• Clinical and Experimental Pediatrics
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• v.53 no.11
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• pp.965-970
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• 2010

Purpose: The life expectancy of patients with spinal muscular atrophy (SMA) type I is generally considered to be less than 2 years. Recently, with the introduction of proactive treatments, a longer survival and an improved survival rate have been reported. In this study, we analyzed the natural courses and survival statistics of SMA type I patients and compared the clinical characteristics of the patients based on their survival periods. Methods: We reviewed the medical records of 14 pediatric patients diagnosed with SMA type I during a 9-year period. We examined the demographic and clinical characteristics of these patients, calculated their survival probabilities, and plotted survival curves as on the censoring date, January 1, 2010. We also compared the characteristics of the patients who died before the age of 24 months (early-death, ED group) and those who survived for 24 months or longer (long-survival, LS group). Results: The mean survival time was $22.8{\pm}2.0$ months. The survival probabilities at 6 months, 12 months, 18 months, 24 months, and 30 months were 92.9%, 92.9%, 76.0%, 76.0%, and 65.1%, respectively. Birth weight was the only factor that showed a statistically significant difference between the ED and LS groups ($P$=0.048). Conclusion: In this study, the survival probabilities at 2 years were far greater than expected. Because of the limited number of patients and information in this study, the contribution of improved supportive care on longer survival could not be clarified; this may be elucidated in larger cohort studies.

• Clinical and Experimental Pediatrics
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• v.51 no.12
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• pp.1350-1354
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• 2008

Spinal muscular atrophy (SMA) is an autosomal recessive disease characterized by diffuse proximal and distal weakness due to deletion of the survival motor neuron (SMN) gene localized on chromosome 5 (5q11.2-13.3). SMA has been considered as a pure lower motor neuron disorder, and a definitive diagnosis can be established by molecular genetic testing. Here, we describe two patients with severe hypotonia and frequent aspirations at early infancy. Nerve conduction studies showed more extensive sensory involvement in these patients diagnosed to have SMA by genetic study than in classical cases of SMA. To the best of our knowledge, this is the first report of SMA Type 1 with sensory nerve involvement in Korea.

• Clinics in Shoulder and Elbow
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• v.22 no.2
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• pp.70-78
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• 2019

Background: This study evaluated postoperative changes in the supraspinatus from time-zero to 6 months, using magnetic resonance imaging (MRI). We hypothesized that restoration of the musculotendinous unit of the rotator cuff by tendon repair immediately improves the rotator cuff muscle status, and maintains it months after surgery. Methods: Totally, 76 patients (29 men, 47 women) with rotator cuff tears involving the supraspinatus tendon who underwent arthroscopic rotator cuff repairs were examined. MRI evaluation showed complete repair with intact integrity of the torn tendon at both time-zero and at 6 months follow-up. All patients underwent standardized MRI at our institution preoperatively, at 1 or 2 days postoperative, and at 6 months after surgery. Supraspinatus muscular (SSP) atrophy (Thomazeau grade) and fatty infiltrations (Goutallier stage) were evaluated by MRI. The cross-sectional area of SSP in the fossa was also measured. Results: As determined by MRI, the cross-sectional area of SSP significantly decreased 11.41% from time-zero (immediate repair) to 6 months post-surgery, whereas the Goutallier stage and Thomazeau grade showed no significant changes (p<0.01). Furthermore, compared to the preoperative MRI, the postoperative MRI at 6 months showed a no statistically significant increase of 8.03% in the cross-sectional area. In addition, morphological improvements were observed in patients with high grade Goutallier and Thomazeau at time-zero, whereas morphology of patients with low grade factors were almost similar to before surgery. Conclusions: Our results indicate that cross-sectional area of the initial repair appears to decrease after a few months postoperatively, possibly due to medial retraction or strained muscle.

• Journal of rehabilitation welfare engineering & assistive technology
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• v.10 no.2
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• pp.107-112
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• 2016

The purpose of this study was to determine the effect of aquatic ramp walking exercise on the activity of the quadriceps, gait and activity of daily living in child with Spinal Muscular Atrophy (SMA) type II. A 5 years-old girl with SMA type II participated in this study. This study used single-subject reverse(A-B-A) design study. There are 12 sessions(4weeks 3 times a week) each during the baseline phase(A), the intervention phase(B), the follow up phase(A). During the baseline phase and the follow up phase performed general aquatic therapy, the intervention phase additional performed walking activity on ramp in pool (60m). Surface electromyogram, Timed Up and Go (TUG) test, ACTIVLIM were used as outcome. During the intervention phase, there were decrease on the activity of the quadriceps. In modified TUG test, gait time reduced during the intervention phase. The ACTIVLIM logit score increased during intervention phase by comparison with the baseline phase. These findings suggest that an aquatic ramp walking exercise activities have the therapeutic possibility on the quadriceps activity and gait ability for child with SMA type II.

• Journal of Life Science
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• v.28 no.4
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• pp.435-443
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• 2018

AMP-activated protein kinase (AMPK) functions as a metabolic master through regulating and restoring cellular energy balance. In skeletal muscle, AMPK increases myofibril protein degradation through the expression of muscle-specific ubiquitin ligases. Mori Folium, the leaf of Morus alba, is a traditional medicinal herb with various pharmacological functions; however, the effects associated with muscle atrophy have not been fully identified. In this study, we confirmed the effects of AMPK activation by examining the effects of 5-aminoimidazole-4-carboxamide ribonucleotide (AICAR), an activator of AMPK, on the induction of atrophy and expression of atrophy-related genes in C2C12 myotubes. We also investigated the effects of the ethanol extract of Mori Folium (EEMF) on the recovery of AICAR-induced muscle atrophy in C2C12 myotubes. It was found that exposure to AICAR resulted in the stimulation of Forkhead box O3a (FOXO3a); an up-regulation of muscle-specific ubiquitin ligases such as Muscle Atrophy F-box (MAFbx)/atrogin-1 and muscle RING finger-1 (MuRF1), and a down-regulation of muscle-specific transcription factors, such as MyoD and myogenin; with the activation of AMPK. In addition, AICAR without cytotoxicity indicated a decrease in diameter of C2C12 myotubes. However, treatment with EEMF significantly suppressed AICAR-induced muscle atrophy of C2C12 myotubes in a dose-dependent manner as confirmed by a decrease in myotube diameter, which is associated with a reversed stimulation of FOXO3a by the inhibition of AMPK activation. These results indicate that the activation of AMPK by AICAR induces muscle atrophy, and EEMF has preeminent effects on the inhibition of AICAR-induced muscle atrophy through the AMPK signaling pathway.

• Journal of Korean Academy of Nursing
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• v.43 no.3
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• pp.361-369
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• 2013

Purpose: The purpose of this study was to examine the effect of anorexia and neuropathic pain induced by cisplatin on hindlimb muscles of rats. Methods: Adult male Sprague-Dawley rats were divided into two groups, a cisplatin-treated group (n=10) and a control group (n=10). In the cisplatin-treated group, cisplatin at a dose of 2 mg/kg was injected intraperitoneally two times a week up to a cumulative dose of 20 mg/kg over 5 weeks, and in the control group saline (0.9% NaCl) was injected intraperitoneally at the same dose and duration as the cisplatin-treated group. At 34 days all rats were anesthetized, after which the soleus and plantaris muscles were dissected. Withdrawal threshold, body weight, food intake, activity, muscle weight, Type I and II fiber cross-sectional areas and myofibrillar protein content of the dissected muscles were determined. Results: Compared with the control group, the cisplatin-treated group showed significant decreases (p<.05) in withdrawal threshold, activity, food intake, body weight, Type I and II fiber cross-sectional areas, myofibrillar protein content and weight of the soleus and plantaris muscles. Conclusion: Muscular atrophy in hindlimb occurs due to anorexia and neuropathic pain induced by the cisplatin treatment.

• The Journal of Korean Physical Therapy
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• v.3 no.1
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• pp.175-188
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• 1991

A study was performed to investigate the effect of electrical stimulation on disused rat soleus muscle, of male rat. The animal's hindlimbs were immobilized 4weeks by plaster of paris, and stimulated with E. S. T for 4weeks (20min/day) The changes on soleus were examined with histochemical, histological, and morphometric method. The results are summarized as follows. 1. Disued atrophy group from immobilization, which margin of sarcolemma and myofibril in sarcoplasm were not cleared, also degenerated from necrosis with phagocytosis. 2. The numbers of nuclear were much increased and accumulation of nuclear were finded, and relatively muscular atrophic changed. 3. Increased inflammatory cyte, also finded neutrophil and macrophage. 4. Relatively atrophic changed from severe fibrosis by incleased connectivetissue. 5. The glycogen granules were much decreased in E. S. T group. It means that electrical stimulation effected the muscle exercise. 6, The activity of the NADH-TR reaction of E. S. T. Tgroup were white muscle group are transformed into red muscle fiber than normal group. 7. These results indicate that the electrical stimulation effected to soleus also prevention and delayed muscular atrophy.

• Journal of Korean Medicine Rehabilitation
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• v.19 no.4
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• pp.151-163
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• 2009

Objectives : In the assessment of the lumbar spine by magnetic resonance imaging (hereinafter, "MRI"), changes in the paraspinal muscles are overlooked. The purpose of our study is to examine the correlation between the multifidus muscle atrophy on MRI findings and the clinical findings in low back pain (hereinafter, "LBP") patients. Methods : The retrospective study on 38 LBP patients, presenting either with or without associated leg pains, was undertaken. The MRI findings on the patients were visually analysed to find out a lumbar multifidus muscle atrophy, disc herniation, disc degeneration, spinal stenosis and nerve root compressions. The clinical history in each case was obtained from their case notes and pain drawing charts. Results : The lumbar multifidus muscle atrophy has occurred from more than 80% of the patients with LBP. Most of lumbar multifidus muscle atrophies have increased from lower level of lumbar spine. It was bilateral in the majority of the cases. In addition, multifidus muscle atrophy was correlated to the patient's age, disc degenerations and spinal stenosis. On the contrary, gender, the duration of LBP, referred leg pain, disc herniation and nerve root compressions had no relevance to multifidus muscle atrophies. Therefore, when assessing the MRIs of the lumbar spine, we should have more attetion on multifidus muscle, because it has lot's of information about spinal neuropathy problems. Conclusions : Therefore, the examination of multifidus muscle atrophies should be considered when assessing the MRIs of the lumbar spine. In addition, it helps to evaluate and plan the treatment modalities of LBP. Moreover, it prevents from LBP by discovering the importance between the multifidus muscle and the spine stabilization exercise.

• Journal of Korean Academy of Nursing
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• v.39 no.3
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• pp.393-400
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• 2009

Purpose: The purpose of this study was to examine the effects of unilateral sciatic nerve injury on unaffected hindlimb muscles of rats. Methods: Adult male Sprague-Dawley rats were assigned to one of three groups: control(C) group(n=10) that had no procedures, sham(S) group(n=10) that underwent sham left sciatic nerve transection, and sciatic nerve transection(SNT) group(n=9) that underwent left sciatic nerve transection. At 15 days rats were anesthetized, and the soleus, plantaris and gastrocnemius muscles were dissected. Results: Muscle weight of the unaffected plantaris muscle in the SNT group was significantly lower than in the other two groups. Type II fiber cross-sectional areas of the unaffected plantaris and gastrocnemius muscles in the SNT group were significantly smaller than in the other two groups. The decrease of muscle weights and Type I, II fiber cross-sectional areas of the unaffected three muscles in the SNT group were significantly less than that of the affected three muscles. Conclusion: Hindlimb muscle atrophy occurs in the unaffected side after unilateral sciatic nerve injury, with changes in the plantaris and gastrocnemius muscle being more apparent than changes in the soleus muscle. These results have implications for nursing care, in the need to assess degree of muscle atrophy in unaffected muscles as well as affected muscles.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.37
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• pp.29.1-29.7
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• 2015

Facial asymmetry is found in patients with or without cosmetic facial alterations. Some patients have facial asymmetry that manifests underlying skeletal problems, while others have only limited soft-tissue facial asymmetry. Orthognathic surgery brings about a dermatic change, as soft tissue covers underlying bones. Limited soft-tissue asymmetry, meanwhile, is difficult to correct. The treatment modalities for the creation or restoration of an esthetically pleasing appearance were autogenous fat grafts, cartilage graft, and silicon injections. A young female patient had right-side facial asymmetry. The clinical assessment involved visual inspection of the face and palpation to differentiate soft tissue and bone. Although the extra-oral examination found facial asymmetry with skin atrophy, the radiographic findings revealed no mandibular atrophy or deviation. She was diagnosed as localized scleroderma with muscle spasm. In conclusion, facial asymmetry patients with skeletal asymmetry can be esthetically satisfied by orthognathic surgery; however, facial atrophy patients with skin or subdermal tissue contraction need treatment by cosmetic dermatological surgery and orthodontic correction.