The Risk of the Next Child Getting Affected by Chronic Granulomatous Disease in Families with at Least One Autosomal Recessive CGD Child

Seyedeh Zalfa Modarresi; Shagayegh Tajik; Mohsen Badalzadeh; Mohammad Reza Fazlollahi; Massoud Houshmand; Marzieh Maddah; Zahra Alizadeh; Mohammad Nabavi; Nasrin Bazargan; Masoud Movahedi; Zahra Pourpak

doi:10.18502/ijaai.v22i1.12014

Seyedeh Zalfa Modarresi Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran
Shagayegh Tajik Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran
Mohsen Badalzadeh Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran
Mohammad Reza Fazlollahi Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran
Massoud Houshmand National Institute for Genetic Engineering and Biotechnology, Tehran, Iran
Marzieh Maddah Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran
Zahra Alizadeh Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran
Mohammad Nabavi Department of Pediatrics, Hazrat Rasool Hospital, Tehran University of Medical Sciences, Tehran, Iran
Nasrin Bazargan Department of Pediatrics, Faculty of Medicine, Kerman University of Medical Sciences. Kerman, Iran
Masoud Movahedi Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran
Zahra Pourpak Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran

DOI: https://doi.org/10.18502/ijaai.v22i1.12014

Keywords: Autosomal recessive; Chronic granulomatous disease; Parental consanguinity; Siblings

Abstract

Chronic granulomatous disease (CGD) is a rare primary immunodeficiency disorder more common in autosomal recessive (AR) than X-linked in Iran.

This study aimed to assess whether having a child with AR-CGD would increase the likelihood of the next child being affected by CGD.

Ninety-one families with at least one child affected by AR-CGD entered this study. Out of the 270 children, 128 were affected by AR-CGD. We used a cross tab for the odds ratio (OR) calculation, in which exposure to a previously affected child and the next child’s status were evaluated. This study illustrated that the chances of having another child afflicted with AR-CGD are significantly increased if the previous child had AR-CGD (OR=2.77, 95% CI=1.35-5.69).

Although AR disorders affect 25% of each pregnancy, we showed that the chance that the next child would be affected by CGD, given that the previous child was affected, is 2.77 times greater than in families with a normal child. It is recommended to warn families with one or more affected children to evaluate the risk of CGD in their subsequent pregnancies with prenatal diagnosis.