Scoping review of clinical practice guidelines on the management of benign liver tumours

OBJECTIVE: Benign liver tumours (BLT) are increasingly diagnosed as incidentalomas. Clinical implications and management vary across and within the different types of BLT. High-quality clinical practice guidelines are needed, because of the many nuances in tumour types, diagnostic modalities, and conservative and invasive management strategies. Yet, available observational evidence is subject to interpretation which may lead to practice variation. Therefore, we aimed to systematically search for available clinical practice guidelines on BLT, to critically appraise them, and to compare management recommendations. DESIGN: A scoping review was performed within MEDLINE, EMBASE, and Web of Science. All BLT guidelines published in peer-reviewed, and English language journals were eligible for inclusion. Clinical practice guidelines on BLT were analysed, compared, and critically appraised using the Appraisal of Guidelines, Research and Evaluation (AGREE II) checklist regarding hepatic haemangioma, focal nodular hyperplasia (FNH), and hepatocellular adenoma (HCA). Preferred Reporting Items for Systematic Reviews and Meta-Analyses recommendations (PRISMA) for scoping reviews were adhered to. RESULTS: The literature search yielded unique 367 papers, 348 were excluded after screening of title/abstract, and 16 after full-text screening. Three guidelines were included: the American College of Gastroenterology (ACG; 2014), Brazilian Society of Hepatology (SBH; 2015), and European Association for the Study of the Liver (EASL; 2016). There was no uniformity in the assessment methods for grading and gravity of recommendations between guidelines. Among observed differences were: (1) indications for biopsy in all three tumours; (2) advices on contraceptive pills and follow-up in FNH and HCA; (3) use of an individualised approach to HCA; (4) absence of recommendations for treatment of HCA in men; and (5) approaches to HCA subtype identification on magnetic resonance imaging. CONCLUSION: Recognising differences in recommendations can assist in harmonisation of practice standards and identify unmet needs in research. This may ultimately contribute to improved global patient care

A female patient with liver failure and pseudocirrhosis

AchtergrondAcuut-op-chronisch leverfalen wordt gekenmerkt door acute, ernstige verslechtering van de leverfunctie bij patiënten met pre-existente cirrose, gerelateerd aan een uitlokkende factor. Dat klinische beeld kan soms het gevolg zijn van een andere aandoening die niet primair de lever treft.CasusEen 65-jarige vrouw, bekend met cirrose, werd overgeplaatst naar ons transplantatiecentrum vanwege het beeld van acuut-op-chronisch leverfalen. Vanwege haar voorgeschiedenis van mammacarcinoom, onverwacht afwijkende laboratoriumuitslagen en een fenotype dat niet geheel bij de diagnose paste, verrichtten we een leverbiopsie. Het biopt toonde diffuse metastasering van mammacarcinoom. Eerder beeldvormend onderzoek had kenmerken van cirrose laten zien, zonder aanwijzingen voor maligniteit; dit misleidende beeld wordt pseudocirrose genoemd.ConclusieEen diffuus hepatogeen gemetastaseerde maligniteit kan lijken op gedecompenseerde cirrose en acuut-op-chronisch leverfalen, zowel in presentatie als bij beeldvormend onderzoek. Levertransplantatie is bij acuut-op-chronisch leverfalen soms de enige levensreddende behandeling, maar is gecontra-indiceerd bij maligniteit. Om een gegronde indicatie voor levertransplantatie te verkrijgen moet een leverbiopsie worden overwogen, ook in spoedsituaties.BACKGROUND: Acute-on-chronic liver failure encompasses an acute deterioration of liver function in patients with pre-existent cirrhosis. Sometimes the clinical picture of acute-on-chronic liver failure is misleading and may not be secondary to primary liver disease, as described in our case.CASE DESCRIPTION: A 65-year-old woman with cirrhosis was transferred to our transplantation centre because of suspected acute-on-chronic liver failure. Given her medical history of breastcancer and suspicious laboratory results, we performed a liver biopsy. This showed diffuse metastases of mammary carcinoma. Earlier CT-scans showed features of cirrhosis without signs of malignancy: a misleading phenomenon called pseudocirrhosis.CONCLUSION: Diffuse malignant hepatic infiltration can resemble cirrhosis and acute-on-chronic liver failure, both in clinical presentation as in imaging. Liver transplantation is contraindicated in malignant liver failure. To assure a solid indication for transplantation, a liver biopsy has to be considered, even in emergency situations.</p

Surgery for Ampullary Cancer in a Patient with Pancreatic Lipomatosis Caused by Cystic Fibrosis

A patient with cystic fibrosis (CF) with pancreatic insufficiency presented with jaundice due to an ampullary tumour. CF is known for a higher incidence of gastrointestinal malignancies. The patient suffered from pancreatic insufficiency. At computed tomography (CT), pancreatic lipomatosis with absence of the pancreatic duct was seen. This is uncommon, also in patients with CF. During surgery, a total pancreatectomy was performed, because there was no possibility to construct a duct to mucosa anastomosis due to the absence of the pancreatic duct and more importantly the pancreas was already afunctional. The presence of lipomatosis increases the risk of leakage at the pancreaticojejunal anastomosis. Therefore, it is important to take this phenomenon, in this case already visible on the preoperative CT scan, into account during the workup for surgery

Behavior and complications of hepatocellular adenoma during pregnancy and puerperium:a retrospective study and systematic review

BACKGROUND: Hepatocellular adenomas (HCA) are benign liver tumors at risk of hemorrhage. The influence of pregnancy on HCA growth and potential bleeding remains unclear. This study investigates HCA-associated behavior and bleeding complications during or shortly after pregnancy. METHODS: (I) Single center retrospective cohort study of HCA during and after pregnancy (II) Systematic literature review. RESULTS: The retrospective study included 11 patients, of which 4 with HCA ≥5 cm. In only two patients HCA showed growth during pregnancy. In this local cohort, no HCA-related hemorrhages occurred during median follow-up of 34 months (interquartile range 19-58 months). The systematic review yielded 33 studies, totaling 90 patients with 99 pregnancies. Of 73 pregnancies without prior HCA-related intervention, 39 HCA remained stable (53.4%), 11 regressed (15.1%), and 23 (31.5%) progressed. Fifteen HCA-related hemorrhages occurred in HCA measuring 6.5-17.0 cm. Eight patients experienced bleeding during pregnancy, two during labor and five postpartum. CONCLUSION: Although hemorrhage of HCA during or shortly after pregnancy is rare and only reported in HCA ≥6.5 cm, it can be fatal. Pregnancy in women with HCA, regardless of size, warrant a close surveillance strategy. Observational studies on behavior and management of HCA ≥5 cm during and immediately after pregnancy are needed

Abrupt appearance of the domain pattern and fatigue of thin ferroelectric films

We study the domain structure in ferroelectric thin films with a `passive' layer (material with damaged ferroelectric properties) at the interface between the film and electrodes within a continuous medium approximation. An abrupt transition from a monodomain to a polydomain state has been found with the increase of the `passive' layer thickness $d$. The domain width changes very quickly at the transition (exponentially with $d^{-2}$). We have estimated the dielectric response $dP/dE$ (the slope of the hysteresis loop) in the `fatigued' multidomain state and found that it is in agreement with experiment, assuming realistic parameters of the layer. We derive a simple universal relation for the dielectric response, which scales as $1/d$, involving only the properties of the passive layer. This relation qualitatively reproduces the evolution of the hysteresis loop in fatigued samples and it could be tested with controlled experiments. It is expected that the coercive field should increase with decreasing lateral size of the film. We believe that specific properties of the domain structure under bias voltage in ferroelectrics with a passive layer can resolve the long-standing `paradox of the coercive field'.Comment: 5 pages, REVTeX 3.1 with two eps-figures. Minor amendments. To appear in Phys. Rev. Letter

Dynamic Phase Transition, Universality, and Finite-size Scaling in the Two-dimensional Kinetic Ising Model in an Oscillating Field

We study the two-dimensional kinetic Ising model below its equilibrium critical temperature, subject to a square-wave oscillating external field. We focus on the multi-droplet regime where the metastable phase decays through nucleation and growth of many droplets of the stable phase. At a critical frequency, the system undergoes a genuine non-equilibrium phase transition, in which the symmetry-broken phase corresponds to an asymmetric stationary limit cycle for the time-dependent magnetization. We investigate the universal aspects of this dynamic phase transition at various temperatures and field amplitudes via large-scale Monte Carlo simulations, employing finite-size scaling techniques adopted from equilibrium critical phenomena. The critical exponents, the fixed-point value of the fourth-order cumulant, and the critical order-parameter distribution all are consistent with the universality class of the two-dimensional equilibrium Ising model. We also study the cross-over from the multi-droplet to the strong-field regime, where the transition disappears

Indeterminate pediatric acute liver failure:Clinical characteristics of a temporal cluster of five children in the Netherlands in the spring of 2022

There is increasing global concern of severe acute hepatitis of unknown etiology in young children. In early 2022, our center for liver transplantation in the Netherlands treated five children who presented in short succession with indeterminate acute liver failure. Four children underwent liver transplantation, one spontaneously recovered. Here we delineate the clinical course and comprehensive diagnostic workup of these patients. Three of five patients showed a gradual decline of liver synthetic function and had mild neurological symptoms. Their clinical and histological findings were consistent with hepatitis. These three patients all had a past SARS-CoV-2 infection and two of them were positive for adenovirus DNA. The other two patients presented with advanced liver failure and encephalopathy and underwent dialysis as a bridge to transplantation. One of these children spontaneously recovered. We discuss this cluster of patients in the context of the currently elevated incidence of severe acute hepatitis in children

Indeterminate pediatric acute liver failure:Clinical characteristics of a temporal cluster of five children in the Netherlands in the spring of 2022

There is increasing global concern of severe acute hepatitis of unknown etiology in young children. In early 2022, our center for liver transplantation in the Netherlands treated five children who presented in short succession with indeterminate acute liver failure. Four children underwent liver transplantation, one spontaneously recovered. Here we delineate the clinical course and comprehensive diagnostic workup of these patients. Three of five patients showed a gradual decline of liver synthetic function and had mild neurological symptoms. Their clinical and histological findings were consistent with hepatitis. These three patients all had a past SARS-CoV-2 infection and two of them were positive for adenovirus DNA. The other two patients presented with advanced liver failure and encephalopathy and underwent dialysis as a bridge to transplantation. One of these children spontaneously recovered. We discuss this cluster of patients in the context of the currently elevated incidence of severe acute hepatitis in children.</p