Apert syndrome, or acrocephalosyndactyly is a complex of associated malformations, which are craniosynostosis and symmetric complex syndactyly of the hands and feet. The syndrome was first described by Apert in 1906. Review of the worlds literature disclosed about 200 similar cases and 9 Cases in Korea. Authors experienced one case of Apert syndrome and treated surgically for acro-syndactyly and polydactyly of both hands and feet. The case was compared with the other previously reported cases in Korea.
Acrocephalosyndactylia ; Craniosynostoses ; Foot ; Hand ; Korea ; Polydactyly ; Syndactyly

BACKGROUND: Balloon mitral valvuloplasty is a favorable procedure as a therapy for mitral stenosis because it minimizes morbidity and shorten hospital stay compared with surgical mitral commissurotomy or mitral valve replacement. Recent reports about concomitant transesophageal echocardiography guide in addition to fluoroscopy suggest that transesophageal echocardiograpy can provide additional benefits during balloon mitral valvuloplasty especially in transseptal puncture, balloon positioning, evaluation of immediate result, and early detection of complications. We performed this study to identify the potential benefits of on-line transesophageal echocardiography guide during balloon mitral valvuloplasty. METHOD: We performed balloon mitral valvuloplasty under on-line transesophageal echocardiography guide in addition to fluoroscopy in 70 patients(male:14, female:56, mean age:44+/-13) with rheumatic mitral stenosis from May 1995 to May 1996. Thirty-two(46%) patients had atrial fibrillation. Included patients were symptomatic with more than NYHA class 2 symptom. Patients with mitral valve score more than 11 and mitral regurgitation more than 2/4 were excluded. Inoue balloons were utilized in all cases. RESULTS: The average mitral valve area increased from 0.9+/-0.2cm2 before valvuloplasty to 1.8+/-0.4cm2 after valvuloplasty(p<0.0001). The averagetransmitral pressure gradient measured by continuous wave Doppler decreased from 14+/-6mmHg before valvuloplasty to 5+/-2mmHg after valvuloplasty(p<0.0001), and the average left atrial pressure measured by catheterization decreased form 22+/-8 mmHg before valvuloplasty to 11+/-5mmHg after valvuloplasty(p<0.0001). The average procedure time was 64+/-22 minutes(ranged from 13 to 150 minutes) and the average fluoroscopy time was 19+/-15 minutes(ranged from 1 to 94 minutes). Two patients underwent surgery due to severe mitral regurgitation associated with papillary muscle rupture which developed after valvuloplasty. In one patient, transesophageal echocardiography detected pericaridal tamponade during the procedure and the transducer was quickly switched to transthoracic transducer to guide the pericardial puncture site. The pericardial tamponade was drained with pigtail catheter and the patient underwent balloon mitral valvuloplasty successfully a week later. Four patients were pregnant at the time of the valvuloplasty procedure and the valvuloplasty was successfully performed with minimal fluoroscopy time(1-3 minutes) without complications in all four patients. Five patients had thrombus in left atrial appendage, but the transesophageal echocardiography was useful in monitoring the ballon position during the procedure and the valvuloplasty was successfully performed without embolic complications in all five patients. CONCLUSION: The transesophageal echocardiography is a very useful guiding adjunct during balloon mitral valvuloplasty in transseptal puncture, balloon positioning, evaluation of immediate result, early detection of complications, and shortening fluoroscopy time especially in pregnant women.
Atrial Appendage ; Atrial Fibrillation ; Atrial Pressure ; Cardiac Tamponade ; Catheterization ; Catheters ; Echocardiography, Transesophageal* ; Female ; Fluoroscopy ; Humans ; Length of Stay ; Mitral Valve ; Mitral Valve Insufficiency ; Mitral Valve Stenosis ; Papillary Muscles ; Pregnant Women ; Punctures ; Rupture ; Thrombosis ; Transducers

BACKGROUND: Percutaneous mitral balloon valvuloplasty(PMV) is a good treatment modality for patient with mitral stenosis(MS). But it is considered relatively contraindicated in patients with left artrial thrombi because of high risk of embolism. Limitted studies have suggested the feasibility of PMV in patients with left atrial appendage(LAA) thrombi. This study was performed to evaluate the feasibility and safty of PMV in patients with LAA thrombi using Inoue balloon under the transesophageal echocardiographic (TEE) monitoring. METHOD: PMV was performed in 5 patients diagnosed as MS with LAA thrombi from October, 1995 to July, 1996. Four cases were female, and one case was male. Their mean age was 525(46-58years old). Two of them had history of cerebrovascular accident(CVA). The duration of anticoagulant treatment was 6-49 moths. All patients underwent PMV using Inoue balloon catheter under the TEE monitoring. RESULTS: EKG finding of all 5 patients were atrial fibrillation(Af). Their mitral valve score were 5-10(Mean score was 82). Transmitral mean pressure gradient was decreased from 14.62.1 to 5.82.0mmHg, and mitral valve increased from 0.840.43 to 1.720.19 after PMV. There was no procedure related complication. In 3 cases of them LAA thrombi diappeared in the follow up TEE. In two patients, the LAA thrombi were calcified and remained unresolved at the time of follow up TEE( 6month-and 12 month-F/U, eath). CONCLUSION: Although the reported number of PMV in patients with LAA thrombi is small in this study, we believe that, with special precaution and TEE monitoring, LAA thrombi is no longer an absolute contraindication to PMV.
Atrial Appendage* ; Balloon Valvuloplasty* ; Catheters ; Echocardiography ; Electrocardiography ; Embolism ; Female ; Follow-Up Studies ; Humans ; Male ; Mitral Valve ; Moths

The mechanism of cytolysis by complement attack of nucleated cells(NC) is of special interest in comparison to that of red blood cells. It is known that NC death by membrane attack comples, C5b-9, is caused by many factors, i.e., efficiency of complex assembly, activation of intrinsic metabolic pathway by signal transduction, cytotoxic effect of the channel itself and natural repair ability. These factors suggest that colloid osmotic lysis, known in red blood cells, does not fully explain the complement-mediated cell death of NC. In this study, the authors investigated correlation between biochemical and morphological changes to prove "Ca2+-mediated metabolic death"8~13) representing a mechanism of NC death caused by C5b-9 attack. The L1210 cells, mouse leukemic cell line carrying small complement channel(TAC5b-91) were used in the experiments. The amounts of intracellular adenine nucleotides to extracellular Ca2+, ouabain, KC1 and dextran were analyzed by bioluminescence method using luminometer. Cell viability was checked by 0.4% trypan blue dye and LDH release. Morphological observation of TAC5b-91 was done by immunocytochemical staining and electron microscope. The results were as follows: 1) The release of ATP, ADP and AMP followed by cell death was rapid and progressive along the incubation time at 37 degrees C and it was accelerated in 1.5 mM of [Ca2+]0. 2) There was no evidence of ATP repairment in the TAC5b-91. 3) Extracellular KC1(150 mM), dextran(0.66 mM) and ATP supplement(0.2 microM) could not effectively inhibit ATP depletion and cell death. Ouabain(27 and 100 microM) enhanced cell death and could not completely prevent ATP loss. 4) Most of the mitochondria showed swelling, loss of cristae and Ca2+ deposit in matrix in the electron microscopic observation. Rapid, sustained and irreversible depletion of adenine nucleotides was due to Ca2+ deposit with destruction of mitochondria and also the leakage through transmembrane channels. Moreover this energy depletion was accelerated by high extracellular Ca2+ concentration. These results indicate that Ca2+-mediated, energy exhaustion is one of the mechanisms of the metabolic cell death by C5b-9 attack of NC.
Mice ; Animals

BACKGROUND: To evaluate the influence of changes in mitral valve area (MVA) and left atrial pressure on pulmonary vein flow (PVF) we analyzed PVF with transesophageal echocardiography (TEE) before, after and one-year after percutaneous mitral valvuloplasty (PMV) in patients with mitral stenosis (MS) in sinus rhythm. METHODS: PMV was guided with TEE. Follow-up TEE was done about one year after PMV. MVA and transmitral mean gradient (TMG) were measured. Systolic velocity (S), diastolic velocity (D), atrial reversal velocity (AR), their time-velocity integral (S-TVI, D-TVI, AR-TVI) and their ratio (S/D ratio, S-TVI/D-TVI ratio were evaluated. RESULTS: The number of patients was twenty-two (F:20). The age was 39+/-9 years (range:26-64). Follow-up duration was 16+/-6 months (range:7-28). MVA increased from 0.9+/-0.2 cm2 to 1.9+/-0.3 cm2 after PMV and decreased to 1.7+/-0.3 cm2 on follow-up TEE significantly. TMG decreased from 15.4+/-4.3 mmHg to 5.5+/-1.9 mmHg after PMV and was 6.2+/-2.4 mmHg on follow-up. S increased significantly on follow-up at both pulmonary vein (PV). D increased on follow-up at left PV. S/D ratio increased on follow-up at both PV. AR increased on follow-up at both PV. S-TVI increased after PMV at left PV and increased on follow-up at both PV. D-TVI had no change. S-TVI/D-TVI ratio increased on follow-up at left PV. AR-TVI increased on follow-up at right PV. CONCLUSIONS: The main changes after PMV in patients with MS in sinus rhythm were increasing tendency in S, S-TVI, S/D ratio, S-TVI/D-TVI ratio and AR. And these changes were statistically significant on follow-up TEE rather than immediately after PMV.
Atrial Pressure ; Echocardiography* ; Echocardiography, Transesophageal ; Follow-Up Studies ; Humans ; Mitral Valve ; Mitral Valve Stenosis* ; Pulmonary Veins

Small and large airways functions were studied in patients with hypertensive heart disease in slightly ro moderately compromised state functionally. In this study, the forced vital capacity and various flow paramaeters reflecting expiratory flow rate were determined from simultaneously recorded forced expiratory volume and maximal expiretory flow volume curves in 86 cases. The closing volume was measured by a single breath nitrogen mrthod in 57 cases and airway resistance with its related parameters by a body plethysmograph in 11 cases. These results were compared with those obtained from the same numbers of healthy controls matched for sex, age and height. In the patient group, the forced vital capadity and all the observed values of flow parameters, execpt for the ratio of the first second vital capacity to the forced vital capacity, were significantly reduced than those in the controls. When the remainder of flow parameters was volume-adjusted to the forced vital capacity, however, the mean of the peak expiratory flow rate and the maximal expiratory flow rate at the 75 percent of the vital capacity were not significantly different from that of controls. In contrast, the volume-adjusted values of maximal expiratory flow were remained significantly smaller than those in the controls. The closing volume and its ratio to the vital capacity were significantly larger in the patient group. Airway resistance and its related parameters revealed no significant differences between two groups. These findings suggest that the patients with hypertensive heart disease in a mild to moderate failure are associated with restrictive ventilatory impairment and a small airways obstruction, but with little or no large airway dysfunction.
Airway Resistance ; Closing Volume ; Forced Expiratory Volume ; Heart Diseases* ; Heart* ; Humans ; Maximal Expiratory Flow Rate ; Nitrogen ; Peak Expiratory Flow Rate ; Vital Capacity

BACKGROUND: Ischemic preconditioning reduces the infarct size and the severity of arrhythmia in a post-ischemic reperfused heart although the detailed mechanism is unknown. In the ischemic heart, a large amount of catecholamine is released from the adrenergic nerve terminal and this aggravates cell destruction and arrhythmia. In this study, the possibility for ischemic preconditioning to inhibit the release of endogenous catecholamine from the ischemic heart was tested to investigate the probable cardioprotective mechanism of ischemic preconditioning. METHODS: In the isolated, Langendorff perfused rat hearts, we observed the protective effect of ischemic preconditioning against post-ischemic reperfusion injury, and measured the amount of catecholamine released into coronary effuent. In addition, we observed the effect of catecholamine depletion on reperfusion injury in non-preconditioned and preconditioned hearts. RESULTS: During the reperfusion(20min) after ischemia(30min), the cardiac function was markedly depressed with the development of severe contracture. In the heart preconditioned by three sequential episodes of 5min ischemia and 5min reperfusion, the reperfusion contracture decreased significantly and the cardiac function was almost recovered to normal after 20min reperfusion. The release of lactate dehydrogenase was also decreased in the preconditioned heart. The release of endogenous catecholamine was abruptly increased immediately after the reperfusion and the release was exponentially decreased throughout the reperfusion period. THe pattern of catecholamine release was much different from that of lactate dehydrogenase release. In the preconditioned heart, the release was significantly decreased to about half of that in non-preconditioned t\heart. Endogenous catecholamine depletion by reserpine treatment did not affect the post-ischemic functional recovery in both non-preconditioned and preconditioned hearts. CONCLUSION: It is suggested from these results that ischemic preconditioning inhibis the release of endogenous catecholamine during ischemic period, which may be partly related to cardioporotective effect of preconditioning in ischemic and reperfused heart.
Animals ; Arrhythmias, Cardiac ; Contracture ; Heart* ; Ischemia ; Ischemic Preconditioning* ; L-Lactate Dehydrogenase ; Rats* ; Reperfusion ; Reperfusion Injury ; Reserpine

No abstract available.
Surveys and Questionnaires*

Ulcerative colitis is a chronic, inflammatory disease of colon which is rare in Korea. In the course of ulcerative colitis numerous extraintestinal complication may develop on the joints, skin, liver, kidney, vessel, oral cavity and eyes. The most common ocular lesions complicated in patients with ulcerative colitis are episcleritis and iritis. And the cases of retinal vascular disease in associated with ulcerative colitis are extremely rare. We experienced nonischemic central retinal vein occlusion complicated in a patient with ulcerative colitis, who is 32-year-old male and complained decreased visual acuity and metamorphosis.
Adult ; Colitis, Ulcerative ; Colon ; Humans ; Iritis ; Joints ; Kidney ; Korea ; Liver ; Male ; Mouth ; Retinal Vein* ; Retinaldehyde ; Scleritis ; Skin ; Ulcer* ; Vascular Diseases ; Visual Acuity

The purpose of this retrospective study was to evaluate the efficacy of arthroscopic repair of Bankart lesion using the biodegradable polyglyconate implant(Suretac) for the treatment of traumatic anterior shoulder instability. Although the arthroscopic procedure using the Suretac device has some technical advantages over others, there have been reports of the higher failure rate than open procedure. Eight shoulders in 8 patients who had traumatic anterior instability of the shoulder with Bankart lesion were managed with this procedure. They were followed up for average 2 years (range 1 year 4 months to 2 years 5 months). During the follow-up period, all the patients showed full range of motion of the shoulder without recurrence of instability. It was our impression that success rate of the procedure could be improved by careful selection of the patient, the accurate arthroscopic technique, and the good rehabilitation program.
Follow-Up Studies ; Humans ; Range of Motion, Articular ; Recurrence ; Rehabilitation ; Retrospective Studies ; Shoulder*