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Porphyria cutanea tarda (PCT) is commonly diagnosed in cases where multiple hyperechoic nodules are observed in the liver. Pathologically, these nodules associated with PCT are focal fatty deposits. We report here, seven cases of PCT with fatty changes over multiple foci in the liver. Furthermore, the characteristics of ultrasonography (US) findings of 32 previously reported cases are summarized. The US features of these nodules showed a homogenous hyperechoic or hyperechoic rim pattern, partial confluence, and no mass effect in the vascular structures. Because multiple hyperechoic liver nodules occasionally mimic malignancies, and because their diagnosis can be challenging, clinicians should consider checking urine porphyrin levels to rule out PCT when such nodules are observed on US.
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Porfiria Cutânea Tardia , Humanos , Porfiria Cutânea Tardia/complicações , Porfiria Cutânea Tardia/diagnóstico por imagem , Ultrassonografia/efeitos adversosRESUMO
BACKGROUND: Direct-acting antivirals (DAAs) rapidly clear hepatitis C virus (HCV), but the lipid dynamics after DAA treatment remain unknown. Low-density lipoprotein (LDL) cholesterolemia is the predicting factor for the onset and death of atherosclerotic cardiovascular diseases. Thus, in this study, we examined the frequency and risk of hyper-LDL cholesterolemia in HCV patients who achieved sustained virologic response (SVR) with DAA treatment. METHODS: A total of 121 patients with HCV genotype 1b, who achieved SVR with DAA treatment, were examined for serum levels of total cholesterol, LDL-cholesterol (LDL-C), high-density lipoprotein, and triglycerides from the start of treatment until 2 years after SVR (SVR-2y). ΔLDL-C was defined as the change in LDL-C levels from treatment initiation to SVR-2y. Hyper-LDL cholesterolemia was defined as ≥ 140 mg/dL LDL-C at SVR-2y. Stepwise multiple regression analysis was performed to determine whether ΔLDL-C and hyper-LDL cholesterolemia are associated with other factors, including viral kinetics. RESULTS: A total of 63, 3, and 55 patients were administered daclatasvir + asunaprevir, ombitasvir + paritaprevir + ritonavir, and ledipasvir + sofosbuvir, respectively. ΔLDL-C in patients with the IL28B (rs8099917) TG/GG genotype was significantly higher than in those with IL28B TT (27.3 ± 27.0 and 9.6 ± 27.3 mg/dL; P < 0.001). In addition, IL28B TG/GG was an independent risk factor for hyper-LDL cholesterolemia (odds ratio: 8.47; P < 0.001). CONCLUSIONS: An IL28B polymorphism is associated with ΔLDL-C and hyper-LDL cholesterolemia after achieving SVR. Thus, lipid markers should be carefully monitored in patients who achieve SVR with DAA.
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Antivirais/uso terapêutico , LDL-Colesterol/sangue , Hepatite C/tratamento farmacológico , Hepatite C/genética , Interferons/genética , Polimorfismo Genético , Idoso , Feminino , Hepatite C/sangue , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Telaprevir (TVR) plays a major role in renal damage and anemia associated with TVR/pegylated interferon/ribavirin therapy for chronic hepatitis C. Adjusting the TVR starting dose may reduce these adverse effects. We aimed to determine whether adjusting the starting dose according to renal function reduces TVR-associated renal damage and anemia and affects the sustained virological response (SVR). PATIENTS AND METHODS: Our study included 112 patients infected with hepatitis C genotype 1 treated with pegylated interferon/ribavirin/TVR triple therapy. The TVR starting dose adjusted according to renal function was calculated as TVR/unadjusted estimated glomerular filtration rate (eGFR) ratio=TVR/(eGFR×body surface area/1.73). RESULTS: A TVR/unadjusted eGFR ratio of 32 or greater was a predictor of renal impairment and anemia in multivariate analysis (odds ratio 12.09, P<0.001, and OR 4.14, P<0.001, respectively). Patients with a TVR/unadjusted eGFR ratio of 32 or greater developed significant renal impairment and anemia (P<0.001 and P=0.002, respectively). SVR was significantly reduced in patients with a TVR/unadjusted eGFR ratio less than 23 versus 23 or greater (66.7 and 87.2%, respectively, P=0.045). SVR tended to increase stepwise [<23.0 (66.7%), ≥23 to <32 (84.8%), and ≥32 (89.6%), respectively]. The TVR/unadjusted eGFR ratio was correlated significantly with the serum TVR concentration (r=0.541, P<0.001). CONCLUSION: Adjusting the TVR starting dose according to the TVR/unadjusted eGFR ratio decreased adverse effects and affected the SVR rate. The TVR starting dose should be adjusted by a TVR/unadjusted eGFR ratio of 23 or greater to less than 32 to safely achieve SVR.
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Injúria Renal Aguda/induzido quimicamente , Anemia/induzido quimicamente , Antivirais/uso terapêutico , Hepatite C Crônica/tratamento farmacológico , Rim/fisiologia , Oligopeptídeos/administração & dosagem , Oligopeptídeos/efeitos adversos , Injúria Renal Aguda/fisiopatologia , Idoso , Anemia/sangue , Antivirais/efeitos adversos , Monitoramento de Medicamentos , Quimioterapia Combinada , Feminino , Genótipo , Taxa de Filtração Glomerular , Hemoglobinas/metabolismo , Hepacivirus/genética , Hepatite C Crônica/virologia , Humanos , Interferon alfa-2 , Interferon-alfa/uso terapêutico , Rim/efeitos dos fármacos , Masculino , Pessoa de Meia-Idade , Polietilenoglicóis/uso terapêutico , Proteínas Recombinantes/uso terapêutico , Estudos Retrospectivos , Ribavirina/uso terapêuticoRESUMO
BACKGROUND/AIMS: The present pilot study aimed to evaluate the safety and efficacy of hepatic arterial infusion chemotherapy (HAIC) with interferon-beta (IFN-ß) and 5-fluorouracil (5-FU) in patients with advanced hepatocellular carcinoma (HCC). METHODOLOGY: We studied 10 patients with advanced HCC and who were unresponsive to previous HAIC using low-dose 5-FU and cisplatin. The median age was 67 years. Eight patients had portal vein tumor thrombosis and four patients had extrahepatic metastasis. Using a drug delivery system, patients were treated with HAIC of IFN-ß (600 MIU/body, three times/week) and 5-FU (250 mg/body, five times/week). Chemotherapy was repeated consecutively for 2 weeks every 4 weeks. RESULTS: Six (60%) patients had a decrease in tumor markers alpha-fetoprotein (APP) or des-gamma-carboxy prothrombin (DCP). The median overall survival was 108 days and the 1-year survival rate was 10.0%. Univariate analysis showed two significant prognostic factors related to long-term survival for more than 60 days: a decrease in APP or DCP 4 weeks after treatment (P = 0.035) and no extra hepatic metastasis (P = 0.035). Severe hepatic injury was not observed. CONCLUSIONS: HAIC with IFN-ß and 5-PU exerts modest antitumor effects and poses no particular safety concerns. This may be a new promising strategy for treatment of advanced HCC.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Carcinoma Hepatocelular/tratamento farmacológico , Neoplasias Hepáticas/tratamento farmacológico , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Biomarcadores/sangue , Carcinoma Hepatocelular/sangue , Carcinoma Hepatocelular/mortalidade , Carcinoma Hepatocelular/secundário , Esquema de Medicação , Feminino , Fluoruracila/administração & dosagem , Artéria Hepática , Humanos , Infusões Intravenosas , Interferon beta/administração & dosagem , Estimativa de Kaplan-Meier , Neoplasias Hepáticas/sangue , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/patologia , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Precursores de Proteínas/sangue , Protrombina , Fatores de Tempo , Resultado do Tratamento , alfa-Fetoproteínas/metabolismoRESUMO
AIM: This prospective study was designed to examine whether consumption of a branched-chain amino acid (BCAA)-enriched nutrient mixture as a late-evening snack (LES) helps maintain and/or improve liver functioning in liver cirrhosis (LC) patients who have undergone radiofrequency ablation (RFA) for hepatocellular carcinoma (HCC). METHODS: An equal number (10) of 30 LC patients who had undergone RFA for HCC was randomly assigned to a standard diet group (control) group, a morning BCAA (M-BCAA) administration group, or a LES with BCAA (LES-BCAA) administration group. Liver function testing was performed and Child-Pugh scores (CPS) calculated for each group to assess the improvement at 1, 4 and 12 weeks post-RFA. RESULTS: Compared to the control and M-BCAA groups, the LES-BCAA group experienced a rapid and significant improvement in albumin and total serum bilirubin levels and in CPS that began during the initial post-RFA period. These results indicate that LES with BCAA supplementation significantly improved the CPS of the LES-BCAA group at 4 and 12 weeks post-RFA. Although no patients experienced serious adverse effects, two patients who had been diagnosed with diabetes mellitus before undergoing RFA required blood sugar management to improve glycemic control and one subject withdrew due to supplement-induced vomiting. CONCLUSION: LES with BCAA supplementation significantly and rapidly improves liver functioning and CPS in LC patients who have undergone RFA for HCC. Control of blood sugar levels is necessary when calorie-containing BCAA is administrated to LC patients with impaired glucose tolerance.
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BACKGROUND AND AIM: Percutaneous radiofrequency ablation (RFA) has been shown to be a highly effective treatment for hepatocellular carcinoma (HCC). We investigated the controllability of HCC and explored the algorithm of therapeutic strategy for HCC in patients who met the RFA criteria. METHODS: We enrolled 472 patients with HCC who met the RFA criteria (≤ 3 nodules, ≤ 3 cm) and underwent RFA for initial therapy. Patients who underwent repeated RFA were evaluated retrospectively when HCC exceeded the RFA criteria, or the functional hepatic reserve progressed to Child-Pugh grade C. RESULTS: Overall survival rates were: 1 year, 96%; 3 years, 79%; and 5 years, 56%. In 5 years, 14% of patients progressed to Child-Pugh grade C. Meanwhile, 47% of patients exceeded the RFA criteria. Annually, 8% of patients deviated from the RFA criteria. The percentage of patients who were able to receive RFA significantly decreased at the fourth session compared with up to the third session. The survival rates decreased at the rate of 7% annually until the third year after the initial RFA. Afterwards, it shifted to a decrease at the rate of 12% annually. In a multivariate analysis, the presence of hepatitis C virus infection and the existence of a single tumor were identified as significant independent factors contributing to probabilities exceeding the RFA criteria. CONCLUSIONS: HCC was controlled by RFA up to three RFA treatments and 3 years from the initial therapy. On this basis, we propose a "three (times) × 3 (years) index" for considering a shift from RFA to other treatment modalities.
Assuntos
Carcinoma Hepatocelular/cirurgia , Ablação por Cateter/métodos , Neoplasias Hepáticas/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Algoritmos , Carcinoma Hepatocelular/patologia , Progressão da Doença , Feminino , Humanos , Neoplasias Hepáticas/patologia , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Recidiva , Estudos Retrospectivos , Análise de Sobrevida , Resultado do TratamentoRESUMO
BACKGROUND: Research in 2008 demonstrated that the majority of out-of-hospital cardiac arrests (OHCAs) occur in the home, and many important characteristics differ between private and public locations. However, the influence of the location of collapse on survival from OHCA is not well understood. Furthermore, most of the reports have been from Western countries; there is little research from Asia that differentiates the conditions of OHCA. OBJECTIVE: To investigate the influence of the location of collapse on being discharged alive from OHCA and whether the location of collapse is also an independent predictor of survival from OHCA in Japan. METHODS: We analyzed 463 consecutive cases of witnessed OHCA with cardiac etiology that occurred between October 2004 and September 2008 in Japan. We investigated the characteristics of OHCA patients who collapsed in private and public locations, and assessed the influence of the location of collapse on survival from OHCA. RESULTS: Patients who collapsed outside the home were younger, more likely to be male, more likely to receive bystander cardiopulmonary resuscitation (CPR), and more likely to have ventricular fibrillation (VF)/pulseless ventricular tachycardia (VT) and had a shorter time interval between collapse and 9-1-1 call than patients who collapsed in the home. Mortality was significantly higher in the group who collapsed in the home. The independent influence of the location of collapse was eliminated by additional adjustment for time interval from collapse to 9-1-1 call, age, bystander CPR, and initial cardiac rhythm. Finally, VF/pulseless VT as the initial rhythm and bystander CPR were independently associated with the patient's being discharged alive; the location of collapse was not an independently associated variable. CONCLUSIONS: The present analysis demonstrated that there were significant differences in survival between groups of patients who suffered from cardiac arrest inside and outside the home in Japan. The outside-the-home group had a higher rate of survival from OHCA; however, the location of collapse was not an independent predictor of survival from OHCA. Education of the families of high-risk patients in placing a rapid emergency call and performing effective CPR might be needed to improve survival from cardiac arrest in the home.
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Ponte Cardiopulmonar/métodos , Serviço Hospitalar de Emergência , Serviços de Assistência Domiciliar , Parada Cardíaca Extra-Hospitalar/mortalidade , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Japão , Masculino , Pessoa de Meia-Idade , Parada Cardíaca Extra-Hospitalar/terapia , Prognóstico , Estudos Prospectivos , Fatores de Risco , Estatísticas não Paramétricas , Taquicardia Ventricular/mortalidade , Taquicardia Ventricular/terapia , Fibrilação Ventricular/mortalidade , Fibrilação Ventricular/terapiaRESUMO
BACKGROUND: Several studies have reported circadian, weekly, and seasonal variations in the rates of out-of-hospital cardiac arrest (OHCA). However, variations in the mortality of OHCA are not well known. METHODS AND RESULTS: We investigated the 1396 consecutive cases of OHCA with cardiac etiology between October 2004 and September 2008. There were 2 peaks in the occurrence of OHCA in early morning and late evening. There was a weekly pattern with an increased incidence on Mondays. We found a significant seasonal variation in the frequency of events, with a maximum during winter. There was a trend of reduced mortality in warmest 3 months, especially among a subgroup of ventricular fibrillation/pulseless ventricular tachycardia with arrest witnessed. CONCLUSION: The present analyses demonstrated circadian, weekly and seasonal variations in the occurrence, and a seasonal variation in mortality in OHCA. Changes in temperature might influence the severity of OHCA and change the rate of success of cardiopulmonary resuscitation.
Assuntos
Parada Cardíaca Extra-Hospitalar/mortalidade , Idoso , Idoso de 80 Anos ou mais , Distribuição de Qui-Quadrado , Ritmo Circadiano , Bases de Dados Factuais , Feminino , Humanos , Incidência , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Parada Cardíaca Extra-Hospitalar/epidemiologia , Estudos Prospectivos , Fatores de Risco , Estações do Ano , Estatísticas não Paramétricas , Taquicardia Ventricular/epidemiologia , Taquicardia Ventricular/mortalidade , Temperatura , Fatores de Tempo , Fibrilação Ventricular/epidemiologia , Fibrilação Ventricular/mortalidadeRESUMO
Hepatitis C-associated osteosclerosis (HCAO) is a rare disorder characterized by a marked increase in skeletal mass in patients who are infected with hepatitis C virus (HCV). The clinical presentation is an acquired deep bone pain with increased serum alkaline phosphatase (ALP) activity. We present a case of a patient with HCAO who was treated with antiviral therapy. A 42-year-old Japanese man presented with severe, stabbing pain in his lower limbs. He was diagnosed with hepatitis C secondary to intravenous drug use 20 years earlier. Serum biochemical studies revealed markedly elevated ALP activity and osteocalcin levels. Skeletal radiographs showed diffuse bony sclerosis with marked cortical thickening in the long bones. The bony findings and clinical symptoms were attributed to HCAO. The HCV RNA viral load was high and the genotype was 2a. The patient was treated with peginterferon alfa-2b and ribavirin for 24 weeks. After 24 weeks of the combination therapy, the patient had a sustained virological response and clinical remission of bone pain and a decrease in the level of serum ALP. In conclusion, HCAO was improved by the combination therapy of peginterferon alfa-2b and ribavirin when the patient achieved sustained virological response. It was confirmed that HCAO was one of the extrahepatic manifestations of HCV.
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A 54-year-old woman visited our hospital because of palpitation and dizziness. Systolic ejection murmur, Levine IV/VI, was heard at the left sternal border and atrial gallop was auscultated at apex. Echocardiogram demonstrated diffuse hypertrophy of the left ventricular walls extending to the apex. The left ventricular walls increased symmetrically; the septal and the posterior wall measured approximately 26 mm and 30 mm in thickness, respectively. Apical two-chamber views revealed a narrow outflow of the left ventricular cavity at apex with a distinct convergence zone, where color flow Doppler imaging showed turbulent jet flow from mid to late systole. The leukocyte α-galactosidase A activity was lower than normal. Percutaneous endomyocardial biopsy showed specific change for Fabry's disease by electron microscopy. On cardiac catheterization, mid-ventricular resting pressure gradient of approximately 90 mmHg was shown. This pressure gradient dramatically disappeared after intravenous administration of 70 mg of cibenzoline. We describe a rare case of Fabry's disease that revealed diffuse hypertrophy having dynamic obstruction at mid-left ventricle, like mid-ventricular obstruction, and the administration of cibenzoline had beneficial effects on pressure gradient of mid-left ventricle.
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AIM: The diagnosis of Wilson disease is based on the results of several clinical and biochemical tests. This study aimed to clarify the clinical features and spectrum of Wilson disease, including severe Wilson disease. METHODS: Between 1985 and 2009, 10 patients with clinical, biochemical or histological evidence of Wilson disease were either diagnosed or had a previously established diagnosis confirmed at Fukuoka University Hospital. Severe Wilson disease was defined by a serum prothrombin time ratio of more than 1.5 or serum prothrombin activity of less than 50%. The 10 Wilson disease patients were divided into two groups, one containing three non-severe patients and the other containing seven severe patients, and the biochemical features of the patients in these two groups were compared. RESULTS: The mean age at diagnosis was 21.5 ± 11.7 years (range, 7-39). Decreased serum ceruloplasmin, enhanced 24-h urinary copper excretion, presence of Kayser-Fleischer rings and histological signs of chronic liver damage were confirmed in 100%, 100%, 66.7% and 100% of patients, respectively. Severe Wilson disease patients had higher levels of serum ceruloplasmin and serum copper (P < 0.05, P < 0.05, respectively) than non-severe patients. CONCLUSION: In severe Wilson disease patients, the serum ceruloplasmin and serum copper levels were higher than those in non-severe Wilson disease patients.
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To examine the mRNA expression of hepatobiliary transporters in primary biliary cirrhosis (PBC) patients and to compare bile acid absorption, synthesis, and efflux in patients with non-end-stage and end-stage PBC, we obtained liver samples from PBC patients by percutaneous needle biopsy. End-stage PBC was defined as follows: histological stage IV; cirrhosis; serum total bilirubin, ≥4.0 mg/dl; and Child-Pugh Class C. The mRNA expression levels of sodium taurocholate cotransporting polypeptide (NTCP), bile salt export pump (BSEP), and hepatic cholesterol 7α-hydroxylase (CYP7A1) were significantly higher in the PBC patients than in the controls (P < 0.01). The mRNA levels of NTCP and BSEP were significantly higher in the end-stage PBC patients than in the controls (P < 0.01). However, hepatic CYP7A1 mRNA expression decreased significantly (by 70%) in the patients with end-stage PBC as compared to the controls and the patients with non-end-stage PBC (P < 0.01). The hepatic expression of transporters mediating bile acid influx and efflux showed sustained elevation, whereas that of the rate-limiting enzyme for bile acid biosynthesis was attenuated in the end-stage PBC patients. Thus, mechanisms may be present preventing the accumulation of toxic bile acids in the hepatocytes of end-stage PBC patients.
Assuntos
Transportadores de Cassetes de Ligação de ATP/biossíntese , Colesterol 7-alfa-Hidroxilase/biossíntese , Doença Hepática Terminal/metabolismo , Cirrose Hepática Biliar/metabolismo , Fígado/metabolismo , Transportadores de Ânions Orgânicos Dependentes de Sódio/biossíntese , Simportadores/biossíntese , Membro 11 da Subfamília B de Transportadores de Cassetes de Ligação de ATP , Transportadores de Cassetes de Ligação de ATP/genética , Ácidos e Sais Biliares/metabolismo , Colesterol 7-alfa-Hidroxilase/genética , Regulação para Baixo , Humanos , Transportadores de Ânions Orgânicos Dependentes de Sódio/genética , RNA Mensageiro/biossíntese , Simportadores/genética , Regulação para CimaRESUMO
There are more than a few risks of hemorrhage complication in patients with amyloidosis. Although most cases with amyloidosis exhibit minor bleeding manifestations, they can be occasionally associated with life-threatening problems. To our knowledge, there are only a few cases of spontaneous pericardial hematoma associated with amyloidosis. We here report a patient who suddenly died of cardiac tamponade with massive pericardial hematoma 7 years after the diagnosis of familial amyloid polyneuropathy (FAP). A 69-year-old female with FAP with cardiogenic shock was admitted to our emergency room. Although she previously underwent permanent pacemaker implantation for atrial fibrillation with slow ventricular response, electrocardiogram showed a critical pacing failure. Emergent telemetry check revealed a sudden extreme increase of pacing capture threshold in the right ventricle. Maximum pacing voltage could not improve the critical condition, and she died 7 h after arrival. Autopsy showed a massive pericardial hematoma in the right ventricular free wall, and microscopic examination revealed typical amyloid deposition in the arterial wall of the pericardium. In this case, it is assumed that a sudden rupture of fragile pericardial vessels with amyloid deposition led to the lethal pericardial hematoma.
Assuntos
Neuropatias Amiloides Familiares/complicações , Neuropatias Amiloides Familiares/fisiopatologia , Hematoma/etiologia , Pericárdio/patologia , Idoso , Evolução Fatal , Feminino , Hematoma/diagnóstico , HumanosRESUMO
We describe a case of isolated adrenocorticotropic hormone deficiency that showed ventricular fibrillation associated with QT prolongation. A 72-year-old man was admitted because of consciousness disorder caused by severe hypoglycemia. On the second hospital day, QT intervals were unexpectedly prolonged and ventricular fibrillation occurred. Electrical defibrillation was performed and restored hemodynamically stable condition without neurologic deficits. He was diagnosed with endocrine tests as having isolated adrenocorticotropic hormone deficiency. QT prolongation was improved after hydrocortisone replacement therapy. We considered the QT prolongation was caused by corticosteroid insufficiency. We should be aware that corticosteroid insufficiency may provoke QT prolongation responsible for sudden cardiac death.
Assuntos
Insuficiência Adrenal/complicações , Insuficiência Adrenal/diagnóstico , Síndrome do QT Longo/etiologia , Fibrilação Ventricular/etiologia , Insuficiência Adrenal/fisiopatologia , Idoso , Eletrocardiografia , Humanos , Síndrome do QT Longo/diagnóstico , Masculino , Fibrilação Ventricular/diagnósticoRESUMO
BACKGROUND: Organic coronary artery stenosis is a significant prognostic factor in patients with coronary spastic angina (CSA), so the present study was focused on assessing the impact of intermediate fixed stenosis at sites of provoked spasm on the long-term outcomes of CSA patients. METHODS AND RESULTS: CSA patients diagnosed on the basis of ergonovine-provoked spasm were enrolled and the clinical background and long-term prognosis of CSA patients with intermediate fixed stenosis at the site of provoked spasm (with-fixed-stenosis group, n=37) and those without fixed stenosis (without-fixed-stenosis group, n=126) were retrospectively compared. During the follow-up period (average 4.01 years for with-fixed-stenosis, 4.47 years for without-fixed-stenosis), the with-fixed-stenosis group had a significantly lower event-free survival rate, as well as a higher frequency of admission for unstable angina and percutaneous coronary intervention than the without-fixed-stenosis group, whereas the survival rate did not differ significantly between the 2 groups. In the multivariate analysis, intermediate fixed stenosis at the site of provoked spasm was a predictor of long-term major adverse cardiac events (MACE). CONCLUSIONS: Intermediate fixed stenosis at the site of ergonovine-provoked spasm is an independent risk factor for MACE during the long-term period in CSA patients.
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Angina Pectoris/mortalidade , Estenose Coronária/mortalidade , Vasos Coronários , Ergonovina/efeitos adversos , Ocitócicos/efeitos adversos , Espasmo/mortalidade , Idoso , Estenose Coronária/induzido quimicamente , Intervalo Livre de Doença , Ergonovina/administração & dosagem , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Ocitócicos/administração & dosagem , Estudos Retrospectivos , Espasmo/induzido quimicamente , Taxa de SobrevidaRESUMO
BACKGROUND/AIMS: The hepatic expression of bile acid transporters is altered in experimental cholestasis and it is unclear whether regulation exists in human cholestatic diseases. We investigated the expression of genes involved in bile acid detoxification, basolateral export and nuclear factor regulation in untreated primary biliary cirrhosis (PBC). METHODS: Liver tissues were obtained from patients with early-stage and late-stage PBC. The hepatic expression levels of messenger RNAs were determined by the real-time reverse transcription polymerase chain reaction. RESULTS: The hepatic expression of multidrug-resistance protein 4 messenger RNA was significantly upregulated in early-stage and late-stage PBC patients compared with controls. The hepatic expression of multidrug-resistance protein 2 and multidrug-resistance protein 3 messenger RNAs was significantly elevated only in early-stage PBC patients. The hepatic expression levels of farnesoid X receptor, fetoprotein transcription factor and constitutive androstane receptor mRNAs were correlated with those of multidrug-resistance protein 2, multidrug-resistance protein 3 and multidrug-resistance protein 4 respectively. CONCLUSIONS: The hepatic expression of multidrug-resistance protein 4 was enhanced in patients with untreated PBC at all stages. However, the hepatic expression of multidrug-resistance protein 2 and multidrug-resistance protein 3 was enhanced only in early-stage patients. The lack of upregulation of these proteins might contribute to the progression of PBC.
Assuntos
Proteínas de Transporte/metabolismo , Regulação da Expressão Gênica/fisiologia , Cirrose Hepática Biliar/metabolismo , Fígado/metabolismo , Glicoproteínas de Membrana/metabolismo , Southwestern Blotting , Receptor Constitutivo de Androstano , Proteínas de Ligação a DNA/metabolismo , Humanos , Microscopia de Fluorescência , Proteínas Associadas à Resistência a Múltiplos Medicamentos/metabolismo , Sondas de Oligonucleotídeos/genética , Receptores Citoplasmáticos e Nucleares/metabolismo , Fatores de Transcrição/metabolismoRESUMO
We report two cases of Budd-Chiari syndrome. Case 1: A 57-year-old man presented with leg edema and esophageal varices. Cavography showed obstruction of the inferior vena cava with antiphospholipid syndrome. Further, the patient showed positive serology for hepatitis C virus and consumed large quantities of alcohol. Percutaneous transluminal angioplasty was performed on this patient and anticoagulants administered; leg edema and esophageal varices were ameliorated although liver biopsy showed cirrhosis without evident congestion. More than 9 months since the diagnosis, restenosis of the inferior vena cava has not occurred. Case 2: A 73-year-old woman presented abdominal pain but no edema or varices. Cavography showed membranous obstruction of the inferior vena cava which required no therapy. Manifestation of portal hypertension was not present and liver function was maintained although liver biopsy showed obvious congestion. These cases showed untypical features against histopathology, and careful observation will be required for emergence of hepatocellular carcinoma.
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PURPOSE: This study aimed to clarify the long-term efficacy of the lamivudine treatment in Japanese patients with chronic hepatitis B either with or without lamivudine resistance or with or without adefovir add-on treatment. METHODS: We followed 110 patients who received lamivudine for more than 12 months, including 67 hepatitis B e antigen (HBeAg)-positive and 43 HBeAg-negative patients. RESULTS: The median follow-up after the onset of lamivudine was 48 (range = 12-86) months. In all the patients with or without lamivudine resistance, the level of alanine aminotransferase (ALT) normalization decreased from 70.0% at 1 year to 36.4% at 5 years and the loss of serum HBV DNA level decreased from 72.7% at 1 year to 31.8% at 5 years. Sixty patients (54.6%) developed a lamivudine-resistant mutation, and this occurrence was more frequently observed in those who were HBeAg-positive (P < 0.01), those with a low level of ALT (P < 0.05), and those with a high level of serum HBV DNA (P < 0.01). Thirty-six of 60 patients received adefovir in addition to lamivudine to treat breakthrough hepatitis. A Cox proportional hazards model analysis revealed the level of baseline HBV DNA to be the best predictive factor for the virus recrudescence (risk ratio = 0.466, 95% confidence interval [CI]: 0.246-0.842, P = 0.011) and the breakthrough hepatitis (risk ratio = 0.444, 95% CI: 0.218-0.879, P = 0.019). We carefully monitored the efficacy of this treatment both in patients who received adefovir and in those who did not since the beginning of the lamivudine treatment. The normalization level of ALT was 61.4% at 5 years and the loss of serum HBV DNA was 61.4% at 5 years since lamivudine was started. A histologic improvement was observed in patients with ALT levels less than two times the upper limit of normal at the time of a second liver biopsy. CONCLUSIONS: Although the efficacy of lamivudine is limited because of breakthrough hepatitis, adefovir was used as a salvage treatment of patients with lamivudine-resistant chronic hepatitis B. In addition, lamivudine was used for the treatment of Japanese patients with chronic hepatitis B with or without lamivudine resistance, and was found to be useful regarding the long-term virologic and biochemical responses.
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BACKGROUND: Primary percutaneous coronary intervention (PCI) for acute myocardial infarction (AMI) is performed in hospitals without on-site coronary artery bypass graft surgery in the ;real world'. However, data on the in-hospital outcomes of primary PCI performed at hospitals with and without on-site cardiac surgery are still lacking in Japan. METHODS AND RESULTS: In the present study, 2,230 AMI patients were enrolled in the AMI-Kyoto Multi-Center Risk Study between January 2000 and December 2005. Of these, 1,817 patients underwent primary PCI. Excluding patients without adequate data, we retrospectively compared clinical background, coronary risk factors, angiographic findings, acute results of primary PCI and in-hospital prognosis between patients undergoing primary PCI in hospitals without on-site cardiac surgery (without surgery group, n=792) and those in hospitals with (with surgery group, n=993). The without surgery group had higher prevalence of previous myocardial infarction, Killip class>or=3 at admission and multivessels as a culprit lesion than the with surgery group. The without surgery group was more likely to have lower frequency of stent usage and lower thrombolysis in myocardial infarction flow grade just after PCI, whereas it was more likely to have intra-aortic balloon pumping and temporary pacing during procedures. The overall in-hospital mortality did not differ between the 2 groups. On multivariate analysis, in AMI patients undergoing primary PCI, Killip class>or=3 at admission, multivessels or left main trunk (LMT) as culprit lesions, number of diseased vessels>or=2 or diseased LMT, and age were the independent predictors of the in-hospital mortality, but the presence of on-site cardiac surgery was not. CONCLUSIONS: These results suggest that in-hospital outcomes in AMI patients undergoing primary PCI at hospitals without on-site cardiac surgery are comparable to those at hospitals with on-site cardiac surgery in Japan.
Assuntos
Angioplastia Coronária com Balão , Ponte de Artéria Coronária , Idoso , Idoso de 80 Anos ou mais , Angioplastia Coronária com Balão/efeitos adversos , Angioplastia Coronária com Balão/mortalidade , Ponte de Artéria Coronária/efeitos adversos , Ponte de Artéria Coronária/mortalidade , Feminino , Mortalidade Hospitalar , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Stents/estatística & dados numéricos , Terapia Trombolítica/estatística & dados numéricos , Resultado do TratamentoRESUMO
OBJECTIVES: Obesity may be linked with coronary atherosclerosis in young males. This study investigated the etiology of acute myocardial infarction (AMI) in young Japanese male patients with or without obesity. METHODS AND RESULTS: This retrospective study included 2,230 AMI patients enrolled in the AMI-Kyoto Multi-Center Risk Study between January 2000 and December 2005. Clinical background, risk factors, angiographic findings, acute results of primary percutaneous coronary intervention (PCI), and in-hospital outcome were evaluated in 33 young male patients < 40 years old. The study group was divided into the obese group [body mass index (BMI) > or =25, n=21] and non-obese group (BMI < 25, n=12). Four of the 12 non-obese patients had underlying disease (Kawasaki disease 2, Buerger's disease 1, drug abuse 1). The non-obese group had a higher prevalence of underlying disease than the obese group. The non-obese group had a higher incidence of left anterior descending coronary artery as culprit lesion and higher Thrombolysis in Myocardial Infarction (TIMI) grade flow in the infarct-related artery before primary PCI. The acquisition rates of TIMI 3 flow after primary PCI and in-hospital outcome did not differ between the two groups. CONCLUSIONS: Non-obese young AMI male patients have a higher frequency of underlying disease. Most young male AMI patients were obese, suggesting that obesity may be important in the pathogenesis of AMI in young male adults.