DIAGNOSIS OF COMMON VARIABLE IMMUNODEFICIENCY IN A 13-YEAR-OLD BOY WITH FATAL NECROTIZING PNEUMONIA
Anuradha KWDA,Sumudu Seneviratne,Pujitha Wickramasinghe,Rajive de Silva,Amitha Fenando
Lady Ridgeway Hospital for Children Department of Paediatrics Faculty of Medicine University of Colombo Medical Research Institute Central Chest Clinic, Colombo
Background : Necrotizing pneumonia is a severe complication of community-acquired pneumonia. Common variable immune deficiency (CVID) is characterized by hypogamaglobulinaemia with phenotypically normal B cells. CVID generally presents late and may first manifest in late childhood, adolescence or young adulthood with an unusually severe infection. Early diagnosis and initiation of lifelong prophylaxis with intravenous immunoglobulin IVIG to prevent/minimize risk of severe infections could be lifesaving.
Case Presentation Summary : A 13-year-old boy with consanguineous parents, was transferred for further management of poorly resolving pneumonia. He had a right lower lobe pneumonia at 9 years and left lower lobe pneumonia complicated with empyema at 11 years of age, but had not been investigated further. On presentation child was febrile, in severe respiratory distress and showed clinical and radiological evidence of an empyema. C-reactive protein (345 mg/dl) and ESR (104mm) were high with neutrophil leucocytosis. Screening for tuberculosis was normal. Sputum culture isolated Methicillin Resistant Staphylococcus aureus sensitive to teicoplanin. Contrast Enhanced Computed Tomography(CECT) showed evidence of necrotizing pneumonia with early bronchiectasis. Thoracoscopic decortication was done on day 3 of admission and patient did not improve despite broad spectrum antibacterials, empirical systemic antifungals and intravenous immunoglobulin (IVIG) therapy Flow cytometry showed normal T and B cell counts. Serum isohamaglutinins were absent. Serum Immunoglobulin(Ig)levels for IgA 28 mg/dL (70-229mg/dl) and IgG 280 mg/dL (726-1085mg/dl) were low for age, and IgM was normal 70 mg/dl (35-72mg/dl). Nitroblue Tetrazolium and retroviral studies were negative. Common variable immune deficiency was diagnosed based on above. Over a period of 2 weeks ventilator settings were escalated and intercostal tubes were placed for bilateral pneumothoraces but patient deteriorated on ventilator, and expired despite aggressive management.
Learning Points/Discussion : Clinicians should be aware that an unusually severe episode of pneumonia warrants screening for underlying immune-deficiency. Earlier diagnosis of CVID and prophylaxis with IVIG may have prevented this death.
Keywords: Necrotizing pneumonia Common Variable Immune Deficiency Prophylaxis
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