Long Term Follow-Up Of Patients With Nonrefluxing Hydronephrosis.

BACKGOUND: The aim of this study is to examine the long-term prognosis of children with ureteropelvic junction obstruction-like hydronephrosis (UPJO-like HN). PATIENTS AND METHODS: The files of children with hydronephrosis (HN) were analyzed retrospectively. Patients with vesicoureteral reflux (VUR) and other genitourinary anomalies were excluded. The final status of the HN, the need for surgery, and urinary tract infection (UTI) frequency were evaluated. RESULTS: The study included 219 patients with 302 renal units (RU) with HN. Surgery rate was higher in RUs with larger kidney size and parenchymal thinning (p:<0.001 for both). Hydronephrosis resolved in 113 (40.2%) RUs, improved in 66 (23.3%), unchanged in 100 (35.5%) and worsened in 4 (1.4%). The frequency of recovery and improvement was found to be less in RUs with severe HN, large kidney size, and thin parenchyma. The UTI frequency was higher in severe HN group (12.2% vs 30.6% p:<0.001). CONCLUSIONS: Children with mild HN had an excellent prognosis. Although the majority of the patients with high-grade HN had also a good prognosis, it seems important to closely follow up patients with severe HN, increased kidney size, and accompanying parenchymal thinning. Clinicians should be aware of the increased frequency of UTIs in children with severe HN.

Características clínicas y evolución del reflujo vesicoureteral en la infancia / Clinical characteristics and outcome of childhood vesicoureteral reflux

Introducción. El objetivo fue evaluar las características clínicas y la evolución del reflujo vesicoureteral (RVU) según el sexo y grado de RVU.Población y métodos. Se incluyeron pacientes con RVU vistos durante el seguimiento de rutina entre enero de 2014 y enero de 2015. Se registraron las características demográficas, la evolución, los laboratorios y las imágenes.Resultados. Se seleccionó a 220 pacientes, cuya media de edad del diagnóstico era 3,17 ± 3,08 años; en ese momento, los varones eran menores que las niñas (2,00 ± 2,59 vs. 3,81 ± 3,15, p < 0,001). La infección urinaria fue la presentación más frecuente, seguida de hidronefrosis prenatal (HNP). El 22 % de los pacientes tuvo reflujo de grado 1-2; el 51 %, de grado 3; y el 27 %, de grado 4-5. En el reflujo de grado 4-5, las ecografías y gammagrafías con ácido dimercaptosuccínico (DMSA) marcado con 99mTc presentaron más anomalías, y se realizaron más cirugías (p < 0,001). En los varones, fueron más comunes el reflujo de grado 4-5 (43,6 % vs. 18,3 %) y las anomalías ecográficas (77 % vs. 54 %) y en la DMSA (77 % vs. 59 %) (p < 0,05). En las niñas, hubo mayores tasas de infección urinaria, disfunción de las vías urinarias inferiores y resolución espontánea (p < 0,05).Conclusiones. A pesar de la menor edad al momento del diagnóstico, la resolución espontánea fue menor en los varones, y estos presentaron HNP, reflujo grave y anomalías radiológicas más frecuentemente.

Introduction. The aim of the study was to assess the clinical features and outcome parameters of children with vesicoureteral reflux (VUR) based on gender and VUR grade.Population and methods. Patients with VUR who were seen during routine follow-up visits at Ankara University Children's Hospital between January 2014-January 2015 were included in this retrospective study. Patient demographics, clinical course, laboratory investigations, imaging were noted.Results. Two hundred and twenty patients were recruited. Mean age at the time of diagnosis was 3,17 ± 3,08 years. Boys were diagnosed at younger ages as compared to girls (2.00 ± 2,59 vs. 3,81 ± 3.15, p < 0.001). Urinary tract infection (UTI) was the most common presentation. The second presentation form was antenatal hydronephrosis (AHN) which was more common in males (25.6 %, p < 0.001). Twenty-two percent of the patients had grade 1-2, 51 % grade 3 and 27 % grade 4-5 reflux. Patients with grade 4-5 reflux had more abnormal ultrasound (US) and Tech 99m dimercaptosuccinic acid scintigraphy (DMSA) findings and surgery was performed more frequently in this group (p < 0.001). In males, grade 4-5 reflux (43.6 % vs. 18.3 %), abnormal US (77 % vs. 54 %) and DMSA (77 % vs. 59 %) findings were more frequent (p < 0.05). In girls higher rates of UTIs, lower urinary tract dysfunction (LUTD) and spontaneous reflux resolution were seen (p < 0.05).Conclusions: Despite younger age at diagnosis, spontaneous resolution was found lower in boys and they had more frequent AHN, more severe reflux, and radiological abnormalities.

Clinical characteristics and outcome of childhood vesicoureteral reflux. / Características clínicas y evolución del reflujo vesicoureteral en la infancia.

INTRODUCTION: The aim of the study was to assess the clinical features and outcome parameters of children with vesicoureteral reflux (VUR) based on gender and VUR grade. POPULATION AND METHODS: Patients with VUR who were seen during routine follow-up visits at Ankara University Children's Hospital between January 2014-January 2015 were included in this retrospective study. Patient demographics, clinical course, laboratory investigations, imaging were noted. RESULTS: Two hundred and twenty patients were recruited. Mean age at the time of diagnosis was 3,17 ± 3,08 years. Boys were diagnosed at younger ages as compared to girls (2.00 ± 2,59 vs. 3,81 ± 3.15, p < 0.001). Urinary tract infection (UTI) was the most common presentation. The second presentation form was antenatal hydronephrosis (AHN) which was more common in males (25.6 %, p < 0.001). Twenty-two percent of the patients had grade 1-2, 51 % grade 3 and 27 % grade 4-5 reflux. Patients with grade 4-5 reflux had more abnormal ultrasound (US) and Tech 99m dimercaptosuccinic acid scintigraphy (DMSA) findings and surgery was performed more frequently in this group (p < 0.001). In males, grade 4-5 reflux (43.6 % vs. 18.3 %), abnormal US (77 % vs. 54 %) and DMSA (77 % vs. 59 %) findings were more frequent (p < 0.05). In girls higher rates of UTIs, lower urinary tract dysfunction (LUTD) and spontaneous reflux resolution were seen (p < 0.05). CONCLUSIONS: Despite younger age at diagnosis, spontaneous resolution was found lower in boys and they had more frequent AHN, more severe reflux, and radiological abnormalities.

Introducción: El objetivo fue evaluar las características clínicas y la evolución del reflujo vesicoureteral (RVU) según el sexo y grado de RVU. Población y métodos: Se incluyeron pacientes con RVU vistos durante el seguimiento de rutina entre enero de 2014 y enero de 2015. Se registraron las características demográficas, la evolución, los laboratorios y las imágenes. Resultados: Se seleccionó a 220 pacientes, cuya media de edad del diagnóstico era 3,17 ± 3,08 años; en ese momento, los varones eran menores que las niñas (2,00 ± 2,59 vs. 3,81 ± 3,15, p < 0,001). La infección urinaria fue la presentación más frecuente, seguida de hidronefrosis prenatal (HNP). El 22 % de los pacientes tuvo reflujo de grado 1-2; el 51 %, de grado 3; y el 27 %, de grado 4-5. En el reflujo de grado 4-5, las ecografías y gammagrafías con ácido dimercaptosuccínico (DMSA) marcado con 99mTc presentaron más anomalías, y se realizaron más cirugías (p < 0,001). En los varones, fueron más comunes el reflujo de grado 4-5 (43,6 % vs. 18,3 %) y las anomalías ecográficas (77 % vs. 54 %) y en la DMSA (77 % vs. 59 %) (p < 0,05). En las niñas, hubo mayores tasas de infección urinaria, disfunción de las vías urinarias inferiores y resolución espontánea (p < 0,05). Conclusiones: A pesar de la menor edad al momento del diagnóstico, la resolución espontánea fue menor en los varones, y estos presentaron HNP, reflujo grave y anomalías radiológicas más frecuentemente.

Campylobacter jejuni: un agente infrecuente de peritonitis en un niño con diálisis peritoneal / Campylobacter jejuni: A rare agent in a child with peritoneal dialysis-related peritonitis

La peritonitis es un problema grave en los niños que reciben diálisis peritoneal. La bacteria Campylobacter jejuni es una causa infrecuente de peritonitis. Un niño de 10 años de edad con insuficiencia renal terminal causada por síndrome urémico hemolítico atípico ingresó a nuestro hospital con dolor abdominal y fiebre. El líquido de la diálisis peritoneal era turbio; en el examen microscópico se observaron leucocitos abundantes. Se inició tratamiento con cefepime intraperitoneal. En el cultivo del líquido peritoneal se aisló Campylobacter jejuni, por lo que se agregó claritromicina oral al tratamiento. Al finalizar el tratamiento, el resultado del cultivo del líquido peritoneal era negativo. Hasta donde sabemos, no se había informado previamente peritonitis por C. jejuni en niños. Conclusión. Si bien la peritonitis por C. jejuni es rara en los niños, debe considerarse como factor etiológico de la peritonitis.

Peritonitis is a serious problem in children receiving peritoneal dialysis. Campylobacter jejuni is an unusual cause of peritonitis. A 10-year-old boy who had end stage renal failure due to atypical hemolytic uremic syndrome was admitted to our hospital with abdominal pain and fever. Peritoneal dialysis fluid was cloudy and microscopic examination showed abundant leukocytes. Intraperitoneal cefepime treatment was started. Campylobacter jejuni was isolated from peritoneal dialysis fluid culture and oral clarithromycin was added to the treatment. At the end of therapy, peritoneal fluid culture was negative. To our knowledge, C. jejuni peritonitis was not reported in children previously. Conclusion : Although C. jejuni peritonitis is rarely encountered in children, it should be considered as an etiologic factor for peritonitis.

Anti-VEGF-related thrombotic microangiopathy in a child presenting with nephrotic syndrome.

BACKGROUND: Targeting the vascular endothelial growth factor (VEGF) signaling pathway has become an important approach to current cancer therapy. Anti-VEGF therapy-related renal adverse effects may present as hypertension, non-nephrotic proteinuria, and rarely as nephrotic syndrome (NS) and acute kidney injury. CASE-DIAGNOSIS/TREATMENT: In this report, we present a 15-year-old boy who had developed nephrotic syndrome and thrombotic microangiopathy 26 months after administration of anti-VEGF therapy. Treatment was discontinued and nephrotic syndrome remitted spontaneously within 3 months. CONCLUSIONS: Nephrologists should be aware of the side effects of anti-VEGF therapy. Early diagnosis and prompt management with withdrawal of the agents will result in spontaneous remission.

Anti-IL-1 treatment in familial Mediterranean fever and related amyloidosis.

Colchicine is the standard treatment in familial Mediterranean fever (FMF) patients. New treatment strategies are needed in FMF patients who were unresponsive to colchicine therapy or who had developed amyloidosis. The aim of this study was to present clinical-laboratory features and treatment responses of pediatric FMF patients that were treated with anti-IL-1 therapies. Files of patients who had been followed in our department with diagnosis of FMF were retrospectively evaluated. Patients that have been receiving anti-IL-1 therapies (anakinra or canakinumab) were included to the study. All patients were interpreted with respect to the demographic data, clinical and laboratory features of the disease, genetic analysis of MEFV mutations and treatment responses. Among 330 currently registered FMF patients, 13 patients were included to the study. Seven of them received anti-IL-1 therapy due to colchicine resistance and 6 due to FMF-related amyloidosis (1 of them with nephrotic syndrome, 2 with chronic kidney disease, 3 with renal transplantation). In all treated patients, attacks completely disappeared or decreased in frequency; partial remission occured in nephrotic syndrome patient; and their life quality improved. Anti-IL-1 therapies can be successfully used in colchicine-resistant FMF patients and patients with amyloidosis during childhood and adolescent period without major side effects.

De novo amyloidosis in a renal transplant patient.

Systemic AA amyloidosis is a serious complication of many chronic inflammatory disorders and chronic infections. Renal involvement is seen in the majority of the patients and can lead to end-stage renal disease. Renal transplantation can be performed in these patients; however, amyloidosis can recur in the transplanted kidneys. On the other hand, de novo AA amyloidosis in renal transplant patients has been rarely reported. We report a 17-yr-old patient with end-stage renal disease due to genitourinary anomalies who developed recurrent pyelonephritis after transplantation. Three yr after transplantation, renal biopsy was performed for proteinuria and AA amyloidosis was identified in the renal allograft. Although rare, chronic infections might cause de novo amyloidosis in renal transplant patients. Therefore, amyloidosis should be kept in mind in those types of patients who present with proteinuria.