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Fibrosis quística / Cystic fibrosis
Escobar Castro, H; Suárez Cortina, L.
Affiliation
  • Escobar Castro, H; Hospital Ramón y Cajal. Alcalá de Henares (Madrid. España
  • Suárez Cortina, L; Hospital Ramón y Cajal. Alcalá de Henares (Madrid. España
Pediátrika (Madr.) ; 21(9): 317-331, oct. 2001. ilus
Article in Es | IBECS | ID: ibc-13193
Responsible library: ES1.1
Localization: ES1.1 - BNCS
RESUMEN
La fibrosis quística es una enfermedad autosómica causada por mutaciones el gen que modula la proteína CFTR responsable del canal de cloro. La enfermedad se caracteriza por infecciones crónicas del aparato respiratorio, insuficiencia pancreática y concentraciones elevadas de electrolitos en sudor. Aunque se trata de una enfermedad multisistémica, la alteración pulmonar es la causa mayor de morbilidad y mortalidad. La insuficiencia pancreática exocrina está presente en la mayoría de los pacientes. Hasta hace poco tiempo, el diagnóstico se basaba en criterio clínicos y esencialmente en la elevación del cloro en sudor. Ahora se conocen más de novecientas mutaciones responsables de la enfermedad y la demostración de alguna de estas o la demostración in vivo de la alteración del transporte iónico en el epitelio nasal, son también criterios diagnósticos. El tratamiento se basa en la prevención controlando cuidadosamente los aspectos nutritivos, gastrointestinales y pulmonares para permitir un crecimiento y desarrollo normal del niño que permita llegar a la edad adulta en buenas condiciones (AU)
Subject(s)
Adolescent; Adult; Female; Child, Preschool; Infant; Male; Child; Infant, Newborn; Humans; Cystic Fibrosis/complications; Cystic Fibrosis/diagnosis; Cystic Fibrosis/etiology; Cystic Fibrosis/therapy; Mutation/physiology; Mutation/genetics; Pancreatitis/complications; Pancreatitis/diagnosis; Pancreatitis/therapy; Diabetes Mellitus/complications; Diabetes Mellitus/diagnosis; Diabetes Mellitus/therapy; Respiratory Tract Infections/complications; Respiratory Tract Infections/diagnosis; Respiratory Tract Infections/microbiology; Enzymes/therapeutic use; Bronchodilator Agents/therapeutic use; Sputum/microbiology; Sputum/enzymology; Cystic Fibrosis Transmembrane Conductance Regulator/physiology; Cystic Fibrosis Transmembrane Conductance Regulator/genetics; Cystic Fibrosis Transmembrane Conductance Regulator/analysis; Cystic Fibrosis Transmembrane Conductance Regulator/therapeutic use; Anti-Inflammatory Agents/therapeutic use; Food and Nutritional Surveillance/methods; Food, Fortified; Child Nutritional Physiological Phenomena/education; Infant Nutritional Physiological Phenomena/education; Lung Diseases/complications; Lung Diseases/diagnosis; Lung Diseases/mortality; Lung Diseases/therapy; Exocrine Pancreatic Insufficiency/complications; Exocrine Pancreatic Insufficiency/diagnosis; Exocrine Pancreatic Insufficiency/mortality; Exocrine Pancreatic Insufficiency/therapy; Exocrine Pancreatic Insufficiency/enzymology; Anti-Bacterial Agents/therapeutic use; Pseudomonas aeruginosa/isolation & purification; Staphylococcus aureus/isolation & purification; Sweat/microbiology; Sweat; Sweating; Consensus Development Conferences as Topic
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Collection: National databases / Spain Health context: SDG3 - Health and Well-Being / SDG3 - Target 3.4 Reduce premature mortality due to noncommunicable diseases / SDG3 - Target 3.2 Reduce avoidable death in newborns and children under 5 Health problem: Target 3.2: Reduce avoidable death in newborns and children under 5 / Digestive System Diseases / Endocrine System Diseases / Other Respiratory Diseases / Infections / Neonatal Healthcare / Noncommunicable Diseases / Nutrition Database: IBECS Main subject: Exocrine Pancreatic Insufficiency / Pancreatitis / Respiratory Tract Infections / Sputum / Food and Nutritional Surveillance / Bronchodilator Agents / Food, Fortified / Child Nutritional Physiological Phenomena / Cystic Fibrosis Transmembrane Conductance Regulator / Cystic Fibrosis Type of study: Practice guideline Limits: Adolescent / Adult / Child / Child, preschool / Female / Humans / Infant / Male / Infant, Newborn Language: Spanish Journal: Pediátrika (Madr.) Year: 2001 Document type: Article Institution/Affiliation country: Hospital Ramón y Cajal/España
Search on Google
Add to My VHL
Print
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Collection: National databases / Spain Health context: SDG3 - Health and Well-Being / SDG3 - Target 3.4 Reduce premature mortality due to noncommunicable diseases / SDG3 - Target 3.2 Reduce avoidable death in newborns and children under 5 Health problem: Target 3.2: Reduce avoidable death in newborns and children under 5 / Digestive System Diseases / Endocrine System Diseases / Other Respiratory Diseases / Infections / Neonatal Healthcare / Noncommunicable Diseases / Nutrition Database: IBECS Main subject: Exocrine Pancreatic Insufficiency / Pancreatitis / Respiratory Tract Infections / Sputum / Food and Nutritional Surveillance / Bronchodilator Agents / Food, Fortified / Child Nutritional Physiological Phenomena / Cystic Fibrosis Transmembrane Conductance Regulator / Cystic Fibrosis Type of study: Practice guideline Limits: Adolescent / Adult / Child / Child, preschool / Female / Humans / Infant / Male / Infant, Newborn Language: Spanish Journal: Pediátrika (Madr.) Year: 2001 Document type: Article Institution/Affiliation country: Hospital Ramón y Cajal/España