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1.
Artigo em Inglês | MEDLINE | ID: mdl-37103527

RESUMO

BACKGROUND AND OBJECTIVE: Common variable immunodeficiency (CVID) is considered the most symptomatic type of inborn errors of immunity in humans. Along with infectious complications, which have numerous consequences, non-infectious complications are also a major challenge among CVID patients. METHODS: All registered CVID patients in the national database were included in this retrospective cohort study. Patients were divided into two groups based on the presence of B-cell lymphopenia. Demographic characteristics, laboratory findings, non-infectious organ involvements, autoimmunity, and lymphoproliferative diseases were evaluated. RESULTS: Among 387 enrolled patients, 66.4% were diagnosed with non-infectious complications; however, 33.6% had only infectious presentations. Enteropathy, autoimmunity, and lymphoproliferative disorders were reported in 35.1%, 24.3%, and 21.4% of patients, respectively. Some complications, including autoimmunity and hepatosplenomegaly, were reported to be significantly higher among patients with B-cell lymphopenia. Among organ involvement, dermatologic, endocrine and musculoskeletal systems were predominantly affected in CVID patients with B-cell lymphopenia. Among autoimmune manifestations, the frequency of rheumatologic, hematologic, and gastrointestinal autoimmunity was reported to be higher compared to other types of autoimmunity independent from the B cell-lymphopenia. Furthermore, hematological cancers, particularly lymphoma, were slightly introduced as the most common type of malignancy. Meanwhile, the mortality rate was 24.5%, and respiratory failure and malignancies were reported as the most common cause of death in our patients without significant differences between the two groups. CONCLUSION: Considering that some of the non-infectious complications might be associated with B-cell lymphopenia, therefore, regular patient monitoring and follow-up along with proper medications (besides immunoglobulins replacement therapy) are highly recommended to prevent further sequels and increase the patients' quality of life.

2.
Eur Ann Allergy Clin Immunol ; 55(4): 174-179, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-35620981

RESUMO

Summary: Background. Immunotherapy may induce sustained unresponsiveness (SU )in which the patient can tolerate the allergen without any severe symptoms after discontinuing immunotherapy. The present study evaluated serum and cutaneous markers for predicting SU in patients with wheat anaphylaxiswho underwent oral immunotherapy. We investigated the effectiveness of a flexible regimen of 5 to 10 g wheat protein (WP) in the maintenance phase of oral immunotherapy (OIT). Methods.This study was conducted on 19 patients with wheat anaphylaxis who underwent OIT. The results of the skin prick test (SPT), besides specific serum IgE (sIgE) and IgG4 (sIgG4) to WP, were evaluated before the desensitization. The maintenance dose started from the preferred dose of 5 to 10 g WP after the build-up phase, if the patient could tolerate it. All patients were recruited 7 to 9 months after undergoing this flexible regimen, and the results of SPT and sIgE, and sIgG4l evels were obtained once more. The patients underwent oral food challenge (OFC) after a 3-4-week avoidance to evaluate SU. Results. There was anassociation between mean IgE reduction and SU (p < 0.0006), while no association was observed between the mean increase in specific IgG4 (p = 0.1), and the mean wheal diameter decrease (p = 0.29). A 50% reductionin sIgE was associated with SU. Thirteen patients were considered to havea SU. There was no association between the flexible regimen and the desensitization rate. Conclusions.The reduction of 50% sIgE is a predictive factor for SU in patients with IgE-mediated wheat allergy.


Assuntos
Dessensibilização Imunológica , Imunoglobulina E , Humanos , Dessensibilização Imunológica/métodos , Administração Oral , Alérgenos , Imunoglobulina G
3.
Eur Ann Allergy Clin Immunol ; 55(2): 78-85, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-35156357

RESUMO

Summary: Background. Anaphylaxis is a life-threatening hypersensitivity reaction. The present study aimed to investigate the cases of anaphylaxis to ant stings in Iran to determine the characteristics of patients, geographical distribution and the type of ants that cause anaphylaxis. Methods. Patients with a history of anaphylaxis to ant sting underwent skin allergy test with extracted substance from Solenopsis invicta. Samples of ants were collected from the sites where each patient was bitten and their species were identified by a medical entomologist. Results. Nineteen patients (mean age: 26.2 years; range: 4-48 years) were included in the study. Most patients (89.5 percent) were female. The lower limb was the most common site of the sting and most stings had occurred in the morning (31.6 %) and evening (31.6%). Skin manifestations were the most common clinical symptoms (94.7%). Most cases of stings were observed in the Hormozgan province (89.5%) located in southern Iran. Sixteen patients had positive skin prick test for ant venom. All collected ants that caused anaphylaxis belonged to the Pachycondyla sennaarensis species. Conclusions. Ant sting anaphylaxis is not uncommon in Iran, especially in its southern regions. All cases of anaphylaxis in this study were due to samsum ant sting (Pachycondyla sennaarensis), which is a species similar to the fire ant (Solenopsis invicta). Allergy skin testing with fire ant extract was positive and helpful in identifying samsum ant allergy in all cases.


Assuntos
Anafilaxia , Venenos de Formiga , Formigas , Mordeduras e Picadas de Insetos , Animais , Humanos , Feminino , Adulto , Masculino , Anafilaxia/diagnóstico , Anafilaxia/epidemiologia , Anafilaxia/etiologia , Mordeduras e Picadas de Insetos/complicações , Alérgenos
4.
Eur Ann Allergy Clin Immunol ; 53(1): 18-22, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-32186350

RESUMO

Summary: Introduction and objective. Gastrointestinal complications are considered as one of the most common manifestations in patients with Common Variable Immunodeficiency (CVID). These complications can result from Small Intestinal Bacterial Overgrowth (SIBO). Hydrogen breath test is extensively used to diagnose SIBO. The objective of this study was to evaluate the prevalence of SIBO using the Hydrogen Breath Test (HBT) in patients with CVID. Materials and methods. Twenty-seven patients with CVID entered this cross-sectional study. Demographic and lower gastrointestinal symptoms were recorded in a check list. Hemoglobin level was measured in all patients. The concentration of IgA and IgG was assessed using nephelometry. Moreover, SIBO was detected by means of Glucose hydrogen breath test. Results. The mean (plus-minus SD) age of the patients was 35.25 (plus-minus 11.69) years. Twenty patients (74.1%) manifested at least one lower gastrointestinal symptom. The most frequent lower gastrointestinal manifestations were bloating (66.7%) and chronic diarrhea (40.7%), respectively. IgA level less than 10 mg/dl and IgG level less than 600 mg/dl were determined in 77.8% and 25.9% of patients, respectively. Positive HBT was detected in 40.7% (n = 11) of the patients. In the positive HBT group, bloating, chronic diarrhea and abdominal pain were the most common lower GI manifestations. There was no significant difference in terms of age, BMI, IgA level, and duration of CVID between the positive and negative HBT groups. The significant association of co-occurrence of anemia and abdominal pain with positive HBT (positive predictive value: 100%) might be considered as a clue to SIBO diagnosis. Conclusions. Regarding the high prevalence and non-specific manifestation of SIBO, it is suggested to consider concurrent symptoms in patients with CVID to manage the timely and precise diagnosis of SIBO.


Assuntos
Dor Abdominal/diagnóstico , Imunodeficiência de Variável Comum , Intestino Delgado/microbiologia , Dor Abdominal/epidemiologia , Testes Respiratórios/métodos , Criança , Imunodeficiência de Variável Comum/diagnóstico , Imunodeficiência de Variável Comum/epidemiologia , Estudos Transversais , Diarreia , Humanos , Hidrogênio/metabolismo , Imunoglobulina A/análise , Imunoglobulina A/química , Imunoglobulina G/análise , Imunoglobulina G/química
6.
Eur Ann Allergy Clin Immunol ; 53(2): 86-90, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-32496031

RESUMO

Summary: Eosinophilic esophagitis (EoE) is a chronic allergen/immune-mediated disease leading to esophageal dysfunction. Food allergens play critical roles in the pathogenesis and treatment of EoE via different mechanisms. This study aimed to present the characteristics and evaluate the ability of skin prick test (SPT), skin prick to prick test (SPP) (IgE-mediated), and atopic patch test (APT) (cell-mediated) individually or simultaneously to diagnose food allergy in patients suffering from EoE. This prospective study was conducted on 58 patients with EoE. Seven patients (12.1%) were positive to only one, 3 (5.2%) were simultaneously positive to two, and 32 (55.2%) were simultaneously positive to three tests. Single and double sensitizations were totally 10.4% in IgE-mediated reactions, while 36.5% in cell-mediated reactions. In contrast, poly sensitization (> 2 allergens) was 51.7% in IgE-mediated tests and 20.7% in the cell-mediated test. Multiple sensitization findings showed egg white, milk, yolk, and soy were the most frequent allergens. Our findings indicate that EoE is early onset and associated with multiple food sensitizations, particularly via IgE-mediated mechanisms. These immune-mediated responses encompass both IgE-mediated (SPT and SPP) and cell-mediated (APT) reactions simultaneously not individually. Therefore, employing multiple assays may strengthen the diagnosis of food sensitization.


Assuntos
Hipersensibilidade Alimentar/diagnóstico , Hipersensibilidade Imediata , Imunoglobulina E/sangue , Testes Cutâneos/métodos , Adolescente , Adulto , Alérgenos , Criança , Esofagite Eosinofílica/sangue , Esofagite Eosinofílica/diagnóstico , Feminino , Humanos , Imunidade Celular , Masculino , Estudos Prospectivos , Adulto Jovem
9.
Allergol. immunopatol ; 45(4): 333-338, jul.-ago. 2017. tab
Artigo em Inglês | IBECS | ID: ibc-165092

RESUMO

Background: A clear picture of interaction of Th1/Th2 cytokines in pathogenesis of chronic spontaneous urticaria (CSU), remains elusive. Impaired IFN-γ production and decreased levels of IL-2 have been reported. The aim of this study was to evaluate the association of Th1 cytokines; IL-2, IL-12 and IFN-γ polymorphisms with CSU. Methods: 90 patients with CSU and 140 age-sex matched subjects were included in this study. DNA samples were evaluated through PCR-SSP assay in order to detect single nucleotide polymorphisms of IL-12 (A/C -1188) or (rs3212227), IFN-γ (A/T UTR5644) or (rs2069717) and IL-2 (G/T -330 and G/T +166) or (rs2069762 and rs2069763). Results: G allele at -330 at promoter region of IL-2 gene was overrepresented in CSU. Heterozygotes (GT) at this locus and heterozygotes at +166 of IL-2 gene (GT) were more prevalent in CSU group. Additionally, the haplotype GT for loci -330 and +166 of IL-2 gene was powerfully associated with CSU (OR (95%CI) = 57.29 (8.43-112.7)). Conclusions: SNP at position -330 and +166 of IL-2 gene are differently expressed in CSU. The haplotype GT of IL-2 at -330 and +166 might confer vulnerability to a number of immunological disorders in Iranian region (AU)


No disponible


Assuntos
Humanos , Urticária/imunologia , Polimorfismo de Nucleotídeo Único/imunologia , Interleucina-2/análise , Interleucina-12/análise , Interferon gama/análise , Doença Crônica , Suscetibilidade a Doenças
10.
J Investig Allergol Clin Immunol ; 27(5): 299-304, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28266921

RESUMO

BACKGROUND: 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. CONCLUSIONS: 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.


Assuntos
Imunodeficiência Combinada Severa/diagnóstico , Biomarcadores , Suscetibilidade a Doenças , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Técnicas de Diagnóstico Molecular , Mutação , Fenótipo , Imunodeficiência Combinada Severa/complicações , Imunodeficiência Combinada Severa/etiologia , Imunodeficiência Combinada Severa/terapia , Avaliação de Sintomas
11.
Allergol Immunopathol (Madr) ; 45(4): 333-338, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28159384

RESUMO

BACKGROUND: A clear picture of interaction of Th1/Th2 cytokines in pathogenesis of chronic spontaneous urticaria (CSU), remains elusive. Impaired IFN-γ production and decreased levels of IL-2 have been reported. The aim of this study was to evaluate the association of Th1 cytokines; IL-2, IL-12 and IFN-γ polymorphisms with CSU. METHODS: 90 patients with CSU and 140 age-sex matched subjects were included in this study. DNA samples were evaluated through PCR-SSP assay in order to detect single nucleotide polymorphisms of IL-12 (A/C -1188) or (rs3212227), IFN-γ (A/T UTR5644) or (rs2069717) and IL-2 (G/T -330 and G/T +166) or (rs2069762 and rs2069763). RESULTS: G allele at -330 at promoter region of IL-2 gene was overrepresented in CSU. Heterozygotes (GT) at this locus and heterozygotes at +166 of IL-2 gene (GT) were more prevalent in CSU group. Additionally, the haplotype GT for loci -330 and +166 of IL-2 gene was powerfully associated with CSU (OR (95%CI)=57.29 (8.43-112.7)). CONCLUSIONS: SNP at position -330 and +166 of IL-2 gene are differently expressed in CSU. The haplotype GT of IL-2 at -330 and +166 might confer vulnerability to a number of immunological disorders in Iranian region.


Assuntos
Interferon gama/genética , Interleucina-12/genética , Interleucina-2/genética , Urticária/genética , Estudos de Casos e Controles , Doença Crônica , Feminino , Frequência do Gene , Estudos de Associação Genética , Predisposição Genética para Doença , Genótipo , Humanos , Irã (Geográfico) , Masculino , Polimorfismo de Nucleotídeo Único
12.
J. investig. allergol. clin. immunol ; 27(5): 299-304, 2017. tab
Artigo em Inglês | IBECS | ID: ibc-167248

RESUMO

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, onethird 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 (AU)


Introducción: La inmunodeficiencia combinada severa (SCID) es una grave enfermedad pediátrica que puede comprometer la vida del paciente. El artículo recoge la evaluación clínica e inmunológica, el análisis molecular y la supervivencia de los pacientes con SCID atendidos en un hospital de referencia de Irán. Métodos: Desde enero de 2006 a diciembre de 2015, se realizó un estudio prospectivo en los pacientes con SCID en el que se realizó un screening inicial junto a diferentes análisis inmunológicos. Se realizó un análisis genético para confirmar el diagnóstico. Resultados: Sesenta y tres pacientes fueron diagnosticados de SCID, cuarenta y tres (63,8%) de los mismos eran varones. La mediana de la edad de inicio de la enfermedad, diagnóstico y retraso en su diagnóstico, fueron de 40, 110 y 60 días respectivamente. Cuarenta y nueve pacientes (77,8%) recibieron vacunación con BCG y un tercio de los mismos presentó complicaciones como consecuencia de la misma. Las manifestaciones clínicas más frecuentes de estos pacientes fueron: neumonía, candidiasis oral recidivante, diarrea crónica y retraso en el crecimiento. Ocho de los treces pacientes que recibieron trasplante de progenitores hematopoyéticos, lograron sobrevivir. Los restantes pacientes fallecieron antes de poder recibir dicho trasplante. El 34,9% de los pacientes tuvieron T-B-NK+ SCID y la mayoría de los pacientes eran portadores de mutaciones en los genes RAG2 o RAG1. Conclusión: La variante autosómica recesiva de la SCID es la forma más común en los pacientes iraníes. Se debe considerar prioritario proporcionar una formación adecuada a los médicos y las familias para reducir el retraso en el diagnóstico. Es igualmente importante concienciar para evitar la vacunación con gérmenes vivos y expandir los registros de donantes de células madre para agilizar el trasplante de estos pacientes (AU)


Assuntos
Humanos , Recém-Nascido , Imunodeficiência Combinada Severa/complicações , Imunodeficiência Combinada Severa/diagnóstico , Transplante de Células-Tronco/métodos , Triagem Neonatal/métodos , Estudos Prospectivos , Estudos de Coortes , Inquéritos e Questionários , Citometria de Fluxo/métodos , 28599
13.
East Mediterr Health J ; 22(7): 432-439, 2016 Oct 02.
Artigo em Inglês | MEDLINE | ID: mdl-27714736

RESUMO

There are few estimates of influenza burden in the WHO Region for the Eastern Mediterranean. In this study we estimated the burden of severe acute respiratory infection (SARI) and influenza-associated SARI (F-SARI) in selected provinces of Islamic Republic of Iran, the trends of SARI and confirmed cases of influenza (F-SARI) over 12 months (seasonality), and the age groups most at risk. Using the electronic Iranian influenza surveillance system and data of cases in sentinel hospitals of 3 selected provinces, we estimated the monthly trend (seasonality) of incidence for SARI and F-SARI, overall incidence of SARI and F-SARI and their disaggregation by age with the aid using the Monte Carlo technique. The age groups most at-risk were children aged under 2 years and adults older than 50 years.


Assuntos
Influenza Humana , Infecções Respiratórias , Vigilância de Evento Sentinela , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Efeitos Psicossociais da Doença , Feminino , Humanos , Incidência , Lactente , Influenza Humana/epidemiologia , Irã (Geográfico)/epidemiologia , Masculino , Pessoa de Meia-Idade , Infecções Respiratórias/epidemiologia , Índice de Gravidade de Doença , Adulto Jovem
14.
Allergol. immunopatol ; 44(4): 322-330, jul.-ago. 2016. graf, tab
Artigo em Inglês | IBECS | ID: ibc-154434

RESUMO

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


No disponible


Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Adulto , Síndromes de Imunodeficiência/diagnóstico , Síndromes de Imunodeficiência/imunologia , Síndromes de Imunodeficiência/patologia , Vigilância Imunológica , Vigilância Imunológica/imunologia , Monitorização Imunológica/instrumentação , Monitorização Imunológica/métodos , Dessensibilização Imunológica , Testes Laboratoriais/métodos , Imunoglobulina G/análise , Imunoglobulina G/imunologia , Estudos de Coortes , Imunocompetência/imunologia
15.
East. Mediterr. health j ; 22(7): 432-439, 2016-07.
Artigo em Inglês | WHO IRIS | ID: who-260093

RESUMO

There are few estimates of influenza burden in the WHO Region for the Eastern Mediterranean. In this study we estimated the burden of severe acute respiratory infection [SARI] and influenza-associated SARI [F-SARI] in selected provinces of Islamic Republic of Iran, the trends of SARI and confirmed cases of influenza [F-SARI] over 12 months [seasonality], and the age groups most at risk. Using the electronic Iranian influenza surveillance system and data of cases in sentinel hospitals of 3 selected provinces, we estimated the monthly trend [seasonality] of incidence for SARI and F-SARI, overall incidence of SARI and F-SARI and their disaggregation by age with the aid using the Monte Carlo technique. The age groups most at-risk were children aged under 2 years and adults older than 50 years


Peu d'estimations sont disponibles sur la charge de la grippe dans la Région OMS de la Méditerranée orientale. Dans la présente étude, nous avons estimé la charge de morbidité due aux infections respiratoires aiguës sévères [IRAS] et aux IRAS associés à la grippe dans certaines provinces de la République islamique d'Iran, ainsi que les tendances des IRAS et des cas de grippe confirmés sur 12 mois [saisonnalité], et les groupes d'âge les plus exposés. En utilisant le système électronique iranien de surveillance de la grippe et les données relatives aux cas des hôpitaux sentinelles de trois provinces sélectionnées, nous avons estimé la tendance mensuelle [saisonnalité] de l'incidence des IRAS et des IRAS dus à la grippe, l'incidence globale des IRAS et des IRAS dues à la grippe et leur ventilation par âge grâce à la méthode de Monte Carlo. Les groupes d'âge les plus exposés au risque étaient les enfants de moins de 2 ans et les adultes de plus de 50 ans


Assuntos
Doenças Transmissíveis , Influenza Humana , Infecções Respiratórias , Insuficiência Respiratória , Criança , Grupos Etários
16.
Allergol Immunopathol (Madr) ; 44(4): 322-30, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26803694

RESUMO

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.


Assuntos
Imunoglobulinas Intravenosas/uso terapêutico , Síndromes de Imunodeficiência , Fatores Imunológicos/uso terapêutico , Infecções Respiratórias/epidemiologia , Adolescente , Adulto , Idade de Início , Criança , Pré-Escolar , Consanguinidade , Diagnóstico Tardio , Feminino , Seguimentos , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Síndromes de Imunodeficiência/diagnóstico , Síndromes de Imunodeficiência/epidemiologia , Síndromes de Imunodeficiência/imunologia , Fatores Imunológicos/administração & dosagem , Irã (Geográfico)/epidemiologia , Masculino , Infecções Respiratórias/tratamento farmacológico , Infecções Respiratórias/etiologia , Infecções Respiratórias/prevenção & controle , Estudos Retrospectivos , Centros de Atenção Terciária/estatística & dados numéricos , Resultado do Tratamento , Adulto Jovem
18.
East Mediterr Health J ; 21(3): 194-8, 2015 May 19.
Artigo em Inglês | MEDLINE | ID: mdl-26074219

RESUMO

No data on the prevalence of asthma in Afghanistan have been published before. In a school-based survey in 2010-2011 the wheezing section of the International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire was completed by a random sample of 1500 children aged 6-7 years and 1500 adolescents aged 13-14 years old. The prevalence of physician-diagnosed asthma was 12.5% in 6-7-year-olds and 17.3% in 13-14-year-olds (P = 0.002). The prevalence of wheeze in the last 12 months was similar in children and adolescents (19.2% and 21.7% respectively). The prevalence of ever wheezing, night attacks, speech-limiting wheeze and exercise-induced wheeze was 23.1%, 4.8%, 12.2% and 9.6% respectively in children and 30.5%, 4.4%, 13.0% and 13.6% respectively in adolescents. These rates are higher than those in neighbouring countries. This first epidemiological survey of asthma in Afghanistan shows that asthma and wheezing are common in Kabul students.


Assuntos
Asma/epidemiologia , Adolescente , Afeganistão/epidemiologia , Asma/diagnóstico , Criança , Feminino , Humanos , Masculino , Prevalência , Inquéritos e Questionários
19.
J Investig Allergol Clin Immunol ; 25(6): 416-25, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26817138

RESUMO

BACKGROUND: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders (PID), with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease. OBJECTIVES: The current study was performed to evaluate and compare demographic and clinical data in the most common types of PAD. MATERIALS AND METHODS: We performed a retrospective review of the medical records of all PAD patients with a confirmed diagnosis of common variable immunodeficiency (CVID), hyper IgM syndrome (HIgM), selective IgA deficiency (SIgAD), and X-linked agammaglobulinemia (XLA) who were diagnosed during the last 30 years at the Children's Medical Center, Tehran, Iran. RESULTS: A total number of 280 cases of PAD (125 CVID, 32 HIgM, 63 SIgAD, and 60 XLA) were enrolled in the study. The median (range) age at the onset of disease in CVID, HIgM, SIgAD, and XLA was 2 (0-46), 0.91 (0-9), 1 (0-26), and 1 (0-10) years, respectively. Gastrointestinal infections were more prevalent in CVID patients, as were central nervous system infections in XLA patients. Autoimmune complications were more prevalent in HIgM patients, malignancies in CVID patients, and allergies in SIgAD patients. The mortality rate for CVID, HIgM, and XLA was 27.2%, 28.1%, and 25%, respectively. No deaths were reported in SIgAD patients. CONCLUSIONS: SIgAD patients had the best prognosis. While all PAD patients should be monitored for infectious complications, special attention should be paid to the finding of malignancy and autoimmune disorders in CVID and HIgM patients, respectively.


Assuntos
Síndromes de Imunodeficiência/complicações , Adolescente , Adulto , Criança , Pré-Escolar , Imunodeficiência de Variável Comum/complicações , Feminino , Humanos , Síndromes de Imunodeficiência/mortalidade , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Centros de Atenção Terciária
20.
East. Mediterr. health j ; 21(3): 194-198, 2015.
Artigo em Inglês | WHO IRIS | ID: who-255087

RESUMO

No data on the prevalence of asthma in Afghanistan have been published before. In a school-based survey in 2010–2011 the wheezing section of the International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire was completed by a random sample of 1500 children aged 6–7 years and 1500 adolescents aged 13–14 years old. The prevalence of physician-diagnosed asthma was 12.5% in 6–7-year-olds and 17.3%in 13–14-year-olds (P = 0.002). The prevalence of wheeze in the last 12 months was similar in children and adolescents (19.2% and 21.7% respectively). The prevalence of ever wheezing, night attacks, speech-limiting wheeze and exercise-induced wheeze was 23.1%, 4.8%, 12.2% and 9.6% respectively in children and 30.5%, 4.4%, 13.0% and 13.6% respectively in adolescents. These rates are higher than those in neighbouring countries. This first epidemiological survey of asthma in Afghanistan shows that asthma and wheezing are common in Kabul students.


Aucune donnée sur la prévalence de l’asthme en Afghanistan n’a encore été publiée. Dans une enquête en milieu scolaire de 2010 à 2011, la partie sur les sibilances du questionnaire International Study of Asthma and Allergies in Childhood (ISAAC) a été remplie par un échantillon aléatoire de 1500 enfants âgés de 6 à 7 ans etde 1500 adolescents âgés de 13 à 14 ans. La prévalence du diagnostic d’asthme posé par un médecin était de 12,5 % chez les enfants de 6 à 7 ans et de 17,3 % chez les adolescents de 13 à 14 ans (P = 0,002). La prévalence des sibilances au cours de 12 mois précédents était similaire chez les enfants et chez les adolescents (19,2 % et 21,7 %, respectivement). La prévalence des sibilances, des crises nocturnes, des sibilances limitant la parole et d’une respiration sifflante induite par l’exercice était de 23,1 %, 4,8 %, 12,2 % et 9,6 %, respectivement chez les enfants et de 30,5 %, 4,4 %, 13,0 % et 13,6 % respectivement chez les adolescents. Ces pourcentages sont supérieurs à ceuxdes pays voisins. La première enquête épidémiologique sur l’asthme en Afghanistan révèle que l’asthme et les sibilances sont fréquents chez les élèves de Kaboul.


Assuntos
Asma , Prevalência , Inquéritos e Questionários , Criança , Adolescente
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