Coração carcinóide – a propósito de um caso clínico

We describe the case of a 76-year-old woman, diagnosed with a neuroendocrine tumor of the cecum in 2004, with liver metastases and carcinoid syndrome since September 2010. The patient had been treated intermittently with chemotherapy cycles, and remained symptomatic, with worsening secondary lesions. In June 2011 she began to present signs and symptoms of right heart failure and was hospitalized in September 2011. Transthoracic two- and three-dimensional echocardiography revealed enlarged right atrium and ventricle and thickened and fixed tricuspid and pulmonary valve leaflets, causing severe tricuspid regurgitation and mild pulmonary regurgitation and stenosis, suggestive of carcinoid heart disease. The authors discuss the clinical importance of transthoracic echocardiography, and the more recent three-dimensional echocardiography, as the diagnostic exam of choice in these cases, as it is especially suitable for assessing the valves and subvalvular apparatus.     

A comparison between central blood pressure values obtained by the Gaon system and the SphygmoCor system

Central pulse pressure is correlated with carotid atherosclerosis and the incidence of cardiovascular events more significantly than brachial pulse pressure. Augmentation index (Aix) has been shown to be an independent predictor of cardiovascular morbidity and mortality. Pulse wave analysis using the Gaon system allows for the estimation of central blood pressure (CBP), corrected augmentation index (Aix@HR75), ejection duration (ED) and subendocardial viability ratio (SEVR), and is widely used in clinical research in Korea. However, the accuracy of this system is controversial. From February 2008 to March 2011, 99 patients were recruited for this study. Measurements were taken both by the Gaon system and the SphygmoCor system on the same day for all study participants. The estimated values of CBP, Aix@HR75, ED and SEVR for the two systems were compared using paired t-tests, simple correlation analyses and Bland-Altman plots. Systolic blood pressure (SBP) estimated by the two systems was significantly (P<0.001) correlated; the coefficient was 0.982. The two s.d. of the difference in SBP between these systems was quite small--<7?mm?Hg. Aix@HR75, ED and SEVR as estimated by the two systems were also significantly correlated, although they, especially SEVR, showed much weaker correlations than were observed in SBP: coefficients for Aix@HR75, ED and SEVR were 0.727, 0.648 and 0.230, respectively. We assessed the CBP of Korean patients estimated by the two systems and observed that the correlations of Aix, ED and SEVR were weaker than that of CBP. Such variations may be due to the difference in measuring methods between the devices. As even a slight change in pulse waveforms may result in a large difference in estimations, parameters, including Aix@HR75, ED and SEVR, should be carefully interpreted by experienced clinicians.     

Study on effectiveness of gemcitabine, dexamethasone, and cisplatin (GDP) for relapsed or refractory AIDS-related non-Hodgkin's lymphoma

Non-Hodgkin's lymphoma (NHL) remains the second most common malignant complication in patients with human immunodeficiency virus (HIV) infection. Even though NHL is commonly chemosensitive to primary treatment, failure or relapse still occurs in a large number of patients. We conducted this retrospective study to evaluate the efficacy and safety of gemcitabine, dexamethasone, and cisplatin (GDP) for relapsed or refractory AIDS-related NHL (AIDS-NHL). Forty-eight patients with relapsed or refractory AIDS-NHL were treated with intravenous combination chemotherapy with GDP. The overall objective response rate was 54.1?% (95?% confidence interval, CI, 40.1–68.3?%), with 10 complete responses and 16 partial responses. The 2-year overall survival rate (OS) was 70.8?% (95?% CI 58.0–83.7?%), and the 5-year OS was 41.7?% (95?% CI 27.7–55.6?%). The 2-year progression-free survival rate (PFS) was 37.5?% (95?% CI 23.8–51.2?%), and the 5-year PFS was 25.0?% (95?% CI 12.8–37.3?%). The median progression-free survival was 8.8?months (95?% CI 0–20.3?months), and the median overall survival was 40.6?months (95?% CI 22.6–58.6?months). Patients with B cell tumors who relapsed but had no B symptoms were clinical stage I/II, had infiltration fewer than two extranodal sites, had CD4⁺ counts >200 cells/μL, and had lactate dehydrogenase (LDH) less than the upper limit of normal benefited from GDP. The level of LDH had a significant impact on the response rate to chemotherapy with GDP (P?=?0.015). Myelosuppression was the main side effect; the incidence of grade 3–4 anemia was 8.3?%; leukopenia, 37.5?%; and thrombocytopenia, 48.3?%. Univariate and multivariate analyses were performed to determine variables for OS and PFS. This study confirms that GDP is an effective and safe salvage regimen in relapsed or refractory AIDS-NHL, was associated with modest declines in CD4⁺ lymphocyte counts, and did not promote HIV-1 viral replication.     

Combined congenital dysfibrinogenemia and factor VII deficiency from mutations in the FGB and F7 genes

Dysfibrinogenemia and factor VII (FVII) deficiency are rare congenital coagulopathies. In this report, the authors describe a man with both defects confirmed by molecular genetic tests. The patient was a 51-year-old man referred for prolonged prothrombin time (PT) that had been accidentally detected on preoperative screening. He had no history of bleeding tendency even on occasions of surgery. Routine coagulation studies revealed prolonged PT (1.53 INR) and thrombin time (42.2 s), and decreased fibrinogen level (57 mg/dl) and FVII activity (44%). Direct sequencing analyses were performed on FGA, FGB, and FGG genes to confirm dysfibrinogenemia and on the F7 gene to confirm FVII deficiency. As a result, the patient was shown to be heterozygous for a point mutation in exon 8 of the FGB gene (c.1475A > G, p.*492Trpext*12; Fibrinogen Magdeburg II) and for a missense mutation in exon 6 of the F7 gene (c.466G > A, p.Gly156Ser). To our knowledge, this is the first report on a case of combined dysfibrinogenemia and FVII deficiency confirmed by molecular genetic tests.     

Effects of vagus nerve preservation and vagotomy on peptide YY and body weight after subtotal gastrectomy

AIM: To investigate the relationship between the function of vagus nerve and peptide YY_3-36 and ghrelin levels after subtotal gastrectomy.METHODS: We enrolled a total of 16 patients who underwent subtotal gastrectomy due to gastric cancer. All surgeries were performed by a single skilled surgeon. We measured peptide YY_3-36, ghrelin, leptin, insulin, growth hormone levels, and body weight immediately before and one month after surgery.RESULTS: Vagus nerve preservation group showed less body weight loss and less increase of peptide YY_3-36 compared with vagotomy group (-5.56 ± 2.24 kg vs -7.85 ± 1.57 kg, P = 0.037 and 0.06 ± 0.08 ng/mL vs 0.19 ± 0.12 ng/mL, P = 0.021, respectively). Moreover, patients with body weight loss of less than 10% exhibited reduced elevation of peptide YY_3-36 level, typically less than 20% [6 (66.7%) vs 0 (0.0%), P = 0.011, odd ratio = 3.333, 95% confidence interval (1.293, 8.591)].CONCLUSION: Vagus nerve preservation contributes to the maintenance of body weight after gastrectomy, and this phenomenon may be related to the suppressed activity of peptide YY_3-36.     

Predictive value of ¹⁸F-fluorodeoxyglucose PET/CT for transarterial chemolipiodolization of hepatocellular carcinoma

AIM: To investigate the correlation of ¹⁸F-fluorodeoxyglucose (¹⁸F-FDG) positron emission tomography (PET) with clinical features and the prediction of treatment response.METHODS: A total of 83 hepatocellular carcinoma (HCC) patients undergoing ¹⁸F-FDG PET before transarterial chemolipiodolization with systemic chemo-infusion between October, 2006 and May, 2009 were retrospectively enrolled. The patients included 68 men and 15 women (mean age, 60 ± 10.7 years). The effect of ¹⁸F-FDG-monitored PET uptake on clinical features and on the evaluated treatment response was ascertained with modified Response Evaluation Criteria in Solid Tumors. The PET parameters of maximal standardized uptake value of the tumor (Tsuv_max), the ratio of the tumor maximal standardized uptake value (SUV) to the liver maximal SUV (Tsuv_max/Lsuv_max) and the ratio of tumor maximal SUV to the liver mean SUV (Tsuv_max/Lsuv_mean) were tested as predictive factors.RESULTS: Among the 3 SUV parameters, the Tsuv_max/Lsuv_mean ratio (cutoff value of 1.90) was significantly associated with tumor burden including tumor size, tumor number, α-fetoprotein levels and tumor stage (P < 0.001, P = 0.008, P = 0.011, P < 0.001, respectively). The objective response rates in patients with a high SUV ratio (≥ 1.90) were significantly better than those with a low SUV ratio (< 1.90) (P = 0.020). The overall survival rates of patients exhibiting a low Tsuv_max/Lsuv_mean ratio (< 1.90) and those with a high SUV ratio (≥ 1.90) was 38.2 and 10.3 mo, respectively (P < 0.01). However, the time to progression showed no significant difference between the groups (P = 0.15).CONCLUSION: ¹⁸F-FDG PET can be an important predictor of HCC treatment. In particular, the Tsuv_max/Lsuv_mean ratio (cutoff value of 1.90) can provide useful information in treatment prognosis for HCC patients treated with locoregional therapy.