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*

BACKGROUND: Methyl alcohol poisoning has been mainly reported in community. Two cases of methyl alcohol poisoning occurred in a small-sized subcontracted factory which manufactured smartphone parts in Korea. CASE PRESENTATION: One young female patient presented with dyspnea and visual disturbance. Another young male patient presented with visual disturbance and myalgia. They treated with sodium bicarbonate infusion and hemodialysis for metabolic acidosis. In addition, he received ethyl alcohol per oral treatment. Her and his urinary methyl alcohol concentration was detected as 7.632 mg/L, 46.8 mg/L, respectively, although they were treated hemodialysis. Results of the working environment measurement showed that the concentration of methyl alcohol (1030.1–2220.5 ppm) in the air exceeded the time weighted average (200 ppm). They were diagnosed with optic neuropathy due to methyl alcohol poisoning and still have visual impairment. CONCLUSIONS: Workers who hired as dispatched employees in a small-sized subcontracted factory were exposed to high concentrations of methyl alcohol. The workplace had poor ventilation system. In addition, workers did not wear proper personal protect equipment. Working environment measurement and annual chekups for workers were not performed. They were in a blind spot to occupational safety and health. More attention is needed to protect vulnerable workers’ health.
Acidosis ; Aluminum* ; Dyspnea ; Ethanol ; Female ; Humans ; Inhalation* ; Korea ; Male ; Methanol* ; Myalgia ; Occupational Health ; Optic Disk ; Optic Nerve Diseases ; Poisoning ; Renal Dialysis ; Smartphone ; Sodium Bicarbonate ; Ventilation ; Vision Disorders

True thymic hyperplasia and ectopic thymus are very rare in children. In embryologic aspect, thymus is distributed around cervical area and ends up in mediastinum. This case is simultaneous thymic hyperplasia of neck and mediastinum. Ectopic thymus in the neck and thymic hyperplasia in the mediastinum in children were reported 2 and 7 cases respectively in Korea. In Clinical aspects, these thymic hyperplasia were presented by mass. So we should suspect these benign condition to avoid unnecessary operation or biopsy. We report a case of true thymic hyperplasia in the mediastinum with ectopic thymus in the neck in a 4-month-old male infant and review the relevant literature. We believe this is the first reported case in the world of true thymus hyperplasia in the mediastinum with cervical ectopic thymus in the neck.
Biopsy ; Child ; Humans ; Infant ; Korea ; Male ; Mediastinum* ; Neck* ; Thymus Gland* ; Thymus Hyperplasia*

PURPOSE: Acute pyogenic osteomyelitis is uncommon in children. Delayed diagnosis and inappropriate treatment are leading to growth failure and deformation. We review the clinical manifestations and treatment of acute osteomyelitis in children according to age. METHODS: A retrospective analysis was made of 32 patients who underwent antibiotic management or operation between Aug 1989 and Dec 2003 for acute pyogenic osteomyelitis in age from 0 to 15 years old. RESULTS: The study group was composed of 21 boys and 11 girls. The subjects were divided into four groups according to age: 0-1 yr (n=6), 1-5 yr (n=11), 6-10 yr (n=8), and 11-15 yr (n=7). Nineteen cases were diagnosed in Winter. Femur was the most common infected site (37.5%). There were no predisposing factor in 17 patients, and 7 of 15 patients had trauma history. Sepsis was important predisposing factor in neonates. The chief complaints were pain, swelling and fever. S. aureus (61%) is the most common organism. Twenty-nine patients were treated with operation and concomitant antibiotics. Two cases had sequelae in follow-up period: One is avascular necrosis of femur and the other is discrepancy of leg length. CONCLUSION: In our review, because of poor prognosis in septic neonates, we recommend to treat actively neonatal sepsis and prevent or detect osteomyelitis early. Because most of patients were diagnosed and treated in orthopedic surgery, the rate of operation was too high. So, protocol for further evaluation and management of acute osteomyelitis in pediatric patients were needed.
Adolescent ; Anti-Bacterial Agents ; Causality ; Child* ; Delayed Diagnosis ; Female ; Femur ; Fever ; Follow-Up Studies ; Humans ; Infant, Newborn ; Leg ; Necrosis ; Orthopedics ; Osteomyelitis* ; Prognosis ; Retrospective Studies ; Sepsis