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Congenital hyperinsulinism – a review of the disorder and a discussion of the anesthesia management
Author(s) -
HARDY OLGA T.,
LITMAN RONALD S.
Publication year - 2007
Publication title -
pediatric anesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.704
H-Index - 82
eISSN - 1460-9592
pISSN - 1155-5645
DOI - 10.1111/j.1460-9592.2007.02192.x
Subject(s) - medicine , hypoglycemia , congenital hyperinsulinism , hyperinsulinism , perioperative , pancreatectomy , pediatrics , intubation , neonatal hypoglycemia , disease , insulin , intensive care medicine , anesthesia , surgery , pregnancy , resection , gestation , insulin resistance , gestational diabetes , biology , genetics
Summary Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in infants and children. In most affected infants, CHI is caused by a specific genetic defect that results in the altered expression of pancreatic beta cells causing unregulated oversecretion of insulin. Infants with CHI may have either focal or diffuse abnormalities of the pancreatic β ‐cells. Both forms of CHI manifest as hypoglycemia, usually in the early newborn period. Focal disease can be treated effectively with surgical resection of the affected area, resulting in a total cure or rendering the patient amenable to medical management. Most children with diffuse disease are unresponsive to medical therapy, and require near‐total pancreatectomy. At The Children's Hospital of Philadelphia, we have developed a multidisciplinary program for diagnosis and treatment of CHI. Anesthesiologists have played an integral role in the perioperative care of these infants, which includes diagnostic procedures, partial or near‐total pancreatectomy, and postoperative pain management. In this review, we describe the clinical features, diagnostic methods and anesthetic concerns in children with CHI.