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Purpose Severe sepsis is associated with functional disability among patients surviving an acute phase of infection. Efforts to improve functional impairment are important. We assessed the effects of early exercise rehabilitation on functional outcomes in patients with severe sepsis. Materials and methods A prospective, single-center, case-control study was conducted between January 2013 and May 2014 at a tertiary care center in Korea. Patients with severe sepsis and septic shock were enrolled and randomized to receive standard sepsis treatment or intervention. Intervention involved early targeted physical rehabilitation with sepsis treatment during hospitalization. Participants were assessed at enrollment, hospital discharge, and 6 months after enrollment. Functional recovery was measured using the Modified Barthel Index (MBI), Functional Independence Measure (FIM), and Instrumental Activities of Daily Living (IADL). Results Forty participants (21 intervention patients) were included in an intention-to-treat analysis. There were no significant differences in baseline MBI, FIM, and IADL between groups. Intervention yielded greater improvement of MBI, FIM, and IADL in the intervention group at hospital discharge, but not significantly. Subgroup analysis of patients with APACHE II scores ≥10 showed significantly greater improvement of physical function at hospital discharge (MBI and FIM) in the intervention group, compared to the control group (55.13 vs. 31.75, p=0.048; 52.40 vs. 31.25, p=0.045). Intervention was significantly associated with improvement of MBI in multiple linear regression analysis (standardized coefficient 0.358, p=0.048). Conclusion Early physical rehabilitation may improve functional recovery at hospital discharge, especially in patients with high initial severity scores.

Purpose This study was designed to investigate the characteristics of Korean patients with calpainopathy. Materials and methods Thirteen patients from ten unrelated families were diagnosed with calpainopathy via direct or targeted sequencing of the CAPN3 gene. Clinical, mutational, and pathological spectra were then analyzed. Results Nine different mutations, including four novel mutations (NM_000070: c.1524+1G>T, c.1789_1790inA, c.2184+1G>T, and c.2384C>T) were identified. The median age at symptom onset was 22 (interquartile range: 15-28). Common clinical findings were joint contracture in nine patients, winged scapula in four, and lordosis in one. However, we also found highly variable clinical features including early onset joint contractures, asymptomatic hyperCKemia, and heterogeneous clinical severity in three members of the same family. Four of nine muscle specimens revealed lobulated fibers, but three showed normal skeletal muscle histology. Conclusion We identified four novel CAPN3 mutations and demonstrated clinical and pathological heterogeneity in Korean patients with calpainopathy.

The purpose of this study was to investigate the function of the autonomic nervous system in children with spastic cerebral palsy (CP) through an analysis of heart rate variability (HRV) occurring with orthostatic stress. Twelve children with spastic CP and twelve normal children participated in this study. The echocardiogram (ECG) signals were recorded for 3 minutes in both the supine and 70 degrees C head-up tilt positions, and then the HRV signals underwent power spectrum analysis at each position. Two components were measured; a low- frequency (LF) component (0.05 - 0.15 Hz) primarily reflecting sympathetic activity during orthostatic stress and a high-frequency (HF) component (0.15 - 0.4 Hz) reflecting parasympathetic activity. In the supine position, there was no significant difference between any of the HRV components of the two groups. In the head-up tilt position, absolute and normalized LF were significantly increased and absolute HF was significantly decreased in the normal children (p < 0.05), but not in the children with spastic CP. The results of this study suggest that cardiac autonomic functions, such as vagal withdrawal and sympathetic activation which occur during head-up tilt position, are not sufficient to overcome the orthostatic stress arising in spastic CP children.

Stem cells are emerging as therapeutic candidates in a variety of diseases because of their multipotent capacities. Among these, mesenchymal stem cells (MSCs) derived from bone marrow, umbilical cord blood or adipose tissue, comprise a population of cells that exhibit extensive proliferative potential and retain the ability to differentiate into multiple tissue-specific lineage cells including osteoblasts, chondrocytes, and adipocytes. MSCs have also been shown to enhance neurological recovery, although the therapeutic effects seem to be derived from an indirect paracrine effect rather than direct cell replacement. MSCs secrete neurotrophic factors, promote endogenous neurogenesis and angiogenesis, encourage synaptic connection and remyelination of damaged axons, decrease apoptosis, and regulate inflammation primarily through paracrine actions. Accordingly, MSCs may prevail as a promising cell source for cell-based therapy in neurological diseases.