Diagnosis of Cystic Fibrosis in Nonscreened Populations.

Objective: Although the majority of cases of cystic fibrosis (CF) are now diagnosed through newborn screening, there is still a need to standardize the diagnostic criteria for those diagnosed outside of the neonatal period. This is because newborn screening started relatively recently, it is not performed everywhere, and even for individuals who were screened, there is the possibility of a false negative. To limit irreversible organ pathology, a timely diagnosis of CF and institution of CF therapies can greatly benefit these patients.

Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation.

Objective: Cystic fibrosis (CF), caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, continues to present diagnostic challenges. Newborn screening and an evolving understanding of CF genetics have prompted a reconsideration of the diagnosis criteria.

Study Design: To improve diagnosis and achieve standardized definitions worldwide, the CF Foundation convened a committee of 32 experts in CF diagnosis from 9 countries to develop clear and actionable consensus guidelines on the diagnosis of CF and to clarify diagnostic criteria and terminology for other disorders associated with CFTR mutations.

Diagnosis of Cystic Fibrosis in Screened Populations.

Objective: Cystic fibrosis (CF) can be difficult to diagnose, even when newborn screening (NBS) tests yield positive results. This challenge is exacerbated by the multitude of NBS protocols, misunderstandings about screening vs diagnostic tests, and the lack of guidelines for presumptive diagnoses. There is also confusion regarding the designation of age at diagnosis.

Applying Cystic Fibrosis Transmembrane Conductance Regulator Genetics and CFTR2 Data to Facilitate Diagnoses.

Objective: As a Mendelian disease, genetics plays an integral role in the diagnosis of cystic fibrosis (CF). The identification of 2 disease-causing mutations in the CF transmembrane conductance regulator (CFTR) in an individual with a phenotype provides evidence that the disease is CF. However, not all variations in CFTR always result in CF.

Cystic Fibrosis Diagnostic Challenges over 4 Decades: Historical Perspectives and Lessons Learned.

Objective: Because cystic fibrosis (CF) can be difficult to diagnose, and because information about the genetic complexities and pathologic basis of the disease has grown so rapidly over the decades, several consensus conferences have been held by the US CF Foundation, and a variety of other efforts to improve diagnostic practices have been organized by the European CF Society. Despite these efforts, the application of diagnostic criteria has been variable and caused confusion.

Study Design: To improve diagnosis and achieve standardization in terms and definitions worldwide, the CF Foundation in 2015 convened a committee of 32 experts in the diagnosis of CF from 9 countries.

Adverse events associated with pelvic organ prolapse surgeries that use implants.

Objective: To estimate the rate of vaginal implant exposure associated with biologic grafts and permanent mesh used for pelvic organ prolapse (POP) surgery, to describe treatments used for these complications, and to estimate response rates to these treatments. The secondary aims were to describe the operative and perioperative complications.

Methods: This was a retrospective analysis of female members of Kaiser Permanente Southern and Northern California and Hawaii who underwent POP surgeries with biologic grafts and permanent mesh between September 2008 and May 2010.

Perioperative complications and reoperations after incontinence and prolapse surgeries using prosthetic implants.

Objective: To estimate the perioperative complication and reoperation rates associated with slings and prolapse repairs using mesh and biologic grafts.

Methods: Analysis of all female members of Kaiser Permanente Southern and Northern California and Hawaii who underwent sling procedures or pelvic organ prolapse surgeries using implanted grafts or mesh between September 1, 2008, and May 31, 2010. Physicians' Current Procedural Terminology Coding System, 4th edition, International Classification of Diseases, 9th Revision, and surgical implant logs were used to identify the procedures performed, implants used, perioperative complications, and readmissions and reoperations within 12 months of the index surgery.

Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis.

Newborn screening for cystic fibrosis (CF) offers the opportunity for early medical and nutritional intervention that can lead to improved outcomes. Management of the asymptomatic infant diagnosed with CF through newborn screening, prenatal diagnosis, or sibling screening is different from treatment of the symptomatically diagnosed individual. The focus of management is on maintaining health by preventing nutritional and respiratory complications.

Correlation of maximum urethral closure pressure with Valsalva leak point pressure using air-charged urodynamic catheters.

Introduction And Hypothesis: The objective of this study was to measure the correlation of maximum urethral closure pressure (MUCP) with Valsalva leak point pressure (VLPP) in women with urodynamic stress incontinence using air-charged urodynamic catheters.

Methods: Records of all women who underwent urodynamic testing for urinary incontinence using air-charged catheters over a 3-year period were reviewed. Data included scores on the Urogenital Distress Inventory (UDI-6) and Incontinence Impact Questionnaire (IIQ-7).

Cystic fibrosis pulmonary guidelines: airway clearance therapies.

Cystic fibrosis (CF) is a genetic disease characterized by dehydration of airway surface liquid and impaired mucociliary clearance. As a result, there is difficulty clearing pathogens from the lung, and patients experience chronic pulmonary infections and inflammation. Clearance of airway secretions has been a primary therapy for those with CF, and a variety of airway clearance therapies (ACTs) have been developed.

Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report.

Newborn screening (NBS) for cystic fibrosis (CF) is increasingly being implemented and is soon likely to be in use throughout the United States, because early detection permits access to specialized medical care and improves outcomes. The diagnosis of CF is not always straightforward, however. The sweat chloride test remains the gold standard for CF diagnosis but does not always give a clear answer.

Pelvic actinomycosis with retained intrauterine fetal bone: a case report.

Background: Pelvic actinomycosis is a rare infection that can manifest as pelvic inflammatory disease and in severe cases can cause extensive fibrosis. Most cases are associated with long-standing use of an intrauterine device (IUD).

Case: A 30-year-old woman presented with abdominal pain, fever and a pelvic mass.

Guidelines for implementation of cystic fibrosis newborn screening programs: Cystic Fibrosis Foundation workshop report.

Newborn screening for cystic fibrosis offers the opportunity for early intervention and improved outcomes. This summary, resulting from a workshop sponsored by the Cystic Fibrosis Foundation to facilitate implementation of widespread high quality cystic fibrosis newborn screening, outlines the steps necessary for success based on the experience of existing programs. Planning should begin with a workgroup composed of those who will be responsible for the success of the local program, typically including the state newborn screening program director and cystic fibrosis care center directors.

Antioxidants in cystic fibrosis. Conclusions from the CF antioxidant workshop, Bethesda, Maryland, November 11-12, 2003.

Although great strides are being made in the care of individuals with cystic fibrosis (CF), this condition remains the most common fatal hereditary disease in North America. Numerous links exist between progression of CF lung disease and oxidative stress. The defect in CF is the loss of function of the transmembrane conductance regulator (CFTR) protein; recent evidence that CFTR expression and function are modulated by oxidative stress suggests that the loss may result in a poor adaptive response to oxidants.

Effects of transdermal and oral contraceptives on estrogen-sensitive hepatic proteins.

Objective: The purpose of this study was to evaluate the effects of the contraceptive patch and an oral contraceptive (OC) on serum concentrations of estrogen-sensitive hepatic proteins, ethinyl estradiol (EE) and levonorgestrel (LNG).

Methods: Twenty-four women were randomized to receive three cycles of a contraceptive patch that delivers EE 20 microg/day and norelgestromin 150 microg/day or an OC that contains EE 35 microg and norgestimate 250 microg. Blood samples were taken at baseline and at the end of Cycle 3.

The impact of pelvic organ prolapse on sexual function in women with urinary incontinence.

The aim of the study is to evaluate the impact of pelvic organ prolapse (POP) on sexual function in women with urinary incontinence (UI). In this retrospective, case-cohort study, we reviewed the medical records of all women evaluated for UI between March and November 2003. All patients completed the short forms of the Urogenital Distress Inventory, Incontinence Impact Questionnaire, and Pelvic Organ Prolapse/Urinary Incontinence Sexual Questionnaire.

Effect of oral versus transdermal steroidal contraceptives on androgenic markers.

Objective: The purpose of this study was to compare biochemical androgen profiles in women treated with the contraceptive patch versus an oral contraceptive (OC).

Study Design: Twenty-four healthy women were randomly assigned to receive 3 cycles of either the contraceptive patch (ethinyl estradiol [EE] 20 microg/d and norelgestromin 150 microg/d) or OC (EE 35 mug and norgestimate 250 microg). Blood samples were taken at baseline and end of treatment.

Guide to bone health and disease in cystic fibrosis.

Cystic fibrosis (CF) is the most common genetic disease within the Caucasian population and leads to premature respiratory failure. Approximately 60,000 individuals are currently living with CF in North America and Europe, 40% of whom are adults. The life span of these patients has increased from approximately 2 to 32 yr of age over the last three decades.

Public attitudes toward the use of psychiatric medications for children.

Psychiatric medication use for children has increased dramatically over the past three decades. Despite substantial media attention to the issue, little is known about how the lay public feels about the use of psychiatric medications for children. Drawing on theories of medicalization, we describe and analyze Americans' attitudes towards the use of psychiatric medications generally and Prozac specifically for children described as having three types of behavioral problems.