BACKGROUND: Neurofibroma, the hallmark of neurofibromatosis, is a cutaneous or subcutaneous lesion, with a variable clinical presentation. Histologically, neurofibroma consists of proliferation of nerve derived cellular elements, together with an abundant, collagenous extracellular matrix. Specifically, neurofibroma has been shown to contain 30-50% collagen in its matrix. Objective 5. METHODS: We examined the expression of extracellular matrix genes (collagen, fibronectin, laminin), TGF-b mRNA and Ha-ras oncogene mRNA by using Northern and slot-blot hybridization and immunoperoxidase stains. Result: In Northern blot analysis, Ha-ras and TGF-b genes revealed respectively, 8.8kb and 2. 5kb sized mRNA transcripts in neurofibroma. These parameters were normal in the control. The expression of these genes were 1.9, 2.0 fold increased in neurofibroma. In slot-blot analysis, expression of type I collagen showed fibronectin genes to be 2,401+210, 540+43, respectively, in neurofibroma. So there were 3.7 fold, 2.1 fold, differences respectively, compared to the normal control. However, there were no significant changes of type IV collagen and laminin Bl mRNA levels between neurofibroma and normal skin tissues. Irnmunoperoxidase staining by rnonoclonal anti type IV collagen antibody in neurofibroma showed type IV collagen to be diffusely and weakly stained in tissue. On staining by monoclonal anti-laminin antibody, laminin was stained in a matrix and around vessels. CONCLUSION: The increased expression of extracellular matrix genes may suggest that there is a subpopulation of fibroic cells in neurofibroma which are stimulated by TGF-b. Ha-ras genes which might have accumulated with the differentiation of neural tissue may be related to the pathogenesis of neurofibroma tissue formation. Further studies are needed to determine whether the other factors are related to the pathogenesis of neurofibroma.
Blotting, Northern ; Collagen ; Collagen Type I ; Collagen Type IV ; Coloring Agents ; Extracellular Matrix* ; Fibronectins ; Genes, ras* ; Laminin ; Neurofibroma ; Neurofibromatoses* ; RNA, Messenger ; Skin

In evaluation of some area within the craniofacial complex posteroanterior cephalcgram together with lateral cephalogram is used for assessment of the exact existence and posture by grasping 3-dimensional concept. The usages of posteroanterior cephalogram in orthodontics are following; 1. The use during diagnosis and treatment planning 1) Assessment of the area and amount of facial asymmetry. 2) Assessment of therapeutic position for diagnosis and treatment 2. The Assessment of success in orthodontic mechanics If we use posteroanterior cephalogram together with lateral cephalogram adequately and simultaneously we can establish the exact diagnosis and appropriate treatment planning.
Diagnosis ; Facial Asymmetry ; Hand Strength ; Mechanics ; Orthodontics* ; Posture

Esthetic is one of the prime goal of the orthodontic treatment. It is represented by the word of harmony or balance. There is no absolute esthetic norm but it is important to study about it in orthodontic and orthognatic surgical treatment planning. The purpose of this study is to obtain the norm of Korean female adolescents who were candidates of 1986, 1987 Miss Korea Beauty Contest. The 77 individual samples were selected and studied by cephalometric analysis. Obtained results were as follows: 1. Korean esthetic norm was obtained. 2. Simple correlation coefficient was obtained between each measurements. 3. Holdaway ratio of Korean beauty was 3.3 : 1. 4. To compare with caucasian, profile of Korean beauty had less prominant nose, more prominent lip and deeper sulcus depth.
Adolescent ; Beauty ; Esthetics* ; Female* ; Humans ; Korea ; Lip ; Nose ; Young Adult*

The author has observed the gross reactions of surrounding maxillary sutures to the widening of midpalatal suture in comparison of young dog with adult dog. Experimental animals used was two young dog and two adult dog with normal palate. After midpalatal suture was seperated with rapid palatal expansion device, experimental animals were sacrificed to observe the surrounding maxillary sutures. The following results were obtained. 1. In adule dog, nasoincisive suture was not expanded, while in young dog it was espanded. 2. In adult dog, pterygopalatine suture was hardly espanded, while in young dog it was expanded unilaterally. 3. In adult dog, frontomaxillary suture was not expanded, while in young dog it was expanded unilaterally. 4. In adult dog, palatoethmoidal suture was not expanded, while in young dog it was expanded unilaterally.
Adult* ; Animals ; Dogs* ; Humans ; Palate ; Sutures*

PURPOSE: There are compelling evidences that house dust mites are important in the pathogenesis of atopic dermatitis. Recently, detection of house dust mite allergens from clothing, skin, and dust from human hair has been reported. To evaluate the importance of house dust mite exposure in the clinical severity of atopic dermatitis. METHODS: We measured house dust mite allergens (Der f1 from Dermatophagoides farinae and Der p1 from D. pteronyssinus) in scalp dander from 27 children with atopic dermatitis and 41 children with asthma only by enzyme-linked immunosorbent assay and examined correlation between house dust mite allergen concentrations in scalp dander and clinical severity of atopic dermatitis in children. RESULTS: Der f1 was detectable in scalp dander samples of 32 patients (47%) from overall 68 patient and Der p1 was detectable in 33 patients (49%). House dust mite allergens (Der f1 or Der p1) were detectable in scalp dander samples of 46 patients (70%) from overall 68 patient. There was no significant differences in house dust mite allergen levels between atopic dermatitis patients and patients with asthma only. There was a tendency of inverse correlation between frequency of scalp washing and concentration of Der f1 in scalp dander extract (r=-0.24, P=0.052). There was a significant correlation between severity grade of atopic dermatitis and concentration of Der f1 in scalp dander (r=0.39, P<0.05) in 27 children with atopic dermatitis. However, there was no significant correlation between severity grade of atopic dermatitis and concentration of Der p1 in scalp dander (r=-0.05, P>0.05). CONCLUSION: There was a significant correlation between house dust mite allergen concentrations in scalp dander and clinical severity in children with atopic dermatitis. And these results suggest that exposure to house dust mite allergen is important in the pathogenesis of atopic dermatitis.
Allergens ; Asthma ; Child* ; Clothing ; Dander* ; Dermatitis, Atopic* ; Dermatophagoides farinae ; Dust* ; Enzyme-Linked Immunosorbent Assay ; Hair ; Humans ; Pyroglyphidae* ; Scalp* ; Skin

Neurocutaneous melanosis is a rare congenital phacomatosis, characterized by the presence of multiple pigmented skin nevi at birth. Meningeal melanosis tending to become malignant and seizure. Dandy-Walker syndrome is a developmental disorder of brain characterized by cystic dilatation of the fourth ventricle and agenesis or hypoplasia of the cerebellar vermis. We studied a case of Dandy-Walker syndrome with neurocutaneous giant melanosis in a 3 years old boy.
Brain ; Child, Preschool ; Dandy-Walker Syndrome* ; Dilatation ; Fourth Ventricle ; Humans ; Male ; Melanosis* ; Neurocutaneous Syndromes ; Nevus ; Parturition ; Seizures ; Skin

BACKGROUND: A number of questionnaires designed for analyzing family members' inconvenience and demands in intensive care unit (ICU) care have been developed and validated in North America. The family satisfaction in the intensive care Unit-24 (FS-ICU-24) questionnaire is one of the most widely used of these instruments. This study aimed to translate the FS-ICU-24 questionnaire into Korean and validate the Korean version of the questionnaire. METHODS: The study was conducted in the medical, surgical, and emergency ICUs at three tertiary hospitals. Relatives of all patients hospitalized for at least 48 hours were enrolled for this study participants. The validation process included the measurement of construct validity, internal consistency, and interrater reliability. The questionnaire consists of 24 items divided between two subscales: satisfaction with care (14 items) and satisfaction with decision making (10 items). RESULTS: In total, 200 family members of 176 patients from three hospitals completed the FS-ICU-24 questionnaire. Construct validity for the questionnaire was superior to that observed for a visual analog scale (Spearman's r = 0.84, p < 0.001). Cronbach's αs were 0.83 and 0.80 for the satisfaction with care and satisfaction with decision making subscales, respectively. The mean (± standard deviation) total FS-ICU-24 score was 75.44 ± 17.70, and participants were most satisfied with consideration of their needs (82.13 ± 21.03) and least satisfied with the atmosphere in the ICU waiting room (35.38 ± 34.84). CONCLUSIONS: The Korean version of the FS-ICU-24 questionnaire demonstrated good validity and could be a useful instrument with which to measure family members' satisfaction about ICU care.
Atmosphere ; Critical Care* ; Decision Making ; Emergencies ; Humans ; Intensive Care Units* ; North America ; Tertiary Care Centers ; Visual Analog Scale

PURPOSE: X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied the cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells from two siblings and one cousin with XLA, as well as additional family members. METHODS: Btk protein expression was analyzed by flow cytometry. Isolation of the coding sequence of the Btk gene was performed by amplification using the reverse transcription-polymerase chain reaction(RT-PCR) technique. Sequence alterations were screened by the single-stranded conformation polymorphism(SSCP) method and characterized by standard sequencing protocols. RESULTS: Cytoplasmic expression of Btk protein in monocytes was not detected in three patients with XLA. In addition, Btk protein analysis clearly showed cellular mosaicism in monocytes from four obligate carriers, findings further supported by SSCP. A single base pair mutation(T to C) in Btk-exon three, which encodes the PH domain, was identified in four XLA patients. A diagnostic sequencing analysis was established to detect heterozygotic pattern in 4 carrier females. Furthermore, we found significant clinical heterogeneity in individuals with the same gene mutation. CONCLUSION: The implicating genetic alteration provided valuable clues to the pathogenesis of XLA in Korea and the flow cytometric analysis was suggested as a useful tool for rapid detection of XLA patients and carriers. The present study has identified a genetic mutation in the Btk coding region and demonstrated heterogeneity in clinical manifestations among patients with the same mutation. A flow cytometric analysis was found to be informative in establishing a deficiency of Btk protein in both patients and carriers and is recommended as a frontline procedure in the molecular diagnosis and work-up of XLA.
Agammaglobulinemia* ; B-Lymphocytes ; Base Pairing ; Clinical Coding ; Cytoplasm ; Diagnosis ; Female ; Flow Cytometry ; Humans ; Hydrogen-Ion Concentration ; Korea ; Monocytes ; Mosaicism ; Polymorphism, Single-Stranded Conformational ; Population Characteristics ; Protein-Tyrosine Kinases* ; Siblings ; Tyrosine*

Lead (Pb), mercury (Hg), and cadmium (Cd) are common heavy metal toxins and cause toxicological renal effects at high levels, but the relevance of low-level environmental exposures in the general population is controversial. A total of 1,797 adults who participated in the KNHANES (a cross-sectional nationally representative survey in Korea) were examined, and 128 of them (7.1%) had chronic kidney disease (CKD). Our study assessed the association between Pb, Hg, Cd exposure, and CKD. Blood Pb and Cd levels were correlated with CKD in univariate logistic regression model. However, these environmental heavy metals were not associated with CKD after adjustment for age, sex, BMI, smoking, hyperlipidemia, hypertension, diabetes, and these metals in multivariate logistic regression models. We stratified the analysis according to hypertension or diabetes. In the adults with hypertension or diabetes, CKD had a significant association with elevated blood Cd after adjustment, but no association was present with blood Pb and Hg. The corresponding odds ratio [OR] of Cd for CKD were 1.52 (95% confidence interval [CI], 1.05-2.19, P=0.026) in adults with hypertension and 1.92 (95% CI, 1.14-3.25, P=0.014) in adults with diabetes. Environmental low level of Pb, Hg, Cd exposure in the general population was not associated with CKD. However, Cd exposure was associated with CKD, especially in adults with hypertension or diabetes. This finding suggests that environmental low Cd exposure may be a contributor to the risk of CKD in adults with hypertension or diabetes.
Adult ; Cadmium/blood/*toxicity ; Cross-Sectional Studies ; Diabetes Mellitus/chemically induced/epidemiology ; *Environmental Exposure ; Female ; Humans ; Hypertension/chemically induced/epidemiology ; Kidney/drug effects/pathology ; Lead/blood/*toxicity ; Male ; Mercury/blood/*toxicity ; Metals, Heavy/*poisoning ; Middle Aged ; Nutrition Surveys ; Poisoning/*epidemiology ; Renal Insufficiency, Chronic/*epidemiology ; Republic of Korea ; Surveys and Questionnaires ; Young Adult

BACKGROUND: A number of questionnaires designed for analyzing family members' inconvenience and demands in intensive care unit (ICU) care have been developed and validated in North America. The family satisfaction in the intensive care Unit-24 (FS-ICU-24) questionnaire is one of the most widely used of these instruments. This study aimed to translate the FS-ICU-24 questionnaire into Korean and validate the Korean version of the questionnaire. METHODS: The study was conducted in the medical, surgical, and emergency ICUs at three tertiary hospitals. Relatives of all patients hospitalized for at least 48 hours were enrolled for this study participants. The validation process included the measurement of construct validity, internal consistency, and interrater reliability. The questionnaire consists of 24 items divided between two subscales: satisfaction with care (14 items) and satisfaction with decision making (10 items). RESULTS: In total, 200 family members of 176 patients from three hospitals completed the FS-ICU-24 questionnaire. Construct validity for the questionnaire was superior to that observed for a visual analog scale (Spearman's r = 0.84, p < 0.001). Cronbach's αs were 0.83 and 0.80 for the satisfaction with care and satisfaction with decision making subscales, respectively. The mean (± standard deviation) total FS-ICU-24 score was 75.44 ± 17.70, and participants were most satisfied with consideration of their needs (82.13 ± 21.03) and least satisfied with the atmosphere in the ICU waiting room (35.38 ± 34.84). CONCLUSIONS: The Korean version of the FS-ICU-24 questionnaire demonstrated good validity and could be a useful instrument with which to measure family members' satisfaction about ICU care.
Atmosphere ; Critical Care ; Decision Making ; Emergencies ; Humans ; Intensive Care Units ; North America ; Tertiary Care Centers ; Visual Analog Scale