[Diagnostics and clinical management of premalignant diseases of the pancreas]. / Diagnostik und klinisches Management prämaligner Erkrankungen des Pankreas.

Pancreatic cancer is one of the most aggressive solid tumors and still has a poor prognosis. A delayed diagnosis at advanced stages and a poor response to systemic treatment frequently make a curative treatment impossible. Therefore, the identification of high-risk patients and screening them regularly is the most promising approach to improve the prognosis. Chronic pancreatitis as well as neoplastic pancreatic cysts can greatly increase the risk of developing pancreatic cancer. Furthermore, familial syndromes and germline mutations also confer an increased risk for development of pancreatic cancer. This article provides an overview of the various premalignant diseases of the pancreas. The value of the various imaging modalities, such as magnetic resonance imaging and endosonography are particularly discussed as well as the screening interval and the indications for surgical treatment are explained.

Study protocol P-MAPS: microbiome as predictor of severity in acute pancreatitis-a prospective multicentre translational study.

BACKGROUND: Acute pancreatitis (AP) is an inflammatory disorder that causes a considerable economic health burden. While the overall mortality is low, around 20% of patients have a complicated course of disease resulting in increased morbidity and mortality. There is an emerging body of evidence that the microbiome exerts a crucial impact on the pathophysiology and course of AP. For several decades multiple clinical and laboratory parameters have been evaluated, and complex scoring systems were developed to predict the clinical course of AP upon admission. However, the majority of scoring systems are determined after several days and achieve a sensitivity around 70% for early prediction of severe AP. Thus, continued efforts are required to investigate reliable biomarkers for the early prediction of severity in order to guide early clinical management of AP patients. METHODS: We designed a multi-center, prospective clinical-translational study to test whether the orointestinal microbiome may serve as novel early predictor of the course, severity and outcome of patients with AP. We will recruit 400 AP patients and obtain buccal and rectal swabs within 72 h of admission to the hospital. Following DNA extraction, microbiome analysis will be performed using 3rd generation sequencing Oxford Nanopore Technologies (ONT) for 16S rRNA and metagenomic sequencing. Alpha- and beta-diversity will be determined and correlated to the revised Atlanta classification and additional clinical outcome parameters such as the length of hospital stay, number and type of complications, number of interventions and 30-day mortality. DISCUSSION: If AP patients show a distinct orointestinal microbiome dependent on the severity and course of the disease, microbiome sequencing could rapidly be implemented in the early clinical management of AP patients in the future. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT04777812.

Scheduling nab-paclitaxel combined with gemcitabine as first-line treatment for metastatic pancreatic adenocarcinoma.

BACKGROUND: Nab-paclitaxel plus gemcitabine (nabP+gemcitabine) offers modest survival gains for patients with metastatic pancreatic ductal adenocarcinoma (PDAC). Sequential scheduling of nabP+gemcitabine in a PDAC mouse model improved efficacy; this hypothesis was tested in a clinical trial. METHODS: Patients with previously untreated metastatic PDAC were randomised to receive nabP+gemcitabine administered either concomitantly on the same day, or sequentially, with gemcitabine administered 24 h after nabP. The primary outcome measure was progression-free survival (PFS). Secondary outcome measures were objective response rate (ORR), overall survival (OS), safety, quality of life (QoL) and predictive biomarkers. RESULTS: In total, 71 patients received sequential (SEQ) and 75 concomitant (CON) treatment. Six-month PFS was 46% with SEQ and 32% with CON scheduling. Median PFS (5.6 versus 4.0 months, hazard ratio [HR] 0.67, 95% confidence interval [95% CI] 0.47-0.95, p = 0.022) and ORR (52% versus 31%, p = 0.023) favoured the SEQ arm; median OS was 10.2 versus 8.2 months (HR 0.93, 95% CI 0.65-1.33, p = 0.70). CTCAE Grade ≥3 neutropaenia incidence doubled with SEQ therapy but was not detrimental to QoL. Strongly positive tumour epithelial cytidine deaminase (CDA) expression favoured benefit from SEQ therapy (PFS HR 0.31, 95% CI 0.13-0.70). CONCLUSIONS: SEQ delivery of nabP+gemcitabine improved PFS and ORR, with manageable toxicity, but did not significantly improve OS. CLINICAL TRIAL REGISTRATION: ISRCTN71070888; ClinialTrials.gov (NCT03529175).

Fibroblast drug scavenging increases intratumoural gemcitabine accumulation in murine pancreas cancer.

OBJECTIVE: Desmoplasia and hypovascularity are thought to impede drug delivery in pancreatic ductal adenocarcinoma (PDAC). However, stromal depletion approaches have failed to show clinical responses in patients. Here, we aimed to revisit the role of the tumour microenvironment as a physical barrier for gemcitabine delivery. DESIGN: Gemcitabine metabolites were analysed in LSL-KrasG12D/+ ; LSL-Trp53R172H/+ ; Pdx-1-Cre (KPC) murine tumours and matched liver metastases, primary tumour cell lines, cancer-associated fibroblasts (CAFs) and pancreatic stellate cells (PSCs) by liquid chromatography-mass spectrometry/mass spectrometry. Functional and preclinical experiments, as well as expression analysis of stromal markers and gemcitabine metabolism pathways were performed in murine and human specimen to investigate the preclinical implications and the mechanism of gemcitabine accumulation. RESULTS: Gemcitabine accumulation was significantly enhanced in fibroblast-rich tumours compared with liver metastases and normal liver. In vitro, significantly increased concentrations of activated 2',2'-difluorodeoxycytidine-5'-triphosphate (dFdCTP) and greatly reduced amounts of the inactive gemcitabine metabolite 2',2'-difluorodeoxyuridine were detected in PSCs and CAFs. Mechanistically, key metabolic enzymes involved in gemcitabine inactivation such as hydrolytic cytosolic 5'-nucleotidases (Nt5c1A, Nt5c3) were expressed at low levels in CAFs in vitro and in vivo, and recombinant expression of Nt5c1A resulted in decreased intracellular dFdCTP concentrations in vitro. Moreover, gemcitabine treatment in KPC mice reduced the number of liver metastases by >50%. CONCLUSIONS: Our findings suggest that fibroblast drug scavenging may contribute to the clinical failure of gemcitabine in desmoplastic PDAC. Metabolic targeting of CAFs may thus be a promising strategy to enhance the antiproliferative effects of gemcitabine.

Detection of peripheral embolic consolidations using contrast-enhanced ultrasonography in patients with no evidence of pulmonary embolism on computed tomography: A pilot study.

AIM: To investigate the value of B-mode imaging and contrast-enhanced ultrasonography (CEUS) in patients with clinically suspected pulmonary embolism (PE) but no evidence of central PE on CT. METHODS: Between May 2004 and February 2015, we included in this retrospective study 19 patients with a risk profile for PE according to their Wells' score, sonographic patterns of peripheral embolic consolidations (EC) on B-mode-imaging and CEUS (ie, missing or inhomogeneous enhancement of the pleural lesions), and exclusion of central PE by CT within 1 week of CEUS. RESULTS: On B-mode imaging, 19 pleural defects presented as hypoechoic. The shape of EC was round in 2, wedge-shaped in 12, polygonal in 3, and presented as atelectasis in 2 cases. On CEUS, 5 of the defects demonstrated, at the arterial and parenchymal phase, a lack of enhancement, and 14 showed an inhomogeneous (mixed) enhancement with wedge-shaped peripheral areas of no contrast enhancement. A second radiologic evaluation of the CT scans revealed PE in two patients and lesions suspicious for malignancy in two other patients. CONCLUSIONS: Despite the lack of definite confirmation of peripheral and central PE on CT, peripheral pleural consolidations with no or inhomogeneous enhancement on CEUS, in combination with the risk profile for a PE, are highly suggestive of EC. If there is still some doubt, histologic confirmation is important to confirm EC and exclude malignancy. Thus, CEUS may close a potential diagnostic gap of small peripheral PE on CT. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 45:575-579, 2017.

[Spontaneous remission of HCV infection after autologous stem cell transplantation in a 58-year-old man]. / Spontane Remission einer HCV-Infektion nach autologer Stammzelltransplantation bei einem 58-jährigen Patienten.

We report about a 58-year-old man with a chronic and treatment-naive hepatitis C virus (HCV) infection of genotype 1b, who had undergone autologous stem cell transplantation twice due to multiple myeloma. Subsequently, a high-level viremic reactivation of an occult hepatitis B virus (HBV) infection and also a reverse seroconversion was observed. Furthermore, a sustained spontaneous remission of HCV infection was seen. Antiviral therapy of HBV infection was initiated with tenofovir. Seven months after therapy initiation, the patient acquired an "anti-HBc-only" status. Antiviral therapy with tenofovir is still continued. The patient is in a good clinical condition.

Gemcitabine diphosphate choline is a major metabolite linked to the Kennedy pathway in pancreatic cancer models in vivo.

BACKGROUND: The modest benefits of gemcitabine (dFdC) therapy in patients with pancreatic ductal adenocarcinoma (PDAC) are well documented, with drug delivery and metabolic lability cited as important contributing factors. We have used a mouse model of PDAC: KRAS(G12D); p53(R172H); pdx-Cre (KPC) that recapitulates the human disease to study dFdC intra-tumoural metabolism. METHODS: LC-MS/MS and NMR were used to measure drug and physiological analytes. Cytotoxicity was assessed by the Sulphorhodamine B assay. RESULTS: In KPC tumour tissue, we identified a new, Kennedy pathway-linked dFdC metabolite (gemcitabine diphosphate choline (GdPC)) present at equimolar amounts to its precursor, the accepted active metabolite gemcitabine triphosphate (dFdCTP). Utilising additional subcutaneous PDAC tumour models, we demonstrated an inverse correlation between GdPC/dFdCTP ratios and cytidine triphosphate (CTP). In tumour homogenates in vitro, CTP inhibited GdPC formation from dFdCTP, indicating competition between CTP and dFdCTP for CTP:phosphocholine cytidylyltransferase (CCT). As the structure of GdPC precludes entry into cells, potential cytotoxicity was assessed by stimulating CCT activity using linoleate in KPC cells in vitro, leading to increased GdPC concentration and synergistic growth inhibition after dFdC addition. CONCLUSIONS: GdPC is an important element of the intra-tumoural dFdC metabolic pathway in vivo.

nab-Paclitaxel: novel clinical and experimental evidence in pancreatic cancer.

The past few decades have seen virtually no treatment advances for patients with metastatic pancreatic cancer. Clinical hallmark features of pancreatic ductal adenocarcinoma (PDA) include late symptom onset, invasive growth, early liver and lymph node metastasis, and resistance to available chemotherapies. nab-Paclitaxel (Abraxane®) is generated through high-pressure homogenization of human albumin and conventional paclitaxel resulting in non-covalently bound, water-soluble albumin-paclitaxel particles with an approximate diameter of 130 nm. Results from the recently completed Metastatic Pancreatic Adenocarcinoma Trial (MPACT) (phase III trial) showed a significant survival benefit for patients treated with nab-paclitaxel in combination with gemcitabine, and this treatment regimen is currently being implemented in national and international guidelines for PDA patients. Therefore, this regimen provides a much needed vantage point of attack for this recalcitrant tumor offering potential new hope for our patients. Mechanisms such as stromal depletion, selective intratumoral accumulation, synergism with gemcitabine metabolism and secreted protein acidic and rich in cysteine (SPARC) mediated anti-tumor activity have been suggested for nab-paclitaxel. This review discusses the clinical and experimental advances of nab-paclitaxel in pancreatic cancer.

[Incidental findings in abdominal ultrasound. Characteristics and clinical interpretation]. / Zufallsbefunde in der Abdomensonographie: Charakteristika und klinische Interpretation.

Abdominal ultrasound is a common diagnostic procedure in internal medicine. The correct interpretation of incidental findings can be difficult at times and often results in expensive and sometimes invasive follow-up examinations. Therefore, detailed knowledge of incidental findings on abdominal ultrasound is of utmost clinical and economical importance. Incidental findings are often benign, however, an accurate evaluation and correct diagnosis is crucial for the subsequent clinical management. To this end B-mode ultrasonography is complemented by color flow Doppler sonography and contrast-enhanced ultrasonography to add dynamic information on blood flow and vessel formation. This article presents frequent incidental findings of the major abdominal organs and vessels, and describes the sonographic and clinical management to find the correct diagnosis.

Claudin-4 as therapeutic target in cancer.

BACKGROUND: Intercellular junctional complexes such as adherens junctions and tight junctions are critical regulators of cellular polarity, paracellular permeability and metabolic and structural integrity of cellular networks. Abundant expression analysis data have yielded insights into the complex pattern of differentially expressed cell-adhesion proteins in epithelial cancers and provide a useful platform for functional, preclinical and clinical evaluation of novel targets. SCOPE OF REVIEW: This review will focus on the role of claudin-4, an integral constituent of tight junctions, in the pathophysiology of epithelial malignancies with particular focus pancreatic cancer, and its potential applicability for prognostic, diagnostic and therapeutic approaches. MAJOR CONCLUSIONS: Claudin-4 expression is widely dysregulated in epithelial malignancies and in a number of premalignant precursor lesions. Although the functional implications are only starting to unravel, claudin-4 seems to play an important role in tumour cell invasion and metastasis, and its dual role as receptor of Clostridium perfringens enterotoxin (CPE) opens exciting avenues for molecular targeted approaches. GENERAL SIGNIFICANCE: Claudin-4 constitutes a promising molecular marker for prognosis, diagnosis and therapy of epithelial malignancies.

Prospective evaluation of duodenogastroesophageal reflux in gastroesophageal reflux disease patients refractory to proton pump inhibitor therapy.

BACKGROUND: Duodenogastroesophageal reflux (DGER) is considered an independent risk factor for complicated reflux disease (gastroesophageal reflux disease; GERD). However, the role of DGER in GERD patients refractory to proton pump inhibitors (PPI) remains poorly understood. METHODS: 85 patients with clinical reflux symptoms and a history of ineffective response to PPIs were enrolled in the study. Patients with elevated reflux measurement (pH and/or Bilitec measurement; n = 47) received pantoprazole 80 mg for 8 weeks. Clinical outcome was defined as response (≤2 symptoms/week) or nonresponse (≥3 symptoms/week). RESULTS: Of the 47 patients with elevated reflux measurement, 30 were classified as responders and 17 as nonresponders. Treatment with pantoprazole resulted in a significant reduction of acidic reflux in both PPI responders and PPI nonresponders. In contrast, DGER was only significantly reduced in the PPI responder group (22.8 ± 22.8 vs. 6.6 ± 10.8%; p < 0.05) but not in the PPI nonresponder group (24.5 ± 18.6 vs. 22.2 ± 12.7%; p > 0.05). CONCLUSIONS: The presented study firstly describes that nonresponsiveness to PPI is associated with a limited effect of PPIs on reducing DGER. Thus, persistent DGER may play a key role in mediating reflux symptoms refractory to high-dose PPIs.

CUX1: target of Akt signalling and mediator of resistance to apoptosis in pancreatic cancer.

BACKGROUND AND AIMS: The transcription factor CUX1 is known as a regulator of cell differentiation and cell cycle progression. Previously, CUX1 was identified as a modulator of invasiveness in various cancers. Based on expression profiles suggesting a role for CUX1 in mediating chemoresistance, the aim of this study was to characterise the effect of CUX1 on apoptosis as well as its regulation by signalling pathways modulating drug resistance in pancreatic cancer. METHODS: The effect of CUX1 on TRAIL- (tumour necrosis factor-related apoptosis-inducing ligand) and drug-induced apoptosis was analysed using overexpression and knock-down strategies. Regulation of CUX1 by phosphatidylinositol-3-kinase (PI3K)/Akt signalling was examined at the mRNA and protein level. The effect of CUX1 knock-down by nanoparticle-complexed small interfering RNA (siRNA) in vivo was analysed in a murine xenograft model. Furthermore, CUX1 RNA and protein expression was evaluated in human pancreatic cancer and adjacent normal tissues. RESULTS: Knock-down of CUX1 resulted in significantly enhanced TRAIL- and drug-induced apoptosis, associated with increased PARP (poly ADP-ribose polymerase) cleavage and caspase activity. Vice versa, overexpression of CUX1 inhibited apoptosis. CUX1 expression was induced by activation of Akt/protein kinase B signalling, and decreased by PI3K inhibitors. The antiapoptotic effect of CUX1 was associated with upregulation of BCL2 and downregulation of tumour necrosis factor alpha. CUX1 was significantly overexpressed in pancreatic cancers, as analysed by in situ hybridisation and immunohistochemistry. In vivo, silencing of CUX1 by intratumourally administered polyethylenimine-complexed siRNA led to reduced tumour growth and increased apoptosis in pancreatic cancer xenografts. CONCLUSION: CUX1 was identified as an important mediator of tumour cell survival in pancreatic cancer in vitro and in vivo.

[Contrast enhanced sonographic patterns of hepatic candidiasis]. / Kontrastunterstützte sonografische Befunde bei hepatischer Candidiasis.

PURPOSE: B-mode ultrasound (US) of hepatic candidiasis (HC) shows an uncharacteristic pattern. The aim of this study is to display the pattern of HC by performing contrast-enhanced ultrasound (CEUS). PATIENTS AND METHODS: Between May 2006 and June 2008 HC was diagnosed in 12 patients (4 female, 8 male) by clinical and sonographic findings. The underlying diseases were acute leukemia (n = 10), aplastic anemia (n = 1), and testicular cancer (n = 1) in either the state of complete remission (n = 10) or relapse (n = 2). Due to neutropenic fever after chemotherapy all of the patients had received antifungal therapy. When HC was diagnosed all patients were afebrile and in a recovered hematological constitution. Additional diagnostic procedures were histological examination (n = 5), computed tomography (n = 8) and sonographic follow-up examinations (n = 12). All patients were examined with B-mode US and CEUS. RESULTS: In B-mode US the lesions were hypoechoic (n = 12), multiple (n = 10) and > 1 cm (n = 8) as well as > 2 cm (n = 4) in diameter. During CEUS no enhancement of contrast media in the centre of the lesions was seen in all cases during both phases. Additionally, in the arterial phase, the lesions showed no rim enhancement (n = 3) (type I), an isoechoic rim enhancement (n = 5) (type II), or a hyperechoic rim enhancement (n = 4) (type III). During sonographic follow-up a complete regression of the lesions (n = 9) or a stable disease (n = 2) was seen. One patient died due to a relapse. CONCLUSION: The CEUS pattern of HC is variable but characteristic. Therefore, CEUS should be performed in all patients with suspected HC.

Contrast-enhanced ultrasound pattern of splenic metastases - a retrospective study in 32 patients.

PURPOSE: To characterize the pattern of contrast-enhanced ultrasonography (CEUS) in splenic metastases compared to standard B-mode ultrasonography. MATERIALS AND METHODS: Between January 2004 and March 2009, about 50,000 abdominal ultrasound examinations were performed, and n = 279 (< 0.6 %) of focal splenic lesions were detected of which n = 32 (11.5 %) were highly suggestive for splenic metastases of various solid tumors. The number of lesions, size, echogenicity, rim appearance, presence of halo sign and necrosis were recorded via B-mode sonography. Contrast enhancement was determined in the arterial phase (5 - 30 sec) and parenchymal phase (3 - 5 min). B-mode sonography and CEUS were compared in terms of the visibility of splenic metastases. All data was evaluated retrospectively. RESULTS: On B-mode sonography lesions were solitary n = 18 (56 %), multiple n = 14 (44 %), < 2 cm n = 11 (34 %), > or = 2 cm n = 21 (66 %), hypoechoic n = 14 (44 %), isoechoic n = 12 (38 %) and hyperechoic n = 6 (19 %), with regular rim appearance n = 27 (84 %), and with irregular rim appearance n = 5 (16 %). During the arterial phase CEUS lesions were hypoechoic n = 21 (66 %), isoechoic n = 2 (6 %), hyperechoic n = 1 (3 %) and complex n = 8 (25 %). During the parenchymal phase lesions were hypoechoic n = 24 (75 %) and complex n = 8 (25 %). CEUS provided improved visualization of splenic metastases in n = 12 (38 %) cases. CONCLUSION: CEUS of splenic metastases is characterized by reduced contrast enhancement in both the arterial and the parenchymal phase in most cases. Moreover, CEUS improved the visualization of splenic metastases in about 40 % of cases in comparison to standard B-mode sonography.

Increased Duodeno-Gastro-Esophageal Reflux (DGER) in symptomatic GERD patients with a history of cholecystectomy.

BACKGROUND: Duodenal-Gastro-Esophageal Reflux (DGER) represents an independent risk factor for the development of complicated Gastro-esophageal-reflux-disease (GERD) and Barrett's esophagus. Clinical and epidemiological data suggest a potential association between cholecystectomy (CCE) and augmented bile reflux. METHODS: 132 patients (67 women, 65 men, median age 55) with typical symptoms of GERD were enrolled in the study and divided in cholecystectomized (CCE-group: n = 107) and non- cholecystectomized (nCCE-group: n = 25) patients. Standardized clinical work-up of patients included combined esophageal 24 h pH-measurement and Bilitec 2000 esophageal manometry and upper endoscopy. RESULTS: In the statistical analysis no differences between the cholecystectomized group (CCE-group, n = 25) and the patients without cholecystectomy (nCCE-group, n = 107) could be observed in quantity or quality of reflux symptoms. Furthermore, neither acid reflux nor severity of inflammation and frequency of Barrett's esophagus significantly differed between the nCCE and CCE-group. However, the percentage of patients with pathological DGER were significantly higher in the CCE-group as compared to the nCCE-group (76 vs. 55 %, p < 0.01). Moreover, the CCE-group revealed significant higher levels of pathological DGER compared to the nCCE-group (15.5 % +/- 14.1 vs. 8.6 % +/- 15.4; p < 0.05). CONCLUSION: To conclude, our data provide first evidence of elevated DGER after CCE in patients with typical clinical symptoms of GERD using the Bilitec device. Both the frequency and the extent of DGER was significantly increased in the CCE-group. Prospective studies are urgently needed to elucidate the impact of CCE on DGER in patients with clinical symptoms of a reflux disease.

Sperm-associated antigen 1 is expressed early in pancreatic tumorigenesis and promotes motility of cancer cells.

Sperm-associated antigen 1 (SPAG1) was recently identified in a rare form of infertility where anti-SPAG1 antibodies derived from the serum of an infertile woman were reported to cause sperm agglutination. Except for its expression and potential role in spermatogenesis, the function of SPAG1 is completely unknown. The unexpected finding of high levels of SPAG1 expression in pancreatic adenocarcinoma compared to normal pancreatic tissue in our previous cDNA array experiments prompted us to look in more detail at the expression and role of this gene in a panel of normal and malignant human tissues as well as in a larger series of pancreatic cancer specimens. We have generated an SPAG1-specific monoclonal antibody and showed high levels of SPAG1 protein in testis and in a large proportion of pancreatic ductal adenocarcinomas (PDAC). In the latter, SPAG1 expression was predominantly cytoplasmic and confined to malignant cells. Furthermore, the extent and intensity of SPAG1 expression was shown to be associated with stage and tumour nodal status, while analysis of precursor lesions, pancreatic intraepithelial neoplasias (PanINs), demonstrated its increased immunoreactivity with increasing PanIN grade, suggesting that SPAG1 is a novel marker of PDAC progression. Immunocytochemical analysis demonstrated colocalization of SPAG1 with microtubules, and their association was confirmed by co-immunoprecipitation; subsequent motility assays further substantiated a potential role of SPAG1 in cancer cell motility. Combined with the finding of its early expression in PDAC development, our data suggest that SPAG1 could contribute to the early spread and poor prognosis of pancreatic adenocarcinoma.

Echo-rich and echo-poor periportal cuffing: pole position for inflammatory bowel diseases.

PURPOSE: To determine the prevalence of echo-rich and echo-poor periportal cuffing in patients from a German tertiary referral hospital and correlate ultrasonographic findings with clinical data. MATERIALS AND METHODS: From April 2002 till April 2008 about 10 500 abdominal examinations were performed by a single physician in our interdisciplinary ultrasound unit. During this time, n = 100 patients (62 male/ 38 female) with periportal cuffing of the liver were detected qualifying for the retrospective study design. Echomorphology of periportal cuffing was evaluated and clinical diagnoses of the underlying diseases were clustered in four main groups: Liver diseases, haematological diseases, bowel diseases and others. Furthermore, liver function tests and body mass index were determined. RESULTS: The mean age of the patients was 57.06 years (SD +/- 19.47). Mean body-mass-index was 24.76 kg/m (2) (SD +/- 4.28). Periportal cuffing was echo-poor in n = 9 (9 %) and echo-rich in n = 91 (91 %). Echo-poor periportal cuffing was significantly more often associated with malignant diseases as compared to echo-rich periportal cuffing (78 vs. 36 %) (p < 0.025). Liver diseases (n = 33) were malign n = 10 (10 %), autoimmune n = 8 (8 %), infectious n = 8 (8 %) and cholestatic n = 7 (7 %). Bowel diseases (n = 34) originated from the upper gastrointestinal tract n = 7 (7 %), lower gastrointestinal tract n = 21 (21 %) and the pancreas n = 6 (6 %). Haematological disorders (n = 15, 15 %) were chronic myeloproliferative n = 2 (2 %), lymphoma n = 8 (8 %), leukemia n = 4 (4 %) and miscellaneous n = 1 (1 %). Other diseases accounted for 18 (18 %) of cases. Aspartat-aminotrasferase (AST) and alanin-aminotransferase (ALT) were elevated in 39 patients (40 %) and 34 patients (35 %), respectively. Total bilirubin was elevated in 35 patients (36 %). Alkaline phosphatase (AP) was detected above normal range in 49 patients (50 %) whereas g-glutamyl-transferase was elevated in 58 patients (59 %). CONCLUSION: Periportal cuffing of the liver is an extremely rare ultrasonographic phenomenon with a prevalence of approximately 0.95 % in our unit. Echo-rich periportal cuffing occurs more frequently than echo-poor periportal cuffing. The majority of echo-poor periportal cuffing is associated with malignant disorders, in particular haematological diseases, whereas echo-rich periportal cuffing is most frequently seen in inflammatory bowel disease patients.