Long-term follow-up of ninety eight Iranian patients with primary immune deficiency in a single tertiary centre
Allergol. immunopatol
; 44(4): 322-330, jul.-ago. 2016. graf, tab
Article
in English
| IBECS
| ID: ibc-154434
Responsible library:
ES1.1
Localization: BNCS
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 patientsRESUMEN
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Collection:
National databases
/
Spain
Database:
IBECS
Main subject:
Monitoring, Immunologic
/
Desensitization, Immunologic
/
Immunologic Deficiency Syndromes
/
Immunologic Surveillance
Type of study:
Etiology study
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Incidence study
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Observational study
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Risk factors
Limits:
Adolescent
/
Adult
/
Child
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Female
/
Humans
/
Male
Language:
English
Journal:
Allergol. immunopatol
Year:
2016
Document type:
Article
Institution/Affiliation country:
Ali-Asghar Hospital/Iran
/
Boston Children´s Hopsital/USA
/
Iran University of Medical Sciences (IUMS)/Iran
/
Iran University of Medical Sciences (IUMS)/IrancTehran
/
Mofid Children's Hospital/Iran
/
Rasool-e-Akram Hospital/Iran
/
Shaheed Beheshti University of Medical Sciences (SBUMS)/Iran
/
Shiraz University of Medical Sciences/Iran
/
Tehran University of Medical Sciences (TUMS)/Iran