Autoimmune polyglandular syndrome type 2 is defined as the occurrence of Addison’s disease concomitantly with autoimmune thyroid disease and/or type 1 diabetes mellitus. An 11-year-old boy with Hashimoto’s disease, Addison’s disease, celiac disease and Langerhans islet cell autoimmunity is described in this case report. Treatment of an endocrine disease may also trigger the onset of another endocrine disease. This case report underlines the importance of early recognition and treatment of critical endocrine diseases as well as the necessity to investigate pediatric patients with autoimmune diseases for coexisting conditions. Furthermore, the role of psychological stress as an inducer of autoimmunity was also discussed.
Endometriosis is the presence of endometrial tissue outside of the uterine cavity. In this paper, we present a patient with nasolacrimal system endometriosis which is a rare clinical condition.
Objective: We aimed to compare the clinical and laboratory findings of patients with juvenile idiopathic arthritis (JIA), to determine the preferred drugs and their efficacy and to evaluate the factors affecting prognosis and development of deformity.Methods: Retrospectively, 75 children with JIA were evaluated. Patients were divided into subgroups according to the International Association of Leagues for Rheumatology diagnostic criteria. The joints, systemic and extraarticular findings, laboratory findings, remission, deformity and relapse rates of JIA subgroups were compared.Results: 75 patients who were diagnosed with JIA, 50.7% were female and 49.3% were male. The largest group of patients were polyarticular JIA patients. Systemic symptoms were detected in 63 patients and extraarticular findings in 45 patients. In the systemic JIA group; anemia, leukocytosis, thrombocytosis, elevated erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and ferritin were most frequently detected. Rheumatoid factor (RF) and immunglobulin elevation were detected in the polyarticular group. Nonsteroidal anti-inflammatory drugs were started as initial treatment in 25 (33.3%) of the patients. Combined drug treatments were applied according to the clinical status of the illness, prognostic features and treatment at the centers. The most commonly used combination was NSAID, corticosteroids and methotrexate (MTX) treatment with a rate of 29.3%. The rate of remission was 85.2% while the deformity rate was 14.7% and the relapse rate was 40.4%. Conclusion:JIA is one of the most common chronic diseases of the childhood that is at the risk of sequelae and is one of the most common rheumatic diseases. In our study, the characteristics of the patients' male/female, subgroups, deformities and relapse rates were determined according to their socioeconomic and racial characteristics and clinical, laboratory features, treatments and remissions were determined.
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The term hemolytic uremic syndrome (HUS) describes a heterogenous group of diseases characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Diarrhea-associated HUS (D+ HUS) accounts for 90 % of cases in childhood, and it is one of the most frequent causes of acute renal failure in children less than five years of age leading to a significant degree of morbidity and mortality. The first step in the pathogenesis of D+HUS is the local inflammation of the intestinal epithelium and endothelium due to Shigatoxin (Stx) producing E. coli infection. This leads to intestinal hemorrhagie, loss of barrier functions, and generation of inflammatory cytokines. Together with TNF α and E.coli derived lipopolysaccharide Stx may act synergistically to damage vascular endothelium. Vascular endothelial injury and its consequences may lead to permanent damage of the kidney, brain, heart, pancreas, and necrosis of intestine. In contrast to D+HUS, the atypical HUS is not associated with diarrhea. Familial and recurrent form of HUS may be associated with low C 3 complement levels and/or genetic mutations in factor H. The main purpose of this article is to review the epidemiological, pathogenetic, and clinical features, current treatment strategies, and outcome of D+ HUS with a brief focus on atypical HUS.
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