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کاربرد نوع شرط:
- جایگاه : پژوهشی
- مجله: Immunology and Genetics Journal
- نوع مقاله: Journal Article
- کلمات کلیدی: Primary immunodeficiency,Hyper IgM,Sporadic HIGM,Familial HIGM
- چکیده:
- چکیده انگلیسی: Background/Objectives: Hyper IgM (HIGM) syndrome or immunoglobulin class-switch recombination deficiency (Ig-CSR) is a group of primary immunodeficiencies (PIDs) where B cells are unable to undergo the process of immunoglobulin class -switching recombination (CSR), a process in which B-cells modify their DNA to switch from production of IgM to other immunoglobulins. Hence, the affected patients exhibit normal to high levels of serum IgM and low or absence of other immunoglobulin isotypes relative to mean values of age. Therefore, the present study was conducted to assess the demographic data, clinical manifestation, and immunological findings in the sporadic and familial types of HIGM.
Methodology: Demographic data, laboratory findings, and clinical presentations of 79 Iranian patients diagnosed with HIgM syndrome were collected. All the patients were classified into two different groups: sporadic and familial types of HIGM.
Results: Male to female ratio was significantly higher in the familial group compared to the sporadic group so that, 94.7% of the patients were male in the familial group, while only 70% of the sporadic patients were male (P=0.032). It was also found that the familial group had a significantly higher consanguinity rate (P=0.047) and a significantly lower delay of diagnosis compared to the sporadic group (P=0.006). The lower respiratory infection (42%) followed by upper respiratory infection (26%) and diarrhea (15%) were the most frequent initial presentations. It was shown that diarrhea, as an initial presentation was about three times more common among the familial group (31.6%) compared to the sporadic group (10%, P=0.028). Otitis was also found to be more prevalent in the sporadic group (P=0.042).
Conclusion: Our findings could be explained by more careful screenings and more vigilant and informative parents in the families with another affected member.- انتشار مقاله: 23-04-1399
- نویسندگان: Salar Pashangzadeh,Kasra Mehdizadeh
- مشاهده
- جایگاه : پژوهشی
- مجله: Immunology and Genetics Journal
- نوع مقاله: Journal Article
- کلمات کلیدی: Hyper IgM (HIgM),hematologic problems,Primary immunodeficiency disorders (PIDs)
- چکیده:
- چکیده انگلیسی: Introduction: Hyper IgM (HIGM) syndrome is a rare kind of primary Immunodeficiency disease (PID) characterized by normal to the increased serum IgM and very low or undetectable IgG, IgA, and IgE. Broad spectrum of clinical manifestations and laboratory findings are observed in the HIGM patients including hematologic problem and malignancy. This study was conducted to assess demographic data, clinical manifestation, and immunological findings in the HIGM patients.
Methods: Lab findings and clinical presentations data of 79 Iranian patients diagnosed with HIgM syndrome were collected. All the patients were classified into two different groups including the patients with hematological problems and those without hematological problems.
Results: Hematologic problems were observed in 34 patients (43%, 23 males and 11 females). The most common hematologic problems types were anemia and leukemia (33 and 25%, respectively). Also, 19 patients (24.1%) had a family history of PID. Significant data that were higher in the patients with hematologic problems, were the oral ulcer (p=0.037), failure to thrive (p=0.022), recurrent diarrhoea (p=0.021), chronic diarrhoea (p=0.022), urinary tract infections (p=0.037), anemia (p=0.000), neutropenia (p=0.000), thrombocytopenia (p=0.001), gastrointestinal problem (p=0.011), neurologic problem (p=0.000), multiple site problem (p=0.000), platelet count (p=0.005), and IgG level (p=0.048).
Conclusion: The association between HIgM syndrome and hematologic problems could lead to severe clinical disorders. Therefore, it is necessary for immunologists to be aware of these situations.- انتشار مقاله: 29-10-1398
- نویسندگان: Mahsa Sohani,Salar PashangZadeh
- مشاهده
- جایگاه : پژوهشی
- مجله: Immunology and Genetics Journal
- نوع مقاله: Journal Article
- کلمات کلیدی: autoimmunity,Hyper IgM Syndrome,Immune trobocytopenia purpura
- چکیده:
- چکیده انگلیسی: Introduction: hyper-IgM (HIGM) syndrome is characterized by normal to increased serum IgM and very low or undetectable IgG, IgA, and IgE. HIGM (also known as class-switch recombination (CSR) defects) patients manifest different clinical manifestations such as autoimmune disorders. Aim of present study was to evaluate demographic data, clinical manifestation and immunological findings in HIGM patients.
Materials and Methods: Clinical features and immunological data were collected from the 79 Iranian HIGM patients’ medical records diagnosed in Children’s Medical Center in Iran. To compare clinical records and laboratory data, all HIGM patients were classified into two different groups patients with autoimmune disease and patients without autoimmune diseases.
Results: A total of 79 patients (60 male and 19 female ) with median (IQR) age at the time of the study of 12 (6-22.45). Autoimmunity diseases were seen in 19 patients (23.75% , 3 females and 16 males). Among the noninfectious manifestations, the hepatomegaly and spelenomegaly were significantly higher in patients with autoimmunity (p= 0.006) than patients without autoimmunity (p=0.006). The most common autoimmune presentations among HIGM patients were ITP (32%), juvenile rheumatoid arthritis (16%), autoimmune hemolytic anemia (11%) and Sclerosing cholangitis (11%), Gullain-Barré syndrome, Evans syndrome, diabetes mellitus and chrohn's disease.
Conclusions: The relation between HIGM syndrome and autoimmunity disorders could lead to sever clinical complications. So, we suggested that immunologists to be aware of this complications.- انتشار مقاله: 27-07-1398
- نویسندگان: Samaneh Delavari,Tannaz Moeini Shad,Salar Pashangzadeh
- مشاهده
- جایگاه : پژوهشی
- مجله: Immunology and Genetics Journal
- نوع مقاله: Journal Article
- کلمات کلیدی: autoimmunity,Primary Immunodeficiency Disorders,Selective IgA Deficiency
- چکیده:
- چکیده انگلیسی: Background and objective: Among primary immunodeficiency (PID), selective immunoglobulin A deficiency (SIgAD) is the most prevalent type. SIgAD patients can be either asymptomatic or symptomatic. Symptomatic patients suffer from a wide range of manifestations including infections, allergy, autoimmunity, and malignancy. SIgAD patients are more susceptible to some autoimmune diseases, while the exact mechanisms behind this association are not found yet. Therefore, this study was conducted in order to evaluate the possible association between autoimmune disease and specific clinical records or immunological data in SIgAD patients.
Methods: The present cohort included 166 SIgAD patients who were diagnosed at the Research Centre for Immunodeficiencies at the children’s medical Centre. A comprehensive history, demographic information, clinical manifestations, laboratory data were obtained from all patients to assess the autoimmune complications.
Results: Autoimmunity was seen in 16 patients (9.6%, 10 males and 6 females). The most common autoimmunity types were juvenile idiopathic arthritis, vitiligo and alopecia (18.8%). 9 patients (6.5%) had a PID family history. Significant data that were higher in patients with autoimmunity were the mean age at the study time (p=0.019), rheumatoid problem (p=0.043), liver problem (p=0.031), IgG level (p=0.006) and IgE level (p=0.004).
Conclusion: The association between SIgA deficiency and autoimmunity could lead to severe clinical complications. So, it is better for immunologists to aware of these problems.- انتشار مقاله: 28-04-1398
- نویسندگان: Salar Pashangzadeh,Mahsa Sohani
- مشاهده