PURPOSE: Newborns may undergo many kinds of minor procedures. Since painful events during procedures may cause adverse effects on growth and/or development, nonpharmacological way to relive pain have been extensively studied including oral glucose and pacifiers. This study was undertaken to evaluate the analgesic effects of orally administered glucose and pacifiers in term neonates during minor procedures using validated behavioral pain rating system. METHODS: Randomized prospective studies with 81 term infants were assigned to one of three treatment groups: placebo (2 ml sterile water), glucose (2 ml 30% glucose), and pacifier (2 ml 30% glucose followed by a pacifier) during heelsticks. Intensity of pain was assessed by Douleur Aigue Nouveau-ne scale (DAN scale). RESULTS: Median pain scores (interquartile) during minor procedure and after 30 seconds were 7 (6-10), 4 (3-8) for sterile water; 7 (3-8), 1 (0-5) for 30% glucose; 4 (2-6), 1 (0-1) for 30% glucose and pacifiers, respectively. P values for comparisons of sterile water versus 30% glucose, and 30% glucose versus 30% glucose plus pacifiers were 0.019, 0.035 for during the procedure and 0.006, 0.034 for after 30 seconds, respectively. CONCLUSION: The analgesic effect of oral glucose during minor procedure is clinically apparent in full term neonates. Pacifiers plus glucose have a synergetic analgesic effect in neonates. These simple and safe interventions should be widely used during minor procedures in term neonates.
Glucose* ; Humans ; Infant ; Infant, Newborn* ; Pacifiers* ; Prospective Studies ; Water

PURPOSE: Infant leukemia is rare and accounts for 5% of leukemia in children. It differs from childhood leukemia in biologic and clinical features and has a poor prognosis. Research on infant leukemia is difficult due to the scarcity of cases. We studied the clinical progress and prognosis of infant leukemia diagnosed in our hospital, in order to contribute to the treatment and prognosis of infant leukemia. METHODS: The patients who were diagnosed with leukemia in the first 12 months of life were analysed between January 1991 and December 2000 in Yonsei Medical Center. We analysed the sex, age, clinical features, treatment outcome, prognostic factor, and survival rate. RESULTS: Among a total of 41 cases, 19 cases were diagnosed with acute lymphoblastic leukemia (ALL), 15 cases with acute myelogenous leukemia (AML), 2 cases with chronic myelogenous leukemia (CML), and 5 cases were unclassifed. Twenty-two were males and 19 females; age at diagnosis was 4 months in ALL, 8 months in AML, and 4 months in CML. Common clinical features at diagnosis were pale appearance and fever, others were poor oral intake, abdominal distension, and irritability. Hyperleukocytosis with average over 20,000/mm3, anemia, and thrombocytopenia were seen. By immunologic surface marker analysis, 8 of 15 B-lineage ALL were CALLA negative, early pre-B ALL. The remission induction rate was 79% in ALL and 60% in AML. The 5 year-survival rate of 41 patients was 29.2%. Sex, age at diagnosis, white blood cell count > 50 109/L, hepatomegaly, and CNS involvement were not prognostic factors. CONCLUSION: Infant leukemia differs from childhood leukemia in biological and clinical features and has a poor prognosis. Therefore, further clinical research is needed to improve the outcome of infant leukemia.
Anemia ; Child ; Diagnosis ; Female ; Fever ; Hepatomegaly ; Humans ; Infant* ; Leukemia* ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Leukocyte Count ; Male ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; Remission Induction ; Survival Rate ; Thrombocytopenia ; Treatment Outcome

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: There are several reports that the risk of development of leukemias is much higher in Down syndrome (DS) children than in non DS children. But there are a few reports about the clinical features of leukemia in Down syndrome and the prognosis in Korea. The object of this study is to evaluate clinical features, treatment results and the prognosis of leukemia of Down syndrome patients. METHODS: We conducted retrospective reviews in 10 children with leukemia of Down syndrome who were admitted to the Department of Pediatrics in Yonsei University Hospital between March 1986 and December 2000. We analyzed the clinical features, laboratory findings and survival rates. RESULTS: A male to female ratio was 1:1.25. Median age at diagnosis was 2 years 8 months. Initial symptoms were hepatosplenomegaly, petechiae, fever and upper respiratory infection symptoms. The number of patients by the type was as followed:acute myeloid leukemia (AML) 7 (70%), acute lymphocytic leukemia 2 (20%), acute mixed lineage leukemia 1 (10%). There were 4 cases of M7 subtype in AML. The median peripheral blood cell counts were as followed; leukocyte was 41,000/muL, hemoglobin was 8.7 g/dL, the platelet was 103,000/muL. The five years event free survival rate after diagnosis was 87.5% (7/8). The one patient relapsed and another one patient died of cardiac anomaly. CONCLUSION: There seemed to be several differences of clinical features between DS leukemia and non DS leukemia, especially prognosis. Multi-centered well organized study should be done to confirm our observation.
Blood Cell Count ; Blood Platelets ; Child ; Chromosomes, Human, Pair 21 ; Diagnosis ; Disease-Free Survival ; Down Syndrome* ; Female ; Fever ; Humans ; Korea ; Leukemia* ; Leukemia, Myeloid ; Leukemia, Myeloid, Acute ; Leukocytes ; Male ; Pediatrics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis* ; Purpura ; Retrospective Studies ; Survival Rate

PURPOSE: Chromosomal analysis has been helpful not only in pathophysiology of leukemia, but diagnosis, classification, management and predicting prognosis. However, little has been studied on chromosomal abnormality of pediatric leukemia in Korea. We have performed chromosomal analysis on childhood leukemia that we experienced, and tried to correlate chromosomal abnormalities with various types of leukemia. METHODS: Subjects were 28 of 84 patients diagnosed with leukemia and have been discovered to have chromosomal abnormalities on chromosomal analysis employing G-banding technique in Yonsei medical center from July 1996 to February 1999. RESULTS: Of the total 84 patients, Acute lymphocytic leukemia (ALL) accounted for 51 cases (61%), Acute myelocytic leukemia (AML), 30 cases (35%), Chronic myeloid leukemia (CML), 3 cases (4%). Chromosomal analysis in ALL: Of 51 cases, 9 cases (18%) showed chromosomal abnormality. Their mean age at diagnosis was 5.6+/-5.1 years. One case (12%) exhibited hyperploid (> 50 chromosomes), 4 cases (44%) pseudodiploid, and marginally-hyperdiploid was seen in 4 cases (44%). Structural abnormality involving translocation was seen in 6 cases, where t(3;9), t(4;11), t(12;?) 1 case respectively, del (13) 2 cases, and I (q9) 1 case. Chromosomal abnormality in AML: Of total 29 cases, 17 cases (55%) were found to have chromosomal abnormalities, with their mean age ranging 7.6+/-6.4 years. t(8;21) was found to be the largest, accounting for 5 cases, and t(15;17), t(1;22), t(1;11), t(10;11), del(5), inv(9) 1 case respectively, 21 trisomy in 1 case, 11 trisomy in 1 case. Other complex chromosomal abnormality was seen in 2 cases. Upon analysis of relationship between the chromosomal abnormality and FAB subtypes, 4 cases of M2- subtype were found amongst 5 cases of t(8;21), but the other chromosomal abnormalities and subtypes failed to show any correlation. Chromosomal abnormality in CML: Two cases (67%) of chromosomal abnormalities were found in 3 with CML. Their mean age at diagnosis was 152.7 years, and all cases showed t(9;22). CONCLUSION: Our study found that in pediatric AML, t(8;21) showed high incidence and was found to be related with M2-subtype. In CML, t(9;22) was found to be frequent, but the data lacks in accuracy as our sample was too small. For more precise information on incidences of chromosomal abnormalities and the prognostic implications that the cytogenetic properties of leukemia, further studies seem to be essential.
Child ; Chromosome Aberrations ; Classification ; Cytogenetics ; Diagnosis ; Humans ; Incidence ; Korea ; Leukemia* ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; Trisomy

PURPOSE: This study is to see chemotherapeutic results followed by Rotational Combination Chemotherapy (R-11 protocol) treatment for the patients with late relapse of childhood ALL six months after the completion of chemotherapy. METHODS: The subjects of study were the 13 children who had been diagnosed as ALL at the Severance Hospital and the Wonju Christian Hospital and completed the chemotherapy. They, however, diagnosed a late relapse of ALL between December, 1996 and December, 2001. After that, they were given an chemotherapy with R-11 protocol. RESULTS: The average age of the 13 patients was 11.8 ( 2.8) years old at relapse. They showed the complete remission (8 patients, 61%), partial remission (4, 31%), and induction failure (1, 8%). The total remission ratio was 92%. Four year Event Free Survival (EFS) after the second remission was 61.5%. They also showed acute toxicity during remission induction with severe than grade III according to the WHO criteria. The leukopenia and thrombocytopenia (hematologic toxicity) were seen at 11 cases (84%) and 9 cases (69%) respectively, and hepatotoxicity (non-hematologic toxicity) was shown at 6 cases (46%). There are also chronic toxicity, one case developed leukomalacia, and 6 cases did various symptoms of infections. CONCLUSION: There is no optimal chemotherapeutic protocol for late relapse of childhood ALL, and the treatment with R-11 protocol is worth a trial.
Child ; Disease-Free Survival ; Drug Therapy* ; Drug Therapy, Combination ; Gangwon-do ; Humans ; Leukopenia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Recurrence* ; Remission Induction ; Thrombocytopenia

Malignant rhabdoid tumor is a clinically aggressive neoplasm that was initially described as a distinctive renal tumor of childhood. But among the malignant rhabdoid tumors, extrarenal rhabdoid tumor is rare. We report an extrarenal neoplasm histologically and ultrastructurally identical to renal rhabdoid tumor that arose in the retroperitoneum of a 4-month-old boy and presented as a right lower abdominal mass. The tumor had an aggressive clinical course despite multimodal therapeutic regimens, and the patient died with disseminated disease 12 months after diagnosis.
Diagnosis ; Humans ; Infant ; Male ; Rhabdoid Tumor*

PURPOSE: The N-myc amplification is one of well known poor prognostic markers in neurblastoma. Because the traditional detection method, Southern blot, is expensive, labor-intensive and time-consuming, the detection of N-myc amplification is not routinely performed in Korea. The purposes of this study are to develop polymerase chain reaction (PCR) for detecting N-myc amplification in neuroblastoma tumor tissue, and to elucidate the clinical significance of N-myc amplification. METHODS: The clinical data and paraffin embedded tumor specimen of 54 neuroblastoma cases were collected from 10 medical centers in Korea. We have developed semiquantitative method of estimating gene copy number that uses differential PCR. N-myc gene primers (RC N-myc, N-myc 7-1) are amplified together with primers from a single-copy internal control gene (beta-globin). After ethidium bromide-stained agarose gel electrophoresis, the ratio of the two PCR products, which stands for N-myc amplification, is determined. Kaplan-Meier survival analysis was performed to evaluate the prognostic significance of N-myc amplification. RESULTS: The differential PCR was very effective, less expensive, less labor-intensive, and simple detection method for N-myc amplification. The percentage of N-myc amplification was higher in the patients older than 1 year old (34.1%: 14/41), when they were compared to the patients younger than 1 year old (16.7%: 2/12). The percentage of N-myc amplification was higher in the patients who have primary tumor at adrenal gland (40.9%: 9/22) than who have primary tumor at retroperitoneum (17.6%: 3/17) or at mediastinum (16.7%: 2/12). In Stage I, II, and III patients, the mean survival time of N-myc amplified group was 18 months and that of N-myc umamplified group was 64 months (Log Rank 4.35, P=0.037). CONCLUSION: The differential PCR was very effective, less expensive, less labor-intensive, and simple detection method for N-myc amplification. The N-myc amplification is one of poor prognostic indicators in Neuroblastoma.
Adrenal Glands ; Blotting, Southern ; Electrophoresis, Agar Gel ; Ethidium ; Gene Dosage ; Genes, myc ; Humans ; Korea ; Mediastinum ; Neuroblastoma* ; Paraffin ; Polymerase Chain Reaction* ; Prognosis ; Survival Rate

PURPOSE: The human chromosome 9p21 region that is a frequent site of deletions and rearrangements in many tumor types including leukemias implied the existence of a tumor suppressor gene within 9p21 which is involved in tumor formation. CDKN2 (p16) gene is located in the same chromosomal region. The loss of CDKN2 function is probably one of the most common genetic alterations and is now thought to play a key role in leukemogenesis. We examined the frequency of the point mutation of CDKN2 gene by analyzing the DNA sequence and demonstrated the prognostic implication of mutations of CDKN2 gene in childhood acute leukemia. METHODS: We investigated the prevalence of the point mutation in thirty patients with 20 cases of acute lymphoblastic leukemia (ALL) and 10 cases of acute myeloid leukemia (AML). The point mutation of CDKN2 gene was analyzed in a PCR generated DNA sequencing technique. RESULTS: There was no point mutation in exon 1 of CDKN2 gene. A missense mutation (G-- Arginine ; Base Sequence ; Chromosomes, Human ; Codon ; Exons ; Genes, p16 ; Genes, Tumor Suppressor ; Glycine ; Humans ; Leukemia* ; Leukemia, Myeloid, Acute ; Mutation, Missense ; Point Mutation ; Polymerase Chain Reaction ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prevalence ; Sequence Analysis, DNA ; T-Lymphocytes

BACKGROUND: Langerhans' cell histiocytosis is a proliferative histiocytic disorder of unknown cause formerly referred to histiocytosis X, with pathologic characteristics of abnormal proliferation of histiocytes which belong to the mononuclear phagocytes. The clinical manifestations range in severity from solitary lytic bone lesions to fatal multisystem disease, typically with indolent clinical courses. The authors reported here, the clinical features and therapeutic outcomes of Langerhans' cell histiocytosis according to stage and prognostic features. METHODS: We reviewed the medical records of 38 cases with Langerhans' cell histiocytosis confirmed by biopsy from March 1983 to March 1998 in Severance hospital for disease course, treatment, and late sequelae. RESULTS: 1) Median age of the patients was 3 years-old, and the male to female ratio was 2.2:1. 2) Fifteen cases were less than 2 years of age, 21 had soft tissue involvements, 10 had more than 4 organ involvement, and 8 had involved organ dysfunction. 3) As for the clinical stages, 19 cases were in stage I, 9 in stage II, 4 in stage III, and 6 in stage IV. As for the pathologic stages, 15 had monostic disease, 2 had polyostic disease, and 21 had multisytemic disease. 4) The incidence of more than 4 organ involvement in cases <2 years was significantly higher than that of cases > or = 2 years [53.3% (8/15) vs 8.7% (2/23), P=0.004], and the incidence of organ dysfunction in cases <2 years of age had a trend toward higher than that of cases > or = 2 years [33.3% (5/15) vs 3% (3/23)], indicating that cases <2 years had more frequent multisystem disease. In contrast, the incidences of more than 4 organ involvement and organ dysfunction in cases <15 years were similar to those of cases > or = 15 years. There was a significant correlation between the presence of more than 4 organ involvement and organ dysfunction (P=0.041). 5) The response rate of all cases was 71% (27 cases), and the response rate of 25 cases who received chemotherapy was 60% (15 cases). There was no difference in the response rate according to the type of chemotherapy. Overall survival rate was 63.4% at 50 months, disease-free survival rate was 56.7% at 24 months. The disease free survival rate was significantly lower in cases younger than 2 years of age than cases older than 2 years of age (P=0.047), in cases with 4 or more organs involvement than 3 or less (P=0.0002), in cases with evidence of organ dysfunction than without evidence of organ dysfunction (P=0.082), and in cases with soft tissue involvement than with only bone involvement (P=0.043). There was significant differences in disease free survival rate according to clinical stage (P=0.001). The overall survival and disease free survival rate of the cases older than 15 years of age were similar to those of the cases younger than 15 years of age were similar to those of the cases younger than 15 years of age. 6) Five cases died during follow-up periods, organ involvement, and organ dysfunction were found to be important prognostic factors, and cases with lesions limited to skeletal system showed more than 90% of survival rate. In the future, clinical investigation enrolled with more cases about the difference of clinical features and therapeutic outcomes between adult patients and pediatric patients should be warranted.
Adult ; Biopsy ; Child, Preschool ; Disease-Free Survival ; Drug Therapy ; Female ; Follow-Up Studies ; Histiocytes ; Histiocytosis* ; Histiocytosis, Langerhans-Cell ; Humans ; Incidence ; Male ; Medical Records ; Phagocytes ; Survival Rate