Multidisciplinary Approach to Treating Chronic Pain in Patients with Ehlers-Danlos Syndrome: Critically Appraised Topic.

The Ehlers-Danlos syndromes (EDS) are a group of 13 related connective tissue disorders with a combined prevalence of 1 in 5000 people, with the most common noted as hypermobile EDS (hEDS). The EDS genetic condition is thought to affect both males and females equally, although most symptomatic patients are female. EDS causes a myriad of symptoms, including skin hyperextensibility and fragility, easy bruising and bleeding, joint hypermobility, subluxation, dislocation, and chronic pain. Pain is one of the most common symptoms of EDS, leading to disability and decreased quality of life. Current guidelines for treating chronic pain in EDS are lacking. Clinicians focus on a conservative multidisciplinary approach in patients with EDS, which avoids surgical interventions and its accompanying risks of morbidity and mortality. The multidisciplinary approach includes physiotherapy, occupational therapy, cognitive behavioral therapy, and pharmacologic interventions to decrease pain. This review identifies literature examining the components of this conservative multidisciplinary approach and their effectiveness across the PubMed, EMBASE, CINAHL, Web of Science, and Trip databases, using the terms "Ehlers-Danlos Syndrome AND Pain Management" that was then subsequently evaluated. The evaluation of this current literature provides weak evidence to support the efficacy of the individual components of the conservative multidisciplinary approach. Lack of alternative approaches leaves medical providers with little choice but to suggest these pain control methods, despite low-grade evidence of weak evidence of their efficacy. More research into the pathophysiology of chronic pain in EDS could help identify additional modes and rationales for therapy.

Contemporary Practice as a Board-Certified Pediatric Clinical Specialist: A Practice Analysis.

PURPOSE: The purpose of the 2019 practice analysis was to identify the elements of contemporary practice as a board-certified pediatric clinical specialist. METHODS: Consistent with the processes of the American Board of Physical Therapy Specialties (ABPTS), a subject matter expert panel used consensus-based processes to develop a survey to gather information concerning the knowledge areas, professional roles and responsibilities, practice expectations, and practice demographics of board-certified pediatric clinical specialists. The web-based survey was divided into 3 parts and administered to 3 separate groups of board-certified pediatric clinical specialists. RESULTS: Survey responses from 323 clinical specialists provided data to support confirmation and revision of the Description of Specialty Practice (DSP) for pediatrics. CONCLUSIONS: The revised DSP will provide contemporary practice information to inform the ABPTS specialist examination blueprint and the curricula of credentialed residency programs in pediatric physical therapy.

Assessment of complete remission after 2-chlorodeoxyadenosine for hairy cell leukemia: utility of marrow immunostaining and measurement of splenic index.

2-Chlorodeoxyadenosine (2-CdA) yields high complete remission (CR) rates in patients with hairy cell leukemia (HCL) Two approaches were used to detect minimal residual disease. We studied two B-lineage antibodies, L26 and MB2, and a T-lineage antibody, UCHL-1, in fixed marrow core biopsies from 34 patients with HCL before and after 2-CdA to detect minimal residual in the marrow. In addition, the splenic index was calculated before and after treatment to detect residual splenomegaly. Prior to therapy, hairy cells exhibited intense cytoplasmic membrane reactivity with L26 and strong intracytoplasmic reactivity with MB2. UCHL-1 did not react with hairy cells. Thirty-one patients were assessable 3 months after therapy. Five of 24 (21%) patients in CR by routine evaluation had residual HCL detected by immunostaining. Four of these 5 patients have been reevaluated at 1 year. One patient relapsed by routine evaluation, 2 remained positive by immunostaining alone, and 1 patient became negative by immunostaining. A total of 19 patients have been evaluated at 1 year and 17 remain in CR. Three of these 17 were positive by immunostaining, 2 of whom had been positive at 3 months and 1 additional patient who became positive by immunostaining at 1 year. Of 9 patients evaluated at 2 years, an additional 2 of 3 patients with minimal residual disease detected previously by immunostaining at 3 months relapsed by routine morphology and 1 had persistent positive immunostaining. Only 1 patient in remission by morphology and immunostaining has relapsed.(ABSTRACT TRUNCATED AT 250 WORDS)

Detection of minimal residual disease by immunostaining of bone marrow biopsies after 2-chlorodeoxyadenosine for hairy cell leukemia.

2-Chlorodeoxyadenosine (2-CdA) yields high complete remission (CR) rates in patients with hairy cell leukemia (HCL). In an effort to detect minimal residual disease, we studied two B-lineage antibodies, L26 and MB2, and a T-lineage antibody, UCHL-1, in fixed marrow core biopsies from 34 patients with HCL before and after 2-CdA. Before therapy, hairy cells exhibited intense cytoplasmic membrane reactivity with L26 and strong intracytoplasmic reactivity with MB2. UCHL-1 did not react with hairy cells. Thirty-one patients were assessable 3 months after therapy. Five of 24 (21%) patients in CR by routine evaluation had residual HCL detected by immunostaining. Four of these 5 patients have been reevaluated at 1 year. One patient relapsed by routine evaluation, 2 remained positive by immunostaining alone, and 1 patient became negative by immunostaining. A total of 19 patients have been evaluated at 1 year. Only 1 additional patient has become positive by immunostaining alone. Immunostaining using the B-lineage antibodies highlighted the presence of hairy cells with preservation of morphology. This assisted in quantifying the extent of disease, particularly when hairy cells were interstitial and blended with surrounding hematopoietic tissue, when hairy cells were present in hypocellular marrows, when hairy cells were spindle-shaped, and when marrows were markedly fibrotic. Because immunostaining can be easily performed on routinely processed marrows, it is an attractive method to detect minimal residual disease. Our data suggest that some patients in apparent CR after 2-CdA may have minimal residual disease. Patients will need to be observed prospectively to determine if residual disease will be predictive of relapse.