1.Study on Current Curriculum Analysis of Clinical Dental Hygiene for Dental Hygiene Students in Korea.
Yong Keum CHOI ; Yang Keum HAN ; Soo Myoung BAE ; Jin KIM ; Hye Jin KIM ; Se Youn AHN ; Kun Ok LIM ; Hee Jung LIM ; Sun Ok JANG ; Yun Jung JANG ; Jin Ah JUNG ; Hyun Sun JEON ; Ji Eun PARK ; Hyo Jin LEE ; Bo Mi SHIN
Journal of Dental Hygiene Science 2017;17(6):523-532
The purpose of this study was to provide basic data to standardize the clinical dental hygiene curriculum, based on analysis of current clinical dental hygiene curricula in Korea. We emailed questionnaires to 12 schools to investigate clinical dental hygiene curricula, from February to March, 2017. We analyzed the clinical dental hygiene curricula in 5 schools with a 3-year program and in 7 schools with a 4-year program. The questionnaire comprised nine items on topics relating to clinical dental hygiene, and four items relating to the dental hygiene process and oral prophylaxis. The questionnaire included details regarding the subject name, the grade/semester/credit system, course content and class hours, the number of senior professors, and the number of patients available for dental hygiene clinical training purposes. In total, there were 96 topics listed in the curricula relating to clinical dental hygiene training, and topics varied between the schools. There was an average of 20.4 topic credits, and more credits and hours were allocated to the 4-year program than to the 3-year program. On average, the ratio of students to professors was 21.4:1. Course content included infection control, concepts for dental hygiene processes, dental hygiene assessment, intervention and evaluation, case studies, and periodontal instrumentation. An average of 2 hours per patient was spent on dental hygiene practice, with an average of 1.9 visits. On average, student clinical training involved 19 patients and 26.6 patients in the 3-year and 4-year programs, respectively. The average participation time per student per topic was 38.0 hours and 53.1 hours, in the 3-year and 4-year programs, respectively. Standardizing the clinical dental hygiene curricula in Korea will require consensus guidelines on topics, the number of classes required to achieve core competencies as a dental hygienist, and theory and practice time.
Consensus
;
Curriculum*
;
Dental Hygienists
;
Electronic Mail
;
Humans
;
Infection Control
;
Korea*
;
Oral Hygiene*
2.A de novo Microdeletion of ANKRD11 Gene in a Korean Patient with KBG Syndrome.
Ji Hun LIM ; Eul Ju SEO ; Yoo Mi KIM ; Hyun Ju CHO ; Jin Ok LEE ; Chong Kun CHEON ; Han Wook YOO
Annals of Laboratory Medicine 2014;34(5):390-394
KBG syndrome is a very rare genetic disorder characterized by macrodontia of upper central incisors, global developmental delay, distinctive craniofacial features, short stature, and skeletal anomalies. Ankyrin repeat domain 11 gene (ANKRD11) has recently been identified as a causal factor of this syndrome. We describe a 6-yr-old Korean boy with features of KBG syndrome. The patient had a short stature, macrodontia, dysmorphic facial features, speech and motor delay with intellectual disability, and partial seizures as indicated by the electroencephalogram, but he was neither autistic nor had autism spectrum disorders. Using high-resolution oligonucleotide array comparative genomic hybridization, we identified a heterozygous 240-kb deletion at 16q24.3 corresponding to ANKRD11. This patient provided additional evidence on the influence of ANKRD11 in KBG syndrome and suggested that deletion limited to ANKRD11 is unlikely to cause autism.
Abnormalities, Multiple/diagnosis/*genetics
;
Asian Continental Ancestry Group/*genetics
;
Bone Diseases, Developmental/diagnosis/*genetics
;
Child
;
Chromosomes, Human, Pair 16
;
Comparative Genomic Hybridization
;
Electroencephalography
;
Facies
;
Gene Deletion
;
Heterozygote
;
Humans
;
Intellectual Disability/diagnosis/*genetics
;
Male
;
Phenotype
;
Repressor Proteins/*genetics
;
Republic of Korea
;
Tooth Abnormalities/diagnosis/*genetics
3.Novel influenza A (H1N1) 2009 infection in the pediatric patients with hematologic and oncologic diseases in the Yeungnam region.
Seok Jeong KANG ; Jae Min LEE ; Jeong Ok HAH ; Ye Jee SHIM ; Kun Soo LEE ; Hyun Jung SHIN ; Heung Sik KIM ; Eun Jin CHOI ; So Eun JEON ; Young Tak LIM ; Ji Kyeong PARK ; Eun Sil PARK
Korean Journal of Pediatrics 2011;54(3):117-122
PURPOSE: Natural history and consequences of the novel 2009 influenza A H1N1 (2009 H1N1) infection in immunocompromised pediatric patients are not yet fully understood. In this study, we investigated the clinical features and outcomes of the 2009 H1N1 infection in pediatric patients with hematological and oncological diseases. METHODS: We retrospectively reviewed the medical records of 528 patients who had hematological and oncological diseases and who were treated at 7 referral centers located in the Yeungnam region. Among the 528 patients, 27 with definite diagnosis of 2009 H1N1 infection were the subjects of this study. All patients were divided into the following 3 groups: patients who were receiving chemotherapy (group 1), patients who were immunosuppressed due to a non-malignant hematological disease (group 2), and patients who were off chemotherapy and had undergone their last chemotherapy course within 2 years from the influenza A pandemic (group 3). RESULTS: All 28 episodes of 2009 H1N1 infection were treated with the antiviral agent oseltamivir (Tamiflu(R)), and 20 episodes were treated after hospitalization. Group 1 patients had higher frequencies of lower respiratory tract infection and longer durations of fever and hospitalization as compared to those in group 2. Ultimately, all episodes resolved completely with no complications. CONCLUSION: These results suggest that early antiviral therapy did not influence the morbidity or mortality of pediatric patients with hematological and oncological diseases in the Yeungnam region of Korea after the 2009 H1N1 infection. However, no definite conclusions can be drawn because of the small sample size.
Child
;
Fever
;
Hematologic Diseases
;
Hospitalization
;
Humans
;
Immunocompromised Host
;
Influenza A virus
;
Influenza, Human
;
Korea
;
Medical Records
;
Natural History
;
Oseltamivir
;
Pandemics
;
Referral and Consultation
;
Respiratory Tract Infections
;
Retrospective Studies
;
Sample Size
4.Workers intake too much salt from dishes of eating out and food service cafeterias; direct chemical analysis of sodium content.
Hae Ryun PARK ; Gye Ok JEONG ; Seung Lim LEE ; Jin Young KIM ; Soon Ah KANG ; Kun Young PARK ; Hyun Joo RYOU
Nutrition Research and Practice 2009;3(4):328-333
The average sodium intake of Koreans was reported to be 5,279.9 mg/day, which is one of the highest intake levels worldwide. The average Koreans intake 19.6% of sodium from kimchi, showing kimchi as the main contributor of sodium in this country (Ministry of Health and Welfare, 2005). The sodium content of dishes that are frequently chosen by workers, and which were served by foodservice cafeterias were chemically analyzed. The average sodium content of one meal provided by 10 foodservice cafeterias was 2,777.7 mg. Twenty-one, one-dish-meals, frequently chosen by workers for a lunch menu, were collected at 4 different restaurants for each menu by one male, aged in the twenties and analyzed chemically also. Workers who eat lunch at a workplace cafeteria everyday could intake about 8 g of salt at a one-time meal and those who eat out for a one-dish-meal would intake 3-8 g of salt without counting sodium content from the side dishes. From these study results, one could estimate that over 10 g of salt could be possible for a single meal for workers who eat out everyday. A nationwide nutrition campaign and education for low salt diets for restaurant owners and foodservice providers should be seriously considered.
Aged
;
Diet
;
Eating
;
Food Services
;
Humans
;
Lunch
;
Male
;
Meals
;
Restaurants
;
Sodium
5.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
6.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
7.Multiple Renal Abscess by Extended-Spectrum beta-Lactamase Producing Escherichia coli.
Min Kyung LIM ; Kye Weol KIM ; Dong Kun LEE ; Hye Kyung LEE ; Yoo Dong WEON ; Young Soo KIM ; Sun Ae YOON ; Young Ok KIM ; Yoon Sik CHANG
Korean Journal of Nephrology 2005;24(3):460-463
Extended-spectrum beta-lactamase (ESBL) producing bacterial infection causes delayed response to antibiotic treatment and consequently results in serious problem, because it can hydrolyze the majority of beta-lactam antibiotics. Here we report a case of multiple renal abscess by ESBL producing Escherichia coli in a patient with diabetes mellitus who had a history of repetitive urinary tract infection. A 49-year old woman was admitted to our hospital because of intermittent fever, left flank pain, and vomiting. She had been diagnosed as acute pyelonephritis and treated with the first and third cephalosporin for 1 month at a clinic. At admission, urinary bacterial culture revealed Escherichia coli resistant to both the first and third cephalosporin such as cefotaxime, ceftriaxone, and ceftazidime. Double disk synergy test confirmed ESBL. Abdominal computed tomography demonstrated multiple renal abscess. After treatment of imipenam for 4 weeks, she discharged with improved condition.
Abscess*
;
Anti-Bacterial Agents
;
Bacterial Infections
;
beta-Lactamases*
;
Cefotaxime
;
Ceftazidime
;
Ceftriaxone
;
Diabetes Mellitus
;
Escherichia coli*
;
Escherichia*
;
Female
;
Fever
;
Flank Pain
;
Humans
;
Middle Aged
;
Pyelonephritis
;
Urinary Tract Infections
;
Vomiting
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

Result Analysis
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