Summary:
Chronic granulomatous disease (CGD) is a primary
immunodeficiency disease which results from absence of the
NADPH oxidase in the professional phagocytic cells
neutrophils, monocytes, macrophages and eosinophils.
Deficiency of this oxidase renders the patient liable to infection
by bacteria and fungi, and, as the name of the disease suggests,
to chronic granulomatous inflammation. Here, a young boy
presented with increasing breathlessness and productive cough
had recurrent episode of pulmonary infection since his
childhood. Repeated Chest X-ray and CT scan showed
homogenous opacities at different places of lung in different
occasions with bilateral reticulo-nodular opacities.
Mulipleoraganisms were isolated from sputum at different
times. A nitroblu-tetrazolium test (NBT) was done abroad
which was positive and confirmed his diagnosis. Since then,
He had prophylactic fluconazol and sulphamethoxazoltrimethoprime
daily and pneumocaccal and influenza
vaccination regularly and proper treatment of acute infective
episodes accordingly. In spite all these measures, repeated
infection caused grievous harm to his lung leading to
irreversible pulmonary fibrosis and bronchiectasis. As a
consequence, he became home bound, oxygen dependant and
dependant on regular use of long acting bronchodilators in
different form. Hematopoeitic stem cell transplantation was
advised which was not affordable for his parents. Now, this
young boy is waiting for further assaults to his lungs and
further deterioration and ultimate hopeless outcomes.
Key words: Chronic granulomatous disease, Immunodeficiency
disorder.
(J Banagladesh Coll Phys Surg 2015; 33: 156-160)