Airway Disease in Children with Primary Ciliary Dyskinesia: Impact of Ciliary Ultrastructure Defect and Genotype.

Primary ciliary dyskinesia (PCD) is characterized by impaired mucociliary clearance, recurrent respiratory infections, progressive airway damage, and obstructive lung disease. Although the association of ciliary ultrastructure defect/genotype with the severity of airflow obstruction has been well characterized, their association with airway abnormalities on chest computed tomography (CT) has been minimally evaluated. We sought to delineate the association of ciliary defect class/genotype with chest CT scores in children with PCD.

Laterality Defects in Primary Ciliary Dyskinesia: Relationship to Ultrastructural Defect or Genotype.

The association between organ laterality abnormalities and ciliary ultrastructural defect or genotype in primary ciliary dyskinesia is poorly understood. To determine if there is an association between presence and/or type of laterality abnormality and ciliary ultrastructural defect or genotype. Participants with primary ciliary dyskinesia in a multicenter, prospective study were grouped based on ciliary ultrastructural defect or genotype.

Airway Inflammation in Children with Primary Ciliary Dyskinesia.

The role of airway inflammation in disease pathogenesis in children with primary ciliary dyskinesia (PCD) is poorly understood. We investigated relationships between sputum inflammation measurements, age, lung function, bronchiectasis, airway infection, and ultrastructural defects in children with PCD. Spontaneously expectorated sputum was collected from clinically stable children and adolescents with PCD ages 6 years and older participating in a multicenter, observational study.

Association of Neonatal Hospital Length of Stay with Lung Function in Primary Ciliary Dyskinesia.

Primary ciliary dyskinesia (PCD), an inherited lung disease, is characterized by abnormal ciliary function leading to progressive bronchiectasis. There is wide variability in respiratory disease severity at birth and later in life. To evaluate the association between neonatal hospital length of stay (neonatal-LOS) and supplemental oxygen duration (SuppO) with lung function in pediatric PCD.

Expression of a Truncated Form of Associated with an Unusually Mild Primary Ciliary Dyskinesia Phenotype.

Primary ciliary dyskinesia (PCD) is a rare lung disease caused by mutations that impair the function of motile cilia, resulting in chronic upper and lower respiratory disease, reduced fertility, and a high prevalence of situs abnormalities. The disease is genetically and phenotypically heterogeneous, with causative mutations in > 50 genes identified, and clinical phenotypes ranging from mild to severe. Absence of (), a component of the outer dynein arm docking complex, results in a failure to assemble outer dynein arms (ODAs), mostly immotile cilia, and a typical PCD phenotype.

Sinus Development and Pneumatization in a Primary Ciliary Dyskinesia Cohort.

Background: Primary ciliary dyskinesia (PCD) is a genetically diverse disease which causes impaired mucociliary clearance, and results in pulmonary, otologic, and rhinologic disease in affected patients. Genetic mutations in multiple genes impair the ability of patients to clear mucous from the lungs, middle ear, and sinonasal cavity and lead to chronic pulmonary and sinonasal symptoms.

Methods: We identified 17 PCD patients who had available CT scans.

Cytoplasmic "ciliary inclusions" in isolation are not sufficient for the diagnosis of primary ciliary dyskinesia.

Background: The diagnosis of primary ciliary dyskinesia (PCD) is difficult and requires a combination of clinical features, nasal nitric oxide testing, cilia ultrastructural analysis by electron microscopy (EM), and genetics. A recently described cytoplasmic ultrastructural change termed "ciliary inclusions" was reported to be diagnostic of PCD; however, no supporting evidence of PCD was provided. In this study, we sought to confirm, or refute, the diagnosis of PCD in subjects with "ciliary inclusions" on EM.

Peripheral Cortisol and Inflammatory Response to a Psychosocial Stressor in People with Schizophrenia.

Objectives: There is growing evidence of both hypothalamic-pituitary-adrenal (HPA) axis and immune system dysfunction in schizophrenia. Additionally, accumulating evidence has linked dysfunction in the kynurenine pathway to schizophrenia as well as to stress and inflammation. The current pilot tested changes in immune, cortisol and kynurenine and kynurenic acid responses to a psychosocial stressor in people with schizophrenia and healthy controls.

Primary Ciliary Dyskinesia: Longitudinal Study of Lung Disease by Ultrastructure Defect and Genotype.

Rationale: In primary ciliary dyskinesia, factors leading to disease heterogeneity are poorly understood.

Objectives: To describe early lung disease progression in primary ciliary dyskinesia and identify associations between ultrastructural defects and genotypes with clinical phenotype.

Methods: This was a prospective, longitudinal (5 yr), multicenter, observational study.

Adjunct Aripiprazole Reduces Prolactin and Prolactin-Related Adverse Effects in Premenopausal Women With Psychosis: Results From the DAAMSEL Clinical Trial.

Purpose/background: Prolactin-related adverse effects contribute to nonadherence and adverse health consequences, particularly in women with severe mental illness. Treating these adverse effects may improve treatment acceptability, adherence, and long-term outcomes.

Methods/procedures: Premenopausal women with a Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision diagnosis of schizophrenia, schizoaffective disorder, or bipolar disorder were recruited for a randomized, double-blind, placebo-controlled 16-week trial of adjunct aripiprazole (5-15 mg/d).

Blood Pressure and Heart Rate Changes During Clozapine Treatment.

People with schizophrenia are 3-4 times more likely to die from cardiovascular disease than the general population. Clozapine (CLZ) is the gold standard of treatment for refractory schizophrenia. It has been associated with tachycardia and recent evidence shows individuals prescribed CLZ may develop blood pressure (BP) elevation and hypertension.

Clinical Features and Associated Likelihood of Primary Ciliary Dyskinesia in Children and Adolescents.

Rationale: Primary ciliary dyskinesia (PCD), a genetically heterogeneous, recessive disorder of motile cilia, is associated with distinct clinical features. Diagnostic tests, including ultrastructural analysis of cilia, nasal nitric oxide measurements, and molecular testing for mutations in PCD genes, have inherent limitations.

Objectives: To define a statistically valid combination of systematically defined clinical features that strongly associates with PCD in children and adolescents.

Blood Draw Barriers for Treatment with Clozapine and Development of a Point-of-Care Monitoring Device.

Background: While clozapine (CLZ) is the most effective antipsychotic drug for schizophrenia treatment, it remains underused. In order to understand the barriers of frequent blood draws for white blood cell counts (WBCs) and clozapine levels, we developed a psychiatrist survey and began an integrative approach of designing a point-of-care device that could eventually have real-time monitoring with immediate results.

Methods: We ascertained barriers related to CLZ management and the acceptance of possible solutions by sending an anonymous survey to physicians in psychiatric practice (n=860).

Adjunctive Minocycline in Clozapine-Treated Schizophrenia Patients With Persistent Symptoms.

Objective: Clozapine is the most effective antipsychotic for treatment refractory people with schizophrenia, yet many patients only partially respond. Accumulating preclinical and clinical data suggest benefits with minocycline. We tested adjunct minocycline to clozapine in a 10-week, double-blind, placebo-controlled trial.

Does mother's smoking influence girls' smoking more than boys' smoking? A 20-year review of the literature using a sex- and gender-based analysis.

A systematic literature review was conducted to examine whether mother's smoking influences girls' smoking more than boys' smoking. Fifty-seven studies, published between 1989 and 2009, were analyzed using a sex and gender lens. Results indicate that mother's prenatal and postnatal smoking influences girls' smoking more than boys' smoking.

"Just because you've got lung cancer doesn't mean I will": lung cancer, smoking, and family dynamics.

Although individuals who are diagnosed with smoking-related disorders are offered smoking cessation programs, little attention has been directed toward reducing tobacco use among healthy relatives who smoke. The purpose of this article is to report smoking relatives' responses to a family member's diagnosis of lung disease, their constructions of smoking in this context, and their interaction patterns with the patient. Interviews with 11 family members where there was a diagnosis of lung cancer and 3 family members where there was another serious smoking-related diagnosis were analyzed.

Do researcher-derived classifications of youths' smoking behavior correspond with youths' characterizations of their behavior?

Aims: To describe the categories employed by researchers to describe adolescents' smoking behavior and to determine how these various categorizations compare with youths' self-defined smoking status.

Study Selection: A search of the PubMed and Science Direct databases, limited to articles in the English language, published between January 2002 and November 2007.

Data Extraction: Employing a mixed methods approach, several categories of youths' smoking status were obtained from a literature review and subsequently reproduced by using responses to detailed questionnaire items.

Continued family smoking after lung cancer diagnosis: the patient's perspective.

Purpose/objectives: To explore the influence of lung cancer diagnosis on interpersonal dynamics in families in which one or more members continue to smoke following diagnosis.

Research Approach: Descriptive, qualitative.

Setting: Three cancer care sites in western Canada.