No abstract available.
De Lange Syndrome*

The Kniest syndrome is characterized by disproportionate dwarfism and Kyphoscoliosis which may be associated with flat facies with prominent eyes, cleft palate, hearing loss, myopia and limited joint motion. The skeletal abnormalities are recognizable at birth with shortening and deformity of the extremities and stiff joints. Marked lumbar lordosis and kyphoscoliosis develop in childhood, resulting in disproportionate shortening of the trunk. We experienced a case of kniest syndrome, confirmed by clinical features, radiological features, and histological examination of cartilage. A brief review of the related literature is presented.
Animals ; Cartilage ; Cleft Palate ; Congenital Abnormalities ; Dwarfism ; Extremities ; Facies ; Hearing Loss ; Joints ; Lordosis ; Myopia ; Parturition

The leukemia of infancy, characterized by a high leukocyte count, massive organomegaly, CNS leukemia at diagnosis, high chromosomal abnormalities and immature phenotype. This is reflected in its poor prognosis. Isolated lesions of the facial nerve have been reported in leukemia patients, but appear to be rare in infancy. We report a case of unilateral facial nerve paralysis occurred in 4-months-old male who had acute lymphoblastic leukemia with review of literature.
Chromosome Aberrations ; Diagnosis ; Facial Nerve* ; Humans ; Infant* ; Leukemia ; Leukocyte Count ; Male ; Paralysis* ; Phenotype ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis

The frequent occurrence of hematuria in children necessitates diagnostic evaluation. Hematuria may occur with either renal or lower urinary tract disease. Clarification of the etiology of hematuria, whether microscopic or gross, is facilited by localizing the site of bleeding to the kidney (glomerular) or the lower urinary tract (non-glomerular). The mean cellular volume (MCV) of urinary red blood cells (RBCs) of pediatric patients with glomerular (group I; n=77) and non-glomerular (group II; n=34) hematuria was determined using Coulter Counter Model S plus IV. We found that re blood cells of glomerular origin had a smaller volume than non-glomerular cells(73.79 9.75 m3 vs 83.55 3.77 m3, p<0.001). If an urinary MCV equal to 80.56 m3 was taken as the cut-off value between glomerular and non-glomerular hematuria, a correct assessment of the site of bleeding was made in 89 (80%) of the 111 patients studied (sensitivity 76%, specificity 88%). The ratio of the urinary erythrocyte MCV to that in blood(Umcv/Bmcv) was compared with the diagnosis. If an Umcv/Bmcv ratio equal to 0.95 was taken as the cut-off value between glomerular and non-glomerular hematuria, a correct assessment of the site of bleeding was made in 93 (83%) of the 111 patients studied (sensitivity 79.6%, specificity 94%). Coulter counter analysis of urine provides a simple noninvasive and objective aid to the diagnosis of hematurai. This test, when used early in the management of pediatric patients with hematuria, may help to avoid invasive investigations.
Blood Cells ; Child ; Diagnosis ; Erythrocytes* ; Hematuria* ; Hemorrhage ; Humans ; Kidney ; Sensitivity and Specificity ; Urinary Tract ; Urologic Diseases

No abstract available.
Mediastinal Emphysema*

Benzoyl peroxide has been known to have bacteriostatic activity against Corynebacterium acnes and to have comedolytic action in patients with acne vulgaris. The present study was undertaken to evaluate the therapeutic effect of Renoxal, a 5% benzoyl peroxide lotion, in patients with acne vulgaris. A total of 35 patients entered this study at the Department of Dermatology, Seoul National University Hospital during the five months period from March to July, 1978. All patients were instructed to apply Benoxal on their affected sites once or twice daily; and the number of lesions on three fixed areas were counted before and every week for four weeks after the treatrnent. Two female patients stopped the application due to developrnent of contact dermatitis to Benoxal The results observed in the 33 patients were as follows: 1. The percentage decrease in total number of lesions in four weeks after treatment was 51.1% 2. Closed and open comedones were gradually eliminated; and their numbers were decreased in four weeks after treatment by 45. 8% and 55. 3% respectively. The papules were decreased. by 59. 1 @2 after the first week of treatment, but showed. a transient lag around tbe second week and then marked 61. 5% in four weeks after treatment. 3. Observed side effects included burning sensation,(3 cases), tightness (3 cases), itching sensation (2 cases) and scaling (1 case). All were tolerable without any specific measure or discontinuance of the application. The authors concluded througb this experiment that Benoxal (5% benzoyl peroxide lotion) is a very effective local therapeutic agent in the treatment of acne vulgaris patients.
Acne Vulgaris* ; Benzoyl Peroxide ; Burns ; Dermatitis, Contact ; Dermatology ; Female ; Humans ; Propionibacterium acnes ; Pruritus ; Sensation ; Seoul ; Thiram

No abstract available.
Acne Vulgaris*

Recent immunological investigations have demonstrated that the patients with psoriasis have various humoral and cellular immune abnormalities, such as increased serum IgG, IgE and secretory IgA, anti-IgC factor in psoriatic lesions, in peripheral blood lymphocytes and in serum, rhumatoid-like factors in IgA and IgG classes of immunoglobulins, antinuclear antibodies (ANA; reacting with the basal cell nuclei of uninvolved skin., anti-stratum corneum antibody and complements in psoriatic scales, immuoglobulin and complement bearing polymorphonuclear leucocytes in the Muro microabscess. These abnormal findings are enough to suggest an autoimmune mechanism in the pathogenesis of psoriasis. Several investigators have also reported the results of T cell enumeration in the peripheral blood in psoriatic patients. However, the results are not in general agreement,. Thee present study was undertaken to clarify any abnormality in the proportion of T cells in the peripheral blood in psoriatic patients. Forty-one patients with active psoriasis registered at the Department of Dermatology, Seoul National University Hospital entered this study from May, 1979 through April, 1980. Twelve healthy medical and paramedical personel the comprised the control group. Active and total T cells were enumerated by the method of E-rosetting technique, and the results were as follows. 1, in normal controls, the active and total T celIs identified as E rosetteforming cells accounted for 61.6+7.4% and 68.1+8.9% of the total lymphocyte population, respectively. 2. In patients with psoriasis, significant decrease of active T cells (54. 2,+11.0%,p<0.005) and total T cells (62.2+11.2%, p<0.05) was observed. More profound reduction of T cells was noted in patients with wide spread psoriasis than those with limited extent.
Antibodies, Antinuclear ; Cell Nucleus ; Complement System Proteins ; Dermatology ; Humans ; Immunoglobulin A ; Immunoglobulin A, Secretory ; Immunoglobulin E ; Immunoglobulin G ; Immunoglobulins ; Lymphocytes ; Psoriasis* ; Research Personnel ; Seoul ; Skin ; T-Lymphocytes* ; Weights and Measures

Condylomata acuminata are benign tumors which are mostly venereally transmitted. Common sites were coronal sulcus, perisnal area and prepuce. Among 28 patients, 21 acuminate lesions and 10 papular lesions were found. Twenty eight human genital warts in Korean were analysed by Southern blot hybridization. Sequences related to HPV6/11 are found in 89.3%(25/28) of the condylomata. HPV16 DNA was not found at sll. Subtype of HPV was determined by the restriction pattern of DNA cleaved with PstI restriction enzyme in 7 cases. Six cases of HPV6a and one case of HPV6c are found. The above results suggest that most of condylomata acuminata are caused by HPV6 and HPV11 in Korea.
Blotting, Southern ; Condylomata Acuminata* ; DNA* ; Humans* ; Korea

BACKGROUND: Fosinopril is a new phosphorous containing angiotensin converting enzyme inhibitor. To assess the antihypertensive efficacy and safety of fosinopril, the clinical trial was done in patients with mild to moderate hypertension. METHODS: In 30 patients with mild to moderate essential hypertension, we administered fosinopril 10-20mg once daily for 10 weeks and checked their blood pressure every 2 weeks. RESULTS: The blood pressure decreased from 158+/-12.3/103.4+/-4.2mmHg to 139+/-7.5/88.4+/-5.9mmHg at the end of treatment(p<0.05). Heart rate did not change significantly during therapeutic period. Of 30 patients, the efficacy of fosinopril therapy disclosed 25 patients(83.7%) with normal diastolic pressure of more than 10mmHg decline of diastolic blood pressure. But two patients had no effects and three patients were not followed up. The adverse reactions due to fosinopril were reported in 3 patients(10%) with dry cough, 2 patients with palpitation(6.7%) and 1 patient with weakness(3.3%), but there were no patients who discontinued fosinopril due to adverse effect. CONCLUSION: Fosinopril has an excellent antihypertensive effect at low dosage as a first line antihypertensive agent or as a substituting agent for other antihypertensives in patients with mild to moderate essential hypertension.
Antihypertensive Agents ; Blood Pressure ; Cough ; Fosinopril* ; Heart Rate ; Humans ; Hypertension ; Peptidyl-Dipeptidase A