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Theo Heller - One of the best experts on this subject based on the ideXlab platform.
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recurrent Nodular Regenerative Hyperplasia post liver transplantation in common variable immunodeficiency
Hepatology, 2021Co-Authors: Julian Hercun, Ivan J. Fuss, David E. Kleiner, Esha Parikh, Gulbu Uzel, Warren Strober, Christopher Koh, Steven M Holland, Theo HellerAbstract:Liver involvement is well described in common variable immunodeficiency (CVID) and is an important prognostic marker. While multiple etiologies for liver disease have been reported, Nodular Regenerative Hyperplasia (NRH) is being increasingly recognized with an overall prevalence above 5% (1). In this case series we describe the pre and post-transplant evolution of CVID-related liver disease (CVID-ld). (Table 1.).
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Nodular Regenerative Hyperplasia in Common Variable Immunodeficiency
Journal of clinical immunology, 2013Co-Authors: Ivan J. Fuss, Julia C. Friend, Zhiqiong Yang, Lubna Hooda, James L. Boyer, Mark Raffeld, David E. Kleiner, Theo HellerAbstract:Purpose Patients with Common Variable Immunodeficiency (CVID) are subject to the development of a liver disease syndrome known as Nodular Regenerative Hyperplasia (NRH). The purpose of this study was to define the characteristics and course of this complication of CVID.
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Nodular Regenerative Hyperplasia and severe portal hypertension in cystinosis
Clinical Gastroenterology and Hepatology, 2006Co-Authors: Kevin Obrien, David E. Kleiner, Theo Heller, Nadeem Hussain, Bradley A Warady, Robert Kleta, Isa Bernardini, William A GahlAbstract:Background & Aims: Cystinosis is a rare autosomal-recessive disorder characterized by the intralysosomal accumulation of cystine, which is responsible for widespread tissue destruction. Liver biopsy specimens of patients with cystinosis show cystine crystal formation in Kupffer cells. However, significant liver disease and portal hypertension is not a common complication of cystinosis. We report the case histories of 2 young men with poorly treated nephropathic cystinosis who developed noncirrhotic portal hypertension with evidence of Nodular Regenerative Hyperplasia (NRH). Methods: Liver biopsy examinations, upper and lower endoscopy with biopsy examination, imaging studies, venous pressure measurements, and laboratory investigations were used to evaluate the causes of the liver disease and portal hypertension. Results: Histologic examination of liver biopsy specimens from both patients showed changes characteristic of NRH with portal hypertension documented by measurement of pressure gradients. In addition, endoscopy in the first patient showed varices and portal hypertensive gastropathy. Conclusions: NRH was confirmed by histologic examination of the liver in both patients and is the likely cause of their portal hypertension. NRH may represent a rare, late complication of cystinosis, although the mechanism remains undefined.
Sammy Saab - One of the best experts on this subject based on the ideXlab platform.
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patients with Nodular Regenerative Hyperplasia should be considered for hepatocellular carcinoma screening
Hepatology Research, 2014Co-Authors: Archita Sood, Gerald A Cox, Justin P Mcwilliams, Hanlin L Wang, Sammy SaabAbstract:The incidence of hepatocellular carcinoma (HCC) appears to be increasing across the globe. Well-established protocols for screening are available, and the most common underlying liver problem associated with the development of HCC is cirrhosis. However, with few exceptions, patients without cirrhosis are generally not screened for HCC. Nodular Regenerative Hyperplasia (NRH) is not associated with the development of significant fibrosis or impaired liver synthetic function. The major clinical impact of NRH appears to be in the development of portal hypertension. Patients with NRH are also not recommended to undergo routine screening for the development of HCC. This report describes a case of a 44-year-old woman with NRH found to have de novo HCC. Emerging evidence suggest a possible pathogenetic relationship between NRH and HCC. The case described here and our review of the published work suggests that additional studies regarding the epidemiological association between NRH and HCC may change the current notion that NRH is not a premalignant lesion, and further studies assessing the utility of routine screening of NRH patients for HCC should be considered.
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human immunodeficiency virus and Nodular Regenerative Hyperplasia of liver a systematic review
World Journal of Hepatology, 2014Co-Authors: Archita Sood, Mariana Castrejon, Sammy SaabAbstract:Human immunodeficiency virus and Nodular Regenerative Hyperplasia of liver: A systematic review
David E. Kleiner - One of the best experts on this subject based on the ideXlab platform.
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Nodular Regenerative Hyperplasia in x linked agammaglobulinemia an underestimated and severe complication
The Journal of Allergy and Clinical Immunology, 2021Co-Authors: Cristiane J Nunessantos, David E. Kleiner, Christopher Koh, Anjali Rai, Keith Sacco, Beatriz E Marciano, Jamie Marko, Jenna Bergerson, Michael Stack, Maria M RiveraAbstract:Background Late-onset complications in X-linked agammaglobulinemia (XLA) are increasingly recognized. Nodular Regenerative Hyperplasia (NRH) has been reported in primary immunodeficiency but data in XLA are limited. Objectives This study sought to describe NRH prevalence, associated features, and impact in patients with XLA. Methods Medical records of all patients with XLA referred to the National Institutes of Health between October 1994 and June 2019 were reviewed. Liver biopsies were performed when clinically indicated. Patients were stratified into NRH+ or NRH− groups, according to their NRH biopsy status. Fisher exact test and Mann-Whitney test were used for statistical comparisons. Results Records of 21 patients with XLA were reviewed, with a cumulative follow-up of 129 patient-years. Eight patients underwent ≥1 liver biopsy of whom 6 (29% of the National Institutes of Health XLA cohort) were NRH+. The median age at NRH diagnosis was 20 years (range, 17-31). Among patients who had liver biopsies, alkaline phosphatase levels were only increased in patients who were NRH+ (P = .04). Persistently low platelet count ( 6 months), mildly to highly elevated hepatic venous pressure gradient and either hepatomegaly and/or splenomegaly were present in all patients who were NRH+. In opposition, persistently low platelet counts were not seen in patients who were NRH−, and hepatosplenomegaly was observed in only 1 patient who was NRH−. Hepatic venous pressure gradient was normal in the only patient tested who was NRH−. All-cause mortality was higher among patients who were NRH+ (5 of 6, 83%) than in the rest of the cohort (1 of 15, 7% among patients who were NRH− and who were classified as unknown; P = .002). Conclusions: NRH is an underreported, frequent, and severe complication in XLA, which is associated with increased morbidity and mortality.
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recurrent Nodular Regenerative Hyperplasia post liver transplantation in common variable immunodeficiency
Hepatology, 2021Co-Authors: Julian Hercun, Ivan J. Fuss, David E. Kleiner, Esha Parikh, Gulbu Uzel, Warren Strober, Christopher Koh, Steven M Holland, Theo HellerAbstract:Liver involvement is well described in common variable immunodeficiency (CVID) and is an important prognostic marker. While multiple etiologies for liver disease have been reported, Nodular Regenerative Hyperplasia (NRH) is being increasingly recognized with an overall prevalence above 5% (1). In this case series we describe the pre and post-transplant evolution of CVID-related liver disease (CVID-ld). (Table 1.).
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Mo1001 Nodular Regenerative Hyperplasia: Many Ways to Get There
Gastroenterology, 2014Co-Authors: Jason L. Eccleston, David E. Kleiner, Christopher Koh, Mary Demino, Preeti A. Reshamwala, Xiongce Zhao, Mark T. Gladwin, Harry L. Malech, Dean D. Metcalfe, Warren StroberAbstract:Introduction: Nodular Regenerative Hyperplasia (NRH) is a rare noncirrhotic portal hypertensive liver disease characterized by the diffuse transformation of hepatic parenchyma into Regenerative nodules without fibrosis. Although the specific etiology has yet to be elucidated, NRH has been associated with various diseases, conditions and medications. Thus, whether NRH occurs from a single unifying pathophysiologic process or multiple different processes is still uncertain. Aims: To characterize biochemical, radiological, and histologic markers of liver disease in patients (pts) with and without NRH in multiple disease cohorts. Methods: Pts with chronic granulomatous disease (CGD), sickle cell disease (SCD), systemic mast cell disease (SMCD), common variable immunodeficiency disease (CVID) and sporadic cases of NRH underwent hepatologic evaluation and liver biopsy for clinical care purposes were evaluated with biomarkers of liver disease, radiologic imaging and histopathologic analysis. Results: 137 pts were evaluated and 56 (41%) were diagnosed with NRH histologically. Of those with NRH, 11 (20%) had CGD, 22 (39%) SCD, 7 (13%) SMCD, 12 (21%) CVID and 4 (7%) sporadic disease. Age at biopsy across all NRH cohorts was significantly older compared to the non-NRH population (mean 40 vs. 33 years, p=0.0128). Patients with NRH had significantly higher alkaline phosphatase (197 vs. 190 U/L, p=0.01), increased liver volumes (2152 vs. 1166 cm3, p=0.01) and increased spleen volumes (after excluding SCD) (1071 vs. 410 cm3, p=0.01), which negatively correlated with platelets ( ρ -0.58, p= 0.002). In the SCD cohort, pts with NRH had increased gamma-glutamyltransferase (128 vs. 67 U/L, p=0.03) and increased lactate dehydrogenase (432 vs. 322 U/L, p=0.01). On histologic evaluation, SCD-NRH pts had increased lobular inflammation (p=0.04). SMCDNRH pts had increased portal and peri-portal inflammation (p=0.005 and p=0.01, respectively), veno-occlusive changes (p=0.04), portal venopathy (p=0.001) and sinusoidal dilation (p=0.03). Conclusions: Despite parallels in clinical disease presentation, histopathologic examination of NRH in separate disease cohorts suggests different mechanistic pathways. Given the potential for multiple etiologies, NRH may be more common than previously thought. Further investigation should focus on the underlying disease process as it may provide a window into biology.
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Nodular Regenerative Hyperplasia in Common Variable Immunodeficiency
Journal of clinical immunology, 2013Co-Authors: Ivan J. Fuss, Julia C. Friend, Zhiqiong Yang, Lubna Hooda, James L. Boyer, Mark Raffeld, David E. Kleiner, Theo HellerAbstract:Purpose Patients with Common Variable Immunodeficiency (CVID) are subject to the development of a liver disease syndrome known as Nodular Regenerative Hyperplasia (NRH). The purpose of this study was to define the characteristics and course of this complication of CVID.
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Nodular Regenerative Hyperplasia and severe portal hypertension in cystinosis
Clinical Gastroenterology and Hepatology, 2006Co-Authors: Kevin Obrien, David E. Kleiner, Theo Heller, Nadeem Hussain, Bradley A Warady, Robert Kleta, Isa Bernardini, William A GahlAbstract:Background & Aims: Cystinosis is a rare autosomal-recessive disorder characterized by the intralysosomal accumulation of cystine, which is responsible for widespread tissue destruction. Liver biopsy specimens of patients with cystinosis show cystine crystal formation in Kupffer cells. However, significant liver disease and portal hypertension is not a common complication of cystinosis. We report the case histories of 2 young men with poorly treated nephropathic cystinosis who developed noncirrhotic portal hypertension with evidence of Nodular Regenerative Hyperplasia (NRH). Methods: Liver biopsy examinations, upper and lower endoscopy with biopsy examination, imaging studies, venous pressure measurements, and laboratory investigations were used to evaluate the causes of the liver disease and portal hypertension. Results: Histologic examination of liver biopsy specimens from both patients showed changes characteristic of NRH with portal hypertension documented by measurement of pressure gradients. In addition, endoscopy in the first patient showed varices and portal hypertensive gastropathy. Conclusions: NRH was confirmed by histologic examination of the liver in both patients and is the likely cause of their portal hypertension. NRH may represent a rare, late complication of cystinosis, although the mechanism remains undefined.
Vincent Mallet - One of the best experts on this subject based on the ideXlab platform.
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acquired protein s deficiency leads to obliterative portal venopathy and to compensatory Nodular Regenerative Hyperplasia in hiv infected patients
AIDS, 2009Co-Authors: Vincent Mallet, Aditi Varthaman, Dominique Lasne, Jeanpaul Viard, Herve Gouya, Delphine Borgel, Sebastien LacroixdesmazesAbstract:OBJECTIVE: To identify the mechanism of Nodular Regenerative Hyperplasia in HIV-infected patients. DESIGN: Case-control study. SETTING: The hepatology and the infectious disease units of two tertiary care centers in France. PATIENTS: We compared 13 consecutive HIV-positive patients with unexplained Nodular Regenerative Hyperplasia to 16 consecutive HIV-positive patients without Nodular Regenerative Hyperplasia, to eight HIV-negative patients with Nodular Regenerative Hyperplasia from an identified cause and to 10 anonymous healthy blood donors. MAIN OUTCOME MEASURE: Patients and controls were screened for diminished protein S activity and antiprotein S immunoglobulin G (IgG) antibodies. The antiprotein S activity of purified IgG from patients and controls was assessed in a functional test of activation of protein C in which protein S serves as a cofactor. A full liver CT portography was realized on the liver explant of a case patient. RESULTS: The CT portography disclosed diffuse obliterative portal venopathy. Levels of protein S activity were lower among patients with HIV-associated Nodular Regenerative Hyperplasia when compared with HIV-positive patients without Nodular Regenerative Hyperplasia and when compared with HIV-negative patients with Nodular Regenerative Hyperplasia (P < 0.005 for all comparisons). HIV-positive patients with Nodular Regenerative Hyperplasia had significantly higher levels of antiprotein S IgG than HIV-positive patients without Nodular Regenerative Hyperplasia and healthy controls. Purified IgG from patients with HIV-associated Nodular Regenerative Hyperplasia specifically inhibited the protein S-dependent protein C activation. CONCLUSION: Acquired autoimmune protein S paucity and secondary thrombophilia appear to be causes of obliterative portal venopathy and compensatory Nodular Regenerative Hyperplasia in HIV-positive patients.
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a new indication for liver transplantation Nodular Regenerative Hyperplasia in human immunodeficiency virus infected patients
Liver Transplantation, 2008Co-Authors: Mariagrazia Tateo, Vincent Mallet, Mylene Sebagh, Mariepierre Bralet, Elina Teicher, Daniel Azoulay, Stanislas Pol, Denis CastaingAbstract:Nodular Regenerative Hyperplasia is one of the causes of noncirrhotic portal hypertension and has recently been described in human immunodeficiency virus-infected patients, and the potential role of a prothrombotic state and hepatotoxic antiretroviral medication has been suggested. Moreover, it is now established that liver transplantation is feasible in HIV-infected patients. We describe here our experience concerning 3 HIV-infected patients with severe complications of Nodular Regenerative Hyperplasia treated with liver transplantation.
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Nodular Regenerative Hyperplasia is a new cause of chronic liver disease in hiv infected patients
AIDS, 2007Co-Authors: Vincent Mallet, Virginie Verkarre, Pierre Blanchard, Anais Valletpichard, Helene Fontaine, Caroline Lascouxcombe, Stanislas PolAbstract:Objective: To describe and explain the syndrome of HIV-associated cryptogenic liver disease in eight consecutive patients suffering from portal hypertension. Methods: The study was undertaken at a liver disease centre in Paris and involved eight of 97 consecutive HIV-infected patients presenting abnormal liver function tests and/or symptomatic portal hypertension of unknown origin. Serology, pathology, and liver function tests were performed. Results: A clear Nodular architecture corresponding to Nodular Regenerative Hyperplasia was observed in seven patients and suggested in one, based on the presence of sinusoidal dilatation in a clinical context of portal hypertension, without overt liver disease. Conclusions: Nodular Regenerative Hyperplasia appears to be a new cause of portal hypertension in HIV-infected patients. This syndrome can be of critical importance as patients can be exposed to the significant complications of portal hypertension and to refractory ascites which may require liver transplantation.
Walter Reinisch - One of the best experts on this subject based on the ideXlab platform.
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Nodular Regenerative Hyperplasia in patients with inflammatory bowel disease treated with azathioprine.
Gut, 2007Co-Authors: G. Vernier-massouille, Walter Reinisch, Philippe Marteau, D. Laharie, J. Cosnes, M. Lemann, G. Cadiot, Y. Bouhnik, M. De Vos, A. BoureilleAbstract:AIM: To assess the characteristics and clinical course of Nodular Regenerative Hyperplasia (NRH) in patients with inflammatory bowel disease treated with azathioprine, so as to estimate the frequency of this complication and search for risk factors. METHODS: Cases were identified through a systematic survey of patients followed at 11 centres. At one centre, the cumulative risk of NRH was estimated and a case-control study was undertaken to identify risk factors. RESULTS: 37 cases of NRH (30 male, 7 female) were identified between 1994 and 2005. The median dose of azathioprine was 2 mg/kg/d (range 1.5 to 3.0). The median time between the start of azathioprine and the diagnosis of NRH was 48 months (range 6 to 187). After a median follow up period of 16 months (range 1 to 138), 14 patients developed complications of portal hypertension. Using multivariate analysis, male sex and stricturing behaviour were the two risk factors associated with NRH in patients treated with azathioprine. The cumulative risk calculated from the database (one centre) was 0.5% at 5 years (95% confidence interval, 0.11 to 0.89) and 1.25% at 10 years (0.29 to 2.21). CONCLUSIONS: NRH is a rare but potentially severe complication of azathioprine in patients with inflammatory bowel disease. Clinicians should be aware of this complication, and should monitor liver function tests and platelet counts closely in their patients.
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6 thioguanine associated Nodular Regenerative Hyperplasia in patients with inflammatory bowel disease may induce portal hypertension
The American Journal of Gastroenterology, 2007Co-Authors: A Ferlitsch, Alexander Teml, Walter Reinisch, G Ulbrich, F Wrba, Monika Homoncik, A Gangl, M Peckradosavljevic, Harald VogelsangAbstract:6-Thioguanine Associated Nodular Regenerative Hyperplasia in Patients With Inflammatory Bowel Disease May Induce Portal Hypertension
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thioguanin induced Nodular Regenerative Hyperplasia of the liver roc analysis of different mr techniques
European Radiology, 2007Co-Authors: Christoph J Zech, Walter Reinisch, F Wrba, Julia Seiderer, Joachim Diebold, Thomas Ochsenkuhn, Wolfgang Schima, Maximilian F Reiser, Stefan O SchoenbergAbstract:The aim of this study was to evaluate the diagnostic value of different magnetic resonance (MR) techniques for the diagnosis of Nodular Regenerative Hyperplasia (NRH). Thirty-one patients with inflammatory bowel disease, who received 6-thioguanin, underwent liver biopsy and liver MRI on a 1.5-T MR system, with gadolinium and superparamagnetic iron oxide particles (SPIO). MR imaging (MRI) was evaluated independently as well as in consensus by two blinded readers, who received the following image sets: pre-contrast; pre-contrast and gadolinium-enhanced; pre-contrast and SPIO-enhanced and all images. The results were correlated with histopathology and diagnostic efficacy parameters were calculated. NRH was found in 13/31 patients. The set "all images" showed the highest sensitivity (84.6%), accuracy (77.4%) and negative predictive value (86.7%). However, results for gadolinium were only slightly inferior. The highest specificity (76.5%) was found for SPIO. The A(z) values of both readers were highest for gadolinium (mean A(z) = 0.824). It can be concluded that gadolinium-enhanced and SPIO-enhanced MRI enable an accurate diagnosis of NRH. Since gadolinium-enhanced MRI is very sensitive, it should be used for screening high-risk patients. SPIO-enhanced MRI is less sensitive, but more specific. The combination of both guarantees a high sensitivity and specificity and, therefore, is the diagnostic procedure of choice.