Search

We report the case of idiopathic non-cirrhotic portal hypertension associated with systemic lupus erythematosus in a 43-year-old woman who suffered from breast cancer. We review this rare condition, as well as its diagnostic and therapeutic approaches., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Suárez-Díaz et al.)

Background: α/β-hydrolase domain-containing protein 5 (ABHD5) plays an important role in the triacylglycerols (TAG) hydrolysis. Indeed, ABHD5 is the co-activator of adipose triglyceride lipase (ATGL), that catalyses the initial step of TAG hydrolysis. Mutations in ABHD5 gene are associated with the onset of Chanarin-Dorfman syndrome (CDS), a rare autosomal recessive lipid storage disorder, characterized by non-bullous congenital ichthyosiform erythroderma (NCIE), hepatomegaly and liver steatosis. Case presentation: We describe here a 5-years-old Brazilian child who presented with NCIE at birth and diffuse micro and macro-vesicular steatosis on liver biopsy since she was 2 years old. Molecular analysis of coding sequence and putative 5′ regulatory region of ABHD5 gene was performed. A homozygous novel deletion, affecting the promoter region and the exon 1, was identified, confirming the suspected diagnosis of CDS for this patient. RT-PCR analysis showed that the genomic rearrangement completely abolished the ABHD5 gene expression in the patient, while only a partial loss of expression was detected in her parents. This is the first report describing the identification of a large deletion encompassing the promoter region of ABHD5 gene. The total loss of ABHD5 expression may explain the early onset of CDS and the severe liver involvement. After molecular diagnosis, the patient started a special diet, poor in fatty acids with medium chain triglycerides (MCT), and showed hepatic and dermatologic improvement in spite of severe molecular defect. Conclusions: This case report extends the spectrum of disease-causing ABHD5 mutations in CDS providing evidence for a novel pathogenic mechanism for this rare disorder. Moreover, our preliminary data show that early diagnosis and prompt treatment of neutral lipid accumulation might be useful for CD patients. [ABSTRACT FROM AUTHOR]

Tick-borne encephalitis (TBE) usually has a biphasic course which begins with unspecific febrile illness, followed by central nervous system involvement. Because TBE is not yet suspected during the initial phase, knowledge of early TBE pathogenesis is incomplete. Herein we evaluated laboratory and immune findings in the initial and second (meningoencephalitic) phase of TBE in 88 well-defined adult patients. Comparison of nine laboratory blood parameters in both phases of TBE revealed that laboratory abnormalities, consisting of low leukocyte and platelet counts and increased liver enzymes levels, were predominately associated with the initial phase of TBE and resolved thereafter. Assessment of 29 immune mediators in serum during the initial phase, and in serum and cerebrospinal fluid (CSF) during the second phase of TBE revealed highly distinct clustering patterns among the three groups. In the initial phase of TBE, the primary finding in serum was a rather heterogeneous immune response involving innate (CXCL11), B cell (CXCL13, BAFF), and T cell mediators (IL-27 and IL-4). During the second phase of TBE, growth factors associated with angiogenesis (GRO-α and VEGF-A) were the predominant characteristic in serum, whereas innate and Th1 mediators were the defining feature of immune responses in CSF. These findings imply that distinct immune processes play a role in the pathophysiology of different phases of TBE and in different compartments.

Clinical complications of cystic fibrosis (CF) include a variety of gastrointestinal (GI) and hepatobiliary manifestations. Recent years have witnessed several advances in the understanding and management of these complications, in addition to opportunities for therapeutic innovations. Herein we review the current understanding of these disorders and also discuss the management of the GI and hepatobiliary complications experienced by persons with CF., Competing Interests: Disclosure C. Velez – Cystic Fibrosis Foundation (CFF) funding for research in the gastrointestinal manifestations of cystic fibrosis, CFF DIGEST faculty award, CFF Data Safety Monitoring Board. S.D. Freedman – CFF funding for research in the gastrointestinal manifestations of cystic fibrosis, CFF DIGEST faculty. D.N. Assis – Prior CFF DIGEST faculty award, CFF research funding., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Abstract: Background: This report examines the patterns of presentation, prognostic factors and survival rate of all patients with gallbladder cancer (GBC) evaluated at our tertiary academic hospital over an 11-year period. Methods: A retrospective review of a prospectively collected database of all patients with GBC presenting between January 1998 and December 2008 was performed. Results: 102 GBC-patients were included: 69 women and 33 men (median age: 65,5 years). Forty-five patients presented with incidental gallbladder cancer (IGC) and 57 with nonincidental cancer (NIGC). Curative surgery rate was 84.4% for IGC and 29.8% for NIGC (p < 0.001). Five-year actuarial survival rate was 63.2% for patients with curative intent surgery and 0% for patients with palliative approach. Patients with IGC had a longer survival rate compared to patients with NIGC (median: 25.8 vs. 4.4 months, p < 0.0001). For patients with radical resection (42 patients), there was no difference between IGC and NIGC. The incidence of liver involvement was respectively 0%, 20.8%, 58.3%, 100% for pT1, pT2, pT3 and pT4 tumors. Univariate analysis showed that survival rate was significantly affected by perineural invasion, T, N and M-stage, R0 resection, liver involvement, CA-19.9. In multivariate analysis, liver involvement was the only independent factor. Conclusions: Majority of patients with a potentially curable disease had IGC. Almost 80% of patients with NIGC presented with unresectable disease. For patients who underwent resection with curative intent, actuarial 5-year survival was 63.2%. Liver involvement was the only independent prognostic factor. All patients with IGC and a pT2 or more advanced T stage should undergo a second radical resection. [Copyright &y& Elsevier]

Background: Organ dysfunction, especially liver injury, caused by dengue virus (DENV) infection has been associated with fatal cases in dengue patients around the world. However, the pathophysiological mechanisms of liver involvement in dengue remain unclear. There is accumulating evidence that miRNAs are playing an important role in regulating viral pathogenesis, and it can help in diagnostic and anti-viral therapies development., Methods: We collected liver tissues of DENV-infected for small RNA sequencing to identify significantly different express miRNAs during dengue virus infection, and the identified target genes of these miRNAs were annotated by biological function and pathway enrichment., Results: 31 significantly altered miRNAs were identified, including 16 up-regulated and 15 down-regulated miRNAs. By performing a series of miRNA prediction and signaling pathway enrichment analyses, the down-regulated miRNAs of mmu-miR-484, mmu-miR-1247-5p and mmu-miR-6538 were identified to be the crucial miRNAs. Further analysis revealed that the inflammation and immune responses involving Hippo, PI3K-Akt, MAPK, Wnt, mTOR, TGF-beta, Tight junction, and Platelet activation were modulated collectively by these three key miRNAs during DENV infection. These pathways are considered to be closely associated with the pathogenic mechanism and treatment strategy of dengue patients., Conclusion: The miRNAs identified by sequencing, especially miR-484 may be the potential therapeutic targets for liver involvement in dengue patients which involves the regulation of vascular permeability and expression of inflammatory cytokines., Competing Interests: Declaration of competing interest The authors declare that they have no conflicts of interest., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Background: Dengue and thalassaemia are prevalent in tropical regions. Thalassaemia patients with dengue present with atypical clinical manifestations and may experience more severe disease and complications. Aims: To investigate the clinical manifestations and outcome in thalassaemia patients with dengue. Methods: Medical records of thalassaemia patients aged from birth to 18 years with serologically-confirmed dengue who were admitted to Chiang Mai University Hospital, Thailand from January 2005 to December 2010 were reviewed retrospectively. Clinical presentation, laboratory results and outcome were analysed. Results: Twenty patients were included and their mean (SD) age was 13·6 (3·1) years. Patients were as follows: haemoglobin H (HbH)/Hb Constant Spring disease 7, HbH disease 3, HbH disease/HbE trait one, beta-thalassaemia/HbE disease 7, beta-thalassaemia/HbS disease one and unspecified thalassaemia one. Four patients had primary and the others secondary dengue. Five patients (25%) had severe dengue, three with severe liver involvement, one with a massive upper gastro-intestinal haemorrhage and one with acute interstitial nephritis, raised creatinine and seizures. Nineteen patients required red cells transfusions. All made a full recovery. Almost all patients presented with haemoglobin lower than baseline. While mean (SD) haemoglobin at baseline was 8·2 (1·6) g/dl, mean (SD) haemoglobin on admission was 6·4 (1·6) g/dl. Median maximum AST and ALT levels were 359 (range 42-5344) and 81 (12-1846) units/L, respectively. Conclusions: Thalassaemia patients with dengue present with anaemia rather than haemoconcentration. Most patients have markedly increased AST levels in relation to ALT, and severe dengue, especially severe liver involvement, is common. In patients with thalassaemia, awareness of the atypical manifestations should aid early recognition of dengue. [ABSTRACT FROM AUTHOR]

Purpose: Liver involvement in syphilis has been studied in cohorts of human immunodeficiency virus (HIV)-negative individuals despite the scarcity of data on such HIV-infected patients. Th aim of this study was to assess hepatic involvement of HIV-infected patients diagnosed with syphilis. Methods: Patients with syphilis and liver involvement, including all stages of syphilis, were systematically identified in our HIV cohort between 2004 and 2008. Results: Of the 1,599 HIV-infected patients identified during the study period, 100 were diagnosed with acute syphilis, all of whom were male. Of these 100 patients, 84 % were men who have sex with men. Laboratory parameters of liver involvement were present in 19 of the 100 HIV-infected patients with syphilis; these resolved after successful antibiotic treatment. Among these 19 patients, six were diagnosed to be in the latent stage, with elevated liver enzymes and parameters of inflammation representing the only distinctive feature. Conclusions: Based on our results, syphilis should be included in the differential diagnosis of increased liver enzymes in HIV-infected patients. [ABSTRACT FROM AUTHOR]

Abstract: Aim: Familial Mediterranean fever is characterised by recurrent, febrile, inflammatory attacks of the serosal membranes. Prolonged inflammatory response is triggered secondary to cytokine stimulation due to reduced activity of pyrin. Inflammatory cytokines play major role in the pathogenesis of acute liver injury; and chronic, recurrent cytokine production may cause chronic hepatitis/cirrhosis. We aimed to analyse liver involvement in children with Familial Mediterranean fever. Patients: The study included 58 patients with Familial Mediterranean fever. Patients with liver involvement were examined in detail. Results: Liver involvement was seen in 11 of 58 patients (18.9%). Two patients (3.4%) had abnormal liver enzymes during the diagnostic evaluation, whilst 9 patients (15.5%) were admitted with the features of liver diseases, and had final diagnosis of Familial Mediterranean fever (2 had Budd–Chiari syndrome, 5 had chronic hepatitis/cirrhosis, 2 had acute hepatitis). None of the demographic factors or laboratory findings was different between the patients with or without liver involvement M694V allele was more common in patients with liver involvement but did not reach significant difference (50% vs. 33.6%, p =0.21). All the patients showed clinical and laboratory improvement after colchicine. Conclusion: Paediatric hepatologists must keep Familial Mediterranean fever in mind in the patients with cryptogenic hepatitis/cirrhosis especially in regions where hereditary inflammatory diseases are common. [Copyright &y& Elsevier]

Introduction: Liver involvement is commonly seen in disseminated T cell lymphoma; however, it is rarely the presenting organ. Here, we describe a case of T cell lymphoma presenting as acute hepatobiliary disease leading to hepatic failure and death. Discussion: Forty-seven-year-old male with history of cirrhosis (etiology undetermined), diabetes mellitus, and pancytopenia was admitted to ICU for hypotension and failure to thrive. He had icterus, minimal ascitis, and hepatosplenomegaly on physical examination. No lymphadenopathy was noted. Laboratory workup on admission showed elevated total bilirubin (10.1 mg/dl) and liver enzymes. Serology for acute viral hepatitis, human immunodeficiency virus, Epstein-Barr virus, and autoimmune hepatitis was negative. CT abdomen showed cirrhotic liver with heterogeneous arterial enhancement of the liver without definite mass lesions. Hospital course was complicated by progressively worsening hypotension, respiratory failure, profound acidosis, disseminated intravascular coagulation, and multi-organ system failure leading to death on hospital day 12. Autopsy of the liver showed cirrhotic changes with infiltration with atypical small lymphocytes confined to septa which were CD3 and CD5 positive (CD4 weakly positive, CD8, CD20, CD57, CD56, CD30, Alk-1, granzyme B, TIA1, and S100 negative). Unusual clinical/histological features include (1) initial clinical presentation of hepatic dysfunction without obvious physical signs of lymphoma, (2) negative workup for viral, toxic, autoimmune, and metabolic liver disease, (3) involvement of the entire liver, observed as heterogeneous enhancement of liver without any focal mass lesion as seen on CT scan, (4) an aggressive nature of disease, and (5) autopsy of liver with T cell infiltration confined to septa. Initial diagnosis was challenging due to unusual clinical presentation suggesting inflammatory hepatobiliary disease and the absence of enlarged lymph nodes. Conclusion: In conclusion, early suspicion of this aggressive lymphoma is important and should be considered in the evaluation of a patient whose course is atypical for hepatitis. Even in the absence of a mass lesion or lymphadenopathy, hepatosplenic T cell lymphoma should be included in the differential diagnosis of acute hepatic dysfunction in a patient who has no evidence of viral, toxic, autoimmune, or metabolic liver disease. [ABSTRACT FROM AUTHOR]

Abstract: Liver involvement is common in connective tissue disorders and usually asymptomatic. However, it may be symptomatic and cases of fulminant hepatitis have been reported. A diagnosis of specific hepatic involvement needs to rule out drug toxicity, viral hepatitis, or auto-immune liver disease. The large panel of auto-antibodies that is now available to the clinician is helpful to differentiate auto-immune hepatitis and specific liver involvement associated with connective tissue disease. In the latter, the outcome is generally favourable with immunosuppressive treatment. [Copyright &y& Elsevier]

Syphilis is an important public health issue and continues to occur at high rates among HIV-infected patients. Although abnormal liver function tests are common among HIV-infected persons, the incidence of syphilitic hepatitis in this population is currently unknown. We present two cases of syphilitic hepatitis and performed a retrospective study to determine the incidence of hepatitis during early syphilis infections among HIV-infected persons. Our study showed that syphilitic hepatitis is common, occurring in 38% (12/32) of HIV-positive patients with early stages of syphilis infection. Most cases occurred during secondary syphilis, with the most common finding being a maculopapular rash. Syphilis should be included in the differential diagnosis of HIV patients presenting with liver test abnormalities, rash and/or sexual risk factors. [ABSTRACT FROM AUTHOR]

Aims: Liver involvement is a common finding in patients suffering from lymphoproliferative disease, and histopathological patterns of infiltration vary according to lymphoma subtype. Data correlating the form of liver involvement with distinct lymphoma subtypes is, however, scarce. The aim was to review 89 liver biopsies diagnosed with lymphoma infiltration and evaluate the infiltration patterns. Methods and results: In equivocal cases, additional immunohistochemical and molecular pathology analyses were performed to differentiate between neoplastic and reactive cell infiltrates and to classify the lymphoma subtypes. Diffuse large B-cell lymphoma (DLBCL), chronic lymphocytic leukaemia (CLL), Hodgkin’s lymphoma (HL) and Burkitt lymphoma (BL) were the most prevalent subtypes in our series, which included 14 different lymphoma entities in total. Whereas DLBCL and BL predominantly demonstrated tumour nodules deranging the normal hepatic architecture, CLL and HL mostly showed infiltration of the portal fields. Interestingly, distinct lymphoma entities, particularly marginal zone B-cell lymphomas (MZL) and HL, commonly revealed lympho-epithelial lesions of bile ducts, which were observed in 10% of all investigated cases. Four cases, initially interpreted as T-cell lymphomas, proved to be reactive T-cell lesions. Conclusions: Distinct lymphoma subtypes show characteristic patterns of liver infiltration. Additional molecular analyses can support diagnosis by verification of clonality or detection of characteristic genetic aberrations. [ABSTRACT FROM AUTHOR]

Abstract: Introduction: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia; mutations in at least three genes (ENG, ACVRL1 et MADH4), which are components of transforming growth factor (TGF)-β, may lead to the clinical picture of HHT. HHT is a multisystemic angiodysplasia, resulting in multiple vascular malformations, involving notably the liver. Exegesis: We report the case of a patient with anicteric cholestasis, revealing HHT. Abdominal CT-scan revealed hepatic artery dilation and multiple arteriovenous fistula. At 2-year follow-up, the patient exhibited liver involvement-associated high output cardiac insufficiency. Conclusion: Liver involvement is frequent in HHT, occurring in 8–31% of patients; it may lead to life-threatening complications, such as high output cardiac failure, portal hypertension or severe cholangitis. Abdominal Doppler ultrasonography is a non-invasive accurate method, suitable for first-line imaging of the liver in patients with HHT; it should be done in all patients, in order to detect HHT-related hepatic vascular malformations. [Copyright &y& Elsevier]

Objective. To study the prevalence and predisposing factors of liver involvement in sickle cell disease (SCD) of patients with acute vaso-occlusive crisis. Material and methods. We prospectively evaluated 41 consecutive patients (44% M, median age 39 years, range 16-56 years) with homozygous (HbSS; 12 cases) or sickle cell-beta thalassemia (HbSβ-thal; 29 cases), admitted to our Medical Department in the period 2002 to 2004. Severity of crisis was graded by in-house-modified APACHE score; presence of asplenia or functional hyposplenism was also considered. Hematological and biochemical parameters including various relevant enzymes/isoenzymes were followed daily. Results. Despite the fact that only 9 patients (22%) presented with acute painful hepatomegaly, liver involvement was evident in 16 (39%); hepatocellular-type injury was found in 1 patient, cholestatic in 8, and mixed in 7. Severity of crisis was not related to liver involvement (score 20.6 versus 18.2), but liver involvement occurred in the presence of normal spleen function (p<0.001) and platelet counts <500,000/mm3 (p<0.001) were. Patients with liver involvement, compared with those without, had higher total and direct bilirubin levels (4.3 versus 2.9 mg/dL, p=0.050; 1.9 versus 0.8 mg/dL, p=0.010, respectively), lower hematocrit (19% versus 23%, p=0.030) and longer hospitalization (10 versus 6.3 days, p<0.001). Conclusions. In SCD, there is a 39% prevalence of acute veno-occlusive involvement of the liver, a figure that is much higher than previously reported. The type of injury is mostly mixed hepatocellular-cholestatic or purely cholestatic and its course is usually benign. Liver involvement occurs more often in patients with normal spleen function and is not associated with the overall severity of the acute episode, both observations being unreported previously. [ABSTRACT FROM AUTHOR]

To formulate recommendations about clinical management of liver involvement in hereditary hemorrhagic telangiectasia (HHT), using a formal consensus development process. Consensus Process: A nominal group technique was used. A list of main clinical, diagnostic and therapeutic issues about liver involvement in HHT was generated by the organizing committee. Panel members then scored their agreement with each statement; the median score, and standard deviation for each statement were determined for each of the three successive panel rounds. These consensus statements formed the basis for recommendations graded with the strength and quality of supporting evidence. Recommendation Statements: Doppler US is sufficiently accurate and suitable for first-line imaging of the liver in the general HHT population. Liver biopsy in any patient with proven or suspected HHT should be avoided. Liver involvement in HHT is generally asymptomatic; in the minority of patients where it is symptomatic, morbidity and mortality can be substantial. The prevalence of focal nodular hyperplasia is much higher in patients with liver involvement by HHT than in the general population. Invasive therapies for liver involvement by HHT (namely liver transplantation) should be considered only in patients who have failed to respond to intensive medical therapy. [ABSTRACT FROM AUTHOR]

The possibility of liver involvement inMycoplasma pneumoniaepneumonia is still controversial. This study investigated 33 adult patients with serologically confirmedM. pneumoniaecommunity-acquired pneumonia (CAP) (median age 31 years) and 38 patients with bacteraemicStreptococcus pneumoniaeCAP (median age 54 years), all without pre-existing liver disease. Serum alanine aminotransferase (ALT) levels were elevated in 12 (36.4%) patients withM. pneumoniaeCAP (median 53.5 U/L), and in four (10.5%) patients withS. pneumoniaeCAP (median 61 U/L) (p 0.025). In most patients withM. pneumoniaeCAP, the elevated ALT levels decreased during macrolide therapy, although this decrease was not significant. [ABSTRACT FROM AUTHOR]

An original experimental model for detecting organ-specific markers of predisposition to ethanol hepatotoxicity is proposed. A relationship between congenital activity of LPO processes in rat liver (before ethanol intoxication) and the type and severity of ethanol-induced damage to the liver was demonstrated using methods of mathematical modeling. It was proven that intact rats with genetically high MDA levels in the liver and more active systems of MDA generation in ascorbate- and NADPH-dependent reactions are prone to ethanol-induced damage to the liver. [ABSTRACT FROM AUTHOR]

Pontocerebellar hypoplasia (PCH) is a heterogeneous neurodevelopmental disorder that is characterized by decreased brainstem and cerebellum volume. Pontocerebellar hypoplasia type 6 (PCH6) is a mitochondrial disease associated with autosomal recessive inheritance that results from mutations in the RARS2 gene. In this case report, we describe a new clinical presentation with a novel RARS2 pathogenic variant. We report here on 2 siblings who presented with neonatal lactic acidosis, microcephaly, growth retardation, persistent seizures, and cholestasis with a previously undefined RARS2 pathogenic variant. In our literature review, we evaluated the clinical features and pathogenic variants of 34 patients reported in 16 publications since the initial identification of RARS2 pathogenic variants in PCH6 in 2007. Both siblings were detected with c.1564G>A (p.Val522Ile), a novel homozygous pathogenic variant of the RARS2 gene. Imaging revealed advanced cerebral atrophy and cerebellar hypoplasia, while the basal ganglia and pons were preserved. At follow-up, the elevations in liver function test results and cholestasis had regressed while the LDH and GGT elevations persisted. Both siblings showed microcephaly on follow-up and started to suffer seizures. Severe developmental delay and nutritional problems were observed, and both died in infancy. RARS2 pathogenic variant is a mitochondrial disease that causes severe mental, motor, and developmental retardation, as well as short life expectancy. Our patients are the first cases with liver involvement in PCH6 and a novel homozygous RARS2 pathogenic variant to be reported in the literature. This additional phenotype can be considered as making a valid contribution to the literature., Competing Interests: The authors have no conflicts of interest to declare., (Copyright © 2022 by S. Karger AG, Basel.)

Copyright of Zeitschrift für Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

We report a case of secondary heart involvement in AIDS-related primary lymphoma of the liver. A worsening dyspnea led to the diagnosis of pericardial effusion, and transesophageal echocardiography revealed the presence of large endocardial ventricular masses. Clinical suspicion of a lymphomatous origin was confirmed at the autopsy, which showed an extranodal dissemination pattern (heart, liver, intestine, and lung). In AIDS patients, both primary and secondary lymphomatous heart involvement are increasing in incidence. Clinical symptoms and signs are vague. Since the hematogenous route is the most common pattern of involvement, even extrathoracic lymphomas can present heart dissemination. Thus, it should be suspected in lymphoma patients who present with even mild aspecific heart symptoms. Appropriate imaging procedures include transesophageal echocardiography and, if possible, ECG-gated MRI. A negative transthoracic echocardiograph does not exclude the presence of myocardial tumor. Chemotherapy is only occasionally beneficial, and the prognosis remains poor. [ABSTRACT FROM AUTHOR]

Prolonged plasma-exchange without addition of cytotoxic agents was employed in 16 patients with mixed cryoglobulinemia and kidney, liver or neurologic involvement. Patients with rapidly progressive renal failure or active and reversible lesions generally improved after plasma-exchange, as well as those with a recently occurring sensory-motor peripheral neuropathy. In 4 out of 6 patients with mixed cryoglobulinemia and chronic active hepatitis, plasma-exchange was followed by either normalization or significant reduction of liver enzymes and bromosulfophthalein retention. In all cases responding to plasma-exchange the beneficial effects were evident after the first 2-3 weeks of treatment, while symptoms did not generally recur when the procedures were either slowly tapered or discontinued. Although the pathogenetic mechanism(s) of action of plasma-exchange remains largely unknown, preliminary data indicate that these procedures induce quantitative as well as qualitative changes in the immune system. [ABSTRACT FROM AUTHOR]

Liver involvement in Langerhans cell histiocytosis (LCH) typically presents with hepatomegaly and other signs of liver dysfunction. We present an 11-month-old child having only minimally elevated liver enzymes as an indication of liver involvement. Using sonography as the initial diagnostic tool followed by MRI, LCH of the liver was revealed. A review of sonographic, CT, MRI and MR cholangiopancreatography findings in liver LCH is presented. We recommend that physicians consider sonography and MRI screening for liver involvement in patients with newly diagnosed LCH, as periportal involvement may be present with little or no liver function abnormality present, as in this patient. [ABSTRACT FROM AUTHOR]

Background: Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma accounting for less than 1% of non-Hodgkin lymphomas. It is generally associated with poor prognosis., Patients and Methods: We performed a cohort study of patients with HSTCL treated at the Mayo Clinic between 1996 and 2020 exploring the clinical characteristics and therapeutic outcomes., Results: Twenty-two cases of HSTCL were identified with a median (range) age at diagnosis of 45.5 (15.5-80.6) years and a male predominance (15/22, 68.2%). Clinical characteristics include massive splenomegaly in 16 patients (73%), hepatic involvement in 13 (59%), and chronic immunosuppressed state in 8 (36%). Phenotypically, lymphoma cells had gamma/delta T-cell receptor expression in 18 (82%) and alpha/beta in 4 patients. Cytogenetic abnormalities included isochromosome 7q (i7q) in 8 (62%) of 13 and trisomy 8 in 4 (44%) of 9. The median (range) follow-up of surviving patients was 33 (2.5-137) months. The median progression-free and overall survival were 9.5 months (95% CI, 1.8, 16.3) and 12.4 months (95% CI, 4.9, 18.5), respectively. Long-term survival was seen in 4 (18%) of 22 patients, with survival of 55, 74, 95, and 137 months. Moreover, 3 of 4 long-term survivors had splenectomy as part of initial treatment, and 2 of 4 long-term survivors received an allogeneic hematopoietic cell transplant (allo-HCT)., Conclusion: Liver involvement and chronic immunosuppression were associated with shorter survival. Although splenectomy and allo-HCT have anecdotal benefit in the literature, our data do not show a statistically significant benefit of splenectomy and/or allo-HCT, likely as a result of our small sample size., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objectives: The seroprevalence of hepatitis A virus antibodies was investigated in a population of 1051 subjects, of whom 376 were controls and 675 were exposed to different degrees of biological risk. Methods: The exposed group was subdivided into subjects at low (242), intermediate (265), and high (168) biological hazard; all subjects were employed in the biomedical field. Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were also determined. Results: The seroprevalence of positive hepatitis A antibodies was 44.9% in all subjects but was significantly higher in males (50.6%) than in females (34.2%) and increased according to age (25.9% in subjects aged ≤40 years and 62.2% in subjects aged >40 years). No difference related to exposure to the biological risk was observed. The prevalence of transaminases at levels above normal values (χ2 = 4.079, P < 0.05 for AST and χ2 = 4.806, P < 0.05 for ALT) and mean values (AST P < 0.05; ALT P < 0.001) appeared significant in hepatitis A virus-positive subjects. On the other hand, excluding individuals with positive hepatitis C virus antibodies (16) and positive hepatitis B virus surface antigen (12), a prevalence of transaminase alterations was not observed, but mean levels of ALT lasted significantly longer in subjects with positive hepatitis A virus antibodies ( P < 0.01). Conclusions: The results confirm that hepatitis A virus is not a risk for employees in the biomedical field, but the presence of hepatitis A virus antibodies suggests a possible, though not clinically evident, liver involvement. [ABSTRACT FROM AUTHOR]

Hepatic involvement in Hodgkin lymphoma (HL) is uncommon (∼5% of patients) but always implies stage IV disease. Accurate staging is mandatory for making the appropriate risk assignment and treatment decisions. The Staging Evaluation and Response Criteria Harmonization for Childhood, Adolescent and Young Adult Hodgkin Lymphoma (SEARCH for CAYAHL) international working group conducted a systematic literature review of liver involvement in HL patients with the aim to propose a universally acceptable definition for liver involvement in pediatric HL. Thirty-three articles describing 6985 pediatric and adult HL patients were reviewed, of which 539 (7.7%) mentioned liver involvement. The literature did not provide a uniform definition of hepatic involvement and we propose consensus criteria derived from the EuroNet and Children's Oncology Group protocols, where liver involvement is defined as any hepatic lesion on computed tomography scan that correlates with 18 F-FDG uptake greater than background liver. A clear definition of liver lesions is necessary to consistently identify liver involvement and compare its impact on outcomes among protocols worldwide., (© 2020 Wiley Periodicals, Inc.)

Background: Liver involvement is not considered a typical extra-articular manifestation and has rarely been studied in rheumatoid arthritis (RA). We aimed to identify the prevalence and aetiologies of liver disease in RA patients., Methods: A cross-sectional study included 150 patients with RA enrolled over 5 years (2010- 2015). The clinical and paraclinical features of RA were analyzed. The clinical and biological characteristics of liver impairment and its aetiologies were collected., Results: One hundred and fifty RA patients (124 women) with a mean age of 57.09 years and a mean RA duration of 7.52 years were included. Liver involvement was diagnosed in 66 patients (44%). The liver disease was asymptomatic in 94% of the cases, revealed by increased gammaglutamyl transferase levels in 74% of the patients. The aetiologies of liver involvement were hepatotoxicity of medications in 38 cases (57%), hepatitis B and C in 14 patients (21%), fatty liver disease in 10 cases (15%), autoimmune liver disease in 2 patients (3%), hydatid cyst in 1 case (2%), and liver angiomas in 1 case (2%). Non-steroidal anti-inflammatory drugs and methotrexate were the drugs most often involved in the genesis of hepatotoxicity (21% and 20% of the cases, respectively)., Conclusion: Liver involvement occurred in 44% of RA patients. Aetiologies were mainly hepatotoxicity and viral hepatitis B and C. Patients with RA should be systematically screened for liver disease, which is rarely symptomatic., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Hepatic abnormalities in patients with lymphoproliferative disorders are common and can occur from direct infiltration by abnormal cells, bile duct obstruction, paraneoplastic syndrome, hemophagocytic syndrome, drug-induced liver injury, opportunistic infections, and reactivation of viral hepatitis. Hepatic involvement by lymphoma is often in association with systemic disease and rarely seen as a primary hepatic lymphoma. Vanishing bile duct syndrome is a well-known complication of Hodgkin disease. Antiviral prophylaxis for hepatitis B virus (HBV) reactivation is recommended for all HBsAg +  patients undergoing chemotherapy and all resolved HBV patients undergoing rituximab therapy and stem cell transplantation., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Actinomycosis is a rare chronic invasive disease caused by Actinomyces spp. Although abdominopelvic actinomycosis, which involves the colon and the pelvic organs extensively, has been frequently reported, abdominopelvic actinomycosis presenting with colon perforation and hepatic involvement concurrently has yet to be reported.Case Presentation: A 55-year-old woman presented at the emergency room with squeezing epigastric pain. Palpation of the abdomen revealed a hard mass with no acute peritoneal signs. Vital signs were normal range except for tachycardia. Initial laboratory testing revealed leukocytosis, anemia, elevated C-reactive protein (CRP), hypoalbuminemia; and normal AST/ALT and BUN/creatinine. CT scan of the abdomen-pelvis revealed a microperforations of the sigmoid colon, abscess in the left lower quadrant and hepatic lesion. Furthermore, there was a large infiltrating conglomerated mass invading the urinary bladder, left adnexa, sigmoid, left inguinal canal and left pelvic wall area. Ultrasound revealed an intra-uterine device (IUD). All these findings initially raised a suspicion of malignancy such as advanced cancer of the colon with liver metastasis. Despite the rarity of the disease, actinomycosis were not excluded because of the IUD found on ultrasound. Parenteral antibiotics and percutaneous drainage of abdomen abscess as well as fasting with total parental nutrition were prescribed for sigmoid perforation and abscess. After 10 days of conservative treatment, no remarkable change was detected in conglomerated mass invading pelvis. Furthermore, the finding of newly developed mechanical small bowel obstruction warranted surgery. Exploratory laparotomy was performed for the removal of perforated colon, obstructive small bowel and organs involved and postoperative histology confirmed a diagnosis of colonic actinomycosis. The patient made an uneventful recovery and was started on a 6-month course of penicillin.Conclusions: Abdominopelvic actinomycosis presenting with colon perforation and hepatic involvement is extremely rare; however, it is clinically similar to advanced colon cancer with liver metastasis, therefore, complicating the preoperative diagnosis. A diagnosis of abdominopelvic actinomycosis should be considered in patients with a history of IUD and chronic abdominal pain, along with an abdominal mass or cutaneous abscess. If surgery is indicated, preoperative empirical antibiotic therapy for actinomycosis and frozen biopsy during surgery may be considered. [ABSTRACT FROM AUTHOR]