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Diretrizes, guidelines e consensos

The purpose of this document is to set out guidelines to ensure standardised care for children with cystic fibrosis looked after at the Royal Brompton & Harefield NHS Foundation Trust and District General Hospitals on a network care basis. They should be used as a guide only. The Royal Brompton Hospital is a Specialist CF Centre as defined by the Specialist Commissioners, NHS England.

With the development of the APP for our guidelines in 2017, we know that aside from the UK, the guidelines have been downloaded 53 countries - Afghanistan, Argentina, Australia, Austria, Bangladesh, Belgium, Brazil, Bulgaria, Ecuador, Egypt, France, Germany, Gibraltar, Greece, Hong Kong, Hungary, India, Iran, Ireland, Italy, Kazakhstan, Kenya, Latvia, Lebanon, Luxembourg, Macedonia, Malaysia, Malta, Mexico, Montenegro, Netherlands, New Zealand, Oman, Pakistan, Portugal, Philippines, Qatar, Romania, Russia, Saudi Arabia, Serbia, South Africa, Spain, Sweden, Switzerland, Thailand, Turkey, Seychelles, UAE, Ukraine, Uruguay, USA, and Yemen. This year we are going paperless, so the guideline will be available online and via APP only. We will only print out the ‘What’s new’ section, contacts and drug formulary for use in our clinics.

Our philosophy of care for patients with cystic fibrosis is based on current guidelines laid down by the Royal College of Physicians, Royal College of Paediatrics & Child Health (formerly British Paediatric Association), CF Trust, British Thoracic Society, and NHSE Service Specifications. These have identified significant advantages in terms of survival and morbidity for patients receiving care from specialist centres. Specialist centres offer access to comprehensive care from a multidisciplinary team consisting of consultants with a special interest in CF, trainee doctors, nurse specialists, dietitians, physiotherapists, clinical psychologists, pharmacists and social workers. The team is also responsible for producing and distributing educational material and carrying out research to improve knowledge about this disease. Special procedures and investigations are provided that may not be available at District General Hospital level (such as formal lung function and bronchoscopy). We are happy to continue with a shared care policy, as long as the NHSE National Service Specification and our signed Service Level Agreement are adhered to. We also run several out-reach clinics whereby our MDT see CF patients in their local hospitals.

Details of the Service Specification can be found –
https://www.england.nhs.uk/commissioning/spec-services/npc-crg/group-a/a01/.

 

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Athanazio RA, Vergara AA, Ribeiro AF, Riedi CA, Procianoy EFA, Adde FA, Reis FJC, Ribeiro JD, Torres LA, Fuccio MB, Epifanio M,Firmida MC, Damaceno N, Ludwig-Neto N, Maróstica PJC, Rached SZ, Melo SFO; Grupo de Trabalho das Diretrizes Brasileiras de Diagnóstico e Tratamento da Fibrose Cística.
J Bras Pneumol. 2017;43(3):219-45.

RESUMO
A fibrose cística (FC) é uma doença genética autossômica recessiva caracterizada pela disfunção do gene CFTR. Trata-se de uma doença multissistêmica que ocorre mais frequentemente em populações descendentes de caucasianos. Nas últimas décadas, diversos avanços no diagnóstico e tratamento da FC mudaram drasticamente o cenário dessa doença, com aumento expressivo da sobrevida e qualidade de vida. Atualmente, o Brasil dispõe de um programa de ampla cobertura para a triagem neonatal de FC e centros de referência distribuídos na maior parte desses estados para seguimento dos indivíduos. Antigamente confinada à faixa etária pediátrica, tem-se observado um aumento de pacientes adultos com FC tanto pelo maior número de diagnósticos de formas atípicas, de expressão fenotípica mais leve, assim como pelo aumento da expectativa de vida com os novos tratamentos. Entretanto, ainda se observa uma grande heterogeneidade no acesso aos métodos diagnósticos e terapêuticos para FC entre as diferentes regiões brasileiras. O objetivo dessas diretrizes foi reunir as principais evidências científicas que norteiam o manejo desses pacientes. Um grupo de 18 especialistas em FC elaborou 82 perguntas clínicas relevantes que foram divididas em cinco categorias: características de um centro de referência; diagnóstico; tratamento da doença respiratória; tratamento gastrointestinal e nutricional; e outros aspectos. Diversos profissionais brasileiros
atuantes na área da FC foram convidados a responder as perguntas formuladas pelos coordenadores. A literatura disponível foi pesquisada na base de dados PubMed com palavras-chave, buscando-se as melhores respostas às perguntas dos autores.
Descritores: Fibrose cística/diagnóstico; Fibrose cística/terapia; Fibrose cística/complicações; Guia de prática clínica.

© 2017 Sociedade Brasileira de Pneumologia e Tisiologia.

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Farrell PM, White TB, Ren CL, Hempstead SE, Accurso F, Derichs N, et al.
J Pediatr. 2017 Feb;181S:S4-S15.e1. doi: 10.1016/j.jpeds.2016.09.064.


Abstract
OBJECTIVE

Cystic fibrosis (CF), caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, continues to present diagnostic challenges. Newborn screening and an evolving understanding of CF genetics have prompted a reconsideration of the diagnosis criteria.

STUDY DESIGN
To improve diagnosis and achieve standardized definitions worldwide, the CF Foundation convened a committee of 32 experts in CF diagnosis from 9 countries to develop clear and actionable consensus guidelines on the diagnosis of CF and to clarify diagnostic criteria and terminology for other disorders associated with CFTR mutations. An a priori threshold of ≥80% affirmative votes was required for acceptance of each recommendation statement.

RESULTS
After reviewing relevant literature, the committee convened to review evidence and cases. Following the conference, consensus statements were developed by an executive subcommittee. The entire consensus committee voted and approved 27 of 28 statements, 7 of which needed revisions and a second round of voting.

CONCLUSIONS
It is recommended that diagnoses associated with CFTR mutations in all individuals, from newborn to adult, be established by evaluation of CFTR function with a sweat chloride test. The latest mutation classifications annotated in the Clinical and Functional Translation of CFTR project (http://www.cftr2.org/index.php) should be used to aid in diagnosis. Newborns with a high immunoreactive trypsinogen level and inconclusive CFTR functional and genetic testing may be designated CFTR-related metabolic syndrome or CF screen positive, inconclusive diagnosis; these terms are now merged and equivalent, and CFTR-related metabolic syndrome/CF screen positive, inconclusive diagnosis may be used. International Statistical Classification of Diseases and Related Health Problems, 10th Revision codes for use in diagnoses associated with CFTR mutations are included.

KEYWORDS: CF-screen positive, inconclusive diagnosis; CFTR-related metabolic syndrome; immunoreactive trypsinogen; intestinal current measurement; nasal potential difference; newborn screening; pancreatitis associated protein; sweat test

PMID: 28129811 DOI: 10.1016/j.jpeds.2016.09.064
Copyright © 2016. Published by Elsevier Inc.

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Smyth AR, Bell SC, Bojcin S, Bryon M, Duff A, Flume P; European Cystic Fibrosis Society.

Abstract
Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above what was seen in the general population over the same period. With the implementation of newborn screening in many European countries, centres are increasingly caring for a cohort of patients who have minimal lung disease at diagnosis and therefore have the potential to enjoy an excellent quality of life and an even greater life expectancy than was seen previously. To allow high quality care to be delivered throughout Europe, a landmark document was published in 2005 that sets standards of care. Our current document builds on this work, setting standards for best practice in key aspects of CF care. The objective of our document is to give a broad overview of the standards expected for screening, diagnosis, pre-emptive treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support. For comprehensive details of clinical care of CF, references to the most up to date European Consensus Statements, Guidelines or Position Papers are provided in Table 1. We hope that this best practice document will be useful to clinical teams both in countries where CF care is developing and those with established CF centres.

Copyright © 2014 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

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Lahiri T, Hempstead SE, Brady C, Cannon CL, Clark K, et al.
Pediatrics March 2016, peds.2015-1784; DOI: https://doi.org/10.1542/peds.2015-1784


Abstract
Cystic fibrosis (CF) clinical care guidelines exist for the care of infants up to age 2 years and for individuals ≥6 years of age. An important gap exists for preschool children between the ages of 2 and 5 years. This period marks a time of growth and development that is critical to achieve optimal nutritional status and maintain lung health. Given that disease often progresses in a clinically silent manner, objective and sensitive tools that detect and track early disease are important in this age group. Several challenges exist that may impede the delivery of care for these children, including adherence to therapies. A multidisciplinary committee was convened by the CF Foundation to develop comprehensive evidence-based and consensus recommendations for the care of preschool children, ages 2 to 5 years, with CF. This document includes recommendations in the following areas: routine surveillance for pulmonary disease, therapeutics, and nutritional and gastrointestinal care.

Copyright © 2016 by the American Academy of Pediatrics

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LeGrys VA Yankaskas JR, Quittell LM, Marshall BC, Mogayzel Jr PJ.

Abstract
The Cystic Fibrosis Foundation (CFF) accredits cystic fibrosis (CF) centers, located in teaching and community hospitals nationwide, which provide comprehensive diagnosis and treatment for people with CF. The CF centers are evaluated by the CFF Center Committee according to specific criteria covering the areas of clinical care, teaching, and research. There are specific requirements for sweat testing, and adherence to them is required for accreditation. In 2006, the CFF Center Committee distributed a sweat testing guidelines memorandum to the CF center directors.1 Although the guidelines are based on the Clinical Laboratory Standards Institute (CLSI), formerly National Committee for Clinical Laboratory Standards, sweat testing document C34-A2 and the College of American Pathologists (CAP) Laboratory Accreditation Program Inspection Checklist items for sweat testing, they are more prescriptive for uniformity and are focused on diagnostic rather than screening sweat tests.2,3 The guidelines are applicable to patients of all ages undergoing sweat chloride testing.
Adherence to the guidelines is mandatory for CFF centers; however, the requirements are appropriate and adaptable for any facility performing diagnostic testing for CF. Although it may be ideal for sweat testing to be centralized at CF centers, in practice this does not occur. According to enrollment in a national proficiency testing program for sweat analysis, more than 600 laboratories performed sweat testing in 2006.4 With widespread implementation of newborn screening programs for CF, the reliance on a well-performed and well-interpreted sweat test is critical to the success of accurately diagnosing CF. Sweat chloride testing should be performed on all infants with a positive newborn screen even in cases in which two CF-causing mutations have been identified. The following represent the 2006 CFF sweat testing guidelines, along with commentary discussing the specific guidelines.

© 2007 Mosby, Inc. Published by Elsevier Inc. All rights reserved.

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