Mortality Among Primary Immunodeficient Patients in Kuwait

Abstract

RATIONALE: The aim of this project is to report mortality among primary immunodeficient patients in Kuwait.METHODS: Data were obtained from Kuwait National Primary Immunodeficiency Disorders Registry which was established in 2004.RESULTS: A total of 114 patients were registered (male: female ratio =1.3), with a follow-up of 65.23 months. The distribution of these patients according to each primary immunodeficiency category was: combined T- and B- cell immunodeficiencies (24%), predominantly antibody immunodeficiency (27%), other well defined immunodeficiencies (27%), diseases of immune dysregulation (13%), congenital defects of phagocyte number, function or both (6%) and complement deficiencies (3%). There were 26 deaths (23%) with male: female ratio of 0.85. The deaths were between 0.2 and 61.33 months after diagnosis. The age of death ranged between 2 to 156 months, with a mean of 23.04+33.32 months. Sixteen deaths (62%) were in the combined T-and B- cell immunodeficiencies group with a mean age of death in this group of 10.06+7.46 months. The overall post-diagnosis survival rate was 77.2% during the whole follow-up period. The patients' survival was influenced by gender, primary immunodeficiency category, consanguineous marriages in the patients' families, family history of primary immunodeficiency, use of IVIG and antimicrobials prophylaxis, and younger age at presentation. The causes of death in the reported patients were respiratory failure with ARDS secondary to pneumonia, sepsis, liver, renal and multiorgan failure.CONCLUSIONS: Our data show a high mortality rate among the registered patients probably related to the high number of patients with combined T- and B- cell immunodeficiencies. RATIONALE: The aim of this project is to report mortality among primary immunodeficient patients in Kuwait. METHODS: Data were obtained from Kuwait National Primary Immunodeficiency Disorders Registry which was established in 2004. RESULTS: A total of 114 patients were registered (male: female ratio =1.3), with a follow-up of 65.23 months. The distribution of these patients according to each primary immunodeficiency category was: combined T- and B- cell immunodeficiencies (24%), predominantly antibody immunodeficiency (27%), other well defined immunodeficiencies (27%), diseases of immune dysregulation (13%), congenital defects of phagocyte number, function or both (6%) and complement deficiencies (3%). There were 26 deaths (23%) with male: female ratio of 0.85. The deaths were between 0.2 and 61.33 months after diagnosis. The age of death ranged between 2 to 156 months, with a mean of 23.04+33.32 months. Sixteen deaths (62%) were in the combined T-and B- cell immunodeficiencies group with a mean age of death in this group of 10.06+7.46 months. The overall post-diagnosis survival rate was 77.2% during the whole follow-up period. The patients' survival was influenced by gender, primary immunodeficiency category, consanguineous marriages in the patients' families, family history of primary immunodeficiency, use of IVIG and antimicrobials prophylaxis, and younger age at presentation. The causes of death in the reported patients were respiratory failure with ARDS secondary to pneumonia, sepsis, liver, renal and multiorgan failure. CONCLUSIONS: Our data show a high mortality rate among the registered patients probably related to the high number of patients with combined T- and B- cell immunodeficiencies.