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Papers by Reza Shiari

International Journal of Pediatrics, Apr 1, 2015

Introduction Systemic Lupus Erythematosus (SLE) is a complex autoimmune and inflammatory disease.... more Introduction Systemic Lupus Erythematosus (SLE) is a complex autoimmune and inflammatory disease. Many studies show HLA alleles can be associated with SLE. The aim of this study was to determine the association of HLA-DRB1 alleles with juvenile-onset in Iranian children. Materials and Methods At a casecontrol study, 31 children with systemic lupus erythematosus (case group) who referred to Mofid Children's Hospital, Shahid Behehsti University of Medical Sciences, Tehran, and 56 healthy children (control group) were participant. Genomic DNA was extracted and HLA typing was performed by Polymerase Chain Reaction (PCR) with Sequence-Specific Primers (SSP) technique. Results HLA-DRB1*01, HLA-DRB1*04, HLA-DRB1*11 and HLA-DRB1*13 were detected to as most frequent alleles associated with SLE in Iranian children. The frequency of HLA DRB1*08 was not significantly different in both groups (P>0.05(. HLA-DRB1*07 had a higher rate of repetition in the control group than patients with SLE. Conclusion There was a significant difference in the frequency of some alleles between patients and controls group, which could be related to susceptibility to SLE. These differences between frequencies of some alleles in both groups may help to determine the onset of lupus in children.

Pediatric Rheumatology, Sep 1, 2014

Juvenile Rheumatoid Arthritis is the most common rheumatologic disease of childhood period. The a... more Juvenile Rheumatoid Arthritis is the most common rheumatologic disease of childhood period. The aim of study was to compare ANA positive and ANA negative cases. This observation study was performed as correlation research. All cases with diagnosis ofJRA who visited Mofid Children's Hospital and Imam Hossein Hospital were included in this study. Duration of this study was from 2006, 1 October till 2008, 31 October. All patients were examined carefully by an experienced pediatric rheumatologist. Age, sex, disease onset, age at diagnosis, RF, ANA, HLA-B27, ESR, type of disease, disease activity, and duration of inactivity were included in this study. ANA titer was measured by immunoflurocence technique. Patients were categorized according to sex, ANA, and type of disease and then group was compared with each other. Data was analyzed by SPSS Ver. 16 (Chicago, IL, USA). This study was approved by Ethical Committee of university. In this study, 61 cases were enrolled. Twenty five cases (41%) were males and 36 cases were females. Mean of age at disease onset was 6.1 +/- 3.1 (Range 6 months to 12.5 years). Mean of age at disease diagnosis was 6.7 +/- 3.2. Mean of age at time of study was 7.6 +/- 3.5 (1.4 to 14 years). From all cases, 38 cases were oligoarticular, 18 cases were polyarticular and 5 cases were systemic onset. From 61 cases, 22 (36.1%) cases, had ANA positive JRA. Of these cases, 14 cases were oligoarticular and 8 cases were polyarticular. Except for sex and morning stiffness, there is significant correlation between type of disease and ANA,RF,HLA-B27, response to treatment, early onset erosion, subcutaneous nodule, and uveitis (P < 0.05). There is significant correlation between sex and ANA, RF, HLA-B27, early erosion, response to treatment, subcutaneous treatment, and uveitis (P < 0.05). There is significant correlation between ANA seropositivity and HLA-B27, early erosion, response to treatment, uveitis and subcutaneous nodule. Our results showed that there is significant correlation among ANA and other factors except morning stiffness. Another prospective study is recommended.

Pediatric Rheumatology, 2021

Background Although, preliminary reports of Severe Acute Respiratory Syndrome (SARS)-CoV-2 infect... more Background Although, preliminary reports of Severe Acute Respiratory Syndrome (SARS)-CoV-2 infection suggest that the infection causes a less severe illness in children, there is now growing evidence of other rare or even serious complications of disease. Case presentation During the recent COVID-19 pandemic in Kerman, Iran, two children (an 8 year-old boy and a 6 year-old girl) were referred to outpatient Clinic of Pediatric Rheumatology with complaints of limping. Both children had experienced fever and mild respiratory tract infection. At the beginning of the second week of infection, they developed joint effusion. They both tested positive for coronavirus infection and were therefore diagnosed with post Coronavirus reactive arthritis. Both children were treated successfully with rest and Non-Steroidal Anti-Inflammatory Drugs (NSAID). They did not have any medical problems in the two months fallow up. Conclusions These two cases suggest that COVID-19 may be rheumatogenic. Highlig...

Archives of Pediatric Infectious Diseases, 2021

Journal of Comprehensive Pediatrics, 2018

Background: Primary immunodeficiency disorders are a diverse group of rare genetic diseases that ... more Background: Primary immunodeficiency disorders are a diverse group of rare genetic diseases that are often under-recognized. Therefore, significant morbidities and mortalities ensue due to diagnosis delay. This is an evaluation of clinical presentations and laboratory data of children referred to Mofid University Hospital during a 10-year period. The aim of this study is to provide epidemiologic data for early diagnosis and proper management of these patients. Methods: A total of 80 children referred to Mofid's Children Hospital suspected to primary immunodeficiencies (PIDs) were evaluated clinically. The demographic, clinical, and laboratory data were collected in detail and were analyzed as a cross-sectional study. Missing data were completed in follow-up visits. Results: The prevalence of antibody deficiencies, phagocytic defects, and combined immunodeficiencies in 80 children with primary immunodeficiency syndromes were respectively 36.25%, 32.5%, and 31.25%. Common variable immunodeficiency (13.75%) and severe combined immunodeficiency (12.5%) had the highest prevalence among these disorders and the lowest prevalence were reported for cyclic neutropenia (5%), ataxia-telangiectasia (5%), and transient hypogammaglobulinemia of infancy (3.75%). The most common mode of inheritance detected was an autosomal recessive pattern. Pneumonia, otitis media, and diarrhea were the most common complications in the course of these disorders, which were seen respectively in 30%, 18.8%, and 15% of the patients. Conclusions: Issues regarding clinical presentation and management of primary immunodeficiencies continue to puzzle the clinical practitioner. Epidemiological data are needed to increase the awareness of the physicians regarding primary immunodeficiency disorders and to support the benefits of early diagnosis and treatment. Diagnosis delay is associated with increased morbidities and even mortalities.

Archives of Pediatric Infectious Diseases, 2021

Background: Kawasaki disease (KD) is a frequent cause of acquired heart disease in infants and yo... more Background: Kawasaki disease (KD) is a frequent cause of acquired heart disease in infants and young children, which may be triggered by infectious agents, with the highest incidence in Asian countries. Several genotypes are identified as susceptibility genes for KD, but none is known in the Iranian population. Objectives: Due to the racial differences in the frequency and significance of the identified genes, we aimed to investigate the 8p22-23-rs2254546 genotypes in a sample of Iranian children and their association with aneurysm and resistance to treatment. Methods: In this prospective cross-sectional study, 100 children with KD as the sample group were compared with 100 matched unrelated healthy Iranian children with no history of KD or immune-related disease. The control subjects were ethnically recruited from the same hospital at the time of a routine physical examination. The 8p22-23-rs2254546 genotypes were analyzed using the polymerase chain reaction (PCR) and direct sequen...

Iranian Journal of Child Neurology, 2019

Juvenile clinically amyopathic dermatomyositis (juvenile CADM) is a rare rheumatologic disease in... more Juvenile clinically amyopathic dermatomyositis (juvenile CADM) is a rare rheumatologic disease in children defined as the presence of the hallmark cutaneous features of dermatomyositis in absence of muscle involvement. In this article, we report an Iranian 14.5-year-old girl presented to Rheumatology Clinic of Mofid Children's Hospital, Tehran, Iran in Jan 2016 with cutaneous complaints diagnosed with juvenile CADM. Finally, we provide a literature review of previous studies on juvenile CADM.

Context: Henoch-Schonlein purpura (HSP) is a significant cause of chronic renal disease in childr... more Context: Henoch-Schonlein purpura (HSP) is a significant cause of chronic renal disease in children. This review determines some risk factors associated with renal involvement in childhood HSP. Evidence Acquisition: Electronic databases, including Google Scholar, PubMed, and Scopus were searched using the following keywords: “children”, “Henoch-Schonlein”, “risk factor”, “renal involvement”, and “IgA vasculitis”. This review was designed to identify the relevant electronic studies published in the English language from December 1998 to August 2018. Results: This review revealed that clinically older age at presentation, persistent rash, atypical rash, rash on unusual location, and gastrointestinal bleeding were significant risk factors for renal involvement. In contrast, joint involvement was not associated with renal involvement. Among biochemical markers, high red blood cell distribution width is a risk marker of renal involvement in HSP. In contrast, peripheral blood immunoglobul...

Iranian Journal of Child Neurology, 2020

Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory disease, which usually presents ... more Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory disease, which usually presents with skin rashes along with muscle weakness. We report a case of JDM in a 10- year-old girl with no skin manifestations presenting with progressive muscle weakness and fatigue. Further laboratory investigations, along with a muscle biopsy, confirmed the diagnosis of adermatopathic JDM. The patient was treated with intravenous immunoglobulin, corticosteroids, methotrexate, hydroxychloroquine, pamidronate, and rituximab. Following treatment, patients’ symptoms subsided, and she gained normal muscular strength over a year.

Clinical Medicine Insights: Arthritis and Musculoskeletal Disorders, 2020

Background: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis of the upper and lowe... more Background: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis of the upper and lower respiratory tract along with glomerulonephritis and is very rare in childhood. Its renal manifestations similarity with IgA vasculitis can be misleading. Case presentation: Herein, we report a 12-years-old girl with the clinical picture of IgA vasculitis and renal involvement at the time of presentation, over time, elevated cytoplasmic Anti-neutrophil Cytoplasmic Antibody (C-ANCA) and tissue biopsy confirmed GPA. Conclusion: In the case of a patient with an unusual presentation of IgA vasculitis, to some degree of suspicion, the GPA should be considered. Also, in approach to non-thrombocytopenic palpable petechia and purpura a wide range of differential diagnosis such as infections, ANCA associated vasculitis, and secondary vasculitis should be considered. Therefore, 2 effective method of GPA diagnosis, the high titer of C-ANCA test and tissue biopsy, should be considered simultaneously.

IgA vasculitis is a hypersensitivity vasculitis, which is usually self-limiting. Renal involvemen... more IgA vasculitis is a hypersensitivity vasculitis, which is usually self-limiting. Renal involvement is the most damaging long-term complication of IgA vasculitis, happening in 20% - 100% of cases. Some factors have been reported to be associated with renal involvement in IgA vasculitis; however, no biomarker has been proved as a risk factor for renal involvement and its severity yet. We followed 48 patients with a confirmed diagnosis of IgA vasculitis for six months. We checked these patients for renal involvement by microscopic urine examination. We checked aPL antibodies in all patients on admission and 12 weeks later. Urinalysis showed renal involvement in 14 of 48 patients with IgA vasculitis (29.16%). Antiphospholipid antibodies were positive in 9 patients with IgA vasculitis and renal involvement (9 out of 14, 64.28%), while they were positive in only six patients with IgA vasculitis without renal involvement (6 out of 34, 17.64%), showing a moderate correlation between positiv...

Clinical Medicine Insights: Arthritis and Musculoskeletal Disorders, 2020

Background: Musculoskeletal symptoms are a presenting manifestation in a number of lymphoprolifer... more Background: Musculoskeletal symptoms are a presenting manifestation in a number of lymphoproliferative disorders including leukemia, especially in children. Among these primary symptoms, midfoot arthritis seems to be an important alarm for malignancy in children. The aim of this study is evaluation association of midfoot arthritis with malignancy in children. Method: In this cross-sectional study, all medical records of patients with arthritis were identified and reviewed. All clinical and laboratory data were recorded in the information form and data were analyzed by SPSS 25 software. Results: A total of 557 cases of arthritis were evaluated, of which 18 (3.2%) cases have primary symptoms of midfoot arthritis. Four of 18 patients (22.2%) had B-cell precursor acute lymphoblastic leukemia, that midfoot arthritis was their first manifestation. Also, their laboratory findings confirmed that platelet, lactic acid dehydrogenesis, and uric acid values were significantly higher in these ch...

BMC Musculoskeletal Disorders, 2020

Background Benign joint hypermobility syndrome (BJHS) is one of the most common hereditary connec... more Background Benign joint hypermobility syndrome (BJHS) is one of the most common hereditary connective tissue disorders in children in which autonomic nervous system involvement has been reported. This study aimed to evaluate the frequency of primary focal hyperhidrosis in children with BJHS. Methods This observational-analytical study was conducted in a case-control setting on children aged 3 to 15 years in 2018 at Mofid Children’s Hospital, Tehran, Iran. Benign joint hypermobility syndrome was diagnosed according to the Brighton criteria; then, the patients referred to a dermatologist for evaluation of hyperhidrosis. Results In total, 130 eligible patients with confirmed BJHS and 160 age- and sex-matched healthy subjects were enrolled in this study. Primary focal hyperhidrosis (PFH) was seen in 56.2 and 16.3% of the cases and controls, respectively, indicating a significant difference (P < 0.05). The severity of hyperhidrosis did not differ between the two groups. Conclusion Alt...

Advances in Rheumatology, 2020

Background The current diagnostic cornerstone for septic arthritis contains gram stains, bacteria... more Background The current diagnostic cornerstone for septic arthritis contains gram stains, bacterial culture, and cell count with a differential of aspirated synovial fluid. Recently, a synovial leukocyte esterase (LE) test has been used for diagnosing septic arthritis. Since this test measures the esterase activity of leukocytes, there is always a dilemma for using this test in patients with inflammatory arthritis. Methods We collected the synovial fluid specimens as part of the general diagnostic protocol for patients suspected of Juvenile Idiopathic Arthritis (JIA) or Septic Arthritis (SA). Each group included 34 patients. We compared the result of the synovial LE test with the result of the culture of each patient. Results The mean ages of patients were 64.14 ± 31.27 and 50.88 ± 23.19 months in the JIA group and septic arthritis group, respectively. The LE test results were positive in 30 specimens, trace in 3 and negative in one in the first-time test and were positive in 31 spec...

Journal of Research in Medical Sciences, 2018

understood, [5,8] several infective pathogens, drugs, and certain toxins can trigger the disease.... more understood, [5,8] several infective pathogens, drugs, and certain toxins can trigger the disease. [1,3] Furthermore, genetic polymorphisms in cytokines and cell adhesion molecules involved in inflammatory responses are considered as potential causes of disease. [1,9,10] Among these genetic factors, human leukocyte antigen (HLA) and particularly HLA-DRB1-encoding the most prevalent beta subunit of HLA-DR-due to its central role in the immune system by presenting peptides derived from extracellular proteins is the principal candidate for increasing incidence of many autoimmune diseases; hence, its association with HSP has been investigated and reported in the literatures. [11-13] Differences and similarities in the frequency of certain HLA alleles have been observed in previous reports and the results of such studies vary depending on the ethnic Background: People of all ages can suffer from Henoch-Schönlein purpura (HSP), but it is the most common vasculitis in childhood. The most important involving gene is located on chromosome 6p21.3, a region coding for human leukocyte antigens (HLAs). Among HLA genes, because of the high rate of polymorphisms, HLA-DRB1 is estimated to have a strong association with HSP. In this study, we aimed to assess the association of HLA-DRB1 alleles with HSP in Iranian children. Materials and Methods: This study consisted of thirty Iranian children with HSP and 35 healthy controls. Genomic DNA was extracted, and HLA typing was performed by polymerase chain reaction with sequence-specific primers technique. Results: The results have shown that HLA-DRB1*01 and HLA-DRB1*11

Iranian Journal of Allergy, Asthma and Immunology, 2019

Chronic granulomatous disease (CGD) is a rare genetic disorder of neutrophil activity, resulting ... more Chronic granulomatous disease (CGD) is a rare genetic disorder of neutrophil activity, resulting in increased rate of recurrent infections with catalase–positive bacteria and fungi, as well as various autoimmune diseases such as sarcoidosis, rheumatoid arthritis, and discoid and/or systemic lupus erythematosus. Few reports have reported lupus erythematosus (LE) in patients with X–linked CGD (XL-CGD) and carriers, and very few in autosomal recessive CGD (AR-CGD). Here, we present 5 patients with CGD developing LE at different ages to emphasize on the importance of appropriate follow–up and treatment in patients with CGD with clinical signs and symptoms of autoimmune diseases and even in those with negative serologic results.

Journal of Lasers in Medical Sciences, 2019

Introduction: Regarding the limited ability of the damaged cartilage cells to self-renew, which i... more Introduction: Regarding the limited ability of the damaged cartilage cells to self-renew, which is due to their specific tissue structure, subtle damages can usually cause diseases such as osteoarthritis. In this work, using laser photobiomodulation and an interesting source of growth factors cocktail called the synovial fluid, we analyzed the chondrogenic marker genes in treated hair follicle dermal papilla cells as an accessible source of cells with relatively high differentiation potential. Methods: Dermal papilla cells were isolated from rat whisker hair follicle (Rattus norvegicus) and established cell cultures were treated with a laser (gallium aluminum arsenide diode Laser (λ=780 nm, 30 mW) at 5 J/cm2 ), the synovial fluid, and a combination of both. After 1, 4, 7, and 14 days, the morphological changes were evaluated and the expression levels of four chondrocyte marker genes (Col2a1, Sox-9, Col10a1, and Runx-2) were assessed by the quantitative real-time polymerase chain rea...

The Lancet Child & Adolescent Health, 2019

Archives of Pediatric Infectious Diseases, 2016

Background: Kawasaki disease (KD) is the most important cause of ischemic heart disease in childr... more Background: Kawasaki disease (KD) is the most important cause of ischemic heart disease in children. Its pathogenesis is not well understood, but geographic, ethnic and familial pattern of this syndrome is reported. Ischemic heart disease (IHD) in parents can be the result of KD in their children. This is a study on the prevalence of IHD in parents of children with severe and non-severe Kawasaki disease. Objectives: The current study aimed to estimate the prevalence of IHD in the families of children with KD. Patients and Methods: Sixty-one children with Kawasaki disease were admitted from December 21, 2004 to January 21, 2008to Mofid Children Hospital (from one month to thirteen-year old) and 50 patients entered the study. Subjects were divided into the severe (24subjects) and non-severe (26 subjects) groups. All of the parents were called for investigation. Data were analyzed by SPSS ver. 21 software. Results: Thirty-two (64%) subjects were male and 18(36%) were female.(1.8/1), mean age of children was 43 ± 33.1 months, and mean age in the severe and non-severe groups were 53.48 ± 37.26 and 32.19 ± 25.76 month, respectively (CI = 2-38.2, P = 0.02). History of IHD was more common in fathers of children in the severe Kawasaki disease group (P = 0.001) with no mean age difference between them. History of cardiac drug usage and hypertension was more common in the severe Kawasaki group (P = 0.009 and P = 0.046). Conclusions: Results of the current study revealed higher incidence of IHD in fathers of the subjects with severe KD. More investigation of genetic predisposition to Kawasaki disease acquisition is recommended.

International Journal of Pediatrics, Apr 1, 2015

Introduction Systemic Lupus Erythematosus (SLE) is a complex autoimmune and inflammatory disease.... more Introduction Systemic Lupus Erythematosus (SLE) is a complex autoimmune and inflammatory disease. Many studies show HLA alleles can be associated with SLE. The aim of this study was to determine the association of HLA-DRB1 alleles with juvenile-onset in Iranian children. Materials and Methods At a casecontrol study, 31 children with systemic lupus erythematosus (case group) who referred to Mofid Children's Hospital, Shahid Behehsti University of Medical Sciences, Tehran, and 56 healthy children (control group) were participant. Genomic DNA was extracted and HLA typing was performed by Polymerase Chain Reaction (PCR) with Sequence-Specific Primers (SSP) technique. Results HLA-DRB1*01, HLA-DRB1*04, HLA-DRB1*11 and HLA-DRB1*13 were detected to as most frequent alleles associated with SLE in Iranian children. The frequency of HLA DRB1*08 was not significantly different in both groups (P>0.05(. HLA-DRB1*07 had a higher rate of repetition in the control group than patients with SLE. Conclusion There was a significant difference in the frequency of some alleles between patients and controls group, which could be related to susceptibility to SLE. These differences between frequencies of some alleles in both groups may help to determine the onset of lupus in children.

Pediatric Rheumatology, Sep 1, 2014

Juvenile Rheumatoid Arthritis is the most common rheumatologic disease of childhood period. The a... more Juvenile Rheumatoid Arthritis is the most common rheumatologic disease of childhood period. The aim of study was to compare ANA positive and ANA negative cases. This observation study was performed as correlation research. All cases with diagnosis ofJRA who visited Mofid Children&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s Hospital and Imam Hossein Hospital were included in this study. Duration of this study was from 2006, 1 October till 2008, 31 October. All patients were examined carefully by an experienced pediatric rheumatologist. Age, sex, disease onset, age at diagnosis, RF, ANA, HLA-B27, ESR, type of disease, disease activity, and duration of inactivity were included in this study. ANA titer was measured by immunoflurocence technique. Patients were categorized according to sex, ANA, and type of disease and then group was compared with each other. Data was analyzed by SPSS Ver. 16 (Chicago, IL, USA). This study was approved by Ethical Committee of university. In this study, 61 cases were enrolled. Twenty five cases (41%) were males and 36 cases were females. Mean of age at disease onset was 6.1 +/- 3.1 (Range 6 months to 12.5 years). Mean of age at disease diagnosis was 6.7 +/- 3.2. Mean of age at time of study was 7.6 +/- 3.5 (1.4 to 14 years). From all cases, 38 cases were oligoarticular, 18 cases were polyarticular and 5 cases were systemic onset. From 61 cases, 22 (36.1%) cases, had ANA positive JRA. Of these cases, 14 cases were oligoarticular and 8 cases were polyarticular. Except for sex and morning stiffness, there is significant correlation between type of disease and ANA,RF,HLA-B27, response to treatment, early onset erosion, subcutaneous nodule, and uveitis (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.05). There is significant correlation between sex and ANA, RF, HLA-B27, early erosion, response to treatment, subcutaneous treatment, and uveitis (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.05). There is significant correlation between ANA seropositivity and HLA-B27, early erosion, response to treatment, uveitis and subcutaneous nodule. Our results showed that there is significant correlation among ANA and other factors except morning stiffness. Another prospective study is recommended.

Pediatric Rheumatology, 2021

Background Although, preliminary reports of Severe Acute Respiratory Syndrome (SARS)-CoV-2 infect... more Background Although, preliminary reports of Severe Acute Respiratory Syndrome (SARS)-CoV-2 infection suggest that the infection causes a less severe illness in children, there is now growing evidence of other rare or even serious complications of disease. Case presentation During the recent COVID-19 pandemic in Kerman, Iran, two children (an 8 year-old boy and a 6 year-old girl) were referred to outpatient Clinic of Pediatric Rheumatology with complaints of limping. Both children had experienced fever and mild respiratory tract infection. At the beginning of the second week of infection, they developed joint effusion. They both tested positive for coronavirus infection and were therefore diagnosed with post Coronavirus reactive arthritis. Both children were treated successfully with rest and Non-Steroidal Anti-Inflammatory Drugs (NSAID). They did not have any medical problems in the two months fallow up. Conclusions These two cases suggest that COVID-19 may be rheumatogenic. Highlig...

Archives of Pediatric Infectious Diseases, 2021

Journal of Comprehensive Pediatrics, 2018

Background: Primary immunodeficiency disorders are a diverse group of rare genetic diseases that ... more Background: Primary immunodeficiency disorders are a diverse group of rare genetic diseases that are often under-recognized. Therefore, significant morbidities and mortalities ensue due to diagnosis delay. This is an evaluation of clinical presentations and laboratory data of children referred to Mofid University Hospital during a 10-year period. The aim of this study is to provide epidemiologic data for early diagnosis and proper management of these patients. Methods: A total of 80 children referred to Mofid's Children Hospital suspected to primary immunodeficiencies (PIDs) were evaluated clinically. The demographic, clinical, and laboratory data were collected in detail and were analyzed as a cross-sectional study. Missing data were completed in follow-up visits. Results: The prevalence of antibody deficiencies, phagocytic defects, and combined immunodeficiencies in 80 children with primary immunodeficiency syndromes were respectively 36.25%, 32.5%, and 31.25%. Common variable immunodeficiency (13.75%) and severe combined immunodeficiency (12.5%) had the highest prevalence among these disorders and the lowest prevalence were reported for cyclic neutropenia (5%), ataxia-telangiectasia (5%), and transient hypogammaglobulinemia of infancy (3.75%). The most common mode of inheritance detected was an autosomal recessive pattern. Pneumonia, otitis media, and diarrhea were the most common complications in the course of these disorders, which were seen respectively in 30%, 18.8%, and 15% of the patients. Conclusions: Issues regarding clinical presentation and management of primary immunodeficiencies continue to puzzle the clinical practitioner. Epidemiological data are needed to increase the awareness of the physicians regarding primary immunodeficiency disorders and to support the benefits of early diagnosis and treatment. Diagnosis delay is associated with increased morbidities and even mortalities.

Archives of Pediatric Infectious Diseases, 2021

Background: Kawasaki disease (KD) is a frequent cause of acquired heart disease in infants and yo... more Background: Kawasaki disease (KD) is a frequent cause of acquired heart disease in infants and young children, which may be triggered by infectious agents, with the highest incidence in Asian countries. Several genotypes are identified as susceptibility genes for KD, but none is known in the Iranian population. Objectives: Due to the racial differences in the frequency and significance of the identified genes, we aimed to investigate the 8p22-23-rs2254546 genotypes in a sample of Iranian children and their association with aneurysm and resistance to treatment. Methods: In this prospective cross-sectional study, 100 children with KD as the sample group were compared with 100 matched unrelated healthy Iranian children with no history of KD or immune-related disease. The control subjects were ethnically recruited from the same hospital at the time of a routine physical examination. The 8p22-23-rs2254546 genotypes were analyzed using the polymerase chain reaction (PCR) and direct sequen...

Iranian Journal of Child Neurology, 2019

Juvenile clinically amyopathic dermatomyositis (juvenile CADM) is a rare rheumatologic disease in... more Juvenile clinically amyopathic dermatomyositis (juvenile CADM) is a rare rheumatologic disease in children defined as the presence of the hallmark cutaneous features of dermatomyositis in absence of muscle involvement. In this article, we report an Iranian 14.5-year-old girl presented to Rheumatology Clinic of Mofid Children's Hospital, Tehran, Iran in Jan 2016 with cutaneous complaints diagnosed with juvenile CADM. Finally, we provide a literature review of previous studies on juvenile CADM.

Context: Henoch-Schonlein purpura (HSP) is a significant cause of chronic renal disease in childr... more Context: Henoch-Schonlein purpura (HSP) is a significant cause of chronic renal disease in children. This review determines some risk factors associated with renal involvement in childhood HSP. Evidence Acquisition: Electronic databases, including Google Scholar, PubMed, and Scopus were searched using the following keywords: “children”, “Henoch-Schonlein”, “risk factor”, “renal involvement”, and “IgA vasculitis”. This review was designed to identify the relevant electronic studies published in the English language from December 1998 to August 2018. Results: This review revealed that clinically older age at presentation, persistent rash, atypical rash, rash on unusual location, and gastrointestinal bleeding were significant risk factors for renal involvement. In contrast, joint involvement was not associated with renal involvement. Among biochemical markers, high red blood cell distribution width is a risk marker of renal involvement in HSP. In contrast, peripheral blood immunoglobul...

Iranian Journal of Child Neurology, 2020

Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory disease, which usually presents ... more Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory disease, which usually presents with skin rashes along with muscle weakness. We report a case of JDM in a 10- year-old girl with no skin manifestations presenting with progressive muscle weakness and fatigue. Further laboratory investigations, along with a muscle biopsy, confirmed the diagnosis of adermatopathic JDM. The patient was treated with intravenous immunoglobulin, corticosteroids, methotrexate, hydroxychloroquine, pamidronate, and rituximab. Following treatment, patients’ symptoms subsided, and she gained normal muscular strength over a year.

Clinical Medicine Insights: Arthritis and Musculoskeletal Disorders, 2020

Background: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis of the upper and lowe... more Background: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis of the upper and lower respiratory tract along with glomerulonephritis and is very rare in childhood. Its renal manifestations similarity with IgA vasculitis can be misleading. Case presentation: Herein, we report a 12-years-old girl with the clinical picture of IgA vasculitis and renal involvement at the time of presentation, over time, elevated cytoplasmic Anti-neutrophil Cytoplasmic Antibody (C-ANCA) and tissue biopsy confirmed GPA. Conclusion: In the case of a patient with an unusual presentation of IgA vasculitis, to some degree of suspicion, the GPA should be considered. Also, in approach to non-thrombocytopenic palpable petechia and purpura a wide range of differential diagnosis such as infections, ANCA associated vasculitis, and secondary vasculitis should be considered. Therefore, 2 effective method of GPA diagnosis, the high titer of C-ANCA test and tissue biopsy, should be considered simultaneously.

IgA vasculitis is a hypersensitivity vasculitis, which is usually self-limiting. Renal involvemen... more IgA vasculitis is a hypersensitivity vasculitis, which is usually self-limiting. Renal involvement is the most damaging long-term complication of IgA vasculitis, happening in 20% - 100% of cases. Some factors have been reported to be associated with renal involvement in IgA vasculitis; however, no biomarker has been proved as a risk factor for renal involvement and its severity yet. We followed 48 patients with a confirmed diagnosis of IgA vasculitis for six months. We checked these patients for renal involvement by microscopic urine examination. We checked aPL antibodies in all patients on admission and 12 weeks later. Urinalysis showed renal involvement in 14 of 48 patients with IgA vasculitis (29.16%). Antiphospholipid antibodies were positive in 9 patients with IgA vasculitis and renal involvement (9 out of 14, 64.28%), while they were positive in only six patients with IgA vasculitis without renal involvement (6 out of 34, 17.64%), showing a moderate correlation between positiv...

Clinical Medicine Insights: Arthritis and Musculoskeletal Disorders, 2020

Background: Musculoskeletal symptoms are a presenting manifestation in a number of lymphoprolifer... more Background: Musculoskeletal symptoms are a presenting manifestation in a number of lymphoproliferative disorders including leukemia, especially in children. Among these primary symptoms, midfoot arthritis seems to be an important alarm for malignancy in children. The aim of this study is evaluation association of midfoot arthritis with malignancy in children. Method: In this cross-sectional study, all medical records of patients with arthritis were identified and reviewed. All clinical and laboratory data were recorded in the information form and data were analyzed by SPSS 25 software. Results: A total of 557 cases of arthritis were evaluated, of which 18 (3.2%) cases have primary symptoms of midfoot arthritis. Four of 18 patients (22.2%) had B-cell precursor acute lymphoblastic leukemia, that midfoot arthritis was their first manifestation. Also, their laboratory findings confirmed that platelet, lactic acid dehydrogenesis, and uric acid values were significantly higher in these ch...

BMC Musculoskeletal Disorders, 2020

Background Benign joint hypermobility syndrome (BJHS) is one of the most common hereditary connec... more Background Benign joint hypermobility syndrome (BJHS) is one of the most common hereditary connective tissue disorders in children in which autonomic nervous system involvement has been reported. This study aimed to evaluate the frequency of primary focal hyperhidrosis in children with BJHS. Methods This observational-analytical study was conducted in a case-control setting on children aged 3 to 15 years in 2018 at Mofid Children’s Hospital, Tehran, Iran. Benign joint hypermobility syndrome was diagnosed according to the Brighton criteria; then, the patients referred to a dermatologist for evaluation of hyperhidrosis. Results In total, 130 eligible patients with confirmed BJHS and 160 age- and sex-matched healthy subjects were enrolled in this study. Primary focal hyperhidrosis (PFH) was seen in 56.2 and 16.3% of the cases and controls, respectively, indicating a significant difference (P < 0.05). The severity of hyperhidrosis did not differ between the two groups. Conclusion Alt...

Advances in Rheumatology, 2020

Background The current diagnostic cornerstone for septic arthritis contains gram stains, bacteria... more Background The current diagnostic cornerstone for septic arthritis contains gram stains, bacterial culture, and cell count with a differential of aspirated synovial fluid. Recently, a synovial leukocyte esterase (LE) test has been used for diagnosing septic arthritis. Since this test measures the esterase activity of leukocytes, there is always a dilemma for using this test in patients with inflammatory arthritis. Methods We collected the synovial fluid specimens as part of the general diagnostic protocol for patients suspected of Juvenile Idiopathic Arthritis (JIA) or Septic Arthritis (SA). Each group included 34 patients. We compared the result of the synovial LE test with the result of the culture of each patient. Results The mean ages of patients were 64.14 ± 31.27 and 50.88 ± 23.19 months in the JIA group and septic arthritis group, respectively. The LE test results were positive in 30 specimens, trace in 3 and negative in one in the first-time test and were positive in 31 spec...

Journal of Research in Medical Sciences, 2018

understood, [5,8] several infective pathogens, drugs, and certain toxins can trigger the disease.... more understood, [5,8] several infective pathogens, drugs, and certain toxins can trigger the disease. [1,3] Furthermore, genetic polymorphisms in cytokines and cell adhesion molecules involved in inflammatory responses are considered as potential causes of disease. [1,9,10] Among these genetic factors, human leukocyte antigen (HLA) and particularly HLA-DRB1-encoding the most prevalent beta subunit of HLA-DR-due to its central role in the immune system by presenting peptides derived from extracellular proteins is the principal candidate for increasing incidence of many autoimmune diseases; hence, its association with HSP has been investigated and reported in the literatures. [11-13] Differences and similarities in the frequency of certain HLA alleles have been observed in previous reports and the results of such studies vary depending on the ethnic Background: People of all ages can suffer from Henoch-Schönlein purpura (HSP), but it is the most common vasculitis in childhood. The most important involving gene is located on chromosome 6p21.3, a region coding for human leukocyte antigens (HLAs). Among HLA genes, because of the high rate of polymorphisms, HLA-DRB1 is estimated to have a strong association with HSP. In this study, we aimed to assess the association of HLA-DRB1 alleles with HSP in Iranian children. Materials and Methods: This study consisted of thirty Iranian children with HSP and 35 healthy controls. Genomic DNA was extracted, and HLA typing was performed by polymerase chain reaction with sequence-specific primers technique. Results: The results have shown that HLA-DRB1*01 and HLA-DRB1*11

Iranian Journal of Allergy, Asthma and Immunology, 2019

Chronic granulomatous disease (CGD) is a rare genetic disorder of neutrophil activity, resulting ... more Chronic granulomatous disease (CGD) is a rare genetic disorder of neutrophil activity, resulting in increased rate of recurrent infections with catalase–positive bacteria and fungi, as well as various autoimmune diseases such as sarcoidosis, rheumatoid arthritis, and discoid and/or systemic lupus erythematosus. Few reports have reported lupus erythematosus (LE) in patients with X–linked CGD (XL-CGD) and carriers, and very few in autosomal recessive CGD (AR-CGD). Here, we present 5 patients with CGD developing LE at different ages to emphasize on the importance of appropriate follow–up and treatment in patients with CGD with clinical signs and symptoms of autoimmune diseases and even in those with negative serologic results.

Journal of Lasers in Medical Sciences, 2019

Introduction: Regarding the limited ability of the damaged cartilage cells to self-renew, which i... more Introduction: Regarding the limited ability of the damaged cartilage cells to self-renew, which is due to their specific tissue structure, subtle damages can usually cause diseases such as osteoarthritis. In this work, using laser photobiomodulation and an interesting source of growth factors cocktail called the synovial fluid, we analyzed the chondrogenic marker genes in treated hair follicle dermal papilla cells as an accessible source of cells with relatively high differentiation potential. Methods: Dermal papilla cells were isolated from rat whisker hair follicle (Rattus norvegicus) and established cell cultures were treated with a laser (gallium aluminum arsenide diode Laser (λ=780 nm, 30 mW) at 5 J/cm2 ), the synovial fluid, and a combination of both. After 1, 4, 7, and 14 days, the morphological changes were evaluated and the expression levels of four chondrocyte marker genes (Col2a1, Sox-9, Col10a1, and Runx-2) were assessed by the quantitative real-time polymerase chain rea...

The Lancet Child & Adolescent Health, 2019

Archives of Pediatric Infectious Diseases, 2016

Background: Kawasaki disease (KD) is the most important cause of ischemic heart disease in childr... more Background: Kawasaki disease (KD) is the most important cause of ischemic heart disease in children. Its pathogenesis is not well understood, but geographic, ethnic and familial pattern of this syndrome is reported. Ischemic heart disease (IHD) in parents can be the result of KD in their children. This is a study on the prevalence of IHD in parents of children with severe and non-severe Kawasaki disease. Objectives: The current study aimed to estimate the prevalence of IHD in the families of children with KD. Patients and Methods: Sixty-one children with Kawasaki disease were admitted from December 21, 2004 to January 21, 2008to Mofid Children Hospital (from one month to thirteen-year old) and 50 patients entered the study. Subjects were divided into the severe (24subjects) and non-severe (26 subjects) groups. All of the parents were called for investigation. Data were analyzed by SPSS ver. 21 software. Results: Thirty-two (64%) subjects were male and 18(36%) were female.(1.8/1), mean age of children was 43 ± 33.1 months, and mean age in the severe and non-severe groups were 53.48 ± 37.26 and 32.19 ± 25.76 month, respectively (CI = 2-38.2, P = 0.02). History of IHD was more common in fathers of children in the severe Kawasaki disease group (P = 0.001) with no mean age difference between them. History of cardiac drug usage and hypertension was more common in the severe Kawasaki group (P = 0.009 and P = 0.046). Conclusions: Results of the current study revealed higher incidence of IHD in fathers of the subjects with severe KD. More investigation of genetic predisposition to Kawasaki disease acquisition is recommended.