Thoracoabdominal compression and respiratory system compliance in HIV-infected infants.

The thoracoabdominal compression technique (TAC) is used to measure expiratory flow in infants. We investigated whether TAC caused a change in total thoracic compliance (Crs), resistance (Rrs), and respiratory system time constant (Trs). We studied 41 infants (mean age, 12.

Mycobacterium abscessus infection in cystic fibrosis. Colonization or infection?

We present a case of a patient with cystic fibrosis who was thought to be colonized with Mycobacterium abscessus for 13 yr prior to developing clinically apparent mycobacterial infection. However, histologic evidence indicated that invasive mycobacterial disease was present from the onset. While accepting that chronic endobronchial colonization with atypical mycobacteria may occur in patients with cystic fibrosis, the repeated isolation of mycobacteria from the sputum of these patients should alert the clinician to the possibility of indolent disease.

MRI-SPECT fusion for the synthesis of high resolution 3D functional brain images: a preliminary study.

Medical imaging being a fast-expanding field, multimodal data fusion appears more and more as a key element for the optimal use of images. By fusion, we mean the combination of several information sources (in particular images), with the aim of providing either more condensed or more pertinent information. The long term scope of this work would be to improve the interpretation of 3D brain images, providing extra elements for the diagnosis and patient follow up.

Epidemiologic study of cystic fibrosis: design and implementation of a prospective, multicenter, observational study of patients with cystic fibrosis in the U.S. and Canada.

Cystic fibrosis (CF) is a complex illness characterized by chronic lung infection leading to deterioration in function and respiratory failure in over 85% of patients. An understanding of the risk factors for that progression and the interaction of these factors with current therapeutic strategies should materially improve the prevention of this progressive lung disease. The Epidemiologic Study of Cystic Fibrosis (ESCF) was therefore designed as a multicenter, longitudinal, observational study to prospectively collect detailed clinical, therapeutic, microbiologic, and lung function data from a large number of CF treatment sites in the U.

Thoracoabdominal asynchrony and ratio of time to peak tidal expiratory flow over total expiratory time in adolescents with cystic fibrosis.

Thoracoabdominal asynchrony (TAA) and the ratio of time to peak tidal expiratory flow over total expiratory time (TME/TE) have been used to assess airway obstruction in infants and adults. We obtained these measurements using calibrated respiratory inductance plethysmography (RIP) on 15 adolescents and young adults with cystic fibrosis (CF) and varying disease severity. The measurements were then compared to 15 normal age-matched controls.

Allergic bronchopulmonary aspergillosis in cystic fibrosis: reported prevalence, regional distribution, and patient characteristics. Scientific Advisory Group, Investigators, and Coordinators of the Epidemiologic Study of Cystic Fibrosis.

Objectives: Using the large database from the Epidemiologic Study of Cystic Fibrosis (ESCF), the objectives of this study were to (1) estimate the reported prevalence of allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis (CF); (2) compare reported prevalence rates across geographic regions; (3) compare reported prevalence rates between patient subgroups based on demographic and disease characteristics; and (4) describe the ABPA group with regard to their sex, age, and disease severity.

Study Design: All patients > or = 5 years of age enrolled in ESCF between December 1993 and May 1996 were eligible. Criteria for the diagnosis of ABPA were defined by the ESCF guidelines.

Unexpected non-HIV causes of death in children born to HIV-infected mothers. Pediatric Pulmonary and Cardiac Complications of Vertically Transmitted HIV Infection Study Group.

Introduction: A high incidence of sudden, unexplained deaths in infants born to HIV-infected mothers has been noted in several epidemiologic studies. During the course of a prospective study of heart and lung disease in children born to HIV-infected mothers, we noted that of 5 unexpected non-HIV-related deaths, 4 were attributed to traumatic events.

Methods: The Pediatric Pulmonary and Cardiac Complications of Vertically Transmitted HIV Infection (P2C2) study is a multicenter, prospective investigation of the incidence of heart and lung disease in HIV-infected children.

Lung abscess versus necrotizing pneumonia: implications for interventional therapy.

Objective: To assess and contrast the role of interventional therapy for two types of cavitating pneumonias: lung abscess and necrotizing pneumonia.

Materials And Methods: We retrospectively reviewed the imaging, interventional therapy, and outcome of 14 children seen between February 1987 and January 1996 with lung abscess and 9 with necrotizing pneumonia. All children were treated with antibiotics prior to intervention.

Cystic fibrosis: predictors of accelerated decline and distribution of disease in 230 patients.

Objective: The purpose of this study was to determine predictors of accelerated deterioration in radiographic manifestations of cystic fibrosis. The incidence and distribution of focally accentuated disease were also studied.

Materials And Methods: From 230 patients, 3038 chest radiographs were scored using the Brasfield system.

Reproductive health in males with cystic fibrosis: knowledge, attitudes, and experiences of patients and parents.

Males with cystic fibrosis (CF) are generally infertile as a result of aberrant development of Wolffian duct derivitives. The personal significance of this and related reproductive and sexual health (RSH) issues is unknown. We set out to describe the knowledge, attitudes, and experiences regarding RSH in a group of adolescent and adult males with CF, as well as the knowledge and attitudes of parents.

Cystic fibrosis: a system for assessing and predicting progression.

Objective: This study presents a radiography-based database scoring changes over time in a large population of patients with cystic fibrosis. The purpose of this database is to provide comparison for groups of patients undergoing experimental treatment to assess effect of the treatment. The data may also be used to compare individuals with their age-matched cohorts with cystic fibrosis.

A novel tool for rapid prototyping and development of simple 3D medical image processing applications on PCs.

A 32-bit PC-based 3D medical image processing software package is presented. Its basic functions are the display and manipulation of medical images and the inclusion of user-written processing routines in C language. This software runs on inexpensive hardware and is easy to learn.

Use of a rapid HPLC assay for determination of pharmacokinetic parameters of ibuprofen in patients with cystic fibrosis.

High doses of ibuprofen have been shown to delay the progression of lung disease without serious adverse effects in patients with cystic fibrosis. To be effective, peak ibuprofen concentration of 50 to 100 mg/L has to be achieved. We developed an HPLC assay to rapidly determine plasma ibuprofen concentration.

Application of textural features to the detection of ocular melanomas in scintigraphy.

A method for automated detection of ocular melanoma in scintigraphic images is described. The algorithm first performs an automatic segmentation of the eyes on specific reference images. The images of the eyes are then analyzed using textural parameters computed in several directions and averaged to damp directional information.

Vitamin A status in acute exacerbations of cystic fibrosis.

Vitamin A is an essential nutrient for epithelial cell maintenance and repair, and it is known that infectious stresses may depress plasma vitamin A concentrations. Patients with cystic fibrosis are at risk for vitamin A deficiency because of fat malabsorption as well as for the inflammatory stresses of pulmonary exacerbations of their underlying disease. We therefore hypothesized that acute pulmonary exacerbations of CF would depress plasma retinol concentrations, and that these concentrations would return to baseline values when clinical symptoms improved.

Pulmonary function and clinical observations in men with congenital bilateral absence of the vas deferens.

Congenital bilateral absence of the vas deferens (CBAVD) was once thought to be a distinct clinical entity, but genetic similarities in men with cystic fibrosis (CF) and CBAVD are described increasingly. We evaluated the clinical status, growth and nutritional state, and respiratory function of 18 men with CBAVD to determine whether these men with different CF transmembrane regulator (CFTR) genotypes may have clinical evidence of mild CF. Following a thorough history and examination, pulmonary function tests, sweat test, and renal ultrasound were performed.

Aerosolized recombinant human DNase in hospitalized cystic fibrosis patients with acute pulmonary exacerbations.

The goal of this study was to evaluate the safety and efficacy of recombinant human DNase (rhDNase) in hospitalized patients with cystic fibrosis (CF) experiencing acute pulmonary exacerbations. Eighty patients with documented CF were enrolled at 11 CF centers when admitted for antibiotic therapy. Patients were at least 5 yr old with a forced vital capacity (FVC) > or = 35% of predicted and an oxygen saturation > or = 90% on a fraction of inspired oxygen (FIO2) < 0.

[Clinical heterogeneity of Townes-Brocks syndrome].

Background: Townes-Brocks syndrome (TBS) is a rare autosomal dominant entity mainly characterized by ano-rectal, ear and extremities abnormalities with variable clinical expression.

Case Reports: The first case had ear and extremities, but not anorectal, abnormalities; a Pierre-Robin sequence with esophageal atresia was also observed. The second case had the classical triad of abnormalities also associated with tetralogy of Fallot which has been only once reported in the literature.

Recombinant human DNase I in cystic fibrosis patients with severe pulmonary disease: a short-term, double-blind study followed by six months open-label treatment.

Chronic pulmonary infection is the major cause of morbidity and mortality in cystic fibrosis (CF). Recombinant human deoxyribonuclease (rhDNase) in vitro has been shown to dramatically reduce the viscoelasticity of the sputum from CF patients. Phase II and III clinical trials have shown the drug to be safe, and that patients with a forced vital capacity (FVC) of > 40% predicted show an improvement in pulmonary function when receiving rhDNase.

Effect of low-fat colostrum on fat accretion and lipogenic enzyme activities in adipose tissue in the 1-day-old pig.

Fat accretion, body fatty acid (FA) composition and adipose tissue lipogenic enzyme activities were determined in 1-day-old piglets fed during the first day of life sow colostrum that was either normal (control) or low in fat and high in lactose, galactose or glucose as the sole source of carbohydrate. Malic enzyme activity did not change during the first postnatal day while increases (P < 0.01) were found for glucose-6-phosphate dehydrogenase and acetyl-CoA-carboxylase.