BACKGROUND AND OBJECTIVES: Idiopathic restrictive cardiomyopathy is a very rare and poorly recognized disease in children. This study is performed to describe the clinical course and to define potential predictors of outcome. MATERIAL AND METHOD: We reviewed the medical records and diagnostic studies of 11 consecutive patients during the period from Jan.1991 to Aug. 2000. RESULTS: The age at diagnosis was 1.2-13 years (median 7 years) and the duration of follow up was 3-90 months (median 3.6 years). All except one were symptomatic (dyspnea in ten, chest pain in four). The chest pain was associated with significant ST depression on both resting and exercise ECG, suggesting myocardial ischemia. Two had complete heart block as either initial or terminal event. Cardiac catheterization was done in nine ( mean pulmonary arterial wedge pressure 23+/-6mmHg, systolic pulmonary arterial pressure 47+/-14mmHg, mean right atrial pressure 11+/-9mmHg). Echocardiographic dimensional ratio of left atrium and aorta (LA/Ao) was 2.41+/-0.58. Mitral E/A inflow ratio was 2.72+/-1.42, E wave deceleration time was 93.6+/-44.2ms. During follow up, six died. The 2 year and 5 year cumulative survival rates were 54.5% and 18.8% respectively. The predictor for nonsurvivor were pulmonary venous congestion and LA/Ao >2.5(p<0.05). Verapamil was tried in 6 cases without favorable effect in all. CONCLUSION: Considerable numbers of restrictive cardiomyopathy have myocardial ischemia associated with ST depression and chest pain. The patients with pulmonary venous congestion and severe left atrial enlargement (LA/Ao>2.5) were at risk for death, requiring prompt definitive treatment such as cardiac transplantation.
Aorta ; Arterial Pressure ; Atrial Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Cardiomyopathy, Restrictive* ; Chest Pain ; Child* ; Deceleration ; Depression ; Diagnosis ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart Atria ; Heart Block ; Heart Transplantation ; Humans ; Hyperemia ; Medical Records ; Myocardial Ischemia ; Pulmonary Wedge Pressure ; Survival Rate ; Verapamil

Unstable Colles' fractures have an inherent capacity for loss of reduction or shortening, or both. The istability can be recognized by the presence of much comminution, severe dorsal angulation(20 degrees or more), or extensive intra-articular involvement. It is difficult to align the fracture fragments and to maintain the reduction. In twenty-nine patients with unstable Colles' fracture from Jan. 1982 to Mar. 1984, several invasive methods were used. The results were as follows: 1. Among 147 patients with Colles' fractures 29 patients(19.7%) had unstable fractures. 2. Colles' fracture was occurred more frequently in women(77%) and in 6th and 7th decades(56%). Unstable fractures showed no significant age and sex related propencity. 3. Mechanism of injury caused unstable Colles' fractures were fall on the outstretched hand(25 patients), traffic accident(3 patients) and crushing injury(1 patient). Open fractures were shown in 4 patients among the unstable fractures. 4. Most of the unstable fractures were comprised of type VII and VIII(49%) but type I and II fractures with marked comminution or displacement also showed instability. 5. Complications were such as, redisplacement of the fractures, temporary neuropathy of the median nerve, compartment syndrome, Sudeck's atrophy, and shoulder-hand syndrome. 6. Overall assessments showed excellent in 4 patients, good in 16 patients, fair in 7 patients and poor in 2 patients. 7. Overall results were mainly correlated with initial severity of the injury and anatomical restoration of the fractures.
Atrophy ; Colles' Fracture ; Compartment Syndromes ; Fractures, Open ; Humans ; Median Nerve ; Radius ; Reflex Sympathetic Dystrophy

Although we made a comparision of fingertip dermatoglyphic patterns between R7 patients with atopic dermatitis(Femals: 54, Male: 35) and 73 their families without atopic dermatitis, no significant results were obtained. So, we compared secondly our results of 2 groups with those of two thousand nonatopic, normal Koreans, as control. Results are as follows: ] In comparison with control, a significant increase in the ulnar loop pattern was detected in families(p<0, ()5), not atopic dermatitis. p. No significant differ ence between right hands and left hands in all three g f OUpS. In atopic dermatitis and control groups, 1 st and 4 th digits showed mostly whorl pattern, and 3 rd and 5 th digits had more ulnar loop pattern than others. Rut families had mostly ulnar loop pattern at all digits except 4 th digit, wl..orl pattern. 4. Each of atopic dermatitis, family and control group had 13, 79% (whor1: 8. 05% ulnar loop: 5 75%), 15. 49p; (whorl: 4. 23%, ulnar loop: 11. 27%), 15. 78%(whorl: 8, 52% ulnar loop: 7. 1 %, arch: 0. 05%), of same dermatoglyphic pattern on all ten digits. Especially, ulnar loop pattern in fanilies was detected significantly higher than other two groups(p<0. 05). Average incidences of bilateral symmetry of pattern type between right and left digits were 78.57%, 74. 67% and 78.74% respectively in atopic dermatitis, family and control group.
Dermatitis, Atopic* ; Dermatoglyphics* ; Hand ; Humans ; Incidence ; Male

We report one case of hair follicle nevus, benign highly differentiating appendageal skin tumor of hair follicle. It arises from pluripotential cells that have formed during life. This patient is a 40-year-old woman who had a localized brownish plaque on the leht cheek for three years. Histologic study revealed many small but mostly mature hair follicles consistent with hair follicle nevus.A Case of Cutaneous Periarteritis Nodosa
Adult ; Cheek ; Female ; Hair Follicle* ; Hair* ; Humans ; Nevus* ; Polyarteritis Nodosa ; Skin

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

A 25-year-old female was seen because of rnultiple, asymptornatic papular eruptions on the vulvar area since 1 year ago. She had been suffered from palmar hyperhidrosis for 3 years. A skin biopsy specimen from the vulval lesion revealed characteristic findings of syringoma.
Adult ; Biopsy ; Female ; Humans ; Hyperhidrosis ; Skin ; Syringoma*

A 72 years old man had an 1 x 1cm sized, solitary glomus tumor on the right knee joint and had suffered from sharp, episodic pain and tenderness, radiating to the right thigh and the buttock for about 20 years. Simple surgical excision was performed for removal of the tumor mass and the subjective symtoms. There is no recurrence until now.
Aged ; Buttocks ; Glomus Tumor* ; Humans ; Knee Joint ; Recurrence ; Thigh

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

Improvement of lichen planus was achieved by 11 weeks of daily oral treatment with griseofulvin. The patient was 59-year-old male, has had hypertension and diabetes mellitus for 9 months, and history of various drug intake to these diseses for 6 months. Four months before first visit, symptome of lichen planus had developed suddenly. Because of treatment failure of oral antihistamine and topical steroid for 6 weeks, we began to use griseofulvin. Praspective studies are needed to better assess the affectiveness of griseafulvin in the treatment of lichen planus.
Diabetes Mellitus ; Griseofulvin* ; Humans ; Hypertension ; Lichen Planus* ; Lichens* ; Male ; Middle Aged ; Treatment Failure