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Liver involvement in patients with sickle cell anemia/trait includes a wide range of alterations, from mild liver function test abnormalities to cirrhosis and acute liver failure. Approximately 15–30% of patients with sickle cell anemia present cirrhosis at autopsy. The pathogenesis of cirrhosis is usually related to chronic hepatitis B or C infection or to iron overload resulting from the many transfusions received by these patients in their lifetime. Thus, cirrhosis has been described almost exclusively in patients with sickle cell anemia, while only mild liver abnormalities have been associated with the sickle cell trait. In the present case study, we describe a young Mediterranean man carrying a sickle cell trait (Hb Sβ+ thalassemia) who developed liver cirrhosis being negative for hepatitis C and B viruses or for other causes of cirrhosis and not receiving chronic blood transfusions.

Background. Although orthotopic liver transplantation (OLT) and combined heart and liver transplantation (CHLT) are accepted therapeutic strategies in ATTR, Centers with an unrestricted access to both facilities are few and long term results are poorly defined. We report results of our transplantation strategy: OLT in symptomatic stage I neuropathy with no or mild heart involvement, CHLT in non-Val30Met stage I neuropathy and severe cardiomyopathy (mean left ventricular wall thickness >14 mm), even in absence of heart failure. Methods. Between 1993 and December 2012, 47 patients underwent organ transplantation (34 OLT and 13 CHLT) and were evaluated for survival and incremental risk factors for mortality. Results. 31 patients were males (66%) with median age of 47 years (range 22-68). Val30Met patients were 15 (32%) and all underwent OLT; the most frequent non-Val30Met mutations were Glu89Gln (n=9, 19%) and Thr49Ala (n=6, 13%). During a median follow-up of 5 years (range 0-20), 19 (40%) patients died (16 OLT, 3 CHLT). Neurologic progression was the cause of death in 6 patients (5 in OLT and 1 CHLT subgroups), severe heart failure in 3 OLT. One-year survival was 77% (71% in OLT and 92% in CHLT). Survival at 5 years was 58% (54% in OLT and 68% in CHLT groups). Cox analysis identified the following independent variables associated with overall mortality: peripheral neuropathy (P=0.03), coexistence of orthostatic hypotension and gastrointestinal symptoms (P=0.02), compromised nutritional status (P=0.03) and long waiting time in list (P=0.04). Conclusions. CHLT improves mid term survival in patients with non-Val30Met mutations, mild neuropathy and definite cardiomyopathy, including those without hemodynamic impairment. However, both OLT and CHLT are suboptimal therapeutical solutions in ATTR since long term efficacy is hampered by disease progression in at least 40% of cases. Pharmacologic strategies against post-transplant wild type transthyretin deposition are needed.

Transcatheter embolization is the mainstay of the therapy of splenic artery aneurysms (SAAs) in patients with portal hypertension. It is indicated when the SAA diameter reaches 20 mm. Although endovascular techniques are effective and safe for the treatment of medium-sized SAAs, little is known about their applicability to large-sized SAAs. Herein, we report a case of giant SAA, which was treated with transcatheter coil embolization. The case was not considered suitable for surgery because of the presence of severe portal hypertension. The procedure was complicated by bacterial infection of the coils within the aneurismatic sac, leading to the development of hepatic failure. A liver transplant was then successfully performed despite the presence of a nonresponsive infection.

Background: Alcoholic liver cirrhosis (ALC) is a leading indication for orthotopic liver transplantation (OLT). Goals: To investigate the long-term outcome of OLT for ALC compared with patients transplanted for hepatitis C virus (HCV) infection. Study: From 1987 to 2001, 49 OLT were performed for ALC and 173 for HCV. From these contemporary groups we matched 1:2 ALC patients (cases) to 98 HCV (controls). The following variables were analyzed: survival, retransplantation, rejection, primary nonfunction, infections, de novo tumors, cardiovascular and neurologic complications, and alcoholic recurrence. Results: Actuarial survival rate at 9 years was comparable for cases and controls. Actuarial graft survival rate at 9 years was significantly higher in cases (78% vs. 60%; P = 0.026). The retransplantation rate was higher in controls (21 % vs. 4%; P = 0.007). Post-OLT complications were not significantly different. The alcoholic recidivism rate was 28% without influence on patients or graft survival, whereas relapse of HCV caused the majority of death in controls (30%; P = 0.042). At multivariate analysis retransplantation was the only predictor of patient survival (odds ratio: 4.35; 95% confidence interval: 2.16-8.74; P < 0.001), whereas HCV was associated with a 2-fold probability of graft failure (odds ratio: 1.97; 95% confidence interval: 1.02-3.81; P = 0.032). Conclusions: The long-term outcome of OLT for ALC is comparable to that for HCV, even if graft survival is significantly better among ALC. These data support ALC as an excellent indication for OLT.

Background. Many investigators have proposed an association between gastro-oesophageal reflux disease and laryngo-pharyngeal symptoms, suggesting that medical or surgical therapy for reflux may be useful. Aim: To perform a meta-analysis assessing the effectiveness of medical or surgical therapy for reflux disease in adult patients with laryngeal or pharyngeal symptoms presumed to be due to gastro-oesophageal reflux disease. Methods: Randomized controlled trials comparing medical or surgical treatments for gastro-oesophageal reflux disease against placebo were identified by searching MEDLINE (1966–September 2005), EMBASE (1974–September 2005), the CCRCT (until September 2005) and abstracts from gastroenterology and ENT meetings. The relative risks of reporting symptomatic improvement or resolution of symptoms was evaluated using a random-effects model. Results: Five studies using high-dose proton pump inhibitor as intervention met the inclusion criteria and were included in the meta-analysis. No surgical studies met inclusion criteria. The pooled relative risk was 1.18 (95% confidence interval: 0.81–1.74). There was no heterogeneity between studies but evidence of significant publication bias. Sub-group analysis performed evaluating Jadad scores and symptom type, did not change the relative risk. Conclusions: Therapy with a high-dose proton pump inhibitor is no more effective than placebo in producing symptomatic improvement or resolution of laryngo-pharyngeal symptoms. Further studies are necessary to identify the characteristics of patients that may respond to proton pump inhibitor therapy.