right suprarenal mass, and liver nodules. A condition of stage 4S neuroblastoma was suspected and supported by an increased ratio of urine vanillylmandelic acid to creatinine. The bone marrow smear was normal. She underwent near total pancreatectomy at the age of 2 months. The suprarenal mass and liver nodules were not found during the operation or during repeated abdominal CT scans at 3 month of age. Spontaneous regression of neuroblastoma was suspected. The pathology of the pancreas was compatible with PHHI.