PURPOSE: The purpose of this study was to analyze the MR findings of childhood adrenoleukodystrophy regarding the extent of white matter degeneration and the pattern of enhancement, and to correlate these findings with clinical manifestations. MATERIALS AND METHODS: Six male patients (5-13 years old;mean age, 8.7 years) of biochemically confirmed adrenoleukodystrophy were included in this study. We evaluated the extent of white matter degeneration which was shown as high signal intensity on T2-weighted image, and the pattern of enhancement. Also we correlated the MR findings with clinical manifestations of symptoms and the results of evoked potential studies. RESULTS:The most common sites of predominant involvement were occipital deep white matter, optic radiation, and splenium of corpus callosum (n = 4, 66.7%). The other sites of predominant involvement were frontal deep white matter (n = 1, 16.7%) and pyramidal tract with cerebellum (n = 1, 16.7%). After contrast enhancement, there was peripheral rim enhancement surrounding the most severely affected areas in five cases (83. 3%). These findings were consistent with clinical symptoms in five cases (83.3%), but the results of evoked potential studies were consistent with clinical symptoms in only two cases (33.3%). CONCLUSION:In childhood adrenoleukodystrophy, MRI can clearly demonstrate specific tracts of involvement and these findings were well correlated with clinical manifestations.
Adrenoleukodystrophy* ; Cerebellum ; Corpus Callosum ; Evoked Potentials ; Humans ; Magnetic Resonance Imaging* ; Male ; Pyramidal Tracts

The goal of cleft lip surgery is to reconstruct normal shape of the lip. To acomplish this goal, various operative method were contrived and concept of the method decided the shape of reconstructed lips. According to the operative result, some operative methods were disappeared or have been developed with a little modifiation. Traditional Millard's rotation-advancement method for unilateral cleft lip patients is largely accepted and developed as proper method for acquiring these functional and anatomic purposes. As a trial for this development, Mulliken add some modifications. He uses exaggerated high rotation incision and it lengthens into midcolumella without backcut. Also he dosen't steal from alar base or lateral lip for vertical height. C-flap is used to lengthen the affected columella and upper lip. The isolated orbicularis oris muscle is coaptated each other for more functional result. With supraperichondral dissection of alar cartilage and transpositioning of caudal septum, he performs synchronous repair of cleft lips, nose and sometimes alveolus. It is still debated when is most suitable age for surgical correction of nasal deformity of cleft lip patients. Done at the time of primary lip repair, there are both some apprehension and inducement. The former are based on technical difficulties due to shortage and fragility of neonatal tissue and possibility of progressive deformities with growing because of iatrogenic injuries to the alar cartilages. But te latter is rationalized because early reposition of deformed nasal cartilage in proper position would induce more natural growth of nasal structures. Some long-term follow up reports reveal the early operation innocent of any growth deterioration. Mulliken treats his cleft lip patient for separated lip and nasal deformities with single operation, and does gingiovoperiosteal alveoloplasty at the same time if necessary. He uses Latham appliance from 4 to 6 week after birth in case of severly collapsed lateral alveolar segment or wide alvolar gap, and perform the definitive opertation at the age of 4 to 6 months. I use lip adhension method to correct more than 10 mm alveolar gap without severe collapse of lateral alveolar segment, but if lateral segment was severely collapsed and away from alvolar arch, I apply the Latham appliance somewhat modificate from original type, which has a metal ring fastened at the front limb of appliance for rubber banding to coaptate easily. I have experienced repair of cleft lip by Mulliken's concept with some modification of my own to 44 cases of unilateral cleft lip patients and conclude that it was very flexible method. Doing with synchonous repair of cleft lip nose, we could get harmonious lip and nose with symmetric nostril sill, cupid's bow and red line. Columella was lengthened primarily. Normal growth of nose was anticipated by anatomic repositioning of alar septal cartilage.
Alveoloplasty ; Cartilage ; Cleft Lip* ; Congenital Abnormalities ; Extremities ; Follow-Up Studies ; Humans ; Lip ; Nasal Cartilages ; Nose ; Parturition ; Rubber

Human membranous nephropathy (MN) is morphologically characterized by subepithelial immune complex deposits and progressive thickening of glomerular basement membranes (GBM). Studies have suggested that the enhanced secretion of classical and novel type IV collagen chains in MN contributes to spike formation and the novel type IV collagen chain is particularly related to thickening of GBM. It is unclear whether the increased accumulation of extracellular matrix (ECM) proteins in GBM is due to the increased mRNA expression for type IV collagen in glomerular visceral epithelial cells (GECs). To answer this question, we analyzed seven renal biopsies of patients with idiopathic MN using in situ hybridization. In MN, the number of GECs expressing mRNA for alpha1(IV) collagen was 2.82+/-1.80/glomerular cross section (gcs), and the number expressing mRNA for alpha4(IV) collagen was 8.42+/-2.85/gcs. The number of GECs expressing mRNA for alpha4(IV) collagen was significantly larger than that of alpha1(IV) collagen mRNA. The expression of mRNA for these ECM proteins in normal controls was negligible. These results suggest that subepithelial immune complexes stimulate the gene expression of alpha1(IV) collagen and alpha4(IV) collagen in glomerular GECs which, in turn, increase the secretion of ECM proteins and contribute to the thickening of GBM in MN.
Antigen-Antibody Complex ; Biopsy ; Collagen ; Collagen Type IV* ; Extracellular Matrix ; Gene Expression ; Glomerular Basement Membrane ; Glomerulonephritis, Membranous* ; Humans* ; In Situ Hybridization ; Podocytes ; RNA, Messenger*

Hurthle cell tumors are an infrequent neoplasm of the thyroid gland in adults. Hurthle cell tumors represent 4. 5% to 10% of all primary thyroid epithelial neoplasms in the foreign literature. It has been known as Hurthle cell tumor since Ewing was the first to use this term in 1928. Tumor occurring in an infant was described by Symmers(1941) and Morrow(1945). The authors experienced a case of congenital Hurthle cell tumor of the thyroid gland in a 2 months old boy. He was admitted to the pediatric surgical department because of a growing mass in the neck since birth. Ultrasonogram showed a huge lobulated homogenous solid mass with medium level echogenicity in the region of the thyroid gland. Subtotal thyroidectomy of right lobe and total thyroidectomy of left lobe were done. The specimen measures 2x3x1.5 cm and 7x3x3 cm, respectively. It was an encapsulated, yellow gray firm and solid mass. The cut surface was smooth, glistening and homogenous. Microscopicully, the tumor was composed of tightly packed regular follicles lined by polyhedral, cuboidal, large cells with a granular acidophilic cytoplasms. The nuclei are vesicular, usually only a little bit larger than those of normal thyroid cells.
Infant ; Adult ; Male ; Female ; Humans

Lymphomatoid papulosis is an enigmatic disease entity which is clinically benign and histologically malignant. Although sporadic cases have been reported, we could not find any comprehensive report on the combined clinical and histologic features of lymphomatoid papulosis in the literature. Perhaps the most controversial aspect of lymphomatoid papulosis is its pathogenesis and categorization as a benign versus a malignant entity. To date, there are no reports on p53 and bcl-2 protein expression in lymphomatoid papulosis. We analysed the clinico-pathological findings of 18 cases with lymphomatoid papulosis during the 10 year period from 1984 to 1995 and examined the prevalence of immunoreactivity for CD30(DAKO, Ber-H2), p53(DAKO, DO-7), and bcl-2(DAKO, 124) using an immunohistochemical(ABC) method. The results obtained are summarized as follows. 1) Age distribution ranged from 20 to 65, with a mean age of 45 years and a sex distribution which showed a male predominence(8:1). The lesions were located on the trunk and extremities(8cases), extremities (7cases), and trunk(3 cases). The morphology of the lesions were papules or plaques(12 cases), and nodules(6 cases). 2) Histopathologic types were classified into 3 types: type A(4 cases), type B(8 cases) and mixed type (6 cases). 3) Positive immunoreactivity for CD30 was seen in 17%(3 of 18cases): type A(2 of 3) and mixed type(1 of 3). 4) The positive immunoreactivity for p53 and bcl-2 was observed in 29%(5 of 18) and 11%(2 of 18), respectively. 5) Cases showing positive immunoreactivity for P53 were type A(1 of 5), type B(1 of 5), and mixed type(3 of 5). 6) Cases showing positive immunoreactivity for bcl-2 were mixed type(2 of 2). One case developed into Ki-1 lymphoma. These results support the idea that lymphomatoid papulosis and Ki-1 lymphoma represent a continuum. The role of p53 gene mutation and bcl-2 activation in the development of lymphomatoid papulosis is currently unknown. But, our results suggest that p53 gene mutation and bcl-2 activation are not a critical step in the development of lymphomatoid papulosis. Further studies are needed to elucidate the role of p53 gene mutation and bcl-2 activation in the development and progression of lymphomatoid papulosis.
Male ; Humans ; Genes, p53

Tamoxifen is a nonsteroidal triphenylethylene delivative that has been widely used in the treatment of breast cancer. tamoxifen is indicated for the adjuvant treatment of breast cancer in women following total mastectomy or segmental mastectomy, breast irradiation and chemotherapy. tamoxifen has been shown to have significant benificial effect in the treatment of breast cancer patient as hormonal therapy. However ,there is mounting evidence that tamoxifen may affect other hormon sensitive organs, including the uterus and ovaries. An increased risk of endometrial polyps, endometrial hyperplasia and adenocarcinoma has been reported in tamoxifen treated women. We have met cases of endometrial polyp and endocervical polyp which were associated with tamoxifen use, after modified radical mastectomy for infilterating ductal carcinoma of breast . So we report these cases with the brief review of literatures.
Adenocarcinoma ; Breast ; Breast Neoplasms ; Carcinoma, Ductal ; Drug Therapy ; Endometrial Hyperplasia ; Female ; Humans ; Mastectomy, Modified Radical ; Mastectomy, Segmental ; Mastectomy, Simple ; Ovary ; Polyps* ; Tamoxifen* ; Uterus

OBJECTIVES: E-cadherin is a transmembrane protein that is one of the key players involved in cell to cell adhesion. Loss of E-cadherin expression is suggested to promote tumor invasion and distant metastasis in tumor development. Recently, it has been proposed E-cadherin function requires its linkage to the cytoskeleton through catenins. So defects in catenins may cause defective E-cadherin function and promote tumor invasion. We intend to evaluate the expression of E-cadherin and alpha-, beta-, gamma- catenin in tissues of human endometrial carcinoma to analyze the patterns of cell adhesion molecules' expression in endometrial carcinoma and to investigate the relationship between status of cell adhesion molecules and various clinicopathological factors. MATERIALS AND METHODS: The present study investigated the immunohistochemical expression of E-cadherin and alpha-, beta-, gamma- catenin in 33 paraffin embedded formalin fixed tissues of endometrial carcinomas. RESULTS: Aberrant E-cadherin, alpha-, beta-, gamma- catenin expression was observed in 33.3(11 of 33), 27.3(9 of 33), 18.2 (6 of 33), and 51.5(17 of 33) % of the specimens, respectively. Statistically significant correlation was found between aberrant expression of E-cadherin and lymph node metastasis and cell types other than endometrioid adenocarcinoma. Aberrant pattern of gamma- catenin expression also correlated with deep myometrial invasion. But alpha-, beta- catenin expression were not correlated with any clinicopathological parameters. Using Kaplan-Meier curves, abnormal expression of E-cadherin correlated closely with poor survival (p<0.05). CONCLUSION: We revealed aberrant expression of these cell adhesion molecules in part of patients with endometrial carcinoma. Aberrant expression of E-cadherin was correlated with lymph node metastasis and cell types other than endometrioid adenocarcinoma and aberrant expression of gamma-catenin was related with deep myometrial invasion.
Cadherins* ; Carcinoma, Endometrioid ; Catenins* ; Cell Adhesion ; Cell Adhesion Molecules ; Cytoskeleton ; Endometrial Neoplasms* ; Female ; Formaldehyde ; gamma Catenin ; Humans ; Lymph Nodes ; Neoplasm Metastasis ; Paraffin

OBJECTIVE: This study was performed to evaluate the effects of mutiple indications and age on the likelihood of finding cervical lesion in large loop excision of transformation zone(LLETZ). METHOD: We reviewed the medical records of 471 evaluable LLETZ of cervix for cervical neoplasia at the Yonsei University College of Medicine, from April 1996 to August l998. The indications for the LLETZ were grouped as being for treatment(biopsy-proven disease)(indication A), significant discrepancy among cytologic and histologic results(indication B), unsatisfactory colposcopic finding(transformation zone not fully visualized)(indication C), possible microinva- sion(indication D), and combinations of the above. RESULTS: The prevalence rates of cervical lesion in performed LLETZ for indication A alone, for indication B alone, for a combination of indication A and C, for a combination B and C, and for indication D alone were 85.7%, 56.5%, 82.3%, 42.6%, 95%, respectively. The data were then reanalyzed to determine the likelihood of finding high-grade disease on LLETZ specimen. Overall, those with preoperative high-grade cytologic or histologic characteristics were much more likely to have high grade disease(78.1%) than were those with preoperative low-grade cytologic or histologic characteristics(27.2%)(p<0.001). The influence of age on the likelihood of identifying diasease on the LLETZ is undertermined. CONCLUSION: Neither age nor the preoperative grade of disease are good discriminators of the likelihood that disease will be found on a LLETZ specimen, However, patients with preoperative high-grade disease are much more likely than those with preoperative low-grade disease to have high-grade dysplasia or invasive cancer on LLETZ.
Cervix Uteri ; Female ; Humans ; Medical Records ; Prevalence

No abstract available.
Brain* ; Hemangioendothelioma* ; Lung* ; Mediastinum*

Alterations in the expression of genes that control the cell cycle may be of critical importance in tumorigenesis and malignant transformation. The major regulatory events leading to cell proliferation occur in Gl phase of cell cycle, and the deregulated expression of Gl cyclins is related to oncogenesis. Cyclins Dl and E play important roles in the progression of cell through Gl phase of the cell cycle. Amplification and/or overexpression of the cyclin Dl gene and aberrant expression of cyclin E has been described in various forms of human cancer. However, the role of cyclins Dl and E in cervical cancer has been poorly defined. In this study, we examined the expression of cyclins Dl and E by Northern blot technique and the status of human papil- lomavirus(HPV) type 16 and 18 by polymerase chain reaction in 25 cases of cervical carcinoma to explore the relationship between cyclins Dl and E and cervical cancer. We found cyclin Dl expression showed down-regulated expression in cervical cancer but cyclin E expression was increased in cancer group. Other clinicopathological prognostic factors were not correlated with cyclins D1 and E expression. Further study based on larger numbers of cases with correlation of cyclins D1 and E status and survival data will be needed to elucidate the use of cyclin expressions as prognostic factor.
Blotting, Northern ; Carcinogenesis ; Cell Cycle ; Cell Proliferation ; Cyclin E ; Cyclins* ; Humans ; Polymerase Chain Reaction ; Uterine Cervical Neoplasms