Mikofenolat mofetil ile remisyon sağlanan steroide dirençli otoimmün hemolitik anemi

Autoimmune hemolytic anemia (AIHA) is a disease that is seen in 1/10.000 people and characterized by forming antibodies against red blood cells and degradation of these red blood cells in reticuloendothelial system. Even autoimmune diseases can accompany, it is seen usually idiopathic. Paleness due to anemia, jaundice, tachycardia, darkening in urine color, hepatosplenomegaly are frequently seen clinical findings. Clinically normochrome and normocytic anemia, reticulocytosis, polychromasia in peripheral smear, spherocytes, indirect hyperbilirubinemia and increased lactate dehydrogenase are seen. Direct coombs test is the diagnostic test. Steroids are the first line drugs in treatment. The dosage and the treatment duration is formed according to patient's clinical situation. The treatment is checked by complete blood count, reticulocyte and Coombs test. Immunosuppressor treatments are given to patients that don't respond to treatment in 4-6 weeks or less patients who has recurrence at the time of decreasing the treatment dosage of corticosteroids. In few patient, that did not respond steroids, immunosuppressive treatments are used. Here, we present a patient, who is diagnosed with AIHA that we couldn't manage remission by steroid treatment, and no response to rituxumab as an immunosuppressor, but treated successfully with microphenolat mofetil

COVID-19 in pediatric patients undergoing chronic dialysis and kidney transplantation

The study aims to present the incidence of COVID-19 in pediatric patients undergoing renal replacement therapy (RRT) and to compare the severity and outcomes of the disease between the dialysis and kidney transplant (KTx) groups. This multicenter observational study was conducted between 1 April and 31 December 2020 in Istanbul. Members of the Istanbul branch of the Turkish Pediatric Nephrology Association were asked to report all confirmed cases of COVID-19 who were on RRT, as well as the number of prevalent RRT patients under the age of 20. A total of 46 confirmed cases of COVID-19 were reported from 12 centers, of which 17 were dialysis patients, and 29 were KTx recipients. Thus, the incidence rate of COVID-19 was 9.3% among dialysis patients and 9.2% among KTx recipients over a 9-month period in Istanbul. Twelve KTx recipients and three dialysis patients were asymptomatic (p = 0.12). Most of the symptomatic patients in both the dialysis and KTx groups had a mild respiratory illness. Only two patients, one in each group, experienced a severe disease course, and only one hemodialysis patient had a critical illness that required mechanical ventilation. In the entire cohort, one hemodialysis patient with multiple comorbidities died. Conclusion: While most cases are asymptomatic or have a mild disease course, pediatric patients undergoing dialysis and a kidney transplant are at increased risk for COVID-19. What is Known: In adult population, both dialysis patients and kidney transplant recipients are at increased risk for severe illness of COVID-19 and have higher mortality rate. Children with kidney transplantation are not at increased risk for COVID-19 and most have mild disease course. Data on children on dialysis are scarce. What is New: Pediatric patients undergoing dialysis and kidney transplantation have an increased risk for COVID-19. Most patients undergoing renal replacement therapy either on dialysis or transplanted develop asymptomatic or mild COVID-19 disease with a favorable outcome

Urinary catheterization and catheter-associated urinary tract infections in childhood

Amaç:Miksiyosistoüretrografi; vezikoüreteral reflü ve mesane anatomisini değerlendirmede kullanılmakta olan önemli bir tetkiktir. Miksiyosistoüretrografi çekimi sırasında kullanılan üriner kateterler idrar yolu enfeksiyonunu kolaylaştıran risk faktörlerindendir. Bu çalışmada üriner kateterizasyonun idrar yolu enfeksiyonu oluşumuna etkisinin değerlendirilmesi amaçlandı.Gereç ve yöntem: Çalışmaya Temmuz 2010-Temmuz 2012 ayları arasında Celal Bayar Üniversitesi Hafsa Sultan Hastanesi Çocuk Nefroloji polikliniğinde tekrarlayan idrar yolu enfeksiyonu nedeniyle izlenen ve miksiyosistoüretrografi çekilen 102 çocuk hasta (62 kız, 40 erkek) alındı. Tüm hastalardan miksiyosistoüretrografiden hemen önce ve iki gün sonra olmak üzere idrar kültürü alındı.Bulgular: Miksiyosistoüretrografiden hemen önce alınan idrar kültürlerinin %2,9' unda, miksiyosistoüretrografiden iki gün sonra alınan idrar kültürlerinin ise %11,7'sinde anlamlı bakteriüri saptandı. Katetere bağlı üriner sistem enfeksiyonu tanısı alan çocukların cinsiyeti arasında istatistiksel olarak anlamlı fark saptanmadı (p= 0.07). Hastalar 5 yaş üzeri ve 5 yaş ve altı olarak iki gruba ayrıldığında, yaş ve katetere bağlı üriner sistem enfeksiyonu arasında istatistiksel olarak anlamlı ilişki olduğu görüldü(p=0.01). Miksiyosistoüretrografi öncesi ve sonrası idrar kültürlerinde en sık izole edilen mikroorganizma Escherichia Coli'ydi.Sonuç: Mesane kateterizasyonunu gerektiren durumlarda temizlik kurallarına uyulması ve vezikoüreteral reflü tanısında hala altın standart olan miksiyosistoüretrografinin yerini alacak yeni tanı yöntemlerinin geliştirilmesi katetere bağlı üriner sistem enfeksiyonlarının önlenmesinde etkili olacaktırPurpose:Micturating cystourethrogram is an important technique used to visualize vesicoureteral reflux and the bladder's anatomy. The catheters that are used during micturating cystourethrogram procedures are one of the risk factors that directly cause urinary tract infections. The aim of this study was to evaluate the effect of urinary catheterization in urinary tract infections. Materials and methods:102 patients (62 boys, 40 girls) who were evaluated at Celal Bayar University Hafsa Sultan Hospital Pediatric Nephrology Polyclinic for recurrent urinary tract infections and who underwent micturating cystourethrogram procedures, between July 2010and 2012 were included in the study. Urine cultures were taken from all the patients before and two days after the micturating cystourethrogram procedure.Results: Of the urine cultures taken right before the micturating cystourethrogram procedures 2.9% and 11.7% of urine cultures taken 2 days after the procedures were positive for significant bacteriuria. Gender had no significant influence (p=0.07) on catheter-associated urinary tract infection. A significant relationship was observed between catheter-associated urinary tract infection and age when the patients were divided into two groups according to their age as under and above 5 years old (p=0,01). Escherichia Coli was the major pathogen that grew on the urine cultures taken both before and after the micturating cystourethrogram procedures.Conclusion: Using the proper sterilization procedures in situations which require urinary catheterization and developing new diagnostic procedures that can replace micturating cystourethrogram which is still the gold standard in vesicoureteral reflux diagnosis would be effective in preventing catheter-associated urinary tract infectio

Hipomagnezemik kalıtsal böbrek hastalıkları

Magnesium, a cofactor of many enzymes in human body, is involved in major cellular events such as energy metabolism, DNA transcription and protein synthesis. Abnormal levels of magnesium affect many organs in our body and can result in fatal complications. Therefore regulation of plasma magnesium concentration is vital. Plasma magnesium concentration can vary between 1.7 and 2.3 mg/dl. Magnesium homeostasis is regulated with bones, kidneys and bowels. 30-50% of dietary magnesium is absorbed from intestines. It is mainly absorbed in distal small intestine and colon. 95% of magnesium filtered by the kidneys is reabsorbed. 10-25% of filtered magnesium is reabsorbed from proximal tubules via passive paracellular transport. 70% of it is reabsorbed from the loop of henle via paracellular transport by means of positive voltage in lumen. The main storage of magnesium in the body is the bone tissue. In case of hypomagnesemia, osteoclastic activity rises in order to maintain normal magnesium levels. Hereditary hypomagnesemia is consisted of genetic diseases causing primary or secondary renal magnesium losses. These diseases can be evaluated in two groups according to the place of magnesium absorption. Familial hypomagnesemic, hypercalciuria, and nephrocalcinosis and Bartter Syndrome develop as a result of the malabsorption of magnesium in the thick ascending limb of Henle's loop. Hypomagnesemia with secondary hypocalcemia, isolated autosomal recessive hypomagnesemia, isolated autosomal dominant hypomagnesemia, Gitelman syndrome, HNF? nephropathy, East / Se Same Syndrome findings occur as a result of malabsorption of magnesium in distal convoluted tubule. This Article provides information for the diagnosis, clinical findings and the treatment of inherited diseases characterized by hypomagnesemia.İnsan vücudunda birçok enzimin kofaktörü olan magnezyum, enerji metabolizması, DNA transkripsiyonu, protein sentezi gibi major hücresel olaylara katılır. Magnezyum homeastazında barsaklar, kemik ve böbrekler rol alır. Diyetle alınan magnezyumun, yaklaşık %30-50' si barsaklardan emilir. Emilim başlıca distal ince barsak ve kolonda olur. Böbreklerden filtre olan magnezyumun da, %95'i geri reabsorbe olur. Magnezyumun %1025 pasif parasellüler transport ile proksimal tubülden reabsorbe olurken %70'i henlenin çıkan kolunda lümen pozitif voltaja bağlı ve parasellüler olarak gerçekleşir. Vücutta en büyük magnezyum deposu kemik doku ise, hipomagnezemi durumunda osteoklastik aktiviteyi arttırararak magnezyum dengesini sürdürmeye çalışır. Kalıtsal hipomagnezemi, primer ya da sekonder olarak renal magnezyum kaybına neden olan genetik hastalıklardan oluşur. Bu hastalıklar magnezyum emilimimin yapıldığı anatomik bölgeye göre iki grupta değerlendirilebilir. Henle kulbu kalın çıkan kolunda magnezyum emilimi bozukluğu sonucu familyal hipomagnezemik, hiperkalsiüri, nefrokalsinozis ve Bartter Sendromu gelişir. Distal kıvrımlı tübülde magnezyum emilim bozukluğu sonucunda ise, sekonder hipokalsemi ile birlikte hipomagnezemi, izole otozomal resesif hipomagnezemi, izole otozomal dominant hipomagnezemi, Gitelman Sendromu, HNF1? Nefropatisi, East/Se Same Sendromu bulguları oluşur. Bu yazıda hipomagnezemi ile seyreden kalıtsal hastalıkların, tanısı, klinik bulguları ve tedavisi hakkında bilgi verildi

Tek taraflı renal agenezi ve hipoplazili çocukların klinik özellikleri ve izlem sonuçları

Objective: Children with an inadequate number of nephrons have an increased risk of developing hypertension, proteinuria and chronic kidney disease in later life. This renal injury is caused by glomerular hyperfiltration. The aim of this study is to evaluate clinical characteristics and follow-up results of the children with unilateral renal agenesis and hypoplasia.Methods: We evaluated the clinical, radiological, and nuclear medicine findings of the children with unilateral agenetic or hypoplastic kidneys who were followed at Celal Bayar University School of Medicine and Dr. Behcet Uz Children's Hospital between 2005 and 2012.Results: The study consisted of 30 (60%) males and 20 (40%) females with a mean age of 8.9+/-4 years. A total of 25 patients were with renal agenesis and 25 with renal hypoplasia were diagnosed. On laboratory evaluation, mean serum creatinine level was 0.45+/-0.22 mg/dl and the mean estimated glomerular filtration rate (eGFR) was 144+/-30.3 ml/min. 29 patient (58%) had hyperfiltration according to an eGFR. Proteinuria and hypertension were noted in 1 (4%) patient. Urological anomalies were found in 15 patients (30%).Conclusion: Our study has determined that children with unilateral renal agenesis and hypoplasia had normal serum creatinine values. Howewer 29 patients (58%) had hyperfiltration according to eGFR. Because many children with hiperfiltration develop renal injury in later life, we emphasize the need for clinical follow-up in these patients starting at birth

Urine Pentraxin 3 Levels as A Marker for the Presence of Renal Parenchymal Scar

[No Abstract Available