18 research outputs found

    MEFV Gene Mutation Analysis in Children with Immunoglobulin A Vasculitis and Its Effects on Clinical Manifestations: A Big Series from a Tertiary Center

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    Aim: Immunoglobulin A vasculitis (IgAV) is the most common vasculitis of childhood, but its pathogenesis is largely unknown, despite evidence pointing to various environmental and genetic factors. We investigated the frequency of MEFV gene mutations that are considered in the pathogenesis and their effect on the clinical features of patients with IgAV. Methods: The study included 244 children diagnosed with IgAV, who underwent MEFV gene analyses. We recorded the demographic and clinical characteristics of the patients, along with their laboratory results. We grouped the patients based on the presence of MEFV gene mutations and MEFV variants. Results: At least one MEFV mutation was detected in 89 (36.5%) patients, with E148Q being the most common (n=31, 34.8%). Age at diagnosis and the frequency of hematuria and recurrence were significantly greater among patients with MEFV mutations (p=0.043, p=0.008, and p=0.009, respectively). Serum IgA levels were significantly higher in patients with the M694V mutation (p=0.040). Conclusion: The presence of MEFV mutations, particularly E148Q and M694V, could be associated with the development and clinical course of IgA vasculitis

    Evaluation of immune response to hepatitis A vaccination and vaccine safety in juvenile idiopathic arthritis

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    Background: Autoimmune mechanisms and drugs used in treatment increase the risk of liver disease in patients with juvenile idiopathic arthritis (JIA) and hepatitis A virus (HAV) vaccination is important, especially in intermediate-endemicity areas like Turkey. In our study, we aimed to evaluate the immune response to hepatitis A vaccine and vaccine safety in children with JIA. Methods: This study was carried out in our hospital’s Pediatric Rheumatology outpatient clinic and Healthy Child clinic between the years 2003 and 2008. The study group consisted of 47 children with JIA (23 male and 24 female) diagnosed according to International League of Associations for Rheumatology diagnostic criteria. The control group consisted of 67 healthy children (31 female, 36 male) who did not have a history of hepatitis A infection or vaccination. Both groups were vaccinated with two doses of hepatitis A vaccine at 6-month intervals. Anti-HAV IgG >80 MIU was accepted as positive response. Results: There was no significant difference between the groups in terms of age and sex. None of the patients with JIA had fever, clinical worsening, or disease activation after vaccination. Anti-HAV IgG positivity rate was significantly higher in the control group (p < 0.05). Anti-HAV IgG was negative in only four cases, and they were all male patients with systemic JIA who had active disease under anti-tumor necrosis factor treatment. Conclusion: Hepatitis A vaccine was safe in patients with JIA, and response to vaccine did not differ between healthy children and patients with JIA except for children with active systemic JIA receiving anti-tumor necrosis factor alpha drugs

    Optic Neuritis Following Hepatitis B Vaccination in a 9-year-old Girl

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    Hepatitis B vaccination can prevent hepatitis B virus infection and its serious consequences, including liver cancer and cirrhosis. Serious side effects reported after receiving hepatitis B vaccination are very uncommon. A majority of the ophthalmological complications seen following hepatitis B vaccination consist of vision loss, optic neuritis, papillary edema, uveitis, acute placoid pigment epitheliopathy and central vein occlusion. We present a 9-year-old girl who was referred to our hospital with decrease in vision and pain in the left eye a week after hepatitis B vaccination. A diagnosis of vaccine- induced optic neuritis was made. The clinical picture improved after systemic corticosteroid treatment. Acute optic neuritis might be a rare complication of hepatitis B vaccination. Parents should be aware of the potential serious side effects of the vaccine

    Vesicoureteral Reflux Incidence In Siblings Of Children With Reflux: Is Screening Required?

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    Aim: The purpose of this study was to evaluate prospectively the age-related incidence and severity of primary vesicoureteral reflux (VUR) in siblings of children with reflux and to conclude whether screening of this group is required or not. Methods: A total of 71 siblings (32 boys, 39 girls) of reflux patients, aged less than 15 years (mean age, 8.4 years) were included in the study. Examination methods including urinalysis, urine culture, renal ultrasonography, and voiding cystourethrography were performed in all of the siblings. Additionally, 99m technetium (Tc)-dimercaptosuccinic acid (DMSA) renal cortical scintigraphy was performed in siblings with VUR. Results: Primary VUR was found in 10 (14%) of the siblings, and the incidence for girls and boys were 41.7% and 9.3%, respectively. The reflux incidence in siblings aged less than 6 years was 40%, whereas in siblings older than 6 years it was found 60%. DMSA scintigraphy was performed on all the siblings with VUR, of whom 5 had normal and 5 had abnormal findings. Conclusion: The incidence of primary VUR in siblings of affected children was found higher compared to the siblings of children without the disease, supporting the necessity to screen this population

    Carotid Sheath Abscess Caused by a Tooth Decay Infection on the Opposite Side

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    Deep neck infections are mortal diseases that need emergency treatment. It can occur at any age but usually in pediatric ages. In this report, a left cervical carotid space abscess of a pediatric patient was discussed. It was interesting that the only origin of the left carotid sheath abscess was right inferior first molar tooth decay. Right neck spaces were all clean. Patient had no immunosupression and also there were no congenital masses such as branchial cleft cysts, foreign bodies, or masses suspicious for malignancies in cervical ultrasound and MRI. We discussed this rare condition under the light of the literature
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