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Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis

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A special issue of Pathogens (ISSN 2076-0817). This special issue belongs to the section "Parasitic Pathogens".

Deadline for manuscript submissions: closed (30 November 2022) | Viewed by 34105

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Dr. Beate Grüner

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Guest Editor

Head of Division of Clinical Infectious Diseases, Department of Internal Medicine III, University Hospital Ulm, Albert-Einstein-Allee 23, 89081 Ulm, Germany
Interests: clinical infectious diseases; infections of the immunocompromized host; HIV; parasitic diseases; alveolar and cystic echinococcosis; tuberculosis
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Dr. Thomas Romig

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Co-Guest Editor

Parasitology Unit 190p, Emil-Wolff-Str. 34, University of Hohenheim, 70599 Stuttgart, Germany
Interests: epidemiology of cestodes; alveolar and cystic echinococcosis; taeniosis; cysticercosis; wildlife parasitology
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Human alveolar echinococcosis (AE) remains a challenge for clinicians and public health systems in affected countries. Experimental, epidemiological, and clinical research has given us some insight into one of the world’s most lethal parasitoses, yet we still are far from fully understanding this disease.

Therefore, we would like to invite you, as experts in this field, to add a piece to the puzzle and share your research results on Echinococcus multilocularis. Compiling and integrating our research will improve our ability to prevent, diagnose, and treat AE und hopefully reduce the burden of disease.

This Special Edition of Pathogens aims to share research with clinical implications from all AE-affected countries. More precisely, the focus lies on the following research questions:

AE or not? How to make first diagnosis of AE easier.
- What are simple and reliable biomarkers to identify AE?
- How can new imaging techniques help to establish the diagnosis?
- When is a biopsy required and how can the examination be conducted?
- Histology, Immunohistology or Nucleic acid detection—quo vadis?

Diagnosis prompts treatment—or not? How to strike the right balance between action and cautious restraint.
- How can we separate active from inactive disease?
- How can we evaluate treatment response?
- Is the PNM classification a reliable prognostic tool?
- When is it appropriate to delay or pause treatment?

Therapy in AE: crossroads, dead-end or roadworks? How to choose a treatment option and overcome current limitations.
- If benzimidazoles fail, what treatment options are left?
- How does treatment differ in vulnerable groups, e.g., the immunocompromised?
- When is conservative treatment and when is surgery the best choice?
- What are the strengths and limitations of different surgical and interventional techniques?
- How can resection margin and perioperative treatment with benzimidazoles affect the long-term outcome?
- Liver transplantation as ultima ratio—quo vadis?

From bench to bedside: How clinicians learn from basic research.
- How does the host’s immune system control AE?
- Which host factors contribute to a stable disease and which do not?
- Is there a difference in the pathogenicity/infectivity of different genetic variants?
- What are underlying mechanisms of benzimidazole intolerance or suspected resistance?

Looking at the greater picture: How can we reduce the burden of disease?
- Understanding the One Health dynamics of AE: what are risk factors for transmission?
- What is the geographical distribution and relative frequency of different genotypes? Are there differences in host predilection?
- Mass screening, baiting or hygiene promotion—what are successful and cost-effective public health strategies?

Dr. Beate Grüner
Guest Editor
Dr. Thomas Romig
Co-Guest Editor

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Keywords

Echinococcus multilocularis
human alveolar echinococcosis
AE
diagnosis
treatment and clinical management of AE
immunology
genotypes
epidemiology
public health

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15 pages, 2351 KiB

Open AccessArticle

¹⁸FDG-PET/CT-Scans and Biomarker Levels Predicting Clinical Outcome in Patients with Alveolar Echinococcosis—A Single-Center Cohort Study with 179 Patients

by Lynn Peters, Wanjie Jiang, Nina Eberhardt, Jürgen Benjamin Hagemann, Beate Grüner and Dennis Tappe

Pathogens 2023, 12(8), 1041; https://doi.org/10.3390/pathogens12081041 - 14 Aug 2023

Cited by 1 | Viewed by 1377

Abstract

Background: Alveolar echinococcosis (AE) is a severe larval tapeworm infection with a variable clinical course of the disease. Reliable imaging techniques and biomarkers are needed to predict the course of the disease. Methods: 179 AE patients that received PET/CT scans between 2008 and 2012 were retrospectively included. From stored blood samples taken on the day of the scan, levels of IgE, parasite-specific serology, amyloid A, C-reactive protein, soluble interleukin 2 receptor, cytokeratin fragments, eosinophilic cell count, and eosinophil cationic protein were measured. Additionally, the current clinical outcome (cured, stable, or progressive disease) after a median duration of 8 years after baseline examination was assessed. Ultimately, an ordinal logistic regression was conducted to evaluate which imaging parameters and biomarkers independently influence the clinical outcome. Results: In general, patients in need of medical treatment or with progressive disease, advanced PNM stages, and positive PET/CT scans exhibited higher levels of the respective biomarkers. However, only the parasite-specific serological markers and total IgE levels differed significantly between clinical groups, WHO PNM stages, and the results of the PET/CT scan. In the multivariate analysis, PET/CT results were a strong predictor of the clinical outcome (OR 8.908, 95%CI 3.019–26.285; p < 0.001), and age at baseline was a moderate predictor (OR 1.031, 95%CI 1.003–1.060; p = 0.029). Conclusions: The PET/CT scan is, preferably in combination with parasite-specific serology and IgE levels, a valuable tool in the clinical management of AE and is able to predict the course of the disease. Full article

(This article belongs to the Special Issue Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis)

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13 pages, 2995 KiB

Open AccessArticle

Robotic Liver Surgery for Alveolar Echinococcosis: A Single-Centre Experience

by Kira C. Steinkraus, Laila Jötten, Benno Traub, Marin Zaimi, Maximilian Denzinger, Christoph W. Michalski, Marko Kornmann and Felix J. Hüttner

Pathogens 2022, 11(11), 1276; https://doi.org/10.3390/pathogens11111276 - 31 Oct 2022

Viewed by 1668

Abstract

Alveolar echinococcosis (AE) is a rare disease caused by Echinococcosis multilocularis, which usually requires multidisciplinary management including surgery as the only curative approach. In recent years, minimally invasive strategies have been increasingly adopted for liver surgery. In particular, robotic surgery enables surgeons to perform even complex liver resections using a minimally invasive approach. However, there are only a few reports on robotic liver surgery for AE. Consecutive patients undergoing robotic liver surgery for AE were analysed based on the prospective database of the Interdisciplinary Robotic Centre of Ulm University Hospital. Between January 2021 and August 2022, a total of 16 patients with AE underwent robotic hepatectomy at our institution. Median age was 55.5 years (23–73), median body mass index (BMI) was 25.8 kg/m² (20.2–36.8) and 12 patients (75%) were female. Anatomic resections were performed in 14 patients (87.5%), of which 4 patients (25%) underwent major hepatectomies (i.e., resection of >3 segments) including two right hemihepatectomies, one left hemihepatectomy and one extended right hemihepatectomy performed as associating liver partition with portal vein ligation staged (ALPPS) hepatectomy. There was no 90-day mortality, no postoperative bile leakage and no posthepatectomy haemorrhage. One patient developed posthepatectomy liver failure grade B after extended right hemihepatectomy using an ALPPS approach. One patient had to be converted to open surgery and developed an organ-space surgical site infection, for which he was re-admitted and underwent intravenous antibiotic therapy. Median length of postoperative hospital stay was 7 days (4–30). To our knowledge, this is the largest series of robotic liver surgeries for AE. The robotic approach seems safe with promising short-term outcomes in this selected cohort for both minor as well as major resections. Full article

(This article belongs to the Special Issue Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis)

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11 pages, 2117 KiB

Open AccessArticle

Initial Risk Assessment in Patients with Alveolar Echinococcosis—Results from a Retrospective Cohort Study

by Lynn Peters, Sanne Burkert, Jürgen Benjamin Hagemann, Rasmus Albes, Jonas Klemptner, Jessica Birkle, Elias Schwaibold, Sofia Siefermann and Beate Grüner

Pathogens 2022, 11(5), 557; https://doi.org/10.3390/pathogens11050557 - 9 May 2022

Cited by 1 | Viewed by 2673

Abstract

Background: Alveolar echinococcosis (AE) is a potentially lethal parasitosis with a broad spectrum of disease dynamics in affected patients. To guide clinical management, we assessed initial prognostic factors for both progressive and controlled AE based on initial staging. Methods: A retrospective cohort study was conducted, examining 279 patients assigned to different clinical groups: cured, stable with and without the need for benzimidazole treatment, and progressive disease. Univariate analysis compared demographic and clinical variables. Significant variables were subsequently entered into two separate logistic regression models for progressive and controlled disease. Results: Based on the multivariate analysis, a large AE lesion (OR = 1.02 per millimetre in size; 95%CI 1.004–1.029), PNM staging (OR = 2.86; 95%CI 1.384–5.911) and especially the involvement of neighbouring organs (OR = 3.70; 95%CI 1.173–11.653) remained significant risk factors for progressive disease. A negative Em2+ IgG (OR = 0.25; 95%CI 0.072–0.835) and a small AE lesion (OR = 0.97; 95%CI 0.949–0.996) were significant protective factors. Conclusions: Patients with large lesions and advanced stages should be monitored closely and most likely require long-term treatment with benzimidazoles if curative resection is not feasible. Patients with small lesions and negative Em2+ IgG seem able to control the disease to a certain extent and a less strict treatment regimen might suffice. Full article

(This article belongs to the Special Issue Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis)

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12 pages, 612 KiB

Open AccessArticle

Characteristics and Clinical Course of Alveolar Echinococcosis in Patients with Immunosuppression-Associated Conditions: A Retrospective Cohort Study

by Ansgar Deibel, Cordula Meyer zu Schwabedissen, Lars Husmann, Felix Grimm, Peter Deplazes, Cäcilia S. Reiner and Beat Müllhaupt

Pathogens 2022, 11(4), 441; https://doi.org/10.3390/pathogens11040441 - 6 Apr 2022

Cited by 4 | Viewed by 1749

Abstract

Objectives: Since the change in the millennium, an increase in cases of alveolar echinococcosis (AE) has been observed in endemic European countries. Previous studies indicate that a significant proportion of the new AE cases have an immunosuppression-associated condition (IAC). The aim of the current study was to determine how IACs impact the number of new AE diagnoses per year and the characteristics of AE at diagnosis and its clinical course at our center. Methods: Retrospective analysis of 189 patients with AE diagnosed between 2000 and 2021 and participating in the Zurich Echinococcosis Cohort Study (ZECS) included clinical characteristics of AE at diagnosis and report of an IAC, as well as the clinical course during follow-up. Results: Of 189 patients participating in this study, 38 had an IAC reported at, or shortly after, AE diagnosis. Over time, there was a steeper increase in the number of newly diagnosed AE patients without an IAC than the number of patients with IAC. Patients with an IAC were older at diagnosis, more frequently had an incidental finding of AE, smaller mean lesion size, and negative Em18 serology. All but two showed favorable outcomes on the last follow-up. Conclusion: IACs have little impact on the increase in new AE cases, as well as on the extent of the disease at diagnosis and clinical course. Full article

(This article belongs to the Special Issue Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis)

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12 pages, 6493 KiB

Open AccessArticle

18F-FDG-PET/MR in Alveolar Echinococcosis: Multiparametric Imaging in a Real-World Setting

by Nina Eberhardt, Lynn Peters, Silke Kapp-Schwoerer, Meinrad Beer, Ambros J. Beer, Beate Grüner and Wolfgang M. Thaiss

Pathogens 2022, 11(3), 348; https://doi.org/10.3390/pathogens11030348 - 12 Mar 2022

Cited by 5 | Viewed by 2248

Abstract

Recent improvements in alveolar echinococcosis (AE) therapy can provide long-term disease control, and even allow structured treatment interruption in selected cases. Imaging has a pivotal role in monitoring disease activity, with 18-fluoro-deoxyglucose positron emission and computed tomography (18F-FDG-PET/CT) in particular having proven beneficial for assessing disease activity. Repetitive regular examinations to monitor therapy response, however, can lead to substantial radiation burden. Therefore, by combining metabolic information and excellent tissue contrast in magnetic resonance imaging (MRI), PET/MR appears ideally suited for this task. Here, we retrospectively analyzed 51 AE patients that underwent 18F-FDG-PET/MR. Patients had a ‘confirmed/probable’ diagnosis in 22/29 cases according to the WHO classification. FDG uptake, diffusion restriction, and MRI morphology were evaluated. We found significant differences in FDG uptake between responders to benzimidazole therapy and progressive manifestations (SUVavg 2.7 ± 1.3 vs. 5.4 ± 2.2, p < 0.001) as well as between Kodama Types 1 and 3 (F = 9.9, p < 0.003). No significant differences were detected for ADC values or MRI morphology concerning response and no correlations were present between FDG uptake and ADC values. The mean radiation dose was 5.9–6.5 mSv. We conclude that the combination of metabolic information and MRI morphology at a low radiation dose proposes PET/MR as a suitable imaging modality for AE assessment. Longitudinal studies are needed to define the role of this imaging modality. Full article

(This article belongs to the Special Issue Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis)

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11 pages, 221 KiB

Open AccessArticle

Salvage Therapy for Alveolar Echinococcosis—A Case Series

by Sanne Burkert, Lynn Peters, Johannes Bloehdorn and Beate Grüner

Pathogens 2022, 11(3), 333; https://doi.org/10.3390/pathogens11030333 - 9 Mar 2022

Cited by 6 | Viewed by 2123

Abstract

Benzimidazoles are the only approved drugs for the treatment of inoperable human alveolar echinococcosis but may be limited due to intolerance or, rarely, ineffectiveness. A medical second-line or salvage therapy is not available, though it is urgently needed. We report long-term follow-up data from 14 patients who underwent salvage therapy with repurposed drugs with cumulatively 53.25 patient-years. Treatment response was evaluated by both clinical outcome and image studies, preferably PET/CT. Eleven patients received amphotericin B, and 70% of evaluable cases showed some positive treatment response, but side effects often limited therapy. Five patients received nitazoxanide, of which two showed clear progression but one achieved a lasting stable disease. One patient was treated with mefloquine combination therapy in advanced disease, and overall, a positive treatment response could not be assessed. Furthermore, we report on one patient receiving pembrolizumab for a concomitant malignancy, which did not result in a reduction of echinococcal manifestation. In summary, current options of salvage therapy can sometimes induce persistent disease control, although with potentially significant side effects and high treatment costs, and mortality remains high. No clear recommendation for a salvage therapy can be given; treatment remains highly experimental, and non-pharmaceutical interventions have to be considered. Full article

(This article belongs to the Special Issue Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis)

12 pages, 1458 KiB

Open AccessArticle

Imaging Aspects of Hepatic Alveolar Echinococcosis: Retrospective Findings of a Surgical Center in Turkey

by Mecit Kantarci, Sonay Aydin, Suat Eren, Hayri Ogul and Okan Akhan

Pathogens 2022, 11(2), 276; https://doi.org/10.3390/pathogens11020276 - 20 Feb 2022

Cited by 6 | Viewed by 2222

Abstract

Radiologists should be aware of the findings of alveolar echinococcosis (AE) due to the diagnostic and management value of imaging. We are attempting to define the most common diagnostic imaging findings of liver AE, along with the prevalence and distribution of those findings. The patients’ US, CT, and MRI images were reviewed retrospectively. CT images were acquired with and without the administration of contrast medium. The MRI protocol includes T2-weighted images (WI), diffusion (WI), apparent diffusion coefficient (ADC) maps, and pre- and post-contrast T1WIs. The current study included 61 patients. The mean age of the population was 58.2 ± 9.6 years According to Kratzer’s categorization (US), 139 lesions (73.1%) were categorized as hailstorm. According to Graeter’s classification (CT), 139 (73.1%) lesions were type 1-diffuse infiltrating. The most frequent types were Kodama type 2 and 3 lesions (MRI) (42.6% and 48.7%, accordingly). P2N0M0 was the most frequent subtype. The current study defines the major, characteristic imaging findings of liver AE using US, CT, and MRI. Since US, CT, and MRI have all been utilized to diagnose AE, we believe that a multi-modality classification system is needed. The study’s findings may aid radiologists in accurately and timely diagnosing liver AE. Full article

(This article belongs to the Special Issue Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis)

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9 pages, 531 KiB

Open AccessArticle

Evaluation of Eosinophilic Cationic Protein as a Marker of Alveolar and Cystic Echinococcosis

by Julian Frederic Hotz, Klaus Kaczirek, Stefan Stremitzer, Fredrik Waneck, Herbert Auer, Thomas Perkmann, Manuel Kussmann, Philipp Karl Bauer, Rui-Yang Chen, Richard Kriz, Heinz Burgmann, Michael Ramharter and Heimo Lagler

Pathogens 2022, 11(2), 261; https://doi.org/10.3390/pathogens11020261 - 18 Feb 2022

Cited by 1 | Viewed by 2090

Abstract

Echinococcosis is a neglected zoonotic disease and a worldwide public health problem caused by infection with the larval stages of taeniid cestodes of the genus Echinococcus. In vitro studies have demonstrated a protoscolecidal effect of eosinophilic cationic protein (ECP), a granule protein of eosinophilic granulocytes, against E. granulosus. Therefore, the main objective of this study was to evaluate ECP as a biomarker in the treatment of alveolar echinococcosis (AE) and cystic echinococcosis (CE). Data were collected retrospectively from the Vienna Echinococcosis Cohort over 7 years until December 2020. Altogether, 32 patients (16 AE and 16 CE) were included. In the selected patients, serum ECP values were compared before and after the beginning of an operative and/or benzimidazole (BMZ) therapy. Mean ECP serum levels before intervention were significantly (p < 0.05) elevated at 34.0 ± 22.9 μg/L in AE patients and at 38.6 ± 19.9 μg/L in CE patients compared to the control group. After the intervention, mean ECP levels decreased significantly (p < 0.05) to 20.4 ± 14.6 μg/L in AE patients and to 22.4 ± 8.3 μg/L in CE patients. Furthermore, ECP showed a significant (p < 0.05) correlation of k = 0.56 with PET–CTI. Based on the significant decrease after operative and/or BMZ treatment and the correlation with clinical markers such as PET–CTI, it is recommended to investigate ECP more intensively as a marker of AE and CE in prospective studies with larger cohorts. Full article

(This article belongs to the Special Issue Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis)

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13 pages, 2108 KiB

Open AccessEditor’s ChoiceArticle

Evaluation of Serological Markers in Alveolar Echinococcosis Emphasizing the Correlation of PET-CTI Tracer Uptake with RecEm18 and Echinococcus-Specific IgG

by Julian Frederic Hotz, Lynn Peters, Silke Kapp-Schwörer, Frauke Theis, Nina Eberhardt, Andreas Essig, Beate Grüner and Jürgen Benjamin Hagemann

Pathogens 2022, 11(2), 239; https://doi.org/10.3390/pathogens11020239 - 12 Feb 2022

Cited by 11 | Viewed by 2527

Abstract

Human alveolar echinococcosis (AE), which is caused by the cestode Echinococcus (E.) multilocularis, is an epidemiologically relevant issue in modern medicine and still poses a diagnostic and therapeutic challenge. Since diagnosis mainly relies on imaging procedures and serological testing, we retrospectively and comparatively analyzed the performance of an Echinococcus IgG screening ELISA, whole serum IgE, and two specific confirmatory ELISA platforms using the defined E. multilocularis antigens Em2-Em18 (Em2+) and recombinant Em18 (recEm18). With special emphasis on the clinical usefulness of recEm18, we correlated the laboratory results with clinical characteristics and imaging findings in a large and well-characterized cohort of N = 124 AE patients, who were followed over several years after either surgical plus subsequent pharmacological treatment or pharmacotherapy alone. All patients had routinely received PET-CTI every two years. Our data reveal strong correlations for both Echinococcus IgG and recEm18 with tracer uptake in PET-CTI and parasitic lesion size and number, suggesting additional clinical usefulness of recEm18 for certain constellations only, while IgG and Em2+ still appear reasonable and sensitive screening methods for initial diagnosis of AE. With this study, we aim to contribute to further optimizing medical care of AE patients. For instance, it might be reasonable to consider the replacement of some PET-CTI follow-ups by imaging procedures with less radiation exposure or serological means alone. Further studies that clarify the correlation of serological markers with ultrasound criteria might be particularly useful, and further retrospective as well as prospective investigations are justified in this context. Full article

(This article belongs to the Special Issue Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis)

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13 pages, 2465 KiB

Open AccessArticle

Alveolar Echinococcosis—A Challenging Task for the Hepatobiliary Surgeon

by Jens Strohaeker, Mihaly Sulyok, Alfred Koenigsrainer and Silvio Nadalin

Pathogens 2022, 11(1), 40; https://doi.org/10.3390/pathogens11010040 - 31 Dec 2021

Cited by 3 | Viewed by 1864

Abstract

(1) Background: Alveolar echinococcosis (AE) is an ultimately fatal disease, whose only curative treatment is surgery. Due to its late presentation extended liver resections are often necessary. The true benefit of extensive surgery has yet to be established; (2) Methods: We present a single center experience of 33 cases of Echinococcus multilocularis that have been treated at a high-volume hepatobiliary surgery center between 2004 and 2021. (3) Results: Of the 33 patients 24 patients underwent major liver resection (73%). In addition to the liver resection patients frequently underwent complex extrahepatic procedures such as lymphadenectomy (n = 21, 61%), vascular resections and reconstructions (n = 9, 27%) or resections and reconstruction of the extrahepatic bile duct (n = 11, 33%). Seven patients suffered from ≥ grade III complications (21%). Complete resection was achieved in 17 patients. Fourteen patients had R1 resections and two had macroscopic parasitic remnant (R2). Progressive disease was reported in three patients (The two R2 patients and one R1 resected patient). At a median follow-up of 54 months no mortality has occurred in our cohort; (4) Conclusions: Liver resection remains the gold standard for AE. Even in extensive disease the combination of complex resection and perioperative benzimidazoles can achieve favorable long-term outcomes. Full article

(This article belongs to the Special Issue Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis)

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20 pages, 4477 KiB

Open AccessArticle

Maca against Echinococcosis?—A Reverse Approach from Patient to In Vitro Testing

by Tanja Karpstein, Sheena Chaudhry, Solange Bresson-Hadni, Michael Hayoz, Ghalia Boubaker, Andrew Hemphill, Reto Rufener, Marc Kaethner, Isabelle Schindler, Yolanda Aebi, Antonio Sa Cunha, Carlo R. Largiadèr and Britta Lundström-Stadelmann

Pathogens 2021, 10(10), 1335; https://doi.org/10.3390/pathogens10101335 - 15 Oct 2021

Cited by 3 | Viewed by 2334

Abstract

Drug-based treatment of alveolar echinococcosis (AE) with benzimidazoles is in most cases non-curative, thus has to be taken lifelong. Here, we report on a 56-year-old male AE patient who received standard benzimidazole treatment and biliary plastic stents, and additionally self-medicated himself with the Peruvian plant extract Maca (Lepidium meyenii). After 42 months, viable parasite tissue had disappeared. Based on this striking observation, the anti-echinococcal activity of Maca was investigated in vitro and in mice experimentally infected with Echinococcus multilocularis metacestodes. Albendazole (ABZ)-treated mice and mice treated with an ABZ+Maca combination exhibited a significantly reduced parasite burden compared to untreated or Maca-treated mice. As shown by a newly established UHPLC-MS/MS-based measurement of ABZ-metabolites, the presence of Maca during the treatment did not alter ABZ plasma levels. In vitro assays corroborated these findings, as exposure to Maca had no notable effect on E. multilocularis metacestodes, and in cultures of germinal layer cells, possibly unspecific, cytotoxic effects of Maca were observed. However, in the combined treatments, Maca inhibited the activity of ABZ in vitro. While Maca had no direct anti-parasitic activity, it induced in vitro proliferation of murine spleen cells, suggesting that immunomodulatory properties could have contributed to the curative effect seen in the patient. Full article

(This article belongs to the Special Issue Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis)

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8 pages, 1999 KiB

Open AccessCase Report

Alveolar Echinococcosis in a Patient with Presumed Autoimmune Hepatitis and Primary Sclerosing Cholangitis: An Unexpected Finding after Liver Transplantation

by Florian Fronhoffs, Leona Dold, Marijo Parčina, Arne Schneidewind, Maria Willis, Thomas F. E. Barth, Tobias J. Weismüller, Taotao Zhou, Philipp Lutz, Julian A. Luetkens, Peter Gerlach, Steffen Manekeller, Jörg C. Kalff, Tim O. Vilz, Christian P. Strassburg and Glen Kristiansen

Pathogens 2023, 12(1), 73; https://doi.org/10.3390/pathogens12010073 - 3 Jan 2023

Cited by 2 | Viewed by 2179

Abstract

Primary sclerosing cholangitis is an important reason for liver transplantation. Hepatic alveolar echinococcosis (AE) is caused by Echinococcus multilocularis and presents characteristic calcified conglomerates detected by ultrasound or computed tomography scan of the liver. Symptoms of AE only occur after a long period of infection when cholestasis or cholangitis becomes apparent. Here, we report on a patient with presumed autoimmune hepatitis and primary sclerosing cholangitis. After liver transplantation, alveolar echinococcosis was diagnosed in the liver explant. Full article

(This article belongs to the Special Issue Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis)

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8 pages, 2598 KiB

Open AccessTutorial

Morphological Characteristics of Alveolar and Cystic Echinococcosis Lesions in Human Liver and Bone

by Thomas F. E. Barth and Adriano Casulli

Pathogens 2021, 10(10), 1326; https://doi.org/10.3390/pathogens10101326 - 14 Oct 2021

Cited by 13 | Viewed by 6987

Abstract

Among echinococcoses diseases of human interest, two have a global public health impact: cystic and alveolar echinococcosis caused by Echinococcus granulosus sensu lato and Echinococcus multilocularis, respectively. Cystic and alveolar echinococcosis are neglected infectious diseases epidemiologically and are clinically vastly different with distinct microscopic features. Because of the rareness of these zoonotic diseases, pathologists have limited diagnostic experience in the analysis of the lesions caused by Echinococcus tapeworms. Here, we describe the main microscopic features to be considered to characterize these lesions: laminated layer, central necrosis, growth pattern, and delineation from adjacent tissue. Moreover, immunohistology using monoclonal antibodies is of great diagnostic help in reaching a definitive diagnosis by identifying the laminated body and small particles of E. multilocularis (spems) and small particles of E. granulosus (spegs). Full article

(This article belongs to the Special Issue Alveolar Echinococcosis: Joining Hands to Tackle a Lethal Parasitosis)

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