No abstract available.
Mitral Valve* ; Quinidine*

The clinical presentation of pheochromocytoma is variable. The classic symptoms are headache, diaphoresis, and tachycardia, with paroxysmal hypertension. Other less common cardiovascular manifestations, such as arrhythmias, angina pectoris, acute myocardial infarction, dilated cardiomyopathy, and acute heart failure, have been reported occasionally. We present the case of a middle-aged woman who had stress-induced cardiomyopathy with a left ventricular thrombus, due to the pheochromocytoma. The thrombus was embolized to the aorto-iliac bifurcation during hospitalization. We removed the thrombus by a catheter thromboembolectomy and performed a surgical left adrenalectomy. After the operation, all of her symptoms and the underlying diseases (hypertension, hyperglycemia, heart failure, dyslipidemia) resolved.
Adrenalectomy ; Angina Pectoris ; Arrhythmias, Cardiac ; Cardiomyopathies* ; Cardiomyopathy, Dilated ; Catheters ; Female ; Headache ; Heart Failure ; Hospitalization ; Humans ; Hyperglycemia ; Hypertension ; Myocardial Infarction ; Pheochromocytoma* ; Tachycardia ; Thrombosis*

Fabry disease is a progressive X-linked disorder of glycosphingolipid metabolism caused by a deficiency of the alpha-galactosidase lysosomal enzyme. The partial or complete deficiency of the lysosomal enzyme leads to an accumulation of neutral glycosphingolipids in the vascular endothelium and visceral tissues throughout the body. In the heart, glycosphingolipids deposition causes progressive left ventricular hypertrophy (LVH). We report a case of Fabry disease which was suspected based upon two-dimensional echocardiographic finding of LVH. A 44-year-old man was admitted to evaluation of aggravated exertional dyspnea of two weeks duration. He had been diagnosed with end-stage renal disease of unknown etiology at age 41 followed by renal transplantation that year. He had been treated with oral immunosuppressive agents. On hospital day two, transthoracic echocardiography revealed concentric LVH. Left ventricular systolic function was preserved but diastolic dysfunction was present. Fabry disease was confirmed by demonstration of a low plasma alpha-galactosidase A (alpha-Gal A) activity. Analysis of genomic DNA showed alpha-Gal A gene mutation. The patient was diagnosed with Fabry disease.
alpha-Galactosidase ; Cardiomyopathies ; DNA ; Dyspnea ; Echocardiography ; Endothelium, Vascular ; Fabry Disease ; Genes, vif ; Glycosphingolipids ; Heart ; Humans ; Hypertrophy, Left Ventricular ; Immunosuppressive Agents ; Kidney Failure, Chronic ; Kidney Transplantation ; Neutral Glycosphingolipids ; Plasma

BACKGROUND AND OBJECTIVES: The combination of platelet glycoprotein IIb/IIIa inhibitors and a low dose thrombolytic agent may produce early Thrombolysis In Myocardial Infarction (TIMI) 3 flow and a high rate of ST elevation resolution in an ST elevation acute myocardial infarction (STEMI). The clinical effect of tirofiban combined with low dose alteplase, prior to primary percutaneous coronary intervention (PCI) in STEMI, were evaluated on the ST elevation resolution, TIMI flow and 30-day clinical outcomes. SUBJECTS AND METHODS: Following aspirin, clopidogrel and standard heparin, 45 patients with STEMI were randomized into 2 groups; tirofiban administration (Group I; n=23, 64+/-10 years; 15 male) or combined administration of tirofiban with 40 mg alteplase prior to primary PCI (Group II; n=22, 59+/-11 years; 19 male). The pre- and post-interventional TIMI flow grades, ST elevation resolution and bleeding complications were compared between the two groups. The major adverse cardiac events (MACE) were compared between the two groups during 30-days of clinical follow-up. RESULTS: Group II had a higher pre-interventional TIMI flow (TIMI flow> or =2: 34.8% vs. 90.9%, p<0.0001) and rate of ST elevation resolution (49.0+/-27.8% vs. 66.6+/-27.2%, p=0.045) than Group I. A major bleeding complication developed in 1 (5.0%) Group II patient, and minor bleeding complications developed 1 patient from each group (Group I; 9.5% vs. Group II; 10%, p=0.959). CONCLUSION: Combined administration of tirofiban with alteplase prior to primary PCI leads to a higher TIMI flow and more frequent ST elevation resolution, without bleeding complications, compared to a single administration of tirofiban.
Angioplasty ; Aspirin ; Blood Platelets ; Electrocardiography ; Follow-Up Studies ; Glycoproteins ; Hemorrhage ; Heparin ; Humans ; Myocardial Infarction* ; Percutaneous Coronary Intervention* ; Thrombolytic Therapy ; Tissue Plasminogen Activator*

PURPOSE: To investigate whether early recombinant human growth hormone (hGH) treatment in elderly patients undergoing surgery for hip fracture improves biochemical and functional recovery. MATERIALS AND METHODS: From September 2007 to December 2008, a total of 25 patients older than 60 years who underwent a gamma nailing surgery for an accidental hip fracture were randomized to be treated with either hGH (Declage(R), 3mg/week) (Group I: 15 patients) or placebo (Group II: 10 patients) at a postoperative 1, 2, 3 weeks, starting within 24 hour after the hip fracture. Mean age was 73.5 years. 6 cases were male and 19 cases were female. Serum IGF-I was measured by radioimmunoassay. Subjective and objective parameters were analyzed such as the mean mid-thigh circumference, change of weight, hand grip strength, functional recovery by Modified Barthel Index of activities of daily living (ADL). RESULTS: There were significant differences in the mean serum IGF-I, mid-thigh circumference, hand grip strength, Modified Barthel Index of activities of daily living between two groups. There were no significant differences in the change of weight. There were three nausea symptom after hGH injection, but no any general adverse effect. CONCLUSION: Early recombinant human growth hormone (hGH) treatment in elderly patients undergoing surgery for hip fracture showed clinical safety and good biochemical and functional recovery.
Activities of Daily Living ; Aged ; Female ; Hand ; Hand Strength ; Hip ; Human Growth Hormone ; Humans ; Insulin-Like Growth Factor I ; Male ; Nails ; Nausea ; Radioimmunoassay

Cardiovascular beriberi is caused by thiamine deficiency and usually presents as high cardiac output failure associated with predominantly right-sided heart failure and rapid recovery after treatment with thiamine. Because of its rarity in developed countries, the diagnosis can often be delayed and missed. We recently experienced a case of cardiovascular beriberi with pulmonary hypertension which successfully treated with thiamine infusion. A 50-year-old man with chronic heavy alcoholics was refered to our department for dyspnea with mental change. Echocardiography showed marked right ventricular (RV) dilatation and flattening of the interventricular septum with a D-shaped deformation of the left ventricle. Moderate tricuspid valve regurgitation was found and estimated RV systolic pressure was 52 mm Hg. Because of his confused mentality and history of chronic alcohol intake, neurological disorder due to thiamine deficiency was suspected and intravenous thiamine was administered and he continuously received a daily dose of 100 mg of thiamine. Follow up echocardiography showed marked reduction of RV dilatation and improvement of a D-shaped deformation of the left ventricle. He finally diagnosed as cardiovascular beriberi on the basis of dramatic response to intravenous thiamine. Thiamine deficiency can cause reversible pulmonary hypertension, and can still be encountered in the clinical setting. Thus high index of suspicion is critically needed for diagnosis.
Alcoholics ; Beriberi* ; Blood Pressure ; Cardiac Output, High ; Developed Countries ; Diagnosis ; Dilatation ; Dyspnea ; Echocardiography ; Follow-Up Studies ; Heart Failure ; Heart Ventricles ; Humans ; Hypertension, Pulmonary* ; Middle Aged ; Nervous System Diseases ; Thiamine ; Thiamine Deficiency ; Tricuspid Valve Insufficiency

Therapy-related ALL (t-ALL) is a rare secondary leukemia that develops after chemotherapy and/or radiotherapy for primary malignancies. Chromosomal 11q23 abnormalities are the most common karyotypic alterations in t-ALL. The t(11;19)(q23;p13) aberration is extremely rare and has not been confirmed at the molecular genetic level. Here, we report a case of t-ALL with t(11;19)(q23;p13.3) and MLL-MLLT1 (alias ENL) gene rearrangement confirmed by cytogenetic analysis, multiplex reverse transcription-PCR (multiplex RT-PCR), and DNA sequencing in a patient who had undergone treatment for breast cancer. A 40-yr-old woman developed acute leukemia 15 months after undergoing 6 cycles of adjuvant chemotherapy (doxorubicin 60 mg/m2 and cyclophosphamide 600 mg/m2), radiation therapy (dose, 5,900 cGy), and anticancer endocrine therapy with tamoxifen. The complete blood cell counts and bone marrow examination showed increased blasts and the blasts showed B lineage immunophenotype (positive for CD19, CD34, and cytoplasmic CD79a). Cytogenetic analysis revealed the karyotype 47,XX,+X,t(11;19)(q23;p13.3)[4]/46,XX[16]. FISH analyses, multiplex RT-PCR, and DNA sequencing confirmed the MLL-MLLT1 gene rearrangement. The patient underwent induction chemotherapy with fractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (Hyper-CVAD) and achieved complete remission. Subsequently, she underwent consolidation chemotherapy, but died of brain ischemia in the pons and the region of the middle cerebral artery. To our knowledge, this is the first case report of t-ALL with t(11;19)(q23;p13.3) and the MLL-MLLT1 gene rearrangement.
Adult ; Antineoplastic Agents/therapeutic use ; Base Sequence ; Breast Neoplasms/drug therapy/radiotherapy ; *Chromosomes, Human, Pair 11 ; *Chromosomes, Human, Pair 19 ; Combined Modality Therapy ; Cyclophosphamide/therapeutic use ; Doxorubicin/therapeutic use ; Female ; Gene Rearrangement ; Humans ; Immunophenotyping ; Karyotyping ; Molecular Sequence Data ; Myeloid-Lymphoid Leukemia Protein/*genetics ; Neoplasm Proteins/*genetics ; Nuclear Proteins/*genetics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/*diagnosis/genetics ; Sequence Analysis, DNA ; Tamoxifen/therapeutic use ; Transcription Factors/*genetics ; *Translocation, Genetic

Primary duodenal carcinoma is rare. Duodenal mucinous adenocarcinoma (DMA) is even rarer, and its associated manifestations and typical endoscopic or imaging findings are not well characterized. Herein, we report a case of primary DMA in an asymptomatic 58-year-old man who visited our hospital for a regular health screening. Upper endoscopy revealed an approximately 4-cm lesion in the second portion of the duodenum, but the mass was not visualized on computed tomography. Biopsies revealed a tubular adenoma that was subsequently resected. Frozen biopsies demonstrated DMA with a background of low-grade tubular adenoma for which we performed Roux-en-Y duodenojejunostomy and jejunojejunostomy. To our knowledge, this is the first report of a patient with DMA in Korea.
Adenocarcinoma ; Adenocarcinoma, Mucinous* ; Adenoma ; Biopsy ; Duodenal Neoplasms ; Duodenum ; Endoscopy ; Humans ; Korea ; Mass Screening ; Middle Aged

A 35-year-old Korean man with a 10-year history of ulcerative colitis (UC) presented with pain and swelling of the right neck. The patient was diagnosed with Takayasu's arteritis (TA) and had human leukocyte antigen (HLA) B-52, which is frequently found in patients having both UC and Takayasu's disease concurrently on HLA analysis. This case is the first report of a patient with both TA and UC in Korea, to the best of our knowledge.
Colitis, Ulcerative ; Humans ; Korea ; Leukocytes ; Neck ; Takayasu Arteritis ; Ulcer

A 35-year-old Korean man with a 10-year history of ulcerative colitis (UC) presented with pain and swelling of the right neck. The patient was diagnosed with Takayasu's arteritis (TA) and had human leukocyte antigen (HLA) B-52, which is frequently found in patients having both UC and Takayasu's disease concurrently on HLA analysis. This case is the first report of a patient with both TA and UC in Korea, to the best of our knowledge.
Colitis, Ulcerative ; Humans ; Korea ; Leukocytes ; Neck ; Takayasu Arteritis ; Ulcer