The uremic toxins indoxyl sulfate (IS) and p-cresyl sulfate (pCS) accumulate in patients with chronic kidney disease (CKD) as a consequence of altered gut microbiota metabolism and a decline in renal ...excretion. Despite of solid experimental evidence for nephrotoxic effects, the impact of uremic toxins on the progression of CKD has not been investigated in representative patient cohorts. In this analysis, IS and pCS serum concentrations were measured in 604 pediatric participants (mean eGFR of 27 ± 11 ml/min/1.73m2) at enrolment into the prospective Cardiovascular Comorbidity in Children with CKD study. Associations with progression of CKD were analyzed by Kaplan-Meier analyses and Cox proportional hazard models. During a median follow up time of 2.2 years (IQR 4.3-0.8 years), the composite renal survival endpoint, defined as 50% loss of eGFR, or eGFR <10ml/min/1.73m2 or start of renal replacement therapy, was reached by 360 patients (60%). Median survival time was shorter in patients with IS and pCS levels in the highest versus lowest quartile for both IS (1.5 years, 95%CI 1.1,2.0 versus 6.0 years, 95%CI 5.0,8.4) and pCS (1.8 years, 95%CI 1.5,2.8 versus 4.4 years, 95%CI 3.4,6.0). Multivariable Cox regression disclosed a significant association of IS, but not pCS, with renal survival, which was independent of other risk factors including baseline eGFR, proteinuria and blood pressure. In this exploratory analysis we provide the first data showing a significant association of IS, but not pCS serum concentrations with the progression of CKD in children, independent of other known risk factors. In the absence of comorbidities, which interfere with serum levels of uremic toxins, such as diabetes, obesity and metabolic syndrome, these results highlight the important role of uremic toxins and accentuate the unmet need of effective elimination strategies to lower the uremic toxin burden and abate progression of CKD.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
The extent and relevance of altered bone metabolism for statural growth in children with chronic kidney disease is controversial. We analyzed the impact of renal dysfunction and recombinant growth ...hormone therapy on a panel of serum markers of bone metabolism in a large pediatric chronic kidney disease cohort.
Bone alkaline phosphatase (BAP), tartrate-resistant acid phosphatase 5b (TRAP5b), sclerostin and C-terminal FGF-23 (cFGF23) normalized for age and sex were analyzed in 556 children aged 6-18 years with an estimated glomerular filtration rate (eGFR) of 10-60 ml/min/1.73 m2. 41 children receiving recombinant growth hormone therapy were compared to an untreated matched control group.
Standardized levels of BAP, TRAP5b and cFGF-23 were increased whereas sclerostin was reduced. BAP was correlated positively and cFGF-23 inversely with eGFR. Intact serum parathormone was an independent positive predictor of BAP and TRAP5b and negatively associated with sclerostin. BAP and TRAP5B were negatively affected by increased C-reactive protein levels. In children receiving recombinant growth hormone, BAP was higher and TRAP5b lower than in untreated controls. Sclerostin levels were in the normal range and higher than in untreated controls. Serum sclerostin and cFGF-23 independently predicted height standard deviation score, and BAP and TRAP5b the prospective change in height standard deviation score.
Markers of bone metabolism indicate a high-bone turnover state in children with chronic kidney disease. Growth hormone induces an osteoanabolic pattern and normalizes osteocyte activity. The osteocyte markers cFGF23 and sclerostin are associated with standardized height, and the markers of bone turnover predict height velocity.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
The study aims to present the incidence of COVID-19 in pediatric patients undergoing renal replacement therapy (RRT) and to compare the severity and outcomes of the disease between the dialysis and ...kidney transplant (KTx) groups. This multicenter observational study was conducted between 1 April and 31 December 2020 in Istanbul. Members of the Istanbul branch of the Turkish Pediatric Nephrology Association were asked to report all confirmed cases of COVID-19 who were on RRT, as well as the number of prevalent RRT patients under the age of 20. A total of 46 confirmed cases of COVID-19 were reported from 12 centers, of which 17 were dialysis patients, and 29 were KTx recipients. Thus, the incidence rate of COVID-19 was 9.3% among dialysis patients and 9.2% among KTx recipients over a 9-month period in Istanbul. Twelve KTx recipients and three dialysis patients were asymptomatic (
p
= 0.12). Most of the symptomatic patients in both the dialysis and KTx groups had a mild respiratory illness. Only two patients, one in each group, experienced a severe disease course, and only one hemodialysis patient had a critical illness that required mechanical ventilation. In the entire cohort, one hemodialysis patient with multiple comorbidities died.
Conclusion
: While most cases are asymptomatic or have a mild disease course, pediatric patients undergoing dialysis and a kidney transplant are at increased risk for COVID-19.
What is Known:
• In adult population, both dialysis patients and kidney transplant recipients are at increased risk for severe illness of COVID-19 and have higher mortality rate.
• Children with kidney transplantation are not at increased risk for COVID-19 and most have mild disease course.
• Data on children on dialysis are scarce.
What is New:
• Pediatric patients undergoing dialysis and kidney transplantation have an increased risk for COVID-19.
• Most patients undergoing renal replacement therapy either on dialysis or transplanted develop asymptomatic or mild COVID-19 disease with a favorable outcome.
Objective: Health-related quality of life and its influencing factors in pediatric patients with voiding dysfunction and their parents was assessed. Method: The patients who were admitted with ...urinary incontinence for at least 6 months to the clinics of pediatrics and pediatric nephrology were enrolled in the study. The patients were divided into three group as daytime urinary incontinence (DUI), enuresis and both DUI and enuresis. The Pediatric Quality of Life InventoryTM (PedsQLTM) was administered to patients and mothers. Results: A total of 100 patients with voiding dysfunction aged from 5 years to 18 years (mean age: 8.8±2.6) were included in the study. The mean dysfunctional voiding symptom score was 15.5±6.6 in all groups and significantly higher in the group who experienced both DUI and NE (p<0.001). No significant difference was observed in terms of mean PedsQL scores of the children and their parents between groups with urinary incontinence (65.5±16 and 58.4±13.5, respectively), but strong positive correlation was obtained (p<0.001). The mean PedsQL score for the children aged from 8 years to 12 years was significantly lower than the scores for the children aged from 5 years to 7 years old (p<0.05). The mean PedsQL score for the children aged from 13 years to 18 years was not different from the other age groups. Conclusions: The early diagnosis and treatment of patients aged from 8 years to 12 years who had the lowest PedsQL score is important. Additionally, the success in improvement of the quality of life of children with voiding dysfunction require cooperation with parents.
Background
This study aims to identify epidemiological and clinical characteristics of patients and report our experience with eculizumab treatment during an outbreak of hemolytic uremic syndrome ...(HUS) caused by Shiga toxin-producing
Escherichia coli
(STEC) in Istanbul in 2015.
Methods
Thirty-two children (21 females, median age 3.25 years) were included in this study. Demographic, clinical and laboratory data, and treatment details were retrospectively collected. Renal outcomes were assessed at last follow-up visit. To assess the effect of eculizumab on prognosis of STEC-HUS, subgroup analysis was performed on patients who required dialysis.
Results
A high number of cases occurred within a certain region of Istanbul. Stool samples were cultured from 21 patients (65%), and enteroaggregative
E. coli
(EAEC;
n
= 7) and enterohemorrhagic
E. coli
(EHEC;
n
= 3) strains were detected. Rates of dialysis treatment, neurological manifestations, and death were 59%, 25%, and 3%, respectively. Mean follow-up duration was 8.6 ± 2.6 months (range 3–12 months). None of the patients (
n
= 25) was on dialysis at the final visit. The complete renal recovery rate was 54%. Nine patients were treated with eculizumab. At final follow-up visit, no differences in estimated glomerular filtration rate, proteinuria level, or hypertension incidence were observed between patients treated with eculizumab and those not treated with eculizumab.
Conclusions
An outbreak of EAEC occurred in a specific region of Istanbul. Livestock markets were suspected as the source. Evidence for beneficial effects of eculizumab on renal outcome was not clear in this cohort.
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.
In this case report, we shared diagnostic and treatment process of a patient-who was first admitted to the hospital with edema, pain and fever in the leg, was diagnosed with multisystem inflammatory ...syndrome in children (MIS-C) later diagnosed hydatid cyst with lung and bone involvement. Ceftriaxone, vancomycin, and clarithromycin treatment which had been already started before were continued. Since her fever and laboratory markers are not responding to treatment, we consulted the patient with pediatric hematology and nephrology departments. In the follow-up pelvic MRI, there was an infiltrative lesion with hyperintense lobulated contours in the left iliac bone and an area of signal change showing heterogeneous contrast enhancement in the adjacent muscle group in the periosteum (Figure 2). The imaging performed in the second month, while the patient was still on albendazole, showed that the lung lesions were almost completely responsive to the treatment, the fluid signal intensity decreased in the lesion in the left iliac bone and there was retraction in the lesion contours.
Background
The optimal time for dialysis initiation in adults and children with chronic kidney disease remains unclear. The aim of this study was to evaluate the impact of dialysis timing on ...different outcome parameters, in particular left ventricular (LV) morphology and inflammation, in pediatric patients receiving peritoneal dialysis and hemodialysis.
Methods
The medical records of pediatric dialysis patients who were followed-up in nine pediatric nephrology centers in Turkey between 2008 and 2013 were retrospectively reviewed. In addition to demographic data, we retrieved anthropometric measurements, data on dialysis treatment modalities, routine biochemical parameters, complete blood count, serum ferritin, parathormone, C-reactive protein (CRP), and albumin levels, as well as echocardiographic data and hospitalization records. The patients were divided into two groups based on their estimated glomerular filtration rate (eGFR) levels at dialysis initiation, namely, an early-start group, characterized by an eGFR of >10 ml/min/1.73 m
2
, and a late-start group, with an eGFR of < 7 ml/min/1.73 m
2
. The collected data were compared between these groups.
Results
A total of 245 pediatric dialysis patients (mean age ± standard deviation 12.3 ± 5.1 years, range 0.5–21 years) were enrolled in this study. Echocardiographic data were available for 137 patients, and the mean LV mass index (LVMI) was 58 ± 31 (range 21–215) g/m
2.7
. The LVMI was 75 ± 30 g/m
2.7
(
n
= 81) and 34 ± 6 g/m
2.7
(
n
= 56) in patients with or without LV hypertrophy (LVH) (
p
< 0.001). Early-start (eGFR >10 ml/min/1.73 m
2
) versus late-start dialysis (eGFR < 7 ml/min/1.73 m
2
) groups did not significantly differ in LVMI and LVH status (
p
> 0.05) nor in number of hospitalizations. Serum albumin levels were significantly higher in the early-dialysis group compared with the late-dialysis group (3.3 ± 0.7 vs. 3.1 ± 0.7 g/dl, respectively;
p
< 0.05). The early-start group had relatively higher time-averaged albumin levels (3.2 ± 0.5 vs. 3.1 ± 0.5 g/dl;
p
= > 0.05) and relatively lower CRP levels (3.64 ± 2.00 vs. 4.37 ± 3.28 mg/L,
p
> 0.05) than the late-start group, but these differences did not reach statistical significance.
Conclusion
Although early dialysis initiation did not have a significant effect on important clinical outcome parameters, including LVH, inflammatory state, and hospitalization, in our pediatric dialysis patients, this area of study deserves further attention.
Urinary tract stones are less common in children than in adults. Determining the etiology is the most important step to achieve successful treatment and prevent future recurrence. The aim of this ...study was to investigate the clinical characteristics and possible risk factors for urinary stone disease in pediatric patients.
The data of 126 patients with urinary stone disease who were treated in a pediatric nephrology clinic between 2000 and 2014 were analyzed retrospectively. A total 126 patients were enrolled in the study: 70 (55%) male and 56 (45%) female patients were included. The complaints, age of diagnosis, family histories, and stone location were examined. Direct urine microscopic examination, complete urinalysis, and urine culture were performed for all of the patients. Calcium, uric acid, oxalate, citrate, magnesium, and cystine levels were measured in urine collected in a 24-hour period. Serum electrolyte, blood urea nitrogen, creatinine, calcium, phosphorus, uric acid, and albumin levels were measured. Urinary ultrasound was performed. Stone analysis was conducted using the X-ray diffraction method. The mean age of the patients was 55 months (range: 1-162 months) at presentation.
In all, 34% of the patients had a family history of urinary stone disease. The rate of previous urinary tract infection was 26%. It was determined that 34% of the patients had been taking vitamin D and 5% had been taking a high dose. Metabolic risk factors determined were: hypercalciuria in 41%, hypocitraturia in 30%, hyperoxaluria in 14%, hyperuricosuria in 10%, and cystinuria in 5%. Among the group, 81% of the patients had kidney stones, 6.5% had ureter stones, and 2.5% had bladder stones. Furthermore, it was determined that 45% of the stones were composed of calcium oxalate, 35% had calcium phosphate stones, 14.2% had uric acid stones, and 13.3% had cystine stones. In 52% of the cases, extracorporeal shock wave lithotripsy was performed, and 71% underwent surgical treatment.
Metabolic evaluation and stone analysis should be performed to prevent future recurrences in children with urinary stone disease and lifelong follow-up should be emphasized.
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