Ehlers-Danlos syndrome, a heritable disorder of connective tissue by autosomal dominant mode, is very rare disease in this country. Authors described a typical case in 39 years old woman who had a daughter of 6 years old also affectect by this disorder. The patient had the characteristic hyperelasticity of the skin and hyperextensibility of the jonts especially at metacarpophalangeal joint of the hands. She also revealed hyperterolism and aortic insufficiency on X-ray and E.C.G. examination.
Adult ; Child ; Connective Tissue ; Ehlers-Danlos Syndrome ; Family Characteristics* ; Female ; Hand ; Humans ; Metacarpophalangeal Joint ; Nuclear Family ; Rare Diseases ; Skin

No abstract available.

Platelet-derived growth factor(PDGF) is known to accelerate soft tissue fracture and periodontal bone healing, but little information is available for characterizing the healing of articualr cartilage. This study was designed to demonstrate the regeneration potential of pasteurized autogeneous osteochondral graft when PDFG was applied locally in vivo. Eighteen rabbits in two groups were used in the experiment. The free osteochondral fragment(1x1x1 cm) were taken from distal femur. The fragment were pasteurized in 65degrees C for 5 minutes. In experimental group(group II), the fragment were soaked in 0.25 mg/ml of recombinant human PDGF mixed 5 ml normal saline and reimplanted to the resected site, and followed with local administration of 0.25 mg/ml PDGF to knee joint. In control group(group I), the pasteurized fragment were reimplanted in the resected site without any treatments. The histologic characteristics of the transplanted autografts for three rabbits in each group were observed at 3, 5 and 7 weeks postoperatively. The results were as follows; Group II revealed the more favorable regeneration of articular cartilage, less arthritic changes and more mature arrangement of chondrocytes than group I. Even thought the cartilage is avascular and the regeneration capacity of pasteurized cartilage is severely limited, the regeneration of grafted articular cartilage is thought to be accellerated by increased newly formed blood vessels and soft tissues due to the diffusion of PDGF on grafted cartilage, and probably also by differentiation of mesenchymal cells in the initial stage of experiment. The results of this study suggest that local administration of PDGF could be used for the treatment of posttraumatic or degenerative arthritis and various cartilage damages.
Autografts ; Blood Vessels ; Cartilage ; Cartilage, Articular ; Chondrocytes ; Diffusion ; Femur ; Humans ; Knee Joint ; Osteoarthritis ; Platelet-Derived Growth Factor* ; Rabbits* ; Regeneration* ; Transplants*

No abstract available.
Breast* ; Glucuronidase* ; Lipase* ; Milk, Human*

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

The study examined the effects of San-Yin- Jiao(SP-6) acupressure treatment on labor pain, length of delivery and anxiety for women in the labor. The effects of using SP-6 acupressure were evaluated by comparing two groups, a SP-6 acupressure group (22) and a control group (17), for a total of 39 women in labor who had a normal vaginal delivery. Data were collected using a structured questionnaire which included general characteristics, a subjective labor pain scale, measurement of duration of delivery time and a subjective anxiety scale. Data were collected before treatment (pre) and after treatment (post). The results of this study are summarized as follows : 1. The post-scores for total labor pain increased over the pre-scores but the difference between the two groups was not statistically significant(p=0.219). Wilcoxon signed rank test of the difference in pre-post labor pain scores for the SP-6 acupressure group was not statistically significant (p=0.081) but the increase for the control group was statistically significant (p=0.001).2. The length of time for the delivery in the group which had the SP-6 acupressure was shorter (143.91+/-67.77) than the control group (197.94+/-89.64). The difference between the two groups was statistically significant (p=0.028).3. The post-scores for anxiety increased over the pre-scores but the difference between the two groups was not statistically significant (p=0.426). The scores of SP-6 acupressure group did not show a significant increase by the Wilcoxon signed rank test (p=0.194) but in the control group showed a significant increase (p=0.008).This study showed that SP-6 acupressure was effective in relation to labor pain, length of time for delivery and anxiety for labor women. But it is necessary to replicate the study with a larger number of participants to generalize the results.
Acupressure* ; Anxiety* ; Female ; Humans ; Labor Pain* ; Pilot Projects* ; Pregnancy ; Surveys and Questionnaires

Lipoprotein(a) [Lp(a)] is considered an additional, independent and largely genetically determined risk factor for the development of premature coronary heart disease. Furthermore abnormal plasma lipoprotein patterns have been associated with increased risk for developing coronary heart disease. Among these lipoproteins, an increased concentration of serum Apo B and decreased level of Apo A are considered as major risk factors. together with elevated serum cholesterol and decreased HDL cholesterol. The aim of this study is to assess serum Lp(a) levels in newborns and to observe their evolution between brith, 7 days and 1 month in 64 healthy newborns by using ELISA method, Other lipid profiles were also measured and compared with feeding formula methods. The results obtained were as follows: 1) There were no significant changes from birth to 7 days, but was increased significantly after 7 days to 1 month of serum Lp(a) concentrations. 2) There were dramatic increases between birth and 7 days for Apo B and did not change between 7 days and 1 month, while Apo A-I was already present in significant levels at birth and was changed continuously between 7 days and 1 month. 3) There were marked increases between birth and 7 days for total cholesterol and triglycerides, while after 7 days the cholesterol levels only progressively increased until month. 4) There were continuous increases between birth and 7 days and 1 month for HDL-C, while LDL-C was markedly increased between birth and 7 days. 5) There were no significant correlations between serum Lp(a)concentrations and other lipid profiles neither age, sex and feeding formulas. In conclusion, our data suggest that the adequate timing for the screening test of Lp(a) in newborns is around 1 month after birth and there are no statistically significant correlations between Lp(a) and other lipid profiles.
Apolipoprotein A-I ; Apolipoproteins B ; Cholesterol ; Cholesterol, HDL ; Coronary Disease ; Enzyme-Linked Immunosorbent Assay ; Humans ; Infant, Newborn* ; Lipoprotein(a)* ; Lipoproteins ; Mass Screening ; Parturition ; Plasma ; Risk Factors ; Triglycerides

The purpose of this study was to analyse the current adult nursing curriculum content in diploma and baccaleurate level nursing schools. The Study was initiated by the Korean Academic Society of Adult Nursing and data were collected from these institutions from March 1997 to April 1998. The questionnaire was constructed after several meetings of researchers which included course description, objectives, conceptual framework, credits(theory and practicum), number of teaching staffs, content and hours for each content. The questionnaire was sent to all of the nursing schools(41 baccalaureate and 62 diploma) in the nation and the response rate were 53.7% in baccalaureate and 25.8% in diploma schools. Primary analysis was took place during summer workship of 1997 Korean Academic Society of Adult Nursing. The second analysis was carried out after additional data of detailed content and hours in adult nursing course were obtained from the schools. The result of primary analysis were as below : 1) The consensus in philosophy of adult nursing was drawn. The client of adult nursing was defined as individual over 18 years who has psychological, physical, social and spiritual aspects and interacting with environment. The client is a member of family and community. The students who finished the course should be able to adapt nursing process to their clients, and improve quality of life. The client, nursing process and nursing were common concepts which included in their conceptual framework. 2) Theory credits ranged 9-18(mean 14.1), practicum credits ranged 5-12(mean 8.1). The number of teaching staffs ranged from 2-6(mean 3.2). 3) The content which most hours assigned to were neuro/endocrine(mean 30.8 hours) gastrointestinal(mean 28.4 hours), cardiovascular(mean 28.6 hours) and respiratory(mean 22.7 hours) systems. 4) Areas taught by other profession(mostly physician) were eye, ear, nose, throat integumentary, radiation therapy, neuromuscular, rehabilitation, respiratory physiology, male genitourinary, pathology and anesthesiology. 5) Contents overlap with other course content should be rearranged in each school curriculums. Improvement of RN national board examination is prerequsite in order to improve adult nursing education in Korea. The result of second analysis were as below : 1) Credits required for graduation were 130-175 in baccalaureate, 93-134 in diploma. Thirteen to Twenty-eight percent of total credits required for nursing major were credits of adult nursing. 2) Mean hours of total adult nursing lecture were 217 in baccalareuate and 212 in diploma. 3) Disease process occupied more hours than nursing process in adult nursing theory. It is suggested to include all nursing educational institutions in order to complete the survey, to delineate the core contents of adult nursing and rate of hours between theory and practise by Korean Academic Society of Adult Nursing.
Adult* ; Anesthesiology ; Consensus ; Curriculum* ; Ear ; Education, Nursing ; Humans ; Korea ; Male ; Nose ; Nursing Process ; Nursing Theory ; Nursing* ; Pathology ; Pharynx ; Philosophy ; Quality of Life ; Rehabilitation ; Respiratory Physiological Phenomena ; Schools, Nursing ; Surveys and Questionnaires

Aggregatibacter aphrophilus is a facultatively anaerobic gram-negative coccobacillus or bacillus that grows with no dependence on X factor and variable requirement for V factor. The organism is normal flora in the human oral cavity and upper respiratory tract and, rarely, causes invasive infections such as bacteremia, endocarditis, brain abscess, or osteomyelitis. We report a case of septic peripheral embolism in left leg from A. aphrophilus endocarditis. A 49-year-old man with known hypertension presented with acute muscle pain in the left leg. On physical examination, a regular heartbeat with a pansystolic murmur was heard. There were decreased pulses in the left popliteal and dorsalis pedis arteries and coldness of the left foot, although sensory and motor functions were intact. Angiography revealed an embolus in a branch of the left femoral artery. He underwent emergency embolectomy, and gram-negative bacilli grew in the embolus cultures. The same microorganism was isolated in two pairs of blood culturs and subsequently identified as A. aphrophilus. Transthoracic echocardiography revealed mitral regurgitation and multiple vegetations on the mitral valve. The patient was treated with a third-generation cephalosporin for 4 weeks and mitral valve replacement in view of the diagnosis of infective endocarditis and septic peripheral embolism.
Angiography ; Arteries ; Bacillus ; Bacteremia ; Brain Abscess ; Cold Temperature ; Echocardiography ; Embolectomy ; Embolism ; Embolism and Thrombosis ; Emergencies ; Endocarditis ; Femoral Artery ; Foot ; Humans ; Hypertension ; Leg ; Middle Aged ; Mitral Valve ; Mitral Valve Insufficiency ; Mouth ; Muscles ; Osteomyelitis ; Physical Examination ; Respiratory System

PURPOSE: To determine the effect of intraluminal brachytherapy on stent patency and survival after metallic stent placement in patients with primary bile duct carcinoma. MATERIALS AND METHODS: Twenty-seven patients with primary bile duct carcinoma underwent metallic stent placement; in 16 of the 27 intraluminal brachytherapy with an iridium-192 source (dose, 25 Gy) was the performed. Obstruction was due to either hilar (n=14) or non-hilar involvement (n=13). For statistical comparison of patients who underwent/did not undergo intraluminal brachytherapy, stent patency and survival were calculated using the Kaplan-Meier method and an independent t test. RESULTS: The mean durations of stent patency and survival were 9.1 and 10.0 months respectively in patients who underwent intraluminal brachytherapy, and 4.2 and 5.0 months in those who did not undergo this procedure (p<0.05). The mean durations of stent patency and survival among the 22 patients who died were 7.6 (range, 0.8 -16.1) and 8.3 (range, 0.8-17.3) months, respectively, in the eleven patients who underwent intraluminal brachytherapy, and 4.2 (range, 0.9-8.0) and 5.0 (range, 0.9-8.4) months in those whom the procedure was not performed (p<0.05). CONCLUSION: Intraluminal brachytherapy after stent placement extended both stent patency and survival in patients with primary bile duct carcinoma.
Bile Ducts* ; Bile* ; Brachytherapy* ; Humans ; Stents*