Journal of Clinical Immunology

, Volume 32, Issue 3, pp 467–473 | Cite as

Survival and Predictors of Death Among Primary Immunodeficient Patients: A Registry-Based Study

  • Waleed Al-HerzEmail author
  • Mohamed A. A. Moussa



The aims of this study were to investigate survival among patients with primary immunodeficiency disorders (PID) in Kuwait and to determine whether certain variables were associated with increased risk of death.


The data of 176 patients (98 males and 78 females) were extracted from the Kuwait National Primary Immunodeficiency Disorders Registry and the observation period was from January 2004 to July 2011.


The distribution of the reported patients was combined T- and B-cell immunodeficiencies (30.1%), predominantly antibody immunodeficiency (19.9%), other well-defined immunodeficiencies (25%), diseases of immune dysregulation (14.8%), congenital defects of phagocyte number, function or both (6.25%), and complement deficiencies (4.0%). In a total of 619.1 patient-years at risk, 48 patients died (mortality incidence rate 77.53 per 1,000 person-years). The overall survival in the studied cohort was 72.7% (72.4% for males and 73.1% for females). The most common cause of death was sepsis (46%) followed by pneumonia (29%). The probabilities that a patient survived 2, 4, and 6 years after onset of symptoms were 76%, 73%, and 69%, respectively. The variables that were found to be predictors for death are parental consanguinity, sepsis, adenovirus and CMV infections, failure to thrive, PID category, and onset age <6 months.


Patients with PID have decreased probabilities of survival that are variable between PID categories. Early diagnosis and aggressive therapeutic interventions specifically of patients with history of the variables associated with increased risk of death may help increase their chance of survival.


Primary immunodeficiency survival death registry consanguinity failure to thrive 



The Kuwait National Primary Immunodeficiency Registry (KNPIDR) was established by a fund from the Kuwait Foundation for the Advancement of Science (KFAS).

Conflicts of Interest

The authors declare that they have no conflict of interest.


  1. 1.
    Ballow M, Notarangelo L, Grimbacher B, Cunningham-Rundles C, Stein M, Helbert M, Gathmann B, Kindle G, Knight AK, Ochs HD, Sullivan K, Franco JL. Immunodeficiencies. Clin Exp Immunol. 2009;158 Suppl 1:14–22.PubMedCrossRefGoogle Scholar
  2. 2.
    Notarangelo LD, Casanova JL. Primary immunodeficiencies: increasing market share. Curr Opin Immunol. 2009;21(5):461–5.PubMedCrossRefGoogle Scholar
  3. 3.
    Morinishi Y, Imai K, Nakagawa N, Sato H, Horiuchi K, Ohtsuka Y, Kaneda Y, Taga T, Hisakawa H, Miyaji R, Endo M, Oh-Ishi T, Kamachi Y, Akahane K, Kobayashi C, Tsuchida M, Morio T, Sasahara Y, Kumaki S, Ishigaki K, Yoshida M, Urabe T, Kobayashi N, Okimoto Y, Reichenbach J, Hashii Y, Tsuji Y, Kogawa K, Yamaguchi S, Kanegane H, Miyawaki T, Yamada M, Ariga T, Nonoyama S. Identification of severe combined immunodeficiency by T-cell receptor excision circles quantification using neonatal Guthri cards. J Pediatr. 2009;155(6):829–33.PubMedCrossRefGoogle Scholar
  4. 4.
    Modell V, Gee B, Lewis DB, Orange JS, Roifman CM, Routes JM, Sorensen RU, Notarangelo LD, Modell F. Global study of primary immunodeficiency diseases (PI)—diagnosis, treatment, and economic impact: an updated report from the Jeffrey Modell Foundation. Immunol Res. 2011;51(1):61–70.PubMedCrossRefGoogle Scholar
  5. 5.
    Al-Herz W. Primary immunodeficiency disorders in Kuwait: first report from Kuwait National Primary Immunodeficiency Registry (2004–2006). J Clin Immunol. 2008;28(2):186–93.PubMedCrossRefGoogle Scholar
  6. 6.
    International Union of Immunological Societies Expert Committee on Primary Immunodeficiencies, Notarangelo LD, Fischer A, Geha RS, Casanova JL, Chapel H, Conley ME, Cunningham-Rundles C, Etzioni A, Hammartröm L, Nonoyama S, Ochs HD, Puck J, Roifman C, Seger R, Wedgwood J. Primary immunodeficiencies: 2009 update. J Allergy Clin Immunol. 2009;124(6):1161–78.PubMedCrossRefGoogle Scholar
  7. 7.
    Grumach AS, Duarte AJ, Bellinati-Pires R, Pastorino AC, Jacob CM, Diogo CL, Condino-Neto A, Kirschfink M, Carneiro-Sampaio MM. Brazilian report on primary immunodeficiencies in children: 166 cases studied over a follow-up time of 15 years. J Clin Immunol. 1997;17(4):340–5.PubMedCrossRefGoogle Scholar
  8. 8.
    Rezaei N, Aghamohammadi A, Moin M, Pourpak Z, Movahedi M, Gharazlou M, Atarod L, Ghazi BM, Isaeian A, Mahmoudi M, Abolmaali K, Mansouri D, Arshi S, Tarash NJ, Sherkat R, Akbari H, Amin R, Alborzi A, Kashef S, Farid R, Mahammadzadeh I, Shabestari MS, Nabavi M, Farhoudi A. Frequency and clinical manifestations of patients with primary immunodeficiency disorders in Iran: update from the Iranian primary immunodeficiency registry. J Clin Immunol. 2006;26(6):519–32.PubMedCrossRefGoogle Scholar
  9. 9.
    Fasth A. Primary immunodeficiency disorders in Sweden: cases among children 1974–1979. J Clin Immunol. 1982;2:86–92.PubMedCrossRefGoogle Scholar
  10. 10.
    Wang LL, Jin YY, Hao YQ, Wang JJ, Yao CM, Wang X, Cao RM, Zhang H, Chen Y, Chen TX. Distribution and clinical features of primary immunodeficiency diseases in Chinese children (2004–2009). J Clin Immunol. 2011;31(3):297–308.PubMedCrossRefGoogle Scholar
  11. 11.
    Moratto D, Giliani S, Bonfim C, Mazzolari E, Fischer A, Ochs HD, Cant AJ, Thrasher AJ, Cowan MJ, Albert MH, Small T, Pai SY, Haddad E, Lisa A, Hambleton S, Slatter M, Cavazzana-Calvo M, Mahlaoui N, Picard C, Torgerson TR, Burroughs L, Koliski A, Neto JZ, Porta F, Qasim W, Veys P, Kavanau K, Hönig M, Schulz A, Friedrich W, Notarangelo LD. Long-term outcome and lineage-specific chimerism in 194 patients with Wiskott–Aldrich syndrome treated by hematopoietic cell transplantation in the period 1980–2009: an international collaborative study. Blood. 2011;118(6):1675–84.PubMedCrossRefGoogle Scholar
  12. 12.
    Bordon V, Gennery AR, Slatter MA, Vandecruys E, Laureys G, Veys P, Qasim W, Friedrich W, Wulfraat NM, Scherer F, Cant AJ, Fischer A, Cavazzana-Calvo M, Bredius RG, Notarangelo LD, Mazzolari E, Neven B, Güngör T. Inborn Error Working Party of the European Bone Marrow Transplantation (EBMT) group. Clinical and immunologic outcome of patients with cartilage hair hypoplasia after hematopoietic stem cell transplantation. Blood. 2010;8:116(1):27–35.CrossRefGoogle Scholar
  13. 13.
    Notarangelo LD, Notarangelo LD, Ochs HD. WASP and the phenotypic range associated with deficiency. Curr Opin Allergy Clin Immunol. 2005;5(6):485–90.PubMedCrossRefGoogle Scholar
  14. 14.
    Lee PP, Chen TX, Jiang LP, Chan KW, Yang W, Lee BW, Chiang WC, Chen XY, Fok SF, Lee TL, Ho MH, Yang XQ, Lau YL. Clinical characteristics and genotype–phenotype correlation in 62 patients with X-linked agammaglobulinemia. J Clin Immunol. 2010;30(1):121–31.PubMedCrossRefGoogle Scholar
  15. 15.
    Gichuhi C, Obimbo E, Mbori-Ngacha D, Mwatha A, Otieno P, Farquhar C, Wariua G, Wamalwa D, Bosire R, John-Stewart G. Predictors of mortality in HIV-1 exposed uninfected post-neonatal infants at the Kenyatta National Hospital, Nairobi. East Afr Med J. 2005;82(9):447–51.PubMedGoogle Scholar
  16. 16.
    Melikian G, Mmiro F, Ndugwa C, Perry R, Jackson JB, Garrett E, Tielsch J, Semba RD. Relation of vitamin A and carotenoid status to growth failure and mortality among Ugandan infants with human immunodeficiency virus. Nutrition. 2001;17(7–8):567–72.PubMedCrossRefGoogle Scholar
  17. 17.
    Bobat R, Coovadia H, Moodley D, Coutsoudis A. Mortality in a cohort of children born to HIV-1 infected women from Durban, South Africa. S Afr Med J. 1999;89(6):646–8.PubMedGoogle Scholar
  18. 18.
    Al-Herz W, Naguib KK, Notarangelo LD, Geha RS, Alwadaani A. Parental consanguinity and the risk of primary immunodeficiency disorders: report from the Kuwait National Primary Immunodeficiency Disorders Registry. Int Arch Allergy Immunol. 2011;154(1):76–80.PubMedCrossRefGoogle Scholar
  19. 19.
    Aghamohammadi A, Abolhassani H, Moazzami K, Parvaneh N, Rezaei N. Correlation between common variable immunodeficiency clinical phenotypes and parental consanguinity in children and adults. J Investig Allergol Clin Immunol. 2010;20(5):372–9.PubMedGoogle Scholar
  20. 20.
    Rivoisy C, Gérard L, Boutboul D, Malphettes M, Fieschi C, Durieu I, Tron F, Masseau A, Bordigoni P, Alric L, Haroche J, Hoarau C, Bérézné A, Carmagnat M, Mouillot G, Oksenhendler E; for the DEFI study group. Parental consanguinity is associated with a severe phenotype in common variable immunodeficiency. J Clin Immunol. 2011 (in press)Google Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Department of Pediatrics, Faculty of MedicineKuwait UniversityKuwaitKuwait
  2. 2.Department of Community Medicine and Behavioral Sciences, Faculty of MedicineKuwait UniversityKuwaitKuwait
  3. 3.Allergy and Clinical Immunology Unit, Department of PediatricsAl-Sabah HospitalKuwaitKuwait

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