Cardiomyopathy in limb girdle muscular dystrophy R9, FKRP related.

INTRODUCTION: Reported frequencies of cardiomyopathy in limb girdle muscular dystrophy R9 (LGMDR9) vary. We describe the frequency and age at onset of cardiomyopathy in an LDMDR9 cohort. METHODS: Echocardiograms from 56 subjects (157 echocardiograms) with LGMDR9 were retrospectively reviewed. The cumulative probability of having an abnormal echocardiogram as a function of age was assessed by survival analysis for interval-censored data by genotype. Correlations between cardiac and clinical function were evaluated. RESULTS: Twenty-five (45%) participants had cardiomyopathy. The median age at first abnormal echocardiogram for subjects homozygous for the c.826C>A variant was 54.2 y compared to 18.1 y for all other fukutin-related protein (FKRP) genotypes (P < .0001). There was a weak correlation between ejection fraction and 10-Meter Walk Test speed (r = 0.25), but no correlation with forced vital capacity (r = 0.08). DISCUSSION: Cardiomyopathy is prevalent among those with LGMDR9 and occurs later in subjects homozygous for the c.826C>A mutation. These data will help to guide surveillance and management.

The Fibromyalgia Transcutaneous Electrical Nerve Stimulation in Physical Therapy Study Protocol: A Multisite Embedded Pragmatic Trial.

OBJECTIVES: Transcutaneous electrical nerve stimulation (TENS) is a nonpharmacological intervention that provides an electrical current through the skin to produce analgesia. The primary purpose of this study is to examine if the addition of TENS to routine physical therapy improves movement-evoked pain in individuals with fibromyalgia in a physical therapy clinical setting. METHODS: Fibromyalgia TENS in Physical Therapy Study is a phase III embedded pragmatic clinical trial funded through the National Institutes of Health Helping to End Addiction Long-Term Initiative. This trial will utilize a randomized cluster design that includes more than 110 physical therapists in 24 to 30 physical therapy clinics within 6 health care systems and 7 states. Clinics will be randomized to TENS or No-TENS, stratified by health care system and clinic size. The plan is to enroll 600 participants, with all participants completing physical therapy as prescribed by their physical therapist. Participants at TENS clinics will utilize TENS for a minimum of 2-hour per day while at the physical therapy clinic and at home when active. The primary outcome is reduction in movement-evoked pain from baseline to day 60 on an 11-point numeric rating scale when participants sit and stand 5 times (Sit and Stand Test). Secondary outcomes include resting pain and fatigue, pain interference, fibromyalgia disease activity, movement-evoked fatigue, multidimensional assessment of fatigue, rapid assessment of physical activity, patient global impression of change, and common data elements shared across studies supported through the Helping to End Addiction Long-Term Initiative. IMPACT: The findings from this study will provide effectiveness data on TENS for individuals with fibromyalgia for health care policymakers, clinicians, and insurers. Data from this study will also inform future pragmatic trials for nonpharmacological interventions and chronic musculoskeletal pain conditions.

One-Day Acceptance and Commitment Therapy Compared to Support for Depressed Migraine Patients: a Randomized Clinical Trial.

In patients with migraine, depression is associated with poorer medical prognosis, decreased quality of life, and increased risk of suicidality and disability; yet, behavioral interventions have rarely been investigated. The current study compared the efficacy of two 1-day (5- to 6-h) interventions for co-occurring migraine and depression: (1) acceptance and commitment therapy plus migraine education (ACT-ED), and (2) support plus migraine education (S-ED). One hundred and thirty-six patients with comorbid depression and migraine were randomized to a treatment. One hundred and three (76%) completed the ACT-ED (N = 56) or S-ED (N = 47) workshop. Primary outcomes were depression diagnosis and symptoms. Secondary outcomes were anxiety symptoms, headache-related disability and general functioning, and quality of life. Assessments were completed at baseline and 3 and 6 months following the workshop. At the 6-month follow-up, on categorical outcomes, a significantly greater number of people in the ACT-ED condition no longer met criteria for a major depressive episode and exhibited a > 50% drop in symptoms on the Hamilton Rating Scale of Depression. Similarly, though, weaker results were found when examining depressive symptoms dimensionally. On secondary outcomes, people in the ACT-ED condition exhibited significantly greater improvements in anxiety, headache-related disability, and quality of social relationships, compared to S-ED, No differences between groups were observed in general functioning. A 1-day (5- to 6-h) ACT workshop can deliver substantial and lasting benefits to depressed migraineurs, over and above those provided by group support and education. This approach is an attractive alternative to weekly psychotherapy. Clinicaltrials.gov # NCT02108678.

Quantitative measurement of retinal hemorrhages in suspected victims of child abuse.

PURPOSE: Child abuse is one of the leading causes of death in early childhood. The presence of retinal hemorrhages often supports the diagnosis. The purpose of this study was to determine whether the specific measurement of retinal hemorrhages when present on fundus photography correlates with other clinical findings typically seen in children suspected of having been abused. METHODS: The medical records of children with retinal hemorrhages who were suspected of being victims of abusive head trauma from June 2003 to June 2013 and who had widefield retinal photography performed were retrospectively reviewed. Data collected included hemorrhage-covered percentage (HCP) of the central retina (posterior pole or 40° circle centered on fovea) measured by ImageJ in relation to death, length of hospital stay, presence of abnormal findings on neuroimaging or skeletal survey, and definite versus possible abuse. RESULTS: Significant difference in retinal hemorrhage measured on fundus photography was found in patients with axial skeletal fracture (P = 0.016), signs of severe brain trauma on neuroimaging (P = 0.014) and definite versus possible abuse (P = 0.023). No correlation of quantitative measurement of the retinal hemorrhage to length of hospital stay, death, or the presence of skull fracture was found in this cohort. CONCLUSIONS: The quantitative measurement of total retinal hemorrhage when present on fundus photography centered on posterior pole in children suspected of having been abused correlated with some but not all findings typically seen in abused children.

Transcutaneous electrical nerve stimulation for the control of pain during rehabilitation after total knee arthroplasty: A randomized, blinded, placebo-controlled trial.

This study evaluated the efficacy of transcutaneous electrical nerve stimulation (TENS) in reducing pain and hyperalgesia and increasing function after total knee arthroplasty (TKA). We hypothesized that participants using TENS during rehabilitation exercises would report significantly lower pain during range-of-motion (ROM) activity and fast walking but not at rest, would have less hyperalgesia, and would have better function than participants receiving placebo-TENS or standard care. We also hypothesized that change in ROM pain would differ based on psychological characteristics (trait anxiety, pain catastrophizing, and depression) and treatment group. This prospective, randomized study used intent-to-treat analyses in 317 participants after primary, unilateral TKA. Assessors, blinded to treatment allocation, measured pain, function (ROM and gait speed), and hyperalgesia (quantitative sensory tests) postoperatively and 6 weeks after surgery. Analgesic intake, anxiety, depression, and pain catastrophizing were also assessed. TENS participants used it 1 to 2 times per day at 42 mA (on average) and had less pain postoperatively during active knee extension (P=.019) and fast walking (P=.006) than standard care participants. TENS and placebo-TENS were not significantly different. TENS participants who scored low on anxiety and pain catastrophizing had a greater reduction in ROM pain at 6 weeks than those who scored high on these factors (P=.002 and P=.03). Both TENS and placebo-TENS participants had less postoperative mechanical hyperalgesia (P=.03-.01) than standard care participants. Supplementing pharmacologic analgesia with TENS during rehabilitation exercises reduces movement pain postoperatively, but a placebo influence exists and the effect is gone by 6 weeks. Patients with low anxiety and pain catastrophizing may benefit most from TENS.

Iron status in toddlerhood predicts sensitivity to psychostimulants in children.

OBJECTIVE: Iron deficiency is associated with impaired dopaminergic signaling and externalizing behavior. The authors examine, whether iron stores in toddlerhood influence later response to psychostimulants. METHOD: Youth participating in a study monitoring the long-term safety of risperidone were included in this analysis if they had received psychostimulant monotherapy for at least 3 weeks and had a complete blood count obtained before psychostimulant treatment. Sensitivity to psychostimulants was defined based on the weight-adjusted dose during the 1st year of treatment. Regression analysis examined whether the hematological tests based on the characteristics of red blood cells were associated with sensitivity to psychostimulants. RESULTS: A total of 29 participants (93% men; 76% Whites), primarily with ADHD (93%), comprised the current sample. The hematological tests were obtained, on average, 3 years before the initiation of psychostimulants monotherapy that occurred at 5.8 years of age and continued for a median of 0.85 years, at an average daily dose of 0.98 mg/kg (SD = 0.38) in methylphenidate equivalent. Compared with those who were poorly sensitive to psychostimulants, after adjusting for age, mean corpuscular volume was significantly higher in the highly and moderately psychostimulants sensitive groups. CONCLUSIONS: If replicated, these findings suggest that more attention should be paid to optimizing body iron in early childhood.

Predictors of postoperative movement and resting pain following total knee replacement.

This study determined preoperative predictors of movement and resting pain following total knee replacement (TKR). We hypothesized that younger patients with higher preoperative pain intensity, pain sensitivity, trait anxiety, pain catastrophizing, and depression would be more likely to experience higher postoperative movement pain than older patients with lower scores on these variables prior to surgery, and that predictors would be similar for resting pain. Demographics, analgesic intake, anxiety, depression, pain catastrophizing, resting pain, movement pain (ie, during active knee range of motion), and quantitative sensory tests were performed preoperatively on 215 participants scheduled for a unilateral TKR. On postoperative day 2, analgesic intake, resting pain, and movement pain were again assessed. Significant predictors of moderate or severe movement pain were higher preoperative movement pain, von Frey pain intensity, and heat pain threshold. People with severe movement pain preoperatively were 20 times more likely to have severe movement pain postoperatively. When the influence of preoperative movement pain was removed, depression became a predictor. Significant predictors of moderate to severe resting pain were higher preoperative resting pain, depression, and younger age. These results suggest that patients with higher preoperative pain and depression are more likely to have higher pain following TKR, and younger patients may have higher resting pain. Cutaneous pain sensitivity predicted movement pain but not resting pain, suggesting that mechanisms underlying movement pain are different from resting pain. Aggressive management of preoperative pain, pain sensitivity, and depression prior to surgery may facilitate postoperative recovery.