Figure legends:
Figure 1: (A) Thoracolumbar scoliosis. (B), (C) and (D) Chest radiographs showing left middle zone (B), lower zone (C), and upper zone pneumonia.
Figure 2: Pedigree of the index patient family indicating X-linked inheritance.
Figure 3: (A) Photomicrograph shows glomerulus with mesangiolysis (encircled) and swollen endothelial cells (arrow), the features of thrombotic microangiopathy (Periodic acid-schiff stain, 40x magnification). Flow cytometric expression of Bruton thymidine kinase (Btk) protein on lymphocytes showing reduced expression in index child (B), and mother (D) when compared to the healthy unrelated control (C).