Methotrexate is a very potent inhibitor of dihydrofolate reductase and causes bone marrow suppression and megaloblastic anemia. It is widely used in combination with other chemotherapeutic agents in lymphoproliferative disorders. A 63 year old man with ischioneuralgia developed exertional dyspnea and dizziness after he had intentionally taken methotrexate in doses of 5mg per day for 2months. Five months after discontinuation of methotrexate, his bone marrow showed the hypercellular marrow with 90% cellularity, 15% blasts and marked dysgranulopoiesis, suggestive of refractory anemia with excess blasts(RAEB). The hematopoietic cells were not enough aspirated for proper diagnosis in follow up bone marrow after three months. The bone marrow aspirates showed 13% blasts, and marked dysgranulopoiesis. The bone marrow biopsy showed hypercellular marrow with 100% cellularity, but marked fibrosis was developed. The cytogenetic study revealed normal karyotype.
Anemia, Megaloblastic ; Anemia, Refractory ; Biopsy ; Bone Marrow ; Cytogenetics ; Diagnosis ; Dizziness ; Dyspnea ; Fibrosis ; Follow-Up Studies ; Humans ; Intention ; Karyotype ; Lymphoproliferative Disorders ; Methotrexate* ; Middle Aged ; Myelodysplastic Syndromes* ; Tetrahydrofolate Dehydrogenase

With the wide and extensive use of immunosuppressive agents and broad-spectrum antibiotics, opportunistic fungla infections have been increased. Fusarium spp. are known to be significant emerging pathogens of opporthunistic local infection. But very rarely it may cause fatalc systemic infection. A 4-year-old boy with acute lymphocytic leukemia develped asymptomatic disseminated purpura with high fever unresponsive to the antibiotics during chemotherapy. The skin lesions gradually increased in size and number, and prgreassed to forming central necrosis. Many septated hyphae and variable sized spore-like fungal elements are found in the epidrmis, dermis and subcuit on histologic sections. The pathogenic fungus was idenified as Fusarium oxysporum by culture and scanning electronic microscopic findings.
Anti-Bacterial Agents ; Child, Preschool ; Dermis ; Drug Therapy ; Fever ; Fungi ; Fusarium* ; Humans ; Hyphae ; Immunocompromised Host* ; Immunosuppressive Agents ; Male ; Necrosis ; Opportunistic Infections ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Purpura ; Skin

OBJECTIVES: This study aimed to identify psychopathologies of sexually abused children and intervening variables of symptom severity. METHODS: Eighty-four school-aged children were identified for sexual abuse from a center for child sexual abuse by psychiatrists, clinical psychologists, and social workers. We analyzed correlations among symptom severity, types of sexual abuse, gender, age, relationship with the abuser, family system, and current and past psychopathologies. RESULTS: The percentage of victims with particular psychiatric disorders (current) were 79.8%. Children without identifiable disorders were 20.2%, but these children had significantly increased scores on self report scales of anxiety (RCMAS), depression (CDI), and withdrawal scores on parental reports of child behavior checklist (K-CBCL). Sixty nine percent of abused children had primary diagnosis related to sexual abuse in DSM-IV diagnositic system. PTSD was 41.7%, depressive disorder was 38.1%, and anxiety disorder was 21.4%. Psychopathologies were more severe if perpetrators were of acquaintance or if victims had previous psychopathologies or parent-child relational problems. Types of primary caregiver and older age were also related to the severity of psychopathologies. CONCLUSION: Present study suggests that most victims of childhood sexual abuse suffer from significant psychological distress. Intervening variables are relationship with the perpetrator, previous mental health status, age of the child, type of the primary care taker, and the quality of parent-child relationship.
Anxiety ; Anxiety Disorders ; Caregivers ; Checklist ; Child Abuse, Sexual ; Child Behavior ; Child* ; Depression ; Depressive Disorder ; Diagnosis ; Diagnostic and Statistical Manual of Mental Disorders ; Humans ; Korea* ; Mental Health ; Parent-Child Relations ; Parents ; Primary Health Care ; Psychiatry ; Psychology ; Psychopathology* ; Self Report ; Sex Offenses ; Social Workers ; Stress Disorders, Post-Traumatic ; Weights and Measures

The erythroleukemic blast crisis in chronic myelogenous leukemia (CML) is rarely reported. We present two cases of erythroleukemic blast crisis of CML. In both cases, they had been treated with interferon and hydroxyurea prior to a blast crisis of CML. On blastic transformation, one patient underwent an acute clinical transformation marked with fever and hematochezia but the other showed no clinical deterioration. The blasts appeared in the peripheral blood. The bone marrow aspirates revealed megaloblastic erythroid hyperplasia (about 72%, 54% of all nucleated cells), increasing the number of myeloblasts (about 46%, 59% of all non-erythroid cells), and erythroblasts with a positive PAS stain. The cytogenetic studies revealed Philadelphia chromosomes with additional chromosomal abnormalities, t(3;21)(q26;q22) and the FISH studies revealed bcr-abl fusion signals in bone marrow cells. One case expired 8 months later despite of hydroxyuria therapy. The other case received allogeneic bone marrow transplantation (alloBMT) without complete remission but expired 34 weeks after alloBMT due to GVHD.
Blast Crisis* ; Bone Marrow ; Bone Marrow Cells ; Bone Marrow Transplantation ; Chromosome Aberrations ; Cytogenetics ; Erythroblasts ; Fever ; Gastrointestinal Hemorrhage ; Granulocyte Precursor Cells ; Humans ; Hydroxyurea ; Hyperplasia ; Interferons ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive* ; Megaloblasts

Nephrogenic diabetes insipidus (DI) is a rare disease in which the patient cannot concentrate urine despite appropriate or high secretion of antidiuretic hormone. Congenital nephrogenic DI is caused by the arginine vasopressin receptor 2 (AVPR2) or aquaporin 2 (AQP2) gene mutation; the AVPR2 genetic mutation accounts for 90% of the cases. National health screening for infants and children was launched in 2007 in order to prevent accidents and promote public health in infants and children in Korea. The program has been widely used as a primary clinical service in Korea. We treated an infant with faltering growth and delayed development detected by the National health screening program, and diagnosed the problem as nephrogenic DI caused by a rare missense mutation of c.490T>C on the AVPR2 gene. This case can be a good educational nephrogenic DI with a rare AVPR2 mutation, which was well screened and traced by the national health screening program for infants and children in Korea.

BACKGROUND: Use of single donor apheresis platelets and concerning for the quality of apheresis platelets has been rapidly increased. Apheresis platelets depleted white blood cell(WBC) are used to prevent or to reduce febrile non-hemolytic transfusion reactions, alloimmunization and cytomegalovirus infection. We compared COBE Spectra LRS (leukoreduction system) and COBE Spectra with PALL PXL 8 in terms of the yield predictors, processing times, and WBC contamination. METHOD: Seventy-two single donors who visited Apheresis Unit(APU) in St. Mary s hospital were prospectively randomized into COBE Spectra LRS and COBE Spectra followed by PALL PXL 8 between September 1997 and October 1998. We used Coulter counting for platelet and Nageotte hemocytometer for WBC count. Data were analyzed by independent t-test. RESULTS: The mean platelet yield per unit was 3.6+ 1.0 x 10 ' with COBE spectra LRS compared to 2.9+ 1.1 X 10 with COBE Spectra(p=0.002), and the mean WBC content per unit with COBE spectra LRS was 4.1 x 104(0.4-23.5) compared to 3.7 x 104(0.43-17.9) with PALL PXL""8(p=0.0728). CONCLUSIONS: This study shows that COBE Spectra LRS has higher platelet yields than that of COBE Spectra, and similar WBC contamination compared to PALL PXL 8. Therefore, this data suggests that COBE Spectra LRS is conveient than COBE Spectra with PALL PXL 8 in clinical practice. (Korean J Blood Transfusion 10(1): 43-51, 1999)
Blood Component Removal ; Blood Group Incompatibility ; Blood Platelets* ; Blood Transfusion ; Cytomegalovirus Infections ; Humans ; Leukocytes* ; Prospective Studies ; Tissue Donors*

BACKGROUND: A previous history of transfusion has been known to be associated with production of anti-HLA class I antibodies. However, platelet glycoproteins are the main target of idiopathic thrombocytopenic purpura (ITP). The mechanism of antibody production is known to differ significantly between glycoproteins and anti-HLA class I. The aim of this study was to evaluate the clinical significance of anti-HLA class I antibodies in childhood ITP. METHODS: Enrollment for the normal control group targeted 48 people who visited Gyeongsang National University Hospital from 1990 to 2010, and 48 young children with ITP. Anti-glycoproteins and anti-HLA class I antibodies were tested using the Modified Antigen Capture Enzyme-linked immunosorbent assay (MACE) kit. RESULTS: The positive rate of anti-HLA antibodies was significantly different [36/39 (92.3%) vs 29/46 (63%)] [ITP group vs normal control group] (P=0.002). The mean positive S/C ratio of anti-HLA antibodies was also significantly different (3.55 vs 1.51) [ITP group vs normal control group] (P=0.0000). The positive rate of anti-HLA did not differ significantly between the transfused group and the non-transfused group [12/12 (100%) vs 24/27 (88%)] [transfused ITP vs non-transfused ITP]. The mean positive S/C ratio of anti-HLA antibodies did not differ significantly between the transfused ITP group and the non-transfused ITP group (4.30 vs 3.25) [transfused ITP vs non-transfused ITP]. Consecutive testing showed that positive rate and positive S/C ratio of anti-HLA antibodies did not change significantly between sampling times in both groups [transfused ITP vs non-transfused ITP] (P=1.00 and P=0.15). CONCLUSION: Anti-HLA class I antibodies may be involved in childhood ITP. Transfusion did not affect the course of childhood ITP.
Antibodies* ; Antibody Formation ; Blood Platelets* ; Child* ; Enzyme-Linked Immunosorbent Assay ; Glycoproteins ; Humans ; Platelet Membrane Glycoproteins ; Purpura, Thrombocytopenic, Idiopathic*

VACTERL association is a disease with multiple congenital anomalies of the vertebrae, anus, cardia, tracheoesophageal(TE) fistula, renal and limb. This disease is derived from VATER anomaly, accompanied by cardiac and limb anomalies. We experienced a case of a 1-day-old boy with anal atresia, who represented multiple anomalies during hospital course. The multiple anomalies were hemivertebra, anal atresia, complex heart disease(coarctation of aorta, secundum aterial septal defect, patent ductus arteriosus), horseshoe kidney, tracheal stenosis, tracheal bronchus and left upper lung agenesis. Because both trachea and esophagus arise from fetal foregut, tracheal or pulmonary anomalies may be developed in VACTERL association instead of TE fistula. VACTERL association with tracheal anomalies, such as tracheal stenosis and tracheal bronchus or pulmonary agenesis, have been reported in foreign scientific society reports. But a case of VACTERL association with both tracheal bronchus and pulmonary agenesis has not reported yet. So we report this case with a brief review of related literature and suggest the consideration of possibility of tracheal or pulmonary anomaly in VACTERL association without TE fistula.
Anal Canal ; Anus, Imperforate ; Aorta ; Bronchi* ; Cardia ; Esophagus ; Extremities ; Fistula ; Heart ; Humans ; Kidney ; Lung ; Male ; Societies, Scientific ; Spine ; Trachea ; Tracheal Stenosis* ; Tracheoesophageal Fistula*

There is a wide variety of genital abnormalities observed in patients with Denys-Drash syndrome (DDS). WT1 is thought to influence the genes related to genital development and mutations in this gene have been associated with DDS. DDS should be considered in the differential diagnosis of newborns with genital anomalies. In contrast to other conditions with 46,XY disorders of sex development, individuals with DDS often have duplicated genital organs (a double vagina, cervix or uterus). A double uterus has not yet been reported with 1390G>A (Arg464 Asn) mutation. However, duplicated genitals have been reported with other genetic mutations in patients with DDS. The duplicated genitals in DDS may be associated with low anti-Mullerian hormone (AMH) secretion. Measurement of the AMH levels may add to our understanding of variations in genital development and their abnormalities in disorders such as DDS. In conclusion, this is first case of low level of AMH and double uterus in 1390G>A (Arg464 Asn) mutations of DDS male.
46, XY Disorders of Sex Development ; Anti-Mullerian Hormone* ; Cervix Uteri ; Denys-Drash Syndrome* ; Diagnosis, Differential ; Female ; Genitalia ; Humans ; Infant, Newborn* ; Male ; Uterus ; Vagina*

Down syndrome is a rare cause of neonatal cholestasis. Neonatal cholestasis in a patient with Down syndrome is usually associated with severe liver diseases, such as neonatal hemochromatosis, myeloproliferative disorder and intrahepatic bile duct paucity. We experienced a case of idiopathic neonatal cholestasis in a patient with Down syndrome, which resolved spontaneously.
Bile Ducts, Intrahepatic ; Cholestasis ; Down Syndrome ; Hemochromatosis ; Humans ; Infant, Newborn ; Liver Diseases ; Myeloproliferative Disorders