Nutcracker syndrome in pediatrics: initial findings and long-term follow-up results.

BACKGROUND: Nutcracker syndrome (NCS) describes a set of symptoms and signs resulting from compression of the left renal vein (LRV). There is a lack of knowledge about its natural course, diagnosis, and management, especially in children. Herein, we present our single-center experience with a large number of patients who have long-term follow-up results. METHODS: All patients with NCS diagnosed between January 2011 and March 2021 were included and their data were obtained retrospectively. RESULTS: A total of 123 NCS patients (85 females) were included. The median age at the time of diagnosis was 12 (IQR 10-14) years, and BMI percentiles were below 5% in 38% of the cases. At the time of diagnosis, two-thirds of the patients were asymptomatic. The most common laboratory finding was nephritic proteinuria (98%), followed by microscopic hematuria (16%). Signs of LRV compression were significantly more evident in upright position Doppler ultrasonography (DUS) examination. All patients have been followed conservatively; hematuria and/or proteinuria resolved in 43 of the 108 patients (40%) within 35.8 ± 25.8 months of follow-up. Control DUS was performed in 52 patients after a mean period of 39.1 ± 21.3 months. The median peak velocity and diameter ratios of the LRV in the upright position were found to be decreased significantly when compared to the initial assessment (p < 0.05). Normal DUS findings were noted in 13 patients at the final evaluation. CONCLUSIONS: In unexplained proteinuria and/or hematuria, NCS should be considered, especially in asthenic adolescents. Our results support conservative management in children as the first-line treatment approach.

Hospitalizations after Renal Transplantation in Children: Risk Factors, Causes, and Outcomes.

INTRODUCTION: The aims of this study were to evaluate the frequency and causes of hospitalizations in the posttransplant period of children, investigate the risk factors, and evaluate the relationship between hospitalizations and renal prognosis in the long term. METHODS: We retrospectively reviewed the files of pediatric renal transplant patients, followed at least 6 months after kidney transplantation, in our center. Clinical information including age at transplantation, gender, primary disease, donor type, immuno-suppressive medication, hospitalization dates, and indications (infections and non-infectious) during follow-up period and graft outcomes was recorded. RESULTS: A total of 74 children (46 males) were followed up for a median of 54 months. Among them, 69 patients (93.2%) were hospitalized 446 times. The most common cause of hospitalizations was infections (314 times, 70%). Urinary tract infections were the most important cause followed by upper respiratory tract infections. Forty (54%) patients were hospitalized 132 times (29.5%) for non-infectious reasons. The most common non-infectious reason was nonspecific graft dysfunction (19 patients, 30 times), followed by rejection (17 patients, 27 times). Younger age, use of induction therapy, and having congenital anomalies of kidney and urinary tract (CAKUT) were found to be risk factors for increased hospitalization rates (p < 0.05). The number of hospitalizations was found to be negatively affecting the final glomerular filtration rate of transplant recipients (p: 0.04, r: -0.023). CONCLUSION: Patients with CAKUT, who received induction therapy, and small children were hospitalized more frequently after transplantation. Strategies to prevent hospitalizations will achieve a better graft prognosis.

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.

Is Office Blood Pressure Measurement Reliable in Obese Adolescents?

BACKGROUND: Although it is not reflected in the OBP measurement in obese children, ambulatory BP changes are known to occur. MH, non-dipper pattern and nocturnal hypertension have been reported to increase in obese children. On the other hand, the factors that indicate a high risk of hypertension are still unclear. The aim of our study is to especially detect masked hypertension by 24-hour BP measurement in obese adolescents and to evaluate the relationship of masked hypertension with metabolic syndrome parameters, anthropometric measurements and hepatosteatosis in these patients. METHODS: A total of 63 adolescents diagnosed with obesity were evaluated between January 2019 and December 2019. Office blood pressure was measured for all children, and all of them underwent ABPM. Patients with and without hypertension in ABPM were compared in terms of clinical and laboratory findings. RESULTS: The mean age was 14.0 ± 1.7 years, females composed 49.2% of the study population. Office blood pressure measurement revealed hypertension in 4 (6.3%) patients and prehypertension in 15 (23.8%) patients. Thirteen patients (20.9%) were diagnosed with masked hypertension, white coat hypertension was diagnosed in 3 (4.7%) patients. Abnormal ABPM patterns were found to be significantly more frequent in patients with severely obesity (with obesity: 26.4% vs severe obesity: 55.6%, p = 0.03) and patients with a higher waist circumference and waist circumference/height ratio. CONCLUSION: The prevalence of masked hypertension in obese adolescents has been found to be quite high. Therefore, we recommend ABPM in adolescents with high waist circumference/height ratio and severe obesity, even if their office blood pressure measurements are normal.

Effect of enuresis on perceived parental acceptance-rejection in children.

OBJECTIVE: Enuresis is a major problem affecting both the child and his family. This study aimed to investigate the effect of enuresis on mother acceptance-rejection perceived by children. MATERIAL AND METHODS: Fourty-six children and their mothers with the diagnosis of primary enuresis were included in the study. Parental Acceptance-Rejection Scale which consisted of 60 questions and consisted of four subscales: affection, hostility, neglect, and undifferentiated rejection was applied to both mothers and children. A dependent Sample t-test was used to compare the scale results of mothers and children, and an independent sample t-test was used to determine the factors affecting perceived high mother hostility in children. RESULTS: The mean age was 10.12±1.34 years and 58.70% of the children were boys. There was no statistically significant difference between total acceptance-rejection, affection, neglect, and undifferentiated rejection scores of mothers and children's perceptions. The perceived hostility score of the children (25.71±8.05) was higher than the mothers' hostility score (22.52±6.26) (p<0.05). The presence of maternal chronic disease was found to increase the perceived high hostility, while other factors were not statistically significant. Thirteen cases with chronic illnesses were excluded and re-analysis revealed that the difference between perceived and mother hostility persisted (p<0.05). CONCLUSION: Children with enuresis perceive their mother's behavior as more hostile than they are. It should be kept in mind that enuresis may affect the mother and child relationship, the family should be informed about the approach to the child.

Ambulatory blood pressure abnormalities in children with migraine.

BACKGROUND: Although there are data showing that the frequency of hypertension increases in adults with migraine, there has been no study on this subject in children. In this study, we aimed to evaluate the presence of hypertension in children with migraine by performing ambulatory blood pressure monitoring (ABPM). METHODS: Thirty-seven children diagnosed with migraine and 30 healthy controls were evaluated between January 2015 and March 2016. Demographic data, clinical and laboratory features, and physical examination findings were recorded for both groups. Office blood pressure was measured for all children, and each also underwent ABPM. The two groups were compared in terms of ambulatory blood pressure parameters. RESULTS: The mean age was 13.3 and 13.1 years and the proportion of females was 73% and 60% in the migraine and control groups, respectively. Although the frequency of hypertension was not higher, abnormal ABPM patterns were found to be significantly more frequent in the migraine group (migraine, 45.9%; control, 16.7%; p, 0.018). Nighttime mean arterial blood pressure, nighttime diastolic blood pressure, and non-dipping pattern were higher in children with migraine than those in the control group (p < 0.05). CONCLUSIONS: These results suggest that ambulatory blood pressure abnormalities may be present in almost half of patients with migraine. Therefore, we suggest that ABPM should be performed even if the office blood pressure measurements of children diagnosed with migraine are normal.

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.

Celiac disease and hematological abnormalities in children with recurrent aphthous stomatitis.

BACKGROUND: Recurrent aphthous stomatitis (RAS) is one of the most common diseases of the oral mucosa and may be related to vitamin deficiencies or systemic diseases such as celiac disease (CD). The aim of this study was to investigate the frequency of hematinic deficiency and CD in children with RAS. METHODS: The medical records of patients diagnosed with RAS were reviewed for the presence of hematinic deficiencies (hemoglobin, mean corpuscular volume, ferritin, vitamin B12 , folic acid), and CD. The study group included 108 children with RAS and 57 healthy children who were evaluated for hematological abnormalities in routine evaluation. RESULTS: The frequency of a family history of RAS was significantly higher in the RAS group compared to the control group (34.2% vs 7%, respectively; P < 0.001). A hematological abnormality was detected in 32.4% of the RAS group and 10.5% of the control group (P = 0.02). The prevalence of iron deficiency anemia was significantly higher in the RAS group (P = 0.037). Three (2.7%) patients with RAS were diagnosed with CD, which is a significantly higher frequency than that observed in healthy children in Turkey (P < 0.01; OR 6.03, 95% CI [2.37, 4.56]). These children had mild malnutrition, iron deficiency, and iron deficiency anemia. CONCLUSIONS: Children with RAS should be evaluated for nutritional status and hematological indices, and in the case of hematological abnormalities and malnutrition screening for CD should be considered.

Mild hypotonia and recurrent seizures in an 8-month-old boy: Questions.

Hypomagnesemia with secondary hypocalcemia is a rare autosomal recessive disorder which manifests in early infancy with generalized seizures, other symptoms of neuromuscular irritability, and growth disturbances. Homozygous mutations in the magnesium transporter gene, transient receptor potential melastatin 6 (TRPM6), cause the disease. Here, we present an 8-month-old Turkish boy with a novel mutation of TRPM6. The patient, son of first-degree cousins, was hospitalized because of recurrent seizures and mild hypotonia. He had seizures since the newborn period and he had been treated with phenobarbital but there was no favorable response to therapy. His past history also revealed hypocalcemia detected on the newborn period but serum magnesium levels were not studied at that time. During hospitalization, we detected hypocalcemia, hypomagnesemia, and normal parathormone levels. Abdominal ultrasound was normal. Magnesium excretion was slightly increased. Considering the consanguinity of the parents and clinical features of the patients, genetic testing of the TRPM6 gene was performed and a novel homozygous mutation was detected as c.3178A>T. He was started on magnesium and calcium supplementation and he is symptom-free for 1 year. We would like to call attention to the measurement of serum magnesium levels in children with hypocalcemic convulsions. Early and appropriate treatment with magnesium supplementation is crucial.

Atypical Hemolytic Uremic Syndrome in Children Aged <2 Years.

BACKGROUND: There are limited data on infants with atypical hemolytic uremic syndrome (aHUS). The aim of this study was to determine the clinical and laboratory features, and to evaluate treatment modalities and outcomes in infants with aHUS. MATERIALS AND METHODS: Relevant data on patients with onset of aHUS at age <2 years were obtained from the Turkish Pediatric aHUS Registry. RESULTS: Among the 146 patients included in the Registry, 53 (36%) (23 male and 30 female) were enrolled for the study. Age at disease onset was ≤1 year in 29 of the patients. In all, 21 (40%) of the patients developed neurological symptoms. Disease-causing mutations were noted in 14 (36%) of the 39 patients in which genetic analysis was performed. Plasma therapy was performed in 42 (79%) patients; eculizumab therapy was administered to treat the first episode of aHUS in 33 (62%) patients and in 5 patients as the first- line therapy. In total, 38 (72%) patients received renal replacement therapy (RRT), 3 (6%) died due to acute illness, and 4 (8%) were discharged from hospital with RRT. Follow-up visit data were available for 46 patients and the median duration was 23 months (range 3-129 months). End-stage renal disease developed only in 1 patient. Proteinuria and hypertension persisted in 17 (37%) and 20 patients (44%) respectively. Eculizumab treatment was continued in 25 of the 39 patients during the follow-up period. CONCLUSION: One-third of the aHUS patients had disease onset during infancy. The prognosis of this life-threatening disease seems to get better with improved treatment modalities.

Proteinuria in pediatric renal transplant recipients.

Proteinuria has been shown to be an important and potentially treatable risk factor for graft loss. The aim of this study was to evaluate prevalence, etiology, and outcome of proteinuria during the follow-up of children with renal transplantation. We retrospectively reviewed the files of renal transplanted children between 2006 and 2016 in our center. All patients were interpreted with respect to the demographic data and clinical and laboratory features including information about proteinuria. Chi-square test and Mann-Whitney U test were used for analysis. Fifty-two children were eligible for the study. Proteinuria was observed in 34 (65%) and nephrotic range proteinuria was detected in 5 (9.6%) patients. Etiology of proteinuria could be identified in 21 patients. Acute rejection and uncontrolled hypertension were the most frequent causes of proteinuria. Proteinuria had resolved during the follow-up in 59% of the patients. We found that children with and without proteinuria had similar glomerular filtration rate at the end of 50 months of follow-up period. Proteinuria seems to be a common complication in renal transplant recipients. Graft functions can be preserved by immediate evaluation of increasing proteinuria, and by fixing treatable causes rapidly and efficiently during the follow-up in majority of the patients.

Hypertension and improved left ventricular mass index in children after renal transplantation.

This study was conducted to evaluate the changes in BP and LVH after the transplantation and to evaluate the effect of BP changes in LVH. Forty-three pediatric renal transplant patients, with a mean age of 16.99 ± 3.88 years, were enrolled in this study. Twenty-three (53.5%) of the patients were male. Medical records for pretransplantation period (closest to the time of transplantation) and for post-transplantation period (9-12 months after transplantation) were reviewed. All the patients had BP measurements and echocardiographic evaluation in pre- and post-transplantation period. Hypertension was defined as an average systolic and/or diastolic BP that is ≥95th percentile for sex, age, and height. Although the number of patients with hypertension increased from 30 (69.76%) to 35 (81.4%), the number of patients with LVH decreased from 19 (44.1%) to 9 (20.9%) after the transplantation. Although the only significant difference in BP measurements was between the mean Z scores of 24 hour and nighttime mean DBP before and after the transplantation; the mean LVMI, and the prevalence of LVH was significantly lower after the transplantation. There was no significant correlation between the LVMI and the BP measurements. Even though hypertension may persist, there is significant improvement in LVH after renal transplantation.

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.

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

UNLABELLED: 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.

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.

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.

Vesicoureteral Reflux and Renal Scarring Risk in Children after the First Febrile Urinary Tract Infection.

BACKGROUND/AIMS: There are many controversies regarding the best approach for evaluating children after their first febrile urinary tract infection (UTI). The aim of this study was to define the clinical, laboratory, and radiological features of patients with their first febrile UTI and to investigate the factors that might predict the presence of vesicoureteral reflux (VUR) and renal scarring. METHODS: The files of patients who were followed due to their first febrile UTI between 2008 and 2013 were retrospectively reviewed (n = 300). Patients were divided into groups based on their age, the resistance state of microorganisms, the presence of VUR, and scarring on Tc99m dimercaptosuccinic acid scintigraphy. The chi-square test and Mann-Whitney U test were used for analysis. RESULTS: The median age at the first febrile UTI was 11 months and girls constituted 77% of the patient population. VUR and renal scarring were detected in 30.9 and 19.4% of the patients, respectively. C-reactive protein levels and the presence of renal scarring were significantly higher in patients with VUR (p < 0.05). Abnormal ultrasonography findings, VUR and recurrent UTIs were significantly higher in patients with renal scars (p < 0.001). In multivariate analysis, we did not detect any factor that might predict the presence of VUR and renal scarring. CONCLUSION: A majority of children had their first febrile UTI at a young age. Although we could not find any factor that might predict the VUR and scar risk in patients with their first febrile UTI, an abnormal renal scan at 6 months after infection was closely related with the presence of VUR and recurrent UTIs.

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.

Two children with steroid-responsive nephrotic syndrome complicated by cerebral venous sinus thrombosis / Dos niños con síndrome nefrótico sensible a corticoides complicado por trombosis de seno venoso cerebral

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