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

The purpose of this study was to assess the use of food materials at elementary school in Busan area and provide school meal management with basic data to improvement of guideline. A survey was done by e-mail with a set of questionnaires, which was responeded by 122 dietitians in elementary school foodservice. The results were as follows; Sixty percent of the dietitians though to the general imported food materials had to use. Regarding mean score of using the imported food materials; safety was 2.12/5.00, freshness 2.48/5.00, sanitation 2.68/5.00, nutrition 2.73/5.00, preference 2.93/5.00, external form 3.09/5.00, package 3.12/5.00, and price 3.63/5.00. Thirty seven percent of the dietitians had purchase the pro-environment farm products(PEFPs) and 32.0% of them purchased those products for the students' health. But 52.4% could not purchase to the PEFPs because of expensive price. They had negative opinions about deficient supplying of PEFPs 4.51/5.00, too expensive price 4.34/5.00, and the truth or false in source of PEFPs 3.96/5.00. Most of them perceived that using the PEFPs had to apply the step by step. Among of them, 33.9% had the knowledge of PEFPs, but 16.4 of them did not know to the PEPPs. 57.4% of them hoped to purchase the food materials through a center of supporting school meal servcie(it called non-profit-making organizations).
Busan* ; Electronic Mail ; Hope ; Humans ; Meals ; Nutritionists* ; Surveys and Questionnaires ; Sanitation

PURPOSE: Acute lymphoblastic leukemia (ALL) accounts for approximately 75% of all cases of childhood leukemia. We investigated epidemiology, clinical and laboratory features and treatment outcome of the children with ALL in Korea during recent 5 years. METHODS: One thousand forty nine patients were enrolled between January 1994 and December 1998 from 37 major hospitals in Korea. The data regarding the clinical and laboratory features including age, WBC counts at diagnosis, immunophenotype, morphology, cytogenetics 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 analyzed by log-rank test. RESULTS: There were 597 males and 452 females. The distribution between the age 2 and 5 years is most common in 46.1%. The annual incidence rate per 100,000 population varied from 1.6 to 2.2. The 5 year event free survival (EFS) rates according to good prognostic factors were as follows: 67% bet ween 1-9 year of age at diagnosis, 69% in under 10,000/mm3of initial WBC count, 74% in early pre-B cell CALLA ( ) immunophenotype, 65% in L3 morphology, 68% in no CNS invasion. Most of patients were treated by CCG treatment protocol. The 5 year EFS was 63%. Main complications were sepsis (21.8%) and hemorrhage (12.5%). The relapse rate was 15.6%. The common causes of death were sepsis, DIC, pneumonia, relapse. CONCLUSION: Our results could provide the most recent and important information about acute lymphoblastic leukemia of children in Korea.
Cause of Death ; Child ; Clinical Protocols ; Cytogenetics ; Dacarbazine ; Diagnosis ; Disease-Free Survival ; Epidemiology* ; Female ; Hemorrhage ; Humans ; Incidence ; Kaplan-Meier Estimate ; Korea* ; Leukemia ; Male ; Medical Records ; Pneumonia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Precursor Cells, B-Lymphoid ; Recurrence ; Retrospective Studies ; Sepsis ; Treatment Outcome

PURPOSE: Of the cancers in childhood, leukemia is the most frequent one. For the desirable control of childhood leukemia, the basic data for the incidence has a great importance. The authors made a report about the incidence of leukemia in childhood, which analyzed the data from 126 cases in Kyongnam province, Korea, during 1991~1995. METHODS: The data were obtained from 126 new cases of childhood leukemia who had been living in the Kyongnam province and were diagnosed at the 26 university hospitals or general hospitals in the Kyongnam area and other cities from 1991 to 1995. RESULTS: The age-and-sex adjusted annual incidence rate per 100,000 population during 1991~1995 varied from 1.82 to 2.86, and cumulative annual incidence rate was 2.41 (male 2.26 and female 2.57 respectively). Male to female sex ratio was 1:1 in total cases. By the major types of childhood leukemia, the cases were composed of acute lymphocytic leukemia 70.6%, acute myelocytic leukemia 26.9% and chronic myelocytic leukemia 2.5%. The cumulative annual incidence rate per 100,000 population (crude rate) during 1991~1995 were 2.77 in Ulsan city, 2.62 in Chinju city and 2.34 in the whole area of Kyongnam province. CONCLUSION: It was concluded that the age-and-sex adjusted annual incidence rate per 100,000 of childhood in Kyongnam province was 2.41, which was lower than that in Pusan city in the same period. And, there was no significant difference of the cumulative annual incidence rate between Ulsan area and Chinju area in the same period.
Busan ; Female ; Gyeongsangnam-do* ; Hospitals, General ; Hospitals, University ; Humans ; Incidence* ; Korea* ; Leukemia* ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Sex Ratio ; Ulsan

Garcin's syndrome is defined as showing total unilateral cranial nerve palsies (at least more than 7 ipsilateral cranial nerve palsies), without either sensory or motor long tract disturbance, and without intracranial hypertension. The present report describes an 8-year-old child who had swelling on the right postauricular area and ptosis on the same side which developed before 2 months ago. Neurologic examination disclosed complete unilateral cranial nerve palsies on the right side. Brain MRI revealed a bulky hypervascular well-enhancing solid tumor, involving the right temporal bone, middle cranial fossa, cerebellopontine angle, infratemporal and pterygopalatine fossae, and parapharyngeal space. A biopsy was performed, and its finding showed a chondrosarcoma.
Biopsy ; Brain ; Cerebellopontine Angle ; Child ; Chondrosarcoma* ; Cranial Fossa, Middle ; Cranial Nerve Diseases ; Cranial Nerves ; Humans ; Intracranial Hypertension ; Magnetic Resonance Imaging ; Neurologic Examination ; Pterygopalatine Fossa ; Temporal Bone

BACKGROUND: Genetic susceptibility to a variety of disease has been shown and it has recently been suggested that aplastic anemia is more common in adults who are HLA-DR2+ than in the general population. METHODS: To investigate whether certain HLA-DR gene is more common in children with aplastic anemia and HLA-DR2+ gene is associated with susceptibility to cyclosporine A (CyA), we analyzed the results of HLA-DR typing in 37 children with aplastic anemia with sequence specific oligonucleotide probe (SSOP) method. RESULTS: Eight patients were DR2+, and there was no more common than the normal population. All of the 8 patients with DR2+ were DRB1*1501+ with high resolution HLA typing. Of the 8 patients with DRB1*1501+, 3 patients were treated with CyA alone (2 patients) or CyA plus ATG (1 patient), 2 patients were treated with ATG (1 patient) or ALG alone (1 patient). Patients treated with CyA only or CyA plus ATG had either a complete (1 patient) or partial (2 patients) response and patients treated ATG or ALG alone had no response. CONCLUSION: Although incidence of HLA-DR2 was not significantly higher in children with aplastic anemia then normal population, response rate to CyA was significantly high in the DR2+ patients.
Adult ; Alleles* ; Anemia, Aplastic* ; Child* ; Cyclosporine ; Genetic Predisposition to Disease ; Histocompatibility Testing ; HLA-DR Antigens* ; HLA-DR2 Antigen ; Humans ; Incidence

BACKGROUND: Modern intensive chemotherapy has dramatically improved the prognosis of acute lymphoblastic leukemia in children. However, quality of life and even survival may be threatened by infection. Immunosuppression is experted due to disease itself or therapy, and sometimes, immunosuppression itself may lead to reactivation of latent viral infections in these patients. Often the viruses involved in the most severe infections suggest that patients suffer from defect in the cellular immunity. The principal defects that predispose leukemia patients to infection are defects of T cell, B cell, stem cell, complement, and macrophage. These contributing factors interact in a complex manner resulting in spectrum of problems. But these may result from a T cell defect and, in this study, 7 cell responsiveness of patients at diagnosis, remission induction, maintenance chemotherapy and after chemotherapy for leukemia has been investigated. Studies of the immune competence of patients undergoing chemotherapy for leukemia is in progress, but results are different from each other. METHOD: Between July 1994 and May 1996, seventy patients with childhood ALL were enrolled in this study. In order to expect frequency and depth of infection and prognosis, we investigated concentrations of immunoglobulins G, A, M, peripheral total lymphocyte count, 7 cell subsets, phytohemmaglutinin responsiveness, interleukln-2(IL-2), gamma-interferon(gamma-INF), and natural killer cell activity. RESULTS: 1) IgA concentrations were often markedly raised at diagnosis, and IgG, IgM concentrations both were within normal limits. During and after chemotherapy, IgA had fallen significantly but IgG, IgM are within normal limits. 2) Total lymphocyte count had fallen during chemotherapy, and returned to normal levels after chemotherapy. CD4+ T cell were markedly decresed at diagnosis, during chemotherapy and returned to normal levels after chemotherapy. 3) In vitro proliferative response of peripheral blood lymphocytes to the T cell mitogen phytohenagglutinin were impaired at diagnosis, during chemotherapy but did not returned to normal levels. 4) Interlekin-2, gamma interferon were normal levels at diagnosis, and had fallen in the induction of remission and quickly returned to normal levels with the swish to maintenance chemotherapy. But Interleukin-2 had fallen during and after chemotherapy. Natural killer cell activity had fallen at diagnosis, during chemotherapy and returned to normal levels after chemotherapy. CONCLUSION: It is assumed that evidence of impaired T cell responses is somewhat definite. These observations suggest that proliferative responses to phytohemagglutinin, CD4+ T cell, natural killer cell activity defects are due to leukemia itself but others more likely are generalizable defects caused by chemotherapy. Further investigations, however, have suggested a persisting defect in IgA, proliferative reponses to phytohemagglutinin, and interleukln-2. Our observations also show that despite normal immunoglobulin levels, most of these children have nonprotective levels for common childhood bacterial or viral disease. These results support to the praxis to withdraw prophylactic antibiotics after discontinuation of intensive chemotherapy and to start the immunization. It is expect to try to use cytokine on treatment and to improve mortality and morbidity for children of acute leukemia also.
Anti-Bacterial Agents ; Child* ; Complement System Proteins ; Diagnosis ; Drug Therapy* ; Humans ; Immunity, Cellular ; Immunization ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Immunoglobulins ; Immunosuppression ; Interferons ; Interleukin-2 ; Killer Cells, Natural ; Leukemia ; Lymphocyte Count ; Lymphocytes ; Macrophages ; Maintenance Chemotherapy ; Mental Competency ; Mortality ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis ; Quality of Life ; Remission Induction ; Stem Cells ; Virus Diseases

The kidney size is of special interest during the diagnostic work up of acute lymphocytic leukemia. But is still uncertain whether a finding of kidney enlargement at presentation has long-term prognostic value. We therefore reviewed the kidney size in children with acute lymphocytic leukemia by abdominal ultrasonograms at the time of diagnosis (n=54) and exmined if there was any statistical significance between the kidney size and prognostic parameters and later outcome. The following results were obtained 1) Among the patients whose kidney size was enlarged over 4SD from the normal, hepatomegaly in 3 cases (20%), splenomegaly in 3 cases (20%), mediastinal widening in 2 cases (13%) were noted. The interrelation between kidney size and infiltration of extramedullary system had no statistical significance. 2) Among the patients whose kidney size was enlarged over 4SD from the normal, patients under age 2 and over age 10 in 5 cases (33%), male in 8 cases (53%), involvement of central nervous system in 1 cases (7%), WBC count over 100x10E9/L in 3 cases (20%), Hb over 10 g/dl in 3 cases (20%) and platelet count below 100x10E9/L in 3 cases (20%) were noted. There was no statistical significance between kidney size and infiltration of extramedullary system. 3) Two Year survival rate based upon kidney size was; 95% in the group below 2SD, 79% between 2SD and 4SD, 59% over 4SD. And 2 Year event free survival rate was 71%, 56% and 58 respectively. In conclusion, the kidney size in childhood acute lymphocytic leukemia at the time of diagnosis influences the late outcome, but it is not a meaningful prognostic parameters.
Central Nervous System ; Child ; Diagnosis ; Disease-Free Survival ; Hepatomegaly ; Humans ; Kidney ; Male ; Platelet Count ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Splenomegaly ; Survival Rate ; Ultrasonography

No abstract available.
Factor VIII* ; Polymorphism, Restriction Fragment Length*

No abstract available.
Abortion, Spontaneous* ; Cytogenetics* ; Female ; Humans ; Pregnancy