Romberg syndrome is a rare disease and characterized by unilaeril atrophy of the skin, subcutaneous tissue and the underlying structure of half the face. The lesion does not usually cross the midline of the scalp. Roriberg syndrome is sometimes mildsnosed as linear scleroderma, although they differ widely in their clinical and histological apperance. A 13-year-old boy was present with a one year history of idefined atrophic patch on the left side of face and neck. We involved skin was not bound down to the underlying structure. The history revealed no prcvious trauma or disease, He had no abnormal neurologic finding. Computerized tomography demonstrated a diminished subcutaneouat volume and also decreased thickness of back muscle is the left side face from cheek to neck.
Adolescent ; Atrophy ; Back Muscles ; Cheek ; Humans ; Male ; Neck ; Neurologic Manifestations ; Rare Diseases ; Scalp ; Scleroderma, Localized ; Skin ; Subcutaneous Tissue

A 63-year-old man had a huge verrucous protruding mass over the suprasternal area. The lesion enlarged rapidly over 3 mooths, and measured about 10×8 cm. The histologic finding of the biopsy specimen showed nests of squamous epithelium with central keratinization, infiltrating the dermis. The neoplasm was treated successfully with surgical excision.
Biopsy ; Dermis ; Epithelium ; Humans ; Keratoacanthoma* ; Middle Aged

Mutations in the p53 tumor suppressor gene have been shown to be one of the most common genetic abnormalities in human cancers. Loss of p53 tumor suppressor gene function can occur through gene mutation or interaction with early proteins of oncogenic viruses such as E6 protein of human papillomaviruses(HPV). We studied 24 squamous cell carcinomas of the larynx(20) and hypopharynx(4) for p53 gene mutations as well as for the presence of oncogenic HPV DNA. Exon 5 through 8 of the p53 gene were examined using polymerase chain reaction-direct sequencing methods. HPV detection was done using polymerase chain reaction amplification with HPV L1 consensus primer. HPV type was determined by the same method using HPV-16 and -18 type-specific E6 primers. The results were as follows: 1) Eight of 24 tumors(33%) had p53 mutations, one of which had 2 mutational sites. All cases of which had p53 mutations or HPV DNA detection were larynx cancer. 2) p53 genetic alteration in larynx cancer included seven missense mutations resulting in single amino acid substitutions, one nonsense mutation encoding a stop codon and one silent mutation. Six of 9(66.7%) mutations occurred in two distinct regions of the genes, codon 245-248(3 mutations) and codon 283-294(3 mutations). 3) The p53 mutational spectrum observed was characterized by G to T transversion(4 of 9), T to A transversion(2 of 9), C to A transversion(1 of 9), G to A transition(1 of 9) and C to T transition(1 of 9). 4) HPV DNA was detected in 3 of 24(12.5%) tumors. According to the type of HPV DNA, HPV-16 was detected in 1 case and HPV non-16, -18 was detected in 2 cases, one of which had p53 mutation. 5) There was no relationship between p53 mutations or HPV DNA detection and clinicopathologic parameters. These results suggest that p53 mutations may play an important role in carcinogenesis of laryngeal cancer. Further study is necessary to clarify the role of p53 mutation and oncogenic HPV infection on clinical outcome of laryngeal cancer.
Amino Acid Substitution ; Carcinogenesis ; Carcinoma, Squamous Cell ; Codon ; Codon, Nonsense ; Codon, Terminator ; Consensus ; DNA* ; Exons ; Genes, p53* ; Genes, Tumor Suppressor ; Human papillomavirus 16 ; Humans* ; Laryngeal Neoplasms ; Larynx* ; Mutation, Missense ; Oncogenic Viruses ; Pharyngeal Neoplasms* ; Pharynx* ; Polymerase Chain Reaction

Microscopic analysis of bone is very useful method for estimating age at death of individual identification, diagnosing metabolic disorder or dietary deficiency in bone tissue, and differentiating human bone or not when applied to fragmentary skeletal remains. The purpose of this study was to establish a systematic method for preparing bone tissues by manual grinding method and offer help in application of related histomorphometric field such as estimating age at death. For this, human bone tissues of rib and femur from cadavers were prepared and considered histomorphological variations of osteon with light microscope. As results of this study, Type II osteons, smaller versions of Intact osteons (Type I) that form by radial remodeling of a preexisting Haversian canal, were distinguished from double-zonal osteons, one of the osteons that exhibit hypercalcified ring within their concentric lamellae, by the lack of an internal reversal line and the parallel contours of lamellae, and it is possible to suggest metric basis for drifting osteons as counting one osteon with the exception of size measurement. By applying this method of present study, one could easily make a bone tissue all oneself that would be helpful for establishing basic data in physical and forensic anthropology.
Bone and Bones ; Cadaver ; Femur ; Forensic Anthropology* ; Haversian System ; Humans ; Ribs

BACKGROUND: Quyyumi et al described circadian variation of ischemic eletrocardio-graphic changes and heart rate in patients with and without significant coronary disease in 1985. Rocco et al also described circadian variation of transient myocaldial ischemia in patients with coronary artery disease. They also noted an increse in ischemic eletrocardiographic abnormalities in the morning hours.In recent years the important of circadian variation of cardiovascular phenomena has generated a great deal of interest. Such phonomena include the occurrence of ischemia in patients with both chronic stable angina and variant angina, myocaldial infarction, sudden cardiac death, ventricular arrhythmia and platelet aggregability. METHOD: To examine whether a significant circadain variation of transient myocardial ischemia exists and to better understand the character of such variation, 35 patients with againa pectoris(15 patients treated with nitrate and/or caclcum channel blocker and 20 patients without treatment) underwent 24 hours ambulatory monitoring of ischemic ST segment changes during daily life. RESULT: Only 21% of the episodes of ST depression were accompanied by chest pain. While 79% were asymptomatic. A significant circadian increase in ischemic activity was found with 44% of episodes and 40% of total ischemic time in not treated group, and 52% of episodes and 56% of total ischemic time in treated group, occuring between 6 A.M. to 12 P.M.(P<0.05 respectively) When the distribution of ischemic episodes was corrected for the variable time of waking, the peak ischemic activity was occuring in the 1st and 2nd hours after rising. There were no significant differences in heart rate at onset and heart rate at 1 min before onset. CONCLUSION: Is summary, transient myocardial ischemia in the absence of pain was more common and there were a significant circadian variation of transient myocardial ischemia with peak activity in the morning. In addition most of transient myocardial were not preceded by a rise in heart rate.
Angina, Stable ; Arrhythmias, Cardiac ; Blood Platelets ; Chest Pain ; Coronary Artery Disease ; Coronary Disease ; Death, Sudden, Cardiac ; Depression ; Heart Rate ; Humans ; Infarction ; Ischemia ; Monitoring, Ambulatory ; Myocardial Ischemia*

PURPOSE: The object of this study was to evaluate entrance angle effects on femoral tunnel length and cartilage damage during anteromedial portal drilling using three-dimensional computer simulation. MATERIALS AND METHODS: Data was obtained from an anatomic study performed using 16 cadaveric knees. The anterior cruciate ligament femoral insertion was dissected and the knees were scanned by computer tomography. Tunnels with different of three-dimensional entrance angles were identified using a computer simulation. The effects of different entrance angles on the femoral tunnel length and medial femoral cartilage damage were evaluated. Specifically, tunnel length and distance from the medial femoral condyle to a virtual cylinder of the femoral tunnel were measured. RESULTS: In tunnels drilled at a coronal angle of 45degrees, an axial angle of 45degrees, and a sagittal angle of 45degrees, the mean femoral tunnel length was 39.5+/-3.7 mm and the distance between the virtual cylinder of the femoral tunnel and the medial femoral condyle was 9.4+/-2.6 mm. The tunnel length at a coronal angle of 30degrees, an axial angle of 60degrees, and a sagittal angle of 45degrees, was 34.0+/-2.9 mm and the distance between the virtual cylinder of the tunnel and the medial femoral condyle was 0.7+/-1.3 mm, which was significantly shorter than the standard angle (p<0.001). CONCLUSION: Extremely low and high entrance angles in both of axial plane and coronal plane produced inappropriate tunnel angles, lengths and higher incidence of cartilage damage. We recommend that angles in proximity to standard angles be chosen during femoral tunnel drilling through the anteromedial portal.
Aged ; Anterior Cruciate Ligament/*surgery ; Anterior Cruciate Ligament Reconstruction/instrumentation/*methods ; Cadaver ; Computer Simulation ; Female ; Femur/anatomy & histology/*surgery ; Humans ; Imaging, Three-Dimensional ; Knee Joint/radiography/*surgery ; Male ; Middle Aged ; Osteotomy/*methods ; Outcome and Process Assessment (Health Care) ; Patient Positioning ; Surgical Instruments ; Tomography, X-Ray Computed

Objectives: To determine whether the clinical aspect of aplastic anemia is influenced by pregnancy. METHODS: We reviewed 37 cases of pregnant aplastic anemia patients during Jan. 1989 to Dec. 1998, and examined age, parity, progress of pregnancy, termination methods, obstetrics & neonatal complications, hematologic change, and treatment modality by medical records. RESULTS: According to onset of disease, patients were divided into pre-pregnant diagnosed group(n=12) and during-pregnancy diagnosed group(n=25). Mean age of diagnosis was 29.4yr, 89.2% were nulliparous, and 51.4% were severe aplastic anemic patients. All patients underwent 50 pregnancy. Mean gestational period was 37wks, birth weight was 2569gram, and, except in 7 cases of abortion, 43 cases were delivered transvaginally or transabdominally(51.2% vs. 48.8%). Preeclampsia, eclampsia, preterm labor, restricted growth, and distress were complicated and decreased hemoglobin, hematocrit, reticulocyte, platelet were reversed after termination in pregnancy associated group. Treatment modality during pregnancy included transfusion, steroid, anti-lymphocytic globulin, anti-thymocytic globulin and IVGV, and remission rate was 45.5% in pregnancy associated group. CONCLUSION: We concluded that pregnancy is associated with aplastic anemia as a high risk factor, and intensive treatment is needed.
Anemia, Aplastic* ; Birth Weight ; Blood Platelets ; Diagnosis ; Eclampsia ; Female ; Hematocrit ; Humans ; Medical Records ; Obstetric Labor, Premature ; Obstetrics ; Parity ; Pre-Eclampsia ; Pregnancy* ; Reticulocytes ; Risk Factors

BACKGROUD: The purpose of our study was to evaluate the usefulness of the ictal semiology, interictal and ictal electro-encephalography(EEG), brain magnetic resonance imaging(MRI), interictal and ictal single photon emission computed tomography(SPECT), positron emission tomography(PET) and neuropsychometry for localization or lateralization of the ictal onset zone, and analyze the result of surgical treatment. METHODS: We evaluated 13 children with intractable temporal lobe epilepsy, who are diagnosed at children's hospital of Seoul National University, from January 1995 to August 1996. The ictal onset zone was determined by ictal semiology, electrophysiologic study, structural and functional neuroimaging, and neuropsychometry. RESULTS: 1) Ictal semiology : Sixty-nine% of the studied children had change of consciousness, 62% had automatism, 31% had aura, 23% had contralateral versive movement, and 23% had contralateral dystonic posturing. Secondarily generalized seizure occured in 38% of them. 2) Interictal EEG showed localized or lateralized value in 8 cases(62%). In interictal EEG, 12 cases(92%) showed epileptiform discharges on the ipsilateral temporal lobe. 3) Magnetic resonance imaging revealed ipsilateral temporal lobe lesion in 8 cases(62%). Interictal SPECT was performed in 6 cases, only one of them had decreased cerebral blood flow on the ipsilateral temporal lobe. Ictal SECT was done in all, 8 cases(67%) had localized or lateralized value. PET was performed in 11 cases, 8 cases(73%) of them had localized or lateralized value. 4) Neuropsychometry was performed in 7 cases, 6 cases(86%) of them had localized or lateralized value. 5) Nine patients underwent epileptic surgery, 6 of them had class I outcome and each one of the rest had class II, class III and class IV. CONCLUSIONS: Electroencephalography, neuropsychometry, MRI, ictal SPECT, and PET are valuable for localization and lateralization of the ictal onset zone, but interictal SPECT is valuless. The outcome after surgery is relatively good, but more extensive study including patient's social, cognitive and emotional status will be necessary.
Automatism ; Brain ; Child* ; Consciousness ; Electroencephalography ; Electrons ; Epilepsy ; Epilepsy, Temporal Lobe* ; Functional Neuroimaging ; Humans ; Magnetic Resonance Imaging ; Seizures ; Seoul ; Temporal Lobe* ; Tomography, Emission-Computed, Single-Photon

No abstract available.
Echocardiography, Transesophageal*

N-acetylcysteine (NAC) is widely recognized as the antidote of choice for acetaminophen overdose. Acetaminophen is a commonly used analgesic and antipyretic agent, and its use is one of the most common causes of poisoning worldwide. Acetaminophen toxicity may occur acutely when supratherapeutic amounts are ingested purposefully or unintentionally. Liver failure may occur in severe toxicity. However, if treated early, patients with acetaminophen poisoning generally recover uneventfully. Acetaminophen is metabolized to N-acetyl-p-benzoquinone imine (NAPQI), which is detoxified by conjugation with glutathione. In overdose, hepatic stores of glutathione are depleted and NAPQI binding to hepatocytes induces cell death and hepatic necrosis. NAC replenishes hepatic glutathione and may also act as a glutathione substitute, combining directly with the toxic metabolite. Intravenous NAC is indicated in patients who present with a history of acetaminophen overdose within the previous 8 to 10 hours, patients unable to tolerate oral NAC, and patients who present with evidence of fulminant hepatic failure. However, caution should be used in patients who have experienced previous hypersensitivity or anaphylactoid reactions to intravenous NAC, as well as in patients with asthma. The most common anaphylactoid reactions include rash, flushing, and bronchospasm. Adults should receive 150 mg/kg administered for 45 minutes, followed by 50 mg/kg administered for 4 hours, followed by 100 mg/kg administered for 16 hours. The total dose is 300 mg/kg delivered over 21 hours. Additionally, caution should always be used when intravenous NAC is prescribed and the amount of diluent is calculated. Monitoring of patients with a should include repeated neurologic and hemodynamic assessment.
Acetaminophen* ; Acetylcysteine* ; Adult ; Antidotes ; Asthma ; Benzoquinones ; Bronchial Spasm ; Cell Death ; Exanthema ; Flushing ; Glutathione ; Hemodynamics ; Hepatocytes ; Humans ; Hypersensitivity ; Imines ; Liver Failure ; Liver Failure, Acute ; Necrosis ; Poisoning*