We report a case of Hunter's syndrome in an 8-year-old boy, who presented with ivory-white colored papules and ridges on the left chest area, which were regarded as pathognomonic cutaneous markers for Hunter's syndrome. He also showed growth retardation, dear corneas, hepatosplenomegaly and fair intellect. The histopathological findings of papular lesions revealed loosely arranged collagen fibers with massive mutinous material which stained positively with alcian blue at both pH 2.0 and 0.5. On quantitation of glycosaminoglycans by hexuronic add assay in 24-hour urine, excessive excretion of creatinine was noted. To the best of our knowledge, this is the first case in Korea.
Alcian Blue ; Child ; Collagen ; Cornea ; Creatinine ; Glycosaminoglycans ; Humans ; Hydrogen-Ion Concentration ; Korea ; Male ; Mucopolysaccharidosis II* ; Thorax

The purpose of this study was to evaluate the effect of EDTA irrigant according to application time and temperature. 31 human mature extracted teeth with a single canal were sectioned with microtome in 3mm thickness and gained 62 samples of root canals. They were distributed randomly into 6 groups of 10 specimens each and control group of 2 specimens. Each specimen was prepared with GT rotary file (Dentsply, Maillefer Co., Swiss) and irrigated with 3 ml sodium hypochlorite every minute. Then smear layer was removed with EDTA solution (PULPDENT(R), PULPDENT Co., USA.) except two control specimens. Specimens of each group were irrigated with 17% EDTA. All specimens were split longitudinally and prepared for examination by scanning electron microscopy. A set of reference micrographs was used to award a debris score as follows: 0 = no smear layer, all tubules clean and open; 1 = no superficial smear layer, tubule openings visible, but some contain debris plug or soft tissue remnants; 2 = moderate smear layer, some tubules open and others closed; 3 = heavy smear layer, most/all tubule openings obscured. Results were evaluated with Kruskal-Wallis test to determine whether there was statistically significant difference among six groups. Pairs of groups were analyzed using the Student-Newman-Keuls Method and Mann-Whitney test. The results were as follows: 1. Control specimens showed heavy smear layer at the canal walls. 2. Among the groups applied with EDTA for 2 minutes, group 1 showed the heaviest smear layer, and there was statistically significant difference between group 1 and the other groups(p<0.05). 3. Among the groups applied with EDTA for 5 minutes, group 4 and group 6 showed smear layer, but there was no significant difference between them. 4. Among the groups applied with EDTA for the same temperature, group 1 showed heavier smear layer than group 4, and there was statistically significant difference(p<0.05). 5. Among the groups applied with EDTA for the same temperature, group 2 showed heavier smear layer than group 5 and group 3 showed heavier smear layer than group 6. But there was no statistically significant difference among them. From the results above, it could be concluded, EDTA solution is effective in removing of smear layer when it is applied for 5 minutes. If EDTA is applied for 2 minutes, it should be applied above room temperature.
Awards and Prizes ; Calcium Hydroxide ; Dental Pulp Cavity ; Edetic Acid ; Humans ; Microscopy, Electron, Scanning ; Smear Layer ; Sodium Hypochlorite ; Tooth

No abstract available.
Skin Manifestations* ; Skin*

Supravalvular aortic stenosis may be defined as an obstructive congenital deformity of the ascending aorta which originates just distal to the level of the origins of the coronary arteries. It may be localized or diffuse, and includes a wide spectrum of pathologic changes. A 16 years old school boy was admitted because of exertional dyspnea for 6 years. Clinical diagnosis of supravalvular and valvular aortic stenosis with bicuspid aortic valves and myocardial hypertrophy was made by echocardiography and angiocardiography. Surgical correction was performed successfully. We presented a case of supravalvular and valvular aortic stenosis with a review of literatures.
Adolescent ; Angiocardiography ; Aorta ; Aortic Stenosis, Supravalvular ; Aortic Valve ; Aortic Valve Stenosis* ; Bicuspid ; Congenital Abnormalities ; Coronary Vessels ; Diagnosis ; Dyspnea ; Echocardiography ; Humans ; Hypertrophy ; Male

No abstract available.
Carbon Dioxide* ; Carbon* ; Lasers, Gas*

Intramembranous tympanic membrane cholesterol granuloma (CG) occurs infrequently. Here, the authors report a case of CG in the tympanic membrane presenting as a blue eardrum in the right ear. In addition, a pinhole perforation noted in the anterosuperior area revealed a brown discharge. High-resolution temporal bone CT showed a bulging mass shadow in the middle ear and a soft tissue dense lesion that filled both the epitympanum and mastoid cavity. Tympanomastoidectomy was performed under general anesthesia. New bone formation was confirmed in the mastoid antrum and epitympanum, and the epitympanum was blocked by new bone. The tympanic membrane revealed a round, brownish mass with a glistening surface and a severely thickened pars tensa. We herein report this case and review pertinent medical literature.
Adult ; Cholesterol/*metabolism ; Granuloma, Foreign-Body/*diagnosis/radiography ; Humans ; Male ; Tympanic Membrane/*pathology/radiography

OBJECTIVE: The presence of the various cytokines in human peritoneal fluid has been evaluated incompletely. Changes in cytokine lefels may be related to activation of peritoneal macrophage and T-lymphocyte, development of endometriosis, and infertility. This study assesses peritoneal fluid levels of interleukin-6 (IL-6), interleukin-8 (IL-8), interleukin-10 (IL-10) and tumor necrosis factor-alpha (TNF-alpha) in infertile women with endometriosis and normal women without endometriosis. Design : Prospective and case-control study in university hospital. MATERIALS AND METHODS: Cytokine levels in peritoneal fluid obtained during laparotomy or laparoscopy from 21 patients in infertile patients in infertile patients with endometriosis and 24 controls undergoing laparotomy or laparoscopy with no evidence of pelvic endometriosis were determined by enzymelinked immunosorbent assay. RESULTS: The mean levels of interleukin-6 in infertile patients with endometriosis and controls were 72.7+/-23.7 pg/ml and 18.5+/-9.7 pg/ml respectively (p=0.02). Similarly, the mean levels of interleukin-8 in infertile patients with endometriosis was significantly higher than that of controls (445.0+/-89.6, vs 45.1+/-48.4, p=0.04). The mean concentration of interleukin-10 in infertile patients with endometriosis was significantly lower than that of controls (1.09+/-0.04 vs 2.19+/-0.03, p=0.03). The level of tumor necrosis factor-alpha was not significantly different between the two study groups. CONCLUSIONS: Increased IL-6 and IL-8 and decreased IL-10 levels in the peritoneal fluid may be related to pathogenesis in the endometriosis and infertility, suggesting that partially contribute to the disturbed immune regulation observed in infertili women with endometriosis.
Ascitic Fluid* ; Case-Control Studies ; Cytokines ; Endometriosis* ; Female ; Humans ; Infertility ; Interleukin-10 ; Interleukin-6 ; Interleukin-8 ; Laparoscopy ; Laparotomy ; Macrophages, Peritoneal ; Prospective Studies ; T-Lymphocytes ; Tumor Necrosis Factor-alpha

The purpose of this study is to investigate the response of human pulp cell on Portland cement mixed with beta-glycerophosphate. To investigate the effect of beta-glycerophosphate and/or dexamethasone on human pulp cell, ALP activity on various concentration of beta-glycerophosphate and dexamethasone was measured and mineral nodule of human pulp cell was stained with Alizarin red S. MTS assay and ALP activity of human pulp cell on Portland cement mixed with various concentration of beta-glycerophosphate (10 mM, 100mM, 1M) was measured and the specimens were examined under SEM. Addition of beta-glycerophosphate or dexamethasone alone had no effect however, the addition of 5 mM beta-glycerophosphate and 100 nM dexamethasone had the largest increasement in ALP activity. There was no toxicity in all samples and the data showed that Portland cement mixed with 10 mM beta-glycerophosphate had more increase in ALP activity compared with control. In conclusion, Portland cement mixed with beta-glycerophosphate has no toxicity and promotes differentiation and mineralization of pulp cell compared with additive-free Portland cement. This implicated that application of Portland cement mixed with beta-glycerophosphate might form more reparative dentin and in turn it would bring direct pulp capping to success.
Anthraquinones ; Dental Pulp Capping ; Dentin ; Dexamethasone ; Glycerophosphates ; Humans

To compare the regenerative effect of direct end-to-end suture, nerve graft and sutureless fibrin glue repair in the rat sciatic model, forty rats were divided into 4 groups: normal control group(NC-G, n=6), direct end-to-end suture group(DS-G, n=12), nerve graft group(NG-G, n=10) and sutureless fibrin glue group(FG-G, n=12). One half of each group except NC-G was sacrificed 4 weeks later and the other half was sacrificed 14 weeks later. The repair site was assessed by nerve conduction studies and quantitative morphometry. Regeneration index (RI) was defined as a total number of remyelinated axons in one quarter of 3X5 inch sized microphotograph. The comparison among groups was analyzed statistically by Kruskal-Wallis 1-way ANOVA and Mann-Whitney test. Nerve conduction study done 14 weeks later revealed that latency was 1.7ms+/-0.18(DS-G), 2.4ms+/-0.35(NG-G), 2.4ms+/-0.17(FG-G) and amplitude was 20.1mV+/-2.89(DS-G), 12.0mV+/-2.47(NG-G), 13.7mV+/-2.95(FG-G). RI was 281+/-37.5(DS-G), 227+/-14.4(NG-G) and 217+/-22.2(FG-G). There was a statistical difference in the latency, amplitude and RI between three groups(p<0.05), but there was no statistical difference in the latency, amplitude and RI between FG-G and NG-G(p>0.05). Regenerative effect of fibrin glue is not different from that of nerve graft, therefore fibrin glue repair can facilitate inaccessible nerve repairs in skull base or spinal surgery.
Animals ; Axons ; Fibrin Tissue Adhesive* ; Fibrin* ; Neural Conduction ; Rats* ; Regeneration ; Sciatic Nerve* ; Skull Base ; Sutures ; Transplants

Cavernous hemangioma seems to most frequently arise in the posterior portion of the external auditory canal. However, they rarely occur in the tympanic membrane. A 49-year-old male patient was referred for evaluation of right-sided pulsatile tinnitus that he'd experienced for the previous 2 years. Temporal bone computerized tomography showed an isolated soft tissue mass just lateral to the tympanic membrane. There was no evidence of bony erosion or middle ear invasion. The patient underwent excision of the mass using a postauricular approach. The mass was removed en bloc and the defect of the tympanic membrane was repaired by tympanoplasty type I. There was no recurrence after 1 year of follow-up.
Caves ; Ear Canal ; Ear, Middle ; Follow-Up Studies ; Hemangioma, Cavernous ; Humans ; Male ; Middle Aged ; Recurrence ; Temporal Bone ; Tinnitus ; Tympanic Membrane ; Tympanoplasty