TY - JOUR
T1 - Combined immunodeficiency in the United States and Kuwait
T2 - Comparison of patients' characteristics and molecular diagnosis
AU - USIDNET Consortium
AU - Al-Herz, Waleed
AU - Notarangelo, Luigi D.
AU - Sadek, Ali
AU - Buckley, Rebecca
AU - Akhter, Javeed
AU - Ballas, Zuhair K.
AU - Ballow, Mark
AU - Bonilla, Francisco A.
AU - Chen, Karin
AU - Cheng, Laurence E.
AU - Cunningham-Rundles, Charlotte
AU - Garabedian, Elizabeth
AU - Haddad, Élie
AU - Kleiner, Gary
AU - Ochs, Hans D.
AU - Patel, Niraj
AU - Secord, Elizabeth A.
AU - Seroogy, Christine
AU - Sullivan, Kathleen
AU - Uygungil, Burcin
N1 - Publisher Copyright:
© 2015 Elsevier Inc.
PY - 2015/12/1
Y1 - 2015/12/1
N2 - 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.
AB - 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.
KW - Combined immunodeficiency
KW - Epidemiology
KW - Genetics
KW - Incidence
KW - Newborn screening
KW - Prevalence
KW - Registry
UR - http://www.scopus.com/inward/record.url?scp=84942599021&partnerID=8YFLogxK
U2 - 10.1016/j.clim.2015.07.013
DO - 10.1016/j.clim.2015.07.013
M3 - Article
C2 - 26248333
AN - SCOPUS:84942599021
SN - 1521-6616
VL - 161
SP - 170
EP - 173
JO - Clinical Immunology
JF - Clinical Immunology
IS - 2
ER -