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Background: Therapeutic apheresis (TA) is already used to treat various diseases in the field of nephrology. The aim of this study was to evaluate the frequency and types of complications that occur during TA in children with kidney disease. Methods: Records of children (≤ 18 years) who underwent TA between 2007 and 2022 were retrospectively reviewed. Children with missing data and those with a diagnosis of nonnephrological disease were excluded. Results: A total of 1214 TA sessions, including 1147 therapeutic plasma exchange (TPE) sessions and 67 immunoadsorption (IA) sessions, were performed on the 108 patients enrolled in the study. Forty-seven percent of the patients were male, and the mean age was 12.22 ± 4.47 years. Posttransplant antibody-mediated rejection (64.8%) and hemolytic uremic syndrome (14.8%) were the most common diagnoses indicating TA. Overall, 17 different complications occurred in 58 sessions (4.8%), and 53 sessions (4.6%) were not completed because of these complications. The distribution of complications among the patients was as follows: 41.4% had technical complications, 25.9% had allergic complications, and 32.7% had others. The most common technical complication was insufficient flow (37.5%). The incidence of complications was greater in patients aged 3–6 years than in patients in the other age groups (p = 0.031). The primary disease, type of vascular access, and rate of fresh frozen plasma/albumin use were similar between patients with and without complications (p values of 0.359 and 0.125 and 0.118, respectively). Conclusions: Our study showed that complications occurred in only 4.8% of TA sessions. The most common complication was technical problems. [ABSTRACT FROM AUTHOR]

Objectives: This study aims to evaluate demographic, clinical, and radiological characteristics of Turkish children with chronic non-bacterial osteomyelitis. Patients and methods: Between January 2008 and December 2018, a total of 28 patients (10 males, 18 females; median age: 12.5 years; range, 4.5 to 21 years) who were diagnosed with chronic non-bacterial osteomyelitis in three pediatric rheumatology centers were retrospectively analyzed. The demographic, clinical, and laboratory findings of the patients were recorded. Results: The median age at the time of diagnosis was 10.2 years. The median time from symptom onset to diagnosis was 6.5 months. The median follow-up was 18.5 months. The most frequent symptom at onset was arthralgia (75.0%). The most frequently involved bone was the femur (67.9%). Eight (63.6%) of 12 patients had at least one Mediterranean fever gene (MEFV) mutation, indicating a significantly higher prevalence than the Turkish population (14.8%). Five of these patients fulfilled the diagnostic criteria for familial Mediterranean fever (FMF). All patients received non-steroidal anti-inflammatory drugs. Other drugs were methotrexate (46.4%), sulfasalazine (39%), corticosteroids (25%), anti-tumor necrosis factor (anti-TNF) agents (32%), pamidronate (25%), and colchicine (21.4%). Six of eight patients with MEFV mutations were administered with colchicine, and all of them responded to treatment. Conclusion: Clinical evolution and imaging investigations should be carefully performed to prevent any delay in the diagnosis of patients with chronic non-bacterial osteomyelitis. Based on our study results, FMF coexistence is worth investigating in patients with chronic non-bacterial osteomyelitis, particularly in the Turkish population. [ABSTRACT FROM AUTHOR]

The article provides answers to questions concerning proteinuria in a male adolescent with hearing loss.

The article presents answers to a clinical quiz about severe anemia and massive proteinuria in a boy with diabetes mellitus.

Objectives: This study aims to evaluate the association between vitamin D deficiency and disease activity in children with juvenile systemic lupus erythematosus (SLE). Patients and methods: A total of 16 children (13 girls and 3 boys; mean age 14.47 years; range 5-18) with SLE who were under followup in our clinic were included in this study. The medical records of the children along with the demographic data, disease duration, treatments received, disease activity, and 25-hydroxyvitamin D [25-(OH)D] levels were evaluated. Systemic lupus erythematosus disease activity (SLEDAI) scores were calculated on the day the serum samples were drawn. Results: The median follow-up period was 30 (range 9 to 55) months. The mean serum vitamin D level was 22.04±16.75 ng/ml. Vitamin D deficiency (serum vitamin D level of <15 ng/ml) was found in half of the patients. Six of them had significantly lower vitamin D levels (≤10 ng/ml). A significant negative correlation between the serum concentrations of vitamin D and the SLEDAI scores was found (r= -0.711, p=0.002). Conclusion: Our study results show that there is a strong negative association between disease activity and vitamin D deficiency in children with SLE. The vitamin D levels of these patients should be evaluated on a regular basis, and vitamin D supplements should be given, if necessary. [ABSTRACT FROM AUTHOR]

The aim of this study was to investigate the prevalence of vitamin D deficiency and factors associated with vitamin D levels in children with juvenile idiopathic arthritis (JIA). The children with JIA who had an outpatient visit between March 2011 and October 2011 were evaluated retrospectively. The patients were divided into three groups according to the 25(OH)D level as: Group 1: >20 ng/ml, Group 2: 15-20 ng/ml, and Group 3: <15 ng/ml. A total of 76 patients, 45 (59.2%) girls with a mean age of 10.0±4.2 years and median follow-up of 12 months (3-110), were included in the study. The mean serum vitamin D level was 21.6±12.7 ng/ml. Vitamin D insufficiency (15-20 ng/ ml) and deficiency (<15 ng/ml) were found in 13 (17.1%) and 28 (36.8%) patients, respectively. Serum alkaline phosphatase (ALP) and parathyroid hormone (PTH) levels were high in 23 (30.2%) and 16 (22.2%) patients, respectively. Vitamin D deficiency is common in children with JIA. 25(OH)D levels should be monitored, and vitamin D supplementation should be given. [ABSTRACT FROM AUTHOR]

Introduction: Micromethod erythrocyte sedimentation rate (MESR) correlates classic Westergren method in newborn. In literature, MESR or microsedimentation rate has been studied especially newborn infections. Some of them showed that this test is more valuable in the sepsis screening and predicting the presence of infection in neonates .To investigate diagnostic evaluation of MESR in infections as its fast, easy, economic method and the relation between other acute phase reactant parameters. Material and Methods: A total 31 patient and 24 healthy children were admitted in this study in Suleyman Demirel University Medical Faculty Department of Pediatric. The patient group had various infections included 14 lower respiratory tract infection, 8 upper respiratory tract infection, 6 urinary tract infection, 3 gastrointestinal infection. The control group had no acute or chronic infections, no history taken any drugs. We performed same tests (Westergren sedimentation and MESR, CRP, WBC, fibrinogen) into two groups. There is no statistical significant between two groups as age and sex. Results: There was no statistically significant difference of age (p=0.59) and sex (p=0.18). between two groups. Sedimentation was 11.75±5.87 mm/h (4-24) in control group, 59.48±26.42 mm/h (10-109) in patient group (p<0.01). MSER was 14.08±5.35 mm/h (4-24) in control group, 32±11.31 (12-60) mm/h in patient group (p<0.01). Mean of sedimentation rate, MESR, CRP, WBC, and fibrinogen were significantly higher in patient group(p<0.01). Conclusion: In several studies, the MESR values higher than the macromethod at values but this difference is not of sufficient magnitude at the clinical decision level . In our study, MESR was as sensitive and specific as erythrocyte sedimentation rate in the patient group than control group. Also, MESR is more practical as it is a simple and quick test which does not need venous sample. [ABSTRACT FROM AUTHOR]

The article discusses some of the issues examined in the case of a child diagnosed with multisystem inflammatory syndrome based on the U.S. Centres for Disease Control and Prevention criteria. Topics discussed include information about graft intolerance syndrome, percutaneous renal artery embolization in patients with symptomatic non-functioning kidney allograft, and reactivation of the inflammatory pathways in the graft.

The article presents a case study of a An 18-year-old boy with detection of proteinuria in the urinary analysis. Topics discussed include results of various medical tests; complications of proteinuria, abdominal pain and burning pain in feet; and the usage of microscopy, echocardiogram and kidney biopsy in the treatment.

Background: Primary hyperoxaluria type 1 (PH1) is characterized by increased endogenous oxalate production and deposition as calcium oxalate crystals. The main manifestations are nephrocalcinosis/nephrolithiasis, causing impaired kidney function. We aimed to evaluate the clinical characteristics and overall outcomes of paediatric PH1 patients in Turkey. Methods: This is a nationwide, multicentre, retrospective study evaluating all available paediatric PH1 patients from 15 different paediatric nephrology centres in Turkey. Detailed patient data was collected which included demographic, clinical and laboratory features. Patients were classified according to their age and characteristics at presentation: patients presenting in the first year of life with nephrocalcinosis/nephrolithiasis (infantile oxalosis, Group 1), cases with recurrent nephrolithiasis diagnosed during childhood (childhood‐onset PH1, Group 2), and asymptomatic children diagnosed with family screening (Group 3). Results: Forty‐eight patients had a mutation consistent with PH1. The most common mutation was c.971_972delTG (25%). Infantile oxalosis patients had more advanced chronic kidney disease (CKD) or kidney failure necessitating dialysis (76.9% vs. 45.5%). These patients had much worse clinical course and mortality rates seemed to be higher (23.1% vs. 13.6%). Patients with fatal outcomes were the ones with significant comorbidities, especially with cardiovascular involvement. Patients in Group 3 were followed with better outcomes, with no kidney failure or mortality. Conclusion: PH1 is not an isolated kidney disease but a systemic disease. Family screening helps to preserve kidney function and prevent systemic complications. Despite all efforts made with traditional treatment methods including transplantation, our results show devastating outcomes or mortality. [ABSTRACT FROM AUTHOR]

A clinical quiz is presented about the case of a seven-year-old boy with a diagnosis of type 1 diabetes mellitus (DM) for two years and was admitted to the hospital with weakness, pallor, and edema of the hands and feet.

Congenital nephrotic syndrome (CNS) is a genetic disease that is present in the antenatal period or during the first 3 months of life. In this study, we aimed to compare growth parameters of patients with CNS who received kidney transplants and either (1) had a normal glomerular filtration rate (GFR) at the time of transplant or (2) chronic kidney disease (CKD) at the time of transplant. Patients with a diagnosis of CNS who had a minimum follow-up period of 6 months were evaluated retrospectively. Children at stages 4 or 5 CKD or patients receiving dialysis during the pretransplant period were defined as group 1; patients with normal GFR at the time of transplantation were classified as group 2. Short stature and low weight were defined as less than −2 standard deviation scores (SDS) for height and weight according to their age. A total of 17 patients were included in the study. Thirteen of 17 patients had NPHS1 gene mutations. Group 1 and group 2 consisted of 8 and 9 patients, respectively. Mean height SDS and mean weight SDS in group 2 were higher than group 1 in the pretransplant period (−4.34 ± 1.74 vs −2.84 ± 1.56; P =.011 and −3.54 ± 0.93 vs −1.83 ± 1.13; P =.008). In the post-transplant period, the significant difference in height SDS continued in favor of group 2 (−3.16 ± 1.11 vs −1.16 ± 0.87; P =.002). The short stature rate was 83% in group 1 and 72% in group 2 in the pretransplant period (P =.62), and 83% in group 1 and 27% in group 2 in the post-transplant period (P =.02). Early renal transplantation seems to be effective for optimal height gain in children with CNS. • Growth retardation is common in congenital nephrotic syndrome. • The treatment of congenital nephrotic syndrome is controversial. • We investigated the effect of early kidney transplantation on growth. • Short stature was common in patients who received transplants and later developed chronic kidney disease. • In the post-transplant period, the low-weight rate was improved in both groups. [ABSTRACT FROM AUTHOR]

The article discusses some of the questions to consider in the case of a child diagnosed with multisystem inflammatory syndrome based on the Centres for Disease Control and Prevention criteria. Highlights include tenderness on the graft in the right pelvic area according to physical examination, absence of leukocytes on microscopic examination of the peritoneal fluid, and the size of the transplanted kidney at the right lower quadrant of the abdomen.

A 4-month-old male baby was admitted because his father and uncles had chronic kidney disease. His father was diagnosed with membranoproliferative glomerulonephritis at the age of 5, underwent a kidney transplant at the age of 22, and lost the graft due to recurrence of the disease. In contrast, the young uncle was diagnosed with C3 glomerulopathy and mycophenolate mofetil and eculizumab were initiated early. It was remarkable that our patient had normal kidney function and urine analyses but low serum C3 level (0.56 g/L; N, 0.9–1.8 g/L). In the disease-associated clinical exome analysis, a heterozygous change in the CFH gene was found. The same mutation was found homozygous in the uncle. In genetically inherited diseases, findings may occur sequentially; early screening of at-risk individuals contributes to kidney survival. [ABSTRACT FROM AUTHOR]

The article presents answers to a quiz about acute kidney injury (AKI) in an allogeneic hematopoietic cell transplant recipient.

A clinical quiz is presented regarding the case of an 11-year 8-month old girl who had an allogeneic stem cell transplantation from a matched unrelated donor due to Fanconi aplastic anemia and had an elevated serum creatinine levels.

Background: Atypical hemolytic uremic syndrome (aHUS) is a rare, mostly complement-mediated thrombotic microangiopathy. The majority of patients are infants. In contrast to infantile-onset aHUS, the clinical and genetic characteristics of adolescence-onset aHUS have not been sufficiently addressed to date.A total of 28 patients (21 girls, 7 boys) who were diagnosed as aHUS between the ages of ≥10 years and <18 years were included in this study. All available data in the Turkish Pediatric aHUS registry were collected and analyzed.The mean age at diagnosis was 12.8±2.3 years. Extra-renal involvement was noted in 13 patients (46.4%); neurological involvement was the most common (32%). A total of 21 patients (75%) required kidney replacement therapy. Five patients (17.8%) received only plasma therapy and 23 (82%) of the patients received eculizumab. Hematologic remission and renal remission were achieved in 25 (89.3%) and 17 (60.7%) of the patients, respectively. Compared with the infantile-onset aHUS patients, adolescent patients had a lower complete remission rate during the first episode (p = 0.002). Genetic analyses were performed in all and a genetic variant was detected in 39.3% of the patients. The mean follow-up duration was 4.9±2.6 years. At the last visit, adolescent patients had lower eGFR levels (p = 0.03) and higher rates of chronic kidney disease stage 5 when compared to infantile-onset aHUS patients (p = 0.04).Adolescence-onset aHUS is a rare disease but tends to cause more permanent renal dysfunction than infantile-onset aHUS. These results may modify the management approaches in these patients.Methods: Atypical hemolytic uremic syndrome (aHUS) is a rare, mostly complement-mediated thrombotic microangiopathy. The majority of patients are infants. In contrast to infantile-onset aHUS, the clinical and genetic characteristics of adolescence-onset aHUS have not been sufficiently addressed to date.A total of 28 patients (21 girls, 7 boys) who were diagnosed as aHUS between the ages of ≥10 years and <18 years were included in this study. All available data in the Turkish Pediatric aHUS registry were collected and analyzed.The mean age at diagnosis was 12.8±2.3 years. Extra-renal involvement was noted in 13 patients (46.4%); neurological involvement was the most common (32%). A total of 21 patients (75%) required kidney replacement therapy. Five patients (17.8%) received only plasma therapy and 23 (82%) of the patients received eculizumab. Hematologic remission and renal remission were achieved in 25 (89.3%) and 17 (60.7%) of the patients, respectively. Compared with the infantile-onset aHUS patients, adolescent patients had a lower complete remission rate during the first episode (p = 0.002). Genetic analyses were performed in all and a genetic variant was detected in 39.3% of the patients. The mean follow-up duration was 4.9±2.6 years. At the last visit, adolescent patients had lower eGFR levels (p = 0.03) and higher rates of chronic kidney disease stage 5 when compared to infantile-onset aHUS patients (p = 0.04).Adolescence-onset aHUS is a rare disease but tends to cause more permanent renal dysfunction than infantile-onset aHUS. These results may modify the management approaches in these patients.Results: Atypical hemolytic uremic syndrome (aHUS) is a rare, mostly complement-mediated thrombotic microangiopathy. The majority of patients are infants. In contrast to infantile-onset aHUS, the clinical and genetic characteristics of adolescence-onset aHUS have not been sufficiently addressed to date.A total of 28 patients (21 girls, 7 boys) who were diagnosed as aHUS between the ages of ≥10 years and <18 years were included in this study. All available data in the Turkish Pediatric aHUS registry were collected and analyzed.The mean age at diagnosis was 12.8±2.3 years. Extra-renal involvement was noted in 13 patients (46.4%); neurological involvement was the most common (32%). A total of 21 patients (75%) required kidney replacement therapy. Five patients (17.8%) received only plasma therapy and 23 (82%) of the patients received eculizumab. Hematologic remission and renal remission were achieved in 25 (89.3%) and 17 (60.7%) of the patients, respectively. Compared with the infantile-onset aHUS patients, adolescent patients had a lower complete remission rate during the first episode (p = 0.002). Genetic analyses were performed in all and a genetic variant was detected in 39.3% of the patients. The mean follow-up duration was 4.9±2.6 years. At the last visit, adolescent patients had lower eGFR levels (p = 0.03) and higher rates of chronic kidney disease stage 5 when compared to infantile-onset aHUS patients (p = 0.04).Adolescence-onset aHUS is a rare disease but tends to cause more permanent renal dysfunction than infantile-onset aHUS. These results may modify the management approaches in these patients.Conclusions: Atypical hemolytic uremic syndrome (aHUS) is a rare, mostly complement-mediated thrombotic microangiopathy. The majority of patients are infants. In contrast to infantile-onset aHUS, the clinical and genetic characteristics of adolescence-onset aHUS have not been sufficiently addressed to date.A total of 28 patients (21 girls, 7 boys) who were diagnosed as aHUS between the ages of ≥10 years and <18 years were included in this study. All available data in the Turkish Pediatric aHUS registry were collected and analyzed.The mean age at diagnosis was 12.8±2.3 years. Extra-renal involvement was noted in 13 patients (46.4%); neurological involvement was the most common (32%). A total of 21 patients (75%) required kidney replacement therapy. Five patients (17.8%) received only plasma therapy and 23 (82%) of the patients received eculizumab. Hematologic remission and renal remission were achieved in 25 (89.3%) and 17 (60.7%) of the patients, respectively. Compared with the infantile-onset aHUS patients, adolescent patients had a lower complete remission rate during the first episode (p = 0.002). Genetic analyses were performed in all and a genetic variant was detected in 39.3% of the patients. The mean follow-up duration was 4.9±2.6 years. At the last visit, adolescent patients had lower eGFR levels (p = 0.03) and higher rates of chronic kidney disease stage 5 when compared to infantile-onset aHUS patients (p = 0.04).Adolescence-onset aHUS is a rare disease but tends to cause more permanent renal dysfunction than infantile-onset aHUS. These results may modify the management approaches in these patients. [ABSTRACT FROM AUTHOR]

Background/aim: Nephrocalcinosis (NC) is defined as calcium deposition in the kidney parenchyma and tubules. This study aims to determine the etiology, risk factors, and follow-up results of patients with NC in Turkey. Materials and methods: Patients diagnosed with NC in the pediatric nephrology Department Units of 19 centers from all geographical regions of Turkey over a 10-year period (2010–2019) were included in the study. The medical records from the centers were reviewed and demographic data, admission complaints, medical history, systemic and genetic disorders, risk factors for NC, treatment details, and presence of NC after one-year follow-up, were recorded retrospectively. Results: The study sample included 195 patients (88 females, 107 males). The mean age at diagnosis was 39.44 ± 47.25 (0.5–208) months; 82/190 patients (43.2%) were diagnosed incidentally; 46/195 patients (23.6%) had an underlying disease; idiopathic hypercalciuria was detected in 75/195 (38.4%) patients. The most common systemic diseases were distal renal tubular acidosis in 11/46 patients (23.9%), primary hyperoxaluria in 9/46 patients (19.6%) and Bartter syndrome in 7/46 patients (15.3%). After one year of follow-up, NC resolved in 56/159 patients (35.2%) and they all did not have an underlying systemic disease. Conclusion: The most common presentation of NC was incidental. Distal renal tubular acidosis and primary hyperoxaluria were the main systemic diseases leading to NC, while hypercalciuria was the most common metabolic risk factor. Nephrocalcinosis was found to remain in most of the patients at a one-year follow-up. It may resolve particularly in patients with no underlying systemic disease. [ABSTRACT FROM AUTHOR]

The article presents answers to a clinical quiz about an uncommon cause of hypophosphatemic rickets.

A quiz about the case of four-and-half-year-old boy with a diagnosis of hypophosphatemic rickets is presented.

Background: C3 glomerulopathy (C3G) is characterized by heterogeneous clinical presentation, outcome, and predominant C3 accumulation in glomeruli without significant IgG. There is scarce outcome data regarding childhood C3G. We describe clinical and pathological features, treatment and outcomes, and risk factors for progression to chronic kidney disease stage 5 (CKD5) in the largest pediatric series with biopsy-proven C3G. Methods: Sixty pediatric patients with C3G from 21 referral centers in Turkey were included in this retrospective study. Patients were categorized according to CKD stage at last visit as CKD5 or non-CKD5. Demographic data, clinicopathologic findings, treatment, and outcome data were compared and possible risk factors for CKD5 progression determined using Cox proportional hazards model. Results: Mean age at diagnosis was 10.6 ± 3.0 years and follow-up time 48.3 ± 36.3 months. Almost half the patients had gross hematuria and hypertension at diagnosis. Nephritic-nephrotic syndrome was the commonest presenting feature (41.6%) and 1/5 of patients presented with nephrotic syndrome. Membranoproliferative glomerulonephritis was the leading injury pattern, while 40 patients had only C3 staining. Patients with DDD had significantly lower baseline serum albumin compared with C3GN. Eighteen patients received eculizumab. Clinical remission was achieved in 68.3%. At last follow-up, 10 patients (16.6%) developed CKD5: they had lower baseline eGFR and albumin and higher frequency of nephrotic syndrome and dialysis requirement than non-CKD5 patients. Lower serum albumin and eGFR at diagnosis were independent predictors for CKD5 development. Conclusions: Children with C3G who have impaired kidney function and hypoalbuminemia at diagnosis should be carefully monitored for risk of progression to CKD5. Graphical abstract [ABSTRACT FROM AUTHOR]

aHUS is caused by the over‐activation and dysregulation of the alternative complement pathway. Data regarding outcomes of pediatric aHUS patients after kidney transplantation are still very scarce. Accordingly, the aim of this study was to describe the clinical findings and outcomes of pediatric aHUS patients after renal transplantation. This is a retrospective, multicenter study including 12 patients from the national registry system. Among the 12 patients, eight had received prophylactic eculizumab and none of those patients (except one) had experienced aHUS recurrence during a median follow‐up period of 58.5 (min‐max, 4‐94) months. Although eculizumab had been started on the day before transplantation in one of them, aHUS recurrence occurred during the transplantation procedure. Eculizumab had been stopped in only one patient who had no complement gene mutation after 35 months of therapy, and recurrence had not been observed during the 19 months of follow‐up. In three patients, maintenance doses had been spaced out without any recurrence. One additional patient with anti‐CFH antibody received only two doses of eculizumab for transplantation and had been followed for 46 months without aHUS recurrence. The remaining three patients had not received anti‐C5 therapy and none of those patients experienced aHUS recurrence during a median follow‐up period of 21 (min‐max, 9‐42) months. Prophylactic eculizumab is a safe and effective treatment for the prevention of aHUS recurrence. Eculizumab interval prolongation, discontinuation, and transplantation without eculizumab prophylaxis can be tried in selected patients with close follow‐up. [ABSTRACT FROM AUTHOR]

Background: Peritoneal dialysis (PD) is the most common kidney replacement therapy in children. Complications associated with PD affect treatment success and sustainability. The aim of this study was to investigate the frequency of PD-related non-infectious complications and the predisposing factors. Methods: Retrospective data from 11 centers in Turkey between 1998 and 2018 was collected. Non-infectious complications of peritoneal dialysis (NICPD), except metabolic ones, in pediatric patients with regular follow-up of at least 3 months were evaluated. Results: A total of 275 patients were included. The median age at onset of PD and median duration of PD were 9.1 (IQR, 2.5–13.2) and 7.6 (IQR, 2.8–11.9) years, respectively. A total of 159 (57.8%) patients encountered 302 NICPD within the observation period of 862 patient-years. The most common NIPCD was catheter dysfunction (n = 71, 23.5%). At least one catheter revision was performed in 77 patients (28.0%). Longer PD duration and presence of swan neck tunnel were associated with the development of NICPD (OR 1.191; 95% CI 1.079–1.315, p = 0.001 and OR 1.580; 95% CI 0.660–0.883, p = 0.048, respectively). Peritoneal dialysis was discontinued in 145 patients; 46 of whom (16.7%) switched to hemodialysis. The frequency of patients who were transferred to hemodialysis due to NICPD was 15.2%. Conclusions: Peritoneal dialysis-related non-infectious complications may lead to discontinuation of therapy. Presence of swan neck tunnel and long duration of PD increased the rate of NICPD. Careful monitoring of patients is necessary to ensure that PD treatment can be maintained safely. [ABSTRACT FROM AUTHOR]

The article discusess the treatment of nunchausen syndrome on an adolescent presenting with acquired acute renal damage. It mentions about the effectivness of laboratory and imaging findings in diagnosing the same; and impact of detailed medical history and systemic examinations in confirming the laboratory test results are also important.

A quiz is presented on an adolescent presenting with acquired acute renal damage.

Background: Although orthostatic proteinuria (OP) is the most common cause of childhood proteinuria, excluding transient proteinuria, data regarding prevalence and long-term prognosis are limited. We aimed to determine prevalence of OP in healthy schoolchildren evaluating relationships with age, gender and body mass index, and determine follow-up. Methods: A total of 1701 healthy children aged 6–15 years were selected using a population-based, stratified, cluster-sampling method; and random urine samples were taken. For proteinuria ≥ 1+ in first urine samples, second and third random samples were taken at least 2 weeks apart to exclude transient proteinuria. For continuing proteinuria after third samples, first morning urine samples were collected. Cases where proteinuria was not detected in first morning urine samples were diagnosed as OP. Results: Sixty-four of 1701 children (3.7%) had proteinuria on first random urine samples. After second and third urine samples, proteinuria persisted in only 16 (0.94%). OP was detected in 11 (0.65%). Prevalence of OP tended to decrease with increasing BMI, though not statistically significant. All 7 cases with OP who were re-evaluated later, had no proteinuria 3 years after diagnosis. Conclusions: Prevalence of OP in our study was lower than the literature. At least three random urine samples should be taken to exclude transient proteinuria in an asymptomatic child/adolescent before making a diagnosis of OP using first morning urine samples. OP is a benign condition and resolves spontaneously in most cases. Underweight children had a tendency for OP compared with overweight and obese children; however, further studies with larger number of patients are needed. [ABSTRACT FROM AUTHOR]

Aim: Viral infections commonly affect kidney transplant recipients and may lead to graft failure and death. The aim of this study was to evaluate the antibody seroprevalence against viral agents in kidney transplant recipients. Material and Methods: The records of children who underwent kidney transplantation between 2008 and 2018 in Akdeniz University Faculty of Medicine were retrospectively reviewed. Epstein-Barr virus, cytomegalovirus, hepatitis A virus, hepatitis B virus, varicella, measles, rubella and mumps serologies evaluated before transplantation, were recorded. The clinical characteristics of seronegative and seropositive patients were compared, and factors that affected seropositivity were investigated. Results: The study included 253 children with a mean age of 16.7±6.23 years. The mean age at transplantation was 11.4±5.01 years. The seropositivity rates for vaccine-preventable viral infections varied: hepatitis B 89.7%, hepatitis A 60.5%, measles 78.7%, rubella 88.1%, mumps 61.2%, and varicella 71.9%. Cytomegalovirus seropositivity was 92.1% and Epstein- Barr virus seropositivity was 82.2%. Hepatitis B antibody positivity was 91.8% in patients undergoing hemodialysis, 94.5% in patients electrocheundergoing peritoneal dialysis, and 84.9% in pre-emptive transplantation patients (p=0.037). The mean age at transplantation was higher in patients with seropositivity for both cytomegalovirus and Epstein-Barr virus compared with seronegative patients (p<0.001 for both). The mean age at transplantation and diagnosis of glomerular disease was found to be effective for varicella seropositivity in multivariate regression analysis (OR 0.860, 95% CI: 0.808--0.915, p<0.001 and OR 2.502, 95% CI: 1.321--4.739, p=0.005, respectively). Conclusion: It is important to screen patients with chronic kidney disease in terms of vaccine-preventable diseases to identify risky groups of patients and to immunize these patients before end-stage kidney disease develops. [ABSTRACT FROM AUTHOR]

Objective: To investigate the interaction between the roles of dietary excess sodium and low potassium intake in the pathogenesis of hypertension. Material and Methods: The study consisted of 56 obese normotensive, 41 obese hypertensive and 29 healthy children as a control group, aged between 6 and 18 years. The ratio of urinary sodium to potassium (U Na/K) was evaluated in the afternoon urine samples in obese children. Also, the total body fat percentages were noted by using Tanita bio-impedance segmental body composition analyzer. Results: Of the 41 obese hypertensive patients, 10 of them were <10 years old, and 31 of them were older than ten years. Insulin levels and HOMA-IR index in the obese normotensive and hypertensive groups were 16.7±8.3 and 15.9±7.9 U/ml, 4.4±3.4 and 5.3±3.4 respectively; these differences were not significant (P>0.05). The association of Na/K with blood pressure was not found statistically significant. However, it was found higher in OHT than ONT and control group. Conclusion: Increased consumption of salty fat foods, high protein diets, and sedentary lifestyles predispose to obesity and related diseases. [ABSTRACT FROM AUTHOR]

Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are very rare in childhood with an increased risk of morbidity and mortality. We aimed to evaluate renal prognostic factors in childhood AAV from the perspective of ANCA serotype, histopathological classification, and five-factor score (FFS).Methods: Pediatric AAV patients from 11 referral centers in Turkey had been included to the study. The demographics, clinical findings, AAV subtypes, outcomes, and FFS were evaluated retrospectively. Kidney biopsies were classified histopathologically.Results: Totally, 39 patients were enrolled in the study. Among all patients, 74.4% had renal involvement, 56.4% ear-throat-nose involvement, and 51.3% had musculoskeletal involvement. Proteinase 3 (PR3)-ANCA was positive in 48.7%, and myeloperoxidase (MPO)-ANCA was positive in 30.8%. 69.2% of patients had impaired renal function, and 28.2% had progressed to end-stage renal disease (ESRD) during the follow-up. At the time of diagnosis, FFS was ≥ 2 in 53.8%. The most common histopathologic classifications were as follows: crescentic type in 40.7% and sclerotic type in 25.9%. Gastrointestinal and renal involvement, MPO-ANCA positivity, serum creatinine levels, and impaired renal function during the follow-up were significantly higher in patients with FFS ≥ 2, compared to patients with FFS < 2. Patients with FFS ≥ 2 had more common crescentic, mixed and sclerotic histopathologic findings in biopsies. By logistic regression analysis forward method, the strongest single-risk factor among all the parameters was the initial level of creatinine in patients with ESRD, compared to the other patients (p = 0,007).Conclusions: Evaluation of the FFS, ANCA serology, and the creatinine levels may help to predict renal prognosis. [ABSTRACT FROM AUTHOR]

Objective: In this study, we aimed to assess renal outcomes of delayed diagnosis of dilating primary vesicoureteral reflux (VUR) following recurrent febrile urinary tract infections (fUTIs) and its diagnostic imaging procedures. Material and methods: The medical records of patients who underwent ultrasonography (US), non-acute dimercaptosuccinic acid (Tc-99mDMSA) scintigraphy and voiding cystourethrography (VCUG), and who were older than 2 years at the time of VUR diagnosis were retrospectively reviewed. Results: A total of 32 children (female, n=27: 84.4%) with a mean age of 7.67±3.34 years at the time of diagnosis of VUR were included in the study. Grade III, IV, V VUR were found in 22%, 69%, and 9% of the patients, respectively. At the time of VUR diagnosis, abnormal US findings were detected in 75% of the cases. Tc-99mDMSA detected abnormalities in 83.9% (7 with a single scar, 7 with multiple lesions, 12 with reduced kidney function) of the patients. Estimated glomerular filtration rate of 3 patients with bilateral grade IV VUR was <75 mL/min/1.73 m². In 5 patients (16%), VUR could not be predicted by US+DMSA scintigraphy (Grade IV VUR in 3 and Grade III in 2 cases ). The sensitivity in predicting VUR was 75.00% (95% CI: 56.60-88.54) and 83.87% (95% CI: 66.27-94.55), respectively, for US alone and combined US+DMSA. Conclusion: VCUG should be performed routinely in addition to US and non-acute DMSA in all children referred with recurrent fUTIs. Awareness of childhood UTI in public and healthcare personnels should be increased in order to refer these patients at a early stage to pediatric urology and nephrology units. [ABSTRACT FROM AUTHOR]

• The prevalence of NPSLE is 13.5% in pediatric SLE patients in Turkey. • Clinical and laboratory features of SLE and NP involvement are grouped in five clusters. • APA positivity, high SLEDAI scores and plasmapheresis are risk factors for neurological sequelae. Neuropsychiatric (NP) involvement is a restricted area in juvenile-onset systemic lupus erythematosus (jSLE). To investigate the prevalence, demographic and clinical features, and outcomes of the neurological involvement in the Turkish jSLE population. This study was based upon 24 referral centers' SLE cohorts, multicenter and multidisciplinary network in Turkey. Patient data were collected by a case report form which was standardized for NP definitions according to American Collage of Rheumatology (ACR). Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) neuropsychiatric part was used to determine NP damage. Variables were evaluated Ward's hierarchical clustering analyses, univariate, and multivariate logistic regression analyses. A hundred forty-nine of 1107 jSLE patients had NP involvement (13.5%). The most common NPSLE findings were headache (50.3%), seizure (38.3%), and acute confusional state (33.6%). Five clusters were identified with all clinical and laboratory findings. The first two clusters involved neuropathies, demyelinating diseases, aseptic meningitis, and movement disorder. Cluster 3 involved headache, activity markers and other SLE involvements. Idiopathic intracranial hypertension, cerebrovascular disease, cognitive dysfunction, psychiatric disorders and SLE antibodies were in the fourth, and acute confusional state was in the fifth cluster. In multivariate analysis, APA positivity; OR: 2.820, (%95CI: 1.002–7.939), P : 0,050, plasmapheresis; OR: 13.804 (%95CI: 2.785–68.432), P : 0,001, SLEDAI scores; OR: 1.115 (%95CI: (1.049–1.186), P : 0,001 were associated with increased risk for neurologic sequelae. We detected the prevalence of juvenile NPSLE manifestations in Turkey. We have identified five clusters that may shed light pathogenesis, treatment and prognosis of NP involvements. We also determined risk factors of neurological sequelae. Our study showed that new definitions NP involvements and sequelae for childhood period are needed. [ABSTRACT FROM AUTHOR]

A 17-year old girl admitted to our outpatient unit with hoarseness and shortness of breath. Her physical examination showed nasal deformity. Her medical history included the diagnosis of uveitis and type 1 diabetes mellitus (DM) as well as asthma and chronic sinusitis. Diagnostic work-up showed involvement of the upper respiratory tract (saddle nose deformity, chronic sinusitis), laryngo-tracheo-bronchial involvement (subglottic and bronchial stenosis) and pulmonary involvement (pulmonary nodules) leading to a diagnosis of granulomatous polyangiitis (GPA). We report this child on the basis of the fact that coexistence of type 1 DM and GPA has never been reported in our country previously. [ABSTRACT FROM AUTHOR]

The article presents answers to a clinical quiz about a rare cause of urolithiasis in an infant who had multiple, bilateral kidney stones which required surgical intervention.

Background: An increase in the number of circulating endothelial cells (CEC) indicates endothelial damage and the risk of cardiovascular disease. The aim of our study was to investigate the association of CEC with various clinical parameters in pediatric renal transplant recipients. Methods: CEC, defined as CD45CD146, were enumerated by flow cytometry from the peripheral blood of 50 pediatric renal transplant recipients and 20 healthy controls. Clinical parameters, including renal function tests, fasting blood glucose, serum cholesterol and triglyceride, cyclosporine A (CsA) (trough and 2nd-hour) and tacrolimus (tac) trough blood levels and their association with CEC numbers were analyzed. Results: CEC numbers of patients were higher than those of controls (respectively, 128 ± 89 cells/ml (42-468 cells/ml), 82 ± 33 cells/ml (32-137 cells/ml), p = 0.024). There was a statistically significant negative correlation between CEC numbers and glomerular filtration rate (GFR) ( r = −0.300, p = 0.012). There was also a statistically positive association between CEC numbers and transplant duration as well as cyclosporine trough level (respectively, r = 0.397, p = 0.004, r = 0.714, p = 0.004). CEC numbers in patients on tac and CsA were similar ( p = 0.716). Conclusions: Our results demonstrate that renal transplant recipients with high CsA trough blood level, longer transplant duration, and lower GFR, are at greater risk of developing endothelial damage. [ABSTRACT FROM AUTHOR]

TUTAR, E.,et al.: Assessment of Ventricular Repolarization in a Large Group of Children with Early Onset DeafnessThis study examined the ECG traces of 397 deaf children (age 12.5± 2.9 years, range 6–19 years), after exclusion of cases with Jervell and Lange-Nielsen syndrome (JLNS), and compared them to those of 361 normal hearing counterparts (age 12.5± 2.7 years; range 7–18 years). An observer, who was unaware of the hearing status of the subjects, measured QT and QTc intervals and calculated dispersions of QT and QTc from standard 12-lead ECGs recorded at a speed of 25 mm/s at rest. Although the mean QT was found to be longer in deaf children than that observed in the control group (P<0.0001), the mean QTc was significantly shorter (P<0.0001). The mean heart rate was significantly lower in deaf children. When QT and QTc data were recompared after the children were grouped according to the heart rate, the observed difference became less significant or disappeared. In conclusion, there are no major abnormalities for repolarization parameters in children with congenital sensorineural deafness, when compared to hearing counterparts, if heart rates are similar. Based on these results, routine ECG screening of deaf children for repolarization abnormalities may be unnecessary unless they have a history of syncope or positive family history of syncope and/or early sudden death.(PACE 2004; 27:1217–1220) [ABSTRACT FROM AUTHOR]

A clinical quiz is presented about a rare cause of urolithiasis in a 13-month-old boy admitted with red-brown spots on his diaper.

A quiz concerning rhabdomyolysis-associated acute kidney injury is presented.

The article presents the case of a 17-year-old boy, who was admitted with complaints of diarrhea, vomiting and decreased urinary output. Diarrhea continued for 1 week and occurred five to six times a day. The stool was green and did not contain blood or mucus. Vomiting and oliguria started 2 days before his presentation to the medical center. He described widespread muscle pain and weakness.