1.A Case of Myasthenia Gravis.
Journal of the Korean Ophthalmological Society 1975;16(3):260-264
Myasthenia Gravis is a chronic disease characterized by excessive fatiguability of striated muscle. The illness has a tendency to exacerbation and to the periods of spontaneous remission. The auther observed a case of ocular myasthenia gravis who has gained spontaneous remission 5 years after from the onset, and reviewed the references related to this disease.
Chronic Disease
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Muscle, Striated
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Myasthenia Gravis*
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Remission, Spontaneous
3.Clinical Characteristics at Diagnosis and Course of Korean Patients with Crohn's Disease.
Jong Beom PARK ; Suk Kyun YANG ; Seung Jae MYUNG ; Jeong Sik BYEON ; Yun Jung LEE ; Gin Hyug LEE ; Hwoon Yong JUNG ; Weon Seon HONG ; Jin Ho KIM ; Young Il MIN
The Korean Journal of Gastroenterology 2004;43(1):8-17
BACKGROUND/AIMS: The aim of this study was to evaluate the clinical characteristics and course of Korean patients with Crohn's disease (CD). METHODS: A total of 113 patients who were newly diagnosed as having CD at the Asan Medical Center between October 1989 and December 2001 were included. RESULTS: The male to female ratio was 1.9:1 and the mean age at diagnosis was 24.2 +/- 7.8 years. The disease location was the small bowel in 19.5%, the large bowel in 11.5%, and the both small and large bowels in 69.0% of the patients. Empirical anti-tuberculous medications were administered to 53 cases (46.9%). Major complications of CD were intestinal obstruction (25.7%) and perianal fistula (42.5%). The cumulative remission rate was 75.7% at 1 year and 80.7% at 3 years from diagnosis. The cumulative relapse rate was 30.7% at 1 year and 53.0% at 3 years from remission. The cumulative operation rate was 11.5% at 1 year and 13.1% at 3 years from diagnosis. CONCLUSIONS: There are no differences in the remission and recurrence rates between Korean and Western patients with CD. Although the cumulative operation rate seems to be lower in Korea than in Western countries, the cause of the difference is uncertain. Further studies are needed to determine the characteristics of CD in Korea.
Adolescent
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Adult
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Crohn Disease/complications/*diagnosis/therapy
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English Abstract
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Female
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Humans
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Korea
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Male
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Remission Induction
4.A Case of Severe Ocular Myasthenia Gravis that Developed Total External Ophthalmoplegia.
Journal of the Korean Ophthalmological Society 1988;29(6):1131-1139
Myasthenia gravis is a chronic disease characterized by voluntary muscle weakness and fatigue. Myasthenia gravis was first described by Thomas Willis in 1672. The etiology of myasthenia gravis is not clarified but recently it has been suggested that it is an autoimmune disease. Ocular myasthenia gravis is characterized clinically by blepharoptosis and external ophthalmopleia. The illness has a tendency to exacerbation and spontaneous remission. The authors observed a case of severe ocular myasthenia gravis which had developed total external ophthalmoplegia. And we reviewed the literature of recent years related to myasthenia gravis, particulary for its etiology, clinical course, pathology, diagnosi, treatment, and prognosis.
Autoimmune Diseases
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Blepharoptosis
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Chronic Disease
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Fatigue
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Muscle, Skeletal
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Myasthenia Gravis*
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Ophthalmoplegia*
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Pathology
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Prognosis
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Remission, Spontaneous
5.Therapeutic Efficacy of Oral Enteral Nutrition in Pediatric Crohn's Disease: A Single Center Non-Comparative Retrospective Study.
Hyun Jin KIM ; Young KIM ; Jin Min CHO ; Seak Hee OH ; Kyung Mo KIM
Yonsei Medical Journal 2016;57(5):1185-1191
PURPOSE: Exclusive enteral nutrition (EEN) therapy effectively induces clinical remission in Crohn's disease (CD). It remains unclear, however, whether partial enteral nutrition (PEN) can maintain remission. This study was performed to determine the abilities of oral EEN and oral PEN to induce and maintain clinical remission in pediatric patients with CD, respectively. MATERIALS AND METHODS: Pediatric patients with CD who received oral EEN at a single center in 2000-2014 were identified retrospectively. Remission rates of the EEN and PEN during the 2 years study period were determined. Risk factors for EEN and PEN failure were identified. RESULTS: Of the 66 patients who started EEN, 61 (92%) completed the course. Clinical remission was achieved in 88% (58/66) of the patients. All 58 patients with remission continued with PEN: 43 (74%) were treatment adherent. The cumulative remission rates at 1 and 2 years were 67% and 52%, respectively. Differing from EEN, limited therapeutic efficacy of PEN was shown in severe CD patients. Female gender associated significantly with non-adherence. CONCLUSION: Oral EEN and PEN effectively induced and maintained remission in a pediatric population. Non-adherence was a limiting factor in the success of therapy.
Adolescent
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Child
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Crohn Disease/*therapy
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Enteral Nutrition/*methods
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Female
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Humans
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Male
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Remission Induction
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Retrospective Studies
6.Diagnosis and Treatment of Anorectal Lesions in Crohn's Disease.
Youn Jung HEO ; Won Kap PARK ; Jae Cheol KIM ; Jong Kyun LEE ; Kwang Yeon KIM
Journal of the Korean Society of Coloproctology 2010;26(3):190-196
PURPOSE: Anorectal lesions in patients with Crohn's disease (CD) are difficult to manage because of frequent recurrences and complications. The aim of this study is to evaluate the relationship between anorectal lesions and CD and to analyze the methods of management and the results of anorectal lesions. METHODS: The records of 33 patients with CD who had anorectal lesions, who visited our institution from July 2001 to June 2007, were reviewed retrospectively. RESULTS: CDs involving the small and the large bowel in 24 patients, the small bowel in 4 patients, the large bowel in 4 patients, and only the anorectum in 1 patient. Twenty-two patients (75.9%) were diagnosed as CD with unusual anorectal findings: unhealed wound or delayed healing of wound after the initial anal operation, multiple ulcers or fissures, broad based or friable fistula tract, non-cryptoglandular type of fistula, multiple fistula tracts, and recurrent or concurrent fistula. The predominant type of anorectal lesion was a perianal fistula (28 patients, 84.8%). Twelve out of 45 anal specimens (26.7%) showed noncaseating epithelioid granulomas, characteristic findings of CD. Conservative treatment was performed in 7 patients (21.2%), anorectal operations in 26 patients (78.8%). Twelve of those 26 patients underwent multiple operations. Anorectal operations were performed as follows: incision and drainage (8), fistulotomy or fistulectomy (17), muscle-preserving surgery (7), seton drainage (12), and modified Hanley's procedure (1). All anorectal operations, except those for an abscess, were performed after induction of remission of the CD. Satisfactory results were achieved in 29 patients (87.9%). CONCLUSION: In patients with unusual anorectal lesions, a diagnostic work-up for CD should be performed. Anorectal lesions with CD may be properly managed using several different methods, depending on the anorectal conditions and the activity of the CD.
Abscess
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Crohn Disease
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Drainage
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Fistula
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Granuloma
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Humans
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Recurrence
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Remission Induction
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Retrospective Studies
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Ulcer
7.A Comprehensive Review of Inflammatory Bowel Disease Focusing on Surgical Management.
Journal of the Korean Society of Coloproctology 2012;28(3):121-131
The two main diseases of inflammatory bowel disease are Crohn's disease and ulcerative colitis. The pathogenesis of inflammatory disease is that abnormal intestinal inflammations occur in genetically susceptible individuals according to various environmental factors. The consequent process results in inflammatory bowel disease. Medical treatment consists of the induction of remission in the acute phase of the disease and the maintenance of remission. Patients with Crohn's disease finally need surgical treatment in 70% of the cases. The main surgical options for Crohn's disease are divided into two surgical procedures. The first is strictureplasty, which can prevent short bowel syndrome. The second is resection of the involved intestinal segment. Simultaneous medico-surgical treatment can be a good treatment strategy. Ulcerative colitis is a diffuse nonspecific inflammatory disease that involves the colon and the rectum. Patients with ulcerative colitis need surgical treatment in 30% of the cases despite proper medical treatment. The reasons for surgical treatment are various, from life-threatening complications to growth retardation. The total proctocolectomy (TPC) with an ileal pouch anal anastomosis (IPAA) is the most common procedure for the surgical treatment of ulcerative colitis. Medical treatment for ulcerative colitis after a TPC with an IPAA is usually not necessary.
Colitis, Ulcerative
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Colon
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Crohn Disease
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Humans
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Inflammation
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Inflammatory Bowel Diseases
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Rectum
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Remission Induction
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Short Bowel Syndrome
8.Clinical Analysis of Pediatric Acute Myeloid Leukemia with CCLG-AML 2015 Regimen.
Ping WANG ; Hao XIONG ; Jian-Xin LI ; Zhuo WANG ; Li YANG ; Fang TAO ; Zhi CHEN ; Yu DU ; Ai-Ping ZHANG ; Lin-Lin LUO
Journal of Experimental Hematology 2022;30(2):373-380
OBJECTIVE:
To analyze the clinical effects of CCLG-AML-2015 protocol on newly diagnosed children with acute myeloid leukemia (AML).
METHODS:
The clinical data of 60 newly diagnosed AML children in the Department of Hematology and Oncology, Wuhan Children's Hospital from August 2015 to September 2019 were summarized, the effect of chemotherapy using the CCLG-AML-2015 regimen (hereinafter referred to as the 2015 regimen) were retrospectively analyzed. 42 children with AML treated by the AML-2006 regimen (hereinafter referred to as the 2006 regimen) from February 2010 to July 2015 were used as control group.
RESULTS:
There were no statistical differences between the 2015 regimen group and the 2006 regimen group in sex, age at first diagnosis, and risk stratification (P>0.05). The complete remission rate of bone marrow cytology after induction of 1 course of chemotherapy (84.7% vs 73.1%, P=0.155), and minimal residual disease detection (MRD) negative (42.3% vs 41.4%, P=0.928) in the 2015 regimen group were not statistically different than those in the 2006 regimen group. The bone marrow cytology CR (98.1% vs 80.6%, P=0.004) and MRD negative (83.3% vs 52.8%, P=0.002) in the 2015 regimen group after 2 courses of induction were higher than those in the 2006 regimen group. The 5-year overall survival (OS) rate in the 2015 regimen group (62.3%±6.4% vs 20.6%±6.4%, P=0.001), the 5-year disease-free survival (EFS) rate (61.0%±6.4% vs 21.0% ±6.4% , P=0.001) were better than those in the 2006 regimen group. The 5-year OS and EFS of high-risk transplant patients in the 2015 regimen group were significantly better than those of high-risk non-transplant patients (OS: 86.6%±9.0% vs 26.7%±11.4%, P=0.000; EFS: 86.6%±9% vs 26.7%±11.4%, P=0.000).
CONCLUSION
The 2015 regimen can increase the CR rate after 2 courses of induction compared with the 2006 regimen. High-risk children receiving hematopoietic stem cell transplantation can significantly improve the prognosis.
Child
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Disease-Free Survival
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Humans
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Leukemia, Myeloid, Acute/drug therapy*
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Prognosis
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Remission Induction
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Retrospective Studies
9.Study of clinical outcome and prognosis in pediatric core binding factor-acute myeloid leukemia.
Jun WU ; Ai Dong LU ; Le Ping ZHANG ; Ying Xi ZUO ; Yue Ping JIA
Chinese Journal of Hematology 2019;40(1):52-57
Objective: To analyze the clinical outcome and the prognostic factor in pediatric patients with core binding factor-acute myeloid leukemia (CBF-AML). Methods: A total of 121 newly diagnosed pediatric CBF-AML patients enrolled from Aug. 2005 to Sep. 2017 were retrospectively reviewed. Cumulative incidence of relapse (CIR), event-free survival (EFS) and overall survival (OS) rates were estimated by Kaplan-Meier method and prognostic factors were evaluated by Cox regression with SPSS. Results: Of the 121 patients, 120 patients were assessed for bone marrow remission after induction chemotherapy. 100 cases (83.3%) achieved complete remission (CR) after the first course of chemotherapy. 119 cases (99.2%) achieved CR after the second course of chemotherapy. Of the 121 patients, 13 patients (10.7%) had recurrence with the median interval of recurrence as 13.8 months (3.7 to 58.8 months). 17 patients (14.0%) died. The CIR, EFS and OS at 3 years were 12.7%, 77.5% and 82.8%, respectively. The factors including age at diagnosis, sex, initial WBC count, presence of extramedullary leukemia, C-KIT expression, additional chromosomal abnormalities, and CR after the first course of chemotherapy were analyzed by multivariate regression analysis of Cox. Multivariate analysis identified that additional chromosomal abnormalities was the only independent risk factor affecting OS (HR=4.289, 95%CI 1.070-17.183, P=0.040). Conclusions: Pediatric CBF-AML was a unique setting of prognostic subtypes. Chemotherapy produced good responses. Additional chromosomal abnormalities was the only independent risk factor for OS in pediatric CBF-AML.
Child
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Core Binding Factors
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Disease-Free Survival
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Humans
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Leukemia, Myeloid, Acute
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Prognosis
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Remission Induction
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Retrospective Studies