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Article
Open AccessHealth-related quality of life of children with X-linked hypophosphatemia in Germany
X-linked hypophosphatemia (XLH) is a rare inherited phosphate-wasting disorder associated with bone and dental complications. Health-related quality of life (HRQoL) is reduced in XLH patients on conventional t...
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Article
Open AccessMorphological changes and their associations with clinical parameters in children with nephropathic cystinosis and chronic kidney disease prior to kidney replacement therapy over 25 years
Infantile nephropathic cystinosis (INC) is a rare lysosomal storage disorder, mostly and often firstly affecting the kidneys, together with impaired disharmonious growth and rickets, eventually resulting in pr...
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Article
Open AccessAssessment and management of vitamin status in children with CKD stages 2–5, on dialysis and post-transplantation: clinical practice points from the Pediatric Renal Nutrition Taskforce
Children with chronic kidney disease (CKD) are at risk for vitamin deficiency or excess. Vitamin status can be affected by diet, supplements, kidney function, medications, and dialysis. Little is known about v...
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Article
Open AccessAddressing the psychosocial aspects of transition to adult care in patients with cystinosis
Cystinosis is a rare autosomal-recessive lysosomal storage disease that progressively affects multiple organs beginning with the kidneys. Patients require lifelong multidisciplinary care for the management of ...
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Article
Open AccessLMS-based continuous pediatric reference values for soluble receptor activator of nuclear factor kappa B ligand (sRANKL) and osteoprotegerin (OPG) in the HARP cohort
Soluble RANKL (sRANKL) and osteoprotegerin (OPG) are regulators of osteoclast differentiation and activation, but adequate pediatric reference values are lacking. Here we provide LMS (Lambda-Mu-Sigma)-based co...
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Article
Open AccessChest configuration in children and adolescents with infantile nephropathic cystinosis compared with other chronic kidney disease entities and its clinical determinants
Infantile nephropathic cystinosis (INC) is a systemic lysosomal storage disease causing intracellular cystine accumulation, resulting in renal Fanconi syndrome, progressive kidney disease (CKD), rickets, malnu...
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Article
Open AccessNutritional management of children with acute kidney injury—clinical practice recommendations from the Pediatric Renal Nutrition Taskforce
The nutritional management of children with acute kidney injury (AKI) is complex. The dynamic nature of AKI necessitates frequent nutritional assessments and adjustments in management. Dietitians providing med...
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Article
IPNA–ESPN Junior Master Class—a decade of successful continuing education and training in pediatric nephrology
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Article
Open AccessAssociations of longitudinal height and weight with clinical outcomes in pediatric kidney replacement therapy: results from the ESPN/ERA Registry
Associations between anthropometric measures and patient outcomes in children are inconsistent and mainly based on data at kidney replacement therapy (KRT) initiation. We studied associations of height and bod...
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Article
Open AccessThe International X-Linked Hypophosphatemia (XLH) Registry: first interim analysis of baseline demographic, genetic and clinical data
X-linked hypophosphatemia (XLH) is a rare, hereditary, progressive, renal phosphate-wasting disorder characterized by a pathological increase in FGF23 concentration and activity. Due to its rarity, diagnosis m...
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Article
Open AccessDiagnosis and management of mineral and bone disorders in infants with CKD: clinical practice points from the ESPN CKD-MBD and Dialysis working groups and the Pediatric Renal Nutrition Taskforce
Infants with chronic kidney disease (CKD) form a vulnerable population who are highly prone to mineral and bone disorders (MBD) including biochemical abnormalities, growth retardation, bone deformities, and fr...
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Article
Open AccessImpact of the COVID-19 pandemic on body mass index in children and adolescents after kidney transplantation
The coronavirus SARS-CoV-2 disease (COVID-19) pandemic affected lifestyles and resulted in significant weight gain in the general population. Its impact on children after kidney transplantation (KTx) is unknown.
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Article
Open AccessEmulation of the control cohort of a randomized controlled trial in pediatric kidney transplantation with Real-World Data from the CERTAIN Registry
Randomized controlled trials in pediatric kidney transplantation are hampered by low incidence and prevalence of kidney failure in children. Real-World Data from patient registries could facilitate the conduct...
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Article
Open AccessIPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome
Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia,...
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Reference Work Entry In depth
Dialysis
Providing optimum care to the infant, child, and adolescent patient requiring acute or chronic dialysis therapy demands that attention be directed to a variety of clinical issues. Pediatric patients with acute...
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Chapter
Disorders of Phosphorus Metabolism
In the past decade, research in genetic disorders of hypophosphatemic disorders has significantly expanded our understanding of phosphate metabolism. X-linked hypophosphatemic rickets is the most common inheri...
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Chapter
Growth and Puberty in Chronic Kidney Disease
Growth failure is a hallmark in children suffering from chronic kidney disease (CKD). It is most pronounced in children with congenital CKD requiring dialysis treatment. Optimal conservative management includi...
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Article
Open AccessNutritional management of the infant with chronic kidney disease stages 2–5 and on dialysis
The nutritional management of children with chronic kidney disease (CKD) is of prime importance in meeting the challenge of maintaining normal growth and development in this population. The objective of this r...
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Article
Open AccessHeterozygous variants in the DVL2 interaction region of DACT1 cause CAKUT and features of Townes–Brocks syndrome 2
Most patients with congenital anomalies of the kidney and urinary tract (CAKUT) remain genetically unexplained. In search of novel genes associated with CAKUT in humans, we applied whole-exome sequencing in a ...
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Article
Open AccessRickets guidance: part II—management
Here, we discuss the management of different forms of rickets, including new therapeutic approaches based on recent guidelines. Management includes close monitoring of growth, the degree of leg bowing, bone pa...
