Prevalence and Risk Factors for Low Bone Mineral Density in Adults With Cystic Fibrosis.

Single-center studies have suggested that up to 70% of adults with cystic fibrosis (CF) have lower than expected bone mineral density (BMD), substantially higher than the 25% prevalence reported from national registries. We determined the prevalence of low BMD in CF adults at our center and assessed risk factors for low BMD. This retrospective cohort study was conducted in all CF patients ≥18 years of age who had a dual-energy X-ray absorptiometry (DXA) scan performed at the Johns Hopkins Adult Cystic Fibrosis center between 2010 and 2018.

Management of pregnancy in cystic fibrosis.

Unlabelled: With recent therapeutic advances in care, people with cystic fibrosis (CF) are living longer and healthier lives. Development of the cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies has led to improved function of the CFTR protein resulting in improved lung function, decreased rates of pulmonary exacerbations and improved nutritional status for the majority of people with CF. Given improved quality and quantity of life, more people with CF are considering becoming pregnant than ever before.

Cystic Fibrosis: Highly Effective Targeted Therapeutics and the Impact on Sex and Racial Disparities.

People with Cystic Fibrosis (CF) are living longer and healthier lives due in part to new therapies, called Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators. However, disparities in outcomes still exist, with females demonstrating a shorter life expectancy than males; this is opposed to the typical female versus male life expectancy in the general United States population. In addition, minority populations such as those of Hispanic ethnicity and African Americans are less frequently eligible for these new CFTR modulators.

Cavitary lung nodules as an extraintestinal manifestation of ulcerative colitis.

A man in his 30s with ulcerative colitis (UC) on immunosuppressive agents and extensive travel history presented with subacute dyspnoea and dry cough. CT of the chest demonstrated numerous cavitary pulmonary nodules. An extensive infectious, malignant and autoimmune evaluation was pursued, ultimately with histopathology most consistent with necrobiotic lung nodules as an extraintestinal manifestation of UC.

Clinician attitudes and practices on pregnancy planning and care in cystic fibrosis.

Background: People with cystic fibrosis (CF) are living longer and healthier lives as a result of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, and are pursuing pregnancy. As the number of pregnancies in CF continue to increase, clinician attitudes and practices regarding care of pregnant people with CF remain largely unknown.

Objective: To evaluate the current attitudes and practices of CF clinicians regarding pregnancy planning and care in CF.

CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor.

The chloride channel dysfunction caused by deleterious cystic fibrosis transmembrane conductance regulator (CFTR) variants generally correlates with severity of cystic fibrosis (CF). However, 3 adults bearing the common severe variant p.Phe508del (legacy: F508del) and a deletion variant in an ivacaftor binding region of CFTR (p.

Indoor air pollution exposure is associated with greater morbidity in cystic fibrosis.

Background: Exposure to higher levels of outdoor air pollution is associated with increased morbidity in individuals with cystic fibrosis. Limited information exist regarding the potential adverse effects of indoor air pollution on those with cystic fibrosis.

Methods: Individuals with cystic fibrosis who were enrolled in the Twin and Sibling Study from 2000-2013, self-reported exposure to four known sources of indoor air pollution (secondhand smoke, forced hot air, wood stove and fireplace).

Challenges Faced by Women with Cystic Fibrosis.

Women with cystic fibrosis (CF) face several unaddressed concerns related to their health. These areas of concern include explanations and guidance on a sex disparity in outcomes, timing of puberty, effects of contraception, prevalence of infertility and impact of pregnancy, and prevention of urinary incontinence and osteoporosis. These understudied topics leave women with numerous unanswered questions about how to manage sexual and reproductive health in the setting of CF.

Sex differences in treatment patterns in cystic fibrosis pulmonary exacerbations.

Background: Females with cystic fibrosis (CF) have been shown to have worse pulmonary exacerbation (PEx) related outcomes compared to males. However, it is unknown if sex differences in treatment patterns are contributing to these outcomes. Thus, we sought to explore sex differences in treatment patterns in the Standardized Treatment of Pulmonary Exacerbations (STOP) cohort.

Catamenial haemoptysis in females with cystic fibrosis: a case series with review of management strategies.

Catamenial haemoptysis, the expectoration of blood during menses, has not been extensively reported in the cystic fibrosis (CF) literature. We describe four cases (age range: 25-34 years) of catamenial haemoptysis across four CF centres in the United States. These cases may represent thoracic endometriosis versus hormonal fluctuations in airway inflammation or infection resulting in bronchial artery bleeding.

Rapidly progressive metastatic cholangiocarcinoma in a postpartum patient with cystic fibrosis: a case report.

Background: Cholangiocarcinoma is a rare gastrointestinal malignancy that arises within the intrahepatic, perihilar, and/or extrahepatic bile ducts. Individuals with cystic fibrosis are at increased risk for gastrointestinal malignancies. The most common gastrointestinal malignancy in cystic fibrosis is colon cancer, but other gastrointestinal malignancies also occur at greater rates than the general population.

Unmasking catamenial hemoptysis in the era of CFTR modulator therapy.

Background: Thoracic endometriosis syndrome (TES) is a rare condition that occurs in women when endometriosis implants into the thoracic cavity. Catamenial hemoptysis, the occurrence of hemoptysis with menstruation, is a recognized clinical manifestation of TES commonly treated with hormonal therapy.

Case Summary: We present the first documented case describing the recrudescence of catamenial hemoptysis in the setting of Lumacaftor/Ivacaftor administration in a 25-year-old woman with cystic fibrosis (CF).

Randomized Evaluation of Heart Failure With Preserved Ejection Fraction Patients With Acute Heart Failure and Dopamine: The ROPA-DOP Trial.

Objectives: This study sought to compare a continuous infusion diuretic strategy versus an intermittent bolus diuretic strategy, with the addition of low-dose dopamine (3 μg/kg/min) in the treatment of hospitalized patients with heart failure with preserved ejection fraction (HFpEF).

Background: HFpEF patients are susceptible to development of worsening renal function (WRF) when hospitalized with acute heart failure; however, inpatient treatment strategies to achieve safe and effective diuresis in HFpEF patients have not been studied to date.

Methods: In a prospective, randomized, clinical trial, 90 HFpEF patients hospitalized with acute heart failure were randomized within 24 h of admission to 1 of 4 treatments: 1) intravenous bolus furosemide administered every 12 h; 2) continuous infusion furosemide; 3) intermittent bolus furosemide with low-dose dopamine; and 4) continuous infusion furosemide with low-dose dopamine.