LUNG FIBROSIS AND RECURRENT PNEUMOTHORAX REVEALING CHRONIC SEPTIC GRANULOMATOSIS IN AN ADOLESCENT

Abstract

TYPE: Late Breaking Case Report TOPIC: Pediatrics INTRODUCTION: Primary spontaneous recurrent pneumothorax is uncommon in children. It may be life threatening and reveal a pulmonary serious disease. CASE PRESENTATION: A 16-year-old adolescent with 3rd degree cousin parents and a history of hypotrophy since his first months of life. At the age of 6 years, he developed chronic cough, wheezing and dyspnea with rapid progression to chronic respiratory failure, pulmonary arterial hypertension and dependence of the oxygen. Chest CT showed pulmonary fibrosis with diffuse septal thickening. Etiological assessment including sweat test, humoral and cellular immunity tests, immunological record and Broncho alveolar lavage was negative. Later, he suffered from spontaneous recurrent pneumothorax managed by pleural drainage despite pleurodesis. The diagnosis of chronic septic granulomatosis was made at the age of 16 years on the basis of a Nitroblue Tetrazolium test not previously performed. Thus, he was treated by corticosteroids and had a bone marrow transplantation wich allowed an improved quality of life. DISCUSSION: severe and recurrent respiratory manifestations require meticulous approach of investigations. Indeed, significant functional and vital complications may occur as a result of delayed diagnosis and treatment. In our case, a Nitroblue Tetrazolium test or a pulmonary biopsy could have accelerated the diagnosis and improved the prognosis of our patient. CONCLUSIONS: Recurrent pneumothorax and pulmonary fibrosis may be manifestations of primary immune deficiency in children. Thus, the etiologic assessment must be exhaustive before an idiopathic origin is considered. DISCLOSURE: Nothing to declare. KEYWORD: Pneumothorax