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*

Flow cytometry, a useful tool for measuring DNA content and cell differentiation as expressed by cell surface markers, is utilized to measure multiple antigens, especially surface antigen, intracellular oncoprotein, and DNA content, simultaneously. For this simultaneous detection, several methods off ixation and permeabilization have been used with limited values. In this study, 20 ng/ml of lysolecithin in 1% paraformaldehyde solution was utilized for fixation and permeabilization of cultured promyelocytic leukemic cells(HL 60). The cells were first stained with phycoerythrin (PE)-conjugated monoclonal antibody to the cell surface My 7 antigen and then were fixed and permeabilized with 20 ng/ml of lysolecithin in 1% partormaldehyde solution. After incubation, the fixed and permeabilized cells were stained with monoclonal antibody to intracellular c-myc antigen, which were followed by fluorescein isothiocyanate (FITC)-conjugated secondary antibody. The c-myc stained cells were finally stained for DNA content with 7-amino-actinomycin D(7-AAD). This procedure permits excellent staining for intracellular oncoproteins and preservation of surface antigens with relatively low cofficients of variation (CV) for the G0G1 peak of the DNA histograms and suggests that the sequential staining procedure of surface antigen, intracellular antigen, and DNA content will be extended for the study of correlations with cellular differentiation, expression of oncoproteins, and cell cycle analysis in the cells which are obtained from human malignant diseases using a 488 nm single laser flow cytometry.
Antigens, Surface* ; Cell Cycle ; Cell Differentiation ; DNA* ; Flow Cytometry* ; Fluorescein ; Humans ; Oncogene Proteins ; Phycoerythrin

To determine the frequency of peripapillary atrophy (zone beta)and the relationship between peripapillary atrophy and glaucomatous optic nerve damage in eyes with primary angle-closure glaucoma, we performed magnification-corrected morphometry of photographs of 88 eyes of 64 patients with primary angle-closure glaucoma. Forty-nine (56%)of 88 eyes with primary angle-closure glaucoma had zone beta. The zone beta/disc area ratio increased significantly (P=0.0001)with a decreasingrim/disc area ratio (r=-0.49).Moreover, the area, angular extent, width, and the frequency of zone betaincreased significantly with increasing stage of glaucoma (P<0.005).In contrast, there was no significant correlation between the zone beta/disc area ratio and perimetric results.We found that zone betawas present in more than half and it is closely associated with glaucomatous optic nerve damage in primary angle-closure glaucoma.
Atrophy* ; Glaucoma ; Glaucoma, Angle-Closure* ; Humans ; Optic Nerve

PURPOSE: The purpose of this study was to assess the usefulness of CT angiography(CTA) with spiral CT in evaluation of intracranial vascular lesions. MATERIALS AND METHODS: CTA and conventional angiography(CA) were performed in 22 patients with suspected intracranial vascular lesion. Nine patients had 10 aneurysms and 2 patients had arteriovenous malformation(AVM)s, while the remaining 11 patients had no vascular lesion. Twenty seconds after beginning injection of contrast media(100 mL with use of a power injector at the rate of 3 mL/sec), CT scanning(30-second continuous exposure and 60-mm length) was performed with a table speed of 2mm/sec and a section thickness of 2mm. The starting point was selected at the floor of the sella turcica. The resulting data were reformatted by MIP and SSD after reconstruction of 1 mm interval. For aneurysm, its size, shape, direction, neck and the relationship to adjacent vessels were compared to CA. RESULTS: The aneurysm diameter was ranged between 3mm and 20mm and all aneurysms were clearly visualized with CTA. CTA findings of the size, shape, direction, and neck of aneurysms and the relationship between aneurysm and adjacent vessels were well correlated with CA. In one case of aneurysm, calcification of the aneurysm wall was demonstrated on CTA. In one case of AVM, the nidus and its arterial supply and venous drainage were well visualized. In the other case of AVM, however, the feeding artery of AVM was not demonstrated on CTA(it was also unclear on CA). CONCLUSION: CTA with spiral CT may be useful in the evaluation of intracranial vascular lesion and valuable as a screening test for intracranial aneurysm.
Aneurysm ; Angiography* ; Arteries ; Drainage ; Humans ; Intracranial Aneurysm ; Mass Screening ; Neck ; Sella Turcica ; Silver Sulfadiazine ; Tomography, Spiral Computed*

We investigated the electrophysiological changes in the cerebral cortical evoked potentials(CEP's) and subsequent changes in the regional cerebral blood flow(rCBF) following increased intracranial pressure(ICP) in cats. A small balloon connected to a fine polyethylene tube was placed on the epidural space of the left parietal region through a small burr hole and inflated with saline in increment of 0.2 ml to simulate the expanding mass. The ICP was maintained at 150 mmH2O and 250 mmH2O levels during the experimental period. The study was conducted with 30 adult cats, weighing between 2.7 and 4.5kg. The animals were anesthetized with pentobarbital (50mg/kg) intraperitonially and tracheostomy was performed to maintain self respiration. Two small burr holes were made on both frontal regions for rCBF measurement and a small burr hole was made on the right primary sensorimotor cortex for a cerebral cortical stimulating electrode. A recording electrode for CEP's was placed on the dura of the left cerebellar hemisphere. For experiments, animals were divided into 3 groups. Group I:Animals(n=10) with 80mmH2O of ICP, Group II:Animals(n=10) with 150 mmH2O of ICP, Group III:Animals(n=10) with 250mmH2O of ICP. The CEP's and rCBF measurements were carried out in each animal before and immediately after increased ICP(IICP), at the 30th min, 60th min, 90th min, 120th min, 150th min and 180th min after IICP. The rCBF was measured by hydrogen clearance method. The results were as follows ; 1. A significant elevation of the systolic blood pressure was observed after the 60th min in both IICP groups. 2. 1) Group II animals showed a significant reduction of rCBF by 10.5% and 39.5% in the right frontal lobe at the 60th min and 180th min after IICP, and by 19.8% and 57.7% in the left frontal lobe at the 60th min and 180th min after IICP, respectively. 2) Group III animals showed also a significant reduction of rCBF by 18.2% and 54.4% in the right frontal lobe at the 60th min and 180th min after IICP, and by 62.9% and 84.7% in the left frontal lobe at the 60th min and 180th min after IICP, respectively. 3) Reductions of the rCBF of the left frontal lobe in the Group III animals were greater than those of the Group II animals. 3. 1) Changes of amplitude and latency in the CEP's were more prominent in the Group III animals than those of the Group II animals. 2) Changes of the late components of CEP's(N2) might represent derangements of the neural activity of the descending reticular formation in brainstem. 4. A close correlation was found between CEP's and rCBF changes, which suggested being a threshold relationship. In conclusion, it is assumed that the detection of CEP's in the cerebellum is a quite valuable prognostic tool to evaluate the neural activity of the non-specific reticular formation and specific somatosensory pathways in the acute intracranial hypertension. The prolongated latencies and suppressed amplitude in the CEP's indicate the presence of damages in brainstem multisynaptic pathways.
Adult ; Animals ; Blood Pressure ; Brain Stem ; Cats ; Cerebellum ; Electrodes ; Epidural Space ; Frontal Lobe ; Humans ; Hydrogen ; Intracranial Hypertension ; Intracranial Pressure* ; Pentobarbital ; Polyethylene ; Rabeprazole ; Respiration ; Reticular Formation ; Tracheostomy