Diazoxide
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Pericardial effusion and hypertrichosis: 3 case reports In a case report, 3 patients (2 boys and 1 girl) aged 7 weeks–17 years were described, who developed pericardial effusion or hypertrichosis during treatment with diazoxide for congenital hyperinsulinism [not all routes and outcomes stated, times to reactions onsets not stated]. Patient 1: A 7-week-old boy, who had a history of cyanosis and apnoea, was found to have hyperinsulinaemic hypoglycaemia. Initially, he was given dextrose and glucagon to maintain euglycaemia, which was weaned after starting treatment with oral diazoxide 15 mg/kg/day along with hydrochlorothiazide. A brain MRI showed typical changes of hypoglycaemic brain injury, including areas of diffusion restriction in both occipital lobes and around the posterior limits of the sylvian fissure bilaterally. His glucose levels were stabilised and he was discharged home on day 40 of life on hydrochlorothiazide and diazoxide. He was also given feeds fortified with powdered carbohydrate supplement. He again presented on day 49 of life due to respiratory distress. Chest X-ray revealed cardiomegaly with pleural spaces and clear lungs. ECG showed a 5–6mm pericardial effusion with normal biventricular systolic function. He was also found to have small restrictive patent ductus arteriosus. He was then fluid restricted. He stopped receiving diazoxide therapy and diuretics were added. He received diazoxide therapy for a total of 6.5 weeks. After stopping diazoxide, he showed a rapid improvement and the pericardial effusion did not recur. However, for glycaemic control he required increased fortification of his feeds. Later, his prolonged transient hyperinsulinism also had resolved. Patient 2: An 8-month-old girl, who had a history of Sotos syndrome (c.6311-6312 deletion of the NSD1 gene), was found to have hypoglycaemia at 12 hours of life. She was initially managed with nasogastric feeds and dextrose. At 11 weeks of age, she was found to have mild hypoglycaemia. Therefore, she was started on diazoxide 10 mg/kg/day along with hydrochlorothiazide and her blood glucose levels were stabilised. With time, she exhibited marked global developmental delay and overgrowth. At 8 months of age, she presented with a 48 hours history of increased work of breathing, worsening lethargy, tachypnoea, fever and pallor. Chest X-ray revealed massive cardiomegaly. ECG showed structurally normal heart without evidence of pulmonary hypertension, but a large global pericardial effusion along with fibrin strands and loculations. She also had right atrial and ventricular collapse indicating cardiac tamponade. Hence, she underwent emergency subxiphoid drainage of turbid pericardial fluid and was stabilised haemodynamically. High numbers of polymorphonuclear cells were found in pericardial fluid without growth of any organisms. Histology of a pericardial biopsy revealed non-specific chronic reactive changes. CRP levels were found markedly elevated. The girl was weaned off on diazoxide over 5 days and was receiving diuretic therapy. S
