The Association of Neonatal Respiratory Distress With Ciliary Ultrastructure and Genotype in Primary Ciliary Dyskinesia.

Objective: To evaluate the relationship between ciliary ultrastructure/genotype and prevalence of neonatal respiratory distress (NRD) in primary ciliary dyskinesia (PCD).

Study Design: This was a retrospective analysis from a multicenter, prospective study of children and adults with PCD. Participants were classified by ultrastructural defect associated with their diagnostic genetic variants: 1) outer dynein arm defect alone (ODA), 2) outer plus inner dynein arm defect (ODA/IDA), 3) inner dynein arm defect with microtubular disorganization (IDA/MTD), 4) DNAH11 (encodes ODA protein but has normal ultrastructure), and 5) normal/near-normal/other.

Extracorporeal photopheresis to attenuate decline in lung function due to refractory obstructive allograft dysfunction.

Background: This study was designed to prospectively evaluate the efficacy of extracorporeal photopheresis (ECP) to attenuate the rate of decline of FEV in lung transplant recipients with refractory bronchiolitis obliterans. Due to an observed higher than expected early mortality, a preliminary analysis was performed.

Study Design And Methods: Subjects from 10 lung transplant centres were assigned to ECP treatment or to observation based on spirometric criteria, with potential crossover for those under observation.

The efficacy of extracorporeal photopheresis to arrest bronchiolitis obliterans in lung allograft recipients was compared between two automated photopheresis instruments.

Background: The most common instruments used for extracorporeal photopheresis (ECP) treatment in the United States are the UVAR XTS and the CELLEX devices (Therakos, West Chester, PA). When compared to the UVAR XTS instrument, the efficacy of the CELLEX instrument to arrest the decline in lung function in patients with chronic lung allograft dysfunction (CLAD) related to bronchiolitis obliterans (BOS) has not been previously evaluated.

Methods: The relative efficacy of the CELLEX vs UVAR XTS ECP instruments was assessed by comparing the difference in rates of FEV decline before and after ECP treatment and survival in two series of lung allograft recipients with BOS who had been treated with these instruments.

Factors Associated With Mortality and Response to Extracorporeal Photopheresis in Lung Allograft Recipients With Bronchiolitis Obliterans Syndrome.

Background: This study was designed to identify factors associated with clinical response to extracorporeal photopheresis (ECP) and mortality after ECP in lung allograft recipients with bronchiolitis obliterans.

Methods: Forced expiratory volume in 1 second (FEV1) values obtained 6 months before (baseline) and 6 months after initiation of ECP were used to plot the linear relationship between FEV1 versus time before and after ECP. Response to ECP was assigned when a positive integer was derived after subtracting the baseline rate of decline from the rate of decline 6 months after ECP.

Pediatric thalamic tumors in the MRI era: a Canadian perspective.

Background: Thalamic gliomas are rare. The natural history is unpredictable, and the optimal management of these tumors in children is poorly defined. The aim was to identify outcomes, prognostic factors, and response to various modalities of treatment in a relatively large population of pediatric thalamic tumors from many centers within a fairly homogeneous health care system.

Trauma association of Canada Pediatric Subcommittee National Pediatric Cervical Spine Evaluation Pathway: consensus guidelines.

Background: The Institute of Medicine calls for the use of clinical guidelines and practice parameters to promote "best practices" and improve patient outcomes. In 2006, The Trauma Association of Canada Pediatric Committee set out to create an evidence-based, national pediatric cervical spine (c-spine) clearance guideline based on the literature, existing algorithms from each pediatric trauma center and from expert opinion from across Canada.

Methods: A review of the literature took place in September 2006 using the PubMed database.

The law of relocation of children.

The trend in the law of relocation of children following divorce or determination of paternity is to decide cases based on their own facts rather by strong presumptions for or against relocation. Another common approach is to divide the burden between parents. Statutes of many states require the custodial parent to give notice, between 30 and 90 days, of the intent to relocate with the child.

Identifying the specific needs of adolescents after a mild traumatic brain injury: a service provider perspective.

Primary Objective: To identify the specific service needs of adolescents with mild traumatic brain injury (MTBI) and those of their parents through the perspective of expert service providers as well as to compare it to the perspective of adolescents and their parents obtained in a prior study.

Study Design: Qualitative design including a focus group held with experts in the field of adolescent TBI and a validation survey of other professionals.

Methods And Procedures: Eight experts from a variety of disciplines participated in a focus group where important needs of adolescents were discussed.