Persistent hyperinsulinemic hypoglycemia of infancy: a clinical and pathological study of 19 cases in a single institution.

OBJECTIVE: To study the clinical and pathological features of persistent hyperinsulinemic hypoglycemia of infancy.
METHOD: The clinical and pathological data of 19 cases of persistent hyperinsulinemic hypoglycemia of infancy were retrieved and reviewed from the medical records in Children's Hospital of Fudan University.
RESULTS: There were 13 boys and 6 girls. The age interval was from 16 days to 7 months, and the average age was 2.71 ± 2.23 months. The blood glucose concentrations ranged from 0.57 to 3.0 mmol/L (average value 1.60 ± 0.75 mmol/L) and the serum insulin concentrations ranged from 3.1 to 79.4 uIU/ml (average value 27.89 ± 21.81 uIU/ml) at the time of one week before operation. The size of lesion was between 2 cm to 6.5 cm in maximum diameter (average value 4.04 ± 1.18 cm). 19 cases were divided into three types according to the pathological classification criteria: focal type (1 case), diffuse type (17 cases) and atypical type (1 case). The enucleation of the nodule was given for the patient of focal type and subtotal pancreatectomy was administrated for diffuse and atypical type patients. The blood glucose concentrations ranged from 3.0 to 12.4 mmol/L (average value 6.21 ± 2.69 mmol/L) at the time of one month after operation. The difference between the preoperative blood glucose concentrations and the postoperative blood glucose concentrations was statistically significant (1.6037 ± 0.7458 mmol/L vs. 6.2105 ± 2.6882 mmol/L, P<0.05). Insulin was positive for the multiple pancreatic islets, and the ki-67 index was between 5% and 8%. P57(kip2) was negative. 19 patients were followed up for a period of 2 to 38 months. 13 cases recovered well without any complications, and 6 cases had postoperative hyperglycemia and need a medication to control the blood glucose concentrations.
CONCLUSION: Persistent hyperinsulinemic hypoglycemia of infancy mainly occurred in infants. Boys had the predominance and the most common pathological type was the diffuse type. The different surgical methods (focal type with lesion enucleation, diffuse and atypical type with subtotal pancreatectomy), were effective for different patients, and most of patients had a good prognosis. Early diagnosis and appropriate treatment to maintain euglycemia remained the mainstay of management to prevent postoperative complications. Therefore, the correct pathological classification could help clinicians to find effective treatment and improve the outcome.