Laaraje, AzzeddineBoucaid, AbdelhalimRadiI, AbdelilahAbilkassem, Rachid2025-10-132025-10-132024https://hdl.handle.net/20.500.14572/2644Azzeddine Laaraje Department of Paediatrics, Mohammed V Military Training Hospital, Mohammed V University, Rabat, Morocco Abdelhalim Boucaid Department of Pulmonology and Allergology, Mohammed V Military Training Hospital, Mohammed V University, Rabat, Morocco Abdelilah RadiI Department of Paediatrics, Mohammed V Military Training Hospital, Mohammed V University, Rabat, Morocco Rachid Abilkassem Department of Paediatrics, Mohammed V Military Training Hospital, Mohammed V University, Rabat, MoroccoAbstract: X-linked agammaglobulinemia (XLA) is characterized by early-onset recurrent bacterial infections, particularly affecting the respiratory tract. We report a 12-year-old male with XLA who presented with chronic cough and recurrent respiratory infections. Genetic testing confirmed a BTK gene mutation. Radiological findings revealed diffuse cylindrical bronchiectasis with bilateral bronchial wall thickening, and pulmonary function tests showed a mixed severe ventilatory pattern. Despite regular immunoglobulin replacement therapy, the patient developed chronic lung disease, highlighting the need for early diagnosis and aggressive management of respiratory manifestations.en-USX-Linked AgammaglobulinemiaProgressive Pulmonary DiseaseImmunoglobulin ReplacementArticle