Cost analysis of telemedicine use in paediatric nephrology —the LMIC perspective
ConclusionsThe use of telemedicine as a follow-up method helps save significant costs and distances travelled by patients.Graphical abstractA higher-resolution version of the Graphical abstract is available asSupplementary information (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 24, 2023 Category: Urology & Nephrology Source Type: research
The manifestations of metabolic acidosis during acetazolamide treatment in a cohort of pediatric idiopathic intracranial hypertension
ConclusionsAlthough laboratory finding of metabolic acidosis is common among patients with idiopathic intracranial hypertension treated with acetazolamide, it is not correlated with clinics. Therefore, we recommend sending blood tests during acetazolamide treatment based on clinical judgment.Graphical abstractA higher resolution version of the Graphical abstract is available as Supplementary information (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 22, 2023 Category: Urology & Nephrology Source Type: research
Constipation and hemolytic uremic syndrome
ConclusionDiagnosis of aHUS remains a diagnosis of exclusion, whereby other causes of HUS are eliminated with reasonable certainty. Exclusion of STEC is necessary and relies on testing availability and recognition of testing limitations. Diarrhea-negative STEC-HUS remains a minority of cases, and future research is needed to explore the clinical characteristics of these patients. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 20, 2023 Category: Urology & Nephrology Source Type: research
Impact of kidney biopsy on deciding when to initiate enzyme replacement therapy in children with Fabry disease
ConclusionsWe demonstrate that histological lesions, typical for FD, can be observed in kidney biopsies at a very young age in patients without classical clinical symptoms or laboratory abnormalities. Thus, we recommend kidney biopsies as a possible tool for early diagnosis of renal involvement in FD. As a consequence of these early biopsy findings without a clinical correlate, an early initiation of ERT should be considered.Graphical abstractA higher resolution version of the Graphical abstract is available asSupplementary information (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 20, 2023 Category: Urology & Nephrology Source Type: research
Ocular manifestations of congenital anomalies of the kidney and urinary tract (CAKUT)
This study reviewed diseases associated with the Genomics England CAKUT-associated gene panel for ocular anomalies. In addition, each gene was examined for expression in the human retina and an ocular phenotype in mouse model s using the Human Protein Atlas and Mouse Genome Informatics databases, respectively. Thirty-four (54%) of the 63 CAKUT-associated genes (55 ‘green’ and 8 ‘amber’) had a reported ocular phenotype. Five of the 6 most common CAKUT-associated genes (PAX2,EYA1,SALL1,GATA3,PBX1) that represent 30% of all diagnoses had ocular features. The ocular abnormalities found with most CAKUT-associated genes...
Source: Pediatric Nephrology - July 20, 2023 Category: Urology & Nephrology Source Type: research
Constipation and hemolytic uremic syndrome
ConclusionDiagnosis of aHUS remains a diagnosis of exclusion, whereby other causes of HUS are eliminated with reasonable certainty. Exclusion of STEC is necessary and relies on testing availability and recognition of testing limitations. Diarrhea-negative STEC-HUS remains a minority of cases, and future research is needed to explore the clinical characteristics of these patients. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 20, 2023 Category: Urology & Nephrology Source Type: research
Impact of kidney biopsy on deciding when to initiate enzyme replacement therapy in children with Fabry disease
ConclusionsWe demonstrate that histological lesions, typical for FD, can be observed in kidney biopsies at a very young age in patients without classical clinical symptoms or laboratory abnormalities. Thus, we recommend kidney biopsies as a possible tool for early diagnosis of renal involvement in FD. As a consequence of these early biopsy findings without a clinical correlate, an early initiation of ERT should be considered.Graphical abstractA higher resolution version of the Graphical abstract is available asSupplementary information (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 20, 2023 Category: Urology & Nephrology Source Type: research
Ocular manifestations of congenital anomalies of the kidney and urinary tract (CAKUT)
This study reviewed diseases associated with the Genomics England CAKUT-associated gene panel for ocular anomalies. In addition, each gene was examined for expression in the human retina and an ocular phenotype in mouse model s using the Human Protein Atlas and Mouse Genome Informatics databases, respectively. Thirty-four (54%) of the 63 CAKUT-associated genes (55 ‘green’ and 8 ‘amber’) had a reported ocular phenotype. Five of the 6 most common CAKUT-associated genes (PAX2,EYA1,SALL1,GATA3,PBX1) that represent 30% of all diagnoses had ocular features. The ocular abnormalities found with most CAKUT-associated genes...
Source: Pediatric Nephrology - July 20, 2023 Category: Urology & Nephrology Source Type: research
Resource use and costs of transitioning from pediatric to adult care for patients with chronic kidney disease
ConclusionsA structured transition pathway for patients with CKD is resource and time –consuming due to the complexity of the disease and should be sufficiently funded.Graphical AbstractA higher-resolution version of the Graphical abstract is available asSupplementary information (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 19, 2023 Category: Urology & Nephrology Source Type: research
Associations between collagen X biomarker and linear growth velocity in a pediatric chronic kidney disease cohort
ConclusionsCXM measurement is potentially a valuable aid for monitoring growth in pediatric CKD. However, future research, including studies of CXM metabolism, is needed to clarify whether CXM can be a surrogate of growth in children with CKD.Graphical AbstractA higher resolution version of the Graphical abstract is available asSupplementary information (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 19, 2023 Category: Urology & Nephrology Source Type: research
Tolvaptan and urea in paediatric hyponatraemia
ConclusionsTolvaptan and urea appear to be safe and effective when fluid restriction is challenging in paediatric SIADH.Graphical abstractA higher resolution version of the Graphical abstract is available asSupplementary information (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 19, 2023 Category: Urology & Nephrology Source Type: research
Is posterior urethral valve a chronic disease, not merely a chronic kidney disease? Reflections on models of (multidisciplinary) care
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 19, 2023 Category: Urology & Nephrology Source Type: research
A child with semaphorin 3b-associated membranous nephropathy effectively treated with obinutuzumab after rituximab resistance
We report the case of a child presenting at 2 years of age with steroid-resistant nephrotic syndrome diagnosed upon kidney biopsy as semaphorin 3B (SEMA3B)-associated primary membranous nephropathy. The patient responded to treatment with cyclosporine, but invariably relapsed upon tapering of this agent. Therefore, at age 9, he was successful ly treated with rituximab to overcome cyclosporine dependence. However, after the second rituximab infusion, a rapid reconstitution of CD19 + B cells and a relapse of proteinuria occurred, requiring reintroduction of cyclosporine. Obinutuzumab, a type II anti-CD20 monoclonal ant...
Source: Pediatric Nephrology - July 19, 2023 Category: Urology & Nephrology Source Type: research
