1.Differences in Therapeutic Responses and Factors Affecting Post-Stroke Depression at a Later Stage According to Baseline Depression.
Eun Jae LEE ; Jong S KIM ; Dae Il CHANG ; Jong Ho PARK ; Seong Hwan AHN ; Jae Kwan CHA ; Ji Hoe HEO ; Sung Il SOHN ; Byung Chul LEE ; Dong Eog KIM ; Hahn Young KIM ; Seongheon KIM ; Do Young KWON ; Jei KIM ; Woo Keun SEO ; Jun LEE ; Sang Won PARK ; Seong Ho KOH ; Jin Young KIM ; Smi CHOI-KWON ; Min Sun KIM ; Ji Sung LEE
Journal of Stroke 2018;20(2):258-267
BACKGROUND AND PURPOSE: The pathophysiology of post-stroke depression (PSD) is complex and may differ according to an individual’s mood immediately after stroke. Here, we compared the therapeutic response and clinical characteristics of PSD at a later stage between patients with and without depression immediately after stroke. METHODS: This study involved a post hoc analysis of data from EMOTION (ClinicalTrials.gov NCT01278498), a placebo-controlled, double-blind trial that examined the efficacy of escitalopram (10 mg/day) on PSD and other emotional disturbances among 478 patients with acute stroke. Participants were classified into the Baseline-Blue (patients with baseline depression at the time of randomization, defined per the Montgomery-Asberg Depression Rating Scale [MADRS] ≥8) or the Baseline-Pink groups (patients without baseline depression). We compared the efficacy of escitalopram and predictors of 3-month PSD (MADRS ≥8) between these groups. RESULTS: There were 203 Baseline-Pink and 275 Baseline-Blue patients. The efficacy of escitalopram in reducing PSD risk was more pronounced in the Baseline-Pink than in the Baseline-Blue group (p for interaction=0.058). Several risk factors differentially affected PSD development based on the presence of baseline depression (p for interaction < 0.10). Cognitive dysfunction was an independent predictor of PSD in the Baseline-Blue, but not in the Baseline-Pink group, whereas the non-use of escitalopram and being female were more strongly associated with PSD in the Baseline-Pink group. CONCLUSIONS: Responses to escitalopram and predictors of PSD 3 months following stroke differed based on the presence of baseline depression. Our data suggest that PSD pathophysiology is heterogeneous; therefore, different therapeutic strategies may be needed to prevent PSD emergence following stroke.
Affective Symptoms
;
Anger
;
Citalopram
;
Depression*
;
Female
;
Humans
;
Random Allocation
;
Risk Factors
;
Stroke
2.Comparison of retrograde intrarenal surgery versus a single-session percutaneous nephrolithotomy for lower-pole stones with a diameter of 15 to 30 mm: A propensity score-matching study.
Gyoo Hwan JUNG ; Jae Hyun JUNG ; Tae Sik AHN ; Joong Sub LEE ; Sung Yong CHO ; Chang Wook JEONG ; Seung Bae LEE ; Hyeon Hoe KIM ; Seung June OH
Korean Journal of Urology 2015;56(7):525-532
PURPOSE: To investigate surgical outcomes between retrograde intrarenal surgery (RIRS) and percutaneous nephrolithotomy (PNL) groups for a main stone sized 15 to 30 mm and located in the lower-pole calyx. MATERIALS AND METHODS: Patients who underwent PNL or RIRS for a main stone sized 15 to 30 mm and located in the lower-pole calyx were retrospectively reviewed. Each patient in the RIRS group was matched to one in the PNL group on the basis of calculated propensity scores by use of age, sex, body mass index, previous treatment history, stone site, maximum stone size, and stone volume. We compared perioperative outcomes between the unmatched and matched groups. RESULTS: Patients underwent PNL (n=87, 66.4%) or RIRS (n=44, 33.6%). After matching, 44 patients in each group were included. Mean patient age was 54.4+/-13.7 years. Perioperative hemoglobin drop was significantly higher and the hospital stay was longer in the PNL group than in the RIRS group. The operative time was significantly longer in the RIRS group than in the PNL group. Stone-free rates were higher and complications rates were lower in the RIRS group than in the PNL group without statistical significance. The presence of a stone located in the lower-anterior minor calyx was a predictor of stone-free status. CONCLUSIONS: RIRS and single-session PNL for patients with a main stone of 15 to 30 mm located in the lower-pole calyx showed comparable surgical results. However, RIRS can be performed more safely than PNL with less bleeding. Stones in the lower-anterior minor calyx should be carefully removed during these procedures.
Adult
;
Aged
;
Female
;
Hemoglobins/metabolism
;
Humans
;
Kidney Calculi/pathology/*surgery
;
Length of Stay/statistics & numerical data
;
Male
;
Middle Aged
;
Nephrectomy/adverse effects/*methods
;
Nephrostomy, Percutaneous/adverse effects/*methods
;
Prognosis
;
Propensity Score
;
Retrospective Studies
;
Treatment Outcome
3.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
4.Analysis of 10,811 Cases with Acute Ischemic Stroke from Korean Stroke Registry: Hospital-Based Multicenter Prospective Registration Study.
Kyung Ho YU ; Hee Jun BAE ; Sun Uck KWON ; Dong Wha KANG ; Keun Sik HONG ; Yong Seok LEE ; Joung Ho RHA ; Ja Seong KOO ; Jong Sung KIM ; Jin Hyuck KIM ; Ju Hun LEE ; Soo Jin CHO ; Sung Hee HWANG ; San JUNG ; Moon Ku HAN ; Ki Hyun CHO ; Byeong Chae KIM ; Dong Jin SHIN ; Dae Il CHANG ; Jae Hyeon PARK ; Eung Gyu KIM ; Dae Soo JUNG ; Moo Young AHN ; Dae Hie LEE ; Kun Woo PARK ; Yong Jae KIM ; Kyung Yul LEE ; Ji Hoe HEO ; Seung Hyun KIM ; Kwang Ho LEE ; Chin Sang CHUNG ; Jae Kwan CHA ; Jun Hong LEE ; Keun Yong UHM ; Byung Chul LEE ; Jae Kyu ROH
Journal of the Korean Neurological Association 2006;24(6):535-543
BACKGROUND: Although several hospital-based stroke studies were published, there has not been any reliable data representing the clinical characteristics of stroke in Korea. We analyzed the clinical characteristics of patients with ischemic stroke registered in the Korean Stroke Registry (KSR), which is the largest prospective hospital-based nation-wide stroke registry in Korea. METHODS: The KSR provided standardized protocols for collecting data, which includes the data of demographics, subtypes of stroke, risk factors, and neurological outcome at discharge. The brain imaging studies, including CT or MRI, were performed in all cases. RESULTS: KSR registered 10,811 patients of acute ischemic stroke between Nov. 2002 and Jun. 2004. The large-artery atherosclerosis was the most common subtype (37.3%), followed by small vessel occlusion (30.8%). Hypertension (65.4%) was the most common risk factor, followed by smoking (34.5%) and diabetes (28.3%). Although most of the hypertensive and diabetic patients had been diagnosed before the stroke, less than 45.4% and 32.5% of them were under regular control. The steno-occlusive lesion of extracranial carotid artery was only 29.3% and the ratio of intra- to extracranial artery disease was more than 2 in KSR. Only 20.5% of patients were admitted within 3 hours after stroke onset and 2.1% were treated with intravenous thrombolysis. In-hospital case-fatality was 5.2%, which is relatively comparable to those of previous studies. CONCLUSIONS: The KSR provided informative data in understanding the clinical characteristics of ischemic stroke in Korea. Further analysis of KSR will facilitate clinical trials and development of guidelines for the management of stroke patients.
Arteries
;
Atherosclerosis
;
Carotid Arteries
;
Cerebrovascular Disorders
;
Demography
;
Epidemiology
;
Humans
;
Hypertension
;
Korea
;
Magnetic Resonance Imaging
;
Neuroimaging
;
Prospective Studies*
;
Registries
;
Risk Factors
;
Smoke
;
Smoking
;
Stroke*
5.The Usefulness of Non-contact Erection Observation for Evaluation Sexual Function in Male Rat.
Sung Hyun PAICK ; Byung Ki LEE ; Sung Wook YOON ; Chang Hoe AHN ; Yong Soo LHO ; Sang Kook YANG ; Hyeong Gon KIM
Korean Journal of Urology 2006;47(10):1107-1110
PURPOSE: Evaluation of sexual function is an important step for animal studies about sexual function. Male rats could display penile erection in the presence of an inaccessible estrous female. We examined the usefulness of observing non-contact erection (NCE) for the evaluation of sexual function in male rats. MATERIALS AND METHODS: Forty five Long-Evans male rats (8 weeks old) were used in this study. Tests for NCE were conducted in a glass chamber divided in half by 2 wire-mesh screens that prevented direct contact, but the rats freely passed other forms of stimulation. If at least 1 NCE could be observed during 45 minutes, we considered this as a positive response. In case of two or three positive responses in three NCE tests, that was considered as NCE (+). After NCE, copulation observations were noted to evaluate sexual function. If at least two ejaculations could be observed during 45 minutes, we considered that a positive response. In case of two or three positive responses in three copulation observations, that was considered as normal sexual function. RESULTS: Twenty one of 45 male rats (47%) were NCE (+) and 34 of 45 male rats (76%) had normal sexual function. In 21 NCE (+) rats, 20 rats had normal sexual function: only one was impotent. The positive predictive value of the NCE observation for the evaluation of sexual function was 95%. In 11 impotent rats, 10 rats were NCE (-). The specificity of the NCE observation was 91%. CONCLUSIONS: These results indicated that NCE observation could be a useful method for evaluating the sexual function of male rat.
Animals
;
Copulation
;
Ejaculation
;
Female
;
Glass
;
Humans
;
Male*
;
Penile Erection
;
Rats*
;
Sensitivity and Specificity
6.Loss of Heterozygosity Analysis of PTEN and DMBT1 Loci in Pediatric Brain Tumors.
Hye Lim JUNG ; Kyu Chang WANG ; Seung Ki KIM ; Young Yim KIM ; Keon Hee YOO ; Ki Woong SUNG ; Hong Hoe KOO ; Hee Young SHIN ; Hyo Seop AHN ; Hyung Jin SHIN ; Byung Kyu CHO
Korean Journal of Pediatric Hematology-Oncology 2004;11(2):195-204
PURPOSE: PTEN and DMBT1, candidate tumor suppressor genes on chromosome 10q, were identified based on deletions in glioblastoma and medulloblastoma cell lines. We examined the occurrences and frequencies of allelic deletions on chromosome 10q23 and 10q25 by loss of heterozygosity (LOH) analysis in 24 pediatric brain tumors to investigate the possible involvement of PTEN and DMBT1 gene deletions in the development of pediatric brain tumors. METHOD: LOH was analyzed by polymerase chain reaction (PCR) of PTEN locus on 10q23 using 2 microsatellite markers, D10S608 and D10S579, and of DMBT1 locus on 10q25-q26.1 using a microsatellite marker, D10S587, in 24 pediatric brain tumor (18 medulloblastomas, 3 ependymomas, 2 glioblastomas and 1 supratentorial primitive neuroectodermal tumor) DNAs extracted from archival tissue specimens (case 1-15, 19) or fresh frozen tissue specimens (case 16-18, 20-24). RESULTS: Allelic deletions were detected in 4 of 23 informative cases (17%) on D10S608, 6 of 24 informative cases (25%) on D10S579, and 8 of 24 informative cases (33%) on D10S587. Overall 11 of 24 cases (46%) showed LOH on chromosome 10q at PTEN or DMBT1 loci, and they were 10 medulloblastomas and 1 ependymoma pathologically. Of 18 medulloblastomas, 7 (39%) exhibited LOH at PTEN locus, 8 (44%) exhibited LOH at DMBT1 locus, and 10 (56%) exhibited LOH at one or both of loci. CONCLUSION: Our results support the notion that PTEN and DMBT1 tumor suppressor gene deletions may be involved in the pathogenesis of pediatric brain tumors. Our results also suggested that PTEN and DMBT1 tumor suppressor gene deletions may not be important in molecular mechanism of glioblastoma development in children as in adults.
Adult
;
Brain Neoplasms*
;
Brain*
;
Cell Line
;
Child
;
DNA
;
Ependymoma
;
Gene Deletion
;
Genes, Tumor Suppressor
;
Glioblastoma
;
Humans
;
Loss of Heterozygosity*
;
Medulloblastoma
;
Microsatellite Repeats
;
Neural Plate
;
Polymerase Chain Reaction
7.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*
8.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
9.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
10.Loss of Heterozygosity Analysis of Chromosome 17p13.1-13.3 and Its Correlation with Clinical Outcome in Medulloblastomas.
Hye Lim JUNG ; Kyu Chang WANG ; Seung Ki KIM ; Ki Woong SUNG ; Hong Hoe KOO ; Hee Young SHIN ; Hyo Seop AHN ; Hyung Jin SHIN ; Byung Kyu CHO
Korean Journal of Pediatric Hematology-Oncology 2003;10(1):72-81
PURPOSE: Cytogenetic studies molecular genetic studies, including loss of heterozygosity (LOH) studies, have shown that deletions on the short arm of chromosome 17 (17p) distal to TP53 locus are the most common genetic events in medulloblastoma, and that these occur in 30 to 50% of medulloblastomas. We examined the occurrences and frequencies of allelic deletions on chromosome 17p13.1-13.3 by LOH analysis to investigate the possible involvement of 17p13.1-13.3 in medulloblastoma development. We also performed survival analysis to determine whether LOH analysis of 17p13.1-13.3 can be used to predict prognosis in medulloblastoma. METHOD: LOH was analyzed by polymerase chain reaction (PCR) on chromosome 17p13.1-13.3 using 3 microsatellite markers, TP53 on 17p13.1, D17S796 on 17p13.1-13.2 and D17S1574 on 17p13.3, in 17 medulloblastoma DNAs extracted from archival tissue specimens (cases 1~15) or fresh frozen tissue specimens (cases 16 and 17). RESULTS: Allelic deletions were detected in 5 of 17 informative cases (29%) on TP53, 8 of 17 informative cases (47%) on D17S796, and 4 of 17 informative cases (24%) on D17S1574. Overall, 9 of 17 cases (53%) showed LOH on chromosome 17p13.1-13.3. The 5-year progression free survival (PFS) and 5-year overall survival (OS) rates were identical (59%). The 5-year PFS for 9 medulloblastoma patients with LOH on 17p13.1-13.3 was 56%, and the 5-year PFS for 8 medulloblastoma patients without LOH on 17p13.1-13.3 was 63%. In our survival analysis, we did not find a significant association between survival and LOH on 17p13.1-13.3. CONCLUSION: Our results support the notion that deletions of chromosome 17p13.1-13.3 may be involved in the pathogenesis of medulloblastoma. From survival analysis, we conclude that LOH on chromosome 17p13.1-13.3 may not be a significant predictor of prognosis in medulloblastoma.
Arm
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Chromosomes, Human, Pair 17
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Cytogenetics
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Disease-Free Survival
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DNA
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Humans
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Loss of Heterozygosity*
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Medulloblastoma*
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Microsatellite Repeats
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Molecular Biology
;
Polymerase Chain Reaction
;
Prognosis

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