囊性纤维化基金会基于证据的婴幼儿囊性纤维化管理指南。
Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis.
机构信息
Women and Children's Hospital of Buffalo, State University of New York at Buffalo, NY, USA.
出版信息
J Pediatr. 2009 Dec;155(6 Suppl):S73-93. doi: 10.1016/j.jpeds.2009.09.001.
Abstract
Newborn screening for cystic fibrosis (CF) offers the opportunity for early medical and nutritional intervention that can lead to improved outcomes. Management of the asymptomatic infant diagnosed with CF through newborn screening, prenatal diagnosis, or sibling screening is different from treatment of the symptomatically diagnosed individual. The focus of management is on maintaining health by preventing nutritional and respiratory complications. The CF Foundation convened a committee to develop recommendations based on a systematic review of the evidence and expert opinion. These guidelines encompass monitoring and treatment recommendations for infants diagnosed with CF and are intended to help guide families, primary care providers, and specialty care centers in the care of infants with CF.
摘要
新生儿筛查囊性纤维化 (CF) 为早期医疗和营养干预提供了机会，这可能会改善预后。通过新生儿筛查、产前诊断或同胞筛查诊断为 CF 的无症状婴儿的管理与症状诊断个体的治疗不同。管理重点是通过预防营养和呼吸并发症来保持健康。CF 基金会召集了一个委员会，根据对证据和专家意见的系统审查，制定建议。这些指南涵盖了对诊断为 CF 的婴儿的监测和治疗建议，旨在帮助指导婴儿 CF 患者的家庭、初级保健提供者和专业护理中心进行护理。
相似文献
1
Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis.囊性纤维化基金会基于证据的婴幼儿囊性纤维化管理指南。
J Pediatr. 2009 Dec;155(6 Suppl):S73-93. doi: 10.1016/j.jpeds.2009.09.001.
2
Management of infants with cystic fibrosis: a summary of the evidence for the cystic fibrosis foundation working group on care of infants with cystic fibrosis.囊性纤维化婴儿的管理：囊性纤维化基金会工作组对囊性纤维化婴儿护理的证据总结。
J Pediatr. 2009 Dec;155(6 Suppl):S94-S105. doi: 10.1016/j.jpeds.2009.09.002.
3
Cystic fibrosis infant care challenges in diagnosis and management in the era of newborn screening.新生儿筛查时代囊性纤维化婴儿护理在诊断和管理方面面临的挑战。
Del Med J. 2012 May;84(5):149-55.
4
Guidelines on the early management of infants diagnosed with cystic fibrosis following newborn screening.新生儿筛查后诊断为囊性纤维化的婴儿早期管理指南。
J Cyst Fibros. 2010 Sep;9(5):323-9. doi: 10.1016/j.jcf.2010.04.008.
5
Outcomes of infants with indeterminate diagnosis detected by cystic fibrosis newborn screening.通过囊性纤维化新生儿筛查检测出诊断不明确的婴儿的结局。
Pediatrics. 2015 Jun;135(6):e1386-92. doi: 10.1542/peds.2014-3698. Epub 2015 May 11.
6
Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome and Cystic Fibrosis Screen Positive, Inconclusive Diagnosis.囊性纤维化跨膜传导调节因子相关代谢综合征及囊性纤维化筛查呈阳性、诊断不确定
J Pediatr. 2017 Feb;181S:S45-S51.e1. doi: 10.1016/j.jpeds.2016.09.066.
7
[National French guidelines for management of infants with cystic fibrosis].[法国囊性纤维化婴儿管理国家指南]
Arch Pediatr. 2014 Jun;21(6):654-62. doi: 10.1016/j.arcped.2014.03.014. Epub 2014 May 10.
8
Nutritional challenges of infants with cystic fibrosis.患有囊性纤维化的婴儿的营养挑战。
Early Hum Dev. 2001 Nov;65 Suppl:S53-61. doi: 10.1016/s0378-3782(01)00206-7.
9
Nutritional benefits of neonatal screening for cystic fibrosis. Wisconsin Cystic Fibrosis Neonatal Screening Study Group.囊性纤维化新生儿筛查的营养益处。威斯康星州囊性纤维化新生儿筛查研究组。
N Engl J Med. 1997 Oct 2;337(14):963-9. doi: 10.1056/NEJM199710023371403.
10
Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID).关于囊性纤维化跨膜传导调节因子相关代谢综合征/囊性纤维化筛查阳性、诊断不确定（CRMS/CFSPID）患儿管理的更新指南。
J Cyst Fibros. 2021 Sep;20(5):810-819. doi: 10.1016/j.jcf.2020.11.006. Epub 2020 Nov 27.
引用本文的文献
1
Organoid-on-a-chip (OrgOC): Advancing cystic fibrosis research.芯片上的类器官（OrgOC）：推动囊性纤维化研究。
Mater Today Bio. 2025 Jul 28;34:102148. doi: 10.1016/j.mtbio.2025.102148. eCollection 2025 Oct.
2
Impact of Elexacaftor/Tezacaftor/Ivacaftor on Fecal Elastase-1 in Children With Cystic Fibrosis.依列卡福妥/替扎卡福妥/依伐卡托对囊性纤维化儿童粪便弹性蛋白酶-1的影响。
Pediatr Pulmonol. 2025 Jun;60(6):e71156. doi: 10.1002/ppul.71156.
3
Innovations in Evaluating Ambulatory Costs of Cystic Fibrosis Care: A Comparative Study Across Multidisciplinary Care Centers in Ireland and the United States.评估囊性纤维化护理门诊费用的创新：爱尔兰和美国多学科护理中心的比较研究
NEJM Catal Innov Care Deliv. 2025 Feb;6(2). doi: 10.1056/CAT.24.0095. Epub 2025 Jan 15.
4
The Frequency and Potential Implications of HFE Genetic Variants in Children With Cystic Fibrosis.囊性纤维化患儿中HFE基因变异的频率及潜在影响
Pediatr Pulmonol. 2025 Mar;60(3):e71042. doi: 10.1002/ppul.71042.
5
Etiologies of exocrine pancreatic insufficiency.外分泌性胰腺功能不全的病因。
Gastroenterol Rep (Oxf). 2025 Mar 10;13:goaf019. doi: 10.1093/gastro/goaf019. eCollection 2025.
6
European guidelines for the diagnosis and treatment of pancreatic exocrine insufficiency: UEG, EPC, EDS, ESPEN, ESPGHAN, ESDO, and ESPCG evidence-based recommendations.欧洲胰腺外分泌功能不全诊断与治疗指南：UEG、EPC、EDS、ESPEN、ESPGHAN、ESDO和ESPCG基于证据的建议。
United European Gastroenterol J. 2025 Feb;13(1):125-172. doi: 10.1002/ueg2.12674. Epub 2024 Dec 5.
7
Impact of acid blocker therapy on growth, gut microbiome, and lung disease in young children with cystic fibrosis.酸阻滞剂疗法对患有囊性纤维化的幼儿生长、肠道微生物群和肺部疾病的影响。
J Pediatr Gastroenterol Nutr. 2024 Dec;79(6):1124-1133. doi: 10.1002/jpn3.12389. Epub 2024 Oct 28.
8
Practical Guidance for Clinical Microbiology Laboratories: Updated guidance for processing respiratory tract samples from people with cystic fibrosis.临床微生物学实验室实用指南：更新的囊性纤维化患者呼吸道样本处理指南。
Clin Microbiol Rev. 2024 Sep 12;37(3):e0021521. doi: 10.1128/cmr.00215-21. Epub 2024 Aug 19.
9
AGA-PancreasFest Joint Symposium on Exocrine Pancreatic Insufficiency.美国胃肠病学会-胰腺节外分泌性胰腺功能不全联合研讨会
Gastro Hep Adv. 2022 Nov 15;2(3):395-411. doi: 10.1016/j.gastha.2022.11.008. eCollection 2023.
10
Breastfeeding, growth, and lung disease in the first 3 years of life in children with cystic fibrosis.囊性纤维化患儿生命最初3年中的母乳喂养、生长发育及肺部疾病
J Cyst Fibros. 2024 Jul 16. doi: 10.1016/j.jcf.2024.07.006.
本文引用的文献
1
Prophylactic anti-staphylococcal antibiotics for cystic fibrosis.用于囊性纤维化的预防性抗葡萄球菌抗生素。
Cochrane Database Syst Rev. 2012 Dec 12;12:CD001912. doi: 10.1002/14651858.CD001912.pub2.
2
Management of infants with cystic fibrosis: a summary of the evidence for the cystic fibrosis foundation working group on care of infants with cystic fibrosis.囊性纤维化婴儿的管理：囊性纤维化基金会工作组对囊性纤维化婴儿护理的证据总结。
J Pediatr. 2009 Dec;155(6 Suppl):S94-S105. doi: 10.1016/j.jpeds.2009.09.002.
3
Cystic fibrosis pulmonary guidelines: airway clearance therapies.囊性纤维化肺部指南：气道清除疗法
Respir Care. 2009 Apr;54(4):522-37.
4
Recommended childhood and adolescent immunization schedules--United States, 2009.2009年美国推荐的儿童及青少年免疫接种计划
Pediatrics. 2009 Jan;123(1):189-90. doi: 10.1542/peds.2008-3306.
5
Safety and tolerability of inhaled hypertonic saline in young children with cystic fibrosis.吸入高渗盐水对患有囊性纤维化幼儿的安全性和耐受性
Pediatr Pulmonol. 2008 Nov;43(11):1100-1106. doi: 10.1002/ppul.20909.
6
Lung function in infants with cystic fibrosis diagnosed by newborn screening.通过新生儿筛查诊断的囊性纤维化婴儿的肺功能
Am J Respir Crit Care Med. 2008 Dec 15;178(12):1238-44. doi: 10.1164/rccm.200804-551OC. Epub 2008 Sep 11.
7
Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice.囊性纤维化突变分析在临床实践中的应用与解读共识。
J Cyst Fibros. 2008 May;7(3):179-96. doi: 10.1016/j.jcf.2008.03.009.
8
Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review.囊性纤维化和胰腺功能不全儿童及成人营养相关管理的循证实践建议：一项系统综述的结果
J Am Diet Assoc. 2008 May;108(5):832-9. doi: 10.1016/j.jada.2008.02.020.
9
Use of Palivizumab for prevention of hospitalization as a result of respiratory syncytial virus in infants with cystic fibrosis.使用帕利珠单抗预防囊性纤维化婴儿因呼吸道合胞病毒感染而住院。
Pediatr Infect Dis J. 2008 Jun;27(6):559-61. doi: 10.1097/INF.0b013e3181673c15.
10
Lung function from infancy to the preschool years after clinical diagnosis of cystic fibrosis.囊性纤维化临床诊断后从婴儿期到学龄前的肺功能
Am J Respir Crit Care Med. 2008 Jul 1;178(1):42-9. doi: 10.1164/rccm.200710-1599OC. Epub 2008 Apr 10.
Zotero 插件