No abstract available.
Ichthyosiform Erythroderma, Congenital*

No abstract available.
Sturge-Weber Syndrome*

PURPOSE:Growth hormone(GH) therapy is very effective for the treatment of short stature, but it is unconvenient that GH should be injected daily because of short half-life. Sustained-release forms of GH preparation is needed for better compliance. This study aimed to measure peak pattern and duration of release of hGH from solid microparticles using sodium hyaluronate. METHODS:In group 1, hGH(EutropinTM) 285microg/kg was injected subcutaneously to 2 Jindo dogs everyday for 7 days. In group 2, hGH solution(EutropinTM) was continuously infused subcutaneously for 12 hours a day for the first 2 days via mini pump(minimed co.) and then for 24 hours a day thereafter until 7th day with the rate of 11.9microg/kg/hr. In group 3, dose of 2mg/kg hGH in sustained-release formulation using sodium hyaluronate, was injected subcutaneously to 3 Jindo dogs. In group 4, two dose levels of 1mg/kg and 2mg/kg hGH in sustained-release formulation using sodium hyaluronate, were injected subcutaneously to each group of 4 Beagle dogs. To evaluate side reactions from continuous injection of sodium hyaluronate, sustained release form of hGH 2mg/kg was injected to 4 Beagle dogs once a week for 4 weeks and compared to 4 control Beagle dogs. Blood samples were withdrawn half- hourly for 6 hour and 2-4 times a day thereafter in Jindo dogs and at 6hr, 12hr, 22hr in the first day and twice a day(at 9:00, 16:00 O'clock) for the following 6 days. RESULTS:In group 1, peak GH conc. of 122+/-27ng/ml was observed at 1 hour after hGH(EutropinTM) 285microg/kg injection and 1/2 of peak GH conc. at 4 hour. and decreased to 2ng/ml at 24 hour. GH AUC(Area under curve) was 670(ng/ml.hr). In group 2, initial steady state GH conc. of 25ng/ml occurred after 6 hour, however, GH conc. decreased gradually to 16ng/ml at the 7th day. GH AUC based on th initial steady state GH conc. was 600(ng/ml.hr). In group 3(Jindo dogs), GH conc. was peaked at 12 hour and 1/2 of peak GH conc at 30-46 hour and decreased to baseline at 70 hour. GH AUC was 2173(ng/ml.hr). In group 4(Beagle dog), peak GH concentrations of 56+/-7ng/ml and 108+/-12ng/ml were observed at 12 hour for the doses of 1mg/ kg and 2mg/kg, respectively and 1/2 of peak GH conc at 48 hour and decreased to baseline at 80 hour. GH AUC was 3560(ng/ml.h) for 2mg/kg treated dogs. Serum IGF-1 was increased to peak levels of 520ng/ml, and 580ng/ml for the doses of 1mg/kg 2mg/kg, respectively, and persisted above the baseline till 120 hour. There was no specific side reaction during experimental period. CONCLUSION: Sustained-release form of hGH with sodium hyaluronate released GH for 70-80 hour with the peak level lower than that resulted from the conventional aqueous formulation of the equivalent dose, and higher concentration IGF-I maintained for 120 hour after injection above baseline. More extensive study is needed to permit for new therapeutic application.
Animals* ; Area Under Curve ; Compliance ; Dogs ; Growth Hormone* ; Half-Life ; Hyaluronic Acid ; Insulin-Like Growth Factor I ; Polymers

Netherton Syndrome is a rare autosomal recessive inherited disorder, characterized by triad of congenital ichthyosiform dermatosis, trichorrhexis invaginata and atopy diathesis. It has been found to be due to mutations in SPINK5, a gene encoding LEKTI (lympho-epithelial Kazal-type related inhibitor). LEKTI is a serine proteinase inhibitor that is expressed in epithelial and lymphoid tissues, and may be important in the keratinocyte terminal differentiation and T and B cell maturation. We herein report two cases of Netherton Syndrome in sisters who had ichthyosis linearis circumflexa, trichorrhexis invaginata and atropic dermatitis.
Dermatitis ; Disease Susceptibility ; Genes, vif ; Humans ; Ichthyosis ; Keratinocytes ; Lymphoid Tissue ; Netherton Syndrome* ; Serine Proteases ; Siblings* ; Skin Diseases

The influence of normal late pregnancy on insulin action and insulin secretion was studied in the Sprague-Dawley female rats. On 20th day after mating, intravenous glucose tolerance test(IVGTI) was performed in non pregnant control and pregnant rats. As results of IVGTT, glucose disappearance rate was not significantly different in both groups, but secretory response of insulin was significantly(p<0.05) increased in pregnant rat. And the ratio of insulin/ .glucose was significantly higher in pregnant rats, which means existence of insulin resistance. These insulin resistance was overcomed by increased secretory response of pancreatic insulin. Insulinogenic index( A insulin/glucose - 5 min) was highly significantly (r=0.62, p<0.01) correlated with progesterone concentration. Glycogen level and amounts of "C-glucose incorporated into glycogen after IVGTT were significantly(p<0. 05) decreased in the liver, but were not changed significantly in soleus. Glycogen synthase activity of soleus and liver was not differ significantly in the both groups. Insulin binding at varying concentrations of insulin to crude membrane of pregnant liver was not significantly different from control. In conclusions, although these pregnant rats were normal glucose tolerance due to increased secretory response of insulin, that was correlated with progesterone concentration, pregnant rat had insulin resistance. The mechanisms of insulin resistance were not related to defect of insulin binding phase and glycogen synthase, but suggest pre-receptor and/or postreceptor phase.
Animals ; Female ; Glucose ; Glucose Tolerance Test ; Glycogen ; Glycogen Synthase ; Humans ; Insulin Resistance* ; Insulin* ; Liver ; Membranes ; Pregnancy ; Progesterone ; Rats* ; Rats, Sprague-Dawley ; Receptor, Insulin

Job analysis was done by the method of interview and survey to find out the human attributes needed for dietitans' job performance. The knowledge, skills, abilities and other characteristics including personality were major items in the questionnaire. The questionnaire was distributed to 340 schools and hospital dietitians. Among these, 201 were compeletely filled out, returned and statistically analyzed according to the frequency scale and importance scale respectively. The results were as follows : 1) The age, education, job experience and monthly pay of hospital dietitians were higher than those of school dietitians. This was partly due to the reflection of short chronicle of school foodservice. 2) The most important human attributes for rwo groups were commonly shown as sincere and faithful personality, good human relationship and responsibility for business outcome. 3) Computer skill and menuplanning ability were human attributes of the most frequently used by school dietitians, whereas knowledge of diet therapy, clinical nutrition, and pathology of disease were frequently used by hospital dietitians. 4) The correlation coefficients between two groups were as follows : Spearman's rho value of personality, skills and abilities, and knowledge were 0.806, -0.432,-0.203 respectively, according to the importance scale. 5) An overall view from t test between two groups, various human attributes, especially knowledge were more frequently used by hospital dietitians.
Commerce ; Diet Therapy ; Education ; Humans* ; Nutritionists* ; Pathology ; Surveys and Questionnaires

We report a case of solitary congenital calcified nodule of the ear in a 1-year-old female. The lesion was a 2 X 2mm-sized, elevated, slightly erythematous and whitish-centered nodule located since birth on the superior helical rim of her left ear. Histopathological findings fram the totally excised lesion showed calcium deposits as amorphous, basophilic masses in the upper dermis and focal discharge of calcium by means of transepidermal elimination. Surrounding the calcium deposits, lymphohistiocytic infiltrations were present.
Basophils ; Calcium ; Dermis ; Ear* ; Female ; Humans ; Parturition

The N-terminal sequence of HIV1 gp41 (amino acid residues 584-623) was known to be the immundominant region of HIV1 gp41 protein. In order to determine epitope for gp41 protein of Korean anti-HIV1 positive sera, multiple antigenic peptides (MAPs) for the sequences corresponding to 584-604, 590-612, 604-623 and 584-618 of HIV1 gp41 were synthesized by solid phase method using Fmoc-Lys (Fmoc)-OH and used as coating antigens for ELISA. The reactivities of the synthetic peptides with Korean HIV1 positive (21 samples) and anti-HIV1 negative sera (22 samples) obtained from healthy blood doner were estimated by an indirect ELISA. MAPs for 584-604, 590-612 and 604-623 of gp41 reacted with 62 %, 100 % and 81 % of Korean anti-HIV1 positive sera tested, respectively. The results suggest that the epitope for HIV1 gp 41 for Korean anti-HIV1 positive sera is located in the region of amino acid 590-612 of gp41. MAP for gp41 (584-618) reacted with all (100 %) of anti-HIV1 positive sera tested, but did not react with anti-HlV1 negative sera. In addition, this MAP reacted stronger with seven samples of anti-HIV1 positive sera of anti-HIV1/2 combo performance panel than the mixture of 584-604, 590-612 and 604-623 of gp41, but did not react with anti-HIV negative serum. The high sensitivity and selectivity of MAP of gp41 (584-618) suggest that this peptide as a coating antigen in an ELISA system will be useful for antibody detection of HIV1.
Enzyme-Linked Immunosorbent Assay ; Epitope Mapping* ; Immune Sera* ; Peptides*

This study was mainly done by factors analyzing to find out the structure and the dimension of the results of a previous study; analyzing the school and hospital dietitians' human attributes needed for successful job performance(Song 1998). The results were as follows: 1) Through factor analysis, the 12 knowledge items were categorized into 4 groups ; 'basic knowledge of food and nutrition', 'knowledge of clinical nutrition and diet therapy', 'knowledge of medial science', and 'knowledge from experience and common sense'. These 4 knowledge factors were more frequently used and importantly recognized by hospital dietians compared with school dietitians. 2) The 38 skill and ability items were categorized into 7 groups ; 'ability of program development and research', 'ability of counseling and nutrition education', 'ability of production control and facility management', 'ability to use computer', 'ability to cooperate with others', 'ability to manage consignment marketing', and 'ability of managing situations and informations'. Different skills and abilities were required for each group. 3) The 11 personality items were categorized as 3 groups ; 'respoinsibility and sincerity', 'positive personality', and 'affirmative and inquisitive personality'.
Counseling ; Diet ; Factor Analysis, Statistical* ; Humans ; Humans* ; Nutritionists* ; Program Development

Cutaneous focal mucinosis was first described by Johnson and Helwig in 1961 and is histo logically characterized by localized accumulation of mucin in the upper and middermis with scattered spindle-shaped fibroblasts. The mucin is jelly-like acid mucopolysaccharide consisting of hyaluronic acid bound to small amounts of chondroitin sulfate and heparin. The pathogenesis of cutaneous mucinosis is unclear. However, studies of various cutaneous mucinoses suggest that there may be an unknown circulating factor that stimulates fibroblasts to produce this material. Differential diagnoses include papular mucinosis, myxoid cyst, cutaneous myxoma. Simple surgical excision is the treatment of choice. In the present case, cutaneous focal mucinosis occurs on the nail bed with onycho mycosis.
Diagnosis, Differential