No abstract available.
Niemann-Pick Disease, Type A*

The goals of this study were 1) to present pre-surgical naso-alveolar molding(PNAM) appliance for bilateral cleft lip and palate treatment and 2) To evaluate the effects of the PNAM appliance on the alveolar molding of the premaxilla and the lateral segments. Subjects consisted of 8 bilateral cleft lip and palate infants (7 males and 1 female, mean age at first visit = 61.6 days after birth) who were treated with PNAM appliances in Department of Orthodontics, Seoul National University Dental Hospital. Average alveolar cleft gap between the premaxilla and lateral segment was 8.09+/- 5.03mm and average duration of alveolar molding treatment was 8.8+/-3.1weeks. These patients' models were obtained at initial visit (T0) and after alveolar molding (T1). 20 linear and 14 angular variables were measured by using photometry and digital caliper. All statistical analyses were performed by Microsoft Excel 97 program. Paired t-test was used to discriminate the effect of alveolar molding by PNAM appliance. 1. Closure of the alveolar cleft gap in bilateral cleft cases by molding therapy was completed successfully. 2. Alveolar molding inhibited outward growth of lateral segments and produced inward bending of lateral segments. 3. By bending the anterior part of the vomer, the premaxilla could be rotated and moved posteriorly via alveolar molding. Conclusion : This appliance can be applied to bilateral cleft lip and palate infants with satisfactory result before cheiloplasty.
Cleft Lip* ; Female ; Fungi* ; Humans ; Infant* ; Male ; Orthodontics ; Palate* ; Photometry ; Seoul ; Vomer

Granular cell tumor is a rare and usually benign tumor that occasionally involves the breast. The physical examination, the mammographic and ultrasonographic findings and the pathologic findings are often suggestive of carcinoma. We report here a rare case of granular cell tumor of the breast that mimicked carcinoma on the mammography, ultrasonography and MR imaging.
Breast* ; Granular Cell Tumor* ; Magnetic Resonance Imaging ; Mammography ; Physical Examination ; Ultrasonography

No abstract available.
Leukemia, Myelogenous, Chronic, BCR-ABL Positive*

PURPOSE: Voice change after thyroidectomy has generally been the result of damage to the recurrent or superior laryngeal nerve. But many patients complain voice alteration without laryngeal nerve injury after thyroidectomy. The purpose of this study is to investigate whether strap muscle division results in any subjective or objective functional sequelae in voice, through long-term follow-up prospectively. METHODS: Twenty-two female patients who had undergone thyroid surgery between July 1998 and December 1999, were studied. The patients who were planned for neck dissection, who had benign laryngeal disease or vocal cord paresis, and whose vocal cord paresis were developed after thyroid surgery, were excluded from this study. Twelve patients had undergone thyroidectomy via retraction of strap muscle and ten patients had undergone thyroidectomy via cutting of strap muscle. For evaluation of voice, questionnaires for changes of voice, acoustics (fundamental frequency, jitter, shimmer, signal to noise ratio, noise to harmonic ratio, voice range), and aerodynamic (maximal phonation time) analyses were done. RESULTS: The subjective voice symptoms after thyroidectomy were disturbances of high pitch, singing, loud voice, and easy fatigue at phonation. There were no significant differences in voice parameters on acoustic and aerodynamic analyses between the strap muscle retraction group and the cutting group through long-term follow-up. CONCLUSION: We conclude that strap muscle division does not result in any subjective or objective functional problems in voice. We suggest that surgical division and reconstruction of these muscles should be employed routinely when operating on large, toxic or neoplastic glands.
Acoustics ; Fatigue ; Female ; Follow-Up Studies ; Humans ; Laryngeal Diseases ; Laryngeal Nerve Injuries ; Laryngeal Nerves ; Muscles ; Neck Dissection ; Noise ; Phonation ; Prospective Studies ; Signal-To-Noise Ratio ; Singing ; Thyroid Gland ; Thyroidectomy* ; Vocal Cord Paralysis ; Voice*

BACKGROUND AND OBJECTIVES: The type of neck dissection performed for head and neck cancers depends on the experience of the surgeon and the status of the neck metastasis. The purpose of this study was to quantify the extent of lymphadenectomy achieved according to the various modifications of neck dissection based on microscopic pathologic analysis and to analyze the difference in the number of lymph nodes between the types of neck dissection. MATERIALS AND METHODS: Charts and pathologic finding of patients who underwent neck dissection from June 1994 to October 1999 were reviewed. Patients who received selective neck dissection or preoperative radiotherapy to the neck were excluded. The samples were 65 necks and were divided into four groups based on Medina's classification: radical neck dissection (group 1), Type I modified radical neck dissection (group 2), Type II modified radical neck dissection (group 3), and Type III modified neck dissection (group 4). The number of lymph nodes was counted by a pathologic microscopic examination for each region of specimens. The intergroup difference was analyzed by a one-way between-group analysis of variance (ANOVA). RESULTS: The mean number of dissected lymph nodes per specimen was 43.6 in group 1, 38.8 in both groups of 2 and 3, and 30.3 in group 4. Group 4 was significantly different from group 1 (p<0.05). Especially, the number of dissected lymph nodes from level II, III, IV of group 4 was significantly different from those of group 1 (p<0.05). There was no significant difference between other groups. CONCLUSIONS: The number of dissected lymph nodes decreases as the number of preserved non-lymphatic tissue structures increases. Level II, III, IV are less completely operated regions in functional neck dissection.
Classification ; Head ; Humans ; Lymph Node Excision ; Lymph Nodes* ; Neck Dissection* ; Neck* ; Neoplasm Metastasis ; Radiotherapy

BACKGROUND AND OBJECTIVES: An accurate, preoperative assessment of tumor extent and lymph node involvement is necessary to plan and tailor therapy for patients with head and neck cancer. Metabolic imaging with tluorine-18-fluorodeoxyglucose (FDG) is a good method to detect primary cancers in the head and neck and to assess the involvement of lymph nodes, but it is not widely available because of high cost of positron emission tomography (PET). Recently, an alternative method for using FDG was developed: the coincidence detection PET (CoDe PET) using a gamma camera. The aim of this study was to evaluate the clinical utility of FDG CoDe PET using a gamma camera in patients with head and neck cancer. MATERIALS AND METHODS: Thirty FDG CoDe PET studies were performed in 7 patients before therapy and 19 patients after therapy with various head and neck cancers (Age : 25- 79 years, mean age : 50+/-13 years, 18 men, 8 women). All patients had fasted for 6 to 12 hours and were injected 1 1 l to 370 MRq of F-18-FDG 1 hour before imaging. With the exception of the physiological FDG uptake, all visually detectable focal FDC uptake in the primary cancer site or in the neck was considered positive. FDG CoDe PET studies were correlated with CT/MRI. The standard procedure for detecting the presence of disease was the combinations of repeated MRIs, 3 months of follow-up clinical evaluation and the result of a needle aspiration cytology or biopsy. RESULTS: FDG CoDe PET had a detcction rate that was comparable to that of CT/MRI in the pre-therapy group. However, in the post-therapy group, FDG CoDe PET could differentiate residual/recurrence of tumor from radiation change more accurately than could MRI. But, it had a less accurate detection rate for cervical metastases because of asymmetric neck muscle uptake. CONCLUSION: FDG CoDe PET is a sensitive and cost-effective method to detect primary tumor and lymph node involvement in primary head and neck cancers. It is also useful in differentiating residual tumor or tumor recurrence from post-therapy changes in patients with head and neck cancers.
Biopsy ; Electrons* ; Follow-Up Studies ; Gamma Cameras ; Head and Neck Neoplasms* ; Head* ; Humans ; Lymph Nodes ; Magnetic Resonance Imaging ; Male ; Neck ; Neck Muscles ; Needles ; Neoplasm Metastasis ; Neoplasm, Residual ; Positron-Emission Tomography* ; Recurrence

The term "amyloidosis" is a disease characterized by deposition of altered proteins in the tissue. The amyloid deposit occurs always in the extracellular matrix and presents a fibrillary conformation. Primary localized tonsillar amyloidosis is a very rare disease of unknown cause that occurs in the absence of systemic involvement of amyloidosis or associated chronic inflammatory diseases. We present a case of primary localized amyloidosis in the left tonsil. Histopathologic examination of the mass revealed findings consistent with amyloidosis, which were stained with Congo Red and apple green birefringence under the polarized light fluorescence microscopy. An extensive workup for systemic involvement of amyloidosis was negative and the patient underwent tonsillectomy. The postoperative recovery was uneventful. At present, the patient has no evidence of disease or recurrence.
Amyloidosis* ; Birefringence ; Congo Red ; Extracellular Matrix ; Humans ; Microscopy, Fluorescence ; Palatine Tonsil* ; Plaque, Amyloid ; Rare Diseases ; Recurrence ; Tonsillectomy

The human papillomavirus (HPV)-18 E7 (E7) oncoprotein is a major transforming protein that is thought to be involved in the development of cervical cancer. It is well-known that E7 stimulates tumour development by inactivating pRb. However, this alone cannot explain the various characteristics acquired by HPV infection. Therefore, we examined other molecules that could help explain the acquired cancer properties during E7-induced cancer development. Using the yeast two-hybrid (Y2H) method, we found that the Elk-1 factor, which is crucial for cell proliferation, invasion, cell survival, anti-apoptotic activity, and cancer development, binds to the E7. By determining which part of E7 binds to which domain of Elk-1 using the Y2H method, it was found that CR2 and CR3 of the E7 and parts 1–206, including the ETS-DNA domain of Elk-1, interact with each other. As a result of their interaction, the transcriptional activity of Elk-1 was increased, thereby increasing the expression of target genes EGR-1, c-fos, and E2F. Additionally, the colony forming assay revealed that overexpression of Elk-1 and E7 promotes C33A cell proliferation. We expect that the discovery of a novel E7 function as an Elk-1 activator could help explain whether the E7 has novel oncogenic activities in addition to p53 inactivation. We also expect that it will offer new methods for developing improved strategies for cervical cancer treatment.

Klippel-Trenaunay-Weber syndrome is a congenital vascular disorder consisted of a variety of vascular malformations, enlargement of the involved limb, and varicose veins. We report a case of Klippel-Trenaunay-Weber syndrome presenting portal hypertension and varices bleeding caused by hypoplasia of the portal vein. Portal hypertension was caused by portal vein hypoplasia associated with Klippel-Trenaunay-Weber syndrome. There were three episodes of variceal bleeding, and hemostasis were achieved by endoscopic band ligation, Sugiura operation, and splenic artery embolization respectively. Although successful hemostasis was achieved, an additional procedures to reduce portal hypertension were needed to prevent repeated episodes of variceal bleeding.
Adult ; English Abstract ; Esophageal and Gastric Varices/*complications ; Gastrointestinal Hemorrhage/*etiology ; Humans ; Hypertension, Portal/complications ; Klippel-Trenaunay-Weber Syndrome/*complications ; Male ; Portal Vein/abnormalities