Bronchial asthma with ABPA presenting as PTE
A 9-year-old boy presented with bluish discoloration of the nails and lips since last one year andanasarca (generalized swelling), worsening cough, respiratory distress, and periumbilical pain abdomen since 20 days previously. He had past history suggestive of asthma (recurrent episodes of cough and respiratory distress since the first year of life), for which he had received irregular treatment. He was not receiving any regular inhaled steroids. He had no significant febrile illnesses and loose stools in the past. His sibling had bronchial asthma. There was no history of tuberculosis or sibling death in family. On admission, patient found to have central cyanosis, tachycardia, marked respiratory distress with normal blood pressure. The child had anasarca, grade III clubbing, and raised jugular venous pressure. His weight and height both were below the third percentile. Examination revealed a hyper-resonant chest with generalized crepts and rhonchi. The second heart sound was loud, but no murmur was detected. He had tender hepatomegaly.\nInvestigations showed hemoglobin of 150 g/L, total leukocyte count 12.8 × 109/L with neutrophils 80%, lymphocytes 18%, monocytes 1%, and eosinophils 1%. Arterial blood gas analysis on 100% oxygen showed pH of 7.36, pCO2 59.5 mm of Hg, pO2 116 mm of Hg, HCO3 32.6 mmol/L, base excess 5.2 and oxygen saturation 98.3%, suggesting chronic respiratory acidosis. Tubercular workup was negative. Sweat chloride level (pilocarpine iontophoresis method) was 42 meq/L (borderline). HIV was non-reactive. Chest x-ray revealed cardiomegaly and bilateral lung infiltrates. Electrocardiography revealed right axis deviation and p-pulmonale. HRCT chest showed central bronchiectasis [], minimal right pleural and pericardial effusion, and gross cardiomegaly. Chest angiography revealed dilated main pulmonary artery and filling defect in right branch of pulmonary artery []. Echocardiography finding were: Dilated right-sided heart, right ventricular dysfunction, severe pulmonary artery hypertension, and right atrial clot. Lung ventilation-perfusion scan was suggestive of pulmonary thrombo-embolism. Serum IgE levels were 1100 IU/ml (normal 50-100 IU/ml). Immediate skin hypersensitivity to Aspergillus fumigatus was positive. Serum precipitants against aspergillus and candida were negative. Coagulogram revealedPprothrombin time index of 82%, APTT 33 seconds, INR 1.2 and presence of fibrin degradation products.\nThe patient was managed with salbutamol (0.15 mg/kg/dose) and ipratropium bromide (0.5 mg/dose) nebulization and intravenous hydrocortisone (10 mg/kg stat followed by 2 mg/kg every 6 hourly) for exacerbation of asthma. For congestive heart failure, digoxin (3 μg/kg/day) and furosamide (2 mg/kg/day) was given. He received injection ceftriaxone (100 mg/kg/day) for 14 days. Low molecular weight heparin (1.0 mg/kg 12 hourly for 5 days) followed by warfarin (0.1 mg/kg/day) was administered for thrombo-embolism after chest angiography. He improved and was discharged on request on warfarin, home oxygen therapy, oral steroids (prednisolone 2 mg/kg/day), and inhaled steroids (budesonide 400 μg/day) after three weeks of admission. He didn’t return for follow-up, and on enquiry, we came to know that he expired after few days of discharge.