Since the independent reports of Charcot and Marie and of Tooth, in 1886, interest in this familial disease has centered largely in the genetic and diagnostic aspects, and yet references in the literature to Surgical management are rare and inexact. Our experience with Charcot-Marie-Tooth disease in 4 patients operated at Seoul National University Hospital during the last 3 years is reported. Of 6 cases of Charcot-Marie-Tooth disease which have been studied, 4 patients have undergone surgery for the correction or amelioration of the crippling foot deformity. The authors' operation has consisted of triple arthrodesis, medial release, lengthening of the Achilles tendon, tendon transfer which was available and present evidence that properly designed surgical treatment can correct, prevent, or materially reduce disability of the deformed feet. Of the 4 cases reported here, all have either been reviewed or the patients have been interviewed in the last year. On the basis of adequate follow-up observations, thesurgical procedure outlined have been shown to improve the crippled condition. In spite of progressive musele wasting, the operative treatment could prevent or ameliorate the deformity. Three cases of Charcot-Marie-Tooth disease were evaluated in a long-term follow-up after having undergone surgery to correct their foot deformity. Excellent results were achieved from three of them which were maintained at final follow-up, and one is being gollowed up.
Achilles Tendon ; Arthrodesis ; Charcot-Marie-Tooth Disease ; Congenital Abnormalities ; Follow-Up Studies ; Foot ; Foot Deformities ; Humans ; Seoul ; Tendon Transfer ; Tooth

We present a case of familial benign chronic pemphigus in 50-year-old male patient who had had recurrent oozing, macerated and eroded skin lesions on the groin and scrotum for 20 years. At first, we diagnosed his case as scrotal eczema and/or intertrigo, but after repeated recurrance, it was confirmed as above disease by characteristic histopathologic findings.
Cytochrome P-450 CYP1A1 ; Eczema ; Groin ; Humans ; Intertrigo ; Male ; Middle Aged ; Pemphigus, Benign Familial* ; Scrotum ; Skin

We present a case of generalized syringoma in a 12-year-old healthy girl. The patient has numerous skin colored or yellowish papules on the face, neck, anterior chest, axillae and abdomen. She has a family history of eyelid syringoma occuring in her mother and maternal grandmother. Diagnosis was confirmed by characteristic histopathologic findings.
Abdomen ; Axilla ; Child ; Diagnosis ; Eyelids ; Female ; Humans ; Mothers ; Neck ; Skin ; Syringoma* ; Thorax

No abstract available.

Transforming growth factor beta(TGF-p), initially identified in vlatelet extracts by virtue of its ability to confer anchorage of independent growth and a necplastic phenotype on mesenchymal cells, has subsequently been identified as a potent inh bit or of proliferation in most cells of epithelial origin. The family of TGF-p peptides is currenly onsisted of four subtypes (TGF-pl, p2, p3, p4). Tkiey are initially translated very larged are urssors of approximately 390 amino acids and produced by a wide variety of cell type. iricluding normal cells and tumor cells. TGF-ps promote deposition of extracellular matrix compcineits, facilitate remodeling events during embryonic development, and suppress immune ce 1 fimetion during the inflammatory process. Several nutaneous diseases are characterized typxcessive and progressive fibrosis of the dermis anil subcutaneous tissues. Prominent amon these disorders are progressive systemic sclerosi(PSS) and generalized morphea(GM), is well as the recently described syndrome of diffuse fasciitis eosinophilia(DF), also known; Shulmans syndrome. The hallmark of the pathologic alteration in these disorders is the excessive deposition of collagen and other connenctive iissues. Macromoleculse in the aerriis and/or the subcutaneous and fascial strutures often accompanied by variable degress of hronic inflammatory cell infiltrates. Now we have examined the expression of TGF-b1 mRNA using of northern blot hybridization with specific sequenced cDNA probe in the normal curltured fibroblasts, placental tissues, and fibrosarcorna derived tumor cell line(HT1080). We found that the size of TGF-b mRNA of each specimen was 2.5kb and theres no alteration of its quality.
Amino Acids ; Blotting, Northern ; Collagen ; Dermis ; DNA, Complementary ; Embryonic Development ; Extracellular Matrix ; Fasciitis ; Female ; Fibroblasts* ; Fibrosis ; Humans ; Peptides ; Phenotype ; Pregnancy ; RNA, Messenger* ; Subcutaneous Tissue ; Transforming Growth Factor beta* ; Transforming Growth Factors ; Virtues

No abstract available.
Administration, Oral* ; Animals ; Intestine, Large* ; Rats*

No abstract available.
Administration, Oral* ; Animals ; Intestine, Large* ; Rats*

Adenoid cystic carcinoma occurs most commonly in the salivary glands, and also has been recognized in numerous other locations. However, it is rarely found in the prostate. We report a case of adenoid cystic c arcinoma of the prostate in a 77-year-old man whose chief complaint was urinary retention. Digital rectal examination transrectal ultrasonography revealed a markedly enlarged prostated and PSA was 4.6ng/ml. The transurethral prostatectomy was performed and the histopathological diagnosis was the adenoid cystic carcinoma. We review the clinical and pathologic feature of this disease.
Adenoids* ; Aged ; Carcinoma, Adenoid Cystic* ; Diagnosis ; Digital Rectal Examination ; Humans ; Prostate ; Salivary Glands ; Transurethral Resection of Prostate ; Ultrasonography ; Urinary Retention

Dermatologist should be aware of the practical importance of proper selection of ski.n lesions for histologic examination, the correct methods for performing biopsy, and steps involved in tissue preparation. They should be acquainted with factors producing various artifacts and ways to prevent these changes. This study was undertaken to investigate the artifacts of 300 dermatopathologic tissue sections which prepared by 3 general hospitals in Seoul in 1983. The results were summarized as follows. [.The percentage of artifacts produced by selection of inapropriate lesion was 4. 0% artifacts during biopsy procedure was 18. 3% inadequate size and depth of specimen was 7. 3%, faulty embedding was 12. 0%, tissue tearing or loss was 49. 3% artifactal dermoepidermal separation was 3.0%, linear knife marking was 17.0% thick or thin section was 8. 3% tissue overlapping was 12. 0% uneven stiining or precipitation was 3. 7%, and foreign body contamination was 3, 7% The percentage of good specimen was 21, 7% diagnostic was 56. 3% not diagnostic but suggestive was 8.7% and reading impossible was 13. 3% 3. The major causes of reading impossible specimen which attributed to the procedures of dermatologist were 40.0% to the proccss of technician were 47.5% and to both were 12. 5%
Artifacts* ; Biopsy* ; Foreign Bodies ; Hospitals, General ; Seoul ; Skin*

OBJECTIVE: The purpose of this project was to suggest guidelines for management of Low grade Squamous Intraepithelial Lesion(LSIL) by evaluating natural course of LSIL of the uterine cervix. MATERIAL & METHODS: Among the women who visited Korea University Kuro Hospital from Jan. 1993 to Oct. 1998, One hundred fifty eight patients who were diagnosed as LSIL with colposcopy directed biopsy were followed up by colposcopy and/or cytology, HPV DNA test every 3 months. RESULT: In I58 patients who were diagnosed as LSIL, colposcopic examination confirmed progression to high grade intraepithelial lesion(HSIL) in 17(10.7%) patients, persistence of LSIL in 87(55%) patients, regression to normal in 54(34.2%) patients during the 3 year follow-up period. In prediction of LSIL subgroup, abnormal Pap test is 39%, 64%, 71% and abnormal HPV test is l6%, 29%, 65% in regression, persistent and progression group respectively. The shortest time of transition from minor lesion(LSIL) to high grade lesion was 12 months (the range, 12-51). CONCLUSION: Of the patients who were diagnosed as LSIL and monitored by colposcopy for 60 months, 34% had disease that regressed, 55% had persistent disease, and 11% had progressive disease. HPV DNA test(p=0.002) is more informative than pap test(p=0.567) in prediction of disease progression.
Biopsy ; Cervix Uteri ; Colposcopy ; Disease Progression ; DNA ; Female ; Follow-Up Studies ; Human Papillomavirus DNA Tests ; Humans ; Korea ; Natural History