Teratogenic Effect of X-ray on Cardiovascular System of Chick Embryo

BACKGROUND AND OBJECTIVES: Laboratory research was carried out to investigate the teratogenic effect of X-ray on chick embryos, especially with regard to cardiovascular malformation. MATERIALS AND METHODS: The chick embryos, 242, 242 and 215 eggs, were irradiated with X-ray at the dose of 500, 750 and 1000 cGy, respectively, during the incubation period between 0 and 9 days. A control group of 90 eggs received no irradiation. After 2 weeks of incubation, the embryos were sacrificed and examined. RESULTS: The survival rate of irradiated group was significantly lower than that of control group (62.5 vs. 87.8%, p<0.0001). The incidence rate of cardiovascular malformation was significantly higher in the irradiated than the control group (16.2 vs. 2.5%, p<0.005). The rate of cardiovascular malformation in the irradiated group increased from 9.4% with 500 cGy to 24.5% with 1000 cGy (p<0.05). There were a total of 33 cases of cardiac malformation, of which 24 were ventricular septal defects and 9 were complex congenital heart diseases. The higher the administered dose of radiation, the higher the observed incidence rate of cardiac malformation and the more complex the cardiac anomaly. Also, the rates of exocardia, exencephaly, beak anomalies and anopia were all increased in the irradiated group. CONCLUSION: X-ray irradiation of chick embryos increased the rates of death and cardiovascular malformation. The highest dose resulted in greater complexity of the cardiovascular malformation.

Measles Antibodies Measured by Plaque Reduction Neutralization Test in Infants during the First Year of Life / 소아과

PURPOSE: The 2000-2001 measles epidemic resulted in more than 50,000 cases with the highest attack rate occuring in infants less than one year of age, indicating the necessity of measles immunization before 12 months of age when a measles outbreak occurs again. The study was conducted to measure maternal measles antibody in infants by plaque reduction neutralization test(PRN), for the first time in Korea, to assess the optimal age for measles vaccination before the first birthday, when necessary. METHODS: Sera were obtained from 95 infants younger than 12 months of age who were healthy or recovered from mild llnesses, and had not had measles vaccination, measles infection, or blood transfusion. Measles antibodies were measured by PRN. RESULTS: Geometric mean titers and seropositive rates of measles antibody measured by PRN were 879.7 mIU/mL(100.0%), 690.0 mIU/mL(83.3%), 182.7 mIU/mL(50.0%), 91.3 mIU/mL(50.0%), 32.2 mIU/ mL(0.0)%, 25.1 mIU/mL(0.0%), 18.1 mIU/mL(0.0%), 38.4 mIU/mL(25.0%), 27.1 mIU/mL(0.0%), 31.2 mIU/mL(0.0%), 54.3 mIU/mL(0.0%), and 27.1 mIU/mL(0.0%) from 0 to 11 months respectively. CONCLUSION: By PRN, which was used for the first time to measure the measles antibody in Korea, placentally transferred measles antibody was detected in all newborns tested and decreased reciprocally to the age of infants, leaving almost all infants older than four months seronegative. These results indicate that measles vaccination at six months of age or older, which is the current recommendation during the period of epidemic issued by the Korean Society of Pediatrics, should not cause the primary vaccine failure. It seems advisable to utilize PRN further in order to find the optimal schedule for measles vaccination to infants born to women who were vaccinated.

Two Pediatric Patients with Neutropenia and Thrombocytopenia Associated with Parvovirus B19 Infection / 소아과

Parvovirus B19, a member of the Erythrovirus genus, is the only member of the Parvoviridae family known to be pathogenic in humans. Haematological consequences of B19 infection arise due to a direct cytotoxic effect on erythroid progenitors in bone marrow, resulting in interruption of erythrocyte production. The physiology of host haematopoiesis and competence of the immune response each determines clinical manifestations of B19 infection. A search of the literature revealed that neutropenia and thrombocytopenia due to B19 have been rarely reported in healthy individuals. In Korea, B19 infection has been reported among individuals with underlying disorders such as leukemia or congenital spherocytosis. Among otherwise healthy persons, there have been few reported cases of B19 infection which resulted in anemia, leukopenia, and thrombocytopenia. We herein report, for the first time in Korea, of two children who experienced leukopenia and thrombocytopenia, but not anemia, after B19 infection and recovered uneventfully.

A Case of Anaphylactic Response to Factor lX Replacement Therapy in Hemophilia B Patient / 대한혈액학회지

We report a first korean case of anaphylactic response to factor lX replacement therapy in 22-month-old male hemophilia B patient. He was admitted to our hospital via emergency room due to anaphylactic response after factor lX infusion. Immediately after the infusion, he became dyspneic and cyanotic. The symptoms completely resolved after sleep. He represented same symptoms after next factor lX infusion, which completely resolved by the administration of oxygen. At the time of anaphylaxis, inhibitors of factor lX was demonstrated(8.8 Bethesda Unit). We planned him to treat with recombinant factor Vll or diluted factor lX at bleeding episode.

Autologous Stem Cell Transplantation for the Treatment of Neuroblastoma in Korea

Autologous stem cell transplantation (ASCT) for the treatment of high-risk neuroblastoma (NBL) is an accepted method for restoring bone marrow depression after high dose chemotherapy. We retrospectively analyzed eighty eight cases of NBL that underwent ASCT following marrow ablative therapy at 12 transplant centers of the Korean Society of Pediatric Hematology-Oncology between January 1996 and September 2000. Seventy nine children were of stage IV NBL and 9 were of stage III with N-myc amplification. Various cytoreductive regimens were used. However, the main regimen was 'CEM' consisting of carboplatin, etoposide and melphalan, and this was used in 66 patients. Total body irradiation was also added in 36 patients for myeloablation. To reduce tumor cell contamination, stem cell infusions after CD34+ cell selection were performed in 16 patients. Post-transplantation therapies included the second transplantation in 18 patients, interleukin2 therapy in 45, 13-cis retinoic acid in 40, 131-meta-iodobenzylguanidine in 4, conventional chemotherapy in 11, and local radiotherapy in 8. Twenty two patients died, sixty six patients are surviving 1 to 46 months after ASCT (median followup duration, 14.5 months). Although the follow-up period was short and the number of patients small, we believe that ASCT might improve the survival rate in high-risk NBL.

Experimental Study for the Teratogenic Effect of Gamma-ray on the Heart of Chick Embryo

PURPOSE: To investigate the teratogenic effect of gamma-ray on the heart of chick embryo. METHODS: 50 rad, 100 rad, 150 rad, 200 rad, 250 rad, and 300 rad of gamma-ray were used to irradiate three days old chick embryos. The control group was not irradiated. After three weeks, the embryos were sacrificed and examined for cardiovascular malformation. RESULTS: The survival rate of the gamma-ray irradiated group was significantly lower than that of the control group(33.3-63.3% vs 76.4%, P=0.001). The cardiac malformation rate of the experimental group was 11.0%. In the control group, no congenital cardiac malformations were observed. The experimental groups had a significantly higher malformation rate(P=0.001). The types of malformation were ventricular septal defect, tricuspid atresia, Ebstein anomaly and aortic arch anomaly. In the gamma-ray irradiated group, the cardiac malformations were : 14 small ventricular septal defects (VSDs), five large VSDs, two tricuspid atresias, and one Ebstein anomaly. The higher the dose of radiation applied, the higher the incidence of cardiac malformation was noted. CONCLUSION: Gamma-ray irradiation of 3 days old chick embryos increased the rate of death and the rate of cardiac malformation significantly.

Epidemiology and Clinical Characteristics of Childhood Acute Lymphoblastic Leukemia in Korea / 대한소아혈액종양학회지

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.

A Case of Extraskeletal Ewing Sarcoma of Retroperitoneum / 대한소아혈액종양학회지

Ewing sarcoma is an uncommon bone malignancy of childhood. Although Ewing sarcoma is mostly a tumor of bone, it may also arise from soft tissues (extraskeletal Ewing sarcoma). We report a case of extraskeletal Ewing sarcoma that arose in the retroperitoneum of a 18-month-old girl and presented with right leg pain and gait disturbance. A brief review of related literatures was also made.

Analysis of Treatment in Pediatric Germ Cell Tumor

PURPOSE: This report is an analysis of our experience with surgery, multi-agent chemotherapy and radiotherapy, in pediatric germ cell tumors(GCTs). METHODS: This report included all children(age <15 years) who were registered between November 1985 and May 2001 with a histologically confirmed diagnosis of GCTand received surgery, chemotherapy and radiotherapy. Retrospective analysis of the medical records of these patients was performed. In addition to the clinicopathological features, the response rate, survival rate, and toxicity were analyzed. RESULTS: Among were a total of 19 patients, 6 had an ovarian tumor and 3 had a testicular tumor. Histologically immature teratoma was the most common type(11/19; 57.9%). Tumor markers were increased in 13/17(76.5%). Complete responses were observed in 17/19(89.5%) of the evaluable patients. Five year actuarial survival was 79.4% and event free survival was 84.2%. Median follow up was 46 months. CONCLUSION: From the present report, it is apparent that chemotherapy is very effective and well tolerated in children with GCTs. The data probably suggests that conservative surgery, when combined with effective chemotherapy, can result in cure of the majority of children with GCTs.

The Effects of Ifosfamide, Carboplatin, and Etoposide in Children with Recurrent/Refractory Solid Tumors / 대한소아혈액종양학회지

PURPOSE: The purpose of this study was to evaluate the effects of ifosfamide, carboplatin, etoposide (ICE) regimen in children with recurrent/refractory solid tumors. METHODS: The medical records of 7 patients diagnosed with recurrent/refractory solid tumors, including osteosarcoma in 2 patients, rhabdomyosarcoma in 2, neuroblastoma in 2 and medulloblastoma in one, and followed at Hanyang University Hospital from January, 1995 until May, 2001, were reviewed. The hematological toxicities above grade III, non-hematological toxicities above grade II, and response rate [complete response (CR) partial response (PR)] after several courses of ifosfamide 1,800 mg/m2/day (day 0 through 4 each cycle), carboplatin 400 mg/m2/day (day 0, 1), etoposide 100 mg/m2/day (day 0 through 4 each cycle) were evaluated. RESULTS: The incidences of hematological toxicities above grade III and non-hematological toxicities above grade II were 89% and 18%, respectively over the total 56 courses of ICE plus granulocyte colony-stimulating factor (G-CSF: 5.0mug/kg/day). Median time from the start of ICE chemotherapy to absolute neutrophil count (ANC) > or =1,000/mm3 for all patients during the total courses was 15 days. Seven patients evaluated for response to ICE. The overall response rate (CR PR) in this study was 57%. The CR rate for all diagnostic categories was 43%. CONCLUSION: Our study indicates that myelosuppression was the major toxicity of ICE chemotherapy and non-hematological toxicity was 20% of hematological toxicity except nausea and vomiting. The combination of ICE chemotherapy was associated with a high CR rate (43%) in children with recurrent/refractory solid tumors.

A Case of Congenital Malaria due to Plasmodium vivax / 감염

No abstract available.

Imaging Findings of the Brain Abnormalities in Acute Lymphoblastic Leukemia of Children during and after Treatment

PURPOSE: We evaluated the imaging abnormalities of the brain observed during and after treatment of acute childhood lymphoblastic leukemia. MATERIALS AND METHODS: The study group consisted of 30patients (male: female= 19:11; mean age, 64months) with acute childhood lymphoblastic leukemia during the previous ten-year period who had undergone pro-phylaxis of the central nervous system. Irrespective of the CNS symptoms, baseline study of the brain involving CT and follow-up CT or MRI was undertaken more than once. We retrospectively evaluated the imaging findings, methods of treatment, associated CNS symptoms, and the interval between diagnosis and the time at which brain abnormalities were revealed by imaging studies. RESULTS: In 15 (50% ; male : female=9:6 ; mean age, 77months) of 30 patients, brain abnormalities that included brain atrophy (n=9), cerebral infarctions (n=4), intracranial hemorrhage (n=1), mineralizing microangiopathy (n=2), and periventricular leukomalacia (n=3) were seen on follow-up CT or MR images. In four of nine patients with brain atrophy, imaging abnormalities such as periventricular leukomalacia(n=2), infarction (n=1) and microangiopathy (n=1) were demonstrated. Fourteen of the 15 patients underwent similar treatment; the one excluded had leukemic cells in the CSF. Six patients had CNS symptoms. In the 15 patients with abnormal brain imaging findings, the interval between diagnosis and the demonstration of brain abnormalities was between one month and four years. After the cessation of treatment, imaging abnormalities remained in all patients except one with brain atrophy. CONCLUSION: Various imaging abnormalities of the brain may be seen during and after the treatment of acute childhood lymphoblastic leukemia and persist for a long time. In children with this condition, the assessment of brain abnormalities requires follow-up study of the brain.

A Case of Jejuno-jejunal Intussusception in Henoch-Schonlein Purpura / 대한소아소화기영양학회지

Gastrointestinal involvement occurs in two thirds of children with Henoch-Schonlein purpura (HSP) and intussusception is by far the most common abdominal complication. Intussusception in HSP almost originates in the small bowel, which is in contrast with idiopathic intussusception. Earlier diagnosis and prompt treatment of intra-abdominal complications can reduce the mortality and ultrasound is the imaging modality of choice in evaluation the bowel manifestations of HSP. We report a case of jejuno-jejunal intussusception associated with HSP in a 5-year-old boy who presented with diffuse abdominal pain and vomiting after a few days of HSP onset. Abdominal ultrasound demonstrated intussusception in the jejunum with well defined target appearance because of the thickened intussusceptum, which disappeared on the computerized tomographic examination checked approximately 24 hours later. A brief review of literature was made.

A Clinical Study on Varicella zoster Virus Infection, Relating Factors and Treatment in Pediatric Cancer Patients

PURPOSE: We performed this study to contribute to the prevention and treatment of Varicella zoster virus infection in pediatric cancer patients through understanding of VZV infection, acyclovir efficacy and varicella vaccination. METHODS: A retrospective study was performed by reviewing chart from January 1988 to January 2001. RESULTS: The diseases among 19 cases were leukemia. 12 cases were solid tumors. Fourteen of leukemic patients were in maintenance chemotherapy, two in off chemotherapy, one in remission state, and two in the intensification when infected by VZV. There were 25 males and 6 females. The average age was 5.1 +/- 1.9 years in the chicken pox(CP) group and 6.5 +/- 3.1 years in the herpes zoster(HZ) group. The mean time of acyclovir treatment was 9.0 +/- 2.4 days and 9.4 +/- 2.9 days, respectively. Acyclovir was very effective. However, 2 patients showed slight increase of ALT/ AST. The HZ group had a more varicella infection history than the CP group. In the CP group, four patients had a history of varicella vaccination. In the HZ group, one patient of two who were without an infection history had a vaccination history. CONCLUSIONS: The most common therapeutic phase when infected was maintenance therapy. The prevalence of CP was higher in a lower age group than the HZ group and the mean time of acyclovir treatment was 9.2 days. All patients were cured. Despite a higher infection history in the HZ group, because of the history of varicella vaccination, we concluded that varicella vaccination can not prevent VZV infection. However, further investigation is necessary for the effect of vaccination on the manifestation.

Current Status and Comprehensive Care of Korean Hemophiliacs / 대한혈액학회지

BACKGROUND: Hemophilia A and B are sex- linked disorders that are due to respective deficiencies of clotting factors VIII and IX. Because hemophilia is life-long and life-threatening bleeding disorder, comprehensive care is very important and a lot of medical resources are required. We analyzed the status of Korean hemophiliacs to promote the comprehensive care of hemophilia and to utilize the limited medical resources appropriately. METHODS: We evaluated the past medical history for the 1,335 hemophilia patients who were registered in the Korea Hemophilia Foundation (KHF) from February 1991 to June 1999. Coagulation factor assay, viral studies, liver function tests and radiologic examination for orthopedic sequales were also performed. RESULTS: Of the total patients, hemophilia A and B were 1,142 cases (85.5%) and 193 cases (14.5%), respectively. Severe cases were 892 (66.8%), moderate 344 (25.8%) and mild 90 (6.7%). Their ages ranged from 7 months to 81 years and the median age was 18 years and 11 months. The number of patients diagnosed under 1 year old was 365 (28.5%). The family history of hemophilia was revealed in 55.4% of patients. 1,144 cases (90.7%) of 1,261 hemophiliacs experienced hemarthrosis, 61.9% epistaxis, 41.2% hematuria, 32.5% gastrointestinal hemorrhage and 15.4% intracranial hemorrhage. 217 cases (25.0%) of 868 severe patients bled over four times a month. Annual usage of factor concentrates per one patient in 1998 was average 44,306 IU/year. Average yearly usage of factor concentrates per kilogram body weight was 1,018 IU/kg/ year. Home treatment program was introduced to 59.2% of patients. Chronic hemophilic arthropathies were recognized in 791 cases (69.9%) of 1,131 patients. The frequencies of inhibitor development in hemophilia A and B were 5.1% and 4.3%, respectively. The prevalence of anti-HCV positivity was 48.9%, HBsAg positivity, 3.7% and anti-HIV positivity, 1.6%. CONCLUSION: The national registries and surveillance systems for hemophilia should be more firmly established to achieve comprehensive care of hemophilia and utilize the limited medical resources appropriately.

A Molecular Genetic Study with EcoRII Restriction Enzyme on the Steroidogenic Acute Regulatory Protein (StAR) Gene / 대한소아내분비학회지

The molecular defect of congenital lipoid adrenal hyperplasia has been discovered to be in the transport of cholesterol into mitochondria due to defective regulatory protein called "Steroidogenic Acute Regulatory Protein (StAR)", while the enzyme P450scc itself is normal. This study with EcoRII restriction enzyme aimed at elucidating more conveniently the molecular defect in the StAR gene. The genomic DNAs were extracted from their peripheral blood. We amplified the exon 7, hot spot, of the StAR gene with 1 set of primers by Polymerase Chain Reaction (PCR). Subsequently, a PCR product corresponding to target sequence (~437 bps) from the patient and her father have been sequenced by automatic sequence analyzer. The PCR-RFLP (Restriction Fragment Length Polymorphism) analysis after restriction digestion with EcoRII restriction enzyme was also performed on 12% polyacrylamide gel electrophoresis. The mutation was identified in the exon 7 of the StAR gene, substituting C for T at codon 258, consequently replacing glutamine by stop codon. This mutation alters EcoRII restriction site. In addition, we obtained the good result of PCR-RFLP (Restriction Fragment Length Polymorphism) analysis on 12% polyacrylamide gel electrophoresis. Therefore, the PCR-RFLP (Restriction Fragment Length Polymorphism) analysis with EcoRII restriction enzyme can be easily utilized to screen carrier, diagnose the patient prenatally or postnatally.

Two Cases of Central Nervous System Atypical Teratoid/Rhabdoid Tumors / 대한소아혈액종양학회지

Central nervous system atypical teratoid/rhabdoid tumor (AT/RT) of infancy and childhood, a new entity among malignant pediatric brain tumors, is unique histologic entity with an extremely aggressive natural history. Histologically AT/RT is defined as a polymorphous neoplasm often featuring rhabdoid, primitive neuroectodermal tumor, epithelial, and mesenchymal components. AT/RT occurs mainly in the posterior fossa, so mimics medulloblastoma. AT/RT is characterized by the cytogenetic finding of monosomy 22 rather than i (17q). Standard chemotherapy for infant and childhood medulloblastoma, for which this entity is often mistaken, has been ineffective. Most children survive less than 12 months (mean survival: 8.5 months) after diagnosis. We report two cases of central nervous system AT/RT in young children, one of them is alive so far with multimodal treatment.

A Case of Multicentric Infantile Myofibromatosis / 대한소아혈액종양학회지

Infantile myofibromatosis is one of the most common fibrous proliferation of infancy, characterized by the development of non-tender, firm, discrete, flesh-colored to purple nodules in skin, muscle, bone, and subcutaneous tissues. Three distinct subtypes are recognized-solitary, multicentric and generalized subtype. In solitary and multicentric subtypes, the prognosis is excellent with expected spontaneous regression of nodules in 1 to 2 years. But the prognosis of generalized subtype is poor with 75% of patients dying within the first few months of life secondary to cardiopulmonary or gastrointestinal complications. We report a case of multicentric infantile myofibromatosis in a 4-year-old boy presenting with multiple, asymptomatic, fixed and protruding masses on the posterior occiput, shoulders, upper and lower back and right lateral chest wall. Over the subsequent 20 monthes, the child developed limitation of motion of back and shoulder with the repeated formation and disappearance of myofibromatosis of the skin and musculature, but without visceral involvement.

A Case of Congenital Leukemia Cutis / 대한피부과학회지

Congenital leukemia is a rare disease developing within the first 4 to 6 weeks of life, whose natural history is quite different from that of childhood and adult leukemia. Leukemia cutis occurs in 25% to 30% of infants with congenital leukemia and is more frequently associated with acute myeloid leukemia than acute lymphocytic leukemia. We report a case of congenital leukemia cutis in which a walnut-sized, slightly erythematous, dome-shaped tumor that had developed at left frontal area since birth was confirmed by skin biopsy. A review and analysis of Korean literature pertaining specially to leukemia cutis in congenital leukemia is presented along with the report of an additional case.

Thyroid Cancer Following Radiation Therapy for Hodgkin's Lymphoma: Report of two cases

The risk of developing thyroid cancer after radiation exposure is well known, but specific modifiers of the dose-response relationship are not. Because Hodgkin's lymphoma affects younger patients with the potential for long-term survival, the subsequent development of thyroid neoplasia is a significant consideration. We experienced two cases of thyroid cancer following radiation exposure for Hodgkin's lymphoma, and we report our experience and present a review of the literature.