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*

BACKGROUND: The effective ventricular function during ejection and filling is likely to depend on the coordinated action of the longitudinally and circumferentially orientated myocardial fibers and the function of these longitudinal fibers has not been extensively studied. METHODS: The role of longitudinally and circumferentially orientated fibers in left ventricular wall motion was evaluated by M-mode echocardiograms of the mitral ring(whose motion reflect long axis change) and the standard minor axis(left ventricular posterior wall), simultaneous recordings of phonocardiograms and electrocardiograms on the paper (speed 100mm/sec), in 24 healty individuals, 17 patients with mitral stenosis, 11 patients with open mitral commissurotomy and 17 mitral valve replaced patients. RESULTS: In the controls long axis shortening significantly preceded minor axis shortening (phase difference between two axes : 20+/-3 msec, mean+/-SEM) during early systole, indicating left ventricle become more spherical. This phase difference was also observed in the patients with mitral stenosis and in those with open mitral commissurotomy. In patients with mitral valve replacement(MVR) whose papillary muscles had been sectioned, the onset of long axis shortening was more delayed during early systole than that of short axis(-33+/-6msec) and the end of shortening was also prolonged to early diastole more than that of normal controls (54+/-3 msec vs 90+/-8 msec, mean+/-SEM, p<0.01 by t-test). CONCLUSION: We observed the time relations between long and short axis motion in normal controls. It can be concluded that the reversed time relation in patients with MVR is one of the important factors which may effect negatively on ventricular function and long-term prognosis, thus the surgical procedures to preserve papillary annular continuity should be considered in patients with mitral valvular disease. And the controlled, prospective, clinical trials with homogenous groups of patients are needed to evaluate the potential benefits of papillary annular continuity in preserving atrio-ventricular interaction in patients undergoing mitral valvular surgery.
Axis, Cervical Vertebra* ; Diastole ; Echocardiography ; Electrocardiography ; Heart Valve Diseases* ; Heart Ventricles ; Humans ; Mitral Valve ; Mitral Valve Stenosis ; Papillary Muscles ; Prognosis ; Systole ; Ventricular Function

OBJECTIVE: This study was aimed to investigate of newborn babies sex ratio in Seoul Seong Ae hospital and review of future problem. METHODS: We reviewed retrospectively the 21,332 cases of deliveries in women visiting at Seoul Seong Ae hospital from Jannuary 1994 to December 2003 (10 year's period). We investigated 1st baby and 2nd baby sex ratio and in case of 3rd baby, subdividing then into tree groups (first and second are female, first and second are male, first and second are male and female). We arranged the data at the base of childbirth registers and patients' obligation records. RESULTS: The results were as follows, 1. The total sex ratio in last 10 years was 111.2:100 (male:female). 2. 1st baby sex ratio was 109.2:100. 3. 2nd baby sex ratio was 110.6:100. 4. 3rd baby sex ratio was 133:100. CONCLUSION: These findings suggest that unbalance of sex ratio in 3rd baby is extremely serious trend in this country and is expected a tremendous social problems in early 21th century.
Female ; Humans ; Infant, Newborn* ; Male ; Parturition ; Retrospective Studies ; Seoul* ; Sex Ratio* ; Social Problems

This study was undertaken to develop species-specific forward and universal reverse PCR primers for the detection of Streptococcus sobrinus. These primers target the variable regions of the 16S ribosomal RNA coding gene (rDNA) and their specificity was tested against 10 strains of S. sobrinus strains and 20 different species of oral bacteria using serial dilutions of the purified genomic DNA of S.sobrinus ATCC 33478T . Our data show that species-specific amplicons were obtained from all the S. sobrinus strains tested but not from other species. Both direct and nested PCR could detect as little as 400 pg and 4 fg of genomic DNA from S. sobrinus ATCC 33478T , respectively. This result suggests that these PCR primers are highly specific and sensitive and applicable to the detection of S. sobrinus.
Bacteria ; Clinical Coding ; Collodion ; DNA ; Polymerase Chain Reaction ; RNA, Ribosomal, 16S ; Sensitivity and Specificity ; Streptococcus ; Streptococcus sobrinus

A 59-year-old man was admitted because of progressive proximal muscle weakness over 4 months. Serum creatine kinase was markedly increased and electromyography revealed myopathic pattern. Muscle biopsy showed nonspecific myopathic changes. No inflammatory change was shown. Thyroid function tests revealed very low thyroxine and low triiodo-L-thyronine, whereas thyroid-stimulating hormone was greatly increased as well as serum anti-thyroglobulin and microsome antibodies. The patient had hypothyroid myopathy due to Hashimoto's thyroiditis, which improved with L-thyroxine replacement.
Antibodies ; Biopsy ; Creatine Kinase ; Electromyography ; Humans ; Hypothyroidism ; Microsomes ; Middle Aged ; Muscle Weakness ; Muscular Diseases* ; Thyroid Function Tests ; Thyroid Gland* ; Thyroiditis* ; Thyrotropin ; Thyroxine