Hepatic Disease In Patients Research Articles

The Key role of intraoperative ultrasound staging in patients with intrahepatic cholangiocarcinoma

Background: Intraoperative ultrasound (IOUS) offers an accurate staging of hepatic disease in patients with liver malignancies but its role in patients with intrahepatic cholangiocarcinoma (IHC) has been poorly evaluated. Aim of the study was to assess the impact of IOUS staging for IHC. Methods: Data from a prospectively collected database of patients with IHC were retrospectively analyzed. All patients scheduled for liver resection were considered for the study. Performance of intraoperative ultrasound staging (both open and laparoscopic) was evaluated. Results: From January 2000 to December 2020 175 patients with IHC were scheduled for hepatectomy. 14 (8%) were considered unresectable at staging laparoscopy: 9 because of peritoneal carcinomatosis and 5 owing to laparoscopic ultrasound (LUS) findings. 161 liver resections were performed. 183 lesions suspected for IHC were preoperatively detected. All patients were staged by thoracoabdominal CT. MRI and PET-CT were also performed in 99 (61.5%) and 51(31.6%) cases respectively. In 22 patients IHC was misdiagnosed (i.e. colorectal metastases or HCC, etc.) at preoperative imaging as well in 12 patients at IOUS. Concordance rate of preoperative diagnosis with IOUS was 93%. Intraoperatively, 34 nodules were newly detected by IOUS in 20 patients (12.4%). 30 were confirmed IHC at pathological examination. The median diameter of new nodules was 5mm (4-8). IOUS also found satellite nodules in 19 patients (12%). Vascular relationships were modified in 14 patients compared to preoperative imaging modalities. Surgical plan was changed according to IOUS or LUS findings in 33(20.5%) cases. Time to first recurrence was worse in patient with new IHC (4 vs 11.6 months, p<0.001). Conclusion: IOUS is mandatory for a correct staging in patients undergoing resection for IHC allowing intraoperative characterization of significant prognostic factors.

Is diffusion-weighted MRI sufficient for follow-up of neuroendocrine tumour liver metastases?

To assess if diffusion-weighted imaging (DWI) alone could be used for follow-up of neuroendocrine hepatic metastases. This was a retrospective study, approved by the institutional review board. Twenty-two patients with neuroendocrine liver metastases who had undergone more than one liver magnetic resonance imaging (MRI) examination, (including DWI and using hepatocyte-specific contrast medium) were evaluated. Up to five metastases were measured at baseline and at each subsequent examination. The reference standard measurement was performed on the hepatocyte phase by one reader. Three independent readers separately measured the same lesions on DWI sequences alone, blinded to other sequences, and recorded the presence of any new lesions. The longest diameters of 317 liver metastases (91 on 22 baseline examinations and a further 226 measurements on follow-up) were measured on the reference standard by one reader and on three b-values by three other readers. The mean difference between DWI measurements and the reference standard measurement was between 0.01-0.08cm over the nine reader/b-value combinations. Based on the width of the Bland and Altman interval containing approximately 95% of the differences between the reader observation and the mean of reference standard and DWI measurement, the narrowest interval over the nine reader/b-value combinations was -0.6 to +0.7cm and the widest was -0.9 to 1cm. In the evaluation of overall response using Response Evaluation Criteria in Solid Tumors (RECIST) 1.1 criteria, the weighted kappa statistic was between 0.49 and 0.86, indicating moderate-to-good agreement between the reference standard and DWI. The visualisation and measurement of hepatic metastases using DWI alone are within acceptable limits for clinical use, allowing the use of this rapid technique to restage hepatic disease in patients with neuroendocrine metastases.

Identifying predictors of hepatic disease in patients after the Fontan operation: A postmortem analysis

We characterized the histologic liver changes that occur after the Fontan operation and identified the factors associated with worsening hepatic disease. From 1979 to 2009, 33 patients who died after the Fontan operation had a postmortem analysis at our institution. The liver histologic findings were graded for sinusoidal and portal fibrosis. The median interval from the Fontan operation to death was 14 days (range, 0 days to 25.6 years). The median age at death was 7.7 years (range, 1.9-52.9 years). Hepatic sinusoidal fibrosis occurred in 25 of 33 patients (76%), with significant fibrosis (grade 2 or greater) in 15 of 25 patients (60%). Portal fibrosis occurred in 17 of 33 patients (52%). The degree of sinusoidal (P<.003) and portal fibrosis (P<.002) correlated with an increased period from the Fontan operation to death. Sinusoidal fibrosis was also associated with increased age at the initial Fontan operation (P<.03). Of the patients who died early (< 35 days postoperatively) after the Fontan operation, sinusoidal fibrosis was present in 15 of the 23 patients (65%) and portal fibrosis in 7 of 23 patients (30%). Significant (grade 2 or greater) sinusoidal fibrosis was seen in 7 patients, and 4 of the 7 had heterotaxy syndrome. Significant portal fibrosis was observed in only 2 patients; both had heterotaxy syndrome. The interval from the initial Fontan operation to death is associated with increased sinusoidal and portal fibrosis. In this small cohort, older age at Fontan operation was associated with sinusoidal fibrosis. Significant sinusoidal fibrosis was observed in patients who died early after the Fontan operation, with more severe disease present in patients with heterotaxy syndrome.

Case 183: Obliterative Portal Venopathy

Obliterative portal venopathy—which is characterized clinically by the presence of portal hypertension in the absence of liver insufficiency—has recently been described as a non–AIDS-defining hepatic disease in patients with the human immunodeficiency virus, and its imaging findings—other than the usual sings of portal hypertension—include liver morphologic changes, multifocal thrombosis of intrahepatic portal branches, parenchymal enhancement abnormalities, focal nodular hyperplasia-like nodules, and extrahepatic portal vein thrombosis.

Non-invasive detection of hepatic amyloidosis: FibroScan, a new tool

Introduction. FibroScan, a non-invasive tool for measuring liver stiffness (LS), is not specific to liver fibrosis. Other extra-hepatic conditions may modify the LS value.Objectives. Our aim was to examine whether amyloid deposition in the liver may modify LS.Methods. LS was measured prospectively in 41 patients with systemic AL amyloidosis (AL) in the French AL Reference Center, comprising: 5 patients with liver involvement (LI) and no cardiac involvement (CI), 11 with CI and no LI, 12 with both LI and CI and 13 with neither (2005 consensus criteria); 26 negative controls, 50 patients infected with Hepatitis C virus (HCV)-infected and 18 AL-free patients with right-sided heart disease (‘cardiac controls’) were also examined.Results. Median LS was significantly higher in patients with AL with liver involvement [27.4 (10.3–75) kPa] than in negative controls [4.8 (2.8–11.9) kPa] (p < 0.0001), and patients infected with HCV [(6.8 (2.9–69.1) kPa] (p = 0.001), and tended to be higher than in the ‘cardiac controls’ [11 (4.1–75) kPa] (p = 0.08). A cut-off value of 17.3 kPa, prioritising specificity, is proposed for routine diagnosis of significant AL liver infiltration.Conclusion. LS > 17.3 kPa is suggestive of AL hepatic disease in patients with non-fibrotic liver changes, and may have diagnostic value in patients with known AL.

A Comparison of Transjugular and Plugged-Percutaneous Liver Biopsy in Patients with Contraindications to Ordinary Percutaneous Liver Biopsy and an “In-House” Protocol for Selecting the Procedure of Choice

The purpose of this study was to evaluate the effectiveness and safety of transjugular liver biopsy (TJLB) and plugged-percutaneous liver biopsy (PB) in consecutive patients with severe liver disease associated with impaired coagulation, ascites, or both and to verify the in-house protocol used to select the appropriate procedure. In 2000-2006, 329 patients (208 male [62.8%] and 121 female [37.2%]), aged 1 month to 81 years (mean, 46.8 years), underwent 150 TJLBs (39.1%) or 233 PBs (60.9%) procedures at a major tertiary center, as determined by an in-house protocol. The groups were compared for specimen characteristics, technical success, and complications. Technical success rates were 97.4% for TJLB (146/150) and 99.1% for PB (231/233). TJLB was associated with a lower average core length (1.29 vs. 1.43 cm) and lower average number of specimens obtained (2.44 vs. 2.8), but both methods yielded sufficient tissue for a definitive diagnosis. There were no major complications in either group. TJLB and PB can be safely and effectively performed for the diagnosis of hepatic disease in patients with contraindications for standard percutaneous liver biopsy. When both are technically available, we suggest PB as the procedure of choice, especially in transplanted livers.

Novel Mutations in NPHP4 in a Consanguineous Family With Histological Findings of Focal Segmental Glomerulosclerosis

Nephronophthisis is a form of autosomal recessive hereditary cystic kidney disease that typically progresses to end-stage renal disease by early adulthood. Conversely, focal segmental glomerulosclerosis is a histological glomerular phenotype that can be familial, primary (idiopathic), or secondary to a multitude of pathological processes affecting the kidney, including such tubulointerstitial diseases as nephronophthisis. Mutations in 6 distinct nephronophthisis genes have been described to date. We describe a consanguineous Filipino family with 2 novel sequence variants in the NPHP4 gene. Affected individuals presented with end-stage renal disease and histological features of focal segmental glomerulosclerosis on biopsy. They also had atypical radiological findings, making the clinical diagnosis of the genetic syndrome difficult. Furthermore, although ocular abnormalities and hearing loss were described previously, this is the first report of hepatic disease in patients with mutations in NPHP4. The diagnosis of nephronophthisis was made by means of mutational analysis of the NPHP4 gene after isolation of a region of homozygosity in affected individuals by using whole-genome single-nucleotide polymorphism analysis. Because establishment of the correct diagnosis has implications for therapeutic interventions, prognosis, and, in the case of heritable diseases, appropriate genetic counseling for affected individuals and their families, this report emphasizes the importance of obtaining meticulous clinical information, considering alternative diagnoses, and, when possible, performing genetic evaluation to confirm the diagnosis. We outline an approach to patients with hereditary kidney disease, focusing specifically on the molecular genetic techniques available to evaluate such families and determine a chromosomal region of interest and, subsequently, the diagnosis.

Freqüência de alterações hepáticas em pacientes com esclerodermia

INTRODUCTION: to determine the frequency of hepatic disease in patients with scleroderma and, secondarily, to investigate the frequency of hepatitis B and C virus infection and determine the frequency of autoantibodies in this disease. MATERIAL AND METHODS: patients with scleroderma followed at Hospital Santa Izabel were included in the study and patients with acne vulgaris served as a comparison group. RESULTS: considering the 65 scleroderma patients, 35% had elevated gamma-glutamyltranspeptidase (gamma-GT), 30% had elevated alkaline phosphatase and 17.1% had alanine aminotransferase (ALT) higher than the reference range. Raised ALT levels were more common in the scleroderma patients than in the control group. Nineteen percent (19%) of the patients tested positive for anti-smooth muscle antibodies and only one patient had anti-mitocondria antibodies. There was no statistical difference between the two groups regarding antibody testing. Anti-HCV antibodies were observed in one patient and HBsAg was detected in another scleroderma patient. There was no patient with clinically significant hepatic disease. CONCLUSIONS: the present study showed that clinical hepatic disease did not occur in our scleroderma patients, despite the relatively increased frequency of liver enzymes abnormalities.

Hepatic disease in patients with Acquired Immunodeficiency Syndrome (AIDS)

The acquired immunodeficiency syndrome is now the leading cause of death in the world. Liver involvement in opportunistic infections and neoplasms affecting patients with human immunodeficiency disease syndrome are common. Many of these patients also take many medicines and toxins that are potentially harmful to the liver. This is an overview on the aetiology and possible diagnostic guide to determine liver involvement in patients with HIV infection. A literature review was performed on major published series on the liver and HIV infection between 1985 and 1999, both years inclusive. Data and opinions from 5 general reviews and 31 original articles from MEDLINE on liver disease in patients with HIV infection regarding aetiology, pathology, presentation and patient evaluation are summarised. The liver is frequently affected in patients with AIDS. The majority of the patients have hepatomegaly and abnormal liver enzymes secondary to involvement with opportunities infections, AIDS associated neoplasms and drug therapy. Most of the infections reach the liver by lymphohaematogeneous spread from other sites in the body. Methodical approach in patient evaluation is therefore essential for prompt diagnosis and treatment to minimise morbidity and early mortality.

Lack of evidence of hepatic disease in patients with oral lichen planus in Serbia.

The reported frequency of chronic liver disease, and particularly, Hepatitis C virus (HCV) infection in patients with oral lichen planus (OLP) shows geographical variation. The aim of this study was to determine the prevalence of chronic hepatic disease, Hepatitis B virus and HCV infection in patients with OLP and control subjects resident in Serbia. In this prospective study 48 patients with OLP (33 women and 15 men, with a mean age of 49.7 years) and 60 control subjects (38 women and 22 men, with a mean age of 46.7 years) were examined for the presence of serological evidence of chronic hepatic disease, Hepatitis B surface antigen (HBsAg) and anti-HCV seropositivity. All patients with OLP had normal liver function. HBsAg was not found in any of the patients with OLP or control group. None of the patients with OLP or control subjects were HCV seropositive. Patients with OLP resident in Serbia do not have evidence of chronic liver disease or infection with HBV or HCV. The exact aetiological role between hepatic disease and OLP remains unclear.

Redox tolerance test: An indicator of preoperative hepatic functional reserve in liver diseases

Changes in the arterial ketone body ratio during oral glucose loading were observed preoperatively in 134 patients with liver disease, and these data were correlated with the pathology findings of liver tissue and the postoperative clinical course. It was found that the redox tolerance index (RTI) could serve as a quantitive indicator of the severity of the underlying chronic hepatic disease in patients with liver disease, and as a reliable indicator of preoperative hepatic functional reserve. When the RTI was 0.70 or more, the underlying hepatic disease was slight and there was good hepatic functional reserve; these patients could tolerate any type of hepatic resection. When the RTI was less than 0.70 and more than 0.5, the underlying hepatic disease was moderate and hepatic functional reserve was poor; if the RTI was less than 0.50, major hepatic resection could not be tolerated.

DIAGNOSIS AND TREATMENT OF HEPATIC DISEASE IN PATIENTS WITH HIV

Liver involvement with opportunistic infections and neoplasms is a well-recognized component of AIDS, affecting most patients. The cause of hepatic disease in these patients may be divided into hepatitis, granulomatous disease, mass lesions, vascular lesions, hepatotoxic drugs, and nonspecific findings. With a rational approach, most patients with AIDS and liver disease can be diagnosed and treated in a cost-effective manner with low morbidity.

Hepatobiliary Dysfunction and Primary Sclerosing Cholangitis in Patients with Crohn's Disease

Background: Only a few studies have attempted to determine the prevalence of long-standing abnormal liver function and primary sclerosing cholangitis (PSC) in patients with Crohn's disease (CD). The aim of the study was to determine the prevalence of long-standing abnormal liver function test results and to describe the clinical, biochemical, and histologic findings in patients with large-duct classic PSC and small-duct PSC (that is, normal cholangiogram) in patients with CD during a 15-year period. Methods: Patients with CD and long-standing abnormal liver function results were investigated individually with endoscopic retrograde cholangiography and liver biopsy. Results: Of 262 consecutive patients with CD, 38 (15%) had long-standing increased alkaline phosphatase (ALP) values (mean, 1065 U/l; range, 321-4165 U/l). Of these, 10 patients were classified as having hepatic disease (4%), of which 9 had PSC and 1 had a non-specific reactive hepatitis. Of nine patients with PSC (3.4%), three were classified as having large-duct PSC; five, small-duct PSC; and one, unclassified. In patients with large-bowel CD (n = 102) the prevalence of PSC was 9%. Mean age at diagnosis of PSC was 35 years (22-46 years), and the female to male ratio, 7:2. All PSC patients had large-bowel involvement (P < 0.00015), and two of them developed colonic carcinoma of the large bowel (P < 0.01). All cases of small-duct PSC were stage 1, whereas large-duct PSC were stage 2-3. During the observation period (mean, 5.4 years) no PSC patients died. Conclusion: The results of our study indicate that PSC is the major hepatic disease in patients with CD and longstanding abnormal liver function tests and is approximately as prevalent as in ulcerative colitis. Patients with PSC and CD may have a milder liver disease than patients with PSC and ulcerative colitis, perhaps because large-duct PSC is less common in patients with CD. Cholangiograms and liver biopsies are both needed to evaluate the extent of the disease.

Iron state and hepatic disease in patients with thalassaemia major, treated with long term subcutaneous desferrioxamine.

Liver biopsies were performed on 51 regularly transfused patients with beta thalassaemia, age range 5-36 (mean 18.6) years, who had received regular subcutaneous desferrioxamine (DFX) treatment for periods between one and eight years (40 for eight years). The biopsy specimens were examined by light microscopy and immunofluorescence for hepatitis B virus surface and core antigens (HBsAg and HBcAg), and the iron content was determined chemically. The results were compared with serum ferritin concentration and aspartate transaminase (AST) activity and with hepatitis B virus serology. Biopsy specimens, in which chemical liver iron had been determined in 12, were also available from 17 patients. Mean serum ferritin (+/- SD) had fallen from 5885 (3245) micrograms/l to 1638 (976) micrograms/l in 36 patients after eight years' chelation, while mean (+/- SD) liver iron concentration had fallen from 2945 (900) micrograms/100 mg dry weight to 857 (435) micrograms/100 mg dry weight in 12 of them. All biopsy specimens examined were negative for HBs and HBc antigens. The presence of histological features of hepatitis was associated with increased liver iron content, increased fibrosis, and with progression of fibrosis between the two biopsies. Procollagen III peptide was assayed in 28 patients but did not correlate with the degree of hepatitis, fibrosis, or with chemical liver iron content. We conclude that with regular subcutaneous DFX, mean concentrations of serum ferritin and liver iron are maintained in these patients at about five and 10 times the normal value, respectively, and that progression of liver damage is more likely to be due to viral hepatitis, presumably related to the parenterally transmitted non-A, non-B agents than to iron overload.

Effects of three H 2 antagonists on the isolated perfused rat liver : Correlation of bile flow changes with potential for causing hepatic disease in patients

The adverse effects of oxmetidine, an H 2 blocking agent which has been shown to produce hepatic injury in 1–4% of patients, on an in vitro model were compared with those of cimetidine and ranitidine which have led to only rare instances of hepatic injury. Bile flow was measured in the isolated perfused rat liver (Wistar rats), comparing the effects of each of the three drugs with control perfusions. Oxmetidine in concentrations of 3 × 10 −3 M or greater led to a decrease in bile flow within 15 min and, at a concentration of 5 × 10 −3 M, to complete cessation of flow within 5 min. Lower concentrations (5 × 10 −4 M) led to a marked choleresis. Ranitidine and cimetidine in concentrations up to 5 × 10 −3 M produced no decrease in bile flow. Ranitidine, however, led to a choleresis at a concentration of 5 × 10 −3 M. The positive correlation between in vivo and in vitro toxicity supports the view that in vitro testing may prove to be of use in predicting the hepatotoxic potential of a drug.

Effects of H2-blocking agents on hepatocytes in vitro: correlation with potential for causing hepatic disease in patients.

The adverse effects on an in vitro model of oxmetidine, an H2-blocking agent which has been shown to produce hepatic injury in 1 to 4% of patients, were compared with those of cimetidine and ranitidine which have led to only rare instances of hepatic injury. Suspensions of hepatocytes, freshly isolated from Sprague-Dawley rats, were exposed to the three drugs. Oxmetidine, in concentrations of 3 X 10(-3) M or greater, led to leakage of AST into the medium after 4 hr of incubation. Ranitidine and cimetidine, in concentrations up to 5 X 10(-3) M, produced no identifiable leakage. Pretreatment of rats with phenobarbital, 3-methylcholanthrene, or SKF 525A resulted in no significant enhancement or inhibition of the oxmetidine effects. These results suggest that the adverse effects of oxmetidine on the hepatocytes are produced by the native compound, not a metabolite. The positive correlation between in vivo and in vitro toxicity supports the view that in vitro testing may prove to be of use in predicting the hepatotoxic potential of a drug.

Peliosis Hepatis in Hematologic Disease

Two patients with hematologic disease, one of whom had received androgenic steroids, had liver damage associated with peliosis hepatis. In one patient with spherocytic hemolytic anemia, peliosis hepatis was an incidental postmortem finding. In the other patient, who was treated with androgenic-anabolic steroids for aplastic anemia, hepatic failure associated with peliosis hepatis developed. Splenic involvement by peliosis was present in both patients. Peliosis hepatis should be considered in the differential diagnosis of hepatic disease in patients with hematologic disorders, especially if treatment has included androgenic-anabolic steroids.

HEPATIC COMPLICATIONS IN THE TREATMENT OF SYPHILIS

Article1 May 1936HEPATIC COMPLICATIONS IN THE TREATMENT OF SYPHILISII. INCIDENCE OF HEPATIC DISEASE IN PATIENTS WITH UNTREATED SYPHILIS AND DURING THEIR SUBSEQUENT TREATMENT*FREDERICK KELLOGG, M.D., NORMAN N. EPSTEIN, M.D., WM. J. KERR, M.D., F.A.C.P.FREDERICK KELLOGG, M.D.Search for more papers by this author, NORMAN N. EPSTEIN, M.D.Search for more papers by this author, WM. J. KERR, M.D., F.A.C.P.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-9-11-1561 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptHepatitis as a complication of anti-syphilitic therapy is not an uncommon occurrence. The injury thus suffered by the liver may be transient or it may be permanent, resulting in cirrhosis. Thus Baldridge1reported that, of a series of 36 patients with definite portal cirrhosis, one-third had histories of treatment for syphilis. During the past 10 years the value of controlling the arsphenamine treatment of syphilis by functional tests of the liver has been repeatedly emphasized. Such tests are indicated especially for patients with histories of hepatic disease or with evidence of disturbance of the liver developing during the course of...Bibliography1. BALDRIDGE CW: The relationship between antisyphilitic treatment and toxic cirrhosis, Am. Jr. Med. Sci., 1934, clxxxviii, 685. Google Scholar2. BISKINDEPSTEINKERR GRNNWJ: Hepatic complications in the treatment of syphilis, ANN. INT. MED., 1934, vii, 966. LinkGoogle Scholar3. ALTHAUSENBISKINDKERR TLGRWJ: The Rose Bengal test of hepatic function; a spectroscopic method, Jr. Lab. and Clin. Med., 1933, xviii, 954. Google Scholar4. ALTHAUSENGUNTHERLAGENKERR TLLJBWJ: Modification of the dextrose tolerance test as an index of metabolic activity of the liver, Arch. Int. Med., 1930, xlvi, 482. CrossrefGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: San Francisco, California*Received for publication March 18, 1936.From the Division of Medicine and the Department of Dermatology, University of California Medical School, San Francisco.†Research Fellow, American College of Physicians. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byAllgemeine Pathologie der Syphilis 1 May 1936Volume 9, Issue 11Page: 1561-1571KeywordsCirrhosisDermatologyHepatitis ALiverLiver diseasesSyphilis Issue Published: 1 May 1936 PDF downloadLoading ...