Objective: To present the report from the Kuwait National Primary Immunodeficiency Registry between 2004 and 2018. Methods: The patients were followed prospectively between January 2004 and December 2018 and their collected data included sociodemographic, diagnosis, clinical presentation, laboratory tests, and treatment. Results: A total of 314 PID patients (165 males and 149 females) were registered during the study period. Most of the patients ( n = 287, 91.4%) were Kuwaiti nationals and the prevalence among Kuwaitis was 20.27/100,000 with a cumulative incidence of 24.96/100,000 Kuwaitis. The distribution of the patients according to PID categories was as follow: immunodeficiencies affecting cellular and humoral immunity, 100 patients (31.8%); combined immunodeficiencies with associated syndromic features, 68 patients (21.7%); predominantly antibody deficiencies, 56 patients (17.8%); diseases of immune dysregulation, 47 patients (15%); congenital defects of phagocyte number or function, 20 patients (6.4%); autoinflammatory disorders, 1 patient (0.3%); and complement deficiencies, 22 patients (7%). The mean age of the patients at onset of symptoms was 26 months while the mean age at diagnosis was 53 months and the mean delay in diagnosis was 27 months. Most of the patients ( n = 272, 86%) had onset of symptoms before the age of 5 years. Parental consanguinity rate within the registered patients was 78% and a positive family history of PID was noticed in 50% of the patients. Genetic testing was performed in 69% of the patients with an overall diagnostic yield of 90%. Mutations were identified in 46 different genes and more than 90% of the reported genetic defects were transmitted by an autosomal recessive pattern. Intravenous immunoglobulins and stem cell transplantation were used in 58% and 25% of the patients, respectively. There were 81 deaths (26%) among the registered patients with a mean age of death of 25 months. Conclusions: PID is not infrequent in Kuwait and the reported prevalence is the highest in the literature with increased proportion of more severe forms. Collaborative efforts including introduction of newborn screening should be implemented to diagnose such cases earlier and improve the quality of life and prevent premature deaths.
Objective: To describe the epidemiological and clinical characteristics of children hospitalized for the 2009 influenza A H1N1 infection in Kuwait. Materials and Methods: A retrospective chart review of hospitalized children with laboratory-confirmed influenza A H1N1 infection in two hospitals in Kuwait was conducted. Epidemiological characteristics, clinical features, risk factors for severe disease, complications and mortality were analyzed. Results: The medical records of 197 children hospitalized for the 2009 pandemic H1N1 infection from August 2009 to January 2010 were reviewed. The majority of the children (104; 52.8%) were admitted during the month of October. The median age was 2 years. Most of the admitted children were in two age categories: 64 infants ≤1 year (32%) and 62 schoolchildren >5 years (31%). The most frequent presentations were fever in 193 (98%), cough in 155 (79%) and runny nose in 105 (53%) cases. The majority of the admitted children (109; 55%) had been previously healthy. All children received an antiviral agent (oseltamivir), and antibiotics were administered to 147 (75%). Bacterial co-infections occurred in 3 (1.5%) of all hospitalized children. Six (3%) children were admitted to the intensive care unit, of whom 4 (66%) required artificial ventilation. There was only 1 mortality. Conclusions: The pandemic H1N1 infection was associated with a wide spectrum of clinical manifestations. The majority of hospitalized children had previously been healthy. Most of the admitted children had an uncomplicated clinical course.
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