BACKGROUND: We compared the clinical outcomes of cardiac valve surgery in adult Jehovah's Witness patients refusing blood transfusion to those in non-Jehovah's Witness patients without any transfusion limitations. METHODS: From 2005 to 2014, 25 Jehovah's Witnesses (JW group) underwent cardiac valve surgery using a blood conservation strategy. Twenty-five matched control patients (non-JW group) were selected according to sex, age, operation date, and surgeon. Both groups were managed according to general guidelines of anticoagulation for valve surgery. RESULTS: The operative mortality rate was 4.0% in the JW group and 0% in the non-JW group (p = 1.000). There was no difference in postoperative major complications between the groups (p = 1.000). The overall survival rate at 5 and 10 years was 85.6% ± 7.9% and 85.6% ± 7.9% in the JW group, respectively, and 100.0% ± 0.0% and 66.7% ± 27.2% in the non-JW group (p = 0.313). The valve-related morbidity-free survival rates (p = 0.625) and late morbidity-free survival rates (p = 0.885) were not significantly different between the groups. CONCLUSIONS: Using a perioperative strategy for blood conservation, cardiac valve surgery without transfusion had comparable clinical outcomes in adult patients. This blood conservation strategy could be broadly applied to major surgeries with careful perioperative care.
Adult ; Blood Transfusion ; Bloodless Medical and Surgical Procedures* ; Heart Valves* ; Humans ; Jehovah's Witnesses* ; Mortality ; Perioperative Care ; Survival Rate

No abstract available.
Mediastinal Emphysema*

Here, we report the case of a 43-year-old female who was diagnosed with a cotyledonoid dissecting leiomyoma (CDL) of the uterus. CDL is a recently described and extremely rare variant of a benign leiomyoma that can grossly masquerade as a malignancy. The 13-cm sized tumor was located primarily on the extrauterine surface as an intrauterine continuity, which showed dark red, congested, bulbous protuberances. It was multinodular appearance, encasing the bilateral adnexae and the left iliac vein. Microscopically, the nodules were separated by extensive hydropic degeneration. The nodules were composed of cigar-shaped spindle cells with no mitosis, cellular pleomorphism or coagulation necrosis. They also showed an intravascular luminal growth pattern. CDL with intravascular growth was diagnosed after excluding intravascular leiomyomatosis, disseminated peritoneal leiomyomatosis, and benign metastasizing leiomyoma. The present case is the second reported case of CDL in Korea. Recognition of this rare and bizarre, malignancy-mimicking leiomyoma is crucial to prevent inappropriate treatment.
Adult ; Estrogens, Conjugated (USP) ; Female ; Humans ; Iliac Vein ; Korea ; Leiomyoma* ; Leiomyomatosis ; Mitosis ; Necrosis ; Phenobarbital* ; Uterus*

BACKGROUND: We compared the clinical outcomes of cardiac valve surgery in adult Jehovah's Witness patients refusing blood transfusion to those in non-Jehovah's Witness patients without any transfusion limitations. METHODS: From 2005 to 2014, 25 Jehovah's Witnesses (JW group) underwent cardiac valve surgery using a blood conservation strategy. Twenty-five matched control patients (non-JW group) were selected according to sex, age, operation date, and surgeon. Both groups were managed according to general guidelines of anticoagulation for valve surgery. RESULTS: The operative mortality rate was 4.0% in the JW group and 0% in the non-JW group (p = 1.000). There was no difference in postoperative major complications between the groups (p = 1.000). The overall survival rate at 5 and 10 years was 85.6% ± 7.9% and 85.6% ± 7.9% in the JW group, respectively, and 100.0% ± 0.0% and 66.7% ± 27.2% in the non-JW group (p = 0.313). The valve-related morbidity-free survival rates (p = 0.625) and late morbidity-free survival rates (p = 0.885) were not significantly different between the groups. CONCLUSIONS: Using a perioperative strategy for blood conservation, cardiac valve surgery without transfusion had comparable clinical outcomes in adult patients. This blood conservation strategy could be broadly applied to major surgeries with careful perioperative care.
Adult ; Blood Transfusion ; Bloodless Medical and Surgical Procedures ; Heart Valves ; Humans ; Jehovah's Witnesses ; Mortality ; Perioperative Care ; Survival Rate

Indication for extracorporeal membrane oxygenation (ECMO) has been extended as the experience of ECMO in various clinical settings accumulates and the outcome after ECMO installation improves. We report two cases of successful mitral valve surgery for severe ischemic mitral regurgitation in patients on ECMO support for cardiogenic shock which developed upon coronary angiography.
Coronary Angiography ; Coronary Artery Disease ; Extracorporeal Membrane Oxygenation ; Humans ; Mitral Valve ; Mitral Valve Insufficiency ; Shock, Cardiogenic

The hypokalemic periodic paralysis is characterized by intermittent falccid paralysis of extremities with spontaneous recovery. It is rarely accompanied by cardiac arrhythmia, especially fatal ventricular tachycardia or torsades de pointes. We observed a 29 year old man, who had suffered from intermittent periodic paralysis and fatal ventricular tachyarrhythmia. He had the first episode of muscle weakness in his low grade of elementary school, which lasted for 20 -30 hours. Similar episodes of muscle weakness occurred 1 -7 times per year, especially after carbohydrate rich food. On admission to emergency room, his chief complaints were generalized weakness and chest tightness, serum potassium level was 1.6mEq/l, and four extremities showed Grade 0 motor weakness. His electrocardiography(ECG) showed Atrioventricular dissociation due to sinus tachycardia and accelerated junctional rhythm, intraventricular conduction distrubance. During intravenous potassium administration, ECG showed sustained ventricular tachycardia and cardiovascular collapse occurred. So we carried out resuscitation and cardioversion. After resuscitation, he recovered from cardovascular collapse and ECG showed sinus tachycardia. But during continuous monitoring ECG showed torsades de pointes with cardiovascular collapse. We carried out resuscitation and defibrillation repeatedly. Serum potassium level was 1.7 - 1.8mEq/L at that time. After successful resuscitation, ECG showed sinus rhythm, and his mental status was fully recovered. After he admitted to intensive care unit, paralytic attack and cardiac arrhythmia did not occurred any more. Serum potassium level was maintained between 3.9 -6.1lmEq/L during his hospital days. He was fully recovered but could not take any medications(e.g. acetazolamide, potassium supplying agent and antiarrhythmic drugs) due to severe gastrointestinal disturbances. During the 30 months of postdischarge period, he experienced three mild paralysis attacks, but they were not accompanied by chest tightness, palpitation or syncope.
Acetazolamide ; Adult ; Arrhythmias, Cardiac* ; Electric Countershock ; Electrocardiography ; Emergency Service, Hospital ; Extremities ; Heart Block ; Humans ; Hypokalemia ; Hypokalemic Periodic Paralysis* ; Intensive Care Units ; Muscle Weakness ; Paralysis ; Potassium ; Resuscitation ; Syncope ; Tachycardia ; Tachycardia, Sinus ; Tachycardia, Ventricular ; Thorax ; Torsades de Pointes

PURPOSE: The purpose of this study was to implement a software to visualize tumor and its surrounding fiber tracts simultaneously using diffusion tensor imaging and examine the feasibility of our software for investigating the influence of tumor on its surrounding fiber connectivity. MATERIALS AND METHODS: MR examination including T1-weigted and diffusion tensor images of a patient with brain tumor was performed on a 3.0 T MRI unit. We used the skull-striped brain and segmented tumor images for volume/surface rendering and anatomical information from contrast-enhanced T1-weighted images. Diffusion tensor images for the white matter fiber-tractography were acquired using a SE-EPI with a diffusion scheme of 25 directions. Fiber-tractography was performed using the streamline and tensorline methods. To correct a spatial mismatch between T1- weighted and diffusion tensor images, they were coregistered using a SPM. Our software was implemented under window-based PC system. RESULTS: We successfully implemented the integrated visualization of the fiber tracts with tube-like surfaces, cortical surface and the tumor with volume/surface renderings in a patient with brain tumor. CONCLUSION: Our result showed the feasibility of the integrated visualization of brain tumor and its surrounding fiber tracts. In addition, our implementation for integrated visualization can be utilized to navigate the brain for the quantitative analysis of fractional anisotropy to assess changes in the white matter tract integrity of edematic and peri-edematic regions in a number of tumor patients.
Anisotropy ; Brain Neoplasms* ; Brain* ; Diffusion ; Diffusion Tensor Imaging ; Humans ; Magnetic Resonance Imaging

Jessner's lymphocytic infiltration of the skin (JLIS) is a well-known but poorly understood disorder. Some doubt still exists about whether it is a distinct disease or a variant of lupus erythematosus or, less commonly, polymorphous light eruption, cutaneous lymphoid hyperplasia. An effective therapy is still unavailable for JLIS. We report a patient with JLIS which was successfully treated with dapsone and intermittent systemic glucocorticoid therapy.
Dapsone ; Humans ; Hyperplasia ; Light ; Skin

Ring chromosome is a structural abnormality that is thought to be the result of fusion and breakage in the short and long arms of chromosome. Wolf-Hirschhorn syndrome (WHS) is a well-known congenital anomaly in the ring chromosome 4 with a partial deletion of the distal short arm. Here we report a 10-month-old male of mosaic ring chromosome 4 with the chief complaint of severe short stature. He showed the height of -4 standard deviation, subtle hypothyroidism and mild atrial septal defect/ventricular septal defect, and also a mild language developmental delay was suspected. Brain magnetic resonance imaging showed multifocal leukomalacia. Chromosomal analysis of the peripheral blood showed the mosaic karyotype with [46,XY,r(4)(p16q35)[84]/45,XY,-4[9]/91,XXYY, dic r(4;4)(p16q35;p16q35)[5]/46,XY,dic r(4;4)(p16q35;p16q35)[2]]. FISH study showed the deletion of the 4p subtelomeric region with the intact 4q subtelomeric and WHS region. Both paternal and maternal karyotypes were normal. We compared the phenotypic variation with the previously reported cases of ring chromosome 4. The ring chromosome 4 with the subtelomeric deletion of short arm seems to be related with the phenotype of short stature.
*Chromosome Deletion ; *Chromosomes, Human, Pair 4 ; Growth Disorders/*diagnosis/genetics ; Humans ; In Situ Hybridization, Fluorescence ; Infant ; Karyotyping ; Male ; *Ring Chromosomes ; Telomere

Lichenoid drug eruption can arise as a result of exogenous compound exoposures and closely mimic idiopathic lichen planus. A 45-year-old man who had taken antituberculosis drugs (isoniazid, rifampin, ethambutol, pyrazinamide) for 2 months developed pruritic violaceous papules and plaques with silvery scales on the whole body. On close inspection, he had superficial erosions on the lips and reticulate white papules on the buccal mucosa. Histopathologic findings were hyperkeratosis, parakeratosis, and hypergranulosis in the epidermis and band-like lymphohistiocytic infiltration, numerous eosinophils, and perivascular lymphohistiocytic infiltration in the dermis. The skin lesions improved leaving hyperpigmentation after cessation of the antituberculosis drugs and application of topical corticosteroid.
Dermis ; Drug Eruptions ; Eosinophils ; Epidermis ; Ethambutol ; Humans ; Hydrazines ; Hyperpigmentation ; Lichen Planus ; Lip ; Middle Aged ; Mouth Mucosa ; Parakeratosis ; Rifampin ; Skin ; Weights and Measures