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Background Exposure to triclosan, an antimicrobial chemical used in some personal care and cleaning products, has been associated with reduced birth weight in some, but not all epidemiological studies. Objectives We conducted a systematic review and meta-analysis to characterize the relation of gestational triclosan exposure with infant birth weight and identify sources of heterogeneity between studies. Methods We identified original studies measuring urinary triclosan concentrations during pregnancy and reporting their association with infant birth weight, gestational age (GA) adjusted birth weight (g), or GA-standardized birth weight z-scores. Using a random effects model, we estimated differences in these outcomes per 10-fold increase in triclosan concentrations and considered triclosan levels and infant sex as sources of heterogeneity. Using Navigation Guide Methods, we evaluated risk of bias within individual studies and across the body of evidence. Results Among thirteen studies, median triclosan concentrations varied by almost 2-orders of magnitude (0.6–29 ng/mL), with higher concentrations in North American and some European studies compared to Asian ones. Associations between triclosan and birth weight ( β :-20 g; 95% CI:-65, 26; n = 6) were stronger than those for GA-adjusted birth weight ( β :-12 g; 95% CI:-29, 5; n = 9). Triclosan was not associated with GA-standardized birth weight z-scores ( β :-0.04; 95% CI:-0.16, 0.07; n = 5). The association between triclosan and GA-adjusted birth weight was stronger in studies with median triclosan values ≥ 10 ng/mL compared to studies with median values β :-27 g; 95% CI:-61, 7; n = 4 vs. β :6g; 95% CI:-20, 31; n = 5). With a limited number of studies, we observed suggestive evidence that inverse associations were more apparent in studies with ≥ 2 prospective triclosan measures compared to those with one measure. Discussion Available evidence, with “low” risk of bias, provides limited evidence that triclosan exposure and reduces infant birth weight. We observed stronger inverse associations between triclosan concentrations and birth weight in populations with higher triclosan exposure.

The prevalence of suboptimal Vitamin D levels is higher in patients with chronic kidney disease (CKD) than in the general population. Recent findings suggest that progression of CKD is linked to a suboptimal Vitamin D level. A high percentage of CKD patients have severe Vitamin D deficiency. These patients also have a low level of 25-hydroxy-vitamin D [25(OH)D] and consequently, a reduced ability to form active 1,25-dihydroxyvitamin D. Various factors underlie the low level of 25(OH)D, including a sedentary lifestyle, decreased intake of Vitamin D due to CKD-related dietary restrictions, and decreased synthesis of Vitamin D in skin due to uremia. All these factors may be particularly influential in patients with progressively worsening CKD, including those receiving chronic dialysis. The objective of our study is to determine the prevalence of Vitamin D deficiency in children with CKD stages three to five and those receiving chronic dialysis, to ascertain whether there is a relationship between Vitamin D deficiency and the stage of CKD, and to identify any clinical correlates associated with the Vitamin D status. A single-center, retrospective review was conducted of 46 children (younger than 18 years) with CKD stages 3-5D who attended the renal clinic of the Red Cross Children's Hospital between October 2013 and November 2014. In total, 73.9% of the study population had suboptimal Vitamin D levels (43.5% and 30.4% had Vitamin D deficiency and insufficiency, respectively). The prevalence of Vitamin D deficiency was significantly higher in older children (≥10 years of age) than in younger children (P = 0.000) but did not significantly differ between males and females (P = 0.693). In total, 12 of 15 black children (80%), 19 of 26 colored children (73.1%), two of four white children (50%), and one Asian child (100%) had suboptimal Vitamin D levels. Neither white nor Asian child had Vitamin D deficiency. In addition, 90% of patients undergoing chronic dialysis, 80% of whom were receiving peritoneal dialysis, had suboptimal Vitamin D levels. Age, weight, height, and the albumin concentration were significantly associated with the Vitamin D level. There was a positive linear relationship between the Vitamin D level and the serum albumin concentration (Spearman's rho correlation coefficient = 0.397, P = 0.007). In total, 87.5% of patients with nephrotic-range proteinuria had suboptimal Vitamin D levels, and 80% were Vitamin D deficient (P = 0.004). A higher percentage of Vitamin D deficiency/insufficiency cases was documented during the winter (24/34, 70.6%) than during the summer (10/34, 29.4%); however, this difference was not statistically significant (P = 0.685). Sub-optimal Vitamin D is high among children with moderate to severe CKD and significantly higher in those undergoing chronic dialysis. The emerging evidence of the role of Vitamin D in slowing progression of CKD highlights the need for monitoring and correction of Vitamin D levels in predialysis children.

Background Systemic lupus erythematosus (SLE) is a life-threatening multisystem autoimmune disease that is more severe in patients of African ancestry and children, yet pediatric SLE on the African continent has been understudied. This study describes a cohort of pediatric SLE (PULSE) patients in South Africa. Methods Patients with a diagnosis of SLE (1997 American College of Rheumatology criteria) diagnosed prior to age 19 years in Cape Town, South Africa, were enrolled in this cross-sectional study from September 2013 to December 2014. Information on clinical and serological characteristics was extracted from medical records. Results were compared to a well-described North American pediatric SLE cohort. Results Seventy-two South African patients were enrolled in the study; mean age 11.5 years; 82% were girls. The racial distribution was 68% Coloured, 24% Black, 5% White and 3% Asian/Indian. Most patients presented with severe lupus nephritis documented by renal biopsy (61%). Of patients with lupus nephritis, 63% presented with International Society of Nephrology/Renal Pathology Society class III or IV. Patients in the PULSE cohort were more likely to be treated with cyclophosphamide, methotrexate and azathioprine. The PULSE cohort had high disease activity at diagnosis (mean Systemic Lupus Erythematosus Disease Activity Index-2K (SLEDAI-2K) 20.6). The SLEDAI-2K at enrolment in the PULSE cohort (5.0) did not differ from the North American pediatric SLE cohort (4.8). Sixty-three per cent of the PULSE cohort had end organ damage with Systemic Lupus International Collaborating Clinics Damage Index (SLICC-DI) score >0 (mean SLICC-DI 1.9), compared to 23% in a previously reported US cohort. Within the PULSE cohort, nine (13%) developed end-stage renal disease with six (8%) requiring transplant, strikingly higher than North American peers (transplant rate Conclusions The PULSE cohort had highly active multiorgan disease at diagnosis and significant disease damage at enrolment in the South African registry. South African patients have severe lupus nephritis and poor renal outcomes compared to North American peers. Our study revealed a severe disease phenotype in the PULSE cohort resulting in poor outcomes in this high-risk population.

Microsporidia are an emerging group of pathogens associated with life-threatening opportunistic infections in immunocompromised hosts, particularly human immunodeficiency virus (HIV)-infected individuals. There have, however, been recent reports of infection in adult solid organ transplant recipients. We report two cases in children, to our knowledge the first in the paediatric literature. Two 13-yr-old, HIV-seronegative females received deceased donor renal transplants from the same donor. Both patients suffered acute cell-mediated rejection and CMV infection reactivation, managed with intensified immunosuppression and ganciclovir. Pyrexia of unknown origin and intermittent diarrhea in both prompted extensive investigations. In both patients, numerous spores of a microsporidial species were demonstrated in renal tissue on biopsy and in the urine, using modified trichrome and quick-hot Gram-chromotrope staining. Electron microscopy and PCR confirmed Encephalitozoon cuniculi infections. Both patients were successfully treated with 400 mg twice daily of albendazole, with sustained clinical improvement. We recommend that microsporidiosis be considered in the differential diagnosis of pyrexia of unknown origin in severely immunocompromised pediatric solid organ transplant recipients, particularly when associated with diarrhea.

Criticism against the use of acute peritoneal dialysis (PD) has been its low clearance and low ultrafiltration (UF) volumes compared to extracorporeal techniques. The aim of our study was to determine whether continuous flow peritoneal dialysis (CFPD) would improve UF in children with acute kidney injury (AKI) in cases where UF on conventional PD was inadequate using 4.25 % glucose concentrations.Five infants were prospectively studied. All had AKI with fluid overload. The median age of the patients was 6 (range 0.43-9) months; the median weight was 6.5 (range 2.7-8.4) kg. Each patient served as his or her own control, undergoing both CFPD and conventional PD. CFPD was performed with two bedside-placed catheters using a 2.5 % glucose concentration. After initial filling, a dialysate flow rate of 100 ml/min/1.73 m(2) was maintained with an adapted continuous venovenous haemofiltration machine. The UF flow rate was set at 2.5 ml/min/1.73 m(2) and adapted as necessary. UF and clearance rates were measured for both PD and CFPD.The median UF rate achieved was 1.7 (range 0.01-5.30) mg/kg/h with conventional PD versus 6.7 (range 2.17-15.7) mg/kg/h with CFPD (p = 0.042). The clearances of urea and creatinine were 6.89 (range 4.50-7.55) and 7.46 (range 4.79-10.50) mL/min/1.73 m(2), respectively, with conventional PD and 19 (17.0-30.0) and 41 (standard deviation17.4, range 12.0-52.0) mL/min/1.73 m(2), respectively, with CFPD (both p = 0.043).Continuous flow peritoneal dialysis improves UF in fluid overloaded infants who are not achieving adequate UF on conventional PD.

Urological complications which develop post-renal transplantation can be associated with significant morbidity especially in children. We evaluated the occurrence and management of all urological complications in a series of unstented pediatric renal transplants in a tertiary pediatric hospital. We reviewed the medical records of children who underwent unstented renal transplant between January 1996 and December 2014. Postoperative urological complications and the outcomes of their management were analyzed. A total of 160 unstented renal transplants were performed, and 32 urological complications were noted in 29 transplants (18%). There were 20 boys and nine girls with an age range of 2.5 years to 18.4 years. Nine (31%) of these patients had LUTD. The most common complication was VUR occurring in 17 patients (10.6%). Urine leaks occurred in six patients (3.8%) and ureteric obstruction in six patients (3.8%), and three patients (1.9%) had unexplained hydronephrosis. Loss of graft occurred in three patients (1.9%), and one patient died from sepsis post-uretero-ureterostomy. Patients with LUTD had more urological complications (P = .037). Unstenting is feasible in most pediatric renal transplants. LUTD is associated with a higher incidence of urological complications, especially VUR.

Surgery for reno-vascular hypertension (RVH) is complex, and the techniques utilized vary with anatomical presentations of the disease. The long-term outcome of revascularization on RVH in children with Takayasu's arteritis (TA)-induced renal artery stenosis (RAS) at our centre was reviewed.This study was a 21-year retrospective review of pre- and post-intervention RVH in children with angiographically confirmed RAS. The outcome of hypertension was defined as follows: (1) cured (normotensive off anti-hypertensives), (2) improved (normotensive on same or reduced number of medications), or (3) failure (no cure or improvement in number of medications).The medical histories of 59 children (median age 9.98 years) were reviewed, of whom 20 (44 %) had revascularization procedures. All were hypertensive, with a mean systolic and diastolic blood pressure of 161.5 ± 36 and 106.5 ± 31 mmHg, respectively. RAS was present in 45 (76.3 %) children. Twenty-four revascularization procedures were performed in 20 children (44 %), of whom five had contralateral nephrectomies. Outcome was available for 17 patients at the 3- and 6-months follow-up, with cure, improvement and failure rates at 3 months of 2/17 (11.8 %), 7/17 (41.2 %) and 8/19 (47 %), respectively, and similar rates at 6 months. Associations between outcome and age (p = 0.51), sex (p = 0.32), number of pre-surgery anti-hypertensives (p = 0.18) and stenosis sites (p = 0.22) were not statistically significant.Revascularization was beneficial to the management of blood pressure control in about half of our RVH patients.

The mutations responsible for cystinosis in South African patients are currently unknown. A pertinent question is whether they are similar to those described elsewhere in the world.Children who were being managed for cystinosis in the Western Cape Province of South Africa between 2002 and 2013 were studied. All underwent molecular analysis to detect sequence variations in the cystinosis gene.This cohort study included 20 patients, 13 of whom were Xhosa-speaking black South Africans and seven were Cape Coloureds (mixed race); none were Caucasian. All had nephropathic infantile-type cystinosis with evidence of proximal tubulopathy, with glycosuria and renal phosphate wasting. Diagnosis was confirmed in 19 cases by demonstrating an elevated cystine concentration in leukocytes. Molecular analysis of the cystinosin gene revealed that 19 patients had a G A mutation in intron 11 (CTNS-c.971-12G A p.D324AfsX44) which caused an out-of-frame 10-bp insertion. Of these 19 patients, 16 were homozygous for this mutation, which was the most frequent mutation identified in the alleles of the black South African and Cape Coloured patients (96 and 71 %, respectively).We recommend that black South African and Cape Coloured patients presenting with cystinosis be tested for CTNS-c.971-12G A in the first instance, with the possibility of prenatal testing being offered to at-risk families.

Background . Renal artery revascularisation procedures are usually carried out on children with renal artery stenosis from varied causes, including Takayasu’s arteritis. Reports on the outcome of such procedures in children usually refer to the improvement in blood pressure, with only minimal mention of effects on renal function. Objective . Salvageability renal function in children who underw ent renal revascularisation for Takayasu’s arteritis-induced renal artery stenosis (TARAS) was the focus of this study. Methods . We undertook a retrospective analysis of children aged ≤16 years with angiographically confirmed TARAS who underwent renal artery revascularisation procedures between 1990 and 2010. Outcomes of renal function were studied over a period of 2 years and were defined as: (i) improvement: >20% increase in estimated glomerular filtration rate (e-GFR) from presurgery value; (ii) stabilisation: e-GFR within 20% of presurgery value; and (iii) failure: >20% deterioration in e-GFR from presurgery value. The GFR was estimated using the Schwartz formula. Results . Twenty children (9 males and 11 females, age range 2 - 14 years) had 27 renal artery revascularisation procedures. Thirteen of the patients (65.0%) had bilateral renal artery stenosis. The baseline mean e-GFR was 88.6 (standard deviation (SD) 25.4) mL/min/1.73 m 2 and the mean duration of follow-up was 28.80 (SD 25.62) months. All the patients had stable or improved renal function until the 2-year follow-up, when the proportion decreased to 92.3% (12/13), as failure was recorded in one child. Bilateral revascularisation was found to be significantly associated with improvement in renal function in the early postoperative period (p=0.04). Conclusion . Renal artery revascularisation procedures are successful in salvaging renal function in children with TARAS

Multidrug-resistant tuberculosis is increasingly common and is associated with long diagnostic delay and high morbidity. We present a 7-year-old child who developed steroid-resistant nephrotic syndrome while receiving treatment for tuberculosis. Renal biopsy results showed systemic amyloidosis; culture of peritoneal tissue confirmed disseminated multidrug-resistant tuberculosis.

The aim of this study was to determine the proportion of children who develop urinary tract infection (UTI) after kidney transplantation (KTx) and to identify the factors associated with UTI and its impact on graft function. To this end, we undertook a chart review of children who underwent KTx at Red Cross Children’s Hospital between January 2003 and December 2009 and were followed-up for at least 6 months after transplantation. Sixty-two children (53.2% males) were followed-up for a mean (standard deviation) period of 36.9 (19.7) months. Mean age at transplantation was 10.0 (4.6) years. Twenty-five (40.3%) children had 89 UTI episodes during the study period, equivalent to 0.94 UTI episodes per one patient-year of follow-up. Acute pyelonephritis occurred in 17 (27.4%) children; another 17 (27.4%) had multiple post-KTx UTI. Klebsiella (40.0%) and Escherichia (28.0%) were the commonest organisms. Those with post-KTx UTI were, at transplantation, younger (8.3 vs. 11.2 years; p = 0.017), had lower urinary tract abnormality (LUTA) (13 vs. 1; p = 0.000) and had pre-KTx UTI (13 vs. 5; p = 0.001). Multivariate analysis revealed that only age

International audience; Mutations of LAMB2 typically cause autosomal recessive Pierson syndrome, a disorder characterized by congenital nephrotic syndrome, ocular and neurologic abnormalities, but may occasionally be associated with milder or oligosymptomatic disease variants. LAMB2 encodes the basement membrane protein laminin β2 which is incorporated in specific heterotrimeric laminin isoforms and has an expression pattern corresponding to the pattern of organ manifestations in Pierson syndrome. Herein we review all previously reported and several novel LAMB2 mutations in relation to the associated phenotype in patients from 39 unrelated families. The majority of disease-causing LAMB2 mutations are truncating, consistent with the hypothesis that loss of laminin β2 function is the molecular basis of Pierson syndrome. While truncating mutations are distributed across the entire gene, missense mutations are clearly clustered in the N-terminal LN domain, which is important for intermolecular interactions. There is an association of missense mutations and small in frame deletions with a higher mean age at onset of renal disease and with absence of neurologic abnormalities, thus suggesting that at least some of these may represent hypomorphic alleles. Nevertheless, genotype alone does not appear to explain the full range of clinical variability, and therefore hitherto unidentified modifiers are likely to exist.

Contains fulltext : 89800raaijmakers.pdf (Publisher’s version ) (Closed access) Peritonitis is a frequent complication of peritoneal dialysis (PD) in children as well in adults. Data on PD and peritonitis in pediatric patients are very scarce in developing countries. A retrospective cohort study was performed between 2000 and 2008 with the aim to evaluate PD treatment and peritonitis epidemiology in pediatric patients in South Africa and identify risk factors for peritonitis. Baseline characteristics and potential risk factors of peritonitis were recorded, including housing, socio-economic circumstances, distance to PD center, type of PD, mode of catheter placement, race, presence of gastrostomy tube, weight, and height. Outcome indices for peritonitis were peritonitis rate, time to first peritonitis, and number of peritonitis-free patients. The patient cohort comprised 67 patients who were on PD for a total of 544 months. The total number of peritonitis episodes was 129. Median peritonitis rate was one episode every 4.3 patient months (2.8 episodes/patient-year, range 0-21.2). Median time to first infection was 2.03 months (range 0.1-21.5 months), and 28.4% of patients remained free from peritonitis. Patients with good housing and good socio-economic circumstances had a significantly lower peritonitis rate and a longer time to first peritonitis episode. Peritonitis rate was high in this cohort, compared to numbers reported for the developed world; the characteristics of causative organisms are comparable. The most important risk factors for the development of peritonitis were poor housing and poor socio-economic circumstances. More intensive counseling may be beneficial, but improvement of general socio-economic circumstances will have the greatest influence on PD success. 01 oktober 2010