Factitious Hyperinsulinism Leading to Pancreatectomy: Severe Forms of Munchausen Syndrome by Proxy

Abstract

ABSTRACT. 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 (؋100) 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. P ersistent hyperinsulinemic hypoglycemia of infancy (PHHI) is a profound hypoglycemia resulting from excess insulin secretion. The diagnosis of PHHI is based on the combination of the clinical history and the laboratory findings. The clinical presentation of PHHI varies with the age at onset of hypoglycemia. The neonatal form (ϳ60% of all patients) is characterized by hypoglycemia within 72 hours after birth, with ϳ50% of the patients exhibiting seizures. Irreversible brain damage may occur. Infantile-onset PHHI (35% of patients) occurs between the 1st and the 12th months of life, and, again, seizures occur in 50% of the patients. Approximately 5% of pediatric patients with PHHI present after 1 year of age. Laboratory findings of PHHI include fasting and postprandial hypoglycemia (Ͻ3 mmol/L) with hyperinsulinemia (plasma insulin concentrations Ͼ3 mU/L) and elevated C-peptide levels, requiring high rates of intravenous glucose (Ͼ10 mg/kg/min) to maintain blood glucose Ͼ3 mmol/L. Plasma glucose concentration increases by 2 to 3 mmol/L in response to 0.5 mg of glucagon injection. In the absence of clearly abnormal insulin levels during hypoglycemia, a 4-to 6-hour fasting study may reveal inappropriately low plasma levels of ketone bodies, free fatty acids, and branched-chain amino acids.The causes of PHHI includ...

Hyperinsulinaemic hypoglycaemia: genetic mechanisms, diagnosis and management

“…Diagnosis is difficult because caregivers are adept at deceiving medical and mental health professionals. Cases of chronic surreptitious administration of insulin or antidiabetic drugs such as sulfonamides to children and adults have been reported, with some being treated with pancreatectomy to control hypoglycaemia (79,80). Management ( Figure 2): 1) Immediate Management Prompt diagnosis with aggressive early intervention to prevent hypoglycaemia remains the mainstay of treatment required to avert irreversible brain damage (4,81).…”

Monsters in the Closet: Munchausen Syndrome by Proxy

“…MSbP case reports describe poisoning with substances such as ipecac, salt, insulin, laxatives, lorazepam, corrosives, diphenhydramine, amitriptyline, lamotrigine, and clonidine to name just a few. 11,14,[17][18][19][20][21][22] Both hematuria and gastrointestinal bleeding can be simulated or produced by a variety of methods. Perpetrators either mix their own blood with a child's specimen or actively induce bleeding.…”

“…13 In a series of 250 Parisian children with a diagnosis of hyperinsulinemic hypoglycemia, 2 cases were found to be MSbP cases. 14 Surveys of pediatric neurologists and gastroenterologists suggest that the condition may be much more common than previously appreciated and that many cases probably go undiagnosed. 15 …”

“…Some children have undergone as many as 100 operations to treat nonexistent conditions, 31 including such invasive procedures as tracheostomy, feeding tube placement, insertion of a central catheter, pancreatectomy, orthopedic procedures, skin biopsy, laparotomy, endoscopy, and ear surgery. 14,25,26,28,29,42 How can this happen? Valentine et al 22 describe MSbP as "a curious triad [that] develops among perpetrator, victim, and medical staff based upon abuse of the child and deception of the physician."…”

Bir Çocuk İstismarı Türü Olarak “Munchausen by Proxy Sendromu”

“…30 Yapılan çalışmalarda çocukların fiziksel müdahale içeren pek çok tıbbi uygulamaya uğradıkları görülmektedir. 43,63,[69][70][71][72][73][74] Uygulanan tüm tetkik ve müdahalelerin çocuğu fiziksel ve duygusal istismara maruz bırakacak nitelikte olduğu düşünülmektedir. Fiziksel olmayan müdahale olarak gruplandırılan olgular ise, fiziksel tetkik ve müdahale içermeyen test ve değerlendirmeler, görüşme gibi duygusal olarak çocuğu örseleyecek durumları içermektedir.…”