Combined immunodeficiency in the United States and Kuwait: Comparison of patients' characteristics and molecular diagnosis

Waleed Al-Herz 1, Luigi D Notarangelo 2, Ali Sadek 3, Rebecca Buckley 4; USIDNET Consortium

Affiliations

01 December 2015

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doi: 10.1016/j.clim.2015.07.013

Abstract

Aim: To compare different variables among (S)CID patients diagnosed in the USA and Kuwait.

Methods: Review of patients registered in The US Immune Deficiency Network registry or Kuwait National PID Registry between 2004 and 2014.

Results: Totals of 98 and 69 (S)CID patients were registered during the study period in the USIDNET registry and the KNPIDR, respectively. The average annual incidence rate for the period 2004-2014 of (S)CID in children in Kuwait was 13.01/100,000 children, with an estimated occurrence of 1/7500 live births. There were differences between the two countries in the following variables: age at onset and diagnosis, family history of (S)CID, parental consanguinity, and outcome. More than 14% of (S)CID patients from USIDNET registry were diagnosed through newborn screening.

Conclusions: Patients' characteristics and molecular causes of S(CID) are different between USA and Kuwait. NBS for SCID should be started in countries where the incidence of (S)CID is high.

Keywords: Combined immunodeficiency; Epidemiology; Genetics; Incidence; Newborn screening; Prevalence; Registry.

Figures

Fig. 1 Characteristics of S(CID) registered in the United States Immune Deficiency Network (USIDNET) registry and Kuwait National Primary Immunodeficiency Disorders Registry (KNPIDR).

Fig. 1 Characteristics of S(CID) registered in the United States Immune Deficiency Network (USIDNET) registry and Kuwait National Primary Immunodeficiency Disorders Registry (KNPIDR).

Fig. 2 Molecular diagnosis of S(CID) registered in the United States Immune Deficiency Network (USIDNET) registry and Kuwait National Primary Immunodeficiency Disorders Registry (KNPIDR).

Fig. 2 Molecular diagnosis of S(CID) registered in the United States Immune Deficiency Network (USIDNET) registry and Kuwait National Primary Immunodeficiency Disorders Registry (KNPIDR).

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