1.Comparison of Sleep Indices between Both Wrist Actigraphies and Nocturnal Polysomnography.
Byung Hak SHIN ; Doo Heum PARK ; Hyun Kwon LEE ; Jaehak YU ; Seung Ho RYU ; Ji Hyeon HA ; Hyeon Sil SHIN ; Seok Chan HONG
Sleep Medicine and Psychophysiology 2007;14(1):20-25
The present study compared the actigraphic indices between both wrist actigraphies (WATGs), and the sleep estimates between each WATG and nocturnal polysomnography (NPSG) to assess their differences and consistencies. We studied 22 right-handed subjects (mean age 43.9+/-13.3 years, M:F=14:8) with untreated primary sleep disorders (primary insomnia=8, simple snorer=2, obstructive sleep apnea=12) undergone by overnight both WATGs and NPSG, simultaneously. Comparison and correlation were analyzed between right and left wrist actigraphic data. In the sleep estimates of both WATGs and NPSG, each WATG was compared and correlated with NPSG in sleep period time (SPT), total sleep time (TST), sleep latency (SL), sleep efficiency (SE) and wake time (WT). Sleep indices between both WATGs showed significant positive correlations with no correlations in SL and fragmentation index (FI). There were no differences in sleep indices between both WATGs. SPTs of both WATGs, SL of left WATG, and TST of right WATG showed positively significant correlations, and SE of right WATG did negatively significant correlation in sleep indices between each WATG and NPSG. As each WATG was compared to PSG, SPTs of both WATGs and WT of right WATG were decreased, and TST and SE of right WATG and SL of left WATG were increased. Inconsistent SL and FI between both WATGs indicate that the activities between both WATGs can differentially happen during wake or arousal. Inconsistent sleep estimates between each WATG and NPSG may indicate the limited usefulness in measuring and analyzing one-night sleep by using WATG.
Arousal
;
Functional Laterality
;
Polysomnography*
;
Sleep Wake Disorders
;
Wrist*
2.The Effect of Insomnia Severity on Daytime Sleepiness in Obstructive Sleep Apnea Syndrome.
Ji Won NAM ; Byoung Hak SHIN ; Hyeon Sil SHIN ; Minji PARK
Sleep Medicine and Psychophysiology 2015;22(1):11-19
OBJECTIVES: Whether daytime sleepiness is proportional to the severity of sleep apnea in obstructive sleep apnea syndrome (OSAS) is controversial. In this study we investigated how insomnia severity affects the association between daytime sleepiness and sleep apnea severity in OSAS. METHODS: The present study included 235 male subjects who were diagnosed with OSAS based on clinical history and nocturnal polysomnography. Pearson's correlation analysis was conducted among sleep and mood-related self-reported data, polysomnographic data and demographic data of all subjects. Based on Pittsburgh Sleep Quality Index (PSQI), the subjects were divided into 2 groups; group A (n = 75; PSQI < or =5) and group B (n = 160; PSQI > 5). Partial correlation analysis was performed between the Epworth Sleepiness Scale (ESS) and other data in both groups. Multiple linear regression analysis was conducted to investigate the factors which affected the ESS in group A. RESULTS: Pearson's correlation analysis showed weak or non-existent correlations between ESS and apnea severity data such as apnea-hypopnea index (AHI) (r = 0.148, p = 0.023), apnea index (AI) (r = 0.137, p = 0.036), hypopnea index (HI) (r = 0.058, p = 0.377), oxygen desaturation index (ODI) (r = 0.149, p = 0.022) and arousal total index (ATI) (r = 0.129, p = 0.048). Positive correlations between ESS and apnea severity data such as AHI (r(p) = 0.313, p = 0.008), AI (r(p) = 0.339, p = 0.004), ODI (r(p) = 0.289, p = 0.015) and ATI (r(p) = 0.256, p = 0.031) were observed only in group A. Multiple regression analysis showed that AI (t = 2.996, p = 0.004) and BAI (t = 2.721, p = 0.008) were associated with ESS in group A. CONCLUSION: The correlation between daytime sleepiness and sleep apnea severity was shown only in group A. This result suggests that associations between daytime sleepiness in OSAS and sleep apnea severity will become prominent when controlling for insomnia-related variables.
Apnea
;
Arousal
;
Humans
;
Linear Models
;
Male
;
Oxygen
;
Polysomnography
;
Sleep Apnea Syndromes
;
Sleep Apnea, Obstructive*
;
Sleep Initiation and Maintenance Disorders*
3.The Construction and the Evaluation of Web Sites on Epilepsy Information.
Eun Sil CHOI ; Yeong Bae LEE ; Hyeon Mi PARK ; Dong Jin SHIN ; Seong Woo CHOI ; Sung Tae YOUN
Journal of Korean Society of Medical Informatics 2003;9(2):131-141
We aimed to construct web sites providing information on epilepsy and to evaluate its practical use. To estimate the demand for such information, a questionnaire was administrated to 106 patients, their 54 families, and 78 nurses, who consented to be interviewed. After using the data collected from the survey to build a epilepsy information web site, data on the level of satisfaction for the website were collected by e-mails and direct surveys from subjects who used this web site; the collected data were analyzed. The results of this study were as follows: The educational level demanded by nurses was slightly higher than those of patients and their families. Patients, their families, and nurses wanted more information on how to handle patients during seizures, especially, and. families wanted to know about drug therapy. The content of the web site was configured based on the educational level of perceived users. Photoshop 6.0, Flash MX and FrontPage 2000 were used to build the web site. Images available on the internet that are shared served as the source of images. The program's URL is www.multimoon.com/black. Regarding user satisfaction with the web site, users found that the Information on the Prognosis of Epilepsy, the Pharmacological Treatment of Epilepsy, Occupation and Marriage Status in Daily Life, and the How to Manage Epileptic Attacks sections were quite helpful. Generally, the reactions to the website were positive. To construct an effective web site to provide information about epilepsy to patients, their families and nurses, educational data and information that meet the demands of users should be added. Its contents should be continuously updated, and its environment should be kept user-friendly so that the information can be accessed with ease.
Drug Therapy
;
Electronic Mail
;
Epilepsy*
;
Humans
;
Internet
;
Marriage
;
Occupations
;
Prognosis
;
Surveys and Questionnaires
;
Seizures
4.How COVID-19 Affected Healthcare Workers in the Hospital Locked Down due to Early COVID-19 Cases in Korea
Hyeon-Ah LEE ; Myung Hee AHN ; Seonjeong BYUN ; Hae-Kook LEE ; Yong-Sil KWEON ; Seockhoon CHUNG ; Yong-Wook SHIN ; Kyoung-Uk LEE
Journal of Korean Medical Science 2021;36(47):e325-
Background:
The healthcare workers (HCWs) were exposed to never-experienced psychological distress during the early stage of the coronavirus disease 2019 (COVID-19) pandemic. The aim of this study was to investigate how the COVID-19 pandemic affected the mental health of HCWs during the hospital lockdown period due to mass healthcare-associated infection during the early spread of COVID-19.
Methods:
A real-time online survey was conducted between April 14–18, 2020 among HCWs who worked at the university hospital where COVID-19 was confirmed in a patient, and the hospital was shut down for 3 weeks. Along with demographic variables and work-related information, psychological distress was measured using the Generalized Anxiety Disorder-7 (GAD-7), the Patient Health Questionnaire-9 (PHQ-9), the Maslach Burnout Inventory-General Survey scale, and the Stress and Anxiety to Viral Epidemics-9.
Results:
The HCWs working in the cohort ward and those who have experienced social discrimination had significantly higher level of depression (PHQ-9 score; 5.24 ± 4.48 vs. 4.15 ± 4.38; P < 0.01 and 5.89 ± 4.78 vs. 3.25 ± 3.77; P < 0.001, respectively) and anxiety (GAD-7 score; 3.69 ± 3.68 vs. 2.87 ± 3.73;P < 0.05 and 4.20 ± 4.22 vs. 2.17 ± 3.06; P < 0.001, respectively) compared to other HCWs. Worries regarding the peer relationship and the skepticism about job were associated with depression (odds ratio [OR], 1.39; 95% confidence interval [CI], 1.07–1.79; P < 0.05 and OR, 1.69; 95% CI, 1.31–2.17; P < 0.001, respectively) and anxiety (OR, 1.73; 95% CI, 1.21–2.49; P < 0.01 and OR, 1.54; 95% CI, 1.09–2.17; P < 0.05, respectively), while fear of infection or worsening of health was not. Path analysis showed that work-related stress associated with the viral epidemic rather than anxiety about the viral epidemic mainly contributed to depression.
Conclusion
The present observational study indicates that mental health problems of HCWs exposed to COVID-19 are associated with distress in work and social relationship. Early intervention programs focusing on these factors are necessary.
5.A Case of Metastatic Epididymal and the Other Metastatic Testicular Carcinoma from Sigmoid Carcinoma.
Yon Sil JUNG ; Dong Kyeong YANG ; Pill Woon KIM ; Chan Il MOON ; Seung Won LEE ; Hyeon Gyoo JI ; Ji Ho CHOI ; Jae Hun LEE ; Dong Bok SHIN ; Seung Phil CHO ; Jeong Cheol YOON
Journal of the Korean Cancer Association 1997;29(4):706-706
Metastatic tumor of the epididymis is a rare tumor. There are around 31 cases in the literature until now. The primary tumor was from the prostate in 18 cases, large and small intestine in 6 cases, kidney in 4 cases, stomach in 2 cases and pancreas in 1 case. We recently experienced a case of metastatic carcinoma to the epididymis from a primary cancer in the sigmoid colon and the other case of metastatic carcinoma to the testis and the liver from a sigmoid adenocarcinoma.
Adenocarcinoma
;
Colon, Sigmoid*
;
Colonic Neoplasms
;
Epididymis
;
Intestine, Small
;
Kidney
;
Liver
;
Male
;
Neoplasm Metastasis
;
Pancreas
;
Prostate
;
Stomach
;
Testis
6.Four Cases of Typhlitis, Developed in Neutropenic State and Treated with Medical Conservative Management.
Pill Woon KIM ; Hyeon Gyoo JI ; Hyun Sik JEONG ; Chan Il MOON ; Dong Kyeong YANG ; Seung Won LEE ; Yon Sil JUNG ; Ji Ho CHOI ; Gui Hyun NAM ; Jae Hoon LEE ; Dong Bok SHIN
Journal of the Korean Cancer Association 1997;29(5):906-913
Typhlitis is a life threatening necrotizing enterocolitis of the cecum, ascending colon and terminal ileum seen in severely neutropenic patients, however its pathogenesis is not identified up to this time.The incidence of typhlitis in leukemic patient is 10~12%, estimated by postmortem examination, and 46% in induction chemotherapy of leukemia. Recently, entity incidence is more high due to increasing challenges to high dose chemotherapy in solid tumors.We experienced four cases of typhlitis, one was developed in the circumstance of neutropenia induced by induction chemotherapy for acute myelocytic leukemia and others in neutropnia due to primary diseases without chemotherapy, ig, chronic myelocytic leukemia, acute lymphocytic leukemia, myelodysplastic syndrome.All cases were treated with high dose broad spectrum antibiotics in early phase of disease and its outcome was good, so that, early diagnosis of typhlitis is essential, then prompt treatment with high dose antibiotics and intravenous fluid before onset of transmural necrosis is associated with lower morbidity and mortality than surgical resection.
Anti-Bacterial Agents
;
Autopsy
;
Cecum
;
Colon, Ascending
;
Drug Therapy
;
Early Diagnosis
;
Enterocolitis, Necrotizing
;
Humans
;
Ileum
;
Incidence
;
Induction Chemotherapy
;
Leukemia
;
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
;
Leukemia, Myeloid, Acute
;
Mortality
;
Necrosis
;
Neutropenia
;
Precursor Cell Lymphoblastic Leukemia-Lymphoma
;
Typhlitis*
7.Current Status of Hematopoietic Stem Cell Transplantation in Korean Children.
Dae Chul JEONG ; Hyung Jin KANG ; Hong Hoe KOO ; Hoon KOOK ; Sun Young KIM ; Soon Ki KIM ; Thad GHIM ; Hack Ki KIM ; Hwang Min KIM ; Hyung Nam MOON ; Kyung Duk PARK ; Byung Kiu PARK ; Sang Gyu PARK ; Young Sil PARK ; Hyeon Jin PARK ; Jong Jin SEO ; Ki Woong SUNG ; Hee Young SHIN ; Hyo Sup AHN ; Kun Hee RYU ; Kyung Ha RYU ; Eun Sun YOO ; Chuhl Joo LYU ; Kwang Chul LEE ; Soon Yong LEE ; Young Ho LEE ; Young Tak LIM ; Jae Young LIM ; Pil Sang JANG ; In Sang JEON ; Nak Gyun CHUNG ; Bin CHO ; Jeong Ok HAH ; Pyung Han HWANG ; Tai Ju HWANG
Korean Journal of Hematology 2006;41(4):235-242
BACKGROUND: Hematopoietic stem cell transplantation (HSCT) is one of the most important armamentarium against various hematologic malignancies or some solid tumors. We investigated the number of patients who might need transplants and compared with that of actual transplants to conceptualize current status and circumstances of HSCTs in Korean children. METHODS: Questionnaires were sent to Korean Society of Hematopoietic Stem Cell Transplantation (KSHSCT) members who were taking care of children with malignancies or hematologic diseases. Almost all of the newly diagnosed patients between Jan, 1st and Dec, 31st, 2003 were enrolled in the study. RESULTS: Seven hundred forty eight children (male to female ratio = 1.4:1) were enrolled. The median age was 6.1 years old (8 days~28.8 years old). Malignant diseases consisted of 695 cases (92.9%), and among them almost half were hematologic malignancies. The participating members speculated that HSCTs should be indicated in 285 children (38.1%) which included 209 allogeneic, and 76 autologous transplants. In reality, however, allogeneic HSCTs were performed only in 140 children (67.0%) with the median interval of 5.9 month, and autologous transplants in 44 children (57.9%) with 8.3 month. In autologous setting, all the patients received peripheral blood stem cells (PBSCs), whereas bone marrow (61%), cord blood (34%), and PBSC (5%) were used in allogeneic HSCTs. Donor types were as follows: unrelated donor (37%), cord blood (34%), sibling donor (25%), and family (4%). The reasons for not performing HSCTs were unfavorable disease status or death, no availability of suitable donor, economical situation, and refusal by parental preferences. Under the strict insurance regulations, many transplants were not covered by insurance. More autologous transplants were performed without insurance coverage than allogeneic HSCTs (P=0.013). Those cases were advanced cases and HLA mismatch transplants for allogeneic setting, and relatively rare diseases still awaiting favorable results of transplants for autologous setting. CONCLUSION: HSCTs are essential part of treatment strategies for children with various diseases. Unfortunately, however, a third of patients who were in need of transplants did not receive HSCTs due to various reasons. It is necessary to expand unrelated donor pool or cord blood banks for the cases lacking HLA-identical sibling donors. Also medical insurances should cover HSCTs for rare diseases as well as for less favorable but novel situations where there are no suitable alternatives.
Autografts
;
Bone Marrow
;
Child*
;
Disulfiram
;
Female
;
Fetal Blood
;
Hematologic Diseases
;
Hematologic Neoplasms
;
Hematopoietic Stem Cell Transplantation*
;
Hematopoietic Stem Cells*
;
Humans
;
Insurance
;
Insurance Coverage
;
Parents
;
Rare Diseases
;
Siblings
;
Social Control, Formal
;
Stem Cells
;
Tissue Donors
;
Unrelated Donors
;
Surveys and Questionnaires
8.Epidemiological Study of Hereditary Hemolytic Anemia in the Korean Pediatric Population during 1997–2016: a Nationwide Retrospective Cohort Study
Ye Jee SHIM ; Hye Lim JUNG ; Hee Young SHIN ; Hyoung Jin KANG ; Jung Yoon CHOI ; Jeong Ok HAH ; Jae Min LEE ; Young Tak LIM ; Eu Jeen YANG ; Hee Jo BAEK ; Hyoung Soo CHOI ; Keon Hee YOO ; Jun Eun PARK ; Seongkoo KIM ; Ji Yoon KIM ; Eun Sil PARK ; Ho Joon IM ; Hee Won CHUEH ; Soon Ki KIM ; Jae Hee LEE ; Eun Sun YOO ; Hyeon Jin PARK ; Jun Ah LEE ; Meerim PARK ; Hyun Sik KANG ; Ji Kyoung PARK ; Na Hee LEE ; Sang Kyu PARK ; Young-Ho LEE ; Seong Wook LEE ; Eun Jin CHOI ; Seom Gim KONG
Journal of Korean Medical Science 2020;35(33):e279-
Background:
Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA.
Methods:
We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey.
Results:
A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia.
Conclusion
In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.
9.Epidemiology and Clinical Outcomes in Children with Malignant Lymphoma in Korea: Retrospective Study.
Chuhl Joo LYU ; Im Joo KANG ; Hong Hoe KOO ; Hoon KOOK ; Soon Ki KIM ; Thad T GHIM ; Hack Ki KIM ; Hwang Min KIM ; Heung Sik KIM ; Hyung Nam MOON ; Kyung Duk PARK ; Kyung Bae PARK ; Byung Kiu PARK ; Sang Gyu PARK ; Young Sil PARK ; Jae Sun PARK ; Joon Eun PARK ; Hyeon Jin PARK ; Jong Jin SEO ; Ki Woong SUNG ; Hee Young SHIN ; Hyo Seop AHN ; Chang Hyun YANG ; Keon Hee YOO ; Kyung Ha RYU ; Eun Sun YOO ; Kun Soo LEE ; Kwang Chul LEE ; Soon Yong LEE ; Young Ho LEE ; Young Tak LIM ; Pil Sang JANG ; Nak Gyun CHUNG ; Dae Chul JEONG ; Hye Lim JUNG ; Bin CHO ; Yong Mook CHOI ; Jeong Ok HAH ; Jung Woo HAN ; Tai Ju HWANG
Korean Journal of Pediatric Hematology-Oncology 2004;11(2):153-163
PURPOSE: Malignant lymphoma is the primary malignant tumor derived from lymphoid organs. It is composed of Hodgkin's disease and non-Hodgkin lymphoma. Recently, survival rate is on the rise due to improved combination chemotherapy, radiotherapy and high dose chemotherapy followed by hematopoietic stem cell transplantation. In South Korea, no epidemiologic studies concerning malignant lymphoma in the pediatric age group has been performed. Therefore, the Korean Society of Pediatric Hematology-Oncology retrospectively analyzed the incidence, pathologic subtypes, treatment strategies, and survival rates of pediatric malignant lymphomas in South Korea. METHOD: Questionnaires were made and sent to a group of training hospitals, with a return of 580 questionnaires from 24 hospitals. Among them, 517 reports were suitable for analysis. RESULTS: Among the 517 cases, Hodgkin's disease accounted for 58 cases and non-Hodgkin's lymphoma for 459 cases. Male to female ratio for malignant lymphoma was 2.7. Mean age at diagnosis was 8.3 years. Among the pathologic subtypes, mixed cellularity was the most frequent subtype for Hodgkin's disease. Most (70.7%) cases of non-Hodgkins lymphoma belonged to high grade NHL. Burkitt lymphoma accounted for 102 cases, and lymphoblastic lymphoma was found in 58 cases. Peripheral lymphadenopathy was the most common presenting sign upon diagnosis. B symptoms were significantly more frequent in Hodgkin's disease patients than in non-Hodgkin lymphoma patients. The Complete response rate was 62.1% for non-Hodgkin's lymphoma, and 82.8% for Hodgkin's disease. Overall 5 year survival rate was 60.0% in non-hodgkin's lymphoma, and 84.8% in Hodgkin's disease. CONCLUSION: The annual incidence of malignant lymphoma in Korea is 4.7 per million. In cases of chemotherapy-sensitive, refractory or relapsed malinant lymphoma, high dose chemotherapy followed by hematopoietic stem cell transplantation is vital for improved survival. For more systematic analysis of epidemiology on malignant lymphomas, better surveillance mechanisms on the occurrence of malignant lymphomas are crucial, and establishment of standardized treatment protocol for malignant lymphoma is required.
Burkitt Lymphoma
;
Child*
;
Clinical Protocols
;
Diagnosis
;
Drug Therapy
;
Drug Therapy, Combination
;
Epidemiologic Studies
;
Epidemiology*
;
Female
;
Hematopoietic Stem Cell Transplantation
;
Hodgkin Disease
;
Humans
;
Incidence
;
Korea*
;
Lymphatic Diseases
;
Lymphoma*
;
Lymphoma, Non-Hodgkin
;
Male
;
Precursor Cell Lymphoblastic Leukemia-Lymphoma
;
Surveys and Questionnaires
;
Radiotherapy
;
Retrospective Studies*
;
Survival Rate
10.Epidemiology and Clinical Outcomes in Children with Aplastic Anemia in Korea: Retrospective Study.
Dae Chul JEONG ; Im Joo KANG ; Hong Hoe KOO ; Hoon KOOK ; Sun Young KIM ; Soon Ki KIM ; Hwang Min KIM ; Heung Sik KIM ; Kyung Duk PARK ; Kyeong Bae PARK ; Young Sil PARK ; Sang Kyu PARK ; Jae Sun PARK ; Jun Eun PARK ; Hyeon Jin PARK ; Jong Jin SEO ; Won Suk SUH ; Ki Woong SUNG ; Hee Young SHIN ; Hyo Seop AHN ; Chang Hyun YANG ; Keon Hee YOO ; Kyung Ha RYU ; Eun Sun YOO ; Chuhl Joo LYU ; Kun Soo LEE ; Kwang Chul LEE ; Soon Yong LEE ; Young Tak LIM ; Pil Sang JANG ; Nak Gyun CHUNG ; Hye Lim JUNG ; Bin CHO ; Yong Mook CHOI ; Jeong Ok HAH ; Tai Ju HWANG
Korean Journal of Pediatric Hematology-Oncology 2004;11(2):137-152
PURPOSE: The annual incidence of aplastic anemia (AA) in Asian countries is higher than in Western countries. The pathogenesis in AA has been investigated in hematopoiesis and immunology. Recently, the survival rate and the quality of life of the patients with AA have been steadily improved by the development of a variety of treatments such as the immunosuppressive therapy (IST), and hematopoietic stem cell transplantation (HSCT). The Korean Society of Pediatric Hematology-oncology retrospectively investigated the incidence, treatment strategies, survival rate, and time to become independent from transfusion in patients with AA, who were diagnosed from January 1st, 1991 to December 31st, 2000 in Korea. METHODS: All the questionnaires were sent to a group of training hospitals, and we collected about 600 questionnaire forms from 27 hospitals. However, 493 reports were available for data analysis. RESULTS: The male and female ratio in AA is 1.1 (259 males vs. 234 female). The median age at diagnosis is 9 years old (range; 0.8~16 years old). The annual incidence of children with AA in Korea is 4.5 per million on the basis of Korean pediatric population. In etiology, there are 20 cases (4.1%) in congenital and others in acquired AA. In acquired AA, the cause of illness was not identifiable for most of the patients, but 1 patient had hepatitis-associated AA, and 3 patients developed the illness after medication. According to the initial laboratory data at diagnosis, the peripheral blood findings showed that hemoglobin is 7.1+/-2.4 g/dL, white blood cell 3, 200/microL (200~16, 550), absolute neutrophil counts 670/microL (0~12, 487), platelets 19, 000/microL (1, 000~500, 000), and corrected reticulocytes 0.18% (0.0~4.7). The bone marrow examination revealed that cellularity was below 25% in 348 patients, and over 25% in 105 patients. In the available data, 269 patients (54.6%) were diagnosed of severe aplastic anemia (SAA) and 224 patients of non-SAA (NSAA). HSCT were done for 96 patients (19.5%) and others received another treatments such as the IST. The anti-thymocyte globulin (ATG) or anti-lymphocyte globulin (ALG) treatment was done for 263 cases, corticosteroids for 259, cyclosporine A (CSA) for 215, and anabolic steroids for 138. The combination IST including ATG or ALG plus corticosteroid plus CSA were applied to 154 children with AA, and transfusion only in 37, as conservative care. In case of those patients with HSCT, the time from diagnosis to transplantation was 12 months (1~144 months) and the sources of stem cells were bone marrow in 82 cases, growth factor mobilized peripheral blood in five, and cord blood in six. There were 57 patients transfused below 40 units of blood products before HSC transplantation. Graft rejection was identified from 16 patients, and booster transplantations were done for 12 patients among them. In complications of HSCT, the graft versus host disease was developed in 20 patients and viral diseases in 12 cases including the CMV, herpetic infection, and hepatitis. Also, one patient suffered from veno-occlusive disease. The overall survival rate in children with AA is 64.3%. The survival rate in HSCT is better than that IST (76.9% vs. 62.6%, P< 0.05). In IST, overall survival rate in very SAA showed lower than SAA and NSAA, and in SAA lower than NSAA in case of absolute neutrophil count below 200/microL (P< 0.05). There was no significant difference in terms of the sex, age at beginning of treatment. In HSCT, transfusion was not related to the survival rate. However, overall survival rate is better in short interval between diagnosis and HSCT than in long interval (P< 0.05). There was no significant difference in the probability of transfusion independence according to treatment strategies, even though it was 71.0% in HSCT and 12.8% in immunosuppressive therapy at the end point of survey (P=0.47). The response pattern was as follows. There were 155 cases of complete response, 110 of partial response and 120 of no response in spite of various treatments. The relapse after treatment was found in 11 patients after IST, of which 6 patients experienced more than 2nd relapse. The median time between the end of treatment and relapse was 16 months (6~84 months). Only three cases developed into other diseases (1 case into acute myeloid leukemia and 2 cases into myelodysplastic syndrome). The median time from diagnosis to the end of treatment was 62 months (0.5~174 months). In fatal cases, the median time between diagnosis and death was 29 months (0~144 months) despite several therapeutic strategies. CONCLUSION: In Korea, the annual incidence of children with AA is 4.5 per million. This result is similar to the ones reported in other Asian countries, but higher than those in Western countries. Although a lot of children with AA received various therapies including IST or HSCT, new treatment strategies have to be developed to improve the survival rate and the quality of life of children with AA
Adrenal Cortex Hormones
;
Allergy and Immunology
;
Anemia, Aplastic*
;
Antilymphocyte Serum
;
Asian Continental Ancestry Group
;
Bone Marrow
;
Bone Marrow Examination
;
Child*
;
Cyclosporine
;
Diagnosis
;
Epidemiology*
;
Female
;
Fetal Blood
;
Graft Rejection
;
Graft vs Host Disease
;
Hematopoiesis
;
Hematopoietic Stem Cell Transplantation
;
Hepatitis
;
Humans
;
Incidence
;
Korea*
;
Leukemia, Myeloid, Acute
;
Leukocytes
;
Male
;
Neutrophils
;
Quality of Life
;
Surveys and Questionnaires
;
Recurrence
;
Reticulocytes
;
Retrospective Studies*
;
Statistics as Topic
;
Stem Cells
;
Steroids
;
Survival Rate
;
Virus Diseases