The multiple lentigines syndrome or LEOPARD syndrome is an autosomal dominantly inherited disorder with a variety of abnormalities and a familial occurrence. This syndrome is characterized by the presence of numerous dark brown macules on the skin but not the mucous surface, and by a marked increase in the number of lentigines from birth to puberty. The eponym LEOPARD stands for lentigines, EKG abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of the genitalia, retardation of growth and deafness. We report a case of multiple lentigines syndrome in 7-year-old boy. He had numerous pinhead to pea sized, dark brownish macules scattered on the entire body and also had pulmonary stenosis, EKG abnormality, ocular hypertelorism and right exotropia. Interestingly, he also had a labial melanotic macule on the lower lip, which is usually spared in the multiple lentigines syndrome. Histologically, the biopsy specimen taken from the macule revealed an elongation of rete ridges, an increase of melanin pigments in the basal layer and mild inflammatory infiltrates intermingled with the melanophages in the upper dermis.
Adolescent ; Biopsy ; Child ; Deafness ; Dermis ; Electrocardiography ; Eponyms ; Exotropia ; Genitalia ; Humans ; Hypertelorism ; Lentigo ; LEOPARD Syndrome* ; Lip ; Male ; Melanins ; Panthera ; Parturition ; Peas ; Puberty ; Pulmonary Valve Stenosis ; Skin

No abstract available.
Blood Pressure* ; Humans ; Infant, Newborn*

No abstract available.
Anesthesiology* ; Education* ; Publications*

Endonasal DCR has been reported for the advantages of lack of a cutaneous scar and short operating time but that the surgical success rates were lower than conventional method. We have performed endonasal DCR and had follow-up of at least 6 months in 107 patients who had been diagnosed with lacrimal pathway obstruction, and also surveyed the age, sex, sac size and nasal cavity abnormality to evaluate the factors related to success rate. Primary success rates were 82.2%(88eyes) and higher success rates were noted in younger age, man and large sac but not significant. But. the cases associated with nasal cavity abnormality as septal deviation, middle turbinate hypertrophy, ethmoiditis and polyp showed poor surgical results due to membranous ostium closure, formation of granuloma within the ostium, and formation of cicatrix with the middle turbinate.(P<0.05) Improvement of success rate may be owing to appropriate preoperative evaluation, proper management of nasal cavity abnormality preoperatively and intraoperatively, and show consider the conventional method in cases of that lower success rate are predict.
Cicatrix ; Dacryocystorhinostomy* ; Follow-Up Studies ; Granuloma ; Humans ; Hypertrophy ; Nasal Cavity ; Polyps ; Turbinates

The purpose of this study was to develop a dental communication course in dental hygiene schools that included theory, practice, and skill for effective dental communication. Thirty-six senior dental hygiene students in a dental hygiene school took a dental communication course and responded to a questionnaire. The instrument used in the study was a modified form of Kim's communication skill self-assessment sheet. The self-assessment questionnaire about communication competencies was administered before and after the class, and the difference between scores at each time point was analyzed using the Wilcoxon signed rank sum test. Among seven elements of dental communication competencies, the competency of “information gathering” was significantly improved (p=0.008). Students' scores on perception of the importance (p=0.019) and necessity (0.016) of a communication course significantly increased after the course. Competencies in communication are essential requirement for dental hygienists. This study showed the possibility of communication skill training. An objective evaluation tool regarding students' communication competencies should be developed. Further studies with larger samples size are needed to develop a standardized comprehensive communication course in dental hygiene schools.
Curriculum ; Dental Hygienists ; Health Communication ; Health Education ; Humans ; Learning* ; Oral Hygiene* ; Self-Assessment

BACKGROUND: The reduction in hematocrit (Hct) by hemodilution tends to cause an increase in cardiac output and a proportional decrease in arterial oxygen content. Additionally the reduction of systemic oxygen delivery (DO2) leads to significant differences in regional blood flow. It is therefore important to characterize the effects of hemodilution on regional oxygen metabolism in individual organs. This study was undertaken to evaluate and compare the effects of acute normovolemic anemia induced by hemodilution. METHODS: Six dogs were anesthetized and mechanically ventilated. Catheters were inserted in the right femoral and pulmonary arteries for blood sampling, and a gastric tonometer catheter was inserted into the gastric lumen. Baseline measurements of systemic hemodynamics, arterial ketone body ratio (AKBR), gastric intramucosal pH (pHi) and arterial lactate were recorded. Hemodilution was then begun by 6% pentastarch and was made in four levels of hematocrit values of 20%, 15%, 10% and 6%. RESULTS: Mean arterial pressures of Hct 10% and 6% was decresaed (P < 0.05) and Hct 15% and 10% increases in cardiac output and pulmonary capillary wedge pressure (PCWP) were observed. Central venous pressure and mean pulmonary arterial pressure were incresed (P < 0.05) at Hct 15%, 10% and 6%. DO2 progressively decreased (P < 0.05). AKBR and pHi began to decreased at Hct 15%. Arterial lactate decrease at Hct 15% and was above 7.4 mmol/L at Hct 6%. CONCLUSIONS: By the measurements of AKBR and pHi, the disturbance of splanchnic oxygenation can be detected early compared to those of O2 in terms of oxygen metabolism and the critical point of DO2 during acute normovolemic anemia induced by hemodilution.
Anemia* ; Animals ; Arterial Pressure ; Cardiac Output ; Catheters ; Central Venous Pressure ; Dogs* ; Hematocrit ; Hemodilution* ; Hemodynamics ; Hydrogen-Ion Concentration* ; Hydroxyethyl Starch Derivatives ; Lactic Acid* ; Metabolism ; Oxygen Consumption* ; Oxygen* ; Pulmonary Artery ; Pulmonary Wedge Pressure ; Regional Blood Flow

Periodic paralysis associated with thyrotoxicosis is characterized by intermittent flaccid paralysis of the skeletal muscle. The paralysis usually involve the skeletal muscle of the limbs, especially lower extrimities. In general, sensory function is intact. Involvement of respiratory, ocular or bulbar muscles is very rare, but bulbar and respiratoy invelvement may prove fatal. It is very rare a case that has severe clinical manifestation such as cardiac arrest. We report a case of thyrotoxic hypokalemic periodic paralysis presenting as cardiac arrest.
Extremities ; Heart Arrest ; Hypokalemic Periodic Paralysis ; Muscle, Skeletal ; Muscles ; Paralysis ; Sensation ; Thyrotoxicosis

BACKGROUND: As single donor platelets (SDP) has been increasingly used, the quality of SDP, especially apheresis-induced platelet activation, has become a major issue. This study evaluated the activation of SDP platelets prepared with three different cell separators that are currently being used at the Korean Red Cross. METHODS: CD62p, CD63 and CD42 were measured in 35 units of SDP prepared with Amicus (Baxter, Deerfield, IL, USA), MCS+ (Haemonetics, Braintree, MA, USA), or Trima (Gambro BCT, Lakewood, USA) using flow cytometry. RESULTS: Expression of CD62p gradually increased with storage time, but no difference in expression was noted between cell separators. Expression of CD63 also increased with storage time and platelets prepared with the Amicus displayed significantly higher CD63 expression 72 and 120 hours after collection compared to those prepared with MCS+ and Trima. Expression of CD42b tended to decrease with storage time, but this was only significant for Amicus 120 hours after collection. No difference in CD42b expression was noted between cell separators. CONCLUSIONS: Platelet activation increased with storage time, and platelet activation was more pronounced in the platelets prepared with the Amicus. However, because in vitro results of platelet activation does not necessarily reflect in vivo platelet function and survival, additional studies are needed to clarify clinical effectiveness of activated platelets.
Blood Platelets* ; Flow Cytometry ; Humans ; Platelet Activation* ; Plateletpheresis* ; Red Cross ; Tissue Donors

BACKGROUND AND OBJECTIVES: We investigated in patients with coronary vasospastic angina whether the exercise ECG test results are influenced by the different modes of exercise load and compared the clinical characteristics including coronary risk factors between patients with positive and negative exercise tests. MATERIALS AND METHODS: This study comprised 34 patients with documented coronary artery spasm without significant stenosis (coronary artery luminal diameter narrowing <70%) and treadmill exercise test. Treadmill exercise ECG test was performed based on Bruce's protocol (graded exercise test, GET) and sudden rapid exercise protocol (non-graded exercise test, NGET) in the morning of the same day. RESULTS: 1) Of 29 patients who underwent both GET and NGET, 19 patients manifested positive result by NGET, whereas only 11 patients did by GET (66 vs. 38%, P=0.04). All patients with positive GET had positive NGET and 8 of 18 patients with negative GET had positive NGET. 2) Of 34 patients who underwent GET, there was no significant difference in frequency of hypertension, diabetes, current smoking, presence of effort chest pain by history, mixed disease (fixed stenosis >50%, <70% of luminal diameter) or total cholesterol level between patients with positive and negative results. In 18 typical variant angina patients by clinical history, 8 of 10 patients with high disease activity (5 times or more attack per week) manifested positive result by GET or NGET, whereas 4 of 8 patients with low disease activity (80 vs. 50%, P=NS). CONCLUSION: In patients with coronary vasospastic angina, sudden rapid exercise could induce more frequently coronary artery spasm than multistage exercise. The result of an exercise test may not be correlated with coronary risk factors, coronary anatomy, effort chest pain, and the disease activity.
Arteries ; Chest Pain ; Cholesterol ; Constriction, Pathologic ; Coronary Vessels ; Electrocardiography ; Exercise Test* ; Humans ; Hypertension ; Phenobarbital ; Risk Factors ; Smoke ; Smoking ; Spasm

BACKGROUND: The p38 mitogen-activated protein kinases (p38Map kinases) are a family of prolinedirected serine/threonine kinases. At least four isoforms of p38Map kinases have been identified; however, their physiological significances remain to be understood. Recently, the role of p38Map kinase in insulin-stimulated glucose uptake has been suggested. The present study aimed to investigate which isoform(s) were responsive to insulin stimulation. In addition, the activities of p38 Map kinase isoforms that may participate in the insulin's antiapoptotic function in CHO-IR cells were also determined. METHODS: Chinese hamster ovary cells, expressing wild- or mutated human insulin receptors (CHO-IR cells), were used to investigate whether insulin can stimulate any of the isoform(s) of the p38Map kinases. The p38Map kinase activity was determined by measuring the degree of 32P-labelling of ATF-2 protein, a specific substrate of p38Map kinase. A DNA laddering assay was performed to examine the degree of apoptosis and a RT-PCR analysis to determine which isoform(s) of the p38Map kinases were expressed in response to insulin. RESULTS: p38Map kinase activation by insulin was sharply suppressed in only the CHO-IR/A1018K cells, which lack the intrinsic tyrosine kinase activity of insulin receptors. Insulin stimulation of p38Map kinase was insensitive to SB203580, an inhibitor of the alpha(alpha)-and beta(beta)-isoforms of p38Map kinases. Moreover, orthovanadate, known as a specific stimulator of the gamma(gamma)-and delta(delta-) isoforms, stimulated the p38Map kinase activity in CHO-IR cells. Insulin increased the degree of mRNA expression of the delta-isoform, but not that of the alpha-isoform p38Map kinase. Interestingly, PD98059, an inhibitor of ERK, suppressed p38Map kinase stimulation, as well as the antiapoptotic protection of cells by insulin. As insulin was found to still protect ERK-lacking cells (CHO-IR/ SOS) from apoptosis, any substantial role(s) of ERK might be excluded. CONCLUSION: Our data suggest that insulin may stimulate the activity and expression of the-isoform of p38Map kinase in a MEK1/2-dependent manner. The involvement of the delta-isoform of p38Map kinase in insulin's antiapoptotic protection was also suggested, but remains to be investigated further to clarify the nature of its mechanism of action
Animals ; Apoptosis ; Cricetinae ; Cricetulus ; DNA ; Female ; Glucose ; Humans ; Insulin ; Ovary ; p38 Mitogen-Activated Protein Kinases ; Phosphotransferases* ; Protein Isoforms ; Protein-Tyrosine Kinases ; Receptor, Insulin ; RNA, Messenger ; Vanadates