• 대한임상검사과학회지
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• 제45권4호
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• pp.159-163
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• 2013

To investigate the cognitive degree about hepatitis and hepatosis, 916 subjects are examined with query and hepatotitis B, C, E test as well as s-AST and s-ALT as liver function test. Based on results, there are 4.9% of positive hepatitis and 8.9% of hepatosis and 13.8% of liver disorder. Among positive hepatitis, there are 93.3% of type B, 42.2% of type E and 6.7% of type C, respectively. From 45% of positive hepatitis B, they carry hepatitis B and E together. The cognitive degree about positive hepatitis is 64.4%, hepatosis 8.6%. The knowledge degree from cognitive group is higher than that of noncognitive group but there is no difference from hepatosis between two groups (p<0.001). The cognitive degree of liver disorder depends on academic background (p<0.001), mother's academic background (p<0.001), job (p<0.05) and family's income (p<0.001), showing significant difference. In summary, hepatitis carrier aware quite well about liver disorder but very low from hepatosis. Accordingly, the plan to increase a cognitive degree and continuous education as well as policy support to minimize spread of disease and to protect not to be worsen disease will be needed.

• Clinical and Experimental Pediatrics
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• 제57권2호
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• pp.91-95
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• 2014

Griscelli syndrome type 2 (GS2) is a rare autosomal recessive disease caused by mutations in the RAB27A gene. It is characterized by cutaneous hypopigmentation, immunodeficiency, and hemophagocytic lymphohistiocytosis. We describe 2 brothers who had GS2 with clinically diverse manifestations. The elder brother presented with a purely neurological picture, whereas the younger one presented with fever, pancytopenia, hepatosplenomegaly, and erythema nodosum. Considering that cutaneous hypopigmentation was a common feature between the brothers, genetic analysis for Griscelli syndrome was performed. As the elder sibling had died, mutation analysis was only performed on the younger sibling, which revealed a novel homozygous mutation in the RAB27A gene on chromosome 15 showing a single-base substitution (c.136T>A p.F46I). Both parents were heterozygous for the same mutation. This confirmed the diagnosis of GS2 in the accelerated phase in both siblings. The atypical features of GS2 in these cases are a novel mutation, isolated neurological involvement in one sibling, association with erythema nodosum, and 2 distinct clinical presentations in siblings with the same genetic mutation.

• Advances in pediatric surgery
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• 제2권2호
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• pp.81-87
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• 1996

Although nonoperative reduction plays a major role in the management of uncomplicated intussusception in the pediatric age group, surgical treatment is still a necessary alternative when nonoperative reduction is unsuccessful. The author analyzed the clinical features of 68 patients requiring operation in order to identify factors which might influence the type of operative management. A nine-year experience at Ewha Womans University Hospital was reviewed, and the findings compared to previous reports. Barium was used for the initial reduction attempt in 33 cases, saline in 35. Manual reduction by milking at operation achieved success in 41 cases(60.3%). Fifteen cases(22.1%) required resection of bowel, and 12 patients(17.6%) were found to have spontaneous and complete reduction of the intussusception at operation. Two cases had pathologic leading points. There were no perforations due to nonoperative reduction. There were no significant differences in demographic data, clinical findings, laboratory data, and anatomic type of intussusception between barium and saline reduction groups. However, a significant number of cases with spontaneous reduction were in saline reduction group(p<0.05). There was a slight chance of spontaneous reduction in infants under 6 month of age(p<0.001). Age under 6 month. body temperature over $38^{\circ}C$, symptom over 24 hours, and ileo-colic and ileo-ileo-colic intussusception contributed significantly to the necessity for bowel resection(p<0.05-0.001). The author believes that the age, body temperature, duration of illness, and anatomic type of intussusception strongly influence operative management.

• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.149-151
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• 2016

Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms involving compression of the cervicomedullary junction by ectopic tonsils. Several studies have reported the clinical symptoms associated with CM-I, including suboccipital headache, weakness in the upper extremities, facial numbness, loss of temperature sensation, ataxia, diplopia, dysarthria, dysphagia, vomiting, vertigo, nystagmus, and tinnitus. Syncope is one of the rarest presentations in patients with CM-I. There are many hypotheses regarding the causes of syncope in patients with CM-I; however, the mechanisms are not clearly understood. Although surgical decompression for CM-I in patients with syncope has yielded good clinical results in some studies, such cases are rarely reported. We report a case of orthostatic syncope in a patient with CM-I who was treated with surgical intervention.

• Journal of mucopolysaccharidosis and rare diseases
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• 제2권1호
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• pp.31-31
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• 2016

Purpose: To describle clinical features and enzyme activity of Vietnamese patients with Mucolipidosis type II. Methods: Clinical features, laboratory and plasma lysosom enzyme activity by 4 MU-Fluorometric assay was studied from 2014-2015 at the Northern referral center of Pediatrics - National Children's Hospital. Results: 16 cases (7 girls and 9 boys) were diagnosed with I-cell bases on clinical symptoms and enzyme activities studies. Diagnosis age was $5.93{\pm}4.28$ years, onset age was recognised from birth to 4 years (median 1.25) with the feature of joint stiffness and bone deformation. All cases presented with the feature of joint stiffness, chest deformation and kyphoscoliosis; Fifteen cases (93.7%) had coarse facial features. No patients had hepatosplenomegaly on abdominal ultrasound, 5/15 patients had heart valves disease. Enzyme assay showed ${\alpha}$-Hexosaminidase of $1,885.9{\pm}338.7$ (nmol/mg plasma/17 hrs), ${\alpha}$-Iduronate sulfatase of $4,534.8{\pm}1,062.9nmol/mg$ plasma/4 hrs). Conclusion: Mucolipidosis II seriously affected the life of the patients with skeletal deformities, contractures develop in all joints and cardiac involvement.

• 턱관절균형의학회지
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• 제4권1호
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• pp.21-26
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• 2014

본 증례에서는 원인 불명의 만성 재발성 악관절 탈구 및 개구 장애에 대해 CBA, TBA를 비롯한 FCST의 구조적 치료를 통해서도 유의한 치료변화가 나타남을 관찰하였다.

• Diabetes and Metabolism Journal
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• 제42권6호
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• pp.451-464
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• 2018

Latent autoimmune diabetes in adults (LADA) is a heterogeneous disease characterized by a less intensive autoimmune process and a broad clinical phenotype compared to classical type 1 diabetes mellitus (T1DM), sharing features with both type 2 diabetes mellitus (T2DM) and T1DM. Since patients affected by LADA are initially insulin independent and recognizable only by testing for islet-cell autoantibodies, it could be difficult to identify LADA in clinical setting and a high misdiagnosis rate still remains among patients with T2DM. Ideally, islet-cell autoantibodies screening should be performed in subjects with newly diagnosed T2DM, ensuring a closer monitoring of those resulted positive and avoiding treatment of hyperglycaemia which might increase the rate of ${\beta}-cells$ loss. Thus, since the autoimmune process in LADA seems to be slower than in classical T1DM, there is a wider window for new therapeutic interventions that may slow down ${\beta}-cell$ failure. This review summarizes the current understanding of LADA, by evaluating data from most recent studies, the actual gaps in diagnosis and management. Finally, we critically highlight and discuss novel findings and future perspectives on the therapeutic approach in LADA.

• Experimental and Molecular Medicine
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• 제50권12호
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• pp.7.1-7.14
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• 2018

Dysregulation of long noncoding RNA (lncRNA) expression is linked to the development of various diseases. Recently, an emerging body of evidence has indicated that lncRNAs play important roles in the pathogenesis of inflammatory bowel diseases (IBDs), including Crohn's disease (CD) and ulcerative Colitis (UC). In IBD, lncRNAs have been shown to be involved in diverse processes, including the regulation of intestinal epithelial cell apoptosis, association with lipid metabolism, and cell-cell interactions, thereby enhancing inflammation and the functional regulation of regulatory T cells. In this review, we aim to summarize the current knowledge regarding the role of lncRNAs in IBD and highlight potential avenues for future investigation. We also collate potentially immune-relevant, IBD-associated lncRNAs identified through a built-by association analysis with respect to their neighboring protein-coding genes within IBD-susceptible loci. We further underscore their importance by highlighting their enrichment for various aspects of immune system regulation, including antigen processing/presentation, immune cell proliferation and differentiation, and chronic inflammatory responses. Finally, we summarize the potential of lncRNAs as diagnostic biomarkers in IBD.

• 한방안이비인후피부과학회지
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• 제35권3호
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• pp.123-132
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• 2022

Objectives : The purpose of this study is to report a case of erythematotelangiectatic type rosacea and peripheral facial palsy improved by Korean medicine treatment with Soshioho-tang gagambang. Methods : The patient visited our clinic due to facial flushing and peripheral facial palsy symptoms. Based on Sanghallon provision, we treated this case with Soshiho-tang gagambang. The result of treatment was evaluated by Visual Analogue Scale(VAS), Dermatology Life Quality Index(DLQI) and House-Brackmann Grading System(HBGS). Results : After 12 days of taking Soshiho-tang gagambang, VAS of rosacea was decreased from 9 to 4 and DLQI was decreased from 22 to 5. After treatment, rosacea with facial flushing was improved and HBGS was reduced from 3 to 1. Conclusions : This study shows the possibility of taking Soshiho-tang gagambang for erythematotelangiectatic type rosacea and peripheral facial palsy derived from stress, fatigue and sleep disturbance according to Sanghallon provision.

• 대한족부족관절학회지
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• 제5권2호
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• pp.112-119
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• 2001

Purpose: The purpose of this study is to analyze the clinical prognostic factors which may affect the postoperative clinical results of the unstable ankle fractures. Materials and Methods: This study is based on 62 unstable ankle fractures treated by open reduction and internal fixation from May 1994 to June 2000, with a minimum follow-up period of 12 months(range: 13 months-7 years 3 months). The 62 patients were average 39.1 years old with male: female ratio of 41:21. Based on Lauge-Hansen classification, the supination-external rotation type was the most common with 36 (58.1%) cases. The clinical results was assessed by American Orthopaedic Foot and Ankle Society(AOFAS) functional scale. The sex, age, body weight, trauma-operation interval, operation time, cause of injury, fracture type were statistically analyzed as the possible postoperative clinical prognostic factors. Results: Postoperative AOFAS functional scale was average 82.1 points with 22(35.5 %) cases excellent, 12(19.4%) good. 16(25.8%) fair and 12(19.4%) cases poor results. The age and the operation time were found to be statistically significant factors affecting the prognosis(p<0.001). The sex, weight, trauma-operation interval factors did not significantly affect the clinical results. The pronation-external rotation type showed better clinical tendency among the fracture types, but without the statistical significance. Conclusion: The surgically treated unstable ankle fractures in patients whose age was above 41 years old or operation time exceeding 90 minutes showed significantly poor clinical results.