Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement

ABSTRACT Pauci-immune glomerulonephritis (GN) is more common in elderly people compared to children and the etiology is not completely understood yet. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. We report a case of a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci-immune crescentic glomerulonephritis with crescents in 20 of 25 glomeruli (80%). Of these 20 crescents, 12 were cellular, 4 fibrocellular, and 4 globally sclerotic. She did not have purpura, arthritis, or systemic symptoms and she responded well to initial immunosuppressive treatment despite relatively severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30 mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2 mg/kg per day) orally for 3 months with prednisone (1 mg/kg per day). In one month, remission was achieved with normal serum creatinine and prednisone was gradually tapered. The case of this child with a relatively rare pediatric disease emphasizes the importance of early and aggressive immunosuppressive treatment in patients with renal-limited ANCA-associated pauci-immune crescentic GN even if with a mild clinical presentation. As in our patient, clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is mandatory in such children to guide the clinical management and predict prognosis

Laparoscopic cholecystectomy results in patients with different age groups

Amaç Laparoskopik kolesistektomi benign safra kesesi hastalıklarında en çok tercih edilen metod olmuştur. İleri yaş ,mortalite ve morbiditenin artmasına neden olur. Bu çalışmanın amacı farklı yaşlardaki hastalarda laparoskopik kolesistektominin sonuçlarını karşılaştırmakır (yaş yaş). Gereç ve Yöntemler Kasım 2001-Mayıs 2009 tarihleri arasında OMÜ Tıp Fakültesi ve Mustafa Kemal Üniversitesi Tıp Fakültesinde benign safra kesesi hastalığı nedeniyle laparoskopik kolesistektomi uygulanan toplam 511 hasta retrospektif olarak analize edildi. Hastalar yaşlarına göre 3 gruba bölündü. Grup A (,n=96). Bulgular Tüm gruplarda laparoskopik kolesistektomi için en sık endikasyon semtomatik kolelitiazisdir. Ko-morbit hastalılar Grup C’de, Grup A ve B’ye göre anlamlı olarak daha yüksekti.İnsidental bilier patolojiler ve abdominal operasyon öyküsü tüm gruplarda benzer şekildeydi. Bununla birlikte bilier kanal ve sistik arter anomalileri Grup A’da,Grup B ve C’ ye göre anlamlı olarak yüksek bulundu. Bu çalışmada 26 hastada (%5.08) açık kolesistektomiye dönmek gerekti. Açığa dönüşün en büyük nedeni Callot üçgeni zor diseksiyonu idi( 21 hastada ;%80). Gruplar arasında morbidite açısından farklılık yoktu. Sonuç Sonuç olarak, genç hastalarda safra yolu anomalilerinin ve yaşlı hastalarda ko-morbid bozuklukların daha yaygın olduğu düşünülmelidir. Bu faktörler hastanın preoperatif iyi değerlendirilmesi sonucu etkilemez. Bununla birlikte cerrah bu durumun farkında olmalı ve intraoperatif ve postoperatif komplikasyonlar nedeniyle dikkatli olmalıdır.Aim Laparoscopic cholecystectomy (LC) has been most preferable method for benign gallbladder disease. Advanced age may be increased morbidity and mortality. The aim of this study was to compare the results of LC in patients according to different ages (age ≤ 30, 31 – 64 years, and age ≥ 65). Material and Methods A retrospective analysis was performed including overall 511 patients who underwent LC for benign disease of gallbladder at Ondokuzmayis University Medical Faculty and Mustafa Kemal University, Medical Faculty between November 2001 and November 2009. The patients are divided into three groups according to ages: Group A (age ≤ 30 years, n = 47), Group B (age = 31 – 64 years, n = 368), Group C (age ≥ 65, n = 96). Results Symptomatic cholelithiasis was the most common indication for LC in all the groups (p > 0.05). Co-morbid diseases were significantly higher in the Group C (≥ 65 years) than in the Group A and B (≤ 30 years, 31 - 64 years) (p=0.001). Co-incidental biliary pathologies, and history of abdominal operation were similar in all the groups. However biliary duct and cystic artery anomalies were significantly more common in the Group A than in the Group B and C (p=0.001). Conversion to OC was required in 26 (5.08 %) patients in this study. The major reason for the (21 cases, 80.76 %) was difficult dissection of the Calot’s triangle. There was no difference in morbidity among the groups (p>0.05). Conclusions As a conclusion, it’s thought that biliary anomalies in young patients and co-morbid diseases disorders in elderly patients are more common. These factors do not affect the results of patient that preoperatively well evaluated. However surgeon should be aware of this condition and be careful for intraoperative and postoperative complication

Renal outcome with eculizumab in two diarrhea-associated hemolyticeuremic syndrome cases with severe neurologic involvement

İstanbul Bilim Üniversitesi, Tıp Fakültesi.The kidney and brain are the two target organs in patients with Shiga toxin-producing Escherichia coli-associated hemolytic–uremic syndrome (STEC-HUS). Activation of the alternative complement pathway occurs in patients with STEC-HUS. A monoclonal antibody (eculizumab) directed against C5 has been reported to be effective against severe neurologic involvement in patients with STEC-HUS. We report on two STEC-HUS children with severe neurologic involvement treated with eculizumab. Despite prompt resolution of initial neurologic findings upon treatment with eculizumab, proteinuria and hypertension persisted in these patients. The persistence of these two risk factors is particularly emphasized to discuss the long-term effects of eculizumab, especially on renal involvement

GENETICALLY INTERESTING FAMILY WITH NEPHROTIC SYNDROME IN THREE SIBLINGS

WOS: 00044399840035

Cryptosporidiosis: A rare and severe infection in a pediatric renal transplant recipient

Acikgoz Y, Ozkaya O, Bek K, Genc G, Sensoy SG, Hokelek M. Cryptosporidiosis: A rare and severe infection in a pediatric renal transplant recipient. Abstract: Cryptosporidium is an intracellular protozoan parasite that causes gastroenteritis in human. In immunocompromised individuals, cryptosporidium causes far more serious disease. There is no effective specific therapy for cryptosporidiosis, and spontaneous recovery is the rule in healthy individuals. However, immunocompromised patients need effective and prolonged therapy. Here, we present our clinical experience in a six-yr-old boy who underwent living-related donor renal transplantation and who was infected with Cryptosporidium spp. Our patient was successfully treated with antimicrobial agents consisting of spiramycin, nitazoxanide, and paromomycin. At the end of second week of therapy, his stool became negative for Cryptosporidium spp. antigen and spiramycin was discontinued. Nitazoxanide and paromomycin treatment was extended to four wk. With this case, we want to emphasize that cryptosporidiosis should be considered in the differential diagnosis of severe or persistent diarrhea in solid organ transplant recipients where rigorous antimicrobial therapy is needed

AUTOSOMAL-DOMINANT POLYCYSTIC KIDNEY DISEASE IN CHILDREN: PRELIMINARY RESULTS OF A TURKISH NATIONAL, MULTICENTER STUDY

WOS: 000443998400522