Frontometaphyseal dysplasia is an uncommon genetic syndrome affecting bone and connective tissue. This condition is characterized by the prominence of supraorbital ridges, hyperostosis of the skull, mandibular hypoplasia and malocclusion with antegonial notching, underdeveloped paranasal flaring of the long bone, and a widened iliac wing. Frontometaphyseal dysplasia has an X-linked dominant trait. Clinical manifestations are more severe in males and have extreme variability in females. We present an 11-year-old boy who had frontometaphyseal dysplasia with disfigured face due to bilaterally urinary tract malformation and chronic urinary tract infection. To improve facial appearance, his prominent supraorbital ridges were contoured by ostectomy and burring through bicoronal incision. Urologic operation was performed simultaneously. Histologically, resected bony specimen revealed bony tissue with normal trabecular pattern suggesting exostotic bone. The patient healed without any postoperative complications and he and his parents were satisfied with his final facial morphology.
Child ; Connective Tissue ; Female ; Humans ; Hyperostosis ; Male ; Malocclusion ; Parents ; Postoperative Complications ; Skull ; Urinary Tract ; Urinary Tract Infections

Restoration of anteroposterior projection of the zygomatic body and facial width are key elements in treatment of complex zygomatic fractures. Traditionally, it has been understood that for most fractures of the zygoma, three-point fixation of the zygomaticofrontal suture, inferior orbital rim, and zygomaticomaxillary buttress generally produces exact structural restoration. Recently, the importance of accurate reduction of the zygomatic arch through coronal incision has been favored in complex zygomatic fractures. Though coronal incision has the advantages of accurate reduction and fixation as a result of extensive exposure of the fractured area, this procedure also has many disadvantages including injury to the facial nerve, paresthesia, alopecia, scar formation, longer operating time, protracted hospitalization, etc. As well, there have been no objective data to prove the fact that four-point fixation through coronal incision is superior to traditional three-point fixation. From May, 1994 to December, 1998, the authors treated 45 patients by traditional three-point (n=20) and coronal four-point fixation (n=25) with random sampling. To assess the difference between the two methods the authors measured the axial angle of the zygoma, the axial angle of the zygomatic arch, and the degree of zygomatic arch inclination on submentovertex X-ray, and then analyzed the measurements by the paired T-test(p < 0.05). As a result, the axial angle of the zygoma and zygomatic arch assessed by anteroposterior projection, as well as the facial width showed no statistical difference between the two groups, respectively(p = 0.26, p = 0.18). Mean while, the degree of zygomatic arch inclination representing the local contour of the fractured zygomatic arch was statistically significant between the two groups(p < 0.05). Thus, the traditional three-point fixation method may be widely acceptable in treatment of complex zygomatic fractures with anterior three-point or zygomatic arch comminution.
Alopecia ; Cicatrix ; Facial Nerve ; Hospitalization ; Humans ; Orbit ; Paresthesia ; Sutures ; Zygoma ; Zygomatic Fractures

Basal cell carcinomas(BCCs) are usually treated with ablative proccdures, but a nonsurgical treatment would conserve tissue and obtain a better cosmetic out come as well as treat patients who refuse surgery or those who are not good surgical candidates. Because of inadequate drug penetration, low cure rates, high recurence rates, and adverse reactions, such as contact dermatitis, topical 5-fluorouracil(5-FU) is unsaitble to treat nodular BCCs. But, int,ralesional 5-FU has been reported to be effective for nodular BCCs. The efficacy and safety of intralesional injection of 5-FU have been evaluated for treatment of BCCs. The results revealed that intralesional injection of 5-FU seems to be effective in the treatment of various premalignant and malignant skin lesions as well as BCCs without side effects.
Carcinoma, Basal Cell* ; Dermatitis, Contact ; Fluorouracil* ; Humans ; Injections, Intralesional* ; Skin

By light and scanning electron microscopy, the morphologies of Korean pubic louse(Phthirus pubis) were described and measured. Especially, electron microscopic structures of palpus, tuft organ, sensilla basiconicum and palpal lobe were reviewed in detail. The variations were obserbed in the number of dorsal setae and the setae of membranous processes of abdomen. And also, the importance of public health and epidemiological aspects was discussed.
Abdomen ; Microscopy, Electron, Scanning* ; Phthiraptera* ; Phthirus* ; Public Health ; Sensilla

No abstract available.
Skull Base* ; Skull*

No abstract available.
Facial Nerve* ; Paralysis* ; Temporal Bone*

Paget's disease is classified, according to location, as mammary or extramammary disease. Extramammary Paget's disease especially in the vulva is extremely rare. Several hundred cases have been described worldwide. Patients with Paget's disease of the vulva are usually white and postmenopausal. The cause of which remains elusive. A case of vulva Paget's disease is presented with brief review of literature.
Humans ; Paget Disease, Extramammary ; Vulva

BACKGROUND/AIMS: Hepatitis C virus(HCV) was known to most common etiologic agent of chronic liver disease in United states and Japan. Although hepatitis B virus(HBV) was well known to be a its major etiologic agent in Korea, it has been showed that HCV and HBV are associated with liver cirrhosis and hepatocellular carcinoma as major causative agent of chronic liver disease. Interferon alpha therapy is generally accepted as effective single agent for chronic hepatitis or to decrease the chronicity of acute hepatitis C. So, we evaluated the efficacy of interferon alpha in hepatitis C. METHODS: 46 patients who were positive for anti-HCV antibody and HCV RNA were included in this study. Liver biopsy was per formed on all patients and all of them were tested as negative for serum HBsAg, anti Hbe. Patients were divided into 2 groups . 30 patients received interferon therapy(treated group) and 16 patients received no therapy(untreated group). We compared the change of liver function test and HCV RNA before and after therapy between two groups. Treated group was subdivided into 5 groups according to response to interferon therapy '. Non-response, partial response, breakthrough, relapse and sustained response. RESULTS: 1) The mean age and sex distribution were 49.9 year old, male 19, female 11 in treated group and 48.7 years, male 12, female 4 in untreated group. 2) The number of patients with acute hepatitis, chronic persistent hepatitis, chronic active hepatitis and liver cirrhosis were 1, 2, 23, 4 in treated group and 0, 1, 12, 3 in untreated group, respectively. 3) The mean follow up period was 1.7 year and 2.3 years in treated and untreated group, respectively. 4) The activity of serum ALT before and after therapy were 195+ 134.6 IU/L, 87.4+ 40.5 IU/L and 186.7+ 106.4 IU/L, 157+ 87.1 IU/L in treated and untreated group, respectively. Serum ALT after therapy in treated group was significantly lower than untreated group(P<0.01). 5) The number of patients for patterns of reponse in treated group was non-response 5, partial response 8, breakthrough 1, relapse 4, sustained response 12 and there was no difference in age among them(P>0.05). 6) The case of negative conversion for HCV RNA in treated group was 12, but there was no case in untreated group. 7) Sex distribution of sustained response was 6(31% ) of 19 male, 6(54.5%) of 11 female and 12 patients(40.0%)(1 of 1 patients with acute hepatitis, 1 of 2 chronic persistent hepatitis, 10 of 23 chronic active heaptitis) included in sustained reponse, but any patients with liver cirrhosis had response. 8) Mean total dose and duration of interferon therapy was non-response 10353.6 million unit(MU)/5.8month(M), partial response 20025.06MU/6.4 M, breakthrough 36000.0MU/5.0M, relapse 11700.0MU/3.3M, sustained response 28100.0MU/6.6M, respectively. 9) 3 of 7 patients who were followed up over 1 year in sustained response and mean time to the relapse was 2.2 years. CONCLUSION: Our results showed that interferon alpha therapy is effective in patients with hepatitis C and further study and attempts should be performed to augument the efficacy of interferon alpha for the treatment of hepatitis C.
Biopsy ; Carcinoma, Hepatocellular ; Female ; Follow-Up Studies ; Hepatitis B ; Hepatitis B Surface Antigens ; Hepatitis C* ; Hepatitis* ; Hepatitis, Chronic ; Humans ; Interferon-alpha* ; Interferons* ; Japan ; Korea ; Liver ; Liver Cirrhosis ; Liver Diseases ; Liver Function Tests ; Male ; Recurrence ; RNA ; Sex Distribution ; United States

No abstract available.
Diagnosis* ; Epilepsy*

No abstract available.
Facial Bones* ; Humans