No abstract available.
DNA* ; Herpesvirus 3, Human*

Craniofacial clefts are rare, mysterious and variable congenital anomalies. The incidence of craniofacial clefts is at least 1 per 100,000 live births. The cause of craniofacial clefts remains obscure, and attempts to classip craniofacial clefts are replete with problems. Tessier presented his classification in 1973 and used the orbit to distinguish the facial clefts from the cranial clefts. He noted that those lesions that involve both the face and cranium tend to do so in a consistent, predictable fashion. The clefts are numbered from 0 to 14 and are oriented around the orbit like spokes of a wheel. Van der Meulen proposed a classification in 1983 on an embryologic basis. The term dysplasia was used instead of cleft, since some of the malformations do not represent true clefts. Between 1997 to 1998, authors experienced 3 cases of median and paramedian facial cleft. One was No.2 facial cleft and the others were No.0-14 facial cleft with orbital hypertelorism. Their ages ranged from 1 to 5 years.
Classification ; Hypertelorism ; Incidence ; Live Birth ; Orbit ; Skull

Vascular leak syndrome(VLS) is characterized by endothelial damage, which causes extravasation of plasma proteins and fluid from capillaries into the extravascular space. It has been suggested that the increased vascular permeability is the result of an IL-2-induced suppression of endothelin-1 secretion by endothelial cells, an IL-2-induced activation of the complement cascade or TNF-alpha release from IL-2-activated T-cells. A 13-year-old male patient visited our hospital with abdominal pain. He was diagnosed with acute lymphoblastic leukemia(T cell type) by bone marrow study on his 2 nd day in hospital. On the 3 rd day of induction chemotherapy(prednisone, L-asparaginase, vincristine, intrathecal methotrexate), pulmonary edema and pleural effusion, ascites and generalized edema developed and lasted for 53 days without responding to supportive care. The laboratoy finding was that TNF-alpha was increased without evidence of infection and hypoalbuminemia was noted. It was suggested that the patient's clinical feature was induced by VLS.
Abdominal Pain ; Adolescent ; Ascites ; Blood Proteins ; Bone Marrow ; Capillaries ; Capillary Permeability ; Complement System Proteins ; Edema ; Endothelial Cells ; Endothelin-1 ; Humans ; Hypoalbuminemia ; Induction Chemotherapy* ; Male ; Pleural Effusion ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Pulmonary Edema ; T-Lymphocytes ; Tumor Necrosis Factor-alpha ; Vincristine

Eosinophilia is a common finding in premature babies during the neonatal period. Serial eosinophil counts were determined in 94 hospitalized, appropriately grown premature in fants whose gestational ages ranged form 28 to 36 weeks. The incidence, severity and etiologic factors of eosinophilia were retrospectively studied in premature infants, who were divided into three groups according to their gestational age. The results were as follows: 1) Absolute eosinophilia (>700/mm3)was documented in 46.8%(44/94). 2) The duration of TRN and antibiotics was cignificantly higher in infants with eosinophilia than withour eosinophilia (p<0.05). The gestational age of the infants with eosinophilia was significantly shorter than that of the infants without eosinophilia (p<0.05). The infants with eosinophilia started with bottle feeding significantly earlier than the infants without eosinophilia (p<0.05). 3) The infants younger than 30 weeks of gestational age have greater incidence of eosinophilia (75%) than the infant with the gestational age between 34 and 36 weeks (34.3%)(p<0.05). 4) The incidence of mild eosinophilia was higher in the group with gewtational age 30 weeks or below(37.5%)than in the group with gestational age between 34 and 36 weeks(17.1%)(p<0.05).Also, the incidence of severe eosinophilia was significantly higher in the group with gestational age 30 weeks or below(37.5%)than in the group with gestational age between 34 and 36 weeks(17.1%)(p<0.05). 5) Eosinophilia was more prevalent in the infants who received parenteral nutrition composed of glucose, amino acid and lipid(77.8%) than the infants who received only glucose(34.1%)(p<0.05). 6) Gestational age and birth weight were significantly lower in infants with severe eosinopilia than those of mild or moderate eosinophilia. The duration of TRN and antibiotics were significantly longer in infants with severe eosinophilia than those of mild or moderate eosinophilia (p<0.05). 7) The peak eosinophil count was observed significantly later in severe eosinophilic group (26.5 13.1 days)than in non-eosinophilic group(14.4 9.0 days)(p<0.05). The above results suggest that eosinophilia in premature infants may be the effecto of immature immunologic responses to the intravenous administration of extemal antigens like amino acid, lipid and antibiotics.
Administration, Intravenous ; Anti-Bacterial Agents ; Birth Weight ; Bottle Feeding ; Eosinophilia* ; Eosinophils ; Gestational Age ; Glucose ; Humans ; Incidence ; Infant ; Infant, Newborn ; Infant, Premature* ; Parenteral Nutrition ; Retrospective Studies

Craniofacial cleft is a rare disease, and has multiple variations with a wide spectrum of severity. Among several classification systems of craniofacial clefts, the Tessier classification is the most widely used because of its simplicity and treatment-oriented approach. We report the case of a Tessier number 3 cleft with wide soft tissue and skeletal defect that resulted in direct communication among the orbital, maxillary sinus, nasal, and oral cavities. We performed soft tissue reconstruction using the straight-line advanced release technique that was devised for unilateral cleft lip repair. The extension of the lateral mucosal and medial mucosal flaps, the turn over flap from the outward turning lower eyelid, and wide dissection around the orbicularis oris muscle enabled successful soft tissue reconstruction without complications. Through this case, we have proved that the straight-line advanced release technique can be applied to severe craniofacial cleft repair as well as unilateral cleft lip repair.
Classification ; Cleft Lip ; Cleft Palate ; Congenital Abnormalities ; Craniofacial Abnormalities ; Eyelids ; Maxillary Sinus ; Orbit ; Rare Diseases ; Reconstructive Surgical Procedures

A case of subgaleal hematoma accompanying ipsilateral exophthalmos is reported. The exophthalmos is due to orbital extension of subgaleal hematoma through potential gap between orbital septum and the lateral horn of the levator palpebral aponeurosis. We must remember that exophthalmos is able to occur after subgaleal hematoma.
Animals ; Craniocerebral Trauma ; Exophthalmos* ; Hematoma* ; Horns ; Orbit

BACKGROUND: Oncogenes and EGF-Receptor(EGFR) may be involved n different stages of the multistep carcinogenesis process. A specific pattern of karyotypic abnormalities in solid tumors can be detected by cytogenetic methods. OBJECTIVE: This study is intnded to observe the cytomolecular kiologic chracterization of c-myc, erb B and EGFR genes in squasnous cell carcinoma(SCC) of the skin and cervix. METHODS: We have eytogenet,ically examined the short-term culturs from SCC. The rearrangement, amplification or expressi.on of erb B, c-myc, and EGFR genes were studied by Southern blot, analysis of genomic DNA and by slot blot analysis of tota! RNA extracted from biopsies of normal skin and SCC tissues. EGFR expression was examined immunohistochemially using monoclonal antibodies and the localizat,ion of the c-myc oncogene mRNA by in situ hybridization. RESULTS: A remarkably structural aberration was del 6(q21-qter) counted 20 metaphases among 28 metaphases ana1yzed. In nunierical aberration, all chromosomes were lost or gained randomly. Amenploid including triploid and tetraploid were observed in 8 metaphases, 6 tumor cells contained marker chromosome. In Southern blot analysis, rearrangement and amglificaton of EGFR in primary squamous cell carcinoma of cervix uteri and skin respectively. In slot blot analysis, the levels of c-myc, erb B and EGFR mRNA increaaed respectively 3.5, 2.5 and 2.8 times in SCC when compared to normal tissues. In immunoperoxidase stain, EGFR was present, in SCC where keratinocytes with strong cyto-plasmic staining but no membr, line labelling, where as in normal skin the were primarily present in t,he membrane and cytoplasm of basal cells. In situ hybridization with c-myc cDNAs allowed detection of grains representative of biotin labelled cDNA-mRNA hybrids in the frozden section of SCC tissues. CONCLUSION: These results suggest that specific patterns of karyotypir abnormalites, rearrangement, or amplification of EGFR gene, and overexpression of oncogenes and EGFR gene may be associated with the carcinogenesis of SCC.
Antibodies, Monoclonal ; Biopsy ; Biotin ; Blotting, Southern ; Carcinogenesis ; Carcinoma, Squamous Cell* ; Edible Grain ; Cervix Uteri ; Cytogenetics ; Cytoplasm ; DNA ; DNA, Complementary ; Epidermal Growth Factor* ; Female ; Genes, erbB-1 ; In Situ Hybridization ; Keratinocytes ; Membranes ; Metaphase ; Oncogenes ; RNA ; RNA, Messenger ; Skin ; Tetraploidy ; Triploidy

Crowns ; Dental Implants ; Finite Element Analysis ; Prostheses and Implants

PURPOSE: The purpose of this study was to evaluate the clinicopathological significance of responders with metastatic colorectal cancer treated with oxaliplatin chemotherapy. METHODS: A total of 52 patients with unresectable metastatic colorectal cancer were enrolled for treatment between March 2000 and August 2005. Patients received first line chemotherapy consisted of oxaliplatin 85 mg/m2 or 130 mg/m2 as a 2-hour infusion on day 1, concurrently with leucovorin (LV) 20 mg/m2 as a bolus infusion on day 1~5, followed by continuous infusion of 5-fluorouracil (5-FU) 425 mg/m2 on day 1~5. This treatment was repeated in 2 or 3 week intervals. All responses were assessed after 4 cycles of therapy by independent radiologic experts and categorized into two groups: responder (major reduction of tumor) and non-responder group (no change or progression of the tumor. RESULTS: The response rate was 51.9 percent (27/52 patients). There were no significant differences in clinicopathologic parameters between two groups. The decrease of CEA value after chemotherapy was significantly more frequent in the responder group than in the non-responder group. CONCLUSIONS: We could not find any clinical differences between the two groups, but these results suggest that oxaliplatin chemotherapy has a beneficial effect on tumor shrinkage and serum CEA value can be an indicator for tumor response of oxaliplatin in advanced colorectal cancer.
Colorectal Neoplasms* ; Drug Therapy* ; Fluorouracil ; Humans ; Leucovorin

Multiple epiphyseal dysplasia (MED) is a clinically and genetically heterogeneous chondroplasia, characterized by delayed development of the ossification centers and, deformities of the extremities that involve only the epiphysis and result in mild short stature. Mutations in the cartilage oligomeric matrix protein (COMP) gene are most commonly found, and most of the mutations are located in the calmodulin-like repeats and the C-terminal domain. We report a Korean kindred of?12 family members with MED in four generations who were found to have a novel mutation in the COMP gene. A pedigree showed early onset osteoarthritis requiring arthroplasty that was an autosomal dominant inherited trait. Radiological examinations demonstrated the presence of osteochondral defects in the medial femoral condyles, and the knee and hip joints showed variable degrees of precocious degenerative changes. Mutation analysis of the COMP gene in the proband and five other affected family members identified a novel missense mutation, c.1280G>C (p.Gly427Ala) in exon 12, which was not found in three unaffected family members. Direct sequencing of the COMP gene may yield pathogenic mutations in dominantly inherited MED cases, and may provide opportunities of carrier detection among high-risk family members, leading to genetic counseling for early diagnosis and intervention before the onset of complications.
Achondroplasia ; Arthroplasty ; Cartilage ; Congenital Abnormalities ; Early Diagnosis ; Epiphyses ; Exons ; Extracellular Matrix Proteins ; Extremities ; Family Characteristics ; Genetic Counseling ; Glycoproteins ; Hip Joint ; Humans ; Knee ; Mutation, Missense ; Osteoarthritis ; Osteochondrodysplasias ; Pedigree