Classic pulmonary blastoma is a variant of carcinosarcoma which is seen almost exclusively in adults. By contrast, most cases of pulmonary blastoma in children have been described as having an exclusive mesenchymal composition, which was proposed as pleuropulmonary blastoma. Recently we experienced a case of pleuropulmonary blastoma, type 1. This 27-month-old male baby was transferred to our hospital due to the left tension pneumothorax. Chest CT revealed a subpleural pulmonary cystic lesion on the left upper lung and an open cystectomy was performed. Histologically the lesion was composed of variable-sized cystic structures lined with a single layer of respiratory-type epithelium. the underlying stroma was composed of sheets of small, round to oval, primitive tumor cells. Some of them had eccentric, eosinophilic cytoplasm, suggestive of rhabdomyoblastic differentiation. These rhabdomyoblastic cells were fuchsinophilic and positive with desmin and vimentin on immunohistochemistry.
Child ; Adult ; Male ; Female ; Humans

No abstract available.

Pseudohypoparathyroidism is a medical disorder characterized by a complex disorder of renal resistance to parathyroid hormone and the mechanism underlying the disease is still unclear. The authors described two cases of pseudohypoparathyroidism in sibling,who had metabolic anomalies(hypocalcemia and hyperphosphatemia, high circulatin immunoreactive PTH)and basal ganglia calcification. Bilateral basal ganglia calcifications, which was not visible on plain skull film, was detected by CT scan of brain MRI. We report these cases with a review of related literatures.
Basal Ganglia ; Brain ; Humans ; Hyperphosphatemia ; Magnetic Resonance Imaging ; Parathyroid Hormone ; Pseudohypoparathyroidism* ; Siblings* ; Skull ; Tomography, X-Ray Computed

Congenital absence of nails usually occur as a rare isolated anomaly or combined with other ectodermal defects. This anomaly is regarded as an inherited disorder either dominantly or recessively but quite a few cases were reported as sporadically developed. The patient was a 2-month-old girl who had no nails on both her 2nd, 3rd toes but had rudimentary nails on her left big toe and both 4th toes at birth. We could not find any other congenital deformity, any family history of inherited diseases related to anonychia. Radiological findings revealed no visualization of both 4th distal phalanges, only. We report this case as congenital anonychia of a sporadic type which may have developed independently from an underlying bone abnormality. We also review other reported cases in the literature.
Congenital Abnormalities ; Ectoderm ; Female ; Hallux ; Humans ; Infant ; Parturition ; Toes*

The VATER syndrome is a group of congenital anomalies with a nonrandom tendency for concurrence. Defects include vertebral, anorectal malformation, tracheoesophageal fisutla with esophageal atresia, radial-limb, vascular, and renal abnormalities. The critical period of organogenesis is at or before the sixth or seventh week of gestation. We experienced one case of VATER syndrome in a 1 day old male neonate having vertebral anomalies, esophageal atresia with tracheoesophageal fistula to the distal esophageal segment, imperforated anus, left renal dysplasia with hydronephrosis of the right kidney and both hydroureter, patent ductus arteriosus. We report a case of VATER syndrome with brief review of related literature.
Anal Canal ; Critical Period (Psychology) ; Ductus Arteriosus, Patent ; Esophageal Atresia ; Humans ; Hydronephrosis ; Infant, Newborn ; Kidney ; Male ; Organogenesis ; Pregnancy ; Tracheoesophageal Fistula

No abstract available.
Dermoid Cyst*

BACKGROUND: Hepatitis B vaccination schedule commonly used in Korea is divided largely into 0, 1, 2 month scheduled vaccination group(0, 1, 2 group) and 0, 1, 6 month scheduled vaccination gorup(0, 1, 6 group). The only difference bet.ween two groups is the interval from 2nd dose to 3rd dose. This st,udy had been carried out, to find whether t.he difference of vaccination interval influence the vaccination complet,ion rate or not. METHODS: Study objects are 135 persons over 20 years old who had heptitis B vaccination more than once in injection room of Dongkuk University Kyong-ju Hospital from Jan. 1st in 1996 to Dec. 31th in 1996. Data about vaccination completion were gathered from record book of injection room and telephone interview. RESULTS: Hepatitis B vaccination completion rate is 73.8% in 0, 1, 2 group and 72.5% in 0, 1, 6 group. The reasons for incomplete vaccination are forgetting vaccinat.ion date(36.4% in 0, 1, 2 group and 50% in 0, 1, 6 group), having no time to spare for vaccination(54.5% in 0, 1, 2 group and 43% in 0, 1, 6 group) and knowing positive HBsAb before completion of scheduled vaccination(9.1% in 0, 1, 2 group and 7% in 0, 1, 6 group). CONCLUSIONS: There is no difference in Hepatitis B vaccination complet,ion rate bet.ween 0, 1, 2 group and 0, 1, 6 group.
Appointments and Schedules ; Gyeongsangbuk-do ; Hepatitis B* ; Hepatitis* ; Humans ; Interviews as Topic ; Korea ; Vaccination* ; Young Adult

A female fetus with short rib(polydactyly) syndrome, Beemer-Langer type was terminated at 28 week's gestation because of abnormalities visualized on sonography. We present the clinical, radiological and autopsy findings of this case with a brief review of the literatures.
Autopsy ; Female ; Fetus ; Humans ; Polydactyly* ; Pregnancy ; Ribs*

PURPOSE: Acute promyelocytic leukemia (APL or AML, M3) represents an unique model for cancer research in terms of biological and clinical features. Since 1988, it has been widely confirmed that all-trans retinoic acid (ATRA) can induce complete clinical remission in over 85% of APL patients by a differentiation process, with PML-RARalpha protein possibly being the direct target of ATRA. However, ATRA treatment has two clinical limitations, namely, retinoic acid syndrome and retinoic resistance. Recently, it has been shown that arsenic trioxide used in some traditional Chinese remedy is very effective in retinoic resistant APL treatment. We tried to observe arsenic effect on cell lines and APL patient cells. MEHTODS: We investigated arsenic trioxide-induced apoptosis on APL, HL60, K562, KPH1 cell lines through MTT assay, DNA fragmentation assay and morphologic features. RESULTS: In MTT assay, cell survival rate decreased as the concentration of arsenic trioxide increased. In DNA fragmentation assay with HL60 cell line, DNA fragmentation was more frequent in high concentrations of arsenic trioxide than in low concentrations. During arsenic trioxide treatment, the morphologic change in bone marrow cells of APL patient, included nuclear differentiation and dark cytoplasmic granule during arsenic trioxide treatment. Serum arsenic reached peak level at 4hr after injection. We experienced a case of a 9-year-old male with APL who had relapsed after cessation of retinoic acid treatment. The patient successfully achieved remission following arsenic trioxide treatment without bone marrow depression and exacerbating bleeding diathesis. CONCLUSION: Arsenic trioxide can be used effectively to treat APL patients by inducing apoptosis and partial differentiation in tumor cells. The precise cellular and molecular mechanisms of its therapeutic effects remain to be determined.
Apoptosis ; Arsenic* ; Asian Continental Ancestry Group ; Bone Marrow ; Bone Marrow Cells ; Cell Line ; Cell Survival ; Child ; Cytoplasmic Granules ; Depression ; Disease Susceptibility ; DNA Fragmentation ; Hemorrhage ; HL-60 Cells ; Humans ; Leukemia, Promyelocytic, Acute* ; Male ; Tretinoin

PURPOSE: This study was designed to identify mother and infant related factors that influence child-care stress among the mother of newborn infants. METHODS: Data were collected through self-administered questionnaires and descriptive statistics, correlation and multiple regression were used to analyze the data. The data survey was conducted with 957 conveniently selected mothers of infants when they visited a public health center in Seoul to have their children immunized. RESULTS: The average item score for the Childcare Stress Inventory was 38.03, for the Edinburgh Postnatal Depression Scale, 9.31 and for the Degree of Bother Inventory, 23.42. The CSI was positively correlated to EPDS (r=.44, p<.001) and DBI (r=.40, p<.001). Also these two variables explained 30.0% of CSI in infants' mothers. CONCLUSION: These findings are expected to expand the understanding about postpartum mothers' child-care stress and can contribute to the development of comprehensive interventions based on community health nursing.
Child ; Community Health Nursing ; Depression ; Depression, Postpartum ; Female ; Humans ; Indoleacetic Acids ; Infant ; Infant, Newborn ; Mothers ; Postpartum Period ; Public Health ; Temperament ; Child Health ; Surveys and Questionnaires