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Journal of Tropical Pediatrics Advance Access published online on December 13, 2006
Journal of Tropical Pediatrics, doi:10.1093/tropej/fml077
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© The Author [2006]. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Mortality and Morbidity in Common Variable Immunodeficiency

Asghar Aghamohammadia,b, Nima Pouladib, Nima Parvaneha, Mehdi Yeganehb, Masoud Movahedia, Mohamad Gharagoloua, Zahra Pourpakb, Nima Rezaeib, Ali Salavatia,c, Sina Abdollahzadea,c and Mostafa Moina,b

aChildren's Medical Center, Department of Pediatrics, bImmunology, Asthma and Allergy Research Institute, and cStudents’ Scientific Research Center (SSRC), Vice Chancellor for Research, Tehran University of Medical Sciences, Tehran, Iran

Correspondence: Asghar Aghamohammadi, Division of Clinical Immunology of Children's Medical Center, Tel.: +98 2166935855; Fax: +98 2166428995. E-mail < aghamohammadi{at}sina.tums.ac.ir>.


  Abstract

Common variable immunodeficiency (CVID) is a heterogeneous group of disorders, characterized by hypogammaglobulinemia, defective specific-antibody production resulting in recurrent bacterial infections. Delay in diagnosis and inadequate treatment result in increased irreversible complications and mortality. To determine persistent morbidities, mortality rate and survival in Iranian patients with CVID, hospital records of 72 (39 males and 33 females) diagnosed CVID patients were reviewed. Probabilities of survival after diagnosis of CVID were estimated from Kaplan–Meier life tables.

Studied patients were enrolled over a 20-year period (1984–2005). The most commonly observed complication was bronchiectasis (24 cases), followed by splenomegaly, intestinal villous atrophy (11 cases), and failure to thrive (10 cases). Post-diagnosis survival was estimated as 65% for the first 6.5 years, which remains the same until 14 years after diagnosis when the survival curve drops to nearly 45%. The mortality rate among patients who had no regular visits and did not receive periodical IVIG was more remarkable when compared with those who had been followed up timely (p-value = 0.001).The most common cause of death was respiratory failure. Based on our observation, it can be highlighted that all patients with CVID, even under regular immunoglobulin replacement, need close monitoring for early detection of complications and introduction of appropriate management.


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