PCRRT Expert Committee ICONIC Position Paper on Prescribing Kidney Replacement Therapy in Critically Sick Children With Acute Liver Failure

Management of acute liver failure (ALF) and acute on chronic liver failure (ACLF) in the pediatric population can be challenging. Kidney manifestations of liver failure, such as hepatorenal syndrome (HRS) and acute kidney injury (AKI), are increasingly prevalent and may portend a poor prognosis. The overall incidence of AKI in children with ALF has not been well-established, partially due to the difficulty of precisely estimating kidney function in these patients. The true incidence of AKI in pediatric patients may still be underestimated due to decreased creatinine production in patients with advanced liver dysfunction and those with critical conditions including shock and cardiovascular compromise with poor kidney perfusion. Current treatment for kidney dysfunction secondary to liver failure include conservative management, intravenous fluids, and kidney replacement therapy (KRT). Despite the paucity of evidence-based recommendations concerning the application of KRT in children with kidney dysfunction in the setting of ALF, expert clinical opinions have been evaluated regarding the optimal modalities and timing of KRT, dialysis/replacement solutions, blood and dialysate flow rates and dialysis dose, and anticoagulation methods

The GoodHope Exercise and Rehabilitation (GEAR) Program for People With Ehlers-Danlos Syndromes and Generalized Hypermobility Spectrum Disorders

Introduction: The Ehlers-Danlos Syndromes (EDS) and Generalized Hypermobility Spectrum Disorders (G-HSD) comprise a heterogeneous group of genetic disorders of abnormal synthesis and/or maturation of collagen and other matricellular proteins. EDS is commonly characterized by manifestations such as multi joint hypermobility that can lead to musculoskeletal pains, subluxations and dislocations, fragile skin, organ dysfunction, and chronic significant diffuse pain with fatigue, deconditioning eventuating to poor quality of life. Evidence suggests exercise and rehabilitation interventions may ameliorate symptoms of unstable joints, recurrent subluxations/dislocations, and chronic widespread musculoskeletal pain. To date, there have only been a few reports describing exercise and rehabilitation care strategies for people with EDS.Methods: In this manuscript, we describe the GoodHope Exercise and Rehabilitation (GEAR) program, its overarching principles, as well as the program development and delivery model. The GEAR program aims to decrease functional impairment, reduce pain, increase confidence in symptom self-management, and provide a community of support for people with EDS/G-HSD. To achieve these goals, we detail the model of care that includes exercise and rehabilitation therapy, education for self-management, and support accessing relevant community resources.Strengths and Limitations of the Study: GEAR represents a novel exercise and rehabilitation care model for people with G-HSD and various clinical EDS subtypes, beyond the commonly included hEDS subtype. Systematic collection of data via validated measurements is ongoing and will guide the refinement of GEAR and support the development of emerging exercise and rehabilitation programs for people with EDS

Bilateral duplex system with overlooked dysplastic moiety: A rare cause of incontinence

Bilateral duplex system with ureteroceles is one of the rarest entities in urology. Incontinence in children is a frequently encountered problem; most often the cause is functional. Less often the cause could be structural such as an ectopic ureter; however bilateral complete renal duplex with bilateral ectopic ureteroceles as a cause of incontinence is even rarer. We herein report a case of bilateral duplex system ectopic ureters with ureteroceles with persistent urinary incontinence despite right upper heminephroureterectomy due to overlooked left dysplastic moiety. This article highlights the importance of thorough clinical and radiological evaluation of contralateral draining system when examining a case of unilateral complete renal duplex system with ureteral ectopia and/or ectopic ureterocele, especially in females to avoid delayed diagnosis

Renal manifestations of COVID 19 in children

Acute kidney injury (AKI) is an important factor affecting the outcome of hospitalized patients under any disease condition. While a lot has been said and studied about pulmonary manifestations of COVID-19 and multisystem inflammatory syndrome in children, this review focuses on its renal manifestations in children with and its complications. For the collection of data, the patient intervention control outcome model was applied to determine all eligible studies. The data was extracted using PubMed/Medline, Embase, and Google Scholar databases using a combination of keywords (AKI, renal failure, kidney disease, children, pediatric, covid-19, SARS COv2). Studies were reviewed after the exclusion of duplicates. The incidence of renal involvement in COVID 19 is up to 10–15%, which is higher than SARS. Both direct and indirect pathogenic mechanisms operate in patients with COVID 19 leading to varied manifestations. While AKI remains the most common manifestation in children admitted to intensive care units, other manifestations like, proteinuria, hematuria, rhabdomyolysis, and thrombotic microangiopathy have also been described in the literature. The children already on immunosuppression due to transplant or immune-mediated renal disorders do not seem to have more severe illness than those without it. The principles of management of AKI in COVID have not been different than other patient groups

Atypical lytic lesions of skull: Clinical and radiological correlation

Imaging alone cannot differentiate various isolated atypical lytic lesions involving the skull. Clinical and radiological correlation is mandatory in reaching to a diagnosis. Histopathology remains the gold standard. We describe few atypical cases presenting as isolated lytic lesions of skull with characteristic imaging findings and a brief clinical approach to reach towards the diagnosis