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Inicio Allergologia et Immunopathologia Long-term follow-up of ninety eight Iranian patients with primary immune deficie...
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Vol. 44. Issue 4.
Pages 322-330 (July - August 2016)
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Vol. 44. Issue 4.
Pages 322-330 (July - August 2016)
Original Article
DOI: 10.1016/j.aller.2015.09.006
Long-term follow-up of ninety eight Iranian patients with primary immune deficiency in a single tertiary centre
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M. Nabavia, S. Arshia, M.H. Bemaniana, A. Aghamohammadib, D. Mansouric, M. Hedayatd, A. Nateghiane, S. Noorbakhshf, F. Ehsanipourf, M. Faranoushg, R. Shakerih, M. Mesdaghii, B. Taghvaeia, B. Ghalebaghia,j, D. Babaiek, A. Bahramia, M. Fallahpoura, H. Esmaeilzadehl, A. Ali Hamidiehm, M. Rekabia, J. Ahmadiana, N. Eslamia, S. Shokria, M. Afsharn, F. Jalalia, N. Akbarpoura, R. Molatefia,
Corresponding author
, N. Rezaeib,o,p
a Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran
b Research Center for Immunodeficiency, Children's Medical Center, Tehran University of Medical Sciences (TUMS), Tehran, Iran
c Department of Internal Medicine, Division of Infectious Diseases and Clinical Immunology, National Research Institute of Tuberculosis and Lung Diseases, Masih-e-Daneshvari Hospital, Shaheed Beheshti University of Medical Sciences (SBUMS), Tehran, Iran
d Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA
e Department of Infectious Diseases, Ali-Asghar Hospital, IUMS, Tehran, Iran
f Research Center of Pediatric Infectious Diseases, Rasool-e-Akram Hospital, IUMS, Tehran, Iran
g Department of Pediatric Oncology, Rasool-e-Akram Hospital, IUMS, Tehran, Iran
h Digestive Oncology Research Center, Digestive Disease Research Institute, TUMS, Tehran, Iran
i Department of Immunology, Mofid Children's Hospital, SBUMS, Tehran, Iran
j Research Center of Otolaryngology and Head & Neck Surgery, IUMS, Tehran, Iran
k Department of Allergy and Clinical Immunology, Mofid Hospital, SBUMS, Tehran, Iran
l Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
m Hematology Oncology and Stem Cell Transplantation Research Center, TUMS, Iran
n Antimicrobial Resistance Research Center, Rasool-e-Akram Hospital, IUMS, Tehran, Iran
o Molecular Immunology Research Center, and Department of Immunology, School of Medicine, TUMS, Tehran, Iran
p Universal Scientific Education and Research Network (USERN), Tehran, Iran
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Tables (4)
Table 1. Demographic characteristics and outcomes of ninety eight Iranian patients with primary immune deficiency.
Table 2. Clinical manifestations and complications of 63 Iranian patients with predominantly antibody deficiency.
Table 3. Common clinical manifestations in twelve Iranian combined immunodeficiency disorders.
Table 4. Clinical manifestations and complications of eleven Iranian patients with chronic granulomatous disease.
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Abstract
Purpose

The aim was to describe the clinical manifestations, complications and long-term outcome of a cohort of Iranian patients with primary immune deficiency (PID).

Method

We retrospectively studied the demographic, clinical and immunological characteristics of the PID patients in a single tertiary centre, from January 1989 to July 2014. The patients were classified according to the International Union of Immunological Societies Expert Committee on PID.

Results

98 patients were diagnosed with and followed-up for 15 disorders. The mean age at onset and diagnosis and the diagnostic delay were 8±10, 14.2±13.1 and 6.1±7 years, respectively. Parental consanguinity rate was 57%. Predominantly Antibody Deficiency was the most common diagnosis (n=63), followed by congenital defects of phagocytes (n=16), combined immunodeficiencies (n=12), well defined syndromes (n=4) and defects in innate immunity (n=3). Recurrent sinopulmonary infection was the most common presentation. Active infections were treated appropriately, in addition to prophylactic therapy with IVIG and antimicrobials. Not all the patients were compliant with prophylactic regimens due to cost and unavailability. One SCID patient underwent successful bone marrow transplantation. The total mortality rate was 19% during the follow-up period (7.8±7.6 years). The mean age of living patients at the time of study was 23±11.7 years.

Conclusions

Physicians awareness of PID has been rising dramatically in Iran, ensuring an increasing number of patients being diagnosed and treated. More effective treatment services, including health insurance coverage and drug availability are needed to improve the outcome of PID patients.

Keywords:
Consanguinity
Complications
Follow-up
Iran
Manifestations
Mortality
Primary immune deficiency

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