[Adverse effects of the tezacaftor/ivacaftor/elexacaftor combination that may lead to discontinuation: About a series of 10 cases]. / Effets indésirables de l'association tezacaftor/ivacaftor/elexacaftor pouvant mener à un arrêt de traitement : à propos d'une série de 10 cas.

INTRODUCTION: Cystic fibrosis transmembrane regulator (CFTR) channel modulators (ivacaftor, lumacaftor, tezacaftor and elexacaftor) represent a major advance in the management of cystic fibrosis. However, few data are available on the real-life safety profile of these medications, in particular on adverse events that may lead to their discontinuation. The aim of this study is to describe the characteristics and evolution of adverse reactions to the tezacaftor/ivacaftor/elexacaftor combination that led to discontinuation and were reported to the Centre régional de pharmacovigilance (CRPV) in Rennes (France). MATERIALS AND METHODS: A retrospective study was conducted from December 2021 to May 2023, focusing on cases of discontinuation of the tezacaftor/ivacaftor/elexacaftor combination due to the occurrence of one or more adverse effects, and reported to the CRPV of Rennes, France. RESULTS: Ten cases of drug discontinuation were reported to the Rennes CRPV (6 women/4 men). Adverse effects mainly involved neuropsychiatric disorders (n=6), followed by liver disorders (n=2), ear, nose and throat disorders (n=1), and digestive disorders (n=1). The average duration of treatment at discontinuation was 339.8 [39-668] days. The drug was reintroduced in 7 patients on average 48.7 [7-123] days after discontinuation, with a dosage adjustments (n=4) consisting of changes in dosing times or a reduction in daily doses, with varying success in alleviating adverse symptoms depending on the case. CONCLUSION: This small case series suggests that neuropsychiatric adverse effects may occur more frequently than initially described after initiation of tezacaftor/ivacaftor/elexacaftor, and should be carefully screened and monitored. Dosage or administration schedule modifications may be considered for patients experiencing these adverse effects. Further pharmacovigilance studies are needed to better understand the adverse effect profiles of "caftors", their possible risk factors, and the impact of adjusting dosing modalities.

[Adherence to chest physiotherapy in adults with cystic fibrosis]. / Adhésion à la kinésithérapie respiratoire chez les adultes atteints de mucoviscidose.

INTRODUCTION: Chest-physiotherapy is a key element in treatment of cystic fibrosis and patient adherence is a major issue in global cystic fibrosis care. This study aims to assess adherence to chest physiotherapy in adults with cystic fibrosis who not treated with tritherapy and to analyze the impact of certain factors on adherence. METHODS: Thus is a cross-sectional study, conducted using a questionnaire and a physiotherapy evaluation. Adherence to this treatment was measured in terms of quantitative and qualitative aspects. The impact on adherence of 15 factors was then assessed. RESULTS: Only 47% of patients could be considered as adherent, with a significant disparity between a quantitative and qualitative assessment. Gender, working time, pathology severity, the fact of being regularly followed by a physiotherapist, the perceived benefit of the sessions and their replacement by physical activity, seem associated with adherence to this treatment. CONCLUSIONS: Taking into account the qualitative aspect of the sessions, our study reveals a low rate, in our cohort, of adherence to respiratory physiotherapy, and highlights six factors likely to predominate. Regular follow-up by a physiotherapist seems to be a determining factor in adherence to this treatment.

[The impact of hypoxia on the ion channels in cystic fibrosis bronchial epithelial cells]. / Impact de l'hypoxie sur les canaux ioniques dans des cellules épithéliales bronchiques mucoviscidosiques.

Cystic fibrosis is a disease caused by a mutation on the CFTR gene coding for a chloride channel. The dominant mutation F508del eliminates the CFTR protein at the surface of epithelial cells, causing an accumulation of viscous mucus in the airways. In advanced stages of the disease, respiratory failure is associated with cellular hypoxia. Our project aims not only to describe the impact of hypoxia on ion channels and to highlight the underlying signaling pathways involved, but also to test the effectiveness of current CF treatments under the above-mentioned conditions.

[Parenthood challenged by cystic fibrosis. Experience of affected parents]. / La parentalité à l'épreuve de la mucoviscidose. Expérience des parents atteints.

INTRODUCTION: Medical progress affords patients with cystic fibrosis (CF) the opportunity to become parents. OBJECTIVE: To assess the psychological issues of parenthood in persons with cystic fibrosis. MATERIAL AND METHODS: Semi-structured interviews on parenthood, conducted by a psychologist with CF parents treated at the Cystic Fibrosis Center and the Transplantation Center of Foch hospital (Suresnes, France). RESULTS: Twenty-one (21) fathers and 22 mothers participated ; 84 % had become parents through pregnancy, 14% via adoption and one woman by means of surrogacy; 73% of the women and 67% of the men had done so prior to lung transplantation, while 25% had achieved parenthood without or against medical advice. As parents, they suffered from a lack of familial and medical support for their parental projects and a lack of recognition of their reproductive difficulties. CONCLUSION: While the desire for a child may be initially repressed by potentially life-threatening risks, a loving couple with a satisfying sexual life may in many instances surmount the repression and express the desire, which is not only the desire for a potentially life-bearing body, but also the desire to start a family.

[How can the transition of adolescents from a children's to an adult CF center be improved? Analysis of adolescents' and parents' needs during the post-transfer period]. / Comment améliorer la transition au sein des CRCM : analyse des besoins des patients et de leurs parents lors de l'arrivée en service adulte.

INTRODUCTION: In 2018, 55.4% of the 7180 French cystic fibrosis (CF) patients were adults. Our study was aimed at identifying young adult patients' needs and those of their parents when the young adults arrived in an adult CF center. METHODOLOGY: Semi-structured interviews, conducted between July 2018 and December 2019and involving all the concerned teenagers and their parents, took place at least 6 months after their transfer. The interview guide dwelt on the aspects having had an impact on their experience of the transition. The interviews were recorded, transcribed and analyzed exhaustively. The results were classified by categorizing the contents according to respondent profile. RESULTS: Thirty-eight young adult patients and 16 parents were interviewed. As regards the young adults, analysis of their needs underlined the importance of their continuing to develop their skills in adaptation, communication and self-care. As regards their parents, they needed support in view of defining their role in their children's new care pathway. CONCLUSION: During and also following the transfer, therapeutic education for the parents as well as the young adults requires reinforcement.

Towards harmonization of solutions used for cystic fibrosis diagnosis by nasal potential difference measurements: A formulation approach with CHESS® software.

BACKGROUND: One diagnosis of cystic fibrosis involves measuring the nasal transepithelial potential difference (NPD) as a complementary technique in the forms of the disease, where the sweat test is non-discriminating. The NPD is measured using solutions with and without chlorides, containing a variety of substances whose activities on nasal mucus membranes are studied or assessed. Among the solutions described in the literature and used in specialized centers, none seems to be best adapted for industrial production for reasons of stability (formulas of the international consensus of Rowe et al. and formulas of Knowles et al.) and/or potential toxicity (formulas of Middleton et al.). OBJECTIVE(S): Defining new formulas, according to those of the international consensus, with greater physicochemical and microbiological stability. METHODS: The reformulation tests were conducted on the formulas of Rowe et al., using CHESS® (CHemical Equilibrium of Species and Surfaces) software for modeling aqueous systems that substantially reduced the number of experiments. CHESS® software was first validated using models of ideal and non-ideal solutions. Thereafter, experimentation was carried out for the sake of comparison with theoretical data. RESULTS: CHESS® software using models of ideal and non-ideal solutions were validated. The experimentation confirmed the theoretical data, and new formulas were assessed based on their physicochemical (pH, content, Osmolality) and microbiological stability. CONCLUSION: The new formulas defined here guarantee excellent physicochemical and microbiological stability of diagnostic solutions, indispensable criteria for harmonizing and comparing results from different specialized centers using NPD measurements. These new formulas apply to the harmonization approach of techniques for measuring the nasal transepithelial potential difference.

Comparação de custos da terapia de reposição enzimática empírica com terapia guiada por diagnóstico laboratorial da insuficiência pancreática / Costs comparison of empirical enzymatic replacement therapy with laboratory diagnosis guided therapy of pancreatic insufficiency

Introdução: a fibrose cística, também conhecida como mucoviscidose, é uma doença genética cujas manifestações resultam da disfunção do gene cystic fibrosis transmembrane conductorance regulator. Cerca de 85% dos indivíduos com essa doença desenvolvem insuficiência pancreática exógena. Objetivo: comparar os custos da terapia de reposição enzimática empírica com a terapia de reposição enzimática empírica guiada pelo teste da elastase fecal, em indivíduos com fibrose cística, acompanhados em um centro de referência para assistência à doença. Metodologia: realizou-se um estudo descritivo e comparativo, que incluiu indivíduos de 0 a 21 anos, com fibrose cística. Coletaram-se dados referentes ao período de janeiro de 2016 a fevereiro de 2020, com registros clínicos, demográficos e laboratoriais. Inicialmente, com base em critérios clínicos, os participantes foram classificados como suficientes pancreáticos ou insuficientes pancreáticos. Após o resultado da dosagem da elastase fecal, o diagnóstico do status pancreático foi reavaliado. Realizouse a estimativa dos custos do teste da elas tase fecal por participante e da terapia por reposição enzimática empírica da insuficiência pancreática em indivíduos que, posteriormente, foram diagnostica dos como suficientes pancreáticos. Resultados: incluíram-se 50 participantes, com média de idade de 9,4 anos, sendo 52% do sexo masculino. Após o resultado da dosagem da elastase fecal, 7 participantes considerados insuficientes pancreáticos e foram reclassificados como suficientes pancreáticos. No período estudado, a economia média estimada, por participante suficiente pancreático, com a suspensão das enzimas, após resultado da elastase fecal, foi de R$ 6.770,13. Conclusão: a terapia de reposição enzimática empírica no tratamento da insuficiência pancreática pode levar a custos desnecessários. A medida de dosagem da elastase fecal contribui para decisão mais objetiva da avaliação da função pancreática.

Introduction: Cystic fibrosis, also known as mucoviscidosis, is a genetic disorder whose manifestations result from dysfunction of the cystic fibrosis transmembrane conductance regulator gene. About 85% of individuals with this disease develop exogenous pancreatic insufficiency. Objetivo: to compare the costs of empirical enzyme replacement therapy with fecal elastase test-guided empirical enzyme replacement therapy in individuals with cystic fibrosis followed up at a referral center for disease care. Methodology: a descriptive and comparative study was carried out, which included individuals aged 0 to 21 years, with cystic fibrosis. Data for the period from January 2016 to February 2020 were collected, with clinical, demographic and laboratory records. Initially, based on clinical criteria, participants were classified as pancreatic sufficient or pancreatic insufficient. After the result of the fecal elastase measurement, the diagnosis of pancreatic status was reassessed. Estimates were made of the costs of the fecal elastase test per participant and of the empiric enzyme replacement therapy for pancreatic insufficiency in individuals who were later diagnosed as pancreatic sufficient. Results: fifty participants were included, with a mean age of 9.4 years, 52% male. After the result of the fecal elastase measurement, 7 participants considered as pancreatic insufficient were reclassified as pancreatic sufficient. In the period studied, the estimated mean savings, per sufficient pancreatic participant, with the suspension of enzymes, after the result of fecal elastase, was R$ 6,770.13. Conclusion: empirical enzyme replacement therapy in the treatment of pancreatic insufficiency can lead to unnecessary costs. The measurement of fecal elastase dosage contributes to a more objective decision on the assessment of pancreatic function.

[Allergic Broncho-Pulmonary Aspergillosis (ABPA) in cystic fibrosis: Mechanisms, diagnosis and therapeutic options]. / Aspergillose bronchopulmonaire allergique (ABPA) et mucoviscidose : mécanismes, diagnostic et alternatives thérapeutiques.

INTRODUCTION: Fungal aspergillosis colonization and allergic bronchopulmonary aspergillosis (ABPA) can have a strong impact on the prognosis in cystic fibrosis (CF). We conducted round table discussions involving French experts from pediatric and adult centers caring for patients with CF, microbiologists, radiologists and pharmacists. The aim was to explore the current state of knowledge on: the pathophysiological mechanisms of Aspergillus and other micromycetes infections in CF (such as Scedosporium sp.), and on the clinico-biological diagnosis of ABPA. In perspective, the experts explored the role of imaging in the diagnosis of APBA, specifically CT and MRI; as well as the role of bronchoscopy in the management. We also reviewed the therapeutic management, including different corticosteroid regimens, antifungals and anti-IgE antibodies. CONCLUSION: The diagnosis of ABPA in CF should be based on more standardized biological assays and imaging to optimize treatment and follow-up.

A influência da idade e do genótipo sobre os níveis de cloreto do suor em uma população pediátrica com fibrose cística no estado da Bahia / The influence of age and genotype on sweat chloride levels in a pediatric population with cystic fibrosis in the state of Bahia

Introdução: a fibrose cística é a doença autossômica recessiva mais comum em populações caucasianas e a sua etiologia está associada a variantes patogênicas no gene CFTR. O teste do suor é considerado o padrão ouro para o diagnóstico dessa enfermidade. Estudos apontam que o genótipo do CFTR e a idade dos indivíduos influenciam as concentrações de cloreto no suor. Objetivos: pesquisar a correlação entre os níveis de cloreto no teste do suor e a idade ao diagnóstico de indivíduos com fibrose cística e comparar as concentrações iônicas do cloreto entre os sexos, diferentes faixas etárias e três grupos diversos de genótipos do CFTR. Metodologia: realizou-se um estudo de corte transversal, incluindo sujeitos de 0 a 20 anos, com diagnóstico confirmado de fibrose cística. Os indivíduos selecionados foram agrupados de acordo com as variáveis analisadas. Calcularam-se os valores descritivos das concentrações de íons cloreto de cada grupo. Utilizou-se o teste de Spearman para a análise da correlação entre a idade ao diagnóstico e os níveis de cloreto no suor. Resultados: 64 indivíduos foram incluídos no estudo, sendo 51,56% do sexo masculino. A mediana (Min ­ Max) da idade ao diagnóstico foi de 7 meses (1-206). Não foi observa da correlação entre a idade dos indivíduos ao diagnóstico e os níveis de cloreto no suor. As concentrações medianas de cloreto foram maiores nos escolares (106 mEq/l), no sexo feminino (102 mEq/l) e nos heterozigotos F508del/Classe I a III (108 mEq/l); e menores nos adolescentes (100 mEq/l) e nos heterozigotos F508del/Classes IV a VI (77 mEq/l). Conclusão: os níveis de cloreto no suor não apresentaram correlação com a idade dos indivíduos ao diagnóstico. A variação considerável dos níveis iônicos entre os grupos de diferentes genótipos corrobora que o teste do suor é um bom preditor da avaliação funcional do canal CFTR.

Introduction: cystic fibrosis is the most common autosomal recessive disorder in Caucasian populations and its etiology is associated with pathogenic variants in the CFTR gene. The sweat test is considered the gold standard for the diagnosis of the disease. Some studies suggest that CFTR genotype and age affect sweat chloride concentrations. Objectives: to investigate the correlation between sweat chloride levels and age at diagnosis of individuals with cystic fibrosis and to compare ionic chloride concentrations among sexes, different age groups and three distinct groups of CFTR genotypes. Methodology: a cross-sectional study was conducted, which included CF subjects from 0 to 20 years of age. The selected individuals were clustered on the variables in analysis. The description values for chloride ion concentrations in each group were calculated. The Spearman's test was used to analyze the correlation between the age at diagnosis and sweat chloride levels. Results: 64 individuals were included, 51,56% male. The median (Min ­ Max) age at diagnosis was 7 months (1-206). There was no correlation between the age at diagnosis and sweat chloride levels. The median of the chloride concentrations were higher for schoolchildren (106 mEq/l), females (102 mEq/l) and heterozygous F508del/Classes I to III (108 mEq/l), and reached the lowest values for teenagers (100 mEq/l) and heterozygous F508del/Classes IV to VI (77 mEq/l). Conclusion: sweat chloride levels did not correlate with the age of individuals at diagnosis. The substantial variation of ionic levels among groups of distinct genotypes corroborates that the sweat test is a good predictor for functional assessment of the CFTR channel.

Avaliação antropométrica e sua associação com variáveis clínicas em pacientes pediátricos com fibrose cística de um centro no Nordeste brasileiro / Anthropometric assessment and its association with clinical variables in pediatric patients with cystic fibrosis from a center in the northeast of Brazil

Introdução: a avaliação e o acompanhamento nutricional fazem parte do cuidado integral dos pacientes com fibrose cística (FC), possibilitando intervenção precoce e tratamento mais efetivo da doença. Objetivo: avaliar o estado antropométrico de crianças e adolescentes com FC e pesquisar sua associação com variáveis clínicas e demográficas. Metodologia: estudo descritivo, incluindo-se indivíduos entre 1-19 anos de idade. Foram registradas medidas de peso, estatura, circunferência do braço (CB) e da prega cutânea tricipital (PCT) e calculados indicadores antropométricos (Peso/Idade- P/I, Altura idade -A/I e IMC/idade-IMC/I) e Circunferência Muscular do Braço (CMB). Comparou-se o indicador CMB com variáveis clínicas e demográficas através do cálculo de razões de prevalência (RP). Resultados: foram incluídos 41 pacientes, 53,6% do sexo masculino, mediana de idade de 78 meses. Quinze (36,6%) pacientes foram classificados como desnutridos pela avaliação da CMB (P15, TRE, mutação F508/del e A/I<-2, observando-se maior associação com as três últimas, RP de 6,25, 3,12; e 2,06; respectivamente. Conclusão: o IMC pode não ser suficiente para a avaliação do estado nutricional na FC, pois subestima a prevalência de desnutrição. Com exceção das infecções respiratórias recorrentes, as demais variáveis tiveram associação com o indicador CMB

Introduction: the assessment and nutritional follow-up are part of the comprehensive care of cystic fibrosis (CF) patients enabling an early intervention and a more effective disease treatment. Objective: to assess the anthropometric status of children and adolescents with cystic fibrosis (CF) and to investigate its association with clinical and demographic variables. Methodology: descriptive study, including individuals between 1-19 years of age. Weight, height, arm circumference (AC) and tricipital skinfold (TSF) measurements were recorded and anthropometric indicators were calculated (Weight/Age-W/A, Height age -H/A and BMI/age-BMI/A) and Muscle Circumference of the Arm (AMC). The AMC indicator was compared with clinical and demographic variables by calculating prevalence ratios (PR). Results: 41 patients were included, 53.6% male, median age 78 months. Fifteen (36.6%) patients were classified as malnourished by the AMC assessment ( P15, TRE, F508del variant and H/A <-2, with a greater association with the last three, PR of 6.25, 3.12; and 2.06; respectively. Conclusions: BMI may not be sufficient to assess nutritional status in CF since it underestimates the prevalence of malnutrition. Except for recurrent respiratory infections, the other variables were associated with the AMC indicator

[Emerging bacteria in cystic fibrosis and non-cystic fibrosis bronchiectasis from a microbiologist's perspective]. / Bactéries émergentes dans la mucoviscidose et les dilatations des bronches hors mucoviscidose. Le point de vue du microbiologiste.

INTRODUCTION: Common major pathogens like Pseudomonas aeruginosa are identified in the airways of patients with cystic fibrosis (CF) and non-CF bronchiectasis. However, other opportunistic bacterial pathogens like Achromobacter xylosoxidans complex, Stenotrophomonas maltophilia and non-tuberculous mycobacteria are currently emerging in CF and are also reported in non-CF bronchiectasis. BACKGROUND: The emergence of opportunistic bacterial pathogens has been recognized in CF through annual national reports of sputum microbiology data. Despite common factors driving the emergence of bacteria identified in CF and non-CF bronchiectasis patients, bronchiectasis registries have been created more recently and no longitudinal analysis of recorded microbiological data is currently available in the literature, thereby preventing the recognition of emerging bacteria in patients with non-CF bronchiectasis. OUTLOOK: A longitudinal follow-up of microbiological data is still needed in non-CF bronchiectasis to identify emerging opportunistic bacterial pathogens. Homogeneity in practice of sputum microbiological examination is also required to allow comparative analysis of data in CF and non-CF bronchiectasis. CONCLUSION: Bacterial pathogens recognized as emerging in CF have to be more carefully monitored in non-CF bronchiectasis in view of their association with deterioration of the lung disease.

[Computed tomography in children with cystic fibrosis: The role of an expiratory protocol]. / Scanner thoracique chez l'enfant atteint de mucoviscidose : intérêt d'un protocole en expiration séquentielle pour réduire la dose d'irradiation.

INTRODUCTION: Chest computed tomography (CT) is essential to monitor lung disease in children with cystic fibrosis, but it involves recurrent exposure to ionizing radiation. The aim of this study was to compare the current complete CT protocol (volumetric end-inspiratory plus sequential expiratory acquisition) to a sequential expiratory acquisition protocol alone in terms of image analysis and ionizing radiation dose. METHODS: Seventy-eight CT scans from 57 children aged 5 to 18 years old were scored on the complete protocol images and on the expiratory sequential images only. Each CT protocol was scored independently, using the Brody scoring system, by two paediatric radiologists. RESULTS: Correlations between the Brody global scores of the two different CT protocols were very good (r=0.90 for both observers), for the bronchiectasis score (r=0.72 and 0.86), mucus plugging score (r=0.87 and 0.83), and expiratory trapped air (r=0.96 and 0.92). Total ionizing radiation dose was reduced, with the measured dose length product (DLP) reduced from 103.31mGy.cm (complete protocol) to 3.06mGy.cm (expiratory protocol) (P<0.001). CONCLUSION: An expiratory chest CT protocol was accurate in diagnosing early signs of CF disease and permitted significant reduction of radiation dose. This protocol would allow spacing out of complete CT scanning with its higher radiation dose and should be considered for the monitoring of lung disease severity in children with CF.

[Focus on cystic fibrosis]. / La mucoviscidose, mise au point.

Cystic fibrosis is a genetic disease whose most common symptoms are of respiratory nature. Mortality has been drastically reduced through better treatment of the various symptoms. Life expectancy at birth is currently over 50 years.

[Family support upon immediate cystic fibrosis announcement]. / L'accompagnement des familles à l'annonce de la mucoviscidose.

Cystic fibrosis, a rare, hereditary and chronic disease, affects the psychic functioning of children and their families. Since it is incurable, constant adjustments are essential to make sense of the inevitable evolution of the disease. The psychologists support the families and the patients so that all live as well as possible with cystic fibrosis.

[An essential tool in the management of cystic fibrosis in childhood]. / La prise en charge de la mucoviscidose dès l'enfance.

The follow-up of a patient with cystic fibrosis requires specific skills. The implementation of a comprehensive management approach gives convincing results in terms of survivability and quality of life. This is why the Cystic Fibrosis Resource and Competence Centres (CRCMs) bring together multidisciplinary teams in which nurse coordinators play a key role with patients, relatives and other healthcare professionals.

[Cystic fibrosis, what follow-up action for adults in 2019?] / Mucoviscidose, quel suivi pour les adultes aujourd'hui ?

Long considered as a paediatric pathology, cystic fibrosis concerns in France more adults than children since 2015. Considering this demographic shift, the way healthcare is organized requires constant adaptation, particularly in the care of adults where new needs are rapidly emerging.

[Cystic fibrosis, support and end of life of patients]. / Mucoviscidose, accompagnement et fin de vie des patients.

Cystic fibrosis is a chronic disease detected at birth that requires multidisciplinary follow-up throughout life. Two singular stories guide us in the reflection on end-of-life care. Firstly, it is a painful stage when it comes to giving up lung transplantation. The care receiver also wants continuity of care in line with his or her philosophy of life. The partnership of the caregivers with the ethical space and/or palliative care of the hospital becomes a necessary third party for a more peaceful end of life.

[Cystic fibrosis, the transition to adult centres]. / Mucoviscidose, le passage vers les centres adultes.

The transition from childhood to adulthood means physical and psychological upheaval. The Safetim study, in a multi-professional team, is looking at the ideal transition for cystic fibrosis patients. Synthesis of the literature on the subject.

[Cystic fibrosis, growing and living better through sport]. / Mucoviscidose, grandir et mieux vivre grâce au sport.

For more than twenty years, the association "Étoiles des neiges" has placed sport at the heart of its actions and promotes its practice adapted for all: children, teenagers, adults and transplant patients. If in 1999, when it launched its projects, young people suffering from cystic fibrosis were rather exempt from sport, since then it has been recognized as one of the pillars of its care. The association deploys its Sport projects to all the CRCMs in France.

[Cystic fibrosis and pregnancy: Outcome, prognostic factors and obstetrical management]. / Mucoviscidose et grossesse : impact, facteurs pronostiques et prise en charge obstétricale.

As a result of improvements in life expectancy and therapies, increasing numbers of patients with cystic fibrosis become pregnant. The first studies were pessimistic and report adverse outcomes on the fetus and the mother. In the recent publications, long-term outcome for women with cystic fibrosis does not appear to be negatively impacted by pregnancy. Furthermore, the number of women successfully completing pregnancy continues to rise. The aim of this review is to assess the outcome of pregnancy in women with cystic fibrosis and the impact of pregnancy on the disease. It is hoped it will improve the counseling for pregnant women with cystic fibrosis and their obstetrical management.