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[PMID]:27778443
[Au] Autor:Krawczyk M; Liebe R; Wasilewicz M; Wunsch E; Raszeja-Wyszomirska J; Milkiewicz P
[Ad] Endereço:Laboratory of Metabolic Liver Diseases, Department of General, Transplant and Liver Surgery, Medical University of Warsaw, Warsaw, Poland.
[Ti] Título:Plasmapheresis exerts a long-lasting antipruritic effect in severe cholestatic itch.
[So] Source:Liver Int;37(5):743-747, 2017 May.
[Is] ISSN:1478-3231
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND & AIMS: The amelioration of refractory cholestatic pruritus after plasmapheresis has been reported in single patients. Here, we analyse the efficacy of plasmapheresis in a cohort of patients with primary biliary cholangitis (PBC). METHODS: Seventeen consecutive patients with PBC (age range 39-85 years, 16 females, 9 with cirrhosis) and refractory pruritus underwent 129 plasmapheresis procedures during 40 admissions. Pruritus was quantified by the 10-point numeric rating scale (NRS) before and after plasmapheresis, as well as ~30 and ~90 days later. RESULTS: The mean pruritus before plasmapheresis did not differ between patients with and without cirrhosis (P>.05). Cirrhotics presented, however, with significantly higher serum alanine aminotransferase (ALT), aspartate transaminase (AST), gamma-glutamyl transpeptidase (GGT), alkaline phosphatase (ALP) and bilirubin before plasmapheresis. Plasmapheresis decreased itching to NRS≤5 in all but five admissions: Mean pruritus decreased from 8.3±1.4 to 3.1±2.2 (P<.0001) in the entire cohort. It also led to a significant decrease in serum ALT, ALP, AST, GGT (all P<.001) and bilirubin (P=.002). Antipruritic effect persisted throughout the 90-days follow-up (P<.0001). The amelioration of pruritus was not affected by the presence of cirrhosis. CONCLUSIONS: Plasmapheresis is a promising method for reducing intractable itch in a significant proportion of PBC patients regardless of liver fibrosis. Long-lasting improvement of symptoms requires repeated procedures.
[Mh] Termos MeSH primário: Colestase/complicações
Cirrose Hepática Biliar/complicações
Plasmaferese
Prurido/terapia
[Mh] Termos MeSH secundário: Adulto
Idoso
Idoso de 80 Anos ou mais
Fosfatase Alcalina/sangue
Aspartato Aminotransferases/sangue
Bilirrubina/sangue
Feminino
Seres Humanos
Fígado/patologia
Masculino
Meia-Idade
Polônia
Prurido/sangue
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
EC 2.6.1.1 (Aspartate Aminotransferases); EC 3.1.3.1 (Alkaline Phosphatase); RFM9X3LJ49 (Bilirubin)
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180307
[Lr] Data última revisão:
180307
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161026
[St] Status:MEDLINE
[do] DOI:10.1111/liv.13281


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[PMID]:29465536
[Au] Autor:Yan X; Jin J
[Ad] Endereço:Department of Hepatology, The First Hospital of Jilin University, Changchun, Jilin, China.
[Ti] Título:Primary cutaneous amyloidosis associated with autoimmune hepatitis-primary biliary cirrhosis overlap syndrome and Sjögren syndrome: A case report.
[So] Source:Medicine (Baltimore);97(8):e0004, 2018 Feb.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Primary cutaneous amyloidosis (PCA) is a localized skin disorder characterized by the abnormal deposition of amyloid in the extracellular matrix of the dermis. The association between PCA and other diseases, although rare, has been documented for various autoimmune diseases. PCA associated with autoimmune hepatitis-primary biliary cirrhosis (AIH-PBC) overlap syndrome and Sjögren syndrome (SS) has not been previously reported in the literature. PATIENT CONCERNS: A 50-year-old woman presented with progressive abnormal liver enzyme levels and was referred to our department. DIAGNOSES: Due to the patient's symptoms, laboratory test results, radiographic findings, and pathologic results, she was diagnosed with PCA associated with AIH-PBC overlap syndrome and SS. INTERVENTIONS: She was subsequently treated with a combination of ursodeoxycholic acid (UDCA), prednisone, and azathioprine. OUTCOMES: While this treatment can achieve therapeutic success, it cannot prevent complications from cirrhosis. This patient remains alive but experienced an emergent gastrointestinal hemorrhage. LESSONS: While we acknowledge that this is a single case, these findings extend our knowledge of immunological diseases associated with PCA and suggest a common, immune-mediated pathogenic pathway between PCA, AIH-PBC overlap syndrome, and SS. After 12 years of follow up, clinical manifestations have developed, and these autoimmune diseases have progressed. The combination of UDCA, prednisone, and azathioprine can achieve therapeutic success but cannot prevent disease progression. Routine follow up for this patient is necessary to document disease progression.
[Mh] Termos MeSH primário: Amiloidose Familiar/imunologia
Hepatite Autoimune/complicações
Cirrose Hepática Biliar/complicações
Síndrome de Sjogren/complicações
Dermatopatias Genéticas/imunologia
Doenças do Tecido Conjuntivo Indiferenciado/complicações
[Mh] Termos MeSH secundário: Amiloidose Familiar/tratamento farmacológico
Anti-Inflamatórios/administração & dosagem
Azatioprina/administração & dosagem
Colagogos e Coleréticos/administração & dosagem
Quimioterapia Combinada
Feminino
Hepatite Autoimune/tratamento farmacológico
Hepatite Autoimune/imunologia
Seres Humanos
Imunossupressores/administração & dosagem
Cirrose Hepática Biliar/tratamento farmacológico
Cirrose Hepática Biliar/imunologia
Meia-Idade
Prednisona/administração & dosagem
Síndrome de Sjogren/tratamento farmacológico
Síndrome de Sjogren/imunologia
Dermatopatias Genéticas/tratamento farmacológico
Resultado do Tratamento
Doenças do Tecido Conjuntivo Indiferenciado/tratamento farmacológico
Doenças do Tecido Conjuntivo Indiferenciado/imunologia
Ácido Ursodesoxicólico/administração & dosagem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Anti-Inflammatory Agents); 0 (Cholagogues and Choleretics); 0 (Immunosuppressive Agents); 724L30Y2QR (Ursodeoxycholic Acid); MRK240IY2L (Azathioprine); VB0R961HZT (Prednisone)
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180302
[Lr] Data última revisão:
180302
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180222
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000010004


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[PMID]:29443746
[Au] Autor:Wang Z; Liu X; Xu H; Qu L; Zhang D; Gao P
[Ad] Endereço:Department of Hepatology.
[Ti] Título:Platelet count to spleen thickness ratio is related to histologic severity of primary biliary cholangitis.
[So] Source:Medicine (Baltimore);97(7):e9843, 2018 Feb.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The aim of this study was to evaluate the ability of noninvasive markers to identify the histological severity of primary biliary cholangitis (PBC).Fifty-eight treatment-naïve PBC patients who had undergone liver biopsy were enrolled in our study. The patients' histological stages were based on the classifications of Ludwig and Scheuer. Aspartate aminotransferase-to-platelet ratio index (APRI), fibrosis index based on the 4 factors (FIB-4), red blood cell distribution width to platelet ratio (RPR), and platelet count to spleen thickness (PC/ST) ratio were calculated. Using the area under the receiver operating characteristic curve (AUROC) to evaluate the accuracy of different markers for predicting the histological severity.Among the 58 treatment-naïve PBC patients, the patients of Scheuer stage I/II/III/IV were 17/25/11/5, respectively. PC/ST ratio (AUROC = 0.807) was superior to RPR (AUROC = 0.717), APRI (AUROC = 0.726), FIB-4 (AUROC = 0.722), and mean platelet volume (MPV) (AUROC = 0.671) in discriminating between stage I and stage ≥II. The AUROC of PC/ST ratio, RPR, APRI, FIB-4, and MPV were 0.939, 0.872, 0.816, 0.831 and 0.572, respectively, for Scheuer stage ≥III; 0.968, 0.795, 0.744, and 0.723, respectively for stage IV. The sensitivity and specificity of PC/ST ratio were 73.4%,79.1%; 81%,100%;88.7%,100% for detection of Scheuer stage ≥ II, Scheuer stage ≥ III and Scheuer stage IV, respectively.Our study findings indicated that compared with previous noninvasive test PRP, APRI, FIB-4 and MPV, PC/ST ratio shows the most accurate for distinguish the histologic severity of PBC patients.
[Mh] Termos MeSH primário: Colangite/sangue
Cirrose Hepática Biliar/sangue
Contagem de Plaquetas
Índice de Gravidade de Doença
Baço/patologia
[Mh] Termos MeSH secundário: Área Sob a Curva
Aspartato Aminotransferases/sangue
Biomarcadores/análise
Colangite/patologia
Índices de Eritrócitos
Feminino
Seres Humanos
Cirrose Hepática Biliar/patologia
Masculino
Meia-Idade
Valor Preditivo dos Testes
Curva ROC
Estudos Retrospectivos
Sensibilidade e Especificidade
[Pt] Tipo de publicação:EVALUATION STUDIES; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers); EC 2.6.1.1 (Aspartate Aminotransferases)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180222
[Lr] Data última revisão:
180222
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180215
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009843


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[PMID]:29366780
[Au] Autor:Lin YC; Wang FS; Yang YL; Chuang YT; Huang YH
[Ad] Endereço:Department of Pediatrics, Chiayi Chang Gung Memorial Hospital, Taiwan.
[Ti] Título:MicroRNA-29a mitigation of toll-like receptor 2 and 4 signaling and alleviation of obstructive jaundice-induced fibrosis in mice.
[So] Source:Biochem Biophys Res Commun;496(3):880-886, 2018 02 12.
[Is] ISSN:1090-2104
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Cholestasis and hepatitis can cause continuous liver damage that may ultimately result in liver fibrosis. In a previous study, we demonstrated that microRNA-29a (miR-29a) protects against liver fibrosis. Toll-like receptor 2 (TLR2) and TLR4 are pattern recognition receptors of bacterial lipoprotein and lipopolysaccharide, both of which participate in activating hepatic stellate cells and liver fibrosis. The purpose of this study is to characterize the biological influence of miR-29a on TLR2 and TLR4 signaling in livers injured with bile duct ligation (BDL). We performed BDL on both miR-29a transgenic mice (miR-29aTg) and wild-type mice to induce cholestatic liver injury. Primary HSCs were transfected with a miR-29a mimic and inhibitor. In the wild-type mice, the BDL demonstrated significant α-smooth muscle actin fibrotic matrix formation and hepatic high mobility group box-1 expression. However, in the miR-29aTg mice, these factors were significantly reduced. Furthermore, miR-29a overexpression reduced the BDL exaggeration of TLR2, TLR4, MyD88, bromodomain-containing protein 4 (BRD4), phospho-p65 as well as proinflammatory cytokines, IL-1ß, MCP-1, TGF-ß, and TNF-α. In vitro, miR-29a mimic transfection reduced α-SMA, BRD4,TLR2, and TLR4 expressions in HSCs. This study provides new molecular insight into the ability of miR-29a to inhibit TLR2 and TLR4 signaling, which thus slows the progression of cholestatic liver deterioration.
[Mh] Termos MeSH primário: Icterícia Obstrutiva/metabolismo
Cirrose Hepática Biliar/metabolismo
MicroRNAs/metabolismo
Receptor 2 Toll-Like/metabolismo
Receptor 4 Toll-Like/metabolismo
[Mh] Termos MeSH secundário: Animais
Citocinas/metabolismo
Icterícia Obstrutiva/complicações
Icterícia Obstrutiva/patologia
Cirrose Hepática Biliar/complicações
Cirrose Hepática Biliar/patologia
Masculino
Camundongos
Camundongos Endogâmicos C57BL
Camundongos Transgênicos
Transdução de Sinais
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Cytokines); 0 (MIRN29 microRNA, mouse); 0 (MicroRNAs); 0 (Tlr2 protein, mouse); 0 (Tlr4 protein, mouse); 0 (Toll-Like Receptor 2); 0 (Toll-Like Receptor 4)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180222
[Lr] Data última revisão:
180222
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180126
[St] Status:MEDLINE


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[PMID]:29172353
[Au] Autor:Class-Vázquez W; Olivero-Rivera S; Rosa-Cruz S; Laboy-Olivieri C; Toro DH
[Ti] Título:The Perils of Diagnosing and Treating Overlap Syndrome.
[So] Source:Bol Asoc Med P R;108(2):57-9, 2016.
[Is] ISSN:0004-4849
[Cp] País de publicação:Puerto Rico
[La] Idioma:eng
[Ab] Resumo:Autoimmune hepatitis (AIH) may present with clinical, laboratory abnormalities and histological features suggestive of primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). These variants of AIH are known as overlap syndromes. We present a case of a 62 year-old-male who presented with altered liver function tests, anemia and unintentional weight loss. Initial laboratories revealed anemia and a cholestatic pattern. Diagnostic work-up was remarkable for a positive antinuclear antibodies (ANA) test and a liver biopsy suggestive of an AIH-PBC overlap syndrome. This case illustrates the complexity of establishing the diagnosis and effective therapy in this condition.
[Mh] Termos MeSH primário: Hepatite Autoimune/diagnóstico
Cirrose Hepática Biliar/diagnóstico
Doenças do Tecido Conjuntivo Indiferenciado/diagnóstico
[Mh] Termos MeSH secundário: Anticorpos Antinucleares/imunologia
Biópsia
Hepatite Autoimune/imunologia
Seres Humanos
Cirrose Hepática Biliar/imunologia
Masculino
Meia-Idade
Doenças do Tecido Conjuntivo Indiferenciado/imunologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antibodies, Antinuclear)
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171214
[Lr] Data última revisão:
171214
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171128
[St] Status:MEDLINE


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[PMID]:28974454
[Au] Autor:Abd El Motteleb DM; Ibrahim IAAE; Elshazly SM
[Ad] Endereço:Clinical Pharmacology Department, Faculty of Medicine, Zagazig University, Egypt.
[Ti] Título:Sildenafil protects against bile duct ligation induced hepatic fibrosis in rats: Potential role for silent information regulator 1 (SIRT1).
[So] Source:Toxicol Appl Pharmacol;335:64-71, 2017 Nov 15.
[Is] ISSN:1096-0333
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Hepatic fibrosis is a potential health problem that may end with life-threatening cirrhosis and primary liver cancer. Recent studies point out to the protective effects of silent information regulator1 (SIRT1), against different models of organs fibrosis. This work aimed to investigate the possible protective effect of sildenafil (SIRT1 activator) against hepatic fibrosis induced by bile duct ligation (BDL). Firstly, three different doses of sildenafil (5, 10, 20mg/kg/day) were investigated; to detect the most protective one against BDL induced liver dysfunction and hepatic fibrosis. The most protective dose is then used; to study its effect on BDL induced SIRT1 downregulation, imbalance of oxidant/antioxidant status, increased inflammatory cytokines and fibrosis. Sildenafil (20mg/kg/day) was the most protective one, it caused upregulation of SIRT1, reduction of hepatic malondialdehyde (MDA) content, increase in expression of nuclear factor erythroid 2-related factor 2 (Nrf2), hemeoxygenease (HO)-1, reduced glutathione (GSH) content and superoxide dismutase (SOD) activity. Hepatic content of tumor necrosis factor-α (TNF-α) and nuclear factor κB (NFκB) expression & content displayed significant reductions with sildenafil treatment, Furthermore, sildenafil caused marked reductions of transforming growth factor (TGF)-ß content, expression of plasminogen activator inhibitor-1 (PAI-1), matrix metalloproteinase-9 (MMP-9), tissue inhibitor of metalloproteinase-1 (TIMP-1), α-smooth muscle actin (α-SMA), fibronectin, collagen I (α1) and hydroxyproline content. However, sildenafil protective effects were significantly reduced by co-administration of EX527 (SIRT1 inhibitor). Our work showed, for the first time that, sildenafil has promising protective effects against BDL induced liver dysfunction and hepatic fibrosis. These effects may be, in part, mediated by up regulation of SIRT1.
[Mh] Termos MeSH primário: Colestase/tratamento farmacológico
Cirrose Hepática Biliar/prevenção & controle
Fígado/efeitos dos fármacos
Substâncias Protetoras/farmacologia
Citrato de Sildenafila/farmacologia
Sirtuína 1/metabolismo
[Mh] Termos MeSH secundário: Animais
Anti-Inflamatórios/farmacologia
Antioxidantes/farmacologia
Biomarcadores/metabolismo
Carbazóis/farmacologia
Colestase/complicações
Colestase/enzimologia
Colestase/patologia
Citoproteção
Modelos Animais de Doenças
Inibidores de Histona Desacetilases/farmacologia
Mediadores da Inflamação/metabolismo
Ligadura
Fígado/enzimologia
Fígado/patologia
Cirrose Hepática Biliar/enzimologia
Cirrose Hepática Biliar/etiologia
Cirrose Hepática Biliar/patologia
Masculino
Estresse Oxidativo/efeitos dos fármacos
Ratos Wistar
Transdução de Sinais/efeitos dos fármacos
Sirtuína 1/antagonistas & inibidores
Regulação para Cima
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (6-chloro-2,3,4,9-tetrahydro-1H-carbazole-1-carboxamide); 0 (Anti-Inflammatory Agents); 0 (Antioxidants); 0 (Biomarkers); 0 (Carbazoles); 0 (Histone Deacetylase Inhibitors); 0 (Inflammation Mediators); 0 (Protective Agents); BW9B0ZE037 (Sildenafil Citrate); EC 3.5.1.- (Sirt1 protein, rat); EC 3.5.1.- (Sirtuin 1)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171025
[Lr] Data última revisão:
171025
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171005
[St] Status:MEDLINE


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[PMID]:28858131
[Au] Autor:Morioka H; Iguchi M; Kuzuya T; Mikamo H; Yagi T
[Ad] Endereço:aDepartment of Infectious Diseases, Nagoya University Hospital bDepartment of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya, Aichi cDepartment of Clinical Infectious Diseases dDepartment of Infection Control and Prevention, Aichi Medical University Hospital, Nagakute, Aichi, Japan.
[Ti] Título:Recurrent bacteremia and liver abscess caused by Clostridium difficile: A case report.
[So] Source:Medicine (Baltimore);96(35):e7969, 2017 Sep.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Clostridium difficile bacteremia (CDB) and liver abscess is a quite rare presentation of C. difficile infection. PATIENTS CONCERNS: A 74-year-old male with primary biliary cirrhosis and hepatocellular carcinoma who underwent transarterial chemoembolization (TACE) developed a high fever on post-TACE day 14. Intravenous ceftriaxone and following meropenem were administered, however, his clinical response was poor. On post-TACE day 24, 2 sets of blood culture were taken due to elevation of C-reactive protein levels. DIAGNOSIS: CDB, caused by bacterial translocation. INTERVENTIONS: Intravenous vancomycin and oral metronidazole were administered for two weeks. OUTCOMES: One month after recurrent CDB, the patient was re-admitted due to a liver abscess at the same site of TACE. C. difficile was isolated from the liver abscess and the patient received 6 weeks of oral metronidazole treatment. CDB and liver abscess have not recurred since completion of antibiotic treatment. LESSONS: The spore-forming ability of C. difficile may contributed to the recurrent CDB episodes and liver abscess formation in necrotic liver tissue following TACE, and long-term metronidazole therapy was considered to be effective to C. difficile liver abscess.
[Mh] Termos MeSH primário: Bacteriemia/microbiologia
Translocação Bacteriana
Infecções por Clostridium/microbiologia
Clostridium difficile/fisiologia
Abscesso Hepático/microbiologia
[Mh] Termos MeSH secundário: Idoso
Antibacterianos/uso terapêutico
Bacteriemia/tratamento farmacológico
Carcinoma Hepatocelular/tratamento farmacológico
Quimioembolização Terapêutica/efeitos adversos
Infecções por Clostridium/tratamento farmacológico
Seres Humanos
Abscesso Hepático/tratamento farmacológico
Cirrose Hepática Biliar/tratamento farmacológico
Neoplasias Hepáticas/tratamento farmacológico
Masculino
Metronidazol/uso terapêutico
Recidiva
Vancomicina/uso terapêutico
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Anti-Bacterial Agents); 140QMO216E (Metronidazole); 6Q205EH1VU (Vancomycin)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170918
[Lr] Data última revisão:
170918
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170901
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000007969


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[PMID]:28836457
[Au] Autor:Silveira MG; Lindor KD
[Ad] Endereço:a Section of Digestive Diseases , Yale School of Medicine , New Haven , CT , USA.
[Ti] Título:Investigational drugs in phase II clinical trials for primary biliary cholangitis.
[So] Source:Expert Opin Investig Drugs;26(10):1115-1121, 2017 Oct.
[Is] ISSN:1744-7658
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease that may lead to biliary fibrosis, and eventually cirrhosis. The primary treatment for PBC is ursodeoxycholic acid (UDCA), which has favorably altered its natural history. However, up to 40% of patients have an inadequate response to UDCA, and are therefore at high risk of liver-related complications. Obeticholic acid has recently been approved for use in patients with PBC with inadequate response or who are intolerant to UDCA, but improvement in long-term outcomes has not yet been demonstrated. Alternative therapeutic options for PBC are needed. Areas covered: Recent advances in research including epidemiological, genetic and pre-clinical studies in animal models of PBC have yielded numerous agents currently at different stages of development for treatment of patients with PBC; in this review, we cover novel therapies that were recently or are recently being investigated in phase II clinical trials. Expert opinion: Despite the evolving landscape in PBC, the main challenges facing development of novel therapies remain the rarity of the disease and the limitations to design and conduct of controlled clinical trials in PBC, which are needed to determine the long-term effects of novel therapies on the clinical outcomes of PBC.
[Mh] Termos MeSH primário: Colangite/tratamento farmacológico
Desenho de Drogas
Drogas em Investigação/uso terapêutico
[Mh] Termos MeSH secundário: Animais
Ácido Quenodesoxicólico/análogos & derivados
Ácido Quenodesoxicólico/uso terapêutico
Colagogos e Coleréticos/uso terapêutico
Colangite/complicações
Progressão da Doença
Seres Humanos
Cirrose Hepática Biliar/tratamento farmacológico
Ácido Ursodesoxicólico/uso terapêutico
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Cholagogues and Choleretics); 0 (Drugs, Investigational); 0462Z4S4OZ (obeticholic acid); 0GEI24LG0J (Chenodeoxycholic Acid); 724L30Y2QR (Ursodeoxycholic Acid)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170922
[Lr] Data última revisão:
170922
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170825
[St] Status:MEDLINE
[do] DOI:10.1080/13543784.2017.1371135


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[PMID]:28570655
[Au] Autor:Li Z; Lin B; Lin G; Wu Y; Jie Y; Li X; Ko B; Chong Y; Luo J
[Ad] Endereço:Department of Infectious Diseases, Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China.
[Ti] Título:Circulating FGF19 closely correlates with bile acid synthesis and cholestasis in patients with primary biliary cirrhosis.
[So] Source:PLoS One;12(6):e0178580, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND AND AIM: Bile acid (BA) synthesis in the liver is regulated by Fibroblast Growth Factor 19 (FGF19) secreted from the ileum as an enterohepatic feedback mechanism. Although FGF19 mRNA is absent in normal liver, FGF19 gene expression was reported to increase in response to both extrahepatic and intrahepatic cholestasis. The impact of upregulated FGF19 expression on BA synthesis is unclear and the overall role of circulating FGF19 and BA synthesis under cholestatic conditions needs to be further investigated. METHODS: BA synthesis was directly quantified by measuring serum concentrations of 7alpha-hydroxycholest-4-en-3-one (C4), along with serum FGF19 and other parameters, in 44 patients with primary biliary cirrhosis (PBC) and 10 healthy subjects. RESULTS: Serum C4 were substantially lower, while those of FGF19 were higher, in cirrhotic PBC patients, as compared to those of either healthy or non-cirrhotic PBC patients. Analyses of the relationships between circulating FGF19, BA synthesis and cholestasis revealed that circulating FGF19 was strongly correlated with BA synthesis (r = -0.735, p<0.0001) and the severity of cholestasis (r = 0.590, p<0.001). Moreover, BA synthesis was found to be strongly correlated with the degree of cholestasis (r = 0.522, p = 0.0005). CONCLUSION: These findings demonstrate that the regulation of BA synthesis in response to cholestasis is primarily controlled by circulating FGF19 and that under cholestatic conditions, the FGF19-BA synthesis feedback mechanism remains intact. Administering FGF19, or suitable mimetic, as a pharmacological intervention to increase circulating levels of FGF19 and suppress BA synthesis by inhibiting CYP7A1 gene expression is likely to provide therapeutic benefits for many PBC patients.
[Mh] Termos MeSH primário: Ácidos e Sais Biliares/biossíntese
Colestase/complicações
Fatores de Crescimento de Fibroblastos/sangue
Cirrose Hepática Biliar/sangue
[Mh] Termos MeSH secundário: Colestase/fisiopatologia
Feminino
Seres Humanos
Cirrose Hepática Biliar/complicações
Cirrose Hepática Biliar/fisiopatologia
Testes de Função Hepática
Masculino
Meia-Idade
Prognóstico
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Bile Acids and Salts); 0 (FGF19 protein, human); 62031-54-3 (Fibroblast Growth Factors)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170926
[Lr] Data última revisão:
170926
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170602
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0178580


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[PMID]:28425413
[Au] Autor:Melchor-Mendoza YK; Martínez-Benítez B; Mina-Hawat A; Rodríguez-Leal G; Duque X; Moran-Villota S
[Ad] Endereço:Social Service and Department of Pathology, National Institute of Medical Sciences and Nutrition Salvador Zubirán, Mexico City.
[Ti] Título:Ursodeoxycholic Acid Therapy in Patients with Primary Biliary Cholangitis with Limited Liver Transplantation Availability.
[So] Source:Ann Hepatol;16(3):430-435, 2017 May - Jun.
[Is] ISSN:1665-2681
[Cp] País de publicação:Mexico
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: There is little information on survival rates of patients with primary biliary cholangtis (PBC) in developing countries. This is particularly true in Latin America, where the number of liver transplants performed remains extremely low for patients with advanced liver disease who fulfill criteria for liver transplantation. The goal of this study was to compare survival rate of patients with PBC in developing countries who were treated with ursodeoxycholic acid (UDCA) versus survival of patients who received other treatments (OT) without UDCA, prescribed before the UDCA era. MATERIAL AND METHODS: A retrospective study was performed, including records of 78 patients with PBC in the liver unit in a third level referral hospital in Mexico City. Patients were followed for five years from initial diagnosis until death related to liver disease or to the end of the study. Patients received UDCA (15 mg/kg/per day) (n = 41) or OT (n = 37) before introduction of UDCA in Mexico. RESULTS: Response to treatment was higher in the group that received UDCA. In the five years of follow-up, survival rates were significantly higher in the UDCA group than in the OT group. The hazard ratio of death was higher in the OT group vs. UDCA group, HR 8.78 (95% CI, 2.52-30.61); Mayo Risk Score and gender were independently associated with the risk of death. CONCLUSIONS: The study confirms that the use of UDCA in countries with a limited liver transplant program increases survival in comparison to other treatments used before the introduction of UDCA.
[Mh] Termos MeSH primário: Colagogos e Coleréticos/uso terapêutico
Colangite/tratamento farmacológico
Acesso aos Serviços de Saúde
Cirrose Hepática Biliar/tratamento farmacológico
Transplante de Fígado
Doadores de Tecidos/provisão & distribuição
Ácido Ursodesoxicólico/uso terapêutico
[Mh] Termos MeSH secundário: Adulto
Colangite/diagnóstico
Colangite/mortalidade
Feminino
Seres Humanos
Estimativa de Kaplan-Meier
Cirrose Hepática Biliar/diagnóstico
Cirrose Hepática Biliar/mortalidade
Masculino
México
Meia-Idade
Modelos de Riscos Proporcionais
Estudos Retrospectivos
Fatores de Risco
Taxa de Sobrevida
Fatores de Tempo
Resultado do Tratamento
[Pt] Tipo de publicação:COMPARATIVE STUDY; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Cholagogues and Choleretics); 724L30Y2QR (Ursodeoxycholic Acid)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171023
[Lr] Data última revisão:
171023
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170421
[St] Status:MEDLINE
[do] DOI:10.5604/16652681.1235486



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