Age of menarche in girls with cystic fibrosis.

Umławska, Wioleta; Sands, Dorota; Zielińska, Anna

doi:10.2478/v10042-010-0051-x

Cited by 25 publications

(18 citation statements)

References 31 publications

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“…Literature on pubertal development/progression and pubertal peak height velocity suggests a significant delay in children with CF. 39,40 Similar delays are reported in CF mice. 41 By using the upper limit of age 11, we believe we selected for prepubertal children with CF with comparable mean ages.…”

Section: Discussionsupporting

confidence: 76%

Growth in Prepubertal Children With Cystic Fibrosis Treated With Ivacaftor

et al. 2017

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BACKGROUND AND OBJECTIVES Cystic fibrosis (CF) is known for its impact on the lung and pancreas of affected individuals; however, impaired growth is also a common complication. We hypothesized that targeting the biological defect in the CF transmembrane conductance regulator (CFTR) protein may impact growth outcomes. METHODS In this post hoc analysis, we assessed linear growth and weight in 83 children (aged 6–11) enrolled in 2 clinical trials, the longitudinal-observation GOAL study and the placebo-controlled ENVISION study, to evaluate the effects of ivacaftor, a CFTR potentiator. We calculated height and weight z scores and height and weight growth velocities (GVs). RESULTS In ivacaftor-treated children in GOAL, height and weight z scores increased significantly from baseline to 6 months (increases of 0.1 [P < .05] and 0.26 [P < .0001], respectively); height GV increased significantly from 3 to 6 months (2.10 cm/y increase [P < .01]). In ivacaftor-treated children in ENVISION, height and weight z scores increased significantly from baseline to 48 weeks (increases of 0.17 [P < .001] and 0.35 [P < .001], respectively). Height and weight GVs from baseline to 48 weeks were also significantly higher with ivacaftor than with placebo (differences of 1.08 cm/y [P < .05] and 3.11 kg/y [P < .001], respectively). CONCLUSIONS Ivacaftor treatment in prepubescent children may help to address short stature and altered GV in children with CF; results from these analyses support the existence of an intrinsic defect in the growth of children with CF that may be ameliorated by CFTR modulation.

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Section: Discussionsupporting

confidence: 76%

Growth in Prepubertal Children With Cystic Fibrosis Treated With Ivacaftor

et al. 2017

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“…Regelmäßige Inhala tionen helfen dem Patienten bei der Mobilisation des zähen Schleims in den Atemwegen, ebenso regelmäßige und konsequente Physiotherapie und Sport. Je nach pulmonaler Situation kommen topische Kortikoide und Makrolide, antiinflammatorische Medikation und antibiotische Therapien zum Einsatz, die inhalativ, oral oder intravenös verabreicht werden [3], sowohl prophylaktisch-suppressiv als auch während Exazerbationen [4] Aspekte vor und während der Schwangerschaft ▼ Fertilität Der Pubertätsbeginn ist bei Mukoviszidose-Patienten im Durchschnitt um 2 Jahre verzögert [6][7][8], er liegt bei 14,9 ± 1,4 Jahren [7]. Auch der puberale Wachstumsschub ist um ungefähr ein Jahr verspätet (bei CF: 12,9 ± 0,8 Jahre, bei Gesunden: 11,9 ± 1,0 Jahre) [7].…”

Section: Abstract ▼unclassified

Schwangerschaft bei Mukoviszidose – ein Überblick

Michl

Mues

Mainz

et al. 2015

Z Geburtshilfe Neonatol

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Life expectancy and quality of life of cystic fibrosis (CF) patients have been steadily increasing for many decades, due to intensified therapy and research. Correspondingly, the number of pregnancies in women with CF rises. Often it is not possible for the patients to assess the consequences of pregnancy in terms of their disease and the impact of their disease on the growing child. A pre-existing poor lung function, low body mass index, CF-related diabetes, chronic microbial colonisation, and transplanted lungs are the main risk factors for complications during pregnancy in CF. Generally, the best outcome for mother and child can be reached under exact planning and meshed multidisciplinary care. The purpose of this summary is to give a practical review of the risks and options associated with pregnancy in CF patients.

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“…The mean age of menarche in girls with CF occurs at the age of 14.4 to 14.6 years, which is higher than 12.9 years as reported in healthy adolescents [91, 92]. Menstrual irregularities have been reported in 52% of adolescents with CF [91].…”

Section: Puberty and Fertilitymentioning

confidence: 99%

Diagnosis and treatment of endocrine comorbidities in patients with cystic fibrosis

Siwamogsatham

Alvarez

Tangpricha

2014

Current Opinion in Endocrinology, Diabetes &Amp; Obesity

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Purpose of review The aim of this review is to provide an update on various relevant endocrine aspects of care in adolescents and adults with cystic fibrosis (CF). Recent findings As life expectancy in CF has continuously improved, endocrine complications have become more apparent. The common endocrine complications include cystic fibrosis related diabetes (CFRD), cystic fibrosis related bone disease, vitamin D deficiency and poor growth and pubertal development. Thyroid and adrenal disorders have also been reported, although the prevalence appears to be less common. Summary Endocrine diseases are an increasingly recognized complication that has a significant impact on the overall health of individuals with CF. This review summarizes the updated screening and management of endocrine diseases in the CF population.

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Age of menarche in girls with cystic fibrosis.

Cited by 25 publications

References 31 publications

Growth in Prepubertal Children With Cystic Fibrosis Treated With Ivacaftor

Growth in Prepubertal Children With Cystic Fibrosis Treated With Ivacaftor

Schwangerschaft bei Mukoviszidose – ein Überblick

Diagnosis and treatment of endocrine comorbidities in patients with cystic fibrosis

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