Patients with CVID had a greater likelihood of developing lung disease, possibly due to delayed diagnosis and immune dysregulation, as compared with XLA patients. Early diagnosis of patients with primary antibody deficiencies and adequate i.v. immunoglobulin replacement therapy substantially reduces the number of pulmonary infections. However, CVID patients are prone to progression of lung disease despite optimal immunoglobulin therapy because of the nature of the disease. This important issue should be addressed in further studies.

Bloodstream and disseminated infections due to Candida species are the most common life-threatening yeast infections among patients with profound and prolonged immunosuppression. 1 The majority of cases are healthcare associated, and common risk factors are the presence of central venous catheters, mechanical ventilation, gastrointestinal surgery, severe malignancies, haemodialysis, diabetes,

To determine the prevalence of vaccine-associated paralytic poliomyelitis (VAPP) in immunodeficient infants, we reviewed all documented cases caused by immunodeficiency-associated vaccine-derived polioviruses in Iran from 1995 through 2008. Changing to an inactivated polio vaccine vaccination schedule and introduction of screening of neonates for immunodeficiencies could reduce the risk for VAPP infection.

Summary Human Griscelli syndrome type 2 (GS‐2) is characterized by partial albinism and a severe immunologic disorder as a result of RAB27A mutations. In melanocytes, Rab27A forms a tripartite complex with a specific effector Slac2‐a/melanophilin and myosin Va, and the complex regulates melanosome transport. Here, we report a novel homozygous missense mutation of Rab27A, i.e. K22R, in a Persian GS‐2 patient and the results of analysis of the impact of the K22R mutation and the previously reported I44T mutation on protein function. Both mutations completely abolish Slac2‐a/melanophilin binding activity but they affect the biochemical properties of Rab27A differently. The Rab27A(K22R) mutant lacks the GTP binding ability and exhibits cytosolic localization in melanocytes. By contrast, neither intrinsic GTPase activity nor melanosomal localization of Rab27A is affected by the I44T mutation, but the Rab27A(I44T) mutant is unable to recruit Slac2‐a/melanophilin. Interestingly, the two mutations differently affect binding to other Rab27A effectors, Slp2‐a, Slp4‐a/granuphilin‐a, and Munc13‐4. The Rab27A(K22R) mutant normally binds Munc13‐4, but not Slp2‐a or Slp4‐a, whereas the Rab27A(I44T) mutant shows reduced binding activity to Slp2‐a and Munc13‐4 but normally binds Slp4‐a.