Tow cases of congenital ectodermal defect in brothers aged 15 and 10 years were pesinted and the literature was reviewed. They had lack of sweating, sparseness of the hard hairs, absence of lanugo hairs, maldevelopment or defect of most of the teeth, etc. They seemed to be angidrotic type associated with a sex-linked reccessive inheritance in their family backgroud.
Ectodermal Dysplasia* ; Hair ; Humans ; Siblings ; Sweat ; Sweating ; Tooth ; Wills

In order to provide more effective and personalized healthcare services to patients and healthcare professionals, intelligent active knowledge management and reasoning systems with semantic interoperability are needed. Technological developments have changed ubiquitous healthcare making it more semantically interoperable and individual patient-based; however, there are also limitations to these methodologies. Based upon an extensive review of international literature, this paper describes two technological approaches to semantically interoperable electronic health records for ubiquitous healthcare data management: the ontology-based model and the information, or openEHR archetype model, and the link to standard terminologies such as SNOMED-CT.
Aluminum Hydroxide ; Carbonates ; Delivery of Health Care ; Electronic Health Records ; Electronics ; Electrons ; Humans ; Knowledge Management ; Semantics

Angiodysplasia of small bowel is uncommon and frequently undiagnosed and presents a taxing surgical problem. It is usually diagnosed for unexplained gastrointestinal bleeding. For the surgeon, the main technical problem is that the lesion is impalpable, and invisible to the naked eye, so it usually cannot be identified unless bleeds actively at the time of surgery. Arteriography gives a little information about wax and wane pattern of bleeding in the lesion. Endoscopy is often unfruitful because the majority of lesions are submucosal and rarely exceed a few millimeters in diameter. Transillumination of the intestinal wall from inside of the lumen to the outside in a dark room can define the precise vascular anatomy of the wall. The delicate lesion of the angiodysplasia can be identified by this transillumination method. We described a simple intraoperstive endoscopic translllumination technique used successfully to identify an angiodysplasia in the small bovwel prior to the bowel resecion. This report summarized our experience and review of literature.
Angiodysplasia* ; Angiography ; Endoscopy ; Hemorrhage ; Taxes ; Transillumination*

Click sound can evoke myogenic potentials in active sternocleidomastoid(SCM) muscle. These are due to activity of vestibular afferent rather than cochlear, which produce very short latency inhibition of ipsilateral motor unit activity. We investigated the click-evoked vestibulocollic reflexes in neck muscles to find out the significance and relationship of latency and amplitude of myogenic potentials between two different methods of muscles activation. EMGs were recorded from surface electrode evoked by sound stimuli(95dB SPL, 5/sec repetitive rate, 512 times) over SCM muscles on each side. Myogenic potentials have two biphasic responses which were termed P1, N1, P2, N2 based on the polarity of their components. The subjects were 24 normal volunteers. EMG recordings were obtained from 12 subjects(Group 1) who keeped head 10 cm above ground in supine position to activate their neck flexors to the degree required through the averaging runs(bilateral SCM muscles activation method). EMGs from another 12 subjects(Group 2) were recorded by rotating head to the opposite side to activate SCM muscle throughout the procedure(unilateral SCM muscle activation method). The latencies and amplitudes of myogenic potentials in the SCM muscle after stimulation were analysed and compared between the two methods. The latencies(msec) of P1 and N1 were 11.2+/-2.4, 21.7+/-1.1 respectively in Group 1 and 13.3+/-2.4, 23.0+/-1.1 respectively in Group 2. The amplitude(uV) of P1-N1 were 38.78.1 in Group 1 and 33.28.1 in Group 2. There were no significant differences in latencies and amplitudes of P1 and N1 between the groups(p<0.05). Unilateral muscle activation is easier and comfortable than the bilateral muscle activation and there were no significant difference in latency and amplitude of P1. The myogenic potential of unilateral vestibular function loss disappeared in a patient who was labyrinthectomized but there was normal response of potential in a patient who is profound sensory neural hearing loss. Click evoked myogenic potentials is a simple method of examining vestibulocollic reflex. Click evoked myogenic potential is related to vestibular status.
Electrodes ; Head ; Healthy Volunteers ; Hearing Loss ; Humans ; Muscles ; Neck ; Neck Muscles ; Reflex* ; Supine Position

Central neurogenic hyperventilation (CNH) is characterized by sustained tachypnea inspite of an elevated arterial PaO2, pH and a low arterial PaCO2. CNH is common in patients with brainstem injury accompanied by a decreased level of consciousness but this also has been described in some alert patients with an invasive brainstem tumor. We report one case with CNH resulting from a unilateral pontine infarction. His consciousness was clear, and CNH spontaneously disappeared without any medication.
Brain Stem ; Brain Stem Neoplasms ; Consciousness ; Humans ; Hydrogen-Ion Concentration ; Hyperventilation* ; Infarction* ; Tachypnea

The lateral medullary syndrome, I. E. Wallenberg syndrome, presents with several forms of sensory deficits. However, the correlation between the sensory deficits and the MRI findings of the medulla has been rarely attempted. We studied 16 patients with lateral medullary infarction who showed appropriate MRI lesions and correlated their sensory findings with the MRI results. In order to examine the extent of lesion of medulla in the MRI, we divided the medulla into three parts, namely upper, middle, and lower parts. The patients exhibited six types of sensory manifestation. In brain MRI, five patients with uncrossed sensory deficit; three patients with contralateral sensory deficit involving only body; and one patient with contralateral sensory level on trunk showed a diagonal band, lateral, or combined lesion in the medulla. Four patients with crossed sensory deficit and one patient with ipsilateral sensory deficit involving only face showed a large, laterodorsal lesion in the medulla. Two patients without sensory deficit exhibited a dorsal lesion in the medulla. In conclusion, lateral medullary syndrome usually has a characteristic lesion in the MRI according to the sensory deficits.
Brain ; Humans ; Infarction ; Lateral Medullary Syndrome* ; Magnetic Resonance Imaging*

A clinical analysis of 1753 patients with dental surgery under the general anesthesia from January 1981 to December 1985 was made according to age, sex, disease, and status of intubation. The results were as follows: 1) Of 1753 patients with oral surgery, 1067 male and 686 female patients were operated on under general anesthesia and the male to female ratio was 1.6:l. 2) Of 1753 patients, there were 501 patients with oral surgery from 20 to 30 years of age and this was the most common age. 3) Benign tumors including cysts were the most common lesions operated on under general anesthesia. 4) Of 1753 patients, 1529 patients had nasotracheal intubations, but the patients with orotracheal intubations were 206, and the patients with endotracheal insertion through tracheostomy were only 18 (l%).
Anesthesia, General ; Female ; Humans ; Intubation ; Male ; Surgery, Oral* ; Tracheostomy

Atrial Septal Defect(ASD) is usually suspected clinically but requires a confirmative diagnostic procedure before surgical repair. Conventional transthoracic echocardiography has relatively high sensitivity and specificity for ASD, but difficulty in visualizing the ASD occasionally. Transesophageal echocardiography has special advantages for investigating the posteriorly located cardiac structures, including the atrial septum, which is imaged perpendicularly at a relatively short distance. We describe a case in which ostium secundum ASD was not visualized by conventional transthoracic echocardiography, but was diagnosed confidently by transesophageal echocardiography. It is concluded that transesophageal echocardiography appears to be a promising diagnostic tools for the evaluation of ASD on the basis of its ability to provide excellent imaging of the entire atrial septum and related posterior cardiac structures.
Atrial Septum ; Echocardiography ; Echocardiography, Transesophageal* ; Heart Septal Defects, Atrial* ; Sensitivity and Specificity

PURPOSE: The purpose is to evaluate the CT or MRI findings to help in the differentiation between benign and malignant meningiomas. MATERIAL AND METHODS: The CT and MRI findings of 8 patients with surgically proven malignant meningioma were reviewed. Four cases of 8 malignant meningiomas and 18 cases of 27 benign lesions were studied with a 1.5T or 0.5T MRI. Radiologic analysis was focused on the heterogeneity of tumor, grade of peritumoral edema, presence of cystic degeneration and calcification, tumoral enhancement pattern, dural enhancement, and tumoral border. RESULT: Histologic types of malignant meningioma were meningotheliomatous (n=4), papillary(n=1), fibroblastic(n=l), angioblastic(n=l), and sarcomatous(n=l). Tumoral Heterogeneity was seen in 8 of 8 malignant lesions(100%) and 8 of 27 benign ones(30%). (p<0.01) Marked peritumoral edema was seen in 8 of 8 malignant lesions(100%) and 2 of 27 benign ones. (7.3%)(p<0.01) Most of malignant lesions(6 of 8 cases, 75%) showed ill defined border. Calcification was not present in malignant lesions in contrast with benign ones (14 of 27 cases, 52%). (p<0.05) No statistical significance was noted in cystic degeneration, dural tail like enhancement or tumoral enhancement pattern between malignant and benign meningiomas. MRI signal intensity of malignant meningiomas was homogeneously or heterogeneously hypointense on T1WI, heterogeneously isointense or hypointense on T2WI and heterogeneous or mixed on Gd-DTPA enhancement study. CONCLUSION: The CT or MRI findings such as heterogeneity, ill defined tumoral border, marked peritumoral edema, and absence of calcification may suggest the possibility of malignancy in meningioma.
Edema ; Gadolinium DTPA ; Humans ; Magnetic Resonance Imaging* ; Meningioma* ; Population Characteristics

PURPOSE: To compare the radiologic and clinical outcomes of total knee arthroplasty (TKA) performed using a navigation system with those obtained using the conventional technique. MATERIALS AND METHODS: Forty-one TKAs were performed using a navigation system (navigation group) and forty-eight by conventional surgery (conventional group). Radiologic results outside the range defined by +/-3degrees of optimum were classified as outliers. Range of motion and HSS scores at final follow-up were also compared. RESULTS: Mean mechanical axes were 0.3degrees valgus for navigation and 0.5degrees varus for the conventional group, which were not statistically different. However, two outliers occurred in the navigation group, which was significantly less than the 14 that occurred in the conventional group. Mean coronal inclination of the femoral component was significantly different in the two groups as were the outlier numbers. Although outlier numbers significantly differed in sagittal inclination of the femoral or tibial components or coronal inclination of the tibial component, there were no statistical differences in terms of mean values. Differences in ROM and HSS scores were also not observed between the two groups. CONCLUSION: The navigation system helped increase alignment accuracy of the lower extremity, but no significant difference was observed in clinical results between the two study groups.
Arthroplasty* ; Follow-Up Studies ; Knee* ; Lower Extremity ; Range of Motion, Articular