A mild case of nesidioblastosis with diagnostic and therapeutic difficulty.
A female infant with nesidioblastosis who showed mild clinical symptoms is reported. In this patient, insulin levels and insulin to glucose ratios (IRI/G) were often normal. Regular milk feedings supplemented with continuous glucose infusion (0.7-2 mg/kg per min) or oral glucose feedings (4.5 mg/kg per min) prevented hypoglycemia. As leucine-sensitivity was diagnosed at 2 months of age, she was started on diazoxide. This was, however, ineffective, and adverse effects appeared. Subtotal pancreatectomy (95%) was therefore attempted at 5 months of age, and persistent normoglycemia as well as normal growth and development followed up to 3 years after the operation. The pancreas showed characteristic signs of nesidioblastosis. The above clinical observation suggests that a patient with nesidioblastosis whose blood glucose level is easily controllable may develop an unexpected episode of hypoglycemia in the presence of a leucine sensitivity. In such a patient, diazoxide or, when it is of no avail, surgical intervention should promptly be instituted to prevent possible neurologic sequelae induced by hypoglycemia.
Yasukura
K
K
Department of Pediatrics, Kansai Medical University, Osaka, Japan.
Ono
A
A
Woo
M
M
Matsuzaki
S
S
Iwase
S
S
Kobayashi
Y
Y
D003131:Combined Modality Therapy; D003981:Diazoxide / Q000627:therapeutic use; D005260:Female; D005947:Glucose / Q000627:therapeutic use; D005951:Glucose Tolerance Test; D006801:Humans; D007225:Infant Food; D007231:Infant, Newborn; D010180:Pancreatectomy; D010182:Pancreatic Diseases / Q000097:blood / Q000175:diagnosis / Q000628:therapy; D012720:Severity of Illness Index
D005947:Glucose; D003981:Diazoxide