1.Correlation between Morphologic Grading and Euploidy Rates of Blastocysts, and Clinical Outcomes in In Vitro Fertilization Preimplantation Genetic Screening.
Min Kyoung KIM ; Jae Kyun PARK ; Yunmi JEON ; Seung Ah CHOE ; Hee Jun LEE ; Jayeon KIM ; Eun Mi CHANG ; Ji Won KIM ; Sang Woo LYU ; Jin Young KIM ; In Pyung KWAK ; Woo Sik LEE ; Tae Ki YOON
Journal of Korean Medical Science 2019;34(4):e27-
BACKGROUND: The standard morphological evaluation has been widely used for embryo selection, but it has limitations. This study aimed to investigate the correlation between morphologic grading and euploidy rate of in vitro fertilization (IVF) preimplantation genetic screening (PGS) and compare the pregnancy rates in young and old ages. METHODS: This is a retrospective study using the medical records of patients who underwent IVF procedures with PGS between January 2016 and February 2017 in a single center. The embryo grades were categorized into 4 groups: excellent, good, fair, and poor. Basic characteristics, euploidy rates, clinical pregnancy (CP) rates and ongoing pregnancy rates were analyzed. RESULTS: The excellent group had significantly higher rate of euploid embryos than fair group (47.82% vs. 29.33%; P = 0.023) and poor group (47.82% vs. 29.60%; P = 0.005). When the four groups were recategorized into two groups (excellent and good vs. fair and poor), they also showed significant difference in euploidy rates (44.52% vs. 29.53%; P = 0.002). When the patients were divided into two groups by age 35, the CP rates for those under and over 35 years old were 44.74% and 47.83%, respectively, which showed no significant difference. CONCLUSION: The significant differences among the euploidy rates of different morphologic embryo grades demonstrated the positive correlations between the morphologic grading of the embryo and the euploidy rate of PGS. Additionally, there was no significant difference between the younger and older patients' CP rates. These findings emphasize the fact that old age patients might benefit from PGS whatever the indication of PGS is.
Blastocyst*
;
Embryonic Structures
;
Fertilization in Vitro*
;
Genetic Testing*
;
Humans
;
In Vitro Techniques*
;
Medical Records
;
Pregnancy
;
Pregnancy Rate
;
Retrospective Studies
2.Effects of Ranibizumab, Bevacizumab, and Aflibercept on Senescent Retinal Pigment Epithelial Cells.
Jae Byoung CHAE ; Chang Rae RHO ; Jeong Ah SHIN ; Jungmook LYU ; Seungbum KANG
Korean Journal of Ophthalmology 2018;32(4):328-338
PURPOSE: Anti-vascular endothelial growth factor (VEGF) agents have been used for the last 10 years, but their safety profile, including cytotoxicity against various ocular cells such as retinal pigment epithelial (RPE) cells, remains a serious concern. Safety studies of VEGF agents conducted to date have primarily relied on healthy RPE cells. In this study, we assessed the safety of three anti-VEGF agents, namely, ranibizumab, bevacizumab, and aflibercept, on senescent RPE cells. METHODS: Senescent human induced pluripotent stem cell-derived RPE cells were generated by continuous replication and confirmed with senescence biomarkers. The viability, proliferation, protein expression, and phagocytosis of the senescent RPE cells were characterized 3 days after anti-VEGF treatment with clinical doses of ranibizumab, bevacizumab, or aflibercept. RESULTS: Clinical doses of ranibizumab, bevacizumab, or aflibercept did not decrease the viability or alter proliferation of senescent RPE cells. In addition, the anti-VEGF agents did not induce additional senescence, impair the protein expression of zonula occludens-1 and RPE65, or reduce the phagocytosis capacity of senescent RPE cells. CONCLUSIONS: Clinical dosages of ranibizumab, bevacizumab, or aflibercept do not induce significant cytotoxicity in senescent RPE cells.
Aging
;
Bevacizumab*
;
Biomarkers
;
Endothelial Growth Factors
;
Epithelial Cells*
;
Humans
;
Phagocytosis
;
Ranibizumab*
;
Retinaldehyde*
;
Vascular Endothelial Growth Factor A
3.A De-identification Method for Bilingual Clinical Texts of Various Note Types.
Soo Yong SHIN ; Yu Rang PARK ; Yongdon SHIN ; Hyo Joung CHOI ; Jihyun PARK ; Yongman LYU ; Moo Song LEE ; Chang Min CHOI ; Woo Sung KIM ; Jae Ho LEE
Journal of Korean Medical Science 2015;30(1):7-15
De-identification of personal health information is essential in order not to require written patient informed consent. Previous de-identification methods were proposed using natural language processing technology in order to remove the identifiers in clinical narrative text, although these methods only focused on narrative text written in English. In this study, we propose a regular expression-based de-identification method used to address bilingual clinical records written in Korean and English. To develop and validate regular expression rules, we obtained training and validation datasets composed of 6,039 clinical notes of 20 types and 5,000 notes of 33 types, respectively. Fifteen regular expression rules were constructed using the development dataset and those rules achieved 99.87% precision and 96.25% recall for the validation dataset. Our de-identification method successfully removed the identifiers in diverse types of bilingual clinical narrative texts. This method will thus assist physicians to more easily perform retrospective research.
Algorithms
;
*Data Anonymization
;
*Electronic Health Records
;
*Health Records, Personal
;
Humans
;
Multilingualism
;
Natural Language Processing
;
Research Design
4.Weaning food practice in children with iron deficiency anemia.
Joo Hee CHANG ; Woo Sik CHEONG ; Yong Hoon JUN ; Soon Ki KIM ; Hung Sik KIM ; Sang Kyu PARK ; Kyung Ha RYU ; Eun Sun YOO ; Chuhl Joo LYU ; Kun soo LEE ; Kwang Chul LEE ; Jae Young LIM ; Du Young CHOI ; Byung Kyu CHOE ; Eun Jin CHOI ; Bong Soon CHOI
Korean Journal of Pediatrics 2009;52(2):159-166
PURPOSE: Iron deficiency anemia (IDA) is one of the most common nutritional deficiencies in children on a weaning diet. We investigated weaning practices in infants and children, as well as their mothers' knowledge about weaning. METHODS: We investigated 129 children with IDA and 166 without IDA (aged 6-36 months) who had visited 10 university hospitals between March 2006 and July 2007. We investigated the hematologic values of both groups. A questionnaire on weaning was answered by the mothers of these children. RESULTS: The hematologic values in the IDA group showed a significant difference from those in the comparison group (P<0.05). Children who were solely breastfed until 6 months of age were 85%, 34% (P<0.05), and weaning was started by 6.3, 6.4 months, respectively (P>0.05). Rice gruel, boiled rice, and fruit juice accounted for approximately 8 0% of the starting foods in both groups (P>0.05). Only 40% of the children in the IDA group had a balanced diet within a month, versus 38% in the comparison group. In response to questions about the necessity of iron-fortified foods for breast-fed infants, less than 50% of mothers in both groups answered correctly. In the IDA group, 42% showed serum ferritin less than 10 ng/ mL, while 92% showed serum MCV less than 72 fL. CONCLUSION: In conclusion, collection of information on history should be thorough for feeding and selective examinations for IDA in high-risk groups. Considering the adaptation period, we suggest beginning children on a weaning diet at 45 months. In addition, we need to educate mothers on weaning practice, especially on the necessity of iron-fortified foods for breast-fed infants.
Anemia, Iron-Deficiency
;
Child
;
Diet
;
Ferritins
;
Fruit
;
Hospitals, University
;
Humans
;
Infant
;
Iron
;
Malnutrition
;
Mothers
;
Surveys and Questionnaires
;
Weaning
5.Prognostic factors in recurrent cervical cancer patients with pulmonary metastasis.
Mi Ok LYU ; Ho Yeon KIM ; Jong Hyuck YOON ; Suk Joon CHANG ; Jung Pil LEE ; Ki Hong CHANG ; Hee Sug RYU ; Jae Hoon KIM
Korean Journal of Obstetrics and Gynecology 2007;50(6):887-892
OBJECTIVE: To characterize prognostic factors in patients with pulmonary metastasis in recurrent cervical cancer. METHODS: The records of 2,042 patients treated for cervical cancer from 1994 to 2004 at two institutions were retrospectively reviewed. Twenty-five (1.04%) patients had pulmonary lesions consistent with metastatic cervical cancer. The data were analyzed retrospectively and reviewed for patient characteristics. RESULTS: Patients were treated with chemotherapy, radiotherapy only, chemoradiotherapy, surgery, and conservative treatment. There were no significant differences in survival rates between each treatment method (p=0.3410). Mean survival after treatment of pulmonary metestasis was 23 months. Histologic type, lymph node metastasis, other organ metastasis, and pretreatment SCC level were not significant prognostic factors, but FIGO stage (p=0.039) and disease-free interval (p=0.033) were significant factors. CONCLUSION: This study revealed that FIGO stage and disease-free interval were independent prognostic factors of pulmonary metestasis in recurrent cervical cancer.
Chemoradiotherapy
;
Drug Therapy
;
Humans
;
Lymph Nodes
;
Neoplasm Metastasis*
;
Prognosis
;
Radiotherapy
;
Retrospective Studies
;
Survival Rate
;
Uterine Cervical Neoplasms*
6.Hereditary Hemolytic Anemia in Korea: a Retrospective Study from 1997 to 2006.
Hee Soon CHO ; Jeong Ok HAH ; Im Ju KANG ; Hyung Jin KANG ; Jae Yong KWAK ; Hong Hoe KOO ; Hoon KOOK ; Byoung Kook KIM ; Soon Ki KIM ; Seung Taik KIM ; Young Dae KIM ; Ji Yoon KIM ; Chul Soo KIM ; Thad GHIM ; Heung Sik KIM ; Sang Gyu PARK ; Seon Yang PARK ; Jun Eun PARK ; Soo Mee BANG ; Jong Jin SEO ; Chang In SUH ; Sang Kyun SOHN ; Ho Jin SHIN ; Hee Young SHIN ; Hyo Sup AHN ; Doyeun OH ; Eun Sun YOO ; Chuhl Joo LYU ; Sung Soo YOON ; Kun Soo LEE ; Kwang Chul LEE ; Kee Hyun LEE ; Soon Yong LEE ; Young Ho LEE ; Jung Ae LEE ; Jong Seok LEE ; Young Tak LIM ; Jae Young LIM ; Ho Joon IM ; Dae Chul JEONG ; So Young CHONG ; Joo Seop CHUNG ; Hye Lim JUNG ; Goon Jae CHO ; Deog Yeon JO ; Jong Youl JIN ; Eun Jin CHOI ; Myung Soo HYUN ; Pyung Han HWANG
Korean Journal of Hematology 2007;42(3):197-205
BACKGROUND: The aim of this study was to investigate the prevalence, clinical and laboratory findings of hereditary hemolytic anemia (HHA) in Korea from 1997 to 2006 and to develop the appropriate diagnostic approach for HHA. METHODS: By the use of questionnaires, information on the clinical and laboratory findings ofHHA diagnosed from 1997 to 2006 in Korea was collected and analyzed retrospectively. A total of 431 cases were enrolled in this study from 46 departments of 35 hospitals. RESULTS: The overall frequency of HHA did not change through the 10-year period for pediatrics but did show an increasing tendency for internal medicine. The overall male to female sex ratio did not show sex predominance (1.17:1), but a significant male predominance with a ratio of 1.49:1 was seen for pediatrics while a significant female predominance with a ratio of 1:1.97 was seen forinternal medicine. Of the total cases, 74.2% (282/431) were diagnosed before the age of 15 years. The etiologies of HHA were classified as red cell membrane defects, hemoglobinopathies, red cell enzyme deficiencies and unknown causes. There were 382 cases (88.6%) of red cell membrane defects with 376 cases (87.2%) of hereditary spherocytosis and 6 cases (1.4%) of hereditary elliptocytosis, 20 cases (4.6%) of hemoglobinopathies with 18 cases (4.2%) of beta-thalassemia, a case (0.2%) of alpha-thalassemia and a case (0.2%) of Hemoglobin Madrid, 7 cases (1.6%) of red cell enzyme deficiencies with 5 cases (1.2%) of glucose-6- phosphate dehydrogenase (G-6-PD) deficiency, a case (0.2%) of pyruvate kinase (PK) deficiency and a case (0.2%) of enolase deficiency, and 22 cases (5.1%) of unknown causes. The most common chief complaint in pediatric patients was pallor and that in adult patients was jaundice. In the red cell membrane defect group of patients, the level of hemoglobin was significantly higher than in adult patients. The mean corpuscular volume, mean corpuscular hemoglobin, corrected reticulocyte count, total and indirect bilirubin level and lactate dehydrogenase levels in the hemoglobinopathy group of patients were significantly lower than the values in the red cell membrane defect group of patients. The mean concentration of G-6-PD was 0.8+/-0.7U/1012RBC in the G-6-PD deficient patients, PK was 1.7U/1010 RBC in the PK deficient patient, and the level of enolase was 0.04U/g of Hb in the enolase deficient patient. CONCLUSION: The most prevalent cause of HHA in Korea during 1997 to 2006 was hereditary spherocytosis, but HHA by other causes such as hemoglobinopathy and red cell enzyme deficiency gradually increased with the development of molecular diagnostic methods and increasing general interest. However, the etiologies of HHA need to be pursued further in 5.1% of the patients. An systematic standard diagnostic approach is needed in a nationwide prospective study for correct diagnoses and appropriate management of HHA.
Adult
;
alpha-Thalassemia
;
Anemia, Hemolytic, Congenital*
;
beta-Thalassemia
;
Bilirubin
;
Cell Membrane
;
Diagnosis
;
Elliptocytosis, Hereditary
;
Erythrocyte Indices
;
Female
;
Hemoglobinopathies
;
Humans
;
Internal Medicine
;
Jaundice
;
Korea*
;
L-Lactate Dehydrogenase
;
Male
;
Oxidoreductases
;
Pallor
;
Pathology, Molecular
;
Pediatrics
;
Phosphopyruvate Hydratase
;
Prevalence
;
Pyruvate Kinase
;
Reticulocyte Count
;
Retrospective Studies*
;
Sex Ratio
;
Surveys and Questionnaires
7.Etiologic Distribution, Diagnostic Tests and Treatment in Prosp??ectively Registered Patients with DILD for Two Years in a Tertiary Medical Center.
Yong Soo KWON ; Man Pyo CHUNG ; Gyeong Man JEON ; Yeon Ju LYU ; Chang Min YU ; Jae Chul CHOI ; Eun Hae KANG ; Won Jung KOH ; Gee Young SUH ; Hojoong KIM ; O Jung KWON
Tuberculosis and Respiratory Diseases 2005;58(6):570-575
INTRODUCTION: Diffuse interstitial lung diseases (DILD) comprise of a large group of lung diseases with diverse etiologies. They are classified into four categories based on the etiology and pathological findings. In Korea, epidemiological data on DILD has never been reported in a prospective manner. METHOD: From May 2002 to April 2004, total 487 patients with DILD were prospectively registered at Samsung Medical Center. The prospective observational analysis of the etiologies, its classification based on 2002 ATS/ERS (American Thoracic Society/European Respiratory Society) guidelines, as well as diagnostic tests and the retrospective analysis of the treatment modalities were carried out. Any infectious and malignant causes were excluded. RESULTS: 1) The patients were classified into idiopathic interstitial pneumonia (IIP) in 269 patients (55.2%), known causes of DILD in 168 patients (34.5%), sarcoidosis in 27 patients (5.5%), other forms of DILD in 14 patients (2.9%), and undetermined DILD in 9 patients (1.9%). 2) The diagnostic test showed that most patients had undergone chest high resolution computed tomography (HRCT) and pulmonary function test (PFT) (97%, 89%). Transbronchial lung biopsy (TBLB) and surgical lung biopsy (SLB) were performed in limited patients (38%, 29%). 3) Among 269 patients with IIP, 220 (82%) had idiopathic pulmonary fibrosis (IPF) while 23 (9%) had nonspecific interstitial pneumonia. SLB was carried out in 36% of patients with IIP. 4) Symptomatic supportive care was given to 67% of IPF, but specific medical treatment including corticosteroids was administered to 89% of non-IPF patients. CONCLUSION: A nationwide registry of DILD patients is required to determine the annual incidence, etiology, and practice pattern of diagnosis and treatment in Korea.
Adrenal Cortex Hormones
;
Biopsy
;
Classification
;
Diagnosis
;
Diagnostic Tests, Routine*
;
Humans
;
Idiopathic Interstitial Pneumonias
;
Idiopathic Pulmonary Fibrosis
;
Incidence
;
Korea
;
Lung
;
Lung Diseases
;
Lung Diseases, Interstitial
;
Respiratory Function Tests
;
Retrospective Studies
;
Sarcoidosis
;
Thorax
8.Epidemiology and Clinical Outcomes of Childhood Wilms Tumor in Korea.
Won Suk SUH ; Im Joo KANG ; Hong Hoe KOO ; Hoon KOOK ; Soon Kee KIM ; Hack Ki KIM ; Hwang Min KIM ; Heung Sik KIM ; Kyung Duk PARK ; Kyung Bae PARK ; Sang Kyu PARK ; Jae Sun PARK ; Jun 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 ; Chuhl Joo LYU ; Kwang Chul LEE ; Kun Soo LEE ; Soon Yong LEE ; Young Ho LEE ; Young Tak LIM ; Pil Sang JANG ; Nak Gyun CHUNG ; Dae Chul JEONG ; Hae Lim JUNG ; Dong Whan CHO ; Bin CHO ; Yong Mook CHOI ; Jeong Ok HAH ; Pyoung Han HWANG ; Tai Ju HWANG
Korean Journal of Pediatric Hematology-Oncology 2004;11(2):164-170
PURPOSE: Wilms tumor is the most common malignant renal tumor in children. We investigated the epidemiology, clinical features and treatment outcome of the children with Wilms tumor in Korea during the recent 10 years. METHODS: Two hundred forty six patients were enrolled between January 1991 and December 2000 from 26 major hospitals in Korea. The data regarding the clinical features including sex, age, pathologic type, prognostic factor and treatment outcome of patients were analyzed retrospectively by review of patient's medical records. Kaplan-Meier survival curves were constructed, The differences between groups were analyzed by log-rank test. RESULTS: There were 130 males and 116 females. The incidence between the age of 1~4 years was the highest with 66.2%. The annual incidence rate per 1, 000, 000 population varied from 1.9 to 2.1. The 10 years overall survival rate according to sex, clinical stage, pathologic type and relapse were as follows: 88.6% in male, 90.9% in female, 100% in stage I, 94.7% in stage II, 92.1% in stage III, 63.4% in stage IV, 85.7% in stage V, 95.3% in favorable histology, 64.1% in unfavorable histology, 94.8% in non-relapse, and 40.9% in relapse. The relapse rate was 12%. The 10 years overall survival rate of 246 patients were 89.1%. CONCLUSION: Our results could provide the most recent and important clinical information on Wilms tumor of children in Korea.
Child
;
Epidemiology*
;
Female
;
Humans
;
Incidence
;
Kaplan-Meier Estimate
;
Korea*
;
Male
;
Medical Records
;
Recurrence
;
Retrospective Studies
;
Survival Rate
;
Treatment Outcome
;
Wilms Tumor*
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

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