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BACKGROUND: Proactively seeking feedback from clinical supervisors, peers or other healthcare professionals is a valuable mechanism for residents to obtain useful information about and improve their performance in clinical settings. Given the scant studies investigating the limited aspects of psychometrics properties of the feedback-seeking instruments in medical education, this study aimed to translate the feedback-seeking behavior scales (frequency of feedback-seeking, motives of feedback-seeking, and promotion of feedback-seeking by supervisors) into Persian and evaluate the psychometric properties of the composite questionnaire among medical residents at Tehran University of Medical Sciences in Iran. METHODS: In this cross-sectional study, feedback-seeking behavior scales were translated through the forward-backward method, and its face validity and content validity were assessed by 10 medical residents and 18 experts. The test-retest reliability was evaluated by administering the questionnaire to 20 medical residents on two testing occasions. A convenience sample of 548 residents completed the questionnaire. Construct validity was examined by exploratory factor analysis and confirmatory factor analysis and concurrent validity was determined by Pearson's correlation coefficient. RESULTS: Content validity assessment showed that the CVR (0.66 to 0.99) and CVI (0.82 to 0.99) values for items and S-CVI values (0.88 to 0.99) for scales were satisfactory. The exploratory and confirmatory factor analysis demonstrated that the models were confirmed with eight items and two factors (explaining 70.98% of the total variance) for the frequency of feedback-seeking scale, with 16 items and four factors (explaining 73.22% of the total variance) for the motives of feedback seeking scale and with four items and one factor (explaining 69.46% of the total variance) for promotion of feedback-seeking by supervisors. AVE values greater than 0.5 and discriminant validity correlations significantly less than 1.0 demonstrated that the total scores of the composite feedback-seeking behavior questionnaire had a favorable fit and the questions could fit their respective factors, and the latent variables were distinct. We found positive and significant correlations between the three scales and their subscales. CONCLUSION: The results of the present study supported the validity and reliability of the Persian composite feedback-seeking behavior questionnaire for assessing feedback-seeking behaviors in medical residents. Applying the questionnaire in residency programs may enhance the quality of clinical education.
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Internado y Residencia , Psicometría , Humanos , Irán , Estudios Transversales , Encuestas y Cuestionarios/normas , Masculino , Reproducibilidad de los Resultados , Femenino , Adulto , Traducciones , Retroalimentación , Competencia ClínicaRESUMEN
BACKGROUND: Hyper-inflammatory syndrome in children and young adult occur 2-6 weeks after COVID-19 infection or closed contact with COVID-19 persons. In this study, the laboratory data and echocardiography and abdominal ultrasonography assessments were evaluated before and after Methylprednisolone pulse as an initial treatment of hyper-inflammatory syndrome. Therefore, the aim of this study is to assessment the clinical manifestations and laboratory data and outcome after methylprednisolone pulse as an initial treatment. METHOD: In this retrospective study, the demographic status, clinical features, laboratory data, echocardiography, abdominal ultrasound, treatment and outcome of 31 pediatric patients under 16 years old, with inflammatory process after COVID-19 were evaluated. The clinical assessments, laboratory data, sonography and echocardiography were evaluated before and after methylprednisolone pulse. The patients were divided in two age group < and ≥ 7 years old and the clinical manifestations were compared with each other. The Mann-Whitney U test was used to assess the difference in quantitative variables between two groups. To compare pre- and post- treatment values, Wilcoxol test was used. To assess the correlation between qualitative variables chi-square test was used. The level of significant was considered 0.05. These patients with fever and hyper-inflammation features admitted to the referral pediatric rheumatology ward in Children Medical Center of Tehran University of medical sciences, from April 2020 to May 2021 were assessed. RESULT: The mean age ± SD were (5.94 ± 3) and 51.6% (16) patients were male and 48.4% (15) patients were female. The most documented of previous COVID infection were antibody positive in about 27 (87%) patients. Moreover, 1 (3.8%) was PCR positive, 2 (7.7%) were positive in both PCR and serology and 3(11.5%) had closed contact with COVID-19 patients. About 9(29%) of patients were admitted in Intensive Care Unit (ICU). There were significant correlation between days of delay in starting treatment and ICU admission (P-value = 0.02). The mortality rate was negative in patients and no re-hospitalization was documented. There were significant differences (P-value < 0.05) between lymphocytes, platelet, Erythrocyte Sedimentation rate, C-reactive protein, Aspartate transaminase, Alanine transaminase and ferritin before and after treatment. Skin rashes and cardiac involvement totally as carditis (myocarditis, vulvulitis and pericarditis) (33.3%) and coronary involvements (53.3%) were the most prominent initial presentation in patients. There were near significant correlation (P-value = 0.066) between ferritin level and carditis before treatment. Cervical lymphadenopathy was seen significantly more in ≥ 7 years old (P-value = 0.01). CONCLUSION: Multisystem inflammatory system in children as a hyperinflammatory syndrome could be treated with first step methylprednisolone pulse with decreasing inflammation in laboratory data and cardiac involvements and good outcome. Furthermore, the ferritin level may be one of the predictor of severe hyper-inflammatory syndrome leading to aggressive and urgent treatment with methylprednisolone pulse.
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BACKGROUND: During childhood, the most common periodic fever is periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. The effective treatment and prevention of febrile attacks improve these patients' and their families' quality of life. However, there is no single strategy or evidence-based guideline to manage this syndrome, and most of them are based on consensus treatment plans. METHODS: This randomized controlled trial was carried out on 67 PFAPA patients referred to three tertiary centers of pediatric rheumatology. The patients were divided into two groups, including group 1 (n = 36) receiving prednisolone plus colchicine and group 2 (n = 31) receiving prednisolone plus cimetidine. Demographic characteristics and the number of febrile episodes were compared between the two groups before and after the intervention. RESULTS: In both groups, the number of febrile episodes after the treatment decreased (P ≤ 0.001). Statistical Analysis showed no significant difference between the two groups (P = 0.88). Moreover, 44 patients from both groups were checked for the MEFV gene. There were no statistical differences between MEFV positive and negative subgroups in response to colchicine (P = 1). CONCLUSION: This study showed that both drug regimens are significantly effective in preventing febrile attacks in PFAPA syndrome, and the presence of a MEFV gene mutation might not be the only significant risk factor for a response to colchicine. TRIAL REGISTRATION: IRCT, IRCT20191222045847N1. Registered 23 October 2019, https://fa.irct.ir/search/result?query=IRCT20191222045847N1.
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Linfadenitis , Linfadenopatía , Faringitis , Estomatitis Aftosa , Niño , Cimetidina/uso terapéutico , Colchicina/uso terapéutico , Fiebre/tratamiento farmacológico , Fiebre/prevención & control , Humanos , Linfadenitis/tratamiento farmacológico , Linfadenitis/prevención & control , Mutación , Faringitis/tratamiento farmacológico , Faringitis/prevención & control , Prednisolona/uso terapéutico , Pirina/genética , Calidad de Vida , Estomatitis Aftosa/tratamiento farmacológico , Estomatitis Aftosa/genética , Estomatitis Aftosa/prevención & control , SíndromeRESUMEN
BACKGROUND: Kawasaki disease (KD) is often complicated by coronary artery lesion (CAL), including dilatation or aneurysms. Intravenous immunoglobulin (IVIG) is used with aspirin to prevent CAL in KD. OBJECTIVE: Given that the primary treatment for other vasculitis is the use of corticosteroids, this study has been performed to evaluate the effect of intravenous methylprednisolone pulse (IVMP) therapy in preventing CAL in KD. METHOD: A randomized, single-blind clinical trial was conducted on 40 KD patients aged six months to five years. Patients were randomized into two groups according to the main treatment plan in addition to aspirin: case group (IVMP for three consecutive days and then oral prednisolone for three days) and control group (intravenous immunoglobulin 2 g/kg). Echocardiography was performed for all children at least three times, during the acute phase, two weeks, and two months later. RESULTS: Data analysis at the end of the study was done on 40 patients (20 patients in each group). There were no significant differences in age and sex distribution, mean fever, and acute phase duration, as well as baseline echocardiography in the two groups. The frequency of CAL was 20% in the case group and 45% in the control group, after two weeks (p<0.05), but there was no significant difference between two groups in types of coronary artery lesion after two weeks and the frequency and severity of CAL after two months. CONCLUSION: IVMP as initial line therapy effectively control systemic and vascular inflammation and decrease coronary artery damage in KD.
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INTRODUCTION: There are no determined treatment agents for severe COVID-19. It is suggested that methylprednisolone, as an immunosuppressive treatment, can reduce the inflammation of the respiratory system in COVID-19 patients. METHODS: We conducted a single-blind, randomised controlled clinical trial involving severe hospitalised patients with confirmed COVID-19 at the early pulmonary phase of the illness in Iran. The patients were randomly allocated in a 1:1 ratio by the block randomisation method to receive standard care with methylprednisolone pulse (intravenous injection, 250â mg·day-1 for 3â days) or standard care alone. The study end-point was the time of clinical improvement or death, whichever came first. Primary and safety analysis was done in the intention-to-treat (ITT) population. RESULTS: 68 eligible patients underwent randomisation (34 patients in each group) from April 20, 2020 to June 20, 2020. In the standard care group, six patients received corticosteroids by the attending physician before the treatment and were excluded from the overall analysis. The percentage of improved patients was higher in the methylprednisolone group than in the standard care group (94.1% versus 57.1%) and the mortality rate was significantly lower in the methylprednisolone group (5.9% versus 42.9%; p<0.001). We demonstrated that patients in the methylprednisolone group had a significantly increased survival time compared with patients in the standard care group (log-rank test: p<0.001; hazard ratio 0.293, 95% CI 0.154-0.556). Two patients (5.8%) in the methylprednisolone group and two patients (7.1%) in the standard care group showed severe adverse events between initiation of treatment and the end of the study. CONCLUSIONS: Our results suggest that methylprednisolone pulse could be an efficient therapeutic agent for hospitalised severe COVID-19 patients at the pulmonary phase.
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Antiinflamatorios/administración & dosificación , Tratamiento Farmacológico de COVID-19 , Metilprednisolona/administración & dosificación , Adulto , Anciano , Femenino , Hospitalización , Humanos , Inyecciones Intravenosas , Masculino , Persona de Mediana Edad , Quimioterapia por Pulso , Índice de Severidad de la Enfermedad , Método Simple CiegoRESUMEN
BACKGROUND: Nailfold capillaroscopy is a noninvasive technique to recognize peripheral microangiopathy, which is an important feature in systemic lupus erythematosus (SLE). The aims of the present study were to investigate the prevalence of nailfold capillaroscopy (NFC) changes in juvenile systemic lupus erythematosus (JSLE), find out patterns of these changes, and correlate findings with clinical and laboratory parameters. METHODS: Forty-nine patients with SLE, all meeting the 1997 revised ACR criteria for SLE classification, and 30 healthy controls were included. A digital video camera was used to capture nailfold capillary images. Computerized image processing was used for analysis. RESULTS: Different abnormal NFC changes were observed with abnormal morphology, the increased diameter and abnormal loop densities in 55.1%, 93.9%, and 26.5% of the patients, respectively. No statistically significant differences were depicted between capillaroscopy with age, gender, autoantibodies (APLs, anti-ds DNA), antiphospholipid antibody syndrome, thrombotic angiopathy, renal function tests (Bun, Cr), and abnormal urine analysis. However, a significant correlation was found between the branched pattern and the CNS involvement group (P value <0.03). CONCLUSIONS: Different abnormal NFC changes are quite common among patients with SLE, and nailfold capillaroscopy is an effective method to monitor such changes. Treatment strategies may change in the branched pattern of nailfold capillaroscopy due to CNS involvement.
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BACKGROUND: Alteration in serum expression of Transforming Growth Factor-beta (TGF-ß) and IL-10 have been suggested to play a role in the pathogenesis of Kawasaki Disease (KD). Inconsistent reports exist on the association of IL-10 polymorphisms with KD susceptibility and Coronary Artery Aneurysms (CAA). METHODS: A number of 110 paediatric patients with KD and 140 healthy individuals were recruited to investigate the frequency of Single Nucleotide Polymorphisms (SNPs) of TGF-ß C/T at codon 10 (rs1982073), C/G at codon 25 (rs1800471) and IL-10 A/G at -1082 (rs1800896), C/T at -819 (rs1800871) and A/C at -592 (rs1800872) and their respective genotype and haplotypes. A comprehensive search was performed in MEDLINE and SCOPUS using the keywords of interleukin 10, transforming growth factor beta, and Kawasaki disease. Moreover, previous studies investigating the TGF-ß and IL-10 polymorphisms in KD were evaluated. Review Manager Version 5.1 Software was used to perform meta-analysis. RESULTS: There was no significant association between allelic or genotypic variants in the mentioned polymorphisms in TGF-ß or IL-10 with KD or CAA. The only significant haplotypic variant was TC variant at codon 10, and 25 of TGF-ß polymorphisms were associated with higher risk of KD. Meta-analysis of a total number of 770 patients vs. 1471 healthy controls showed no difference in the frequency of any of the IL-10 genetic variants in KD patients, regardless of the presence of CAA. CONCLUSION: Polymorphisms of TGF-ß or IL-10 are not associated with additional risk for KD in Iranian population. IL-10 polymorphisms at -1082, -819 and -592 positions are not associated with KD, nor do they predict coronary artery aneurysm formation.
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AIM: Kawasaki disease (KD) is a systemic vasculitis of children associated with cardiovascular sequelae. Proinflammatory cytokines play a major role in KD pathogenesis. However, their role is both influenced and modified by regulatory T-cells. IL-1 gene cluster, IL-6 and TNF-α polymorphisms have shown significant associations with some vasculitides. Herein we investigated their role in KD. METHODS: Fifty-five patients with KD who were randomly selected from referrals to the main pediatric hospital were enrolled in this case-control study. Single nucleotide polymorphisms (SNPs) of the following genes were assessed in patients and 140 healthy subjects as control group: IL-1α at -889 (rs1800587), IL-1ß at -511 (rs16944), IL-1ß at +3962 (rs1143634), IL-1R at Pst-I 1970 (rs2234650), IL-1RN/A at Mspa-I 11100 (rs315952), TNF-α at -308 (rs1800629), TNF-α at -238, IL-6 at -174 (rs1800795) and IL-6 at +565. RESULTS: Twenty-one percent of the control group had A allele at TNF-α -238 while only 8% of KD patients had A allele at this position (P = 0.003, OR [95%CI] = 0.32 [0.14-0.71]). Consistently, TNF-α genotype GG at -238 had significant association with KD (OR [95% CI] = 4.31 [1.79-10.73]). Most controls carried the CG genotype at IL-6 -174 (n = 93 [66.9%]) while GG genotype was the most common genotype (n = 27 [49%]) among patients. Carriers of the GG haplotype at TNF-α (-308, -238) were significantly more prevalent among the KD group. No association was found between IL-1 gene cluster, allelic or haplotypic variants and KD. CONCLUSION: TNF-α GG genotype at -238 and GG haplotype at positions -308 and -238 were associated with KD in an Iranian population.
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Síndrome Mucocutáneo Linfonodular/genética , Polimorfismo de Nucleótido Simple , Factor de Necrosis Tumoral alfa/genética , Distribución de Chi-Cuadrado , Preescolar , Femenino , Frecuencia de los Genes , Estudios de Asociación Genética , Predisposición Genética a la Enfermedad , Haplotipos , Humanos , Lactante , Interleucina-1alfa/genética , Interleucina-1beta/genética , Interleucina-6/genética , Irán , Masculino , Síndrome Mucocutáneo Linfonodular/diagnóstico , Síndrome Mucocutáneo Linfonodular/inmunología , Oportunidad Relativa , Fenotipo , Receptores de Interleucina-1/genética , Estudios Retrospectivos , Factores de RiesgoRESUMEN
AIM: Kawasaki disease (KD) is a systemic vasculitis of medium-sized arteries. High levels of interleukin 4 (IL-4) and the dominance of Th2 cytokines seem to be a key feature in the acute phase of KD. In this study, the role of IL-4 and IL-4R gene polymorphisms were investigated in Iranian children with KD. METHODS: Fifty-five patients with KD and 140 healthy subjects as a control group were enrolled in this study. Single nucleotide polymorphisms (SNPs) of IL-4 gene at positions -1098 (rs2243248), -590 (rs2243250) and -33 (rs2070874), as well as IL-4RA gene at position +1902 (rs180275) were assessed in patients and the control group. RESULTS: The C allele and CC genotype of IL-4 gene at position -590 and at position -33 had positive associations and the CT genotype at -590 was negatively associated with KD (odds ratio (95% CI) = 0.04 [0.01-0.09]). The haplotype TCC was more frequent among the patients, while the haplotypes TTT and TTC had a negative association with KD. CONCLUSION: IL-4 polymorphisms might be associated with KD in an Iranian population.
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Interleucina-4/genética , Síndrome Mucocutáneo Linfonodular/genética , Polimorfismo de Nucleótido Simple , Factores de Edad , Estudios de Casos y Controles , Preescolar , Femenino , Frecuencia de los Genes , Estudios de Asociación Genética , Predisposición Genética a la Enfermedad , Haplotipos , Heterocigoto , Homocigoto , Humanos , Lactante , Subunidad alfa del Receptor de Interleucina-4/genética , Irán , Masculino , Síndrome Mucocutáneo Linfonodular/diagnóstico , Síndrome Mucocutáneo Linfonodular/inmunología , Fenotipo , Factores de RiesgoRESUMEN
BACKGROUND: Familial Mediterranean Fever (FMF) is an autosomal recessive disorder, characterized by recurrent and self-limited episodes of fever, abdominal pain, synovitis and pleuritis. FMF as the most common inherited monogenic autoinflammatory disease mainly affects ethnic groups of the Mediterranean basin, Arab, Jewish, Turkish, Armenian North Africans and Arabic descent. MATERIALS AND METHODS: In the present study, we selected 390 unrelated FMF patients according to the Tel-Hashomer criteria, and analyzed all patients for 12 most common mutations of MEFV gene by reverse hybridization assay (FMF strip assay). We also investigated exon 2 and 10 of MEFV gene in 78 patients by Sanger sequencing. RESULTS: According to strip assay results, at least one mutation was found in 234 patients (60%), and no mutation was found in other 156 patients (40%). The five most common mutations and allelic frequencies were M694V (13.6%), E148Q (10.4%), M694I (6.5%), V726A (4.1%), and M680I (3.8%). Moreover, we detected a novel missense variant (R204H, c.611 G > A) (SCV000297822) and following rare mutations among sequenced samples; R202Q, P115T, G304R, and E230K. CONCLUSION: This study describes the MEFV mutations spectrum and distribution in Iranian population, and shows different mutation patterns among Iranian ethnicities. Moreover, M694V is the most common MEFV mutation in Iran.
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Fiebre Mediterránea Familiar/genética , Frecuencia de los Genes , Mutación Missense , Pirina/genética , Niño , Fiebre Mediterránea Familiar/patología , Femenino , Genotipo , Humanos , Irán , MasculinoRESUMEN
Children with juvenile idiopathic arthritis (JIA) are prone to the problems that can delay their psychosocial development; however, the existing literature has not reached a consensus on the psychological problems related to JIA. A total of 51 children and adolescents with JIA and 75 healthy controls aged 6 to 18 years were examined using the Child Behavioral Checklist (CBCL). Our results represented that 70 percent of JIA group reached "borderline clinical" range or "clinical" range in internalizing problems, while this percentage in the control group was 18 percent. In addition, our results indicated that JIA group has gotten significantly higher scores (more than twofold) in externalizing behaviors compared to control group. Furthermore, children with JIA showed higher rate of anxiety/depression, withdrawal/depression, somatic complaints, rule breaking behaviors, and aggressive behaviors as well as thought and social problems compared to control group (p < 0.001). As a conclusion, children and adolescents with JIA compared to healthy controls may show higher rate of both internalizing and externalizing problems. Furthermore, our novel findings on externalizing, social, and thought problems in JIA warrant further investigation on affected children who may be at greater risk of future psychopathologies.
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BACKGROUND: Various indices have been raised as predictors of activity and severity of juvenile idiopathic arthritis. OBJECTIVES: This study was conducted to investigate the changes of platelet indices in acute phase and two months after treatment in these patients. PATIENTS AND METHODS: In a cohort study, platelet count, mean platelet volume (MPV), platelet distribution width (PDW), plateletcrit (PCT) were evaluated in children referred to children's medical center, Tehran due to juvenile idiopathic arthritis from March 2013 to March 2014 during the acute phase and two months after standard treatment. The statistical data were analyzed by SPSS 19 software, and the significance level was set as P < 0.05. RESULTS: In this study, 55 children (24 boys and 31 girls) with mean ± SD age of 7.50 ± 3.35 years were studied. The mean ± SD value of platelet count was 441872.7 ± 151836.9 in the acute phase and reached 395418.2 ± 119601.6 two months after treatment (P = 0.01). The mean ± SD PCT in the acute phase of various subtypes of the disease was 0.32 ± 0.11, which reached 0.29 ± 0.10 after treatment (P = 0.09). However, the PDW range in different subtypes of the disease reached 13.4 ± 8.0 from 13.9 ± 2.9 and MPV reached 8.7 ± 0.9 from 8.8 ± 1.1 after treatment, but they were not significantly different from the results in the acute phase (P = 0.5). CONCLUSIONS: Platelet count is one of the most remarkable indices in JIA. Evaluation of PCT can also help determine the severity of the inflammatory process in the follow-up and treatment process.
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INTRODUCTION: Headache is a common neuropsychiatric manifestation of juvenile systemic lupus erythematous (JSLE). Pseudotumor cerebri (PTC) is an uncommon cause of headache, presenting especially in active JSLE. In this paper, we report a case of missed intractable headache that was eventually diagnosed as PTC and presented as the first manifestation of JSLE. CASE PRESENTATION: A 9-year-old girl with a history of progressive headache for four months, fever, fatigue, myalgia, arthralgia, small-joint arthritis of the hands, and recent diplopia was referred to our clinic. The diagnosis of PTC in the background of JSLE was made based on her history, physical examination, positive laboratory findings, and increased intracranial pressure, with normal neuroimaging. Treatment with high-dose prednisolone led to dramatic resolution of the headache. CONCLUSIONS: It seems that a complete neurologic examination in newly diagnosed SLE patients is mandatory, especially in the presence of any neuropsychiatric manifestations, such as headache.
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This study evaluated the clinical features, course, and outcomes of Iranian children with juvenile dermatomyositis (JDM), juvenile polymyositis (JPM), and other uncommon connective tissue disorders. A chart review of 85 Iranian children with JDM and JPM was performed during a 10-year period from 2003 to 2013. The patients' clinical signs and symptoms, laboratory data, and other factors affecting clinical outcomes were recorded using questionnaires. Statistical analysis was performed using SPSS software version 20. In all, 40 boys and 45 girls were included in the study (F/M, 1.1:1). Disease frequency was significantly higher in boys aged <5 years (F/M, 0.4:1) and girls aged >5 years (F/M, 1.6:1). The combined mean age at diagnosis was 7.5 years. Muscle weakness, particularly in the proximal muscles of lower extremities (96 %); fatigue (83 %); and heliotrope rash (71 %) were the most frequently recorded symptoms. Elevated lactate dehydrogenase level was the most common enzyme disturbance (98 %). Monocyclic course was seen in 60 % of patients. The mean treatment duration was 3 years. The incidence rate of complications such as calcinosis, lipodystrophy, and growth disturbances was 20, 9, and 30 %, respectively. The occurrence of these complications in patients with monocyclic disease was significantly lower. Vital organ involvement led to the death of four patients. The incidence of calcinosis was significantly lower in patients having a shorter interval between disease onset and treatment. Two important complications, failure to thrive and lipodystrophy, were significantly higher in patients having antinuclear antibodies. The incidence of the above three complications was higher in patients with polycyclic or continuous chronic disease. Respiratory failure was the most common cause of patient mortality.
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Antirreumáticos/uso terapéutico , Dermatomiositis/diagnóstico , Glucocorticoides/uso terapéutico , Prednisona/uso terapéutico , Adolescente , Niño , Preescolar , Dermatomiositis/tratamiento farmacológico , Dermatomiositis/mortalidad , Progresión de la Enfermedad , Quimioterapia Combinada , Femenino , Humanos , Lactante , Irán , Masculino , Pronóstico , Factores SexualesRESUMEN
BACKGROUND: Infantile Systemic Hyalinosis (ISH) is a very rare autosomal recessive disorder characterized by connective tissue involvement as hyaline deposition in skin, gastrointestinal tract, muscles, glands and other organs. CASES PRESENTATION: We report eight Iranian children (4 male and 4 female) with ISH referred to our hospital from 1996 to 2013. The illness had been diagnosed by clinical manifestations and disease progression. Six of them died and two are alive but very sick. CONCLUSION: ISH is a very rare disorder with poor prognosis. Seventy five percent of our 8 patients died before 2 years old due to severe diarrhea, malabsorption and/or infection.
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Wegener's granulomatosis or granulomatosis polyangiitis (GPA) is an uncommon chronic systemic vasculitis in children. The aim of this study was to describe pediatric patients with GPA in Iran. We studied records of all patients with GPA diagnosis who were referred to all Iranian Pediatric Rheumatology Division from 2002 to 2011. A total of 11 patients (5 females and 6 males) enrolled in this study. In children less than 15 years old, the prevalence of GPA is 0.6 per million. The mean age of GPA diagnosis was 11 years and average delay diagnosis was 20 months. Mortality rate was 18.1% due to pulmonary vasculitis and infection. The most common organ system involvement was upper and lower respiratory tract involvement (81.8% and 63.9%, resp.). Other common manifestations were renal (36.3), skin (27.2%), and eye involvement (18.2%).
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BACKGROUND AND OBJECTIVES: Brucellosis is considered the leading zoonotic disease of the Middle East. The disease has a wide spectrum of clinical manifestations and can result in complications with severe morbidity. The aim of this study was to evaluate the frequency, distribution and characteristics of arthritis in Iranian children with brucellosis. DESIGN AND SETTING: A retrospective descriptive study conducted in a referral children center in Tehran from 1997 to 2005. PATIENTS AND METHODS: Arthritis in children with brucellosis who were admitted to a specialized hospital was detected by clinical signs of the involved joint(s) and characteristics of joint fluid aspiration. Socio-demographic information was recorded. RESULTS: Of 96 patients diagnosed with brucellosis, 24 (25%) had Brucella arthritis 14 (58.3%) males and 10 (41.7%) females. Most common manifestations were fever in 21 patients (87.5%) and fatigue in 18 patients (75%). Monoarthritis was recorded in 15 patients (62.5%) of the cases with involvement of the knee in 8 (45%) and hip in 5 (29%), the ankle in 2 (8%) patients while 9 (37.5%) patients suffered from polyarthritis. None of the patients had axial joints involvement. Seventy-five percent of the subjects (18 patients) were from urban areas and 66.7% (16 patients) had consumed un-pasteurized cheese. Recurrence was not seen in any of the 24 patients who received a combination of co-trimoxazole for 6 weeks and gentamicin for 5 days. CONCLUSION: Childhood brucellosis is a challenging disease in Iran that has serious complications like arthritis. Therefore all physicians who work in endemic areas should be familiar with this disease and consider the possibility of brucellosis in all children who present with arthritis and arthralgia.