The study highlights socioeconomic factors to be significant to the stigma and illness uncertainty experiences in SCD. Efforts by healthcare workers to reduce patient illness uncertainty may have additional impact, reducing their stigma.
Keloids result from abnormal proliferative scar formation with scar tissue expanding beyond the margin of the original wound and are mostly found in individuals of sub-Saharan African descent. The etiology of keloids has not been resolved but previous studies suggest that keloids are a genetically heterogeneous disorder. Although possible candidate genes have been suggested by genome-wide association studies using common variants, by upregulation in keloids or their involvement in syndromes that include keloid formation, rare coding variants that contribute to susceptibility in non-syndromic keloid formation have not been previously identified. Through analysis of whole-genome data we mapped a locus to chromosome 8p23.3-p21.3 with a statistically significant maximum multipoint LOD score of 4.48. This finding was followed up using exome sequencing and led to the identification of a c.1202T>C (p.(Leu401Pro)) variant in the N-acylsphingosine amidohydrolase (ASAH1) gene that co-segregates with the keloid phenotype in a large Yoruba family. ASAH1 is an acid ceramidase known to be involved in tumor formation by controlling the ratio of ceramide and sphingosine. ASAH1 is also involved in cell proliferation and inflammation, and may affect the development of keloids via multiple mechanisms. Functional studies need to clarify the role of the ASAH1 variant in wound healing.
Treatment of HIV infection with highly active antiretroviral therapy (HAART) requires high levels of adherence in order to obtain maximum benefit and minimize the development of antiviral resistance. Many patients in community clinical settings have imperfect adherence that may lead to poor clinical outcomes. The Connecticut HIV Medication Project (CHaMP) is a multidisciplinary program designed to evaluate patients receiving antiviral therapy. Based on results of a multifaceted assessment, a variety of targeted interventions and follow-up are offered. A retrospective analysis was performed on patients who were referred to the program over a 35-month period from March 2002 through January 2005. Two hundred forty-nine patients who were referred for adherence services had baseline and follow-up data available for analysis. Participants who maintained an unchanged antiretroviral regimen experienced a significant increase in self-reported adherence (89.1% to 96.9%, p < 0.001) and likelihood of reporting more than 95% adherence (36.6% to 73.1%, p < 0.001) by 7-day recall. Improvements in plasma HIV viremia (3.10 +/- 1.21 log copies to 2.78 +/- 0.98, p < 0.01) were also demonstrated. Limitations to this study included the unusually high level of baseline adherence, the large fraction of patients (28.6%) who were lost to follow-up, and follow-up that was limited to one time point at 12-16 weeks such that attrition of the intervention effect could not be assessed. The CHaMP experience demonstrates that the development of a multifaceted clinical program can have significant impact on medication adherence and viral burden in HIV infection.
This quality improvement project addressed the gaps in sickle cell pain management at a suburban teaching hospital emergency department. The aims were to (1) measure baseline pain management practices for patients with sickle cell disease in the ED and (2) implement an adapted emergency department sickle cell pain management clinical pathway. A retrospective chart review was conducted for data abstraction on pain management practices. Medical record review from 44 patient visits showed a high use of monotherapy, delay in time to medical evaluation, medication order, and time to first and subsequent analgesic administration and pain reassessments. Results were used by a multidisciplinary team to develop and implement an evidence-based clinical pathway to improve sickle cell disease pain management. The clinical pathway development was guided by both the advancing research through close collaboration model (ARCC) and Levine's principles of conservation to provide holistic care while preserving vital organs. arrives to your unit with a 3-day history of his usual sickle cell related backache and left leg pain. He took 15 mg morphine and Evidence-Based Sickle Cell Pain Management 103diphenhydramine 25 mg 2 hr ago. He reports that warm pad, gentle massage, dark room, and chicken noodle soup usually help but he has no appetite. He remains stoic and is asking for ketorolac (Toradol) and intravenous morphine 5 mg.
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