Background and objectives: Atypical hemolytic uremic syndrome (aHUS) is associated with a congenital or acquired dysregulation of the complement alternative pathway that leads to continuous complement activation on host cells causing inflammation and damage. Eculizumab, a humanized mAb against complement protein C5, inhibits activation of the terminal complement pathway.Design, setting, participants, & measurements: We report an adolescent with relapsing unclassified aHUS. On admission, a high plasma creatinine level indicated a poor prognosis, and hemodialysis had to be started. Plasma exchanges were initially effective against the microangiopathic hemolytic activity and allowed a temporary improvement of renal function with termination of hemodialysis after 7 wk. Subsequently, plasma exchanges (three times per week) failed to prevent ongoing aHUS activity and progressive renal failure. After 12 wk, aHUS treatment was switched to eculizumab.Results: Eculizumab was effective in terminating the microangiopathic hemolytic process in two aHUS relapses; however, after normalization of complement activity, aHUS recurred and ultimately led to anuric end-stage renal failure.Conclusions: In this patient, complement inhibition by eculizumab temporarily terminated the microangiopathic hemolytic activity. Nevertheless, renal damage as a result of preceding and subsequent aHUS activity resulted in end-stage renal failure; therefore, therapeutic success may depend on early administration of eculizumab. The optimal duration of treatment may be variable and remains to be determined.
We describe the case of an 18-year-old girl with chronic recurrent multifocal osteomyelitis (CRMO) over a period of 10 years. She had suffered predominantly from very painful recurrent swelling of her cheeks. Various therapeutic regimens including nonsteroidal antiinflammatory drugs and steroids had shown only a partial or temporary response. Because tumor necrosis factor-alpha-blocking agents have been successfully applied in Crohn's-associated CRMO and the related SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome, tumor necrosis factor-alpha-blocking therapy with infliximab was initiated. Thereafter, apart from 1 mild episode, no additional recurrences were observed during 21 months of follow-up. Infliximab was well tolerated, and steroids were tapered off. Our observation indicates that infliximab may be an effective therapeutic option in CRMO.
Our objective was to assess the prevalence and history of arteriovenous fistula after renal biopsy in pediatric patients by the use of color Doppler sonography. Fifty patients, aged 6 days to 18.5 years, underwent sonographically guided renal biopsy. Color Doppler sonography was performed prior to, during, and after the procedure. Postbiopsy hematoma was detected in 46 patients (92%), of whom 10 suffered clinically significant bleeds. Six patients (age range, 8 to 18.5 years) developed postbiopsy arteriovenous fistula. In three cases the arteriovenous fistula diminished spontaneously, one persisted without symptoms, and two symptomatic arteriovenous fistulas needed coil implantation. Color Doppler sonography demonstrated all arteriovenous fistulas 4 hours after the procedure and duplex Doppler sonography demonstrated the flow disturbances in the feeding and draining vessels. We conclude that color Doppler sonography allows noninvasive detection and follow-up study of postbiopsy arteriovenous fistula, thus helping to define management after interventional procedures.
The aim of this study was to describe the potential of three-dimensional ultrasound (3DUS) in the urinary tract of neonates, infants, and children. The potential applications are illustrated based on our experience in 80 patients using two different 3DUS techniques. Various disease entities throughout the neonatal and pediatric age have been evaluated. The potential of 3DUS is discussed based on comparison with conventional 2DUS or other imaging (as clinically indicated), focused on the potentially improved renal parenchymal volume assessment. In our experience, 3DUS is feasible in neonates, infants, and children without sedation. It reduces imaging time, improves demonstration of complex anatomy and allows for evaluation of anatomy/pathology in any plane. The 3DUS improves volume assessment and follow-up comparison by offering an improved standardization and documentation. Rendered views of the dilated collecting system enable a comprehensive demonstration of hydronephrosis similar to intravenous urography or MR urography images. Additionally, 3DUS offers an ideal tool for training and education. Yet, limitations have to be acknowledged: areas inaccessible for 2DUS; poor quality of the original 2DUS acquisition; limited resolution; patient motion and breathing; cardiac pulsation creating artifacts and misregistration; equipment cost; lack of 3D DICOM standards creating problems with data storage; as well as system-inherent technical limitations. Nevertheless, the 3DUS holds the potential to become a valuable additional imaging tool for sonographic evaluation of the pediatric urinary tract.
Sonography was compared to pH-metry and/or oesophagomanometry to evaluate the accuracy of sonography in the early diagnosis of gastro-oesophageal reflux. Thirty children with a mean age of 72 days (21-252 days) were studied. The results showed that specificity of sonographic diagnosis was 87.5% and sensitivity was 100% (with P less than 0.001). Sonography also proved helpful in providing both functional and morphological data in addition to pH-metric results. This study therefore suggests that sonography is useful as the first approach in the diagnosis of vomiting babies as it is non-invasive and provides sufficient diagnostic accuracy.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.