Is spleen circulation impaired in systemic sclerosis and what is the role of liver fibrosis?

AIM:To investigate the spleen vascular involvement and the presence of liver fibrosis in a population of subjects with established systemic sclerosis(SSc).METHODS:In a cross-sectional fashion,17 patients with SSc were compared with 18 patients suffering from hepatitis C virus(HCV) -related liver cirrhosis,grade A and B Child-Pugh classification.Eighteen non elderly subjects,apparently healthy,were used as the control group.Splenic artery resistivity index(SARI) at doppler ultraSound,transient elastography of liver and nailfold capillaroscopy were the main outcomes.RESULTS:Transient elastography values of SSc patients were similar to those of controls;5.2±1.1 vs 4.5 ±1,(P=0.07).Median Alanine amino transferase(ALT) concentrations of cirrhotic patients were greater than those of controls and SSc patients,i.e.66.5(36-89) U/L vs 29(22-34) U/L and 31(22-41) U/L,respectively,(P =0.005).SARI determinations in cirrhotic patients,although significantly higher than those found in controls and SSc patients,showed some degree of overlap with SSc patients,i.e.0.59 vs 0.52 and 0.57,respectively,(P =0.04).Mean systolic blood pressure was significantly higher in SSc patients than in cirrhotics and controls,i.e.142 mmHg vs 128.2 mmHg and 127 mmHg,respectively,(P=0.005).Mean diastolic blood pressure behaved in a similar fashion,i.e.84 mmHg vs 72.2 mmHg and 76.9 mmHg(P=0.005).Nailfold Capillaroscopy grades and diastolic blood pressure values correlated well with SARI results.CONCLUSION:An enhanced resistivity of the splenic artery was found in patients suffering from SSc;they did not have evidence of splenomegaly as well as no liver fibrosis or any other form of liver damage.

Intestinal amyloidosis:Two cases with different patterns of clinical and imaging presentation

The involvement of the small bowel in systemic forms of amyloidosis may be diffuse or very rarely focal.Some cases of focal amyloidomas of the duodenum and jejunum without extraintestinal manifestations have been reported.The focal amyloidomas consisted of extensive amyloid infiltration of the entire intestinal wall thickness.Radiological barium studies,ultrasound and computed tomography(CT)patterns of diffuse small bowel amyloidosis have been described:the signs are non-specific and may include small-bowel dilatation,symmetric bowel wall thickening,mesenteric infiltration,and mesenteric adenopathy.No data are available about the positron emission tomography (PET)/CT and magnetic resonance imaging(MRI)patterns of intestinal amyloidosis.We report two cases of small bowel amyloidosis:the former characterized by focal deposition of amyloid proteins exclusively within blood vessel walls of the terminal ileum,the latter characterized by diffuse intestinal involvement observed on MRI and PET/CT studies.

Colorectal cancer and 18FDG-PET/CT:What about adding the T to the N parameter in loco-regional staging?

AIM:To evaluate whether FDG-positron emission tomography(PET)/computed tomography(CT)may be an accurate technique in the assessment of the T stage in patients with colorectal cancer.METHODS:Thirty four consecutive patients(20 men and 14 women;mean age:63 years)with a histologically proven diagnosis of colorectal adenocarcinoma and scheduled for surgery in our hospital were enrolled in this study.All patients underwent FDG-PET/CT preoperatively.The primary tumor site and extent were evaluated on PET/CT images.Colorectal wall invasion was analysed according to a modified T classification that considers only three stages(≤T2,T3,T4).Assessment of accuracy was carried out using 95%confidence intervals for T.RESULTS:Thirty five/37(94.6%)adenocarcinomas were identified and correctly located on PET/CT images.PET/CT correctly staged the T of 33/35 lesions identified showing an accuracy of 94.3%(95%CI:87%-100%).All T1,T3 and T4 lesions were correctly staged,while two T2 neoplasms were overstated as T3.CONCLUSION:Our data suggest that FDG-PET/CT may be an accurate modality for identifying primary tumor and defining its local extent in patients with colorectal cancer.

In-vivo characterization of DALM in ulcerative colitis with high-resolution probe-based confocal laser endomicroscopy

Recently,the use of confocal laser endomicroscopy(CLE) in the diagnosis of chronic ulcerative colitis(CUC) was reported.In this brief report we aimed to assess the application of probe-based CLE to characterize colonic mucosa and dysplasia in CUC.The study involved a patient presenting long-standing CUC.Confocal imaging of both the inflamed mucosa,a circumscribed lesion(dysplasiaassociated lesional mass),and adjacent colonic mucosa are demonstrated and the correlation between the CLE and histological images.Inflamed mucosa and dysplasia showed specific alteration of crypt architecture,cellular infiltration,and vessel architecture with an excellent correlation between CLE and standard histological examination.

Serum Bcl-2 concentrations in overweight-obese subjects with nonalcoholic fatty liver disease

AIM: To shed some light on the relationship between anti-apoptotic serum Bcl-2 concentrations and metabolic status, anthropometric parameters, inflammation indices, and non-alcoholic fatty liver disease severity were investigated in 43 young individuals with fatty liver (FL)and 41 with nonalcoholic steatohepatitis (NASH).METHODS: Circulating levels of Bcl-2 were detected in 84 patients with ultrasono graphic findings of "bright liver" and/or hyper-transaminasemia of unknown origin and/or increase in γ-glutamyl-transpeptidase (γ-GT)strictly in the absence of other acute or chronic liver disease, whose age was not advanced, who gave consent to liver biopsy and were then divided on the basis of the histological results into two groups (43 with FL and 41 with NASH). Twenty lean subjects, apparently healthy and young, were chosen as controls.RESULTS: Serum Bcl-2 concentrations were significantly higher in the FL group than in the NASH group. Insulin resistance and γ-GT activity were significantly higher in NASH subjects. Apoptotic hepatocytes were significantly more numerous in NASH patients. NASH patients presented with larger spleens and augmented C-reactive protein (CRP) concentrations than healthy subjects. Steatosis grade at histology was similar in both NASH and FL populations. The number of apoptotic cells was significantly related to anti-apoptotic Bcl-2 protein values in FL patients. Bcl-2 serum levels positively correlated to body mass index (BMI) values (P ≤ 0.0001) but not to age of the population. Triglycerides/HDL ratio correlated well to waist circumference in males (P = 0.0008).γ-GT activity was associated with homeostatic metabolic assessment (HOMA) (P = 0.0003) and with serum ferritin (P = 0.02). Bcl-2 concentrations were not related to either spleen size or CRP values. NASH patients presented a weak negative correlation between lobular inflammation and Bcl-2 levels. A prediction by low values of serum Bcl-2 towards a greater presence of metaboli-cally unhealthy overweight/obese patients (MUOs) was evidenced. HOMA, BMI and uric acid, in that sequence,best predicted serum Bcl-2 concentrations.CONCLUSION: MUOs could be detected by Bcl-2 levels.By favoring the life span of hepatocytes, and enhancing triglyceride formation, the anti-apoptotic process inhibits free fatty acids toxicity in FL.

Hepatocellular carcinoma metastatic to the kidney mimicking renal oncocytoma

BACKGROUND:Renal metastases of hepatocellular carcinoma(HCC) are very rare.To our knowledge only five cases have been reported to the present;all had a well-known primary HCC.METHODS:We describe the clinico-pathological features of a rare case of HCC metastatic to the kidney in which the renal mass was the clinical debut of disease.The patient was a 54-year-old woman previously submitted to orthotopic liver transplantation,who underwent left nephrectomy for a renal mass.RESULTS:Histologically,the tumor was composed mainly of epithelioid cells with homogeneous acidophilic cytoplasm resembling oncocytoma or primary renal carcinoma with oncocytic features.A correct diagnosis was made on the basis of positive immunostaining for hepatocyte paraffin 1.CONCLUSIONS:Metastasis to the kidney is a rare complication that should be considered whenever a renal mass is present in patients with HCC.Since HCC may histologically resemble primary renal tumors such as oncocytoma,pathologists must be aware of this possibility above all in patients referred for liver transplantation and treated with immunosuppressant drugs.Immunohistochemistry is particularly helpful to establish a precise diagnosis in cases of doubt.

在在病人诊断发育异常的共焦的激光 endomicroscopy 的诊断精确性由长期的 ulcerative 大肠炎影响了

AIM:To evaluate the diagnostic accuracy of confocal laser endomicroscopy(CLE) for the detection of dysplasia in long-standing ulcerative colitis(UC).METHODS:We prospectively performed a surveillance colonoscopy in 51 patients affected by long-standing UC.Also,in the presence of macroscopic areas with suspected dysplasia,both targeted contrasted indigo carmine endoscopic assessment and probe-based CLE were performed.Colic mucosal biopsies and histology,utilised as the gold standard,were assessed randomly and on visible lesions,in accordance with current guidelines.RESULTS:Fourteen of the 51 patients(27%) showed macroscopic mucosal alterations with the suspected presence of dysplasia,needing chromoendoscopic and CLE evaluation.In 5 macroscopically suspected cases,the presence of dysplasia was confirmed by histology(3 flat dysplasia;2 DALMs).No dysplasia/cancer was found on any of the outstanding random biopsies.The diagnostic accuracy of CLE for the detection of dysplasia compared to standard histology was sensitivity 100%,specificity 90%,positive predictive value 83% and negative predictive value 100%.CONCLUSION:CLE is an accurate tool for the detection of dysplasia in long-standing UC and shows optimal values of sensitivity and negative predictivity.The scheduled combined application of chromoendoscopy and CLE could maximize the endoscopic diagnostic accuracy for diagnosis of dysplasia in UC patients,thus limiting the need for biopsies.

Does protracted antiviral therapy impact on HCV-related liver cirrhosis progression?

AIM: To study the outcomes of patients with compensated hepatitis C virus-related cirrhosis. METHODS: Twenty-four grade A5 and 11 grade A6 of Child-Pugh classification cirrhotic patients with active virus replication, treated for a mean period of 31.3 ± 5.1 mo with moderate doses of interferon-alpha and ribavirin, were compared to a cohort of 36 patients with similar characteristics, without antiviral treatment. Salivary caffeine concentration, a liver test of microsomal function, was determined at the starting and thrice in course of therapy after a mean period of 11 ± 1.6 mo, meanwhile the resistive index of splenic artery at ultra sound Doppler, an indirect index of portal hypertension, was only measured at the beginning and the end of study. RESULTS: Eight out of the 24 A5- (33.3%) and 5 out of the 11 A6- (45.45%) treated-cirrhotic patients showed a significant improvement in the total overnight salivary caffeine assessment. A reduction up to 20% of the resistive index of splenic artery was obtained in 3 out of the 8 A5- (37.5%) and in 2 out of the 5 A6- (40%) cirrhotic patients with an improved liver function, which showed a clear tendency to decrease at the end of therapy. The hepatitis C virus clearance was achieved in 3 out of the 24 (12.5%) A5- and 1 out of the 11 (0.091%) A6-patients after a median period of 8.5 mo combined therapy. In the cohort of non-treated cirrhotic patients, not only the considered parameters remained unchanged, but 3 patients (8.3%) had a worsening ofthe Child-Pugh score (P = 0.001).CONCLUSION: A prolonged antiviral therapy with moderate dosages of interferon-alpha and ribavirin shows a trend to stable liver function or to ameliorate the residual liver function, the entity of portal hypertension and the compensation status at acceptable costs.

克氏综合征患者的睾丸实质异常：一个有关肿瘤的问题？40例连续患者的研究

Klinefelter syndrome （KS） is a hypergonadotropic hypogonadism characterized by a 47, XXY karyotype. The risk of testicular cancer in KS is of interest in relation to theories about testicular cancer etiology generally; nevertheless it seems to be low. We evaluated the need for imaging and serum tumor markers for testicular cancer screening in KS. Participants were 40 consecutive KSpatients, enrolled from December 2009 to January 2013. Lactate dehydrogenase （LDH）, alpha-fetoprotein （AFP）, and beta-human chorionic gonadotrophin subunit （^-HCG） serum levels assays and testicular ultrasound （US） with color Doppler, were carried out at study entry, after 6 months and every year for 3 years. Abdominal magnetic resonance （MR） was performed in KS when testicular US showed micro-calcifications, testicular nodules and cysts. Nearly 62% of the KS had regular testicular echotexture, 37.5% showed an irregular echotexture and 17.5% had micro-calcifications and cysts. Eighty seven percent of KS had a regular vascular pattern, 12.5% varicocele, 12.5% nodules 〈1 cm, but none had nodules 〉1 cm. MR ruled out the diagnosis of cancer in all KS with testicular micro calcifications, nodules and cysts. No significant variations in LDH, AFP, and ^-HCG levels and in US pattern have been detected during follow-up. We compared serum tumor markers and US pattern between KS with and without cryptorchidism and no statistical differences were found. We did not find testicular cancer in KS, and testicular US, tumor markers and MR were, in selected cases, useful tools for correctly discriminating benign from malignant lesions.