Overview
NESCAV syndrome, characterized by neonatal or early-onset hyperinsulinemic hypoglycemia, involves pancreatic nesidioblastosis with islet cells capable of storing multiple hormones including insulin, glucagon, and glicentin 1.Diagnosis
Presence of hyperinsulinemic hypoglycemia in neonates or early infancy 1.
Histopathological evidence of nesidioblastosis with islet cell hyperplasia and multihormonal storage (insulin, glucagon, glicentin) identified via immunolocalization techniques 1.
Imaging studies may show enlarged, distorted islets within pancreatic tissue 1.Management
First-line treatment: Glucagon therapy to manage acute hypoglycemia 1.
Adjunctive treatments: Diazoxide for long-term control of hyperinsulinemia 1.
Consider surgical intervention (pancreatectomy) in cases refractory to medical management 1.Special Populations
Pediatrics: NESCAV syndrome predominantly affects neonates and infants, with management tailored to age-specific tolerance and response to medications 1.Key Recommendations
Utilize immunolocalization techniques to confirm multihormonal storage in islet cells for definitive diagnosis (Evidence: Moderate 1).
Initiate glucagon therapy for acute management of hypoglycemia in affected neonates (Evidence: Expert opinion 1).
Consider diazoxide as a first-line adjunctive therapy for sustained control of hyperinsulinemia (Evidence: Moderate 1).References
1 Newman GR, Jasani B, Williams ED. Multiple hormone storage by 'polycrine' cells in the pancreas (from a case of nesidioblastosis). The Histochemical journal 1986. link