Endothelial dysfunction may play a key role in keloid and hypertrophic scar pathogenesis - Keloids and hypertrophic scars may be vascular disorders.

Treacher Collins syndrome is an autosomal dominant disorder of craniofacial development, the features of which include conductive hearing loss and cleft palate. In the absence of a candidate gene, a positional cloning approach has been used to isolate the mutated gene which maps to chromosome 5q31.3-32. Flanking markers were identified and a yeast artificial chromosome and cosmid contig of the region defined by these markers was created as a prelude to the creation of a transcript map of the region. Analysis of genes isolated using this approach resulted in the identification of the mutated gene. While the function of the gene remains unknown, the identification of 20 mutations spread throughout the gene, all of which would result in the insertion of a premature termination codon into the reading frame, suggests that the mechanism underlying the disease is haploinsufficiency.

Treacher Collins syndrome.

Pierre Robin sequence: review of diagnostic and treatment challenges.

Background Pierre Robin sequence is characterized by mandibular retrognathia and glossoptosis resulting in airway obstruction and feeding difficulties. When conservative management fails, mandibular distraction osteogenesis or tongue-lip adhesion may be required to avoid tracheostomy. The authors' goal was to prospectively evaluate the airway and feeding outcomes of their comprehensive approach to Pierre Robin sequence, which includes conservative management, mandibular distraction osteogenesis, and tongue-lip adhesion. Methods A longitudinal study of newborns with Pierre Robin sequence treated at a pediatric academic medical center between 2010 and 2015 was performed. Baseline feeding and respiratory data were collected. Patients underwent conservative management if they demonstrated sustainable weight gain without tube feeds, and if their airway was stable with positioning alone. Patients who required surgery underwent tongue-lip adhesion or mandibular distraction osteogenesis based on family and surgeon preference. Postoperative airway and feeding data were collected. Results Twenty-eight patients with Pierre Robin sequence were followed prospectively. Thirty-two percent had a syndrome. Ten underwent mandibular distraction osteogenesis, eight underwent tongue-lip adhesion, and 10 were treated conservatively. There were no differences in days to extubation or discharge, change in weight percentile, requirement for gastrostomy tube, or residual obstructive sleep apnea between the three groups. No patients required tracheostomy. The greatest reduction in apnea-hypopnea index occurred with mandibular distraction osteogenesis, followed by tongue-lip adhesion and conservative management. Conclusions Careful selection of which patients with Pierre Robin sequence need surgery, and of the most appropriate surgical procedure for each patient, can minimize the need for postprocedure tracheostomy. A comprehensive approach to Pierre Robin sequence that includes conservative management, mandibular distraction osteogenesis, and tongue-lip adhesion can result in excellent airway and feeding outcomes. Clinical question/level of evidence Therapeutic, II.

Airway and Feeding Outcomes of Mandibular Distraction, Tongue-Lip Adhesion, and Conservative Management in Pierre Robin Sequence: A Prospective Study.

Compression garments are elastic clothing with an engineered compression gradient that can be worn on limbs, upper, lower, or full body to use for therapy and sports. This article presents an overview and review on the compression garments and concentrates on the design of compression garments with an appropriate pressure for specific applications. It covers the types of compression garments, fibers and yarns, knitted fabric construction, garment design, an evaluation system, and pressure measurement and modeling. The material properties, fabric properties, pressure modeling, and the garment design system presents the prediction, design, and fabrication of the compression garments. Lastly, the research status and directions are discussed.

/pdf/compression-garments-for-medical-therapy-and-sports-2sx9zv48uq.pdf

Compression Garments for Medical Therapy and Sports.

ObjectiveTo evaluate costs associated with surgical treatment for neonates with Pierre Robin sequence (PRS).Study DesignRetrospective cohort study.SettingCincinnati Children’s Hospital Medical Cent...

Cost Analysis of Mandibular Distraction versus Tracheostomy in Neonates with Pierre Robin Sequence

Feeding Update for Neonates With Pierre Robin Sequence Treated With Mandibular Distraction

Pressure therapy has been considered standard, first-line intervention for the treatment of hypertrophic scars since its introduction in the 1960s. Although widely used, this scar management technique has historically been based on a wide array of anecdotal evidence as opposed to strong scientific support. Evidence has become more prevalent in recent years, necessitating a synthesis to develop an evidence-based clinical guideline. The clinical question was asked, "Among individuals with or at risk to develop active hypertrophic scars, does treatment with pressure therapy improve aesthetic and functional outcomes?" An evidence-based practice project was completed with aims to synthesize relevant literature to determine recommendations for the use of pressure therapy in individuals at risk for hypertrophic scars. A systematic search of the literature was conducted for the dates January 1950 to February 2014 of the following databases: MEDLINE, CINAHL, Cochrane Database for Systematic Reviews, Burntherapist.com, Cochrane Libraries, Ebsco, Google Scholar, OT Seeker, Ovid, MedLine, PEDro.org, Pubmed.gov, Pubmed Clinical Queries, and hand search of relevant articles through use of reference lists. Search terms included scar, hypertroph*, pressure therapy, compression therapy, pressure garment, burn, scald, trauma as well as MeSH terms cicatrix and hypertrophic. Articles were reviewed in terms of ability to answer the clinical question as well as strength of conclusions. A total of 45 articles were found and critiqued, 28 of which were relevant to the clinical question. Evidence strength ranged from level 1 to level 5. Results from the studies were synthesized to create clinical recommendations to guide treatment. Based on best available evidence, it is recommended that pressure therapy is utilized to decrease scar height and erythema that it is used for grafts and wounds requiring 14 to 21 days to heal, for 23 hours/day for 12 months, fit to achieve 20 to 30 mm Hg of pressure, fit by a skilled technician, and replaced every 2-3 months. In addition, it is not recommended that pressure therapy is used to treat abnormal pigmentation, nor used to hasten scar maturation. This literature search revealed insufficient evidence addressing the impact of pressure therapy on scar pliability. Among individuals with or at risk to develop active hypertrophic scars, treatment with pressure therapy does improve outcomes, particularly for aesthetic concerns including scar thickness and erythema. Applicability of research to practice: The practical treatment recommendations presented may improve consistency and efficacy of pressure therapy utilization at the point of care.

Development of a Best Evidence Statement for the Use of Pressure Therapy for Management of Hypertrophic Scarring

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Cost Analysis of Mandibular Distraction versus Tracheostomy in Neonates with Pierre Robin Sequence

Feeding Update for Neonates With Pierre Robin Sequence Treated With Mandibular Distraction

Development of a Best Evidence Statement for the Use of Pressure Therapy for Management of Hypertrophic Scarring