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

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

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*

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

Generalized distinct hyperpigrnented macules developed at 2. 7 years after starting photochemotherapy(psoralen and UVA) in a 66-year-old psoriatic male patient. The histopathologic feature of this macule was characterized as a lentiginous p roliferation. The significance and long term course of PUVA lentigo are still unknown. So, we will further monitor this patient continually for melanocytic dysplasias, including melanoma.
Aged ; Humans ; Lentigo* ; Lichen Planus ; Male ; Melanoma ; Mouth Mucosa

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

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