Database : MEDLINE
Search on : Caroli and Disease [Words]
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[PMID]: 25726036
[Au] Autor:Lee JM; Ahn YH; Kang HG; Ha II; Lee K; Moon KC; Lee JH; Park YS; Cho YM; Bae JS; Kim NK; Park WY; Cheong HI
[Ad] Address:Department of Pediatrics, Seoul National University Children's Hospital, 101 Daehak-Ro, Jongno-Gu, Seoul, 110-744, Korea.
[Ti] Title:Nephronophthisis 13: implications of its association with Caroli disease and altered intracellular localization of WDR19 in the kidney.
[So] Source:Pediatr Nephrol;30(9):1451-8, 2015 Sep.
[Is] ISSN:1432-198X
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:BACKGROUND: Nephronophthisis 13 (NPHP 13) is associated with mutations in the WDR19 gene, which encodes for a protein in the intraflagellar transport complex. Herein, we describe six additional cases accompanied by Caroli syndrome or disease. METHODS: Targeted exome sequencing covering 96 ciliopathy-related genes was performed for 48 unrelated Korean patients with a clinical suspicion of NPHP. Mutations were confirmed by Sanger sequencing. We evaluated the expression of WDR19 in the biopsied kidney by immunohistochemistry in patients and controls. RESULTS: We detected three (3/48, 6.3 %) unrelated index cases with WDR19 mutations. One of the cases involved two siblings with the same mutation. Later, we detected an additional index case with a similar phenotype of kidney and liver involvement by Sanger sequencing of WDR19. The p.R1178Q mutation was common in all patients. All of the six affected patients from four families progressed to chronic kidney disease. Of note, all six patients had Caroli syndrome or disease. Immunohistochemistry for WDR19 showed localized expression along the luminal borders of the renal tubular epithelium in controls, whereas it showed diffuse cytoplasmic staining in the affected patients. CONCLUSIONS: Caroli disease is a major extra-renal phenotype associated with mutations in WDR19 in the Korean population. In this study, we visually validated the expression pattern of mutant WDR19 protein in the kidneys of NPHP 13 patients. More data are needed to identify the true frequency of p.R1178Q. Functional studies including transfection assay will provide solid grounds for the pathogenicity of each mutation.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1508
[Js] Journal subset:IM
[St] Status:In-Data-Review
[do] DOI:10.1007/s00467-015-3068-8

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[PMID]: 25130384
[Au] Autor:Dell'Amore A; Monteverde M; Martucci N; Davoli F; Caroli G; Pipitone E; Bini A; Stella F; Dell'Amore D; Casadio C; Rocco G
[Ad] Address:Division of Thoracic Surgery, S.Orsola Malpighi University Hospital, Bologna, Italy. Electronic address: dellamore76@libero.it....
[Ti] Title:Surgery for non-small cell lung cancer in younger patients: what are the differences?
[So] Source:Heart Lung Circ;24(1):62-8, 2015 Jan.
[Is] ISSN:1444-2892
[Cp] Country of publication:Australia
[La] Language:eng
[Ab] Abstract:BACKGROUND: Non-small cell lung cancer (NSCLC) in young adults is uncommon. The objective of this study was to evaluate the clinicopathological characteristics, outcomes and prognosis of people younger than 50 years old treated surgically for NSCLC. METHODS: A retrospective study was conducted using the institutional database of four thoracic surgery units to collect patients with NSCLC younger than 50 years who had undergone surgery. These patients were compared with older patients (>75-years) operated in the same institutions and in the same period. RESULTS: We identified 113 young patients and 347 older patients. Younger patients were more likely to be female, non-smokers, with fewer comorbidities. Younger patients were more likely to be symptomatic at the time of diagnosis. Risk factors for poor prognosis in younger patients were T-stage, and disease-free-interval less than 548 days. Kaplan-Meier analysis showed a lower five-year survival in older patients compared with the younger ones (66% vs 38%, p=0.001). CONCLUSIONS: In conclusion NSCLC in younger patients has some distinct clinicopathological characteristics. The overall-survival of young patients is better than in older patients. Young patients receive more complete and aggressive treatment that could explain better survival. Further prospective studies with larger patient populations are required, to clarify the biological and genetic variance of NSCLC in younger patients.
[Mh] MeSH terms primary: Carcinoma, Non-Small-Cell Lung/mortality
Carcinoma, Non-Small-Cell Lung/surgery
Lung Neoplasms/mortality
Lung Neoplasms/surgery
[Mh] MeSH terms secundary: Adult
Age Factors
Aged
Aged, 80 and over
Databases, Factual
Disease-Free Survival
Female
Humans
Male
Middle Aged
Retrospective Studies
Sex Factors
Survival Rate
[Pt] Publication type:COMPARATIVE STUDY; JOURNAL ARTICLE
[Em] Entry month:1508
[Js] Journal subset:IM
[Da] Date of entry for processing:141208
[St] Status:MEDLINE

  3 / 1239 MEDLINE  
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[PMID]: 25425976
[Au] Autor:Galea R; Cardillo MT; Caroli A; Marini MG; Sonnino C; Narducci ML; Biasucci LM
[Ti] Title:Inflammation and C-reactive protein in atrial fibrillation: cause or effect?
[So] Source:Tex Heart Inst J;41(5):461-8, 2014 Oct.
[Is] ISSN:1526-6702
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Atrial fibrillation is associated with substantial morbidity and mortality rates. The incompletely understood pathogenesis of this cardiac dysrhythmia makes it difficult to improve approaches to primary and secondary prevention. Evidence has accumulated in regard to a relationship between inflammation and atrial fibrillation. Investigators have correlated the dysrhythmia with myocarditis, pericardiotomy, and C-reactive protein levels, suggesting that inflammation causes atrial fibrillation or participates in its onset and continuation. Conversely, other investigators suggest that atrial fibrillation induces an inflammatory response. In this review, we summarize and critically discuss the nature and clinical role of inflammation and C-reactive protein in atrial fibrillation.
[Mh] MeSH terms primary: Atrial Fibrillation/blood
Atrial Fibrillation/etiology
C-Reactive Protein/metabolism
Myocarditis/complications
[Mh] MeSH terms secundary: Humans
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T; REVIEW
[Nm] Name of substance:9007-41-4 (C-Reactive Protein)
[Em] Entry month:1507
[Cu] Class update date: 150106
[Lr] Last revision date:150106
[Js] Journal subset:IM
[Da] Date of entry for processing:141126
[St] Status:MEDLINE
[do] DOI:10.14503/THIJ-13-3466

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[PMID]: 26034365
[Au] Autor:Lai YH; Duan WD; Yu Q; Ye S; Xiao NJ; Zhang DX; Huang ZQ; Yang ZY; Dong JH
[Ad] Address:Yan-Hua Lai, Wei-Dong Duan, Qiang Yu, Sheng Ye, Nian-Jun Xiao, Dong-Xin Zhang, Zhi-Qiang Huang, Jia-Hong Dong, Hospital and Institute of Hepatobiliary Surgery, Chinese PLA General Hospital, Beijing 100853, China....
[Ti] Title:Outcomes of liver transplantation for end-stage biliary disease: A comparative study with end-stage liver disease.
[So] Source:World J Gastroenterol;21(20):6296-303, 2015 May 28.
[Is] ISSN:2219-2840
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:AIM: To evaluate the outcomes of patients with end-stage biliary disease (ESBD) who underwent liver transplantation, to define the concept of ESBD, the criteria for patient selection and the optimal operation for decision-making. METHODS: Between June 2002 and June 2014, 43 patients with ESBD from two Chinese organ transplantation centres were evaluated for liver transplantation. The causes of liver disease were primary biliary cirrhosis (n = 8), cholelithiasis (n = 8), congenital biliary atresia (n = 2), graft-related cholangiopathy (n = 18), Caroli's disease (n = 2), iatrogenic bile duct injury (n = 2), primary sclerosing cholangitis (n = 1), intrahepatic bile duct paucity (n = 1) and Alagille's syndrome (n = 1). The patients with ESBD were compared with an end-stage liver disease (ESLD) case control group during the same period, and the potential prognostic values of multiple demographic and clinical variables were assessed. The examined variables included recipient age, sex, pre-transplant clinical status, pre-transplant laboratory values, operation condition and postoperative complications, as well as patient and allograft survival rates. Survival analysis was performed using Kaplan-Meier curves, and the rates were compared using log-rank tests. All variables identified by univariate analysis with P values < 0.100 were subjected to multivariate analysis. A Cox proportional hazard regression model was used to determine the effect of the study variables on outcomes in the study group. RESULTS: Patients in the ESBD group had lower model for end-stage liver disease (MELD)/paediatric end-stage liver disease (PELD) scores and a higher frequency of previous abdominal surgery compared to patients in the ESLD group (19.2 ± 6.6 vs 22.0 ± 6.5, P = 0.023 and 1.8 ± 1.3 vs 0.1 ± 0.2, P = 0.000). Moreover, the operation time and the time spent in intensive care were significantly higher in the ESBD group than in the ESLD group (527.4 ± 98.8 vs 443.0 ± 101.0, P = 0.000, and 12.74 ± 6.6 vs 10.0 ± 7.5, P = 0.000). The patient survival rate in the ESBD group was not significantly different from that of the ESBD group at 1, 3 and 5 years (ESBD: 90.7%, 88.4%, 79.4% vs ESLD: 84.9%, 80.92%, 79.0%, χ(2) = 0.194, P = 0.660). The graft-survival rates were also similar between the two groups at 1, 3 and 5 years (ESBD: 90.7%, 85.2%, 72.7% vs ESLD: 84.9%, 81.0%, 77.5%, χ(2) = 0.003, P = 0.958). Univariate analysis identified MELD/PELD score (HR = 1.213, 95%CI: 1.081-1.362, P = 0.001) and bleeding volume (HR = 0.103, 95%CI: 0.020-0.538, P = 0.007) as significant factors affecting the outcomes of patients in the ESBD group. However, multivariate analysis revealed that MELD/PELD score (HR = 1.132, 95%CI: 1.005-1.275, P = 0.041) was the only negative factor that was associated with short survival time. CONCLUSION: MELD/PELD criteria do not adequately measure the clinical characteristics and staging of ESBD. The allocation system based on MELD/PELD criteria should be re-evaluated for patients with ESBD.
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Em] Entry month:1506
[Cu] Class update date: 150604
[Lr] Last revision date:150604
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.3748/wjg.v21.i20.6296

  5 / 1239 MEDLINE  
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[PMID]: 26157755
[Au] Autor:Gu DH; Park MS; Jung CH; Yoo YJ; Cho JY; Lee YH; Seo YS; Yim HJ; Um SH; Ryu HS
[Ad] Address:Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea....
[Ti] Title:Caroli's disease misdiagnosed as intraductal papillary neoplasm of the bile duct.
[So] Source:Clin Mol Hepatol;21(2):175-9, 2015 Jun.
[Is] ISSN:2287-285X
[Cp] Country of publication:Korea (South)
[La] Language:eng
[Ab] Abstract:Caroli's disease is a rare autosomal-recessive disorder caused by malformation of the ductal plate during embryonic development. Although it is present at birth, Caroli's disease is typically not diagnosed until between the second and fourth decades of life, as it was in the present patient. Here we report a rare case of Caroli's disease limited to one liver segment, which was initially misdiagnosed as an intraductal papillary neoplasm of the bile duct. The asymptomatic patient was treated with liver segmentectomy.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1507
[Cu] Class update date: 150711
[Lr] Last revision date:150711
[Js] Journal subset:IM
[St] Status:In-Data-Review
[do] DOI:10.3350/cmh.2015.21.2.175

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[PMID]: 25437302
[Au] Autor:Caroli A; Prestia A; Wade S; Chen K; Ayutyanont N; Landau SM; Madison CM; Haense C; Herholz K; Reiman EM; Jagust WJ; Frisoni GB; Alzheimer's Disease Neuroimaging Initiative
[Ad] Address:*Medical Imaging Unit, Biomedical Engineering Department, IRCCS Istituto di Ricerche Farmacologiche Mario Negri, Bergamo †Laboratory of Epidemiology and Neuroimaging, IRCCS Fatebenefratelli, Brescia §Department of Decision Science, Bocconi University, Milan, Italy ‡Department of Engineering, University of Cambridge **Institute of Brain, Behaviour, and Mental Health University of Cambridge, Cambridge ∥Banner Alzheimer's Institute, Phoenix, AZ ¶Helen Wills Neuroscience Institute, University of California, Berkeley, CA #Hannover Medical School, Clinic for Nuclear Medicine, Hannover, Germany ††Departments of Internal Medicine and Psychiatry, University Hospitals and University of Geneva, Geneva, Switzerland.
[Ti] Title:Alzheimer Disease Biomarkers as Outcome Measures for Clinical Trials in MCI.
[So] Source:Alzheimer Dis Assoc Disord;29(2):101-9, 2015 Apr-Jun.
[Is] ISSN:1546-4156
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: The aim of this study was to compare the performance and power of the best-established diagnostic biological markers as outcome measures for clinical trials in patients with mild cognitive impairment (MCI). METHODS: Magnetic resonance imaging, F-18 fluorodeoxyglucose positron emission tomography markers, and Alzheimer's Disease Assessment Scale-cognitive subscale were compared in terms of effect size and statistical power over different follow-up periods in 2 MCI groups, selected from Alzheimer's Disease Neuroimaging Initiative data set based on cerebrospinal fluid (abnormal cerebrospinal fluid Aß1-42 concentration-ABETA+) or magnetic resonance imaging evidence of Alzheimer disease (positivity to hippocampal atrophy-HIPPO+). Biomarkers progression was modeled through mixed effect models. Scaled slope was chosen as measure of effect size. Biomarkers power was estimated using simulation algorithms. RESULTS: Seventy-four ABETA+ and 51 HIPPO+ MCI patients were included in the study. Imaging biomarkers of neurodegeneration, especially MR measurements, showed highest performance. For all biomarkers and both MCI groups, power increased with increasing follow-up time, irrespective of biomarker assessment frequency. CONCLUSION: These findings provide information about biomarker enrichment and outcome measurements that could be employed to reduce MCI patient samples and treatment duration in future clinical trials.
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, N.I.H., EXTRAMURAL; RESEARCH SUPPORT, NON-U.S. GOV'T; RESEARCH SUPPORT, U.S. GOV'T, NON-P.H.S.
[Em] Entry month:1505
[Cu] Class update date: 150521
[Lr] Last revision date:150521
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.1097/WAD.0000000000000071

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[PMID]: 25409057
[Au] Autor:Olano C; Mönkemüller K
[Ad] Address:Basil I. Hirschowitz Endoscopic Center of Excellence, Division of Gastroenterology and Hepatology, University of Alabama at Birmingham, Alabama, USA.
[Ti] Title:Novel ERCP technique using a pushing catheter as a "mini-overtube" to remove a migrated metal stent from the bile duct.
[So] Source:Endoscopy;46 Suppl 1 UCTN:E534-5, 2014.
[Is] ISSN:1438-8812
[Cp] Country of publication:Germany
[La] Language:eng
[Mh] MeSH terms primary: Cholangiopancreatography, Endoscopic Retrograde/methods
Device Removal/methods
Lithiasis/therapy
Liver Diseases/therapy
Prosthesis Failure
[Mh] MeSH terms secundary: Bile Ducts
Caroli Disease/complications
Caroli Disease/therapy
Cholangiopancreatography, Endoscopic Retrograde/instrumentation
Cholangitis, Sclerosing/complications
Cholangitis, Sclerosing/therapy
Device Removal/instrumentation
Female
Humans
Lithiasis/complications
Liver Diseases/complications
Middle Aged
Stents/adverse effects
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1507
[Js] Journal subset:IM
[Da] Date of entry for processing:141121
[St] Status:MEDLINE
[do] DOI:10.1055/s-0034-1377637

  8 / 1239 MEDLINE  
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[PMID]: 24621061
[Au] Autor:Mezzelani A; Landini M; Facchiano F; Raggi ME; Villa L; Molteni M; De Santis B; Brera C; Caroli AM; Milanesi L; Marabotti A
[Ti] Title:Environment, dysbiosis, immunity and sex-specific susceptibility: a translational hypothesis for regressive autism pathogenesis.
[So] Source:Nutr Neurosci;18(4):145-61, 2015 May.
[Is] ISSN:1476-8305
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BACKGROUND: Autism is an increasing neurodevelopmental disease that appears by 3 years of age, has genetic and/or environmental etiology, and often shows comorbid situations, such as gastrointestinal (GI) disorders. Autism has also a striking sex-bias, not fully genetically explainable. OBJECTIVE: Our goal was to explain how and in which predisposing conditions some compounds can impair neurodevelopment, why this occurs in the first years of age, and, primarily, why more in males than females. METHODS: We reviewed articles regarding the genetic and environmental etiology of autism and toxins effects on animal models selected from PubMed and databases about autism and toxicology. DISCUSSION: Our hypothesis proposes that in the first year of life, the decreasing of maternal immune protection and child immune-system immaturity create an immune vulnerability to infection diseases that, especially if treated with antibiotics, could facilitate dysbiosis and GI disorders. This condition triggers a vicious circle between immune system impairment and increasing dysbiosis that leads to leaky gut and neurochemical compounds and/or neurotoxic xenobiotics production and absorption. This alteration affects the 'gut-brain axis' communication that connects gut with central nervous system via immune system. Thus, metabolic pathways impaired in autistic children can be affected by genetic alterations or by environment-xenobiotics interference. In addition, in animal models many xenobiotics exert their neurotoxicity in a sex-dependent manner. CONCLUSIONS: We integrate fragmented and multi-disciplinary information in a unique hypothesis and first disclose a possible environmental origin for the imbalance of male:female distribution of autism, reinforcing the idea that exogenous factors are related to the recent rise of this disease.
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Em] Entry month:1504
[Cu] Class update date: 150711
[Lr] Last revision date:150711
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.1179/1476830513Y.0000000108

  9 / 1239 MEDLINE  
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[PMID]: 25966425
[Au] Autor:Plentz RR; Malek NP
[Ad] Address:Department of Internal Medicine I, Medical University Hospital, Otfried-Müller-Str. 10, 72076 Tübingen, Germany. Electronic address: Ruben.Plentz@med.uni-tuebingen.de.
[Ti] Title:Clinical presentation, risk factors and staging systems of cholangiocarcinoma.
[So] Source:Best Pract Res Clin Gastroenterol;29(2):245-52, 2015 Apr.
[Is] ISSN:1532-1916
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:Cholangiocarcinoma (CCA) is the second most common primary liver tumour. Intra-hepatic CCA develops within the liver parenchyma while extrahepatic CCA involves the biliary tree within the hepatoduodenal ligament. Hilar CCA are also called Klatskin tumour. The CCA incidence has increased worldwide over the last years, but there are also geographic differences, with focus in Asian countries. Known risk factors are primary sclerosing cholangitis (PSC), hepatolithiasis, Caroli's disease, hepatitis B and C infection, liver flukes, cirrhosis, diabetes, obesity, alcohol consumption and probably tobacco smoking. Patients with early CCA have only little discomfort, but can later show episodes with jaundice and other non-specific tumour symptoms. For the staging of the disease different classifications are available, which consider various factors like tumour size, location, regional lymph nodes, metastasis, vascular involvement and tumour marker.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1505
[Js] Journal subset:IM
[St] Status:In-Process

  10 / 1239 MEDLINE  
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[PMID]: 24730630
[Au] Autor:Viswanathan S; Kumar D
[Ad] Address:Department of Pediatrics, Rainbow Babies and Children's Hospital, University Hospitals, Cleveland, Ohio, USA.
[Ti] Title:Diagnostic challenge of large congenital liver cyst in the newborn.
[So] Source:Pediatr Int;56(2):267-70, 2014 Apr.
[Is] ISSN:1442-200X
[Cp] Country of publication:Australia
[La] Language:eng
[Ab] Abstract:Liver cysts in the newborn often pose significant diagnostic challenges. Described herein is a case of large congenital liver cyst that was difficult to diagnose both antenatally and postnatally and which was later diagnosed as Caroli disease.
[Mh] MeSH terms primary: Caroli Disease/diagnosis
Cysts/diagnosis
Liver Diseases/diagnosis
[Mh] MeSH terms secundary: Cysts/congenital
Diagnosis, Differential
Female
Humans
Infant, Newborn
Liver Diseases/congenital
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1507
[Js] Journal subset:IM
[Da] Date of entry for processing:140415
[St] Status:MEDLINE
[do] DOI:10.1111/ped.12260


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