Fibrose cística em adultos: aspectos clínicos e espirométricos

Lemos, Antônio Carlos Moreira; Matos, Eliana Dias; Franco, Rosana; Santana, Pablo López; Santana, Maria Angélica

doi:10.1590/s1806-37132004000100004

Cited by 12 publications

(13 citation statements)

References 7 publications

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“…(15)(16)(17) One group of authors described 28 cases of patients living in Salvador, Brazil and diagnosed with CF in adult life. (18) The mean age was 31.1 years, 53.7% were black or mulatto, and 43% presented P. aeruginosa in the sputum culture. The authors emphasized the importance of investigating CF in adult patients with recurrent respiratory infection, sinusitis, and bronchiectasis.…”

Section: Antibiotic Therapymentioning

confidence: 97%

Fibrose cística no adulto: aspectos diagnósticos e terapêuticos

Dalcin¹,

Silva²

2008

J. bras. pneumol.

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Once considered a childhood disease, cystic fibrosis is now also a disease of adults. Increased longevity has resulted in the aging of the cystic fibrosis population. The consequent age-related medical problems among adults with cystic fibrosis have increased medical care needs. These needs are being met by a growing number of nonpediatric pulmonologists and other nonpediatric specialists. The objective of this review was to summarize the current knowledge about diagnosis and treatment in adult cystic fibrosis. In most cases, the diagnosis is suggested by manifestations of chronic sinopulmonary disease and exocrine pancreatic insufficiency. The diagnosis is confirmed by a positive sweat test result. Adult patients may, however, present pancreatic sufficiency and atypical clinical features, sometimes in combination with normal or borderline sweat test results. In such cases, identifying cystic fibrosis mutations and measuring nasal potential difference can have diagnostic utility. The standard therapeutic approach to pulmonary disease includes the use of antibiotics, airway clearance, exercise, mucolytics, bronchodilators, oxygen therapy, anti-inflammatory agents and nutritional support. Appropriate application of these therapies results in most cystic fibrosis patients surviving into adulthood with an acceptable quality of life.

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Section: Antibiotic Therapymentioning

confidence: 97%

Fibrose cística no adulto: aspectos diagnósticos e terapêuticos

Dalcin¹,

Silva²

2008

J. bras. pneumol.

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“…No aparelho digestivo, ocorre insuficiência pancreática por obstrução dos ductos excretores da glândula, (5,8) .…”

Section: Acometimento Do Trato Gastrintestinalunclassified

“…O diagnóstico da FC é sugerido somando-se as manifestações clínicas com a história familiar, e confirmado pela demonstração de níveis elevados de cloreto no suor, pelo método de Gibson e Cooke (1)(2)(3)(4)(5)(6)(7)(8) . Este teste mensura a quantidade de cloreto e sódio no suor e tem sensibilidade são considerados normais (1) .…”

Section: Diagnósticounclassified

“…A doença é mais comum nas crianças da raça branca (1: 2.500 nascimentos de caucasianos) (4,7) , enquanto sua incidência é considerada rara na raça negra (um para 17 mil negros americanos) e nos Orientais (1: 25.000) (8,7,2) . No…”

Section: Introductionunclassified

“…O diagnóstico é baseado nos achados clínicos, e confirmado pela demonstração laboratorial de concentrações anormalmente elevadas de cloreto no suor (> 60 mlEq/ l) (7,8,12) e pela análise genética (7,13) . O aumento da suspeição clínica e o diagnóstico através de análise genética têm levado à identificação de pacientes adolescentes e adultos com esta patologia (13,14) .…”

Section: Introductionunclassified

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Canais Iônicos E Fibrose Cística

Coutinho¹,

Figueiredo²,

Tintino³

et al. 2014

Rev. Interf. FLS

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_________________________________________________________________________________ RESUMOIntrodução: A Fibrose Cística (FC) é uma doença autossômica recessiva letal, que acomete preferencialmente crianças brancas. Objetivo: Descrever e discutir os mecanismos moleculares envolvidos na FC. Materiais e métodos: Artigos obtidos nos bancos de dados internacionais SCIELO, PUBMED, BIOLINE, DOAJ e HIGHWIRE que tratam dos mecanismos moleculares envolvidos na FC. Resultados e discussões: Decorre de mutações no gene que codifica a proteína "regulador de condutância transmembrana da fibrose cística" (CFTR). Esta contribui para a regulação do fluxo iônico nas superfícies apicais das células epiteliais, e é um canal de cloreto. A fisiopatologia da FC envolve o bloqueio da secreção do cloreto através da membrana apical das células epiteliais glandulares, resultando em seu acúmulo intracelular, acompanhado do influxo de sódio e água para este compartimento. Como consequência, ocorre desidratação da superfície celular com formação de secreções espessas -características da doença. O diagnóstico é baseado na clínica, demonstração laboratorial de concentrações elevadas de cloreto no suor e análise genética. O tratamento inclui: fluidificação das vias aéreas, antibioticoterapia, broncodilatadores, suporte nutricional e reposição enzimas. Na tentativa de aumentar o efluxo epitelial de Cl -ou inibir o influxo do Na + , alvos moleculares podem ser atacados por diferentes drogas como reguladores de migração citoplasmática, ativadores da expressão gênica, entre outros, para o primeiro, e inativação dos ENaC (amilorida ou benzamil) ou inibição do influxo de sódio (ouabaína, digoxina ou loperamida). Considerações Finais: Novas modalidades terapêuticas e a detecção precoce da FC por genotipagem podem melhorar o prognóstico e a qualidade de vida dos afetados. Palavras -chave:Fibrose Cística, Canalopatias, CFTR, EnaC, F508. ABSTRACTIntroduction: Cystic Fibrosis (CF) is a lethal autossomic recessive disease occurring mainly in white kids. Objective: Describe and discuss the molecular mechanisms involved in the CF. Material and methods: article obtained from the international databanks SCIELO, PUBMED, BIOLINE, DOAJ and HIGHWIRE about the molecular mechanisms of CF. Results and discussions: Its due mutations on the gene of cystic fibrosis Transmembrane conductance regulator (CFTR). This protein contributes for the regulation of the ionic flow in the apical surfaces of the epithelial cells, and is a chloride canal. The physiopathology of the CF involves the blockade of the chloride secretion through the apical membrane of the epithelial cells, resulting in intracellular accumulation, followed by the sodium and water influx. As consequence will occur a dehidratation of the cellular surface with thick secretion. The diagnosis is based on the clinic, the laboratorial identification of high concentrations of chloride in the sweat and genetic analysis. The treatment includes: rehidratation of the airway, antibiothicotherapy, nutritional support and en...

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Evaluation of autonomic modulation of lung function and heart rate in children with cystic fibrosis

Carvalho

Aletti

Costa

et al. 2020

Pediatric Pulmonology

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The autonomic nervous system (ANS) plays an important role in modulating bronchial smooth muscle contractility, which is altered in cystic fibrosis (CF). A convenient approach to probe ANS regulation is the quantitative analysis of heart rate variability (HRV). The purpose of this study was to evaluate ANS regulation in children with CF and to investigate the influence of colonization by Pseudonomas aeruginosa via assessment of HRV in colonized CF (CCF) children and noncolonized CF (NCCF) children. Sixteen children with CF (7 CCF and 9 NCCF) and seven healthy age‐matched control children were enrolled in the study. Heart rate was recorded for 10 min at rest in the supine and standing positions and HRV analysis was carried out using autoregressive spectral analysis. The CCF group was characterized by lower forced expiratory volume than NCCF, indicating an impairment of respiratory function. The HRV parameters further confirmed the possible sympathetic overactivity in CCF. Children with CF exhibited hyperactivity of the sympathetic nervous system. In particular, the CCF group presented a greater impairment of ANS modulation. Both CCF and NCCF children showed lower supine vagal activation in the HRV indices related to sympathetic activation and reduction of indices indicating vagal activity with the postural change from supine to standing when compared to the NCCF group.

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Fibrose cística em adultos: aspectos clínicos e espirométricos

Cited by 12 publications

References 7 publications

Fibrose cística no adulto: aspectos diagnósticos e terapêuticos

Fibrose cística no adulto: aspectos diagnósticos e terapêuticos

Canais Iônicos E Fibrose Cística

Evaluation of autonomic modulation of lung function and heart rate in children with cystic fibrosis

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