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).