No abstract available.
De Lange Syndrome*

No abstract available.
Temporal Bone*

The Kniest syndrome is characterized by disproportionate dwarfism and Kyphoscoliosis which may be associated with flat facies with prominent eyes, cleft palate, hearing loss, myopia and limited joint motion. The skeletal abnormalities are recognizable at birth with shortening and deformity of the extremities and stiff joints. Marked lumbar lordosis and kyphoscoliosis develop in childhood, resulting in disproportionate shortening of the trunk. We experienced a case of kniest syndrome, confirmed by clinical features, radiological features, and histological examination of cartilage. A brief review of the related literature is presented.
Animals ; Cartilage ; Cleft Palate ; Congenital Abnormalities ; Dwarfism ; Extremities ; Facies ; Hearing Loss ; Joints ; Lordosis ; Myopia ; Parturition

BACKGROUND: Pacing-induced atrial electrical remodeling (AER) is characterized by shortening of atrial effective refractory period (A-ERP) and its altered rate adaptation. In paroxysmal atrial fibrillation (AF), periods of AF occur with interveneing normal sinus rhythm (NSR) when atria recover from the preceding AER. Previous episodes of AF may precondition the atrial myocardium and cause different time course of AER in subsequent episodes of AF. But the influence of the preceding AER on the subsequent AER has not been described. METHODS: Four mongrel dogs were anesthetized with enflurane. After thoracotomy, silicon band with 3 pairs of electrodes was sutured to the lateral wall of the left atrium. Atrial pacing was performed after 2 wks of recovery and autonomic blockade. Pacing protocol consisted of rapid atrial pacing (RAP) at 500 bpm (for 60 min) and recovery in NSR (for 60 min) which was repeated three times. A-ERP was measured every 10 min. The same pacing protocol was repeated after pretreatment with verapamil (0.1 mg/kg/hr). RESULTS: 1) With 60 min of RAP, A-ERP decreased significantly (126+/-6 ms vs. 105+/-7 ms, p<0.005). 2) After cessation of pacing, A-ERP returned to 98% of baseline value in 15 minutes. Recovery from AER occurred faster than AER (78 vs 21 ms/h). 3) After pretreatment with verapamil, RAP decreased A-ERP from 127+/-5 ms to 116+/-5 ms. AER, the reduction in A-ERP, was significantly attenuated by pretreatment with verapamil (deltaERp=17+/-7 vs. 9+/-0.2 %, p<0.05). 4) When RAPs were repeated, AER showed a tendency of acceleration, but it was not statistically significant (deltaERp=22 ms, 24 ms, 28 ms at the end of 60 min pacing for the 1st, 2nd, 3rd pacing). CONCLUSION: RAP induced AER in conscious dog atria and it was reduced by pretreatment with calcium channel blocking agent, verapamil. Upon repeated atrial stimulations, AER did not accelerate or decelerate when the atria recovered from the preceding AER.
Acceleration ; Animals ; Atrial Fibrillation ; Atrial Remodeling* ; Calcium ; Calcium Channels ; Dogs* ; Electrodes ; Enflurane ; Heart Atria ; Myocardium ; Silicones ; Thoracotomy ; Verapamil

Background: Failure of mitral valve repair sometimes may be ascribed to severe or progressive alteration of the subvalvar apparatus. The aim of this study was to evaluate the effects of new chordae formation on mitral repair. Material and Method: From March 1997 to February 1999, 26 patients underwent mitral valve repairs with new chordae formation, we compared the symptoms and echocardiographic findings checked at preoperative state, and intraoperative period, discharge, and their last OPD visit. There were 15 male , and 11 female patients, and their mean age was 51.2+/-13.4 years. Etiology of the lesions was degenerative (18), rheumatic (6), infective (1) and ischemic (1). Chordal lesions were caused by rupture (18), elongation (6), and a combination of two causes (2). Associated lesions included atrial septal defect (2), tricuspid insufficiency (7), aortic insufficiency(4), and a combination of previous two factors (2). The number of mean artificial chordae was 3.6+/-1.6. Annuloplasty was performed in all cases. The CPB time was 182.1+/-63.7 minutes and the ACC time was 133.1+/-45.6 minutes. Average follow up period was 49.2+/-7.1 months. Result: There was no early death. Early reoperation was performed in two patients, one patient received mitral valve replacement because of an abnormality of annuloplasty and another received pericardiostomy due to postoperative pericardial effusion. During the follow up of 49.2+/-7.1 moths, there was no late mortality. Postoperative NYHA functional class checked at last OPD visit was class I in 22 patients (88%), class II in 2 (8%), and class III in 1 (4%). Regarding the late echocardiogram MR was absent in 20 patients (78%), I in 4 (15%), and II in 1 (4%). The postrepair mitral valve area was 2.2+/-0.35 cm(2). Conclusion: This study suggests that mitral valve repair using new chordae formation provides good early and mid term survivals and functional improvement. We think that the artificial chorda formation with polytetrafluoroethylene suture might be safe and effective technique for mitral valve repair.
Echocardiography ; Female ; Follow-Up Studies ; Heart Septal Defects, Atrial ; Humans ; Intraoperative Period ; Male ; Mitral Valve* ; Mortality ; Moths ; Pericardial Effusion ; Pericardial Window Techniques ; Polytetrafluoroethylene ; Reoperation ; Rupture ; Sutures

We present a case of an 8 year-old girl with a large aneurysmal bone cyst (ABC) of the 4th lumbar vertebra that involved the adjacent posterior element and which was treated by an en bloc excision. An aneurysmal bone cyst is an uncommon lesion. Among lesions that involve the extremities, standard treatment with curettage and a bone graft or other alternatives, such as embolization and radiation therapy, may be sufficient; but those that involve the spinal column require special consideration because of its anatomical and biomechanical complexity. We removed a large aneurysmal bone cyst from the posterior element of the lumbar spine with an en bloc excision without fusion or instrumentation and the clinical result was satisfactory. At the 1-year follow-up, there was no evidence of clinical recurrence or radiological instability. We describe a case of a large aneurysmal bone cyst of a lumbar vertebra of a child and present a review of the pertinent literature.
Aneurysm* ; Bone Cysts* ; Child* ; Curettage ; Extremities ; Female ; Follow-Up Studies ; Humans ; Recurrence ; Spine ; Transplants

BACKGROUND: Unlike patterns observed in the general population, obesity is associated with better survival among hemodialysis patients, which could be explained by reverse causation or illness-related weight loss. However, the time-varying effect of body mass index (BMI) on hemodialysis survival has not been investigated. Therefore, this study investigated the time-varying effect of BMI on mortality after starting hemodialysis. METHODS: In the present study, we examined Korean Society of Nephrology data from 16,069 adult patients who started hemodialysis during or after the year 2000. Complete survival data were obtained from Statistics Korea. Survival analysis was performed using Cox regression and a non-proportional hazard fractional polynomial model. RESULTS: During the median follow-up of 8.6 years, 9,272 patients (57.7%) died. Compared to individuals with normal BMI (18.5–24.9 kg/m²), the underweight group (< 18.5 kg/m²) had a higer mortality hazard ratio (HR, 1.292; 95% confidence interval [CI], 1.203–1.387; P < 0.001) and the overweight group (25.0–29.9 kg/m²) had a lower mortality HR (0.904; 95% CI, 0.829–0.985; P = 0.022). The underweight group had increasing HRs during the first 3 to 7 years after starting hemodialysis, which varied according to age group. The young obese group (< 40 years old) had a U-shaped temporal trend in their mortality HRs, which reflected increased mortality after 7 years. CONCLUSION: The obese hemodialysis group had better survival during the early post-dialysis period, although the beneficial effect of obesity disappeared 7 years after starting hemodialysis. The young obese group also had an increased mortality HR after 7 years.
Adult ; Body Mass Index ; Follow-Up Studies ; Humans ; Korea ; Models, Statistical ; Mortality ; Nephrology ; Obesity ; Overweight ; Renal Dialysis ; Thinness ; Weight Loss

BACKGROUND: Transcranial Doppler ultrasonography (TCD) can detect microembolic signals (MES) in the patients with a potential embolic source. Clinical significance of MES has not been demonstrated in patients with prosthetic mechanical heart valves. We studied the correlation between cerebral thromboemoblic events after the mechanical heart valve surgery (MHVS) and residual MES during TCD monitoring with 100% oxygen inhalation in patients with mechanical heart valves. MATERIAL AND METHOD: Twenty patients with previous cerebral thromboemoblic events after MHVS and a sex- and age-matched control group (n=30) were studied. TCD monitoring was performed from unilateral middle cerebral artery. After baseline monitoring for 20 minutes, 6L of oxygen was inspired for 40 minutes. RESULT: The site of valve and the duration after MHVS of the patients did not differ from those of controls. During baseline monitoring, there was no significant difference in MES prevalence or counts compared to controls. During oxygen inhalation, patients showed a higher MES prevalence (55%, 27.6%, p=0.045) and a more frequent MES counts (p=0.027) compared to controls. CONCLUSION: TCD monitoring with oxygen inhalation may be useful to differentiate clinically significant MES in patients with mechanical heart valve.
Embolism ; Heart Valve Prosthesis ; Heart Valves* ; Heart* ; Humans ; Inhalation* ; Middle Cerebral Artery ; Oxygen* ; Prevalence ; Ultrasonography ; Ultrasonography, Doppler, Transcranial

Fusobacteria are anaerobic gram-negative, non-spore forming bacilli found in normal flora of the oral cavity, urogenital tract, and gastrointestinal tract. Fusobacterium nucleatum has been seldom reported as a cause of liver abscess, particularly in immunocompetent hosts. A 55-year-old man with frequent periodontal disease visited our hospital with intermittent fever and headache for 2 months. Abdominal CT scan revealed an 8.2x6 cm mass in the right hepatic lobe with central low density. Abscess culture revealed F. nucleatum as the causative organism. Percutaneous abscess drainage and intravenous administration of antibiotics for 4 weeks improved symptoms and decreased the abscess size. We report a rare case of liver abscess due to F. nucleatum in an immunocompetent man with periodontal disease.
Ampicillin/therapeutic use ; Anti-Bacterial Agents/therapeutic use ; Fusobacterium Infections/complications/*diagnosis/drug therapy ; Fusobacterium nucleatum/*isolation & purification ; Humans ; Injections, Intravenous ; Liver Abscess/*diagnosis/etiology/microbiology ; Male ; Middle Aged ; Periodontal Diseases/*diagnosis ; Sulbactam/therapeutic use

BACKGROUND: Many autopsy studies have shown that the extent of extracranial carotid and coronary artherosclerosis is correlated and B-mode ultrasonographic intima-media thickness(IMT) and histologic IMT have been good correlation. In recent years. as it has been reported that IMT of carotid artery had something to do with risk factors of atherosclerosis and occurrence of coronary artery disease, in this study, we tried to investigate if the grade of atherosclerosis in B-mode ultrasonography of carotid artery could predict coronary artery disease and have something to do with the severity of coronary artery disease. METHODS: We classified the patients who were examined coronary angiography into control group without significant(>50%) stenosis(11 patients) and coronary artery disease(CAD) group(45 patients) according to the existence of significant stenosis, and we subdivided CAD group into single vessel disease(SVD) group(25 patients) and multivessel disease(MVD) group(20 patients). Practicing B-mode ultrasonography of common carotid artery(CCA), carotid artery bifurcation(BIF) and internal carotid artery(ICA), we measured IMT and IMT/L(lumen diameter) of each segment. Adding all values of each segment, we got mean aggregated IMT and mean aggregated IMT/L. RESULTS: 1) As IMT of left BIF in both six segments, control group was 0.55+/-0.16mm, SVD group was 0.71+/-0.36mm and MVD group was 1.02+/-0.61mm. So compared with control group and SVD, MVD group were significantly thick. As IMT/L, control group was 0.07+/-0.02, SVD group 0.08+/-0.05 and MVD group was 0.13+/-0.08. So compared with control group and SVD, MVD group was ignificantly high. 2)IMT of BIF in three segments, control group was 0.59+/-0.16mm, CAD group was 0.82+/-0.47mm and MVD group was 0.90+/-0.54mm. So compared with control group and CAD, MVD group were significantly thick. Also as IMT/L of BIF, compared with control group(0.07+/-0.02) and CAD(0.10+/-0.06), MVD(0.11+/-0.07) group was high.= 3) As mean aggregated IMT, control group was 0.57+/-0.34mm, CAD group was 0.69+/-0.45mm, SVD group was 0.63+/-0.12mm and MVD group was 0.74+/-0.21mm. So CAD group was thicker than control group and MVD group was thicker than SVD group. As mean aggregated IMT/L, control group was 0.07+/-0.03, CAD group was 0.10+/-0.05, SVD group was 0.09+/-0.01 and MVD group was 0.11+/-0.03. So CAD group was higher than control group and MVD group was higher than SVD group. CONCLUSION: These data support use of the mean aggregated B-mode ultrasonographic IMT and IMT/L in carotid bifurcation for correlation with the status of coronary atherosclerosis.
Atherosclerosis ; Autopsy ; Carotid Arteries* ; Constriction, Pathologic ; Coronary Angiography ; Coronary Artery Disease* ; Coronary Vessels* ; Humans ; Risk Factors ; Ultrasonography