We report two cases of angiokeratoma circumcriptum. One of them was 20 year- old male, who had multiple dark erythematous 0.5 cm to 2 cm sized keratotic papules and nodules surrounding irregular shaped erythema scattered or grouped on the right lower leg. The other case was 36 year-old male, who had Q. 7 cm sized dark erythematous papules and 2 cm sized dark brown colored nodule with central ulceration on the medial part of the left upper thigh, The two patients had skin lesions at birth. Histopathologic finding disclosed markedly dilated vessels containing many red blood cells mainly in the papillary dermis, which in some, were surrounded by elongated rete ridge. Cavernous hemangioma like lesion was present in the lower dermis and subcutaneous fat in case 2.
Adult ; Angiokeratoma* ; Dermis ; Erythema ; Erythrocytes ; Hemangioma, Cavernous ; Humans ; Leg ; Male ; Parturition ; Skin ; Subcutaneous Fat ; Thigh ; Ulcer

Bone destruction is primarily mediated by osteoclastic bone resorption, and cancer cells stimulate the formation and activation of osteoclasts next to metastatic foci. Accumulating evidences indicate that receptor activator of NF-kB ligand (RANKL) is the ultimate extracellular mediator that stimulates osteoclast differentiation into mature osteoclasts. In contrast, osteoprotegerin (OPG) inhibits osteoclast development. In order to elucidate a mechanism for cancer-induced osteoclastogenesis, cells from a human breast cancer line, MDA-MB-231, were directly co-cultured with ST2, MC3T3-E1, or with primary mouse calvarial cells. Osteoclast-like cells and tartarate resistant acid phosphatase (TRAP) activities were then quantitated. We examined these cell lines and samples from breast cancer by RT-PCR for the expressions of OPG and RANKL mRNA. Compared to controls, co-culture of MDA-MB-231 cells with stromal or osteoblastic cells induced an increase in number of osteoclasts and TRAP activities. MDA-MB-231 cells alone or breast cancer samples did not express RANKL mRNA. However, co-culture of these cancer cells with stromal or osteoblastic cells induced RANKL mRNA expression and decreased OPG mRNA expression. These experiments demonstrate that direct interactions between breast cancer and stromal or osteoblastic cells induce osteoclastogenesis in vitro through modulating RANKL expression.
3T3 Cells ; Acid Phosphatase/metabolism ; Animals ; Bone Neoplasms/*metabolism/*secondary ; Breast Neoplasms/*pathology ; Carrier Proteins/*biosynthesis ; Cell Differentiation ; Cell Line, Tumor ; Cells, Cultured ; Coculture ; Culture Media, Conditioned/pharmacology ; Glycoproteins/*biosynthesis ; Human ; Isoenzymes/metabolism ; Male ; Membrane Glycoproteins/*biosynthesis ; Mice ; Mice, Inbred C57BL ; Neoplasm Metastasis ; Osteoblasts/metabolism ; Osteoclasts/metabolism ; Protein Binding ; RNA, Messenger/metabolism ; Receptors, Cytoplasmic and Nuclear/*biosynthesis ; Reverse Transcriptase Polymerase Chain Reaction ; Time Factors

Recently identified second messenger molecules of signal transmission were derived from inositol phospholipid and several phospholipase C(PLC) isoenzyme were reconized. But the role of PLC in embryonal organ development, differentiation and growth is not described, yet. And, Siah gene of mouse is expressed at a higher level in a restricted number of sites during development. So, it is suggested that the Siah gene plays a significant role in development of vertebrates.Therefore, this study was aimed to investigate the role of PLC isoenzymes and Siah gene during murine fetal development by use of immunehistochemistry. The results are as follow: 1. PLC-beta1 and PLC-gammal are expressed apparently at early fetal stage, PLC-betal is observed focally, whereas PLC-gamma1 is diffusely expressed. 2. PLC-delta1 is not expressed during early and mid murine fetal age but strongly expressed in placental tissue. 3. PLC-beta1 is observed mainly in neural tissues including brain at mid fetal stage. PLC-gamma1 is expressed in entire organs, especially in liver tissue, and then their expression is reduced after mid stage. 4. Siah gene product is significantly expressed in olfactory epithelium, retina, neuron of brain and choroid plexus from mid fetal stage to late gestation. These results suggest that PLC-betal play a important role in central neural development and PLC-gamma1 in entire orgaans, especially in liver development. The strong expression of PLC-deltal in placenta suggest its involvement in early development, indirectly. Siah gene product expressed at a higher levels in olfactory epithelium, retina, neuron and choroid plexus, so Siah gene may play a important role in these organ developments.
Animals ; Brain ; Choroid Plexus ; Fetal Development* ; Gestational Age ; Immunohistochemistry* ; Inositol ; Isoenzymes ; Liver ; Mice ; Neurons ; Olfactory Mucosa ; Phospholipases* ; Placenta ; Pregnancy ; Retina ; Second Messenger Systems ; Type C Phospholipases*

A 26-year-old woman had an ovarian Sertoli-Leydig cell tumor (SLCT) associated with an elevated level of serum alpha-fetoprotein (AFP). The tumor had a heterologous element of intestinal-type mucinous epithelium, retiform and intermediately differentiated tubules of the Sertoli cells, and AFP-producing Leydig cells. AFP was demonstrated within the Leydig cells by an immunohistochemical technique. After surgery, the serum AFP level of the patient fell to the normal range. The present case is the first documented case of AFP producing a SLCT of the ovary reported in Korea.
Adult ; alpha-Fetoproteins* ; Epithelium ; Female ; Humans ; Korea ; Leydig Cells ; Male ; Mucins ; Ovary* ; Reference Values ; Sertoli Cells ; Sertoli-Leydig Cell Tumor*

BACKGROUND: Epidural "top-up" has been known to enhance spinal anesthesia in combined spinal-epidural anesthesia. Saline and local anesthetics were reported to have a volume effect when infused as epidural "top-up". In cesarean deliveries where high sensory block has been achieved using a combined spinal-epidural technique, we evaluated the effects of epidural "top-up" on the underlying spinal block. METHODS: Sixty-six parturients were allocated randomly into group C (control, n = 21), S (saline, n = 21), or B (bupivacaine, n = 24). Ten minutes after they received 8 mg of 0.5% hyperbaric bupivacaine intrathecally, nothing, 10 ml saline, or 10 ml of 0.25% bupivacaine were infused, respectively. The sensory level at 10 min, the maximal level and the time to reach it, and degrees of motor block and muscle relaxation were compared. We also investigated intraoperative side effects and postoperative findings in the PACU. RESULTS: There were significant changes in sensory level after epidural top-ups in the group S and B, but no differences in maximal height of sensory block and degree of muscle relaxation among the groups. Intraoperative pain was complained in fewer patients in the group B. Times to sensory and motor recovery were longer in patients of the group B. CONCLUSIONS: Eight milligrams of hyperbaric bupivacaine using a combined spinal-epidural technique was not sufficient for cesarean section. Epidural saline top-up showed volume effect, which didn't improve the spinal block. Epidural "top-up" of 0.25% 10 ml bupivacaine enhanced the spinal block and sustained the block postoperatively.
Anesthesia ; Anesthesia, Epidural* ; Anesthesia, Spinal ; Anesthetics, Local ; Bupivacaine ; Cesarean Section* ; Female ; Humans ; Muscle Relaxation ; Pregnancy

Cystic adventitial disease of the popliteal artery (CADPA) is a rare but a well-known cause of intermittent claudication, especially in young patients. The etiology of the disease is still controversial. Diagnosis starts with thorough history taking, physical examination, and radiography. A 44-year-old man presented with intermittent left calf claudication that had begun 9 months earlier (; the symptom-free interval was about 500 m). 3D-CT and CT-angiography revealed an oval cystic mass that compressed the popliteal artery, causing severe stenosis. Surgery was performed; the affected segment of the artery was successfully excised and replaced with an autogenous saphenous vein graft. Follow-up is on going. No cyst recurrence has so far been detected either clinically or by duplex-sonography during the 6-month postoperative period. The graft was patent and the patient was completely symptom free. Severe claudication in young patients, possibly without significant vascular risk factors, should prompt the clinical suspicion of CADPA.
Adult ; Arteries ; Constriction, Pathologic ; Diagnosis ; Follow-Up Studies ; Humans ; Intermittent Claudication ; Physical Examination ; Popliteal Artery* ; Postoperative Period ; Radiography ; Recurrence ; Risk Factors ; Saphenous Vein ; Transplants

When a Korean child is living in close contact with a person with active pulmonary tuberculosis, the risk of his acquiring the disease is great. In seeking an effective means of reducing this risk to the chi1d, isoniazid was given as secondary chemoprophylaxis to 233 tuberculin-positive children under 5 years of age who were clinically free of disease but were living in close contact with a household member with active tuberculosis. The condition of these children after 9 to 12 months of chemoprophylaxis was compared with a similar control group of 216 children living under exact1y comparable circumstances. An analysis of tile results of this study indicates that secondary chemoprophylaxis with isoniazid is effective and worthwhile for children under 4 years of age who are under constant exposure to tuberculosis. That there was such a protection is indicated by the fact that (1) isoniazid markedly reduced the size of the tuberculin reaction and that (2) only 2 children receiving isoniazid developed active tuberculosis (0.8%) as against the 8 controls who did (3.70%). The faithful cooperation of parents in administering prophylactic isoniazid to small children is often difficult to obtain. This difficulty is suggested by the fact that in the case of the 2 children in the isoniazid test group who developed active tuberculosis, the actual drug intake had been irregularly given and amounted to less than half the prescribed dose.
Chemoprevention ; Child ; Family Characteristics* ; Humans ; Isoniazid ; Parents ; Tuberculin ; Tuberculosis ; Tuberculosis, Pulmonary*

BACKGROUND: A spectrophotometric approach to minimum inhibitory concentration (MIC) determination for filamentous fungi may provide an objective and rapid MIC reading, and quantify the hyphal growth of molds. In this study, we evaluated two spectrophotometric broth microdilution methods (SBM) to determine amphotericin B and itraconazole MICs for Aspergillus species isolated from clinical specimens. METHODS: A total of 80 clinical isolates (20 A. fumigatus, 20 A. flavus, 18 A. niger, 20 A. terreus, and 2 A. nidulans) were tested for amphotericin B and itraconazole susceptibility by the broth microdilution method. The MIC endpoint was calculated by the spectrophotometer with microplate reader (SBM-Spec method) or colorimetric XTT (tetrazolium dye) method (SBM-XTT method). The results of the SBM method were compared with those of NCCLS M38-A broth microdilution method. RESULTS: The MICs of amphotericin B by the NCCLS M38-A method ranged from 0.125 to 8 g/mL, and those of itraconazole ranged from 0.25 to 2micrograms/mL. The agreement of SBM-Spec and SBM-XTT methods within one dilution of the NCCLS M38 reference were 98.8% and 96.3% for the ampho-tericin B, and 98.8% and 100% for itraconazole, respectively. The agreements between SBM-Spec and SBM-XTT methods were 97.5% for amphotericin B and 98.8% for itraconazole. CONCLUSIONS: In antifungal susceptibility testing of Aspergillus species, the SBM method includ-ing SBM-Spec and SBM-XTT methods showed high levels of agreements with the NCCLS M38-A method. The SBM methods can be useful in the clinical laboratory.
Amphotericin B ; Aspergillus* ; Fungi ; Itraconazole ; Microbial Sensitivity Tests ; Niger

Insulinoma is the most important cause of hyperinsulinemic hypoglycemia. Insulinoma can be cured by surgical management but diagnosis and localization are difficult due to their small size and variable clinical manifestation. We studied clinical manifestation of the patients who were diagnosed as insulinoma or nesidioblastosis. METHODS: We studied clinical charts of 16 patients who underwent operation under the impression of insulinoma from 1990 to 1997. RESULTS: 1) Of the 16 patients, 13 had insulinoma and 3 had nesidioblastosis. 2) Neuroglycopenic symptoms were present in all cases. Among them, loss of consciousness or confusion were present in 12 cases (75%). Adrenergic symptoms were present in 9 cases (56%). Mean symptom duration until diagnosis was 39 months(range; 15days~10years). 3) 72 hr fasting test showed hypoglycemia in all cases. 4) Insulin/glucose ratio was above 0.3 except for two cases. 5) CT and MRI had low sensitivity (38%) and specificity (33%) as a preoperative tumor localization tool, and more invasive technique, i.e., selective angiography and transhepatic portal vein sampling, were necessary in most cases. CONCLUSION: Nsidioblastosis may be more prevalent than previously suspected. Diagnosis of insulinoma is not always easy and invasive diagnostic methods should be considered when the clinical suspicion is high, even if biochemical and noninvasive localization tools give negative results.
Angiography ; Asian Continental Ancestry Group ; Diagnosis ; Fasting ; Humans ; Hypoglycemia ; Insulinoma* ; Magnetic Resonance Imaging ; Nesidioblastosis* ; Portal Vein ; Sensitivity and Specificity ; Unconsciousness

The form and function of the craniofacial structure critically depend on genetic information. With recent advances in the molecular technology, genes that are important for normal growth and morphogenesis of the craniofacial skeleton are being rapidly uncovered, shaping up modern craniofacial biology. One of them is fibroblast growth factor receptor 2 (FGFR2). Specific point mutations in the FGFR2 gene have been linked to Apert syndrome, which is characterized by premature closure of cranial sutures and craniofacial anomalies as well as limb deformities. To study pathogenic mechanisms underlying craniosynostosis phenotype of Apert syndrome, we used a transgenic approach; an FGFR2 minigene construct containing an Apert mutation (a point mutation that substitute proline at the position 253 to arginine; P253R) was introduced into fertilized mouse germ cells by DNA microinjection. The injected cells were then allowed to develop into transgenic mice. We used a bone-specific promoter (a DNA fragment from the type I collagen gene) to confine the expression of mutant FGFR2 gene to the bone tissue, and asked whether expression of mutant FGFR2 in bone is sufficient to cause the craniosynostosis phenotype in mice. Initial characterization of these mice shows prematurely closed cranial sutures with facial deformities expected from Apert patients. We also demonstrate that the transgene produces mutant FGFR2 protein with increased functional activities. Having this useful mouse model, we now can ask questions regarding the role of FGFR2 in normal and abnormal development of cranial bones and sutures.
Acrocephalosyndactylia* ; Animals ; Arginine ; Biology ; Bone and Bones ; Collagen Type I ; Congenital Abnormalities ; Cranial Sutures* ; Craniosynostoses ; DNA ; Extremities ; Germ Cells ; Humans ; Mice ; Mice, Transgenic* ; Microinjections ; Morphogenesis ; Phenotype ; Point Mutation ; Proline ; Receptor, Fibroblast Growth Factor, Type 2 ; Skeleton ; Sutures ; Transgenes