Nesidioblastosis is a rare disorder characterized by hyperinsulinemic hypoglycemia. Correction of hypoglycemia in these infants is frequently difficult despite high glucose infusion. Diazoxide, a nondiuretic benzothiadiazine, has been used in various doses to achieve euglycemia. [1][2][3][4][5] In this paper, we describe the development of cardiorespiratory failure during diazoxide treatment of one patient with nesidioblastosis.
Case ReportA full-term Saudi male infant was born at an Aramco outlying clinic by spontaneous vaginal delivery. Birth weight was 4300 g. Apgar scores were 5 and 8 at 1 and 5 minutes, respectively. There was no history of maternal diabetes; the parents were first cousins. The baby was referred to Dhahran Health Center at 1 day of age because of refusal to feed and poor sucking. On admission, temperature was 36.4°C, respiratory rate 54 breaths per minute, heart rate 108 beats per minute, and blood pressure 82/38 mm Hg. Findings on physical examination, including chest, heart, and abdomen, were unremarkable with no cardiorespiratory distress. Blood glucose level on admission was 0.4 mmol/L. Despite glucose infusion up to 20 mg/kg/min, frequent episodes of hypoglycemia were observed. Glucagon injections were started after 2 days, and hydrocortisone was added 4 days after admission but was given for only 3½ days. Hypoglycemia continued to occur despite the above treatment. Hyperinsulinemia was confirmed by serum insulin levels of 122 and 309 pmol/L with simultaneous blood glucose of 1.0 and 1.2 mmol/L (insulin/glucose ratio of 122 and 257.5, respectively). At 6 days of age diazoxide was started at a dose of 17 mg/kg/day intravenously. Chlorothiazide was added as recommended in several reports (10 to 40 mg/kg/day).
1-5Hypoglycemic episodes were less frequent but not controlled, and occasional injections of glucagon and/or intravenous glucose boluses were required. After instituting the diazoxide and chlorothiazide therapy, heart and respiratory rates increased gradually. Diaphoresis, intercostal retraction, and a grade 3/6 systolic ejection murmur maximum at the left sternal border were observed. Periods of lethargy, fussiness, and irritability also were noted. Heart size, as measured by cardiothoracic ratio in chest roentgenograms, showed a steady increase (Figure 1).By 2 weeks of age, frank heart failure was evident with increased intensity of the murmur, hyperactive precordium, cardiomegaly, and hepatomegaly. Electrocardiogram showed right atrial enlargement and suggested left ventricular hypertrophy. Echocardiogram showed no structural abnormality; indices of left ventricular function were not calculated. The chest roentgenogram showed cardiomegaly and pulmonary edema, and several doses of furosemide were given. The child also became febrile, and he developed seizures. He was intubated and mechanically ventilated. Ampicillin and gentamicin were given after obtaining the appropriate cultures. Metabolic screening and the cultures were negative.Because of the continued hypoglycemic episodes, ...
