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*

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

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

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

PURPOSE: In general, genetic disorders have the numerical or structural abnormalities of chromosome. There are clinically two different disorders, Prader-Willi syndrome(PWS) and Angelman syndrome(AS), which have the deletion of specific region of 15 chromosome originated from father or mother by genetic imprinting phenomenon. We reported the results of genetic study including DNA methylation analysis and fluorescence in situ hybridization(FISH), in addition to clinical findings. METHODS: We studied 20 patients of PWS and AS diagnosed in pediatric department of Asan Medical Center from 1992 Feb. to 2001 Apr., retrospectively. We analyzed the clinical manifestations of the disorders and performed cytogenetic studies from the peripheral blood. And we the methylation pattern and FISH, using the probe for single nucleotide ribonucleoprotein N(SNRPN) locus within the Prader-Willi/Angelman region (15q11-q13). RESULTS: Among 16 patients of PWS, the age at initial visit was from 3 days to 12 years and the male to female ratio was 1.67:1. Seven patients had the history of intrauterine growth retardation or fetal distress. The chief complaints at initial presentation were classified on age. Under 3 years(n=10), there were hypotonia(4), development delay (3), feeding difficulties(2), and tachycardia(1). Above 3 years(n=6), we found short stature(2), respiratory difficulty(1), seizure(1), obesity(1), and undescended testes(1). DNA methylation study showed the methylation of 15 chromosome originated from paternal chromosome and the microdeletion by FISH was detected in all 12 among 12 patients (100%). In AS(n=4), the sex ratio(M:F) was 3:1 and age at presentation was from 15 months to 6 years. The important sign was severe developmental delay in all patients, combined with mental retardation in one case. Other clinical manifestations were depigmentation of skin and hair(4), prognathism(3), seizure(3), frequent laughing(2), undescended testes(1), hypotonia(1), and hyperactivity(1). FISH study was performed and showed the microdeletion of 15 chromosome in all 4 patients. CONCLUSION: We studied the clinically two different disorders, PWS and AS, with the deletion of same region of 15 chromosome and reported the results of the clinical and genetic analysis based on clinical findings and DNA analysis and FISH study, respectively.
Angelman Syndrome* ; Chungcheongnam-do ; Cytogenetics ; DNA ; DNA Methylation ; Fathers ; Female ; Fetal Distress ; Fetal Growth Retardation ; Fluorescence ; Genomic Imprinting ; Humans ; Intellectual Disability ; Male ; Methylation ; Mothers ; Prader-Willi Syndrome* ; Retrospective Studies ; Ribonucleoproteins ; Skin ; snRNP Core Proteins

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: Tracheal intubation can cause sympathetic stimulation such as hypertension and tachycardia. Many drug are used for reducing the incidence of hypertension and tachycardia induced by intubation. Among these drugs, fentanyl can be used with good result. The purpose of this study was to find the appropriate fentanyl injection time before intubation when a small dose (3 microgram/kg) was used. METHODS: We studied 82 ASA class 1 2 patients, scheduled for gynecological elective surgery, randomized into 5 groups. Group 1 received no fentanyl before intubation, and groups 2, 3, 4 and 5 received 3 microgram/kg fentanyl at 7 min, 5 min, 3 min and 1 min before intubation, respectively. The blood pressure and heart rate were checked at preinduction and post-intubation periods for 5 minutes. RESULTS: The increase of the heart rate was significantly lower in groups 2 and 3 than in group 5 just after intubation. Furthermore the increase of the mean blood pressure was significantly lower in groups 2, 3 and 4 than in group 5 just after intubation. The incidence of tachycardia and hypertension was significantly lower in groups 2, 3 and 4 than in group 5 just after intubation. CONCLUSIONS: When a small dose of fentanyl was used to blunt the intubation induced sympathetic stimulation, the appropriate time was prior to 3 minutes before intubation.
Blood Pressure ; Fentanyl* ; Heart Rate ; Humans ; Hypertension* ; Incidence* ; Intubation* ; Tachycardia*