A case-control study on pregnancy in Italian Cystic Fibrosis women. Data from the Italian Registry

Giordani, Barbara; Quattrucci, Serena; Amato, Annalisa; Salvatore, Marco; Padoan, Rita

doi:10.1016/j.rmed.2018.11.009

Cited by 13 publications

(19 citation statements)

References 23 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…As an extension of a study of pregnancies in Italian CF women [10], this paper describes for the first time the good perinatal outcomes documented by the Italian CF Registry (ICFR) data of 2010-2015. The previous study evaluated the effects of pregnancy on the women's clinical condition, and…”

Section: Discussionmentioning

confidence: 99%

“…However, the subsequent improvements in diagnostic and therapeutic methods leading to longer and healthier life have meant that women with CF could plan a pregnancy as a real possibility. A number of reports [5][6][7][8][9][10] have documented the effect of pregnancy on the clinical status of women with CF, but less is known about perinatal outcomes [11][12][13]. Other than the annual number of pregnancies, the American, British, Canadian and Australian CF registries provide no information in terms of gestational age or type of labour; only the French registry reports some data concerning perinatal outcomes [14,15].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Perinatal Outcomes in Cystic Fibrosis Women: Data From the Italian Cystic Fibrosis Registry.

Padoan¹,

Quattrucci²,

Amato³

et al. 2020

Preprint

View full text Add to dashboard Cite

Data from the Italian Cystic Fibrosis Registry (ICFR) concerning pregnancies in the period 2010-2015 were used to investigate the association between the pre-conception clinical status and perinatal outcomes of women with CF. The assessed clinical variables were genotype, pre-conception age, the body mass index (BMI), and the percentage of predicted forced expiratory volume in one second (ppFEV1); the analysed outcomes were gestational age, birth weight (BW), and the frequency of caesarean deliveries. Complete information concerning mother and child was available for 56 pregnanciesthat came to term. The median pre-conception age was 30.8 years (18.7-42.3); median BMI was 21.5 kg/m2(16.5-26.8); and median ppFEV1was 73.9% (30-128%).Twenty-four women (42.8%) carried class I-III mutations, and 25 (44.8%) at least one mutation conferring residual CFTR function or having variable clinical consequences. The median duration of pregnancy was 37 weeks (31-41), and the frequency of prematurity was 28.30%. Median BW was 2,910 g (1,300-3,650). There was a positive correlation between BMI and ppFEV1 in comparison with BW (r=0.29 andr=0.31 by p=0.048 and p=0.032, respectively), but no correlation between age and BW. The overall frequency of caesarean sections was 63.64%, but it was 77.27% among the women with severe genotypes. A lower ppFEV1 corresponded to a lower BW (p=0.047). Respiratory function had no effect on the percentage of caesarean deliveries. The nutritional status and respiratory function of CF mothers correlate with their newborn’s BW. Caesarean sections are very frequent, even in young women with normal lung function.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Perinatal Outcomes in Cystic Fibrosis Women: Data From the Italian Cystic Fibrosis Registry.

Padoan¹,

Quattrucci²,

Amato³

et al. 2020

Preprint

View full text Add to dashboard Cite

show abstract

“…of their genotype or lung function impairment, and that the only adverse effect was on their nutritional status. 10 This observation suggests that pregnancy is feasible in such women, and can be successfully managed by means of the integrated multidisciplinary care provided by specialized CF centers following specific guidelines. 18 This paper analyses the outcomes of two thirds of the previously studied pregnancies 10 for which full outcome data were available (the duration of pregnancy, BW, and the type of delivery).…”

Section: Re Sultsmentioning

confidence: 96%

“…However, subsequent improvements in diagnostic and therapeutic methods that led to longer and healthier lives have meant that women with CF can now plan a pregnancy as a real possibility. A number of reports [5][6][7][8][9][10] have documented the effect of pregnancy on the clinical status of women with CF but less is known about perinatal outcomes, [11][12][13] as the American, British, Canadian and Australian CF registries provide no information concerning gestational age or type of labor, and only the French registry reports some data concerning perinatal outcomes. 14,15 As clinicians are consequently very interested in learning more about the management and outcomes of pregnancy in fertile women with airway disease and possibly reduced respiratory function, 16 the aim of this study was to use data from the Italian Cystic Fibrosis Registry (ICFR) to investigate the perinatal outcomes of women with CF who gave birth between 2010 and 2015.…”

Section: Introductionmentioning

confidence: 99%

Perinatal outcomes in women with cystic fibrosis: Data from the Italian Cystic Fibrosis Registry

Padoan

Quattrucci

Amato

et al. 2021

Acta Obstet Gynecol Scand

View full text Add to dashboard Cite

show abstract

“…Sollte eine Schwangerschaft eintreten, ist eine multidisziplinäre Betreuung von Patientinnen mit PCD empfehlenswert, welche mindestens eine regelmäßige gynäkologische und internistisch-pneumologische Versorgung umfasst. Von der Betreuung von Patientinnen mit CF ist bekannt, dass eine Schwangerschaft Effekte auf die Lungenfunktion haben kann 104 105 . Das Hauptproblem während einer Schwangerschaft ist das erhöhte Risiko für Frühgeburtlichkeit und das (mit Voranschreiten der Schwangerschaft und sich verschlechternder Atemmechanik steigende) Risiko für bronchopulmonale Exazerbationen.…”

Section: Nicht-respiratorische Manifestationenunclassified

Management der Primären Ciliären Dyskinesie

Raidt

Brillault²,

Brinkmann

et al. 2020

Pneumologie

View full text Add to dashboard Cite

ZusammenfassungDie Primäre Ciliäre Dyskinesie (PCD, MIM 242650) ist eine seltene hereditäre Multisystemerkrankung mit klinisch heterogenem Phänotyp. Leitsymptom ist eine chronische Sekretretention der oberen und unteren Atemwege, welche durch die Dysfunktion motiler respiratorischer Zilien entsteht. In der Folge kommt es zur Ausbildung von Bronchiektasen, häufig zu einer Infektion durch Pseudomonas aeruginosa sowie einer abnehmenden Lungenfunktion bis hin zum Lungenversagen.Bislang gibt es kaum evidenzbasierte Therapieempfehlungen, da randomisierte Langzeitstudien zur Behandlung der PCD fehlten. In diesem Jahr wurden die Daten einer ersten placebokontrollierten Medikamentenstudie bei PCD veröffentlicht. Anlässlich dieses Meilensteins im Management der PCD wurde der vorliegende Übersichtsartikel als Konsens von Patientenvertretern sowie Klinikern, die langjährige Erfahrung in der Behandlung der PCD haben, verfasst. Diese Arbeit bietet eine Zusammenfassung aktuell eingesetzter Behandlungsverfahren, die überwiegend auf persönlichen Erfahrungen und Expertenmeinungen beruhen oder von anderen Atemwegserkrankungen wie der Cystischen Fibrose (CF), COPD oder Bronchiektasen-Erkrankung abgeleitet werden. Da es derzeit keine kurative Therapie für PCD gibt, stehen symptomatische Maßnahmen wie die regelmäßige Reinigung der Atemwege und die Behandlung von rezidivierenden Atemwegsinfektionen im Fokus. Nicht respiratorische Manifestationen werden organspezifisch behandelt. Um neben der ersten Medikamentenstudie mehr evidenzbasiertes Wissen zu generieren, werden weitere Projekte etabliert, u. a. ein internationales PCD-Register. Hierüber wird Patienten der Zugang zu klinischen und wissenschaftlichen Studien erleichtert und die Vernetzung behandelnder Zentren gefördert. Des Weiteren können Erkenntnisse über eine Genotyp-spezifische Erkrankungsschwere erlangt werden, um folglich die therapeutische Versorgung der Patienten zu verbessern und somit zu individualisieren.

show abstract

A case-control study on pregnancy in Italian Cystic Fibrosis women. Data from the Italian Registry

Cited by 13 publications

References 23 publications

Perinatal Outcomes in Cystic Fibrosis Women: Data From the Italian Cystic Fibrosis Registry.

Perinatal Outcomes in Cystic Fibrosis Women: Data From the Italian Cystic Fibrosis Registry.

Perinatal outcomes in women with cystic fibrosis: Data from the Italian Cystic Fibrosis Registry

Management der Primären Ciliären Dyskinesie

Contact Info

Product

Resources

About