Pamela M Schuler

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Recent progress in scientific research has facilitated accurate genetic and neuropathological diagnosis of congenital myopathies. However, given their relatively low incidence, congenital myopathies remain unfamiliar to the majority of care providers, and the levels of patient care are extremely variable. This consensus statement aims to provide care(More)
Patients who have cystic fibrosis (CF) are frequently hospitalized for long-term intravenous (IV) treatment. We evaluated clinical effectiveness of the Drum-Cartridge Catheter (Abbott Laboratories) for such patients. The catheter is placed peripherally under local anesthesia via an antecubital vein into the superior vena cava or right atrium. Patients who(More)
OBJECTIVE Lung retransplantation is a controversial practice due to increased morbidity and mortality and the scarcity of available donor organs. Living donor lobar lung transplantation increases the number of available donor organs and facilitates a more organized procedure than traditional cadaveric donation for this complex reoperation. The purpose of(More)
Mutations in the surfactant protein (SP)-C gene are responsible for familial and sporadic interstitial lung disease (ILD). The consequences of such mutations on pulmonary surfactant composition and function are poorly understood. To determine the effects of a mutation in the SP-C gene on surfactant, we obtained lung tissue at the time of transplantation(More)
BACKGROUND Evidence on the effectiveness of respiratory syncytial virus (RSV) immunoprophylaxis with palivizumab in children with cystic fibrosis (CF) is lacking. METHODS We utilized Medicaid Extract files from 27 states from 1999 to 2006 linked to the National Cystic Fibrosis Registry to establish a cohort of children 0-2 years with CF diagnosis.(More)
We present the case of a 32-year-old woman with high-grade right pulmonary artery stenosis secondary to fibrous mediastinitis. The patient was managed with balloon angioplasty and stent placement. Only 15 cases of this nature have been reported in the literature, and this is one of the first to be managed with endovascular stent placement.
Congenital muscular dystrophies are a group of rare neuromuscular disorders with a wide spectrum of clinical phenotypes. Recent advances in understanding the molecular pathogenesis of congenital muscular dystrophy have enabled better diagnosis. However, medical care for patients with congenital muscular dystrophy remains very diverse. Advances in many areas(More)
Omalizumab is a recombinant DNA-derived humanized immunoglobulin G (IgG) anti-IgE monoclonal antibody approved for use in patients with allergic asthma. However, it is not approved for allergic bronchopulmonary aspergillosis (ABPA). Conflicting reports exist about the effects of omalizumab on ABPA in patients with cystic fibrosis (CF). We report 2 patients(More)
Extralobar pulmonary sequestration (ELS) and congenital cystic adenomatoid malformation have been reported to coexist in several variations. This suggests a common embryologic origin. A 6-month-old boy presented with a history of recurrent pneumonias. The patient was diagnosed with a right lower lobe congenital cystic adenomatoid malformation (CCAM) and a(More)
A cross-sectional study was carried out in order to determine the influence of cardiovascular fitness on age-related declines in cognitive performance. Forty-eight volunteers were divided into Young (n = 13, 18-27 years), Middle-Aged (n = 22, 60-65 years) and Old (n = 13, 65-88 years) groups and tested on a battery of cardiovascular, pulmonary, hemodynamic,(More)