Lasten munuaissairaudet

Vertaisarvioitu. Teema : munuaissairaudetLasten munuaissairaudet voivat olla synnynnäisiä kehityshäiriöitä, munuaiskerässairauksia tai tubulaarisia sairauksia. Ne ovat merkittävä erotusdiagnostinen vaihtoehto selvitettäessä lapsen kasvuhäiriön, huonon menestymisen tai korkean verenpaineen syytä. Lasten vaikeiden munuaiskorvaushoitoon johtavien sairauksien ilmaantuvuus on pysynyt vakaana, mutta munuaisten vajaatoiminnan syyt ovat osittain muuttuneet. Vaikeaakin munuaissairautta sairastavien lasten eloonjäämisennuste on hyvä ja heitä siirtyy yhä enemmän aikuisten terveydenhuollon seurantaan.Peer reviewe

Incidence of urinary tract infections in infants with antenatally diagnosed hydronephrosis-A retrospective single center study

Objective: To evaluate the incidence of urinary tract infections (UTIs) in infants with antenatal hydronephrosis (AHN). Materials and Methods: A cohort of AHN patients admitted to our institution between 2003 and 2013 were identified. Altogether 192 patients with nonrefluxing hydronephrosis (HN, n = 135), nonrefluxing hydroureteronephrosis (HUN, n = 21), or vesicoureteral reflux (VUR, n = 36) were identified. Patients with complex anomalies or neonatal decompression of the urinary tract were excluded. Information about UTIs diagnosed among the AHN patients was collected and compared with data from 58 controls. Results: During the median follow-up time of 2.6 (0.3-11.2) years, 24 (13%) patients (15 (10%) males and 9 (19%) females) and 2 (3%) controls experienced at least one UTI (p = 0.033). Eighteen (69%) UTIs were febrile. The males had the first UTI at significantly younger age than the females (0.3, 0.0-1.7 years vs. 1.0, 0.4-4.8 years, p = 0.010). UTI was detected in 15 (63%) patients with grade 4-5 VUR, in 8 (6%) patients with HN, and in one (5%) patient with HUN (p-values 0.999 against the controls). None of the patients with grade = 3 VUR had UTI. Fifty-eight percent of the patients with UTI were on antimicrobial prophylaxis. In five (12%) cases UTI appeared within one week after voiding cystourethrography (VCUG). Conclusions: Infants with AHN and grade 4-5 VUR had the highest risk of UTI. UTIs tented to be more common in females than in males; however, males experienced UTI at younger age than females. VCUG caused UTI in 2.3% in our material. (C) 2017 Elsevier Inc. All rights reserved.Peer reviewe

Prenatal complicated duplex collecting system and ureterocele-Important risk factors for urinary tract infection

Purpose: To evaluate the risk of urinary tract infections (UTIs) in infants with prenatally detected complicated duplex collecting system (CDS) or ureterocele. Materials and methods: All patients with prenatally detected CDS (n= 34) or single system ureterocele (n= 7) who were admitted to our institution between 2003 and 2013 were enrolled in this retrospective analysis. Duplex collecting systems with ureterocele (n = 13), vesicoureteral reflux (VUR) (n = 20) or nonrefluxing megaureter without ureterocele (n = 7) were determined as complicated. Twenty-six (63%) patients were females. The prevalence of UTI was compared to 66 controls. Results: The median follow-up time was 5.5 (1.7-12.2) years. Eighteen (44%) patients and 3 (5%) controls had at least one UTI (p <0.001) at themedian age of 0.8 and 0.4 years, respectively (p= 0.481). Fifty-seven percent of the UTIs were breakthrough infections and 82% of those were non-Escherichia coli infections. UTIs occurred prior to any surgical intervention in 4/13 (31%) patients with ureterocele, in 2/14 (14%) patientswith VUR, in 4/7 (57%) patients with both ureterocele and VUR, and in 3/7 (43%) patientswith nonrefluxingmegaureterwithout VUR or ureterocele (p-values 0.012, 0.209, 0.001 and 0.010, respectively, compared to controls). Postoperative UTIswere observed in 29% of the girls and in none of the 11 boys (p = 0.072). The incidence of UTI after perforation of ureterocele was only 14%. Conclusions: Children with prenatally detected ureterocele or duplex collecting system associated with nonrefluxing megaureter are at high risk of UTI despite prophylactic antibiotics. In case of prenatally detected ureterocele we suggest to consider early endoscopic perforation. Level of evidence: III. (c) 2017 Elsevier Inc. All rights reserved.Peer reviewe

Association of Renal Glomerular and Tubular Function With Renal Outcome in Patients With Posterior Urethral Valves

OBJECTIVE To analyze renal glomerular and tubular function and their association in patients operated for posterior urethral valves and to prognosticate the risk for end-stage kidney disease (ESKD) METHODS Sixty-three previously treated patients were evaluated for renal function during 1987-1991. The patients' age at evaluation was 11 years (range 2-24). Glomerular function was assessed by measuring glomerular filtration rate (GFR) and urine albumin excretion. Tubular function was determined by measuring urine concentration capacity and excretion of electrolytes (Na, K, Cl, Ca, P, Mg) and 0-2-microglobulin. Additionally, the prevalence of hypertension and serum parathyroid hormone and aldosterone values were registered. Tubular function was compared with GFR and the risk of developing ESKD before November 2018. RESULTS Twenty of the study patients (32%) had decreased GFR. In addition, 19% had proteinuria and 56% were hypertensive. Those without proteinuria or hypertension had better GFR values (P < .01 for both). There was a significant correlation between GFR and all the tubular function (P < .05) variables (except excretion of chloride) measured. Compared to the patients with favorable renal outcome, the patients (n = 10) who later developed ESKD had significantly (P < .01) lower GFR and reduced urinary excretion of all measured electrolytes except calcium. Consistently, urine 0-2 microglobulin and serum parathyroid hormone and aldosterone values were significantly higher in the patients who developed ESKD (P CONCLUSION Both glomerular and tubular function decline was common and several parameters were likely to predict ESKD in posterior urethral valves patients. UROLOGY (C) 2020 Elsevier Inc.Peer reviewe

Physical performance after pediatric solid organ transplantation

Introduction Low physical activity is a well-recognized problem in pediatric solid organ transplant recipients; however, little is known about the differences between transplant groups. Physical performance testing was performed in a cohort of pediatric kidney, liver, and heart transplant recipients. Methods Fifty-one patients (54.9% boys), including 17 liver, 20 kidney, 2 combined liver-kidney, and 12 heart transplant recipients, were tested at the median age of 11.5 (7.5-14.9) years. The results were compared with a control group, which consisted of 425 healthy schoolchildren. The physical performance test included six different tests of endurance, strength, flexibility, and speed. Results The transplant recipients performed worse on most tests when compared with the control subjects (leg-lift test 42.0 vs. 44.9 repetitions, p = .002; repeated squatting 21.6 vs. 23.9 repetitions, p < .001; sit-up test 9 vs. 17 vs. 9 repetitions, p < .001, back extension 20 vs. 35 repetitions, p < .001; and shuttle run test 26.5 vs. 23.7 seconds, p < .001). None of the test results differed statistically significantly between the transplant groups. Conclusion The physical performance of pediatric solid organ transplant recipients is lower than that of their healthy peers but do not differ between different transplant groups. More systematic rehabilitation programs and follow-up are needed.Peer reviewe

Cancer morbidity and mortality after pediatric solid organ transplantation-a nationwide register study

Background The prevalence of malignancies after pediatric solid organ transplantation was evaluated in a nationwide study. Methods All patients who had undergone kidney, liver, or heart transplantation during childhood between the years 1982 and 2015 in Finland were identified. The inclusion criteria were age under 16 years at transplantation and age over 18 years at the last follow-up day. A total of 233 (137 kidney, 53 liver, and 43 heart) transplant recipients were enrolled. Controls (n = 1157) matched by the year of birth, gender, and hometown were identified using the Population Register Center registry. The cancer diagnoses were searched using the Finnish Cancer Registry. Results Altogether 26 individuals diagnosed with cancer were found, including 18 transplant recipients. Cancer was diagnosed at a median of 12.0 (IQR 7.8-17.8) years after the transplantation. The transplant recipients' risk for cancer was significantly higher when compared with the controls (HR 14.7; 95% CI 6.4-33.9). There was no difference for different graft types. Sixty-one percent of cancers among the transplant recipients were diagnosed at age older than 18 years. Conclusion The risk for cancer is significantly higher among young adults having undergone solid organ transplantation during childhood in comparison with population controls. Careful follow-up and attention to prevent cancers throughout adulthood are warranted.Peer reviewe

Reduction of inflammation by high-dose methylprednisolone does not attenuate oxidative stress in children undergoing bidirectional Glenn procedure with or without aortic arch or pulmonary arterial repair

Objective Corticosteroids attenuate inflammatory reaction in pediatric heart surgery. Inflammation is a source of free oxygen radicals. Children with a cyanotic heart defect are prone to increased radical stress during heart surgery. We hypothesized that high-dose methylprednisolone reduces inflammatory reaction and thereby also oxidative stress in infants with a univentricular heart defect undergoing bidirectional Glenn procedure. Design A double-blind, placebo-controlled, randomized clinical trial. Setting Operation theatre and pediatric intensive care unit of a university hospital. Participants Twenty-nine infants undergoing bidirectional Glenn procedure with or without aortic arch or pulmonary arterial repair. Interventions After anesthesia induction, the patients received intravenously either 30 mg/kg of methylprednisolone (n=15) or the same volume of saline as placebo (n=14). Measurements and Main Results Plasma interleukin-6, interleukin-8 and interleukin-10 (biomarkers of inflammation) as well as 8-hydroxydeoxyguanosine concentrations (a biomarker of oxidative stress) were measured at four different time points: preoperatively, during CPB, after protamine administration, and six hours postoperatively. The study parameters did not differ between the study groups preoperatively. Methylprednisolone reduced the pro-inflammatory cytokines interleukin-6 and interleukin-8 and increased the anti-inflammatory cytokine interleukin-10 postoperatively. Despite reduced inflammation, there were no differences in 8-hydroxydeoxyguanosine between the methylprednisolone and placebo groups. Conclusions Pro-inflammatory reaction and increase in free radical stress were not interrelated during congenital heart surgery in cyanotic infants with a univentricular heart defect undergoing bidirectional Glenn procedure. High-dose methylprednisolone was ineffective in attenuating free radical stress.Peer reviewe

Anemia and low-grade inflammation in pediatric kidney transplant recipients

Anemia and low-grade inflammation are reported to be associated with impaired long-term graft outcome in renal transplant (RTx) recipients. In this study, hemoglobin (Hb) and inflammation marker levels were correlated with measured glomerular filtration rate (GFR) in 128 pediatric RTx recipients over a median follow-up period of 10 years. Serum levels of erythropoietin (EPO), hepcidin-25, high-sensitivity C-reactive protein (CRP) (hsCRP) and interleukin-6 (IL-6) were analyzed by enzyme-linked immunosorbent assays, and GFR was analyzed by Cr-51-EDTA clearance. The median levels of Hb (115 g/L), hsCRP (0.4 mg/L) and IL-6 (1.4 pg/mL) and the median erythrocyte sedimentation rate (ESR; 19 mm/h) remained stable after the first post-operative year. However, approximately half of the patients had a normocytic, normochromic anemia, and one-third had elevated levels of hsCRP (> 1 mg/L) and ESR (> 25 mm/h), indicating continuous low-grade inflammation. Low Hb levels preceded increased fibrosis in protocol biopsies taken at 1.5 and 3 years after transplantation and preceded decreased GFR by several years. Hb levels showed an inverse correlation with EPO levels (r = -0.206, p = 0.038) and ESR (r = -0.369, p <0.001), but not with hepcidin-25, hsCRP or IL-6 levels. The levels of the major inflammatory markers IL-6 and hsCRP did not show a significant correlation with GFR at either the early maintenance phase or later. In the multivariable analysis, low Hb levels performed better than any other marker with respect to predicting concomitant and subsequent GFR. Anemia, but not elevated inflammatory indices, was associated with poor concomitant and subsequent graft function during a 10-year follow-up in pediatric RTx patients.Peer reviewe

IL-10 polymorphisms+434T/C,+504G/T, and-2849C/T may predispose to tubulointersititial nephritis and uveitis in pediatric population

Background Tubulointerstitial nephritis (TIN) and uveitis syndrome (TINU) are likely to be autoimmune diseases. Based on previous studies, adults with isolated idiopathic uveitis have polymorphisms in interleukin 10 (IL-10) and tumor necrosis factor a (TNF-alpha) genes. We aimed to evaluate the presence of IL-10 and TNF-alpha polymorphisms in a nationwide cohort of pediatric TIN/TINU patients. Methods Single nucleotide polymorphisms in IL-10 (+434T/C, +504G/T, -1082G/A, -2849C/T) and in TNF alpha (-308G/A, -238G/A, -857C/T) genes were genotyped in 30 well-defined pediatric patients with idiopathic TIN/TINU syndrome. Control group frequencies for these SNPs were obtained from 393 independent Finnish subjects. Results The homozygous minor allele in IL-10 +434T (rs2222202) and IL-10+504G (rs3024490) was found in all patients with TIN or TINU syndrome while the frequency of these minor alleles in the control population was 44% and 23%, respectively (p <0.001). In IL-10 SNP -2849 (rs6703630) a significant difference was found with genotype TT in all patients (p = 0.004) and in subgroups with TINU syndrome (p = 0.017) and TINU syndrome with chronic uveitis (p = 0.01) compared to reference population. There were no statistical differences in any of the studied TNF-alpha genotypes between TIN/TINU patients and control population. Conclusions A significant difference in the frequency of IL-10+434T and +504G alleles was found between TIN/TINU patients and control population. Genotype -2849TT was more frequently present in patients with TINU syndrome than in the reference subjects. Genetic variation in the inflammatory mediators may predispose to autoimmune nephritis and uveitis.Peer reviewe

Intestinal failure as a significant risk factor for renal impairment in children

Objective: Although impaired renal function has been a frequent finding among adults with intestinal failure (IF), the data on children is scarce. The aim of this study was to assess renal function in pediatric-onset IF. Methods: Medical records of 70 patients (38 boys) with pediatric-onset IF due to either short bowel syndrome (n = 59) or primary motility disorder (n = 11) and a history of parenteral nutrition (PN) dependency for >= 1 mo were evaluated. Renal function at the most recent follow-up was studied using plasma creatinine, cystatin C, and urea concentrations and estimated glomerular filtration rate (eGFR). Results: At a median age of 5.7 y and after PN duration of 3.2 y, 20 patients (29%) had decreased eGFR and higher cystatin C and urea concentrations. Patients with decreased renal function had significantly longer duration of PN (3.2 versus 0.9 y; P = 0.030) and shorter percentage of age adjusted small bowel length remaining (22 versus 32%; P = 0.041) compared with patients with preserved renal function. No other predisposing factors for decreased eGFR were identified. Conclusions: Patients with pediatric-onset IF are at significant risk for impaired renal function, which is associated with the duration of PN and the length of the remaining small bowel. In the present study, no other predisposing factors for decreased eGFR were found. Further studies using measured GFR are needed. (C) 2017 Elsevier Inc. All rights reserved.Peer reviewe