No abstract available.
Klippel-Trenaunay-Weber Syndrome*

The onset of systemic scleroderma in the first. decade of lif occurs in less than 1.5% of instances. Systemic sclerodrma in childhood have been characterized by less severe visceral involvement and a benign course We report a boy, aged 7 years, with a history of Raynaid; phenomenon and dysphagia. He had a mask-like face, flexion contracture of hands and knee joints, digital pitting scars, ichthyosiform skin change on leg and hyperpigrnented induration on all extremities and trunk. Antinuclear antibodies were positive in nucleolar type and Scl-70 antiocy was also present. On radiologie study, the pattern of reflux esophagitis and resorption of the listal ends of all phalanges were nated. Histopathological findings were compatible with sclerodma. He was treated with Penicillamine-D and oral corticosteroic with good improvernent.
Antibodies, Antinuclear ; Cicatrix ; Contracture ; Deglutition Disorders ; Esophagitis, Peptic ; Extremities ; Hand ; Humans ; Knee Joint ; Leg ; Male ; Scleroderma, Systemic* ; Skin

Eosinophilic granuloma, Hand-Schuller-Christian and Abt-Letterer-Siewe syndrome are known to be the same disease entity involving the reticulohistiocytic system of the body, although their clinical manifestations are different. So they were included under the term of Histiocytosis X and this concept has been generally accepted. The authors have experienced one case of Hand-Schuller-Christian disease and that of eosinophilic granuloma. The former was a 23 years old man with complaints of exophthalmos, polyuria and a tender palpable mass on the right parietal area and in the latter a soft pulastile mase located on the left parietal area in a 3 year old boy. There was no neurological deficits on their admission. We managed them with radiation and steroid therapy following surgical curettage.
Child, Preschool ; Curettage ; Eosinophilic Granuloma ; Exophthalmos ; Histiocytosis* ; Histiocytosis, Langerhans-Cell* ; Humans ; Male ; Polyuria ; Rabeprazole ; Young Adult

OBJECTIVE: This study was performed to evaluate and compare the embryonic developmental capacity and pregnancy rates in conventional in vitro fertilization (IVF) and intracytoplasmic sperm injection (ICSI) with ejaculated sperm or testicular sperm cycles. MATERIALS AND METHODS: Fertilization was examined in the following morning after IVF (group I), ICSI (group II) or TESE-ICSI cycles (group III). Fertilized oocytes were co-cultured with Vero cells until embryo transfer (ET). On day 2 and 5~7, grades of embryos (<4- or > or =4-cell) and blastocysts (BG1, 2, 3 or early) were evaluated. Clinical pregnancy rate was determined by detecting G-sac with transvaginal ultrasonogram. We analyzed the results bychi2 and Student's t-test and considered statistically significant when P value was less than 0.05. RESULTS: Fertilization rate was significantly higher (p<0.05) in group I (79.0+/-21.2%) than in group II and III (56.8+/-21.6% and 36.7+/-25.3%). Cleavage and blastulation rate of group I (95.8+/-13.8% and 59.5+/-25.3%) were significantly higher (p<0.05) than those of group III (83.4+/-18.6% and 40.4+/- 36.5%). Clinical pregnancy rate was significantly higher (p<0.05) in group I and II (40.7% and 41.7%) than that in group III (12.5%). No differences were found in the rates of multiple pregnancy and abortion among three groups. Embryonic implantation rate was higher in group I (15.1+/-20.2%, p<0.05) and II (14.7+/-20.6%, NS) than that in group III (5.1+/-15.6%). However, embryonic implantation rate was increased in ET with blastocyst(s) among three groups. CONCLUSIONS: Fertilized oocytes obtained from TESE-ICSI were harder to be successfully cultured to blastocyst stage for 5~7 days than that from IVF cycles. However, all blastocyst(s) ET increased the embryonic implantation rate equally in IVF, ICSI and TESE-ICSI cycles.
Blastocyst ; Embryo Transfer ; Embryonic Development* ; Embryonic Structures ; Female ; Fertilization ; Fertilization in Vitro ; Oocytes* ; Pregnancy ; Pregnancy Rate* ; Pregnancy* ; Pregnancy, Multiple ; Sperm Injections, Intracytoplasmic* ; Spermatozoa ; Ultrasonography ; Vero Cells

The eukaryotic cell is compartmentalized by a series of vesicular organelles which constitute the endocytic and exocytic transport pathways. Each vesicular compartment has distinct sets of membrane proteins, structures and functions. Despite continuous vesicular transport, each vesicular compartment maintains its structure and function by use of retention and retrieval signal for its own resident proteins. Proteins in transit along the endocytic and exocytic pathway are transported without admixing with cytoplasmic constituents by successive steps of budding from the donor vesicles, formation of intermediate transport vesicles, transport, targeting to and fusion with acceptor vesicles. Specificity and fidelity of the vesicular transport are conferred by vesicular membrane proteins and small molecular weight GTP-binding proteins of the Rab subfamily. Proteins for export are packaged into specific vesicles for their final destinations. Insertion into and retrieval from the plasma membrane of transport proteins in response to cellular stimulus are a new paradigm of cellular regulatory mechanism. Secretion of neurotransmitters, hormones and enzymes by exocytosis involves a complex set of cytosolic proteins, G-proteins, proteins on the secretory granule membrane and plasma membrane. Much progress has been recently made in identifying proteins and factors involved in the exocytosis. But the molecular interactions among identified proteins and regulatory factors are unknown and remain to be elucidated. Finally our chemiosmotic hypothesis which involves the H+ electrochemical gradient across the secretory granule membrane generated by an ATP-dependent electrogenic H(+)-ATPase as the potential driving force for fusion and release of granule contents will be discussed.
Biological Transport ; *Exocytosis ; Human ; Organelles/*metabolism ; Support, Non-U.S. Gov't

We present a case of transient acantholytic dermatosis which developed on the face of a 19-year-old Korean girl. Asyrnptomatic pinhead to miliary-grain sized brownish papules arranged in a somewhat band-like outbreak along the left side of the nose for about 3 months. Histopathologic findings were similar to Dariers disease. Immunoperoxidase staining showed that IgG was deposited on the intercellular area of keratinocytes around the suprabasal clefts. Skin lesions disappeared spontaneously in 5 weeks after a skin biopsy.
Biopsy ; Darier Disease ; Female ; Humans ; Immunoglobulin G ; Keratinocytes ; Nose ; Skin ; Skin Diseases* ; Young Adult

No abstract available.
Animals ; Mice*

OBJECTIVE: In vitro fertilization (IVF) and a prolonging the time of culture may be helpful in establishing a viable pregnancy through a selection effect. Some embryos do not develop beyond the 4-cell stage and some may not develop to the blastocyst stage. We have evaluated the safety of SET and the outcomes of pregnancy. METHODS: Sperms were treated with Ham's F-10 supplemented with 10% human follicular fluid (hFF). Oocytes or fertilized oocytes were cultured in Dulbecco's Modified Eagle Medium (DMEM) with 10% or 20% hFF respectively. Up to five oocytes were inseminated with approximately 200,000 sperm cells/2 ml in each well. Fertilization was examined in the following morning and fertilized oocytes were co-cultured until embryo transfer. Vero cells for co-culture were prepared in Tissue Culture Medium - 199 (TCM-199) with 10% fetal bovine serum. At the two to four cell and blastocyst on day 2 and day 5, embryo and blastocyst grading were evaluated. Pregnancy rate was determined after transfer of human embryos at the two to four cell stage on day 2 (Group I) or subsequent transfer of embryos on day 2 and at the blastocyst stage on day 5 (Group II). For statistically significant when rho value was less than 0.05. RESULTS: No differences was found in the fertilization between Group I(81.0%, 98/121) and Group II(81.8%, 180/220). In case of cleavage rate, no difference was found in Group I(95.9%, 94/98) and Group II(66.7%, 12/18) than in Group (26.3%, 5/19). CONCLUSION: The results of this study showed that SET is safe and effective, and significantly increases the pregnancy rate.
Blastocyst ; Coculture Techniques ; Eagles ; Embryo Transfer* ; Embryonic Structures* ; Female ; Fertilization ; Fertilization in Vitro ; Follicular Fluid ; Humans ; Oocytes ; Pregnancy ; Pregnancy Rate ; Spermatozoa ; Vero Cells

OBJECTIVE: To evaluate the diagnostic value of computed tomography-myelography (CTM) compared to that of magnetic resonance imaging (MRI) in patients with lumbar radiculopathy. METHODS: The study included 91 patients presenting with radicular leg pain caused by herniated nucleus pulposus or lateral recess stenosis in the lumbar spine. The degree of nerve root compression on MRI and CTM was classified into four grades. The results of each imaging modality as assessed by two different observers were compared. Visual analog scale score for pain and electromyography result were the clinical parameters used to evaluate the relationships between clinical features and nerve root compression grades on both MRI and CTM. These relationships were quantified by calculating the receiver-operating characteristic curves, and the degree of relationship was compared between MRI and CTM. RESULTS: McNemar's test revealed that the two diagnostic modalities did not show diagnostic concurrence (p<0.0001). Electromyography results did not correlate with grades on either MRI or CTM. The visual analog pain scale score results were correlated better with changes of the grades on CTM than those on MRI (p=0.0007). CONCLUSION: The present study demonstrates that CTM could better define the pathology of degenerative lumbar spine diseases with radiculopathy than MRI. CTM can be considered as a useful confirmative diagnostic tool when the exact cause of radicular pain in a patient with lumbar radiculopathy cannot be identified by using MRI. However, the invasiveness and potential complications of CTM are still considered to be pending questions to settle.
Constriction, Pathologic ; Electromyography ; Humans ; Leg ; Magnetic Resonance Imaging* ; Myelography ; Pain Measurement ; Pathology ; Radiculopathy* ; Spine ; Visual Analog Scale

Borderline malignant ovarian tumor are characterized as neoplasrns exhibiting cellular priliferative changes greater than those seen in the benign form, but not showing destnictive invasion of the ovarian strama. We reviewed 14 cases of borderline ovarian tumor who had been diagnosed and treated at Kyung Pook university hospital from 1987 to 1992. We nnalysed the clinical features, treatment and prognosis which were compared to 24 cases of the epithelial ovarian cancer at the same period The most common histological thpe of boderline tumnr was mucinous type (1 l cases, 79%), the next one was serous type (3 cases, 21%). In FIGO stage of Borderline tumor, 11 caseS weve stage I a, 1 with stage II c, 1 with stage gb and 1 with stage lV, and the FIGO stage of 24 cases of ovarian cancer was 8 cases with stage I a, 1 with stage I b, 2 with stage I c, 1 with II c, 2 with stage III a, 1 with stage III b, 7 with stage III c and 2 with stage 1V. The mean age of borderline and malignant tumor was 37.1 and 47.6 years.. 3 cases were being pregnant on the operatian. In the 14 cases of borderline turnor, 7 cases were managed by conservative operation, but 1 case recurred 4 years later so extirpative operation and pastoperative chemotherapy were given. The other 4 cases were managed by extirpative operation but postoperative chemotherapy was given in only 1 patient. Follow up ranged from 7 to 72 months. 12 cases were alive. 2 cases died, one was stage III b, the other was atage IV. This atudy suggeat, that it is neceasary to follow up carefully even the early stage of botderline tumor and manage as oyarian carcinama in the advanced stage.
Drug Therapy ; Female ; Follow-Up Studies ; Humans ; Mucins ; Ovarian Neoplasms ; Ovary* ; Prognosis