The migration percentage measured on EOS (R) standing full-leg radiographs: equivalent and advantageous in ambulant children with cerebral palsy

BACKGROUND: During ambulatory follow-up of patients with cerebral palsy (CP) systematic radiographic screening is required firstly to evaluate hip migration and development in the prevention of hip dislocation and secondly to analyse lower limb alignment and leg length. The Migration Percentage (MP) is a radiographic measurement used to describe the extent of femoral head lateralisation on conventional supine pelvic radiographs. Our goal was to assess the comparability of the MP measured on low radiation dose EOS® standing full-leg radiographs with that of conventional supine pelvic radiographs. METHODS: Patients presenting with CP were prospectively selected from our outpatient follow-up consultation at our institutions CP reference centre and underwent conventional supine pelvic and EOS® standing full-leg radiographs the same day for diagnostic and screening reasons. RESULTS: Out of 28 prospectively selected patients we included 21 (42 hips), of which 10 were female, with a mean age of 9.25 years and GMFCS levels of I, II and III. Seven out of 28 patients were excluded due to insufficient quality of radiographic images. The absolute differences in MP measured on both conventional supine pelvic and EOS® standing full-leg radiographs ranged between - 8 and 6% with an absolute mean difference of 0% (SD ±3.5) and were not statistically significant (p = 0.99). A Bland-Altman plot showed acceptable agreement between both measurements without proportional bias. CONCLUSION: There is no statistical significant difference between the Migration Percentage measured on conventional supine pelvic radiographs and EOS® standing full-leg radiographs in ambulant patients. These images use lower radiation doses and contain more radiographic information. TRIAL REGISTRATION: Approved by the Medical Research Ethics committee of the University Hospitals Leuven ( MP001492 ).status: publishe

Fulminant Wilson Disease in Children: Recovery After Plasma Exchange Without Transplantation

OBJECTIVES: Since 2005, a New Wilson Index (NWI) ≥11 is used as a predictor of death without transplantation in fulminant Wilson disease (WD). Plasma exchange is advocated as a new treatment modality. METHODS: We present a patient with fulminant WD treated with plasma exchange. All published cases applying plasma exchange for fulminant WD were reviewed systematically. RESULTS: A 14-year-old girl presented with hemolysis and fulminant liver failure. She had no encephalopathy; NWI was 14. As a bridge to transplantation plasma exchange was started immediately. Complete remission was achieved with plasma exchange and later chelation therapy with D-penicillamine. She is now at 3-year transplant-free survival. Literature review identified 37 patients presenting with fulminant WD and NWI ≥11 who were treated with plasma exchange. Seventeen of these patients (ie, 46%) recovered without transplantation. CONCLUSIONS: Multiple case reports and case series demonstrate transplant free survival after plasma exchange and subsequent chelation therapy, despite a NWI ≥11. Plasma exchange affects the clinical course and is a therapeutic option in children and young adults presenting with fulminant WD.status: publishe

The Effect of ss-Glucan Prebiotic on Kidney Function, Uremic Toxins and Gut Microbiome in Stage 3 to 5 Chronic Kidney Disease (CKD) Predialysis Participants: A Randomized Controlled Trial

There is growing evidence that gut dysbiosis contributes to the progression of chronic kidney disease (CKD) owing to several mechanisms, including microbiota-derived uremic toxins, diet and immune-mediated factors. The aim of this study was to investigate the effect of a ß-glucan prebiotic on kidney function, uremic toxins and the gut microbiome in stage 3 to 5 CKD participants. Fifty-nine participants were randomized to either the ß-glucan prebiotic intervention group (n = 30) or the control group (n = 29). The primary outcomes were to assess kidney function (urea, creatinine and glomerular filtration rate), plasma levels of total and free levels of uremic toxins (p-cresyl sulfate (pCS), indoxyl-sulfate (IxS), p-cresyl glucuronide (pCG) and indoxyl 3-acetic acid (IAA) and gut microbiota using 16S rRNA sequencing at baseline, week 8 and week 14. The intervention group (age 40.6 ± 11.4 y) and the control group (age 41.3 ± 12.0 y) did not differ in age or any other socio-demographic variables at baseline. There were no significant changes in kidney function over 14 weeks. There was a significant reduction in uremic toxin levels at different time points, in free IxS at 8 weeks (p = 0.003) and 14 weeks (p < 0.001), free pCS (p = 0.006) at 14 weeks and total and free pCG (p < 0.001, p < 0.001, respectively) and at 14 weeks. There were no differences in relative abundances of genera between groups. Enterotyping revealed that the population consisted of only two of the four enterotypes: Bacteroides 2 and Prevotella. The redundancy analysis showed a few factors significantly affected the gut microbiome: these included triglyceride levels (p < 0.001), body mass index (p = 0.002), high- density lipoprotein (p < 0.001) and the prebiotic intervention (p = 0.002). The ß-glucan prebiotic significantly altered uremic toxin levels of intestinal origin and favorably affected the gut microbiome