A coordinated transition model for patients with cystinosis: from pediatrics to adult care

Gema Ariceta1, Juan Antonio Camacho2, Matilde Fernández-Obispo3

  • 1Servicio de Nefrología Pediátrica, Hospital Universitari Vall d'Hebron, Grupo Renaltube, Redinren, Universidad Autónoma de Barcelona, Barcelona, España.

Abstract

Related Concept Videos

Chronic Kidney Disease III: Interprofessional Care01:28

Chronic Kidney Disease III: Interprofessional Care

Chronic kidney disease (CKD) requires collaborative and comprehensive management. CKD progresses through stages and can lead to end-stage kidney disease (ESKD) if untreated. Interprofessional collaboration and patient education are crucial, enabling patients to manage their health and improve their quality of life.Diagnostic approach for chronic kidney diseaseThe diagnosis of CKD primarily focuses on the glomerular filtration rate (GFR), which assesses kidney function by measuring how well...
580
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
603
Chronic Kidney Disease IV: Nursing Management01:18

Chronic Kidney Disease IV: Nursing Management

Nursing management is essential for preventing complications, maintaining stability, and improving patients' quality of life in chronic kidney disease (CKD). By using a structured approach, nurses help slow CKD progression and support effective patient care​.1. Comprehensive patient assessmentEffective management begins with nurses reviewing the patient’s medical history, and identifying key risk factors like diabetes, hypertension, and nephrotoxic drug use. Nurses assess signs of...
547
Kidney Transplant III: Nursing Management01:16

Kidney Transplant III: Nursing Management

Postoperative Nursing Management for Kidney Transplant PatientsPostoperative nursing management care includes monitoring the surgical site, encouraging early movement, and promoting lung health through breathing exercises. Nurses also administer prescribed medications like H2-blockers, such as famotidine, or proton pump inhibitors, like omeprazole, to help prevent gastrointestinal ulcers and bleeding. Fungal infections in the mouth and bladder can result from immunosuppressive and antibiotic...
502
Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses...
312
Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
1000