Disparities in treatment and outcome of kidney replacement therapy in children with comorbidities: an ESPN/ERA Registry study.

Background: Data on comorbidities in children on kidney replacement therapy (KRT) are scarce. Considering their high relevance for prognosis and treatment, this study aims to analyse the prevalence and implications of comorbidities in European children on KRT.

Methods: We included data from patients <20 years of age when commencing KRT from 2007 to 2017 from 22 European countries within the European Society of Paediatric Nephrology/European Renal Association Registry.

Vitamin D-Dependent Rickets Type 1A in Two Siblings with a Hypomorphic Variant Frequent in the African Population.

Vitamin D-dependent type 1A rickets (VDDR-1A) is a rare autosomal recessive disease due to the inability to convert 25-hydroxyvitamin D [25(OH)D] to the active form 1.25-dihydroxyvitamin D [1.25(OH) D] by the enzyme 25(OH)D-1α-hydroxylase leading to low or low-normal serum levels of [1.

IgA vasculitis (Henoch-Schönlein purpura) nephritis and psoriasis in a child: is there a relationship?

Background: Psoriasis is a chronic immune-mediated disorder that primarily affects the skin in both adults and children but can also have systemic involvement, particularly with arthritis and kidney injury. IgA nephropathy is the most frequent kidney disorder associated with psoriasis. Approximately one third of all cases of psoriasis begin in childhood, but association between psoriasis and renal disorders has scarcely been reported in pediatric patients.

[Diagnostic and Therapeutic Challenges in Atypical Hemolytic Uremic Syndrome: A Case Report].

The atypical hemolytic uremic syndrome comprises a thrombotic microangiopathy resulting from the complement alternate pathway hyperactivation. Its severity requires early diagnosis and treatment. The differential diagnosis includes typical hemolytic uremic syndrome (associated with Shiga toxin) and thrombotic thrombocytopenic purpura (associated with deficient activity of ADAMTS13).

[Distal Renal Tubular Acidosis: Clinical Variability in the Same Family].

Primary distal renal tubular acidosis is a genetic disorder characterized by the inability in acidification of urine. Symptoms are usually non-specific and highly variable. We described six cases in a family with four generations affected.

Pediatric Kidney Transplantation: Experience of a Center Over 4 Decades.

Introduction: Chronic kidney disease in the pediatric population is associated with numerous comorbidities and an increased risk of mortality. Kidney transplantation (KT) is considered to be the option of choice in children with end-stage renal disease.

Aim: To characterize a single center experience in pediatric KT in the last 35 years.

Cerebral Venous Sinus Thrombosis in a Child with Idiopathic Nephrotic Syndrome: a case report.

Complications are rare in pediatric cases of idiopathic nephrotic syndrome (NS). Thromboembolism ranks among the most uncommon and difficult complications to diagnose, particularly in the first episode of NS, since clinical signs might be unspecific. This report describes the case of a 5-year-old girl with NS for the first time presenting with severe hypoalbuminemia (< 2g/dL).

Bilateral renal cell carcinoma in a paediatric patient with tuberous sclerosis complex.

Tuberous sclerosis complex (TSC) is a genetic neurocutaneous disorder characterised by seizures, mental retardation and hamartoma formation in multiple organs, mainly in the brain, skin, kidney, liver, lung and heart. Renal manifestations occur in about 60-80% of all patients with TSC and their rate increases with age. We report the case of a 17-year-old boy with tuberous sclerosis who presented with abdominal pain associated with kidney failure.

[Asymptomatic cardiac tumor in a child: an incidental diagnosis].

Cardiac tumors in the pediatric population are rare, their incidence range between 0.001% and 0.003%.

Severe axillary lymphadenitis after BCG vaccination: alert for primary immunodeficiencies.

The bacilli Calmette-Guérin (BCG) vaccine is administered to all newborns in countries where tuberculosis is endemic. Immunocompromised hosts, namely patients with human immunodeficiency virus infection or primary immunodeficiencies, are especially prone to serious complications from this vaccine. We report three cases of BCG disease in children with primary immunodeficiencies: one with a partial recessive interferon-γ receptor 1 deficiency, who developed BCG dissemination; and two relatives with ZAP70 deficiency, a severe combined immunodeficiency, both of whom presented with regional and distant BCG disease.