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

Juvenile xanthogranuloma (JXG) is a fibrohistiocytic proliferative disorder predominant in infancy and childhood. It is a benign and transient skin lesion that usually appears in the cephalic area. Recently, the concurrent finding of neurofibromatosis (NF), juvenile chronic myelogenous leukemia (JCML), and JXG has been repeatedly reported. Especially, the family history of NF may represent a risk factor for the development of JCML in patients with NF and in patients with NF and JXG. So, a finding of JXG and NF in infants should alert the physician to a possible development of JCML. We report a case of JXG developed in a neurofibromatosis child with family history.
Child ; Humans ; Infant ; Leukemia, Myelomonocytic, Juvenile ; Neurofibromatoses ; Risk Factors ; Skin ; Xanthogranuloma, Juvenile

It is well known that the electroencephalographic finding in patients with moyamoya disease demonstrates the characteristic "re-build up" phenomenon a few minutes after hyperventilation. To evaluate the usefulness of an electroencephalogram (EEG) in the postoperative management of children with moyamoya disease, we studied the presence or absence of improvement in the clinical, single photon emission computed tomography (SPECT) and EEG findings, before and after surgery. Twenty-two patients, who underwent indirect revascularization surgery for moyamoya disease, were included in our study. Clinical improvement was assessed as the disappearance or decrease of a transient ischemic attack or headache. The findings on the EEG and SPECT were considered improved when the re-build up phenomenon was absent and when there was improvement in the cerebrovascular reserve as a result of the acetazolamide challenge test. The statistical correlation analysis for both clinical and EEG improvement were consistent (kappa value=0.409, p< 0.05). However, the result from the clinical and SPECT improvement as well as that from EEG and SPECT improvement were not statistically significant. Our results suggest that EEG can be used as a noninvasive and simple follow-up test for moyamoya disease after indirect revascularization surgery if the hyperventilation procedure is effectively performed during EEG recording.
Tomography, Emission-Computed, Single-Photon/methods ; Postoperative Period ; Moyamoya Disease/*diagnosis/*therapy ; Models, Statistical ; Male ; Ischemic Attack, Transient/diagnosis ; Humans ; Headache/diagnosis ; Female ; Electroencephalography/*methods ; Child, Preschool ; Child ; Cerebral Revascularization ; Adolescent

PURPOSE: To evaluate the clinical results of the pilon fractures treated with arthroscopically assisted limited open reduction and Ilizarov external fixation. MATERIALS AND METHODS: This is a retrospective study of the clinical result, bone union, complication and postoperative ankle function of 22 pilon fractures treated with arthroscopically assisted limited open reduction and Ilizarov external fixation between January 1999 to March 2004. RESULTS: Clinical follow up averaged 16 months, with an average age of 39.2. All patients with type 1 and 2 fracture had excellent or good score by Ovadia and Beals criteria. Closed fractures healed within 13 weeks and open fractures within 16 weeks after surgery in average. Average range of motion of the ankle was 12o dorsiflexion (0~20 degree) and 25o plantar flexion (15~35 degree). CONCLUSION: Minimal soft tissue dissection and anatomical reduction are very important factor for minimizing complication and satisfactory ankle function. So, arthroscopically assisted limited open reduction and Ilizarov external fixation is an effective treatment option for tibial pilon fractures.
Ankle ; Follow-Up Studies ; Fractures, Closed ; Fractures, Open ; Humans ; Range of Motion, Articular ; Retrospective Studies