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

No abstract available.

A 19-month old male patient developed mutiple, firm, plum-colored, finger tip to chestnut sized nodules on the left cheek, right preauricular area, both elbow joints, and right wrist. On physical examination, a right cervical lymph node was palpable. Histology from one of the nodules revealed a diffuse infiltration of atypical, hyperchromatic, polymorphous lymphocytes in the dermis and subrutaneous fat. Bone marow aspiration, chest X-ray, ultrasonogram, and computerized tomogram were performed revealing bone marrow and lymph node involvement. The patient was treated with a POG @8617 schedule. Skin lesions markedly improved after one cycle regimen. We present a case of diffuse, ponrly differentiated, malignant lymphocytic lymphoma in the early childhood.
Appointments and Schedules ; Bone Marrow ; Cheek ; Dermis ; Elbow Joint ; Fingers ; Humans ; Infant ; Leukemia, Lymphocytic, Chronic, B-Cell ; Lymph Nodes ; Lymphocytes ; Lymphoma* ; Male ; Physical Examination ; Skin ; Thorax ; Ultrasonography ; Wrist ; Prunus domestica

No abstract available.
Phenylketonurias*

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.
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

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

Mass screening of cardiomegaly by chest X-ray in 144,021 (male 55,491, female 88,530) students of primary, middle and high school (6~17 years of age) in Seoul was performed and electrocardiograms of 217 cases of cardiomegaly were studied. The results were as follows; 1) Cardiomegaly (cardiothoracic ratio over 0.5) was seen in 0.19% of each sex and it was higher in middle and high school ages (12~17 years) than primary school ages (6~11 years). 2) Incidences of abnormal electrocardiogram in cardiomegaly were 59.5% in male and 54.7% of female students. Major abnormal electrocardiograms in cardiomegaly were right ventricular hypertrophy (24.8%), left ventricular hypertrophy (11.6%), biventricular hypertrophy(4.8%), complete right bundle branch block (7.7%), incomplete right bundle branch block (8.7%), first degree A-V block (5.8%) and premature beat (4.3%). 3) Left ventficular hypertrophy was seen most frequently in high school ages (15~17 years) and decreased with decreasing age. Right ventricular hypertropy was seen most frequently in primary school ages (9~11 years) and decreased with increasing age. Biventricular hypertrophy was seen most frequently in primary school ages (6~8 years) and decreased with increasing age. 4) Complete and incomplete right vundle branch block were seen commonly in 12~17 years of age and first degree A-V block in 9~11 years of age.
Female ; Male ; Humans ; Incidence