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
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Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Immunologic Deficiency Syndromes/diagnosis , Immunologic Deficiency Syndromes/immunology , Immunologic Deficiency Syndromes/pathology , Immunologic Surveillance , Immunologic Surveillance/immunology , Monitoring, Immunologic/instrumentation , Monitoring, Immunologic/methods , Desensitization, Immunologic , Laboratory Test/methods , Immunoglobulin G/analysis , Immunoglobulin G/immunology , Cohort Studies , Immunocompetence/immunologyABSTRACT
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.
Subject(s)
Immunoglobulins, Intravenous/therapeutic use , Immunologic Deficiency Syndromes , Immunologic Factors/therapeutic use , Respiratory Tract Infections/epidemiology , Adolescent , Adult , Age of Onset , Child , Child, Preschool , Consanguinity , Delayed Diagnosis , Female , Follow-Up Studies , Humans , Immunoglobulins, Intravenous/administration & dosage , Immunologic Deficiency Syndromes/diagnosis , Immunologic Deficiency Syndromes/epidemiology , Immunologic Deficiency Syndromes/immunology , Immunologic Factors/administration & dosage , Iran/epidemiology , Male , Respiratory Tract Infections/drug therapy , Respiratory Tract Infections/etiology , Respiratory Tract Infections/prevention & control , Retrospective Studies , Tertiary Care Centers/statistics & numerical data , Treatment Outcome , Young AdultABSTRACT
BACKGROUND: Risk factors for colonization with vancomycin-resistant enterococci (VRE) vary by population and locale. The objective of this study was to determine the prevalence of and risk factors for VRE colonization in children with acute lymphoblastic leukemia (ALL) in Tehran. METHODS: Stools were collected from children with ALL at the Ali Asghar Children's Hospital and the Mahak Pediatric Oncology Center between March 2007 and October 2008. Demographic features and potential risk factors for VRE colonization, including duration of ALL, presence of severe neutropenia in the preceding month, receipt of antibiotics in the preceding 3 months, concurrent medical problems, days of hospitalization, and the need for intensive care since the time of diagnosis of ALL, were recorded. RESULTS: VRE was identified from stools in 33 of 130 children with ALL (25%). No clear risk factors were identified for VRE colonization in the current study, but there was a trend towards an increased prevalence in children admitted to the intensive care unit since their ALL diagnosis (p=0.07). The VanA genotype was found in 28 of the 33 stools (85%), with all other enterococci being VanB. CONCLUSIONS: The prevalence of VRE colonization in children with ALL in Tehran is high. Modifiable risk factors have not been identified. The implementation of routine surveillance for colonization and an increased emphasis on adherence to standard infection control precautions may prevent spread.
Subject(s)
Enterococcus/isolation & purification , Gram-Positive Bacterial Infections/epidemiology , Precursor Cell Lymphoblastic Leukemia-Lymphoma/complications , Vancomycin Resistance , Adolescent , Bacterial Proteins/genetics , Carbon-Oxygen Ligases/genetics , Child , Child, Preschool , Cross Infection/epidemiology , Cross Infection/microbiology , DNA, Bacterial/isolation & purification , Enterococcus/drug effects , Enterococcus/genetics , Feces/microbiology , Female , Gram-Positive Bacterial Infections/diagnosis , Gram-Positive Bacterial Infections/microbiology , Humans , Infant , Infection Control/standards , Intensive Care Units , Iran/epidemiology , Male , Microbial Sensitivity Tests , Polymerase Chain Reaction , Prevalence , Risk FactorsABSTRACT
Brucellosis is being reported with increasing frequency in the Islamic Republic of Iran. Serum antibodies in high-risk and general populations help to define cut-off levels and can be used as a simple and rapid diagnostic tests in infected areas. We performed the rose Bengal test (RBT), serum agglutination test (SAT) and 2-mercaptoethanol (2ME) titre determination on 415 healthy individuals including butchers, slaughterers and others. Positive results were found by RBT, SAT titre (1:80) and 2ME titre > or = 1:20 in slaughterers (10%, 20% and 6% respectively), butchers (6%, 4% and 1% respectively) and the general population (1%, 2% and < 1% respectively). A single SAT titre > or = 1:80 in the presence of 2ME titre > or = 1:20 can be diagnostic in this region.
Subject(s)
Abattoirs , Antibodies, Bacterial/blood , Brucella/immunology , Brucellosis/epidemiology , Occupational Diseases/epidemiology , Abattoirs/statistics & numerical data , Adult , Aged , Agglutination Tests , Animals , Brucellosis/blood , Brucellosis/immunology , Case-Control Studies , Cross-Sectional Studies , Female , Food Microbiology , Humans , Iran/epidemiology , Male , Meat/microbiology , Mercaptoethanol , Middle Aged , Milk/microbiology , Occupational Diseases/blood , Occupational Diseases/immunology , Population Surveillance , Risk Factors , Rose Bengal , Sensitivity and Specificity , Seroepidemiologic Studies , Surveys and QuestionnairesABSTRACT
Brucellosis is being reported with increasing frequency in the Islamic Republic of Iran. Serum antibodies in high-risk and general populations help to define cut-off levels and can be used as a simple and rapid diagnostic tests in infected areas. We performed the rose Bengal test [RBT], serum agglutination test [SAT] and 2-mercaptoethanol [2ME] titre determination on 415 healthy individuals including butchers, slaughterers and others. Positive results were found by RBT, SAT titre [1:80] and 2ME titre >/= 1:20 in slaughterers [10%, 20% and 6% respectively], butchers [6%, 4% and 1% respectively] and the general population [1%, 2% and < 1% respectively]. A single SAT titre >/= 1:80 in the presence of 2ME titre >/= 1:20 can be diagnostic in this region
