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Persistent hyperinsulinemic hypoglycemia of infancy: long-term results
C Dacou-Voutetakis1, F Psychou, M Maniati-Christidis
1First Pediatric Department, Athens University Medical School, Aghia Sophia Children's Hospital, Goudi, Greece.
Insights
Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) can be managed with diazoxide therapy and dietary changes. Early identification of the anatomical lesion is recommended before considering surgery.
Area of Science:
- Pediatrics
- Endocrinology
- Metabolic Disorders
Background:
- Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is a rare condition causing severe hypoglycemia.
- Management strategies for PHHI vary, with surgery often considered.
- Long-term outcomes and optimal treatment approaches require further investigation.
Purpose of the Study:
- To retrospectively analyze the management and outcomes of infants with PHHI.
- To evaluate the efficacy of conservative management, including diazoxide therapy and dietary modifications.
- To assess the necessity of surgical intervention and the importance of lesion localization.
Main Methods:
- Retrospective analysis of data from 15 infants with PHHI treated between 1976 and 1995.
- Review of treatment modalities: diazoxide therapy, frequent low-protein feeds, pancreatectomy, and focal lesion excision.
- Assessment of patient outcomes, including euglycemia, neurological status, and long-term complications.
Main Results:
- Nine patients achieved euglycemia with prolonged diazoxide therapy and dietary management.
- Four patients were managed successfully with dietary changes alone.
- Surgical intervention in two patients led to diabetes mellitus in one; no neurological damage was observed in any patient.
Conclusions:
- Conservative management, though demanding, is effective for PHHI when euglycemia is maintained.
- Diazoxide therapy and dietary adjustments are viable long-term treatment options.
- Pre-surgical anatomical lesion definition (focal vs. diffuse) is crucial for guiding treatment decisions.
Abstract:
Data from 15 infants with persistent hyperinsulinemic hypoglycemia of infancy (PHHI), presented to our hospital from 1976 to 1995, were retrospectively analyzed. Nine patients were successfully managed with prolonged (1.25-13 yr) diazoxide therapy and frequent, low protein feeds. Three of them are still being treated with diazoxide, 7.75, 9.25 and 13 yr post diagnosis. Four patients were managed only with frequent, low protein feeds. One patient had 2/3 pancreatectomy, and another had excision of a focal lesion which proved to be focal nesidioblastosis. Two patients were lost to follow up. No patient has shown neurological damage. One girl developed seizures at age 11.5 yr without hypoglycemia. The patient with 2/3 pancreatectomy developed diabetes mellitus at the age of 20 years. Although conservative management of PHHI is very laborious for the family and the physician, it should be applied as long as euglycemia is accomplished. Definition of the anatomical lesion (focal or diffuse) prior to pancreatectomy is recommended.