Nevoid basal cell carcinoma syndrome is inherited as an autcsonal dominant trait with a high degree of penetrane and variable expressivity. The mair features are basal cell carcinoma. jaw cysts, skeletal anornalies, ectopic calcifications and palmoplantar pits. We report varous clinical ma.nifestations in brother and sister, wnich are jaw cyst,, bifid rib, shortened fourth metacarpals, ectopie calcification, palrnoplanar pits and basal cell carcinoma, Microscopically, histologic patterns of basal cell cacinomas are superficial, nodulocystic, keratotic, pigmented, adenoid and follicullar nevoid. Lange numbers of basal cell carcinomas were electrodessicated and curetted under general,nesthesia.
Adenoids ; Basal Cell Nevus Syndrome* ; Carcinoma, Basal Cell ; Humans ; Jaw Cysts ; Metacarpal Bones ; Ribs ; Siblings

A study of effect of local hypothermia upon the paravertebral muscles which also become sensitized during spinal cord cooling was performed using cats. With a cuff, a cooler, to which was attached tubes connected to a refrigerator, the experimental technique was deviced to cool the paravertebral muscles locally at cervical and lumbar level. Cold, liquid saline at a temperature of 2.8+/-0.6 degrees C was circulated in closed system through the tube into the cuff which was snugly rested on the surface of paravertebral muscles as a heat exchanger. The temperature was measured at intervals of ten minutes with thermocouples before and during cooling for thirty minutes. In the muscle surface underneath the cuff, the mean precooling temperature of 30.1 degrees C in the normal muscle was lowered to 15.4 degrees C at the end of the first 10 minutes of cooling. After this initial rapid drop in temperature, there was a further gradual decrease of temperature to 13.6 degrees C at the end of 20 minutes of cooling. In the muscle 1cm beneath the cuff, the mean precooling temperature of 31.4 degrees C in the normal muscle was lowered to 17.3 degrees C at the end of first 10 minutes of cooling. After this initial rapid drop in temperature, there was a further gradual decrease of temperature to 16.2 degrees C at the end of 20 minutes of cooling. For comparison, the temperature in the clipped muscle and nonclipped muscle were also measured. The temperature in the clipped muscle surface was lower than that of non-clipped. Topical ice application resulted in rapid drop of temperature from 31.2 degrees C to 13.9 degrees C in skin, 32.3 degrees C to 13.1 degrees C in subcutaneous layer and 32.5 degrees C to 13.9 degrees C in muscle, simultaneously. Another aspect of this experiment was an evaluation of the protective effect of local hypothermia with respect to muscle injury associated with clipping, of muscles. The injured(clipped) muscles with or without local hypothermia was biopsied and stained with Hematoxylin Eosin and Hematoxylin Basic Funchsin Picric acid and sectioned specimens were observed under the light microscope. The clipped muscle examined thirty minutes after release of clipping showed 20~30% of red stain in HBFP stain. The normal muscle showed less than 5% of red stain in the field. In intermittent hypothermia, 2 minute cooling group showed 20% of red stain in the field. The clipped muscle with local hypothermia showed less red stain than that of non-hypothermia. The results of this study confirmed the belief that the cold liquid and ice of physiologic saline can be used in clinical neurosurgery for extravascular local cooling of scalp and paravertebral muscles and for irrigating or perfusing operative field.
Animals ; Cats ; Eosine Yellowish-(YS) ; Hematoxylin ; Hot Temperature ; Hypothermia ; Ice ; Muscles ; Neurosurgery ; Scalp ; Skin ; Spinal Cord

PURPOSE: The ability to perform chromosome analysis of cryopreserved cord blood mononuclear cells is important for future retrospective studies. We compared the karyotypes of cryopreserved cells with cells before cryopreservation. METHODS: One cord blood (CB) sample was obtained from normal healthy volunteer. Karyotype analysis was performed before cryopreservation. After mononuclear cell separation with Ficoll-Hypaque, the mononuclear cells were cryopreserved by programmed controlled-rate freezer and then transferred into the liquid nitrogen (-196 degrees C) for 3 days. After rapid thawing, cytogenetic analysis was performed as the same method for each sample by different conditions. The samples were divided by three groups. The first group was no culture before cryopreservation, the second group was 72 hours culture before cryopreservation, but no 24 hours culture after thawing and the third group was 72 hours culture before cryopreservation and 24 hours culture after thawing. RESULTS: The chromosome analysis was successful in the second and third groups of CB sample. CONCLUSION: The successful result from CB samples may suggest the usefulness of long-term cryopreservation for retrospective study in various clinical settings including hematologic malignancies.
Cell Separation ; Cryopreservation ; Cytogenetic Analysis ; Fetal Blood ; Karyotype ; Nitrogen ; Retrospective Studies

Scrub typhus is a zoonosis transmitted by a chigger of trombiculid mite and manifested with fever, skin rash, myalgia, and hepatitis etc. The renal involvement of the disease is prese-ted with transient microscopic hematuria and/or proteinuria. However acute renal failure or meningoencephalitis is very rare. We report a case of scrub typhus manifested with acute renal failure, meningoencephalitis, and hepatitis. The patient was treated with doxycycline and continuous arteriovenous hemofiltration (CAVH) and recovered successfully.
Acute Kidney Injury* ; Doxycycline ; Exanthema ; Fever ; Hematuria ; Hemofiltration ; Hepatitis* ; Humans ; Meningoencephalitis* ; Myalgia ; Proteinuria ; Scrub Typhus* ; Trombiculidae

For those with allergy, vaccination with a specific allergen has often been used as a major therapeutic measure. However, the universal application of this technique in clinics have been restricted due to its low success rates and the risk of active systemic anaphylactic shock (ASAS). In this regard, we constructed a fusion protein (OVA-DT), ovalbumin (OVA) fused with diphtheria toxin protein (DT), which may exert a specific cytotoxicity to cells bearing OVA-specific IgE. Its therapeutic effect was evaluated in mice (BALB/c) sensitized with OVA (Os-mice). OVA challenges to the OVA-sensitized mice (Os-mice) caused ASAS to death within 30 min, but OVA-DT treatment afforded mice complete protection. When OVA-DT was treated to the Os-mice, none showed the signs of ASAS when re-challenged 48 h after the treatment. OVA-DT itself was not found to be toxic or allergenic in normal mice. The effect of OVA-DT on the biological functions of mast cells was also studied. Binding of OVA-DT to OVA-specific IgE bearing mast cells and the inhibition of histamine release from these cells were observed. In addition, OVA-DT treatment inhibited the proliferation of OVA-specific B cells in mice. In Os-mice treated with OVA-DT, levels of anti-OVA IgG2a in serum and the production of IFN-gamma by splenic lymphocytes were found to increase, but the production of IL-4 by these cells decreased. Re-direction of cytokine profiles from OVA-specific Th2 to OVA-specific Thl is suggested. These results indicate that OVA-DT can protect Os-mice from ASAS due to OVA challenge, because it inactivates OVA-specific IgE-expressing cells, including mast cells and B cells.
Anaphylaxis/prevention | control* ; Animal ; B-Lymphocytes/immunology ; Female ; Histamine Release/drug effects ; IgE/metabolism ; Interferon Type II/biosynthesis ; Interleukin-4/biosynthesis ; Lymphocyte Transformation/drug effects ; Mast Cells/metabolism ; Mice ; Mice, Inbred BALB C ; Ovalbumin/immunology* ; Recombinant Fusion Proteins/therapeutic use*

Higenamine was widely used as traditional remedy for the treatment of rheumatoid arthritis. Nitric oxide (NO) may be a critical mediator in this inflammatory disease. Synovial tissue from humans with inflammatory arthritis expresses NOS2 (iNOS) mRNA and protein, and generates NO in vitro. We therefore, investigated the effect of higenamine on the induction of nitric oxide synthase (NOS) promoted by lipopolysaccharide (LPS). Prophylactic application of higenamine selectively prevented LPS-primed initiation of L-arginine-induced relaxation and restored phenylephrine(PE)-induced contraction in rat aorta. LPS-stimulated nitrite production in the incubation medium was reduced by higenamine. Furthermore, RT-PCR and Northern analysis indicated that higenamine reduced iNOS expression primed by LPS in rat aorta. These results suggest that higenamine prevents LPS-promoted induction of NOS in vascular smooth muscle.
Animals ; Aorta* ; Arthritis ; Arthritis, Rheumatoid ; Humans ; Muscle, Smooth, Vascular ; Nitric Oxide ; Nitric Oxide Synthase ; Rats* ; Relaxation ; RNA, Messenger*

OBJECTIVE: We investigated a possible use of the induced apoptosis as a biomarker in the cells and their media treated with commonly used anti-cancer agents in gynecologic malignancies. METHODS: After treatments with low and high concentrations of paclitaxel, cisplatin, and camptothecin in HeLa and OVCAR-3 cells, the levels of M30 antigen were detected in the cells and their media by immunofluorescence staining and ELISA methods, respectively. RESULTS: The percentages of M30-fluoresein isothiocyanate (FITC) positive cells in HeLa and OVCAR-3 cells treated with paclitaxel, cisplatin, and camptothecin were 4.3% vs 18.1% vs 34.87% and 4.07% vs 18.6% vs 32.63%, 4.3% vs 17.87% vs 32.38% and 4.07% vs 16.83% vs 32%, and 4.3% vs 16.75% vs 31.3% and 4.07% vs 15.18% vs 29.9% in control, low dose, and hight dose groups, respectively (P<0.001). M30 antigen levels (U/L) measured in culture media of HeLa and OVCAR-3 cells treated with paclitaxel, cisplatin, and camptothecin were 53.03 vs 101.53 vs 355.59 and 86 vs 114.41 vs 412.04, 53.03 vs 79.84 vs 327.64 and 86 vs 125.44 vs 385.09, and 53.03 vs 88.41 vs 295.005 and 86 vs 108.42 vs 263.1 in control, low dose, and hight dose groups, respectively (P<0.001). CONCLUSION: Our results obtained in this preclinical study suggests that measurement of the levels of M30 antigen may help to predict the clinical responses and to select the effective anti-cancer agents in clinical settings, rapidly and quantitatively.
Apoptosis ; Camptothecin ; Cell Line ; Cisplatin ; Culture Media ; Enzyme-Linked Immunosorbent Assay ; Fluorescent Antibody Technique ; HeLa Cells ; Humans ; Isothiocyanates ; Paclitaxel

PURPOSE: Controversy still exists over in the prognostic significance of microscopic tumor cell dissemination in patients with cancer. This study evaluated the prognostic implication of isolated tumor cells in the bone marrow of patients with gastric cancer. MATERIALS AND METHODS: Four hundred nineteen (419) patients who underwent surgery for gastric cancer between June 1998 and July 2000 were enrolled in the study. Bone marrow aspirate was obtained from the iliac crest before removal of the primary tumor. Mononuclear cells were isolated and stained with AE-1/AE-3 PAN-CYTOKERATIN. RESULTS: Cytokeratin-positive cells were found in the bone marrow of 219 patients (52.3%). The incidence varied significantly with the depth of invasion (P=0.021) and the stage (P=0.026). The five-year survival rate of patients with cytokeratin-positive cells was 74.1% and that of patients without cytokeratin-positive cells was 81.1% (P=0.2481). There were no significant differences in the recurrence rate and the site of recurrence according to whether or not cytokeratin-positive cells were present in the bone marrow. CONCLUSION: The presence of cytokeratin-positive cells in the bone marrow of patients with gastric cancer did not predict outcome and recurrence. Therefore, it cannot be used as a prognostic factor.
Bone Marrow* ; Humans ; Incidence ; Prognosis ; Recurrence ; Stomach Neoplasms* ; Survival Rate

PURPOSE: p53 is one of the most commonly mutated genes in human tumors. The aim of this study was to analyze p53 mutation in gastric cancer and its correlations with the clinicopathologic variables to clarify the usefulness of p53 mutation as a prognostic factor. MATERIALS AND METHODS: Specimens from 331 patients with gastric cancer who underwent a gastrectomy between March 1999 and April 2001 at the Kyungpook National University Hospital were used. p53 gene mutations were assessed by using a polymerase chain-reaction single-strand conformation polymorphism (PCR-SSCP) analysis. The correlations between p53 gene mutation and clinocopathologic parameters were analyzed. RESULTS: p53 mutations were found in 66 (19.9%) tumors. Among those 66 cases, mutations were seen in 23 tumors at exon 5, in 8 at exon 6, in 21 at exon 7, and in 17 at exon 8. Two mutations were shown in 3 tumors. Thirty-six (23.1%) of 156 intestinal-type tumors and 19 (13.1%) of 145 diffuse-type tumors showed p53 gene mutation (P=0.007). The frequency of p53 gene mutation didn't show any significant differences according to age, sex, stage, location, or gross type. Exon 5 mutations showed more frequently in intestinal-type tumors than in diffuse-type tumors (9.7% vs. 2.8%, P=0.024), and p53 mutation were more frequent in lymph nodes metastasis group than lymph nodes non-metastasis group with statistical significance (25.0% vs 15.6%, P=0.034). The five-year survival rate showed no statistically significant difference with p53 mutation (P=0.704). CONCLUSION: p53 mutations assessed by PCR-SSCP had little value as a prognostic factor after gastrectomy in patients with gastric cancer.
Exons ; Gastrectomy ; Genes, p53* ; Gyeongsangbuk-do ; Humans ; Lymph Nodes ; Neoplasm Metastasis ; Stomach Neoplasms* ; Survival Rate

OBJECTIVES: Apoptosis may play a major role in determining the growth and progression of the tumors. Certain oncogenes and tumor supressor genes are known to modulate apoptosis. The aim of study was to investigate whether apoptosis is related to the degree of differentiation, MIB-1 indicies, and expression of mutated p53 and bcl-2 in cervical neoplasms. MATERIALS AND METHODS: We examined 57 samples of normal, premalignant(i.e. mild, moderate and severe dysplasia and carcinoma in situ), malignant cervical tissue to evaluate whether differences in the apoptotic activity. Apoptotic cells and bodies were visualized by 3' end labelling. Simultaneously, quantitative immunostaining was performed for bcl-2 and p53, two known regulators of apoptosis. RESULTS: The cell proliferation index as determined by MIB-1 immunohistochemistry increased with progression from normal to cervical intraepithelial neoplasm and invasive cancer. The apoptotic index(AI) also increased with grade of lesion and was significantly associated with cell proliferation. However, the extent of apoptosis did not correlate with the expression of p53 and bcl-2. CONCLUSIONS: These results suggest that the elevation of AI in cervical neoplasm is associated with cell proliferation activity but is independent of the expression of p53 and bcl-2. It is likely that the effects on apoptosis of bcl-2 and p53 are countered by those of other oncogene products and/or additional factors that regulate apoptosis in vivo.
Apoptosis* ; Cell Proliferation ; Cervical Intraepithelial Neoplasia* ; Immunohistochemistry ; Oncogene Proteins ; Oncogenes ; Uterine Cervical Neoplasms