Canavan disease(CD) is a rare autosomal recessive leukodystrophy caused by the deficiency of aspartoacylase and the accumulation in brain of N-acetylaspartate(NAA). CD has been reported mainly Ashkenazi Jews but also occurs in other ethnic groups. Usually it presents as early as the third month of life with megalencephaly, hypotonia later progressing to hypertonia, psychomotor and mental retardation, blindness, occasionally deafness and seizure. Diagnosis is based on the clinical feature, N-acetylaspartic aciduria, radiologic and pathologic findings. Histologically, the affected white matter shows extensive vacuolation and demyelination. There is no treatment for CD and the only prevention is through genetic counselling and prenatal diagnosis. We experienced a case of Canavan disease that was presented with hypotonia and developmental delay. Diagnosis was confirmed histologically. Radiologic findings are extensive high signal throughout the white matter on T2-weighted MRI and increased NAA peak and decreased choline peak of the white matter on MR spectroscopy.
Blindness ; Brain ; Canavan Disease* ; Choline ; Deafness ; Demyelinating Diseases ; Diagnosis ; Ethnic Groups ; Humans ; Intellectual Disability ; Jews ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Muscle Hypotonia ; Prenatal Diagnosis ; Seizures

No abstract available.

PURPOSE: This research was conducted to identify the effects of providing support programs to families of elderly with dementia on family empowerment and attitudes toward dementia. METHODS: A nonequivalent control group pre- and post-test design was used. Participants were 49 family caregivers, experimental (24) and control (25), recruited from families through a dementia support center in Y district. This program implemented Haearim, a support program for family caregivers of demented elders developed by the National Institute of Dementia in 2016. Outcome measures were empowerment and attitudes toward to dementia. Data were analyzed with a χ2-test, independent t-test, Fisher's exact probability test, paired t-test, and repeated measures ANOVA with SPSS/PC version 20.0. RESULTS: Family empowerment (F=6.84, p=.002) and family caregivers' attitudes (F=16.48, p < .001) toward dementia in the experimental group improved significantly more than that of the control group after intervention. CONCLUSION: Results indicate that support programs for families of elderly with dementia are effective in positively changing empowerment and attitudes toward dementia among family caregivers.
Aged ; Caregivers ; Dementia ; Humans ; Outcome Assessment (Health Care) ; Power (Psychology)

Purpose: The purpose of this study was to identify the mediating effect of work engagement and job crafting on the relationship between nurses’ Grit on nursing performance. Methods: The data were collected using structured questionnaires through online surveys from 142 clinical nurses in five hospitals. Data were analyzed using SPSS 24.0, Hayes’s SPSS Process Macro 4.0 version program. Results: Nursing performance was significantly associated with Grit, work engagement and job crafting. The mediating effect of job crafting on the Grit on nursing performance was confirmed. Conclusion These results suggest that it is necessary to develop an intervention program that focuses on improving job crafting. Further, job crafting needs to be considered in developing nursing interventions to nurses' Grit and improve nursing performance for nurses.

Purpose: Intrahepatic recurrence has a significant effect on the survival of hepatocellular carcinoma (HCC) patients. We aimed to determine if there are useful indicators in predicting the recurrence of liver cancer after a hepatic resection. Methods: We retrospectively reviewed medical records of 210 HCC patients who underwent hepatectomy between January 2009 and December 2015. We examined clinic-pathological variables comparing 2 groups of HCC patients, either intrahepatic recurrence or not. Results: We divided 184 patients into 2 groups; 94 patients (51.1%) with intrahepatic recurrence and 90 patients (48.9%) without intrahepatic recurrence. Multivariate analysis showed operation type, preoperative α-FP, postoperative protein induced by vitamin K absence-II (PIVKA-II) elevation, and multiple tumor number were closely associated with intrahepatic recurrence. The preoperative PIVKA-II level was not statistically significant in postoperative intrahepatic recurrence rate. The recurrence rate was 46.2% in 132 of 184 cases of α-FP < 100 ng/mL group. Of the 184 cases, α-FP 100–200 ng/mL were 14 cases (12 cases recurred (85.7%)). Alpha-fetoprotein > 200 ng/mL was 38 of 184 cases, 21 of which recurred (55.3%). According to the multivariate analysis, OR ratio was 8.003 (95% confidence interval [CI], 1.549–41.353) in the α-FP 100–200 ng/mL group and 1.867 (95% CI, 0.784–4.444) in α-FP 200 ng/mL or higher group (P = 0.013). Three-year survival rate of intrahepatic recurrence patients was 80.7%, 3-year survival rate of no intrahepatic recurrence patients was 95.0%. Conclusion The intrahepatic recurrence rates were high in the group preop α-FP over 100 ng/mL. Close observation is needed. Prospective study for α-FP genes of HCC patients should be planned for predicting intrahepatic recurrence after hepatectomy.

Intradural schwannoma accompanied with torsion is rare. Intradural lumbar schwannoma was found in a 63-year-old man presenting with right sacral pain radiating to the lower extremity. This mass showed minimal enhancement on MRI. The tumor's location changed each time on MRI and CT-myelography. The patient underwent surgical resection and the cauda equina, linked to this schwannoma, was severely twisted.

Intradural schwannoma accompanied with torsion is rare. Intradural lumbar schwannoma was found in a 63-year-old man presenting with right sacral pain radiating to the lower extremity. This mass showed minimal enhancement on MRI. The tumor's location changed each time on MRI and CT-myelography. The patient underwent surgical resection and the cauda equina, linked to this schwannoma, was severely twisted.

PURPOSE: To find the effect of visual feedback of head angle with using a mobile posture-aware system on craniocervical angle and neck and shoulder muscles fatigue for preventing or decreasing the forward head posture. METHODS: Twenty-four healthy young adults in Chungbuk to participate in this study. The subjects started to watch a movie clip for 10 minutes with visual feedback in 0°, 30°, and 60° of head angles. During the task, surface electromyography (EMG) was used to collect data from the upper trapezius (UT), sternocleidomasetoid muscle (SCM), cervical erecter spinae (CES) during watching the smartphone. Craniocervical angles were measured using a sagittal-view photograph of the subject in a sitting posture. A one-way repeated analysis of variance with a significant level of 0.05 used for statistical analysis. RESULTS: Craniocervical angle with 0° visual feedback was significantly greater than 30°and 60°. Craniocervical angle with 30° visual feedback was significantly greater than 60°. In addition, MDF of UT muscles in 0° and 30° of visual feedback was significantly greater than 60°. CONCLUSION: We concluded that 0° visual feedback of head angle with using a mobile posture-aware system would be beneficial to prevent or decrease forward head posture during watching a smartphone. We also could recommend using of 30° visual feedback in case of caring UT muscle fatigue primarily.
Chungcheongbuk-do ; Electromyography ; Fatigue* ; Feedback, Sensory* ; Head* ; Humans ; Muscle Fatigue ; Muscles* ; Neck* ; Posture ; Shoulder* ; Smartphone* ; Superficial Back Muscles ; Young Adult

Retroperitoneal hemorrhage is a potentially lethal complication in patients on anticoagulant therapy and can be caused by ovarian artery bleeding, regardless of the patients’ age and obstetric history. This case illustrates the clinical presentation of ovarian artery bleeding in a postmenopausal female on anticoagulant therapy, the diagnostic utility of transcatheter angiography, and successful embolization.

PURPOSE: To determine accurately the extent of the disease and risk of recurrence is important in enhancing the therapeutic success rate of breast cancer. Primary tumor state and axillary node invasion were some well known factors to predict the prognosis of breast cancer. However, some patients with early stage cancer developed systemic metastasis later despite of little possibility of recurrence based on some previously establised prognostic system. These results demand another approach to predict systemic metastases in patient without gross evidence of further recurrence. Micrometastases is a promising key to explain the recurrence in these patients and micrometastases in bone marrow could raised the ongoing recurrence in skeletal system which is the most frequent metastatic site in breast cancer. Therefore we tricd to determine the rate of micrometastasis in surgically resectable Korean breast cancer patients and the relationship with clinicopathological characteristics of the cancer. MATERIALS AND METHODS: We studied bone marrow aspirate specimens from 38 patients with breast cancer who underwent curative resection at Chonnam University Hospital from January 1996 to February 1997. And reverse transcription polymerase chain reaction (RT-PCR) to detect messenger RNA for cytokeratin 19 was performed. RESULTS: Metastases in bone marrow were detected in 8/38 patients (21.1%). No sta- tistically significant relationship existed between bone marrow metastasis and clinicopa- thological parameters for predicting prognosis that consisted of tumor state, lymph node invasion, histologic grade, steroid receptor, and c-erbB2 overexpression. In particular, bone marrow metastasis developed even from ductal carcinoma in situ. CONCLUSION: Bone marrow metastasis may be developed from the extremely early stage of breast cancer and we can not make the corelationship between the bone marrow metastasis and establised some prognostic factors. Based on these results, we recommand the evaluation of bone marrow metastasis in all breast cancer patients and require the close follow-up to allow more sensitive prediction of ongoing recurrence and higher curability.
Bone Marrow* ; Breast Neoplasms* ; Breast* ; Carcinoma, Intraductal, Noninfiltrating ; Humans ; Jeollanam-do ; Keratin-19 ; Lymph Nodes ; Neoplasm Metastasis ; Neoplasm Micrometastasis* ; Polymerase Chain Reaction ; Prognosis ; Receptors, Steroid ; Recurrence ; Reverse Transcription ; RNA, Messenger