Factitious hyperinsulinism leading to pancreatectomy: severe forms of Munchausen syndrome by proxy.
Clinical history and inappropriate insulin secretion during hypoglycemic episodes permit the diagnosis of hyperinsulinism. We report 2 cases of factitious hyperinsulinism leading to partial pancreatectomy. Case 1 was an 8-year-old girl who presented with severe hypoglycemia and elevated insulin and C-peptide levels. Catheterization of pancreatic veins was performed to localize the excess insulin secretion. Insulinoma was suspected, and partial pancreatectomy was performed. Ten days after surgery, severe hypoglycemia recurred with severely elevated plasma insulin levels (x100) but very low C-peptide plasma levels, suggesting factitious hyperinsulinemia. Hypoglycemic episodes before surgery were provoked by oral sulfonamides; postoperative episodes were caused by parenteral insulin. Falsified prescriptions for sulfonamides and insulin by the mother, a nurse, were found. Case 2 was a 6-month-old girl who presented with seizures and hypoglycemia but had a symptom-free interval of many months afterward. At 2 years of age, repeated hypoglycemic seizures and elevated insulin plasma levels suggested congenital hyperinsulinism. C-peptide plasma level, measured once, was normal, but blood sampling was performed 15 minutes after a hypoglycemic episode. Partial pancreatectomy was performed. Two weeks after surgery, hypoglycemic seizures recurred, and the patient was admitted for pancreatic vein catheterization. This investigation was performed during hypoglycemia and revealed high insulin levels and undetectable C-peptide levels, suggesting factitious hypoglycemia. Insulin/C-peptide ratio analysis is crucial to assess factitious hypoglycemia, although sulfonamide-induced hypoglycemia is not thereby detected. One percent (2 of 250) of all cases of hyperinsulinemic hypoglycemia in our unit have been identified as Munchausen syndrome by proxy. Atypical disease history should raise the question of factitious hypoglycemia.
Department of Metabolic Disorders, Hôpital Necker Enfants Malades, 75015 Paris, France. giurgea@im3.inserm.fr
Irina
I
Giurgea
Tim
T
Ulinski
Guy
G
Touati
Christine
C
Sempoux
Fanny
F
Mochel
Francis
F
Brunelle
Jean-Marie
JM
Saudubray
Claire
C
Fekete
Pascale
P
de Lonlay
D001786:Blood Glucose / Q000032:analysis; D002096:C-Peptide / Q000097:blood; D002648:Child; D002675:Child, Preschool; D044903:Congenital Hyperinsulinism / Q000175:diagnosis; D005260:Female; D006801:Humans; D006946:Hyperinsulinism / Q000139:chemically induced / Q000175:diagnosis*; D007003:Hypoglycemia / Q000139:chemically induced / Q000175:diagnosis; D007328:Insulin / Q000097:blood; D016735:Munchausen Syndrome by Proxy / Q000175:diagnosis*; D010180:Pancreatectomy
D001786:Blood Glucose; D002096:C-Peptide; D007328:Insulin