2026-04-13T04:12:28Zhttps://recercat.cat/oai/request

oai:recercat.cat:11351/61442025-10-24T10:38:11Zcom_2072_378070com_2072_378040col_2072_378092

00925njm 22002777a 4500 dc Serramontmany Morante, Eugènia author Muñoz López, Marina author Fernandez Polo, Aurora author Morillo Soriano, Maria author Gomez Ganda, Laura author Cañete Ramirez, Carme author Ariceta Iraola, Gema author 2021-07-09T12:22:02Z 2021-07-09T12:22:02Z 2021-01-14 NPHS1; Albúmina; Síndrome nefròtica congènit NPHS1; Albúmina; Síndrome nefrótico congénito NPHS1; Albumin; Congenital nephrotic syndrome Background: Congenital nephrotic syndrome of the Finnish type (CNF) is a rare, severe glomerular disease caused by mutations in the NPHS1 gene, which codes for nephrin. It is characterised by massive proteinuria and severe edoema. Progression to end-stage kidney failure occurs during early childhood and the only curative treatment is kidney transplantation. Nowadays, patients need aggressive medical treatment, which includes daily albumin infusions (for months) until they get clinical stability to receive transplant. Objective: In our paediatric hospital, we implemented a multidisciplinary program for the home infusion of albumin with outpatient follow-up. The aim of the study was to assess the safety and efficacy of this program for the first four years of its implementation. Material and Methods: Retrospective observational study of CNF paediatric patients treated with home albumin infusion therapy from March 2014 to July 2018 at a tertiary care paediatric hospital. Information on albumin administration was obtained from the electronic prescription assistance program and details on clinical and care-related variables from the hospital's electronic information systems. Results: Four patients with CNF received albumin infusions for 18, 21, 22 months, and 3 years. The treatment was safe, and the complication rates were to be expected considering the severity of disease. Patients required a median of two hospital admissions a year (19 in total); 47% due to catheter-related complications, but there were just three catheter infections. Conclusions: In our experience, home albumin infusion therapy is safe and effective and helps to improve children health and quality of life. http://hdl.handle.net/11351/6144 Nefrologia pediàtrica Albúmina - Ús terapèutic - Eficàcia DISEASES::Male Urogenital Diseases::Urologic Diseases::Kidney Diseases::Nephrosis::Nephrotic Syndrome CHEMICALS AND DRUGS::Amino Acids, Peptides, and Proteins::Proteins::Albumins Other subheadings::Other subheadings::/therapeutic use NAMED GROUPS::Persons::Age Groups::Infant ENFERMEDADES::enfermedades urogenitales masculinas::enfermedades urológicas::enfermedades renales::nefrosis::síndrome nefrótico COMPUESTOS QUÍMICOS Y DROGAS::aminoácidos, péptidos y proteínas::proteínas::albúminas Otros calificadores::Otros calificadores::/uso terapéutico DENOMINACIONES DE GRUPOS::personas::Grupos de Edad::lactante Home Albumin Infusion Therapy, Another Alternative Treatment in Patients With Congenital Nephrotic Syndrome of the Finnish Type