Nodular regenerative hyperplasia rarely leads to liver transplantation: A 20-year cohort study in all Dutch liver transplant units.
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ABSTRACT:
SUBMITTER: Meijer B
PROVIDER: S-EPMC5548351 | biostudies-other | 2017 Aug
REPOSITORIES: biostudies-other
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Project description: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. METHODS:CVID patients were evaluated by retrospective and prospective clinical course review. Liver biopsy specimens were evaluated for evidence of NRH and studied via RT-PCR for cytokine analysis. RESULTS:NRH in our CVID patient population occurred in approximately 5 % of the 261 patients in our total CVID study group, initially presenting in most cases with an elevated alkaline phosphatase level. While in some patients the disease remained static, in a larger proportion a more severe disease developed characterized by portal hypertension, the latter leading to hypersplenism with neutropenia and thrombocytopenia and, in some cases, to ascites. In addition, a substantial proportion of patients either developed or presented initially with an autoimmune hepatitis-like (AIH-like) liver disease that resulted in severe liver dysfunction and, in most cases to death due to infections. The liver histologic findings in these AIH-like patients were characterized by underlying NRH pattern with superimposed interface hepatitis, lymphocytic infiltration and fibrosis. Immunologic studies of biopsies of NRH patients demonstrated the presence of infiltrating T cells producing IFN-?, suggesting that the NRH is due to an autoimmune process. CONCLUSION:Overall, these studies provide evidence that NRH may not be benign but, can be a severe and potentially fatal disease complication of CVID that merits close monitoring and intervention.
| S-EPMC3731765 | biostudies-literature
Project description:Nodular regenerative hyperplasia (NRH) is associated with high morbidity and mortality in patients with common variable immunodeficiency (CVID). While liver biopsy is the gold standard for NRH diagnosis, a non-invasive technique could facilitate early disease recognition, monitoring, and/or immune intervention. We performed a cross-sectional analysis of ultrasound-based transient elastography (TE) in patients with CVID to evaluate liver stiffness and compared this between patients with (N = 12) and without (N = 6) biopsy-proven NRH. Additionally, these data were compared to a cohort followed at our institution for non-alcoholic fatty liver disease (NAFLD) (N = 527), a disease for which TE has routine diagnostic use. Clinical and pathologic features of NRH were evaluated as correlates of liver stiffness, and receiver operating characteristic curves were used to define a liver stiffness cutoff with diagnostic utility for NRH among CVID patients. CVID patients with NRH had a more severe disease presentation compared to those without. This included increased autoinflammatory disease comorbidities, combined B-cell and T-cell dysfunction, and abnormal liver biochemistries (specifically an increased mean alkaline phosphatase level [proximal to TE, 250 vs. 100 U/L; p = 0.03; peak, 314 vs. 114 U/L; p = 0.02). Results of TE demonstrated a significantly elevated liver stiffness in CVID patients with NRH (mean 13.2 ± 6.2 kPa) as compared to both CVID patients without NRH (mean 4.6 ± 0.9 kPa) and non-CVID patients with NAFLD (mean 6.9 ± 5.5 kPa) (p < 0.01). No single or composite histopathologic feature of NRH correlated with liver stiffness including nodule size, nodule density, sinusoidal dilation, fibrosis, and/or lymphocytosis. In contrast, liver stiffness by TE was significantly correlated with clinical parameters of portal hypertension, including an elevated hepatic venous pressure gradient, an increased splenic longitudinal diameter, presence of varices, and presence of peripheral edema. A liver stiffness of greater than or equal to 6.2 kPa was a clinically significant cutoff for NRH in CVID patients. We propose that TE has diagnostic utility in CVID, particularly in the presence of immunophenotypic features such as combined B-cell and T-cell dysfunction, autoinflammatory comorbidities, and/or abnormal liver tests. Elevated liver stiffness by TE should raise suspicion for NRH in patients with CVID and prompt expedited evaluation by hepatology.
| S-EPMC9121126 | biostudies-literature
Project description:Focal nodular hyperplasia (FNH) is a benign condition of the liver that is often discovered incidentally on radiological investigation. FNH has no malignant potential, is rarely symptomatic and surgical intervention is almost never required. However, eight patients with a diagnosis of FNH associated with upper abdominal pain or rapid growth were referred for surgery. All patients had been extensively investigated for other causes of pain and had been observed for between 1 and 7 years prior to surgical referral. The FNH lesions were between 1 cm and 8 cm in diameter. One FNH lesion 7.5 cm in diameter lay in segment VII/VIII and was related to the right and middle hepatic veins. All patients were resected with immediate and lasting control of their symptoms. Based on this experience FNH should be managed in a manner similar to haemangiomas with most lesions being safe to observe. However, it should be recognized that symptomatic FNH does occur, as well as FNH behaving in an unusual fashion such as rapid growth. Both of these findings are indications for resection.
| S-EPMC2043107 | biostudies-other
Project description:IntroductionNodular hyperplasia of parathyroid glands (PG) is the most probable cause of medical treatment failure in secondary hyperparathyroidism (sHPT). This prospective cohort study is located at the interface of medical and surgical consideration of sHPT treatment options and identifies risk-factors for nodular hyperplasia of PG.Material and methodsOne-hundred-eight resected PG of 27 patients with a broad spectrum of sHPT severity were classified according to the degree of hyperplasia by histopathology. Twenty routinely gathered parameters from medical history, ultrasound findings of PG and laboratory results were analyzed for their influence on nodular hyperplasia of PG by risk-adjusted multivariable binary regression. A prognostic model for non-invasive assessment of PG was developed and used to weight the individual impact of identified risk-factors on the probability of nodular hyperplasia of single PG.ResultsIndependent risk-factors for nodular hyperplasia of single PG were duration of dialysis in years, PG volume in mm3 determined by ultrasound and serum level of parathyroid hormone in pg/mL. Multivariable analyses computed a model with an Area Under the Receiver Operative Curve of 0.857 (95%-CI:0.773-0.941) when predicting nodular hyperplasia of PG. Theoretical assessment of risk-factor interaction revealed that the duration of dialysis had the strongest influence on the probability of nodular hyperplasia of single PG.ConclusionsThe three identified risk-factors (duration of dialysis, PG volume determined by ultrasound and serum level of parathyroid hormone) can be easily gathered in daily routine and could be used to non-invasively assess the probability of nodular hyperplasia of PG. This assessment would benefit from periodically collected data sets of PG changes during the course of sHPT, so that the choice of medical or surgical sHPT treatment could be adjusted more to the naturally changing type of histological PG lesion on an individually adopted basis in the future.
| S-EPMC5645091 | biostudies-literature
Project description:Follicular nodular hyperplasia (FNH) is a common benign liver tumor for which conservative management is indicated. Surgical or interventional management is indicated in symptomatic cases. Transarterial embolization (TAE) has been extensively used to manage unresectable liver tumors. Sublimation describes a change of physical state from solid to gas. Hepatic tissue sublimation following TAE has so far not been reported in medical literature.A 30 year - old male patient presenting with pain to the upper abdomen due to a large FNH was managed with TAE. Routine radiographic control on post-intervention day one was within normal limits. Imaging due to right upper quadrant pain with fever and elevated inflammatory markers and liver enzymes on day two after TAE revealed a marked reduction of the FNH accompanied by the presence of a large volume of gas collection without signs of abscess formation. This change of state from solid to gas without sign of abscess formation within 2 days after TAE was described as hepatic tissue sublimation. The patient was managed conservatively and discharge 12 days after TAE.Tissue sublimation has hardly been reported in medical literature. This to the best of our knowledge is the first documented case of hepatic tissue sublimation following TAE.
| S-EPMC5540510 | biostudies-other
Project description:Previous work on agreement computation in sentence comprehension motivates a model in which the parser predicts the verb's number and engages in retrieval of the agreement controller only when it detects a mismatch between the prediction and the bottom-up input. It is the error-driven second stage of this process that is prone to similarity-based interference and can result in the illusory licensing of a subject-verb number agreement violation in the presence of a structurally irrelevant noun matching the number marking on the verb ('The bed by the lamps were…'), giving rise to an effect known as 'agreement attraction'. Here we ask to what extent the error-driven retrieval process underlying the illusory licensing alters the structural and thematic representation of the sentence. We use a novel dual-task paradigm that combines self-paced reading with a speeded forced choice task to investigate whether agreement attraction leads comprehenders to erroneously interpret the attractor as the thematic subject, which would indicate structural reanalysis. Participants read sentence fragments ('The bed by the lamp/lamps was/were undoubtedly quite') and completed the sentences by choosing between two adjectives ('comfortable'/'bright') which were either compatible with the subject's head noun or with the attractor. We found the expected agreement attraction profile in the self-paced reading data but the interpretive error occurs on only a small subset of attraction trials, suggesting that in agreement attraction agreement checking rarely matches the thematic relation. We propose that illusory licensing of an agreement violation often reflects a low-level rechecking process that is only concerned with number and does not have an impact on the structural representation of the sentence. Interestingly, this suggests that error-driven repair processes can result in a globally inconsistent final sentence representation with a persistent mismatch between the subject and the verb.
| S-EPMC6524724 | biostudies-literature
Project description:INTRODUCTION: Unlike malignant liver tumours, the indications for hepatic resection for benign disease are not well defined. This is particularly true for focal nodular hyperplasia (FNH). Here we summarize a single-centre experience of the diagnosis and management of FNH. MATERIALS AND METHODS: Using a prospectively collected database, a retrospective analysis of consecutive patients who were managed at our centre for FNH between January 1997 and December 2006 was performed. RESULTS: The cohort was divided into two groups of patients: those who were managed surgically (n=15) and those managed conservatively (n=37). There was no correlation between tumour size and number of lesions with oral contraceptive use (p=0.07 and 0.90, respectively) and pregnancy (p=0.45 and 0.60, respectively). However, tumour size (p=0.006) and number of lesions (p=0.02) were associated with the occurrence of pain in these patients. Pain was the commonest symptom of patients (13/15) who were managed surgically. All patients underwent radiological imaging before diagnosis. The sensitivities of ultrasound, CT scanning and MRI scanning in characterizing these lesions were 30%, 70% and 87%, respectively. There were no postoperative deaths and three postoperative complications that were successfully managed non-operatively. With a median follow-up of 24 months in the surgically treated group, one patient has developed recurrent symptoms of pain. CONCLUSION. In this series, there was no mortality directly due to the surgical procedure and a modest morbidity, justifying surgical resections in selected patients.
| S-EPMC2225515 | biostudies-other
Project description:BackgroundObserved long-term outcomes no longer reflect the survival trajectory facing pediatric liver transplant (LT) recipients today. We aimed to use national registry data and parametric models to project 20- and 30-year post-transplant outcomes for recently transplanted pediatric LT recipients.MethodsWe conducted a retrospective cohort study of 13,442 first-time pediatric (age <18) LT recipients using 1987 to 2018 Scientific Registry of Transplant Recipients data. We validated the proposed method (ie, to project long-term patient and graft survival using parametric survival models and short-term data) in 2 historic cohorts (1987-1996 and 1997-2006) and estimated long-term projections among patients transplanted between 2007 and 2018. Projections were stratified by raft type, recipient age, and indication for transplant.ResultsParsimonious parametric models with Weibull distribution can be applied to post-transplant data and used to project long-term outcomes for pediatric LT recipients beyond observed data. Projected 20-year patient survival for pediatric LT recipients transplanted in 2007 to 2018 was 84.0% (95% confidence interval 81.5-85.8), compared to observed 20-year survival of 72.8% and 63.6% among those transplanted in 1997 to 2006 and 1987 to 1996, respectively. Projected 30-year survival for pediatric LT recipients in 2007 to 2018 was 80.1% (75.2-82.7), compared to projected 30-year survival of 68.6% (66.1-70.9) in the 1997 to 2006 cohort and observed 30-year survival of 57.5% in the 1987 to 1996 cohort. Twenty- and 30-year patient and graft survival varied slightly by recipient age, graft type, and indication for transplant.ConclusionsProjected long-term outcomes for recently transplanted pediatric LT recipients are excellent, reflective of substantial improvements in medical care, and informative for physician-patient education and decision making in the current era.
| S-EPMC8573715 | biostudies-literature
Project description:Background: We conduct a study in developing and validating two radiomics-based models to preoperatively distinguish hepatic epithelioid angiomyolipoma (HEAML) from hepatic carcinoma (HCC) as well as focal nodular hyperplasia (FNH). Methods: Totally, preoperative contrast-enhanced computed tomography (CT) data of 170 patients and preoperative contrast-enhanced magnetic resonance imaging (MRI) data of 137 patients were enrolled in this study. Quantitative texture features and wavelet features were extracted from the regions of interest (ROIs) of each patient imaging data. Then two radiomics signatures were constructed based on CT and MRI radiomics features, respectively, using the random forest (RF) algorithm. By integrating radiomics signatures with clinical characteristics, two radiomics-based fusion models were established through multivariate linear regression and 10-fold cross-validation. Finally, two diagnostic nomograms were built to facilitate the clinical application of the fusion models. Results: The radiomics signatures based on the RF algorithm achieved the optimal predictive performance in both CT and MRI data. The area under the receiver operating characteristic curves (AUCs) reached 0.996, 0.879, 0.999, and 0.925 for the training as well as test cohort from CT and MRI data, respectively. Then, two fusion models simultaneously integrated clinical characteristics achieved average AUCs of 0.966 (CT data) and 0.971 (MRI data) with 10-fold cross-validation. Through decision curve analysis, the fusion models were proved to be excellent models to distinguish HEAML from HCC and FNH in comparison between the clinical models and radiomics signatures. Conclusions: Two radiomics-based models derived from CT and MRI images, respectively, performed well in distinguishing HEAML from HCC and FNH and might be potential diagnostic tools to formulate individualized treatment strategies.
| S-EPMC7573543 | biostudies-literature
Project description:AimTo identify clonality and genetic alterations in focal nodular hyperplasia (FNH) and the nodules derived from it.MethodsTwelve FNH lesions were examined. Twelve hepatocellular adenomas (HCAs) and 22 hepatocellular carcinomas (HCCs) were used as references. Nodules of different types were identified and isolated from FNH by microdissection. An X-chromosome inactivation assay was employed to describe their clonality status. Loss of heterozygosity (LOH) was detected, using 57 markers, for genetic alterations.ResultsNodules of altered hepatocytes (NAH), the putative precursors of HCA and HCC, were found in all the FNH lesions. Polyclonality was revealed in 10 FNH lesions from female patients, and LOH was not detected in any of the six FNH lesions examined, the results apparently showing their polyclonal nature. In contrast, monoclonality was demonstrated in all the eight HCAs and in four of the HCCs from females, and allelic imbalances were found in the HCAs (9/9) and HCCs (15/18), with chromosomal arms 11p, 13q and 17p affected in the former, and 6q, 8p, 11p, 16q and 17p affected in the latter lesions in high frequencies (> or = 30%). Monoclonality was revealed in 21 (40%) of the 52 microdissected NAH, but was not found in any of the five ordinary nodules. LOH was found in all of the 13 NAH tested, being highly frequent at six loci on 8p, 11p, 13q and 17p.ConclusionFNH, as a whole, is polyclonal, but some of the NAH lesions derived from it are already neoplastic and harbor similar allelic imbalances as HCAs.
| S-EPMC2754518 | biostudies-literature