Polycystic liver diseases (PLD) represent a group of genetic disorders in which cysts occur in the liver (autosomal dominant polycystic liver disease) or in combination with cysts in the kidneys (autosomal dominant polycystic kidney disease). Regardless of the genetic mutations, the natural history of these disorders is alike. The natural history of PLD is characterized by a continuous increase in the volume and the number of cysts. Both genders are affected; however, women have a higher prevalence. Most patients with PLD are asymptomatic and can be managed conservatively. Severe symptoms can affect 20% of patients who develop massive hepatomegaly with compression of the surrounding organs. Rrarely, patients with PLD suffer from acute complications caused by the torsion of hepatic cysts, intraluminal cystic hemorrhage and infections. The most common methods for the diagnosis of PLD are cross sectional imaging studies. Abdominal ultrasound and computerized tomography are the two most frequently used investigations. Magnetic resonance imaging is more sensitive and specific, and it is a valuable test for patients with intravenous contrast allergies or renal dysfunction. Different treatment modalities are available to physicians caring for these patients. Medical treatment has been ineffective. Percutaneous sclerotherapy, trans-arterial embolization, cyst fenestration, hepatic resection and liver transplantation are indicated to specific groups of patients and have to be tailored according to the extent of disease. This review outlines the current knowledge of the pathophysiology, clinical course, diagnosis and treatment strategies of PLD.

BackgroundControversy still exists on the effect that obesity has on the morbidity and mortality in severe acute pancreatitis (SAP). The primary purpose of this study was to compare the mortality rate of obese versus nonobese patients admitted to the ICU for SAP. Secondary goals were to assess the potential risk factors for abdominal compartment syndrome (ACS) and to investigate the performance of validated scoring systems to predict ACS and in-hospital mortality.MethodsA retrospective cohort of adults admitted to the ICU for SAP was stratified by their body mass index (BMI) as obese and nonobese. The rates of morbidity, mortality, and ACS were compared by univariate and multivariate regression analyses. Areas under the curve (AUC) were used to evaluate the discriminating performance of severity scores and other selected variables to predict mortality and the risk of ACS.ResultForty-five patients satisfied the inclusion criteria and 24 (53 %) were obese with similar characteristics to nonobese patients. Among all the subjects, 11 (24 %) died and 16 (35 %) developed ACS. In-hospital mortality was significantly lower for obese patients (12.5 vs. 38 %; P = 0.046) even though they seemed to develop ACS more frequently (41 vs. 28 %; P = 0.533). At multivariable analysis, age was the most significant factor associated with in-hospital mortality (odds ratio (OR) = 1.273; 95 % confidence interval (CI) 1.052–1.541; P = 0.013) and APACHE II and Glasgow-Imrie for the development of ACS (OR = 1.143; 95 % CI 1.012–1.292; P = 0.032 and OR = 1.221; 95 % CI 1.000–1.493; P = 0.05) respectively. Good discrimination for in-hospital mortality was observed for patients’ age (AUC = 0.846) and number of comorbidities (AUC = 0.801). ACS was not adequately predicted by any of the clinical severity scores (AUC = 0.548–0.661).ConclusionsPatients’ age was the most significant factor associated with mortality in patients affected by SAP. Higher APACHE II and Glasgow-Imrie scores were associated with the development of ACS, but their discrimination performance was unsatisfactory.

SummaryHepatic abscesses caused by ingested foreign bodies have been reported in the medical literature but represent very uncommon events. Extra-luminal migration of sawing needles and pins is the most common cause of perforation of the gastrointestinal tract associated with liver infections. Other non-metallic sharp objects such as animal bones and toothpicks have been described but are less frequent. The authors present a case of a 45-year-old woman who suffered from sepsis and a liver abscess because of the migration of a toothpick that lodged in the left hepatic lobe. Review of the literature on the pathogenesis and clinical management of liver abscesses caused by ingested foreign is presented. BACKGROUND

Potential donors were risk takers and were willing to donate when given the opportunity. They accepted significant risks, especially if they had a close emotional relationship with the recipient.

IFALD is a common and potentially life-threatening condition for patients with SBS requiring long-term PN. There exists the potential for decreasing its incidence by optimizing the composition and the rate of infusion of parenteral solutions, by advocating a multidisciplinary approach, and by early referral for intestinal-liver transplantation to ensure long-term survival of patients with SBS.

The diagnosis and the treatment of dominant strictures (DS) in patients with primary sclerosing cholangitis (PSC) is challenging and the scientific literature on the subject is quite limited. Only level II and level III evidence is available to guide physicians managing patients with DS and PSC. For the diagnosis, intraductal endoscopic ultrasound is the most sensitive (64%) and specific (95%) test. However, the majority of cases require a combination of several different diagnostic tests, as there is no single investigation that can rule out malignancy in this group of patients. For the treatment, serial endoscopic or percutaneous dilatations provide 1- and 3-year biliary duct patency in 80 and 60% of patients, respectively. Dilatation and stenting are the most common interventions, although the optimal duration of treatment has still not been clearly defined. Bile duct resection and/or bilioenteric bypass are currently indicated only for patients with preserved liver function. For all other patients, benign DS can be treated with endoscopic dilatation with short-term stenting. This approach is effective and safe and does not increase the risk of malignant transformation or complications for liver transplant candidates. During the last decade, the use of self-expandable metallic stents for benign diseases has become an innovative option. The aim of this article is to review the diagnostic and therapeutic strategies for patients affected by PSC and DS with specific emphasis on the outcomes of patients treated with temporary stents.

Inflammatory pseudotumours (IPTs) of the biliary tract are extremely rare and heterogeneous by aetiology and clinical presentation. They might cause biliary obstruction and mimic cholangiocarcinomas and their final diagnosis is usually achieved only after surgical excision. The most characteristic feature of IPT is the presence of chronic inflammatory cell infiltrates with variable degree of proliferating fibrous tissue. IPTs have the potential for recurrence even after resection and if untreated they can grow causing a variety of symptoms due to compression of the surrounding structures and organs. Despite the significant improvement of modern imaging techniques, preoperative distinction between IPTs of the biliary system and malignancies is extremely difficult. Histological diagnosis poses a clinical challenge because sampling is often suboptimal. Although rare, IPTs should be in the differential diagnosis of patients who present with painless jaundice and no other clinical symptoms or signs characteristics of cholangiocarcinomas.

SUMMARYMalignant serous cystic neoplasms (SCN) of the pancreas are exceptionally rare, and only a few cases have been reported. As a result, SCN have been unanimously classified as benign tumours. Contrary to this conviction, in 1989, George et al published the very first case of a patient found to have a malignant pancreatic SCN. Up to the time of the submission of this paper, 27 cases of serous cystoadenocarcinomas have been published. In all the previously published cases of malignant SCN, the correct diagnosis was made postoperatively or at the time of autopsy. The authors present a case of a 68-year-old patient who was incidentally found to have a large liver mass on transthoracic echocardiogram ordered for suspected coronary artery insufficiency. Subsequent investigations revealed an additional large mass in the pancreas and percutaneous biopsies of both lesions revealed histological features consistent with malignant SCN metastasised to the left hepatic lobe. BACKGROUND