Highlights d Cities possess a consistent ''core'' set of non-human microbes d Urban microbiomes echo important features of cities and city-life d Antimicrobial resistance genes are widespread in cities d Cities contain many novel bacterial and viral species
Factor H autoantibodies can impair complement regulation, resulting in atypical hemolytic uremic syndrome, predominantly in childhood. There are no trials investigating treatment, and clinical practice is only informed by retrospective cohort analysis. Here we examined 175 children presenting with atypical hemolytic uremic syndrome in the United Kingdom and Ireland for factor H autoantibodies that included 17 children with titers above the international standard. Of the 17, seven had a concomitant rare genetic variant in a gene encoding a complement pathway component or regulator. Two children received supportive treatment; both developed established renal failure. Plasma exchange was associated with a poor rate of renal recovery in seven of 11 treated. Six patients treated with eculizumab recovered renal function. Contrary to global practice, immunosuppressive therapy to prevent relapse in plasma exchange–treated patients was not adopted due to concerns over treatment-associated complications. Without immunosuppression, the relapse rate was high (five of seven). However, reintroduction of treatment resulted in recovery of renal function. All patients treated with eculizumab achieved sustained remission. Five patients received renal transplants without specific factor H autoantibody–targeted treatment with recurrence in one who also had a functionally significant CFI mutation. Thus, our current practice is to initiate eculizumab therapy for treatment of factor H autoantibody–mediated atypical hemolytic uremic syndrome rather than plasma exchange with or without immunosuppression. Based on this retrospective analysis we see no suggestion of inferior treatment, albeit the strength of our conclusions is limited by the small sample size.
Objectives To describe the epidemiological and clinical characteristics and outcome of hospitalized children with COVID-19 during the initial phase of the pandemic. Methods This was a cross-sectional descriptive study conducted at the dedicated COVID-19 hospital of a tertiary care referral center in North India. Consecutive children aged 14 y or younger who tested positive for SARS-CoV-2 by RT-PCR from nasopharyngeal swab between 1 April 2020 and 15 July 2020 were included. Results Of 31 children with median (IQR) age of 33 (9-96) mo, 9 (29%) were infants. About 74% (n = 23) had history of household contact. Comorbidities were noted in 6 (19%) children. More than half (58%) were asymptomatic. Of 13 symptomatic children, median (IQR) duration of symptoms was 2 (1-5.5) d. Fever (32%) was most common followed by cough (19%), rapid breathing (13%), diarrhea (10%) and vomiting (10%). Severe [n = 4, 13%] and critical [n = 1, 3%] illnesses were noted more commonly in infants with comorbidities. Three (10%) children required PICU admission and invasive ventilation; one died. Median (IQR) length of hospital stay was 15 (11-20) d. Follow up RT-PCR before discharge was performed in 17 children and the median (IQR) duration to RT-PCR negativity was 16 (12-19) d. Conclusions In the early pandemic, most children with COVID-19 had a household contact and presented with asymptomatic or mild illness. Severe and critical illness were observed in young infants and those with comorbidities.
Children with chronic kidney disease stage 5 requiring dialysis can be treated by peritoneal or hemodialysis. In the United Kingdom nearly twice as many children receive peritoneal dialysis compared with hemodialysis. Technical aspects of pediatric hemodialysis are challenging and include the relative size of extracorporeal circuit and child's blood volume, assessment of adequacy,technical and complications of vascular access. Alternatives to standard hospital-based hemodialysis are also increasingly available. Optimizing nutritional status with the support of specialist pediatric dietitians is key to the management of children receiving hemodialysis. The effects of chronic illness on growth and school achievement, as well as the psychological, emotional, and social development of the child should not be underestimated. This review focuses on the above elements and highlights common pediatric practice in the United Kingdom.
Background Biomarkers and dual-energy X-ray absorptiometry (DXA) are thought to be poor predictors of bone mineral density (BMD). The Kidney Disease: Improving Global Outcomes guidelines suggest using DXA if the results will affect patient management, but this has not been studied in children or young adults in whom bone mineral accretion continues to 30 years of age. We studied the clinical utility of DXA and serum biomarkers against tibial cortical BMD (CortBMD) measured by peripheral quantitative computed tomography, expressed as Z-score CortBMD, which predicts fracture risk. Methods This was a cross-sectional multicentre study in 26 patients with CKD4 and 5 and 77 on dialysis. Results Significant bone pain that hindered activities of daily living was present in 58%, and 10% had at least one low-trauma fracture. CortBMD and cortical mineral content Z-scores were lower in dialysis compared with CKD patients (P = 0.004 and P = 0.02). DXA BMD hip and lumbar spine Z-scores did not correlate with CortBMD or biomarkers. CortBMD was negatively associated with parathyroid hormone (PTH; r = −0.44, P < 0.0001) and alkaline phosphatase (ALP; r = −0.22, P = 0.03) and positively with calcium (Ca; r = 0.33, P = 0.001). At PTH <3 times upper limit of normal, none of the patients had a CortBMD below −2 SD (odds ratio 95% confidence interval 7.331 to infinity). On multivariable linear regression PTH (β = −0.43 , P < 0.0001), ALP (β = −0.36, P < 0.0001) and Ca (β = 0.21, P = 0.005) together predicted 57% of variability in CortBMD. DXA measures did not improve this model. Conclusions Taken together, routinely used biomarkers, PTH, ALP and Ca, but not DXA, are moderate predictors of cortical BMD. DXA is not clinically useful and should not be routinely performed in children and young adults with CKD 4–5D.
There are five genetic mutations identified to date which classify BS in to five different types. Clinical presentation and severity varies with each type [4,5,[7][8][9]. BS I, II and IV tend to present antenatally or in the neonatal period. BS III and V tend to have milder symptoms and present later in life. The correlation between genotype and the observed phenotype is variable and clinical manifestations and disease severity often differ [8,10,11].
Introduction Cardiovascular disease (CVD) is a common cause of morbidity and mortality even in young people with chronic kidney disease (CKD). We examined structural and functional cardiovascular changes in patients with CKD stages 4-5 and on dialysis under 30 years of age. Methods 79 children and 21 young adults underwent cardiac CT for coronary artery calcification (CAC), ultrasound for carotid intima media thickness (cIMT), carotid-femoral pulse wave velocity (cfPWV), and echocardiography. Differences in structural [CAC, cIMT z-score, left ventricular mass index] and functional [carotid distensibility z-score, cfPWV z-score] measures were examined between CKD stages 4-5 and dialysis patients. Results Overall the cIMT z-score was raised (median 2.17, IQR 1.14-2.86) and 10 (10%) had CAC. 16/23(69.5%) of CKD4-5 and 68/77(88.3%) on dialysis had at least one structural or functional CV abnormality. There was no difference in the prevalence of structural abnormalities in CKD or dialysis cohorts, but functional abnormalities were more prevalent in patients on dialysis (p < 0.05). The presence of > 1 structural abnormality was associated with a 4.5-fold increased odds of > 1 functional abnormality (95% CI 1.3 to 16.6, p < 0.05). Patients with structural and functional abnormalities (cIMT z-score >2SD or distensibility <-2SD) had less carotid dilatation (lumen/wall cross sectional areas ratio) compared to those with normal cIMT and distensibility. Conclusion There is a high burden of subclinical cardiovascular disease in young CKD patients, with a greater prevalence of functional abnormalities in dialysis compared to CKD patients. Longitudinal studies are required to test these hypothesis generating data and define the trajectory of CV changes in CKD.
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