Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience

Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience Publisher Pubmed

Fazlollahi MR¹ ; Pourpak Z¹ ; Hamidieh AA² ; Movahedi M³ ; Houshmand M^{1, 4} ; Badalzadeh M¹ ; Nourizadeh M¹ ; Mahloujirad M¹ ; Arshi S⁵ ; Nabavi M⁵ ; Gharagozlou M³ ; Khayatzadeh A³ ; Dabbaghzade A⁶ ; Atarod L⁷ Show All Authors

Fazlollahi MR¹
Pourpak Z¹
Hamidieh AA²
Movahedi M³
Houshmand M^{1, 4}
Badalzadeh M¹
Nourizadeh M¹
Mahloujirad M¹
Arshi S⁵
Nabavi M⁵
Gharagozlou M³
Khayatzadeh A³
Dabbaghzade A⁶
Atarod L⁷
Zandieh F⁸
Sadeghi Shabestary M⁹
Mesdaghi M¹⁰
Mohammadzadeh I¹¹
Mahdaviani SA¹²
Eslamian MH¹³
Pesaran F¹
Bahraminia E³
Abolnezhadian F¹⁴
Arij Z¹
Moin M¹

Show Affiliations

1. Immunology Asthma and Allergy Research Institute (IAARI), Tehran University of Medical Sciences, Tehran, Iran
2. Hematology-Oncology and Stem Cell Transplantation Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
3. Department of Allergy and Clinical Immunology, Childrenâ€™s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
4. Department of Medical Genetics, National Institute of Genetic Engineering and Biotechnology (NIGEB), Tehran, Iran
5. Department of Allergy and Clinical Immunology, Hazrat Rasoul Hospital, Iran University of Medical Sciences, Tehran, Iran
6. Department of Allergy and Clinical Immunology, Mazandaran University of Medical Sciences, Sari, Iran
7. Department of Pediatrics, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran
8. Department of Allergy and Clinical Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran
9. Department of Allergy and Clinical Immunology, Tabriz Childrenâ€™s Hospital, Tabriz University of Medical Sciences, Tabriz, Iran
10. Department of Allergy and Clinical Immunology, Mofid Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
11. Department of Immunology and Allergy, Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran
12. Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
13. Allergy and Clinical Immunology Group, Faculty of Medicine, Hamedan University of Medical Sciences, Hamedan, Iran
14. Department of Immunology and Allergy, Ahvaz University of Medical Sciences, Ahvaz, Iran

Source: Journal of Investigational Allergology and Clinical Immunology Published:2017

Abstract

Introduction: Severe combined immunodeficiency (SCID) is a life-threatening pediatric disease. We report on the clinical evaluation, immunological assessment, molecular analysis, and outcomes of SCID patients in a tertiary referral center in Iran. Methods: From January 2006 to December 2015, we performed a prospective cohort study in which initial screening and advanced immunological tests were carried out on patients suspected of having SCID. Genetic analysis was also performed to confirm the diagnosis. Results: A total of 63 patients were diagnosed with SCID (43 male [68.3%]). The median age at onset and diagnosis and diagnostic delay were 40 and 110 and 60 days respectively. A total of 49 patients (77.8%) had a history of BCG vaccination, and of these, one-third experienced BCG-associated complications. The most common clinical manifestations were pneumonia, recurrent oral candidiasis, chronic diarrhea, and failure to thrive. Of the thirteen patients who underwent hematopoietic stem cell transplantation, 8 survived and 5 died before they could receive the transplant. Most patients (34.9%) were classified as having T–B–NK+ SCID and had a mutation in the RAG2 or RAG1 gene. Conclusion: Autosomal recessive SCID is the most common type in Iranian patients. Providing high-quality training to physicians and patients’ families to reduce the diagnostic delay should be prioritized. It is also important to raise awareness of live vaccination and to expand stem cell donor registries to speed up the transplantation process. © 2017 Esmon Publicidad.

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Style	Citing Format
MLA	Fazlollahi MR, et al.. "Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience." Journal of Investigational Allergology and Clinical Immunology, vol. 27, no. 5, 2017, pp. 299-304.
APA	Fazlollahi MR, Pourpak Z, Hamidieh AA, Movahedi M, Houshmand M, Badalzadeh M, Nourizadeh M, Mahloujirad M, Arshi S, Nabavi M, Gharagozlou M, Khayatzadeh A, Dabbaghzade A, Atarod L, Zandieh F, Sadeghi Shabestary M, Mesdaghi M, Mohammadzadeh I, Mahdaviani SA, ... Moin M (2017). Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience. Journal of Investigational Allergology and Clinical Immunology, 27(5), 299-304.
Chicago	Fazlollahi MR, Pourpak Z, Hamidieh AA, Movahedi M, Houshmand M, Badalzadeh M, Nourizadeh M, et al.. "Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience." Journal of Investigational Allergology and Clinical Immunology 27, no. 5 (2017): 299-304.
Harvard	Fazlollahi MR et al. (2017) 'Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience', Journal of Investigational Allergology and Clinical Immunology, 27(5), pp. 299-304.
Vancouver	Fazlollahi MR, Pourpak Z, Hamidieh AA, Movahedi M, Houshmand M, Badalzadeh M, et al.. Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience. Journal of Investigational Allergology and Clinical Immunology. 2017;27(5):299-304.
BibTex	@article{ author = {Fazlollahi MR and Pourpak Z and Hamidieh AA and Movahedi M and Houshmand M and Badalzadeh M and Nourizadeh M and Mahloujirad M and Arshi S and Nabavi M and Gharagozlou M and Khayatzadeh A and Dabbaghzade A and Atarod L and Zandieh F and Sadeghi Shabestary M and Mesdaghi M and Mohammadzadeh I and Mahdaviani SA and Eslamian MH and Pesaran F and Bahraminia E and Abolnezhadian F and Arij Z and Moin M}, title = {Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience}, journal = {Journal of Investigational Allergology and Clinical Immunology}, volume = {27}, number = {5}, pages = {299-304}, year = {2017} }
RIS	TY - JOUR AU - Fazlollahi MR AU - Pourpak Z AU - Hamidieh AA AU - Movahedi M AU - Houshmand M AU - Badalzadeh M AU - Nourizadeh M AU - Mahloujirad M AU - Arshi S AU - Nabavi M AU - Gharagozlou M AU - Khayatzadeh A AU - Dabbaghzade A AU - Atarod L AU - Zandieh F AU - Sadeghi Shabestary M AU - Mesdaghi M AU - Mohammadzadeh I AU - Mahdaviani SA AU - Eslamian MH AU - Pesaran F AU - Bahraminia E AU - Abolnezhadian F AU - Arij Z AU - Moin M TI - Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience JO - Journal of Investigational Allergology and Clinical Immunology VL - 27 IS - 5 SP - 299 EP - 304 PY - 2017 ER -

Style

Citing Format

MLA

Fazlollahi MR, et al.. "Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience." Journal of Investigational Allergology and Clinical Immunology, vol. 27, no. 5, 2017, pp. 299-304.

APA

Fazlollahi MR, Pourpak Z, Hamidieh AA, Movahedi M, Houshmand M, Badalzadeh M, Nourizadeh M, Mahloujirad M, Arshi S, Nabavi M, Gharagozlou M, Khayatzadeh A, Dabbaghzade A, Atarod L, Zandieh F, Sadeghi Shabestary M, Mesdaghi M, Mohammadzadeh I, Mahdaviani SA, ... Moin M (2017). Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience. Journal of Investigational Allergology and Clinical Immunology, 27(5), 299-304.

Chicago

Fazlollahi MR, Pourpak Z, Hamidieh AA, Movahedi M, Houshmand M, Badalzadeh M, Nourizadeh M, et al.. "Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience." Journal of Investigational Allergology and Clinical Immunology 27, no. 5 (2017): 299-304.

Harvard

Fazlollahi MR et al. (2017) 'Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience', Journal of Investigational Allergology and Clinical Immunology, 27(5), pp. 299-304.

Vancouver

Fazlollahi MR, Pourpak Z, Hamidieh AA, Movahedi M, Houshmand M, Badalzadeh M, et al.. Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience. Journal of Investigational Allergology and Clinical Immunology. 2017;27(5):299-304.

BibTex

@article{ author = {Fazlollahi MR and Pourpak Z and Hamidieh AA and Movahedi M and Houshmand M and Badalzadeh M and Nourizadeh M and Mahloujirad M and Arshi S and Nabavi M and Gharagozlou M and Khayatzadeh A and Dabbaghzade A and Atarod L and Zandieh F and Sadeghi Shabestary M and Mesdaghi M and Mohammadzadeh I and Mahdaviani SA and Eslamian MH and Pesaran F and Bahraminia E and Abolnezhadian F and Arij Z and Moin M}, title = {Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience}, journal = {Journal of Investigational Allergology and Clinical Immunology}, volume = {27}, number = {5}, pages = {299-304}, year = {2017} }

RIS

TY - JOUR
AU - Fazlollahi MR
AU - Pourpak Z
AU - Hamidieh AA
AU - Movahedi M
AU - Houshmand M
AU - Badalzadeh M
AU - Nourizadeh M
AU - Mahloujirad M
AU - Arshi S
AU - Nabavi M
AU - Gharagozlou M
AU - Khayatzadeh A
AU - Dabbaghzade A
AU - Atarod L
AU - Zandieh F
AU - Sadeghi Shabestary M
AU - Mesdaghi M
AU - Mohammadzadeh I
AU - Mahdaviani SA
AU - Eslamian MH
AU - Pesaran F
AU - Bahraminia E
AU - Abolnezhadian F
AU - Arij Z
AU - Moin M
TI - Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade’S Experience
JO - Journal of Investigational Allergology and Clinical Immunology
VL - 27
IS - 5
SP - 299
EP - 304
PY - 2017
ER -

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Abstract