Makale Arama

Thrombotic thrombocytopenic purpura (TTP) is a rare and fatal disorder characterized with microangiopathic hemolytic anemia, thrombocytopenia, neurological symptoms, fever, and kidney failure. The reason of TTP and optimal treatment method of TTP is still unknown. However, today, therapeutic plasma exchange (plasmapheresis) and / or plasma infusion is considered as the standard treatment method for these patients. In this study, the clinical characteristics and outcome of 18 patients with TTP treated with plasma exchange at the Department of Hematology, Meram Faculty of Medicine, Necmettin Erbakan University, between 2008 and 2011 years were evaluated retrospectively. Among the patients, 6 were male, 12 were females, median age was 39 (21-66), 8 were idiopathic, 7 were postpartum, 1 was post operative periods of prosthetic operation, 1 was pesticide exposure and 1 was NSAII induced TTP. The first admission of patients were evaluated for symptoms and complete blood counts, coagulation tests, BUN (blood urea nitrogen), creatinine, LDH (lactic dehydrogenase) levels were performed. All patients were initially treated with a daily plasma exchange plus steroids, which was continued until the normalization of platelet count and the level of LDH. In this study, a total of 165 plasmapheresis procedures were performed to total 18 cases. Fresh frozen plasma (FFP) was used as fluid of replacement. Of the 18 patients, 15 were discharged with complete remission (n:12) and partial remission (n:3), and 3 had no response and died of progressive neurological fingings and acute renal failure. Untreated TTP has a high mortality that may be as great as 90%. However, the rate of this mortality is dramitacally changed with plasma exchange treatment. As a result, today, plasma exchange is the most effective treatment available for treatment of TTP.

Aim: In this retrospective study, our patients with nephrotic syndrome (NS) were evaluated according to age, sex, etiology, response to steroid, prognosis, and compared with national data and the literature. Material and method: 141 children with nephrotic syndrome (NS) who admitted to the Department of Pediatric Nephrology of Selcuk University between January 2000 and December 2006 were assessed. Patients were classified into two groups according to etiology as primary and secondary NS. Results: 59% of the patients were male and 41% of them were female. Male/female ratio was 1.44, and median age was 9 (0.3-18.5) years. It is observed that 81.5% of the cases had primary and 18.5% had secondary NS. 67 of the patients with primary NS were male and 48 of them were female. Median age was 8 (0.3-18) years. 16 of the patients with secondary NS were male and 10 of them were female. Median age was 14 (7.5-18.5) years. Of patients with primary NS, 76.5% had steroid-responsive, and 22.6% steroide resistant. %64.8 of the patients with steroid responsive NS developed one and more relapses. Percentage of rare and frequent relapses were 3.4% and 61.3%, respectively. Our results showed that mesangioproliferative glomerulonephritis (MesPGN) was the most common histopathologic diagnosis, 15 (45%) of the 31 biopsied patients with primary NS were found to have MesPGN. Henoch Schonlein purpura nephritis was diagnosed in 14 (53.8%) patients and it was the most common cause of secondary NS. As a result, NS frequency and response to rate of steroid therapy are similar to present literature finding. However, frequent relaps ratio was lower than the others studies. Conclusion: We think that further research including more patients population and multiple center and control studies to investigate NS events to reveal causes of concerning with regional, national or race differences at the childhood period is warranted.

Aim: We aimed to evaluate the relationship between magnetic resonance (MR) enterography and diffusion magnetic resonance imaging (MRI) investigations in inflammatory bowel disease (IBD) patients during active inflammation and remission phases. 

Patients and Methods: We included patients diagnosed with IBD between 01.12.2015-31.08.2016 at our hospital. 47 patients were included; 30 were male (63.8%) and 17 were female (36.2%). Thirty two (68.1%) of them had ulcerative colitis and 15 (31.9%) had Crohn’s disease. Standard institutional sequences for upper and lower abdomen scan included: sagittal and axial T2 TRUFI, axial and coronal fat suppressed T2 TRUFI and coronal and axial T1 VIBE following intravenous (at 60 seconds) contrast medium administration. First group of patients (n=10) had MRI investigations both at active and remission phases, whereas in the second group of patients (n=37) apparent diffusion coefficient (ADC) values, bowel wall thickness and contrast enhancement grades were compared between actively inflamed and non-inflamed bowel segments.

Results: A statistically significant difference was found regarding ADC values and bowel wall thickness measurements in the first group of patients when compared between active and remission phase MRI investigations (p=0.005). Bowel wall contrast enhancement degree did not differ between active and remission phases in the first group (p=0.059). In the second group, there was a statistically significant difference between active and normal bowel segments regarding ADC values, bowel wall thickness and contrast enhancement (p<0.001).

Conclusion: MR enterography and diffusion MRI may be beneficial to determine the degree of inflammation and response to treatment in IBD patients.

Aim: It was aimed to compare with healthy volunteers the changes in heat shock protein (HSP) 90 alpha and vitamin levels during exacerbation and remission periods in our patients with familial Mediterranean fever (FMF), to research whether these molecules will benefit in control of FMF exacerbation and prevention of amyloid accumulation, and also to investigate the relationship of these molecules with FMF gene mutations.

Patients and Methods: HSP 90 alpha, vitamin A, vitamin B 12, vitamin D, vitamin E and folic acid levels were studied in 30 patients with AAA diagnosed in our hospital's pediatric nephrology department during the exacerbation and remission periods and 30 healthy volunteers. The results were compared statistically.

Results: In our series, 19 (63.3%) of the patients were male, 11 (36.7%) were female, with a mean age of 11 years. The most common presenting complaint was abdominal pain (n=19; 63.3%) after fever and only high fever was found to be present in five (16.7%) patients. The most common genetic mutation was M694V (n=14; 46.7%), whereas no mutation was detected in five (16.7%) patients. Although HSP 90 alpha was the highest in the exacerbation group and the lowest in the remission group, there was no significant difference between the groups. Vitamin B12 was significantly different between the groups and the highest value was in the control group. Vitamin D was significantly different between the exacerbation and the remission groups and the highest value was in the exacerbation group. In addition, vitamin D levels were significantly higher in boys. There was no significant difference between vitamin A, vitamin E and folic acid levels. FMF gene mutations did not affect HSP 90 alpha and vitamin levels.

Conclusion: Our findings showed that serum HSP 90 alpha, vitamin D, vitamin E and folic acid levels were normal in AAA, but vitamin B12 and vitamin A levels were low during exacerbation and remission. However, we think that prospective studies including larger series should be performed about the subject.

\r\n Silent thyroiditis and Graves’ disease are autoimmune disorders which are common cause of thyrotoxicosis and should be given different treatments. Anamnesis and physical examination are not sufficient in the differential diagnosis of these two diseases, and advanced examinations such as thyroid scintigraphy, iodine reuptake study and thyroid stimulating receptor antibody could be needed. In this case, we present a patient diagnosed with a silent thyroiditis as a result of a thyrotoxicosis of a patient who was following up due to Graves' disease in remission and we aimed to emphasize the importance of differential diagnosis of these two diseases.

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Granulocytic sarcoma is extramedullary infiltration of leukemic cells and the involvement of the gastrointestinal tract is rare. These cases may present with abdominal pain, ileus, gastrointestinal bleeding and perforation. We present an acute myeloid leukemia patient that admitted with abdominal pain whose bone marrow is in remission after chemotherapy in whom the reason of abdominal pain was granulocytic sarcoma that occurred in the colon.