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Öğe Canakinumab treatment in four children with colchicine resistant familial mediterranean fever(Pakistan Medical Assoc, 2017) Ozkan, Solmaz; Atas, BulentFamilial Mediterranean Fever (FMF) is an autosomal recessive and autoinflammatory disease, characterized with inflammation of serous membranes such as peritoneum, pleura, synovium with fever and pain. Colchicine is the main treatment of FMF, but 5-10 % of patients are unresponsive to colchicine. We report using anti-interleukin-1 agents anakinra and canakinumab in four colchicine-resistant patients who were successfully treated. Three of the patients were siblings.Öğe Congenital Nephrotic Syndrome: Case Report and Review of Literature(Cukurova Univ, Fac Medicine, 2012) Konak, Murat; Annagur, Ali; Altunhan, H.; Atas, Bulent; Ors, RahmiCongenital nephrotic syndrome (CNS) is a rare kidney disease which reveals itself after birth and characterized with severe proteinuria, hypoalbuminemia and edema. It is a genetic disorder that occurs with deterioration of glomerular filtration barrier especially as a result of mutation that develops in genes called nephrin and podocin. CNS could be as a result of perinatal infection as well as a part of a genetic syndrome. Immune suppressive treatment is ineffective in genetically originated CNS, however renal transplantation yields curative treatment. In many cases to prevent from life threatening edema, daily albumin infusion is needed. Additionally, high caloric diet, thyroxin and mineral supply are applied. Also prophylaxis of thrombolytic complications and opportunistic infection that could develop due to immune deficiency is needed. In this report we discussed a case with the review of literature by reporting a newborn which had CNS diagnosis as a result of persistent hypoalbuminemia and proteinuria without edema.Öğe Early kidney injury in immunoglobulin A vasculitis: Role of renal biomarkers(Wiley, 2021) Ture, Esra; Yazar, Abdullah; Akin, Fatih; Topcu, Cemile; Aydin, Arif; Balasar, Mehmet; Atas, BulentBackground We aimed to determine whether urine kidney injury molecule 1 (KIM-1) and neutrophil gelatinase-associated lipocalin (NGAL) can be used as early noninvasive biomarkers of kidney injury in immunoglobulin A vasculitis. Methods Patients who were diagnosed with immunoglobulin A vasculitis were included in the study. Urine samples were collected for determination of urine KIM-1 and NGAL levels. The control group consisted of age-matched healthy children. Results Sixty-one patients who were diagnosed with immunoglobulin A vasculitis were included in the study; 37.7% of these patients were determined to have renal involvement. Median KIM-1 was found to be significantly higher in the patient group (69.59 pg/mL) than the control group (40.84 pg/mL) (P = 0.001). Median NGAL was determined to be statistically significantly higher in the patient group (59.87 ng/mL) compared with the control group (44.87 ng/mL) (P = 0.013). In 23.6% of the patients without renal involvement at admission renal involvement developed within the following 6 months. When median KIM-1 and NGAL at admission of these patients were compared with the control group, they were determined to be statistically significantly higher (P = 0.001, P = 0.003). Conclusions The fact that our patients with late-term nephropathy had no hematuria and / or proteinuria and that KIM-1 and NGAL levels were determined to be high indicates that these biomarkers might be potentially reliable, noninvasive and early determinants of kidney injury.Öğe Paediatric systemic lupus erythematosus: A single referral centre experience(Pakistan Medical Assoc, 2021) Atas, Bulent; Bulut, Mustafa; Sap, Fatih; Yazar, Abdullah; Akin, Fatih; Poyraz, Necdet; Tokgoz, HuseyinIn this study, the clinical and laboratory findings, management and follow-up of 32 children with paediatric systemic lupus erythematosus (pSLE) were evaluated to determine the prognostic factors in pSLE. Of the 32 patients, 25 (78.1%) were females. Age at onset of symptoms and diagnosis in the patients were 147.6 +/- 49 months and 154.3 +/- 48 months, respectively. The most common symptom on admission were joint problems, seen in 25 (78.1%) patients. Haematological alterations were seen in 25 (78.1%) cases during follow-up. Lupus nephritis was diagnosed in 10 (31.2%) patients. Malar rash was seen in a total of 12 (37.5%) patients during follow up, however it had been noted in five (15.6%) patients on admission. Antinuclear antibody and anti-dsDNA were positive in all patients and 31 (96.8%) patients, respectively. Decreased complement 3 and 4 levels were noted in 23 (71.8%) patients. Antiphospholipid antibody was studied in 27 patients and it was found to be positive in 13 (48.1%) patients. In conclusion, based on our findings, we would like to emphasize that pSLE has a large and remarkable clinical and laboratory findings.Öğe Spontaneous Rupture of the Spleen in a Patient With Systemic Lupus Erythematosus Initially Presented as Evans Syndrome(Lippincott Williams & Wilkins, 2014) Tokgoz, Huseyin; Caliskan, Umran; Atas, Bulent; Ozbek, Orhan; Tavil, BetulBackground: Although splenic abnormalities are common in patients with lupus, spontaneous rupture of spleen is extremely rare. Observations: A 15-year-old boy with new-onset Evans syndrome subsequently diagnosed as systemic lupus erythematosus developed spontaneous rupture of spleen during the course of his illness. Despite the severe thrombocytopenia, he was managed conservatively with gradual regression of hematoma without further complication. Conclusions: Splenic rupture may occur spontaneously in the course of systemic lupus erythematosus. We conclude that conservative treatment of splenic rupture may be preferred especially in immunocompromised patients to avoid surgical complications.