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Wound contact layer (WCL) dressings are intended to protect tissue during the healing process. A randomised controlled trial was undertaken to compare 2 such dressings. Outpatients with acute wounds were randomly allocated to treatment with either a soft silicone‐coated WCL (intervention group, n = 59) or a lipidocolloid‐impregnated WCL (control group, n = 62). At the first dressing removal (day 3), 89.8% of patients in the intervention group experienced non‐painful dressing removal (defined as a pain rating <30 mm on a 100 mm visual analogue scale), compared with 73.6% of patients in the control group (P = .017) (per protocol population). At day 21, wounds were considered as healed in 66.1% of patients in the intervention group compared with 43.5% in the control group (P = .012) (intention‐to‐treat population). Both dressings were well tolerated and rated highly in terms of in‐use characteristics, although the soft silicone‐coated WCL was rated significantly higher than the lipidocolloid‐impregnated WCL in terms of its ability to remain in place (P= .016). The results indicate that the soft silicone‐coated WCL is suitable for the management of acute wounds as it can minimise dressing‐associated pain and support healing.  相似文献   
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To describe the clinical and electrophysiological features evoking CMT4C, an autosomal recessive (AR) form of Charcot-Marie-Tooth disease (CMT) due to mutations in the SH3TC2 gene, we screened the coding sequence of SH3TC2 gene in 102 unrelated patients with a demyelinating or intermediate CMT and a family history compatible with an AR transmission. We identified among this cohort 16 patients carrying two mutations in the SH3TC2 gene, but medical records finally analyzed 14 patients. We report clinical, electrophysiological, and molecular data of 14 patients (9 men, 5 women) with CMT4C. Mean age at examination was 43.6 years (median = 42.5). Among the 14 studied cases 6 had scoliosis as the presenting sign. Cranial nerve involvement affecting either the VIIIth, VIIth, XIIth or a combination of the IXth and Xth nerves was noted in 10 patients. Remarkably, 50% of the patients had proximal limb involvement at the time of examination. The hallmark of the electrophysiological study was the presence of probable conduction block and temporal dispersion. Thus the clinical and paraclinical spectrum of CMT4C can guide the clinician to perform analysis of the SH3TC2 gene.  相似文献   
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BACKGROUND: Asking psychiatric in-patients about their drug consumption is unlikely to yield reliable results, particularly where alcohol and illicit drug use is involved. The main aim of this study was to compare spontaneous self-reports of drug use in hospitalized psychiatric patients to biological measures of same. A secondary aim was to determine which personal factors were associated with the use of tobacco, alcohol, and illicit drugs as indicated by these biological measures. METHODS: The consumption of substances was investigated using biological measures (urine cotinine, cannabis, opiates, cocaine, amphetamines and barbiturates; blood carbohydrate-deficient transferrin [CDT] and gamma-glutamyl transferase [GGT]) in 486 consecutively admitted psychiatric patients, one day following their hospitalization. Patients' self-reports of alcohol, tobacco and illicit drugs consumption were recorded. Socio-professional and familial data were also recorded. RESULTS: The results show a low correlation between biological measures and self-reported consumption of alcohol and illicit drugs. Fifty-two percent of the patients under-reported their consumption of illicit drugs (kappa=.47). Patients with schizophrenia and personality disorders were more likely to disclose their illicit drug consumption relative to patients suffering from mood disorders and alcohol dependence. Fifty-six percent of patients underreported alcohol use, as evaluated by CDT (kappa=.2), and 37% underreported when using the CDT+GGT measure as an indicator. Smoking appeared to be reported adequately. In the study we observed a strong negative correlation between cannabis use and age, a strong correlation between tobacco and cannabis use, and correlations between tobacco, cannabis and alcohol consumption. CONCLUSION: This study is the first to compare self-reports and biological measures of alcohol, tobacco and illicit drug uses in a large sample of inpatients suffering from various categories of psychiatric illnesses, allowing for cross-diagnosis comparisons.  相似文献   
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Very Long-Chain Acyl-CoA dehydrogenase (VLCAD) deficiency is an inborn error of mitochondrial long-chain fatty acid oxidation (FAO) most often occurring in childhood with cardiac or liver involvement, but rhabdomyolysis attacks have also been reported in adults. We report in this study the clinical, biochemical and molecular studies in 13 adult patients from 10 different families with VLCAD deficiency. The enzyme defect was demonstrated in cultured skin fibroblasts or lymphocytes. All patients exhibited exercise intolerance and recurrent rhabdomyolysis episodes, which were generally triggered by strenuous exercise, fasting, cold or fever (mean age at onset: 10 years). Inaugural life-threatening general manifestations also occurred before the age of 3 years in four patients. Increased levels of long-chain acylcarnitines with tetradecenoylcarnitine (C14:1) as the most prominent species were observed in all patients. Muscle biopsies showed a mild lipidosis in four patients. For all patients but two, molecular analysis showed homozygous (4 patients) or compound heterozygous genotype (7 patients). For the two remaining patients, only one mutation in a heterozygous state was detected. This study confirms that VLCAD deficiency, although being less frequent than CPT II deficiency, should be systematically considered in the differential diagnosis of exercise-induced rhabdomyolysis. Measurement of fasting blood acylcarnitines by tandem mass spectrometry allows accurate biochemical diagnosis and should therefore be performed in all patients presenting with unexplained muscle exercise intolerance or rhabdomyolysis.  相似文献   
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There are recent reports of effective treatment of cutaneous hemangiomas with Propranolol. The current study aims to assess efficacy of systemic Propranolol for subglottic hemangiomas and to discuss its place among the other available therapies. We report 2 infants with subglottic hemangiomas, which were resistant to other established medical treatments. One infant presented with PHACES association, the other with widespread cutaneous congenital hemangiomas. Both were subsequently treated with systemic Propranolol. Both patients’ subglottic hemangiomas responded dramatically to systemic Propranolol. No side effects of the therapy occurred, and a safety protocol previously designed for Propranolol prescribed for other indications was applied to our patients. Propranolol appears to be an effective treatment for subglottic hemangiomas and should now be used as a first-line treatment in subglottic hemangiomas when intervention is required.  相似文献   
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