Database : MEDLINE
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[PMID]: 25447106
[Au] Autor:Obusez EC; Udayasankar U
[Ad] Address:Imaging Institute, Cleveland Clinic Children's Hospital, Cleveland, Ohio.
[Ti] Title:Autosomal recessive polycystic kidney disease with caroli syndrome.
[So] Source:J Urol;193(2):679-80, 2015 Feb.
[Is] ISSN:1527-3792
[Cp] Country of publication:United States
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1501
[Js] Journal subset:AIM; IM
[St] Status:In-Data-Review

  2 / 1209 MEDLINE  
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[PMID]: 25467097
[Au] Autor:Forny DN; Ferrante SM; Silveira VG; Siviero I; Chagas VL; Méio IB
[Ad] Address:Federal University of Rio de Janeiro....
[Ti] Title:Choledochal cyst in childhood: review of 30 cases.
[So] Source:Rev Col Bras Cir;41(5):331-5, 2014 Sep-Oct.
[Is] ISSN:1809-4546
[Cp] Country of publication:Brazil
[La] Language:eng; por
[Ab] Abstract:OBJECTIVE: To analyze and discuss the clinical data, diagnosis and treatment of a number of patients with cystic dilatation of the common bile duct of a Brazilian pediatric hospital. METHODS: We analyzed 30 patients treated at the Martagão Gesteira Institute of Pediatrics and Child Care of the Federal University of Rio de Janeiro for 23 years ,with statistical analysis of epidemiological data, clinical manifestations, diagnosis, treatment and postoperative outcome. RESULTS: We observed a marked female predominance (73.4% of cases), the diagnosis being made in the first decade of life in 90% of patients. The most prevalent clinical manifestation was jaundice (70% of cases) and the classic triad of choledochal cyst was not observed. Abdominal ultrasound was the first imaging examination performed, with a sensitivity of 56.6%, with diagnostic definition in 17 children. Two patients (6.6%) had prenatal diagnosis. All patients underwent surgical treatment, cyst resection with Roux-en-Y hepaticojejunostomy being performed in 80% of cases. The incidence of postoperative complications was 13.3% and the mortality rate was 6.6%, ie two patients were diagnosed with Caroli's disease. CONCLUSION: The non-observance of the classic triad of choledochal cyst suggests that its incidence is lower than that reported in the medical literature. The surgical treatment of choledochal cysts, with resection and bilioenteric anastomosis, is safe even for small children.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1412
[Js] Journal subset:IM
[St] Status:In-Process

  3 / 1209 MEDLINE  
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[PMID]: 25058754
[Au] Autor:Knapp J; Sako Y; Grenouillet F; Bresson-Hadni S; Richou C; Gbaguidi-Haore H; Ito A; Millon L
[Ad] Address:Laboratory of Chrono-environnement, UMR/CNRS 6249, Faculty of Medicine and Pharmacy, Besançon, France - WHO Collaborating Centre for prevention and treatment of human echinococcosis, Besançon, France....
[Ti] Title:Comparison of the serological tests ICT and ELISA for the diagnosis of alveolar echinococcosis in France.
[So] Source:Parasite;21:34, 2014.
[Is] ISSN:1776-1042
[Cp] Country of publication:France
[La] Language:eng
[Ab] Abstract:Serological diagnosis of alveolar echinococcosis (AE) is a key element for efficient patient treatment management. A rapid immunochromatography test kit (ICT) using the recombinant Em18 antigen (rEm18) was recently developed. The aim of our study was to assess this test on a panel of sera from French patients with alveolar echinococcosis and control patients. In a blind test, a total of 112 serum samples were tested including samples of AE (n = 30), cystic echinococcosis [CE] (n = 15), and polycystic echinococcosis [PE] (n = 1). For the comparison, 66 sera from patients with hepatocarcinoma, fascioliasis, toxocariasis, Caroli's disease, or autoimmune chronic active hepatitis were used. The diagnostic test sets we used were the rEm18-ICT and two validated ELISAs with rEm18 and Em2-Em18 antigens, respectively. For the ICT, 27/30 sera from AE patients, 4/15 sera from CE patients and the PE patient serum were positive. One serum from the control panel (toxocariasis) was positive for the ICT. The rEm18-ICT sensitivity (90.0%) and specificity (92.7%) for detection of Em18-specific antibodies confirmed it as a relevant tool for AE diagnosis. The rEm18-ELISA had a sensitivity of 86.7% and specificity of 91.5%, and the Em2-Em18-ELISA had a sensitivity of 96.7% and specificity of 87.8%. However, when AE patient sera are recorded as weak in intensity with the ICT, we recommend a double reading and use of a reference sample if the ICT is used for patient follow-up.
[Mh] MeSH terms primary: Antibodies, Helminth/blood
Antigens, Helminth/immunology
Echinococcosis, Hepatic/diagnosis
Echinococcus multilocularis/immunology
Enzyme-Linked Immunosorbent Assay
Immunochromatography
[Mh] MeSH terms secundary: Animals
Antibodies, Helminth/immunology
Diagnosis, Differential
Echinococcosis, Hepatic/epidemiology
False Negative Reactions
False Positive Reactions
France/epidemiology
Humans
Liver Diseases/diagnosis
Neoplasms/diagnosis
Parasitic Diseases/diagnosis
Recombinant Proteins/immunology
Reproducibility of Results
Sensitivity and Specificity
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T; VALIDATION STUDIES
[Nm] Name of substance:0 (Antibodies, Helminth); 0 (Antigens, Helminth); 0 (Em18 antigen); 0 (Em2 antigen, Echinococcus multilocularis); 0 (Recombinant Proteins)
[Em] Entry month:1501
[Js] Journal subset:IM
[Da] Date of entry for processing:140725
[St] Status:MEDLINE
[do] DOI:10.1051/parasite/2014037

  4 / 1209 MEDLINE  
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[PMID]: 25548748
[Au] Autor:Jang MH; Lee YJ; Kim H
[Ad] Address:Department of Pathology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.
[Ti] Title:Intrahepatic cholangiocarcinoma arising in Caroli's disease.
[So] Source:Clin Mol Hepatol;20(4):402-5, 2014 Dec.
[Is] ISSN:2287-285X
[Cp] Country of publication:Korea (South)
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1412
[Cu] Class update date: 150113
[Lr] Last revision date:150113
[Js] Journal subset:IM
[St] Status:In-Data-Review
[do] DOI:10.3350/cmh.2014.20.4.402

  5 / 1209 MEDLINE  
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[PMID]: 24952892
[Au] Autor:Della Rosa PA; Cerami C; Gallivanone F; Prestia A; Caroli A; Castiglioni I; Gilardi MC; Frisoni G; Friston K; Ashburner J; Perani D; EADC-PET Consortium
[Ad] Address:Institute of Molecular Bioimaging and Physiology, National Research Council, Segrate, MI, Italy, pasquale.dellarosa@ibfm.cnr.it.
[Ti] Title:A standardized [18F]-FDG-PET template for spatial normalization in statistical parametric mapping of dementia.
[So] Source:Neuroinformatics;12(4):575-93, 2014 Oct.
[Is] ISSN:1559-0089
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:[18F]-fluorodeoxyglucose (FDG) Positron Emission Tomography (PET) is a widely used diagnostic tool that can detect and quantify pathophysiology, as assessed through changes in cerebral glucose metabolism. [18F]-FDG PET scans can be analyzed using voxel-based statistical methods such as Statistical Parametric Mapping (SPM) that provide statistical maps of brain abnormalities in single patients. In order to perform SPM, a "spatial normalization" of an individual's PET scan is required to match a reference PET template. The PET template currently used for SPM normalization is based on [15O]-H2O images and does not resemble either the specific metabolic features of [18F]-FDG brain scans or the specific morphological characteristics of individual brains affected by neurodegeneration. Thus, our aim was to create a new [18F]-FDG PET aging and dementia-specific template for spatial normalization, based on images derived from both age-matched controls and patients. We hypothesized that this template would increase spatial normalization accuracy and thereby preserve crucial information for research and diagnostic purposes. We investigated the statistical sensitivity and registration accuracy of normalization procedures based on the standard and new template-at the single-subject and group level-independently for subjects with Mild Cognitive Impairment (MCI), probable Alzheimer's Disease (AD), Frontotemporal lobar degeneration (FTLD) and dementia with Lewy bodies (DLB). We found a significant statistical effect of the population-specific FDG template-based normalisation in key anatomical regions for each dementia subtype, suggesting that spatial normalization with the new template provides more accurate estimates of metabolic abnormalities for single-subject and group analysis, and therefore, a more effective diagnostic measure.
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Em] Entry month:1410
[Cu] Class update date: 141230
[Lr] Last revision date:141230
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.1007/s12021-014-9235-4

  6 / 1209 MEDLINE  
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[PMID]: 25518446
[Au] Autor:Circella E; Legretto M; Pugliese N; Caroli A; Bozzo G; Accogli G; Lavazza A; Camarda A
[Ti] Title:Psittacine beak and feather disease-like illness in Gouldian finches (Chloebia gouldiae).
[So] Source:Avian Dis;58(3):482-7, 2014 Sep.
[Is] ISSN:0005-2086
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Beak and feather disease virus (BFDV) is a member of the genus Circovirus and causes psittacine beak and feather disease (PBFD) in Psittaciformes. PBFD is a severe disease generally characterized by immunodeficiency and beak and feather disorders. Although Circovirus spp. have been detected in several nonpsittacine species, little is known about the symptoms and the disease associated with this infection in birds other than Psittaciformes. In this study, we report the identification of Circovirus infection in a flock of Gouldian finches showing beak and feather disorders. Sequence analyses on the rep gene of the virus highlighted a strong similarity at nucleotide and amino acid levels with the corresponding regions of BFDV from psittacine species. By contrast, it was more distant to circoviruses identified in finch and canary.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1412
[Js] Journal subset:IM
[St] Status:In-Process

  7 / 1209 MEDLINE  
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[PMID]: 25501066
[Au] Autor:Marzano AV; Ceccherini I; Gattorno M; Fanoni D; Caroli F; Rusmini M; Grossi A; De Simone C; Borghi OM; Meroni PL; Crosti C; Cugno M
[Ad] Address:From the Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti (AVM, DF, CC), Università degli Studi di Milano, Unità Operativa di Dermatologia, IRCCS Fondazione Ca' Granda, Ospedale Maggiore Policlinico, Milano; UOC Genetica Medica (IC, FC, MR, AG), Istituto Giannina Gaslini; Pediatria II (MG), Istituto Giannina Gaslini, Genova; Dipartimento di Dermatologia (CDS), Università Cattolica del Sacro Cuore, Roma; Dipartimento di Scienze Cliniche e di Comunità (OMB, PLM), Università degli Studi di Milano, Cattedra di Reumatologia, Istituto G. Pini, Milano; and Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti (MC), Università degli Studi di Milano, Unità Operativa di Medicina Interna, IRCCS Fondazione Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy.
[Ti] Title:Association of Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis (PASH) Shares Genetic and Cytokine Profiles With Other Autoinflammatory Diseases.
[So] Source:Medicine (Baltimore);93(27):e187, 2014 Dec.
[Is] ISSN:1536-5964
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:The association of pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) has recently been described and suggested to be a new entity within the spectrum of autoinflammatory syndromes, which are characterized by recurrent episodes of sterile inflammation, without circulating autoantibodies and autoreactive T-cells. We conducted an observational study on 5 patients with PASH syndrome, analyzing their clinical features, genetic profile of 10 genes already known to be involved in autoinflammatory diseases (AIDs), and cytokine expression pattern both in lesional skin and serum. In tissue skin samples, the expressions of interleukin (IL)-1ß and its receptors I and II were significantly higher in PASH (P = 0.028, 0.047, and 0.050, respectively) than in controls. In PASH patients, chemokines such as IL-8 (P = 0.004), C-X-C motif ligand (CXCL) 1/2/3 (P = 0.028), CXCL 16 (P = 0.008), and regulated on activation, normal T cell expressed and secreted (RANTES) (P = 0.005) were overexpressed. Fas/Fas ligand and cluster of differentiation (CD)40/CD40 ligand systems were also overexpressed (P = 0.016 for Fas, P = 0.006 for Fas ligand, P = 0.005 for CD40, and P = 0.004 for CD40 ligand), contributing to tissue damage and inflammation. In peripheral blood, serum levels of the main proinflammatory cytokines, that is, IL-1ß, tumor necrosis factor-α, and IL-17, were within the normal range, suggesting that in PASH syndrome, the inflammatory process is mainly localized into the skin. Four out of our 5 PASH patients presented genetic alterations typical of well-known AIDs, including inflammatory bowel diseases, and the only patient lacking genetic changes had clinically evident Crohn disease. In conclusion, overexpression of cytokines/chemokines and molecules amplifying the inflammatory network, along with the genetic changes, supports the view that PASH syndrome is autoinflammatory in origin.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1412
[Js] Journal subset:AIM; IM
[St] Status:In-Data-Review
[do] DOI:10.1097/MD.0000000000000187

  8 / 1209 MEDLINE  
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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.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1412
[Js] Journal subset:IM
[St] Status:In-Data-Review

  9 / 1209 MEDLINE  
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[PMID]: 24022473
[Au] Autor:Issar P; Issar SK
[Ad] Address:Department of Radiodiagnosis, J L N Hospital and Research Centre, Bhilai, Chhattisgarh, 490 006, India, mareesh_23@yahoo.co.in.
[Ti] Title:Caroli's disease.
[So] Source:Indian J Gastroenterol;33(5):500, 2014 Sep.
[Is] ISSN:0975-0711
[Cp] Country of publication:India
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1410
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.1007/s12664-013-0403-5

  10 / 1209 MEDLINE  
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[PMID]: 24114580
[Au] Autor:Büscher R; Büscher AK; Weber S; Mohr J; Hegen B; Vester U; Hoyer PF
[Ad] Address:Children's Hospital, Pediatrics II, University of Duisburg-Essen, Hufelandstr. 55, 45122, Essen, Germany, rainer.buescher@uk-essen.de.
[Ti] Title:Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD): kidney-related and non-kidney-related phenotypes.
[So] Source:Pediatr Nephrol;29(10):1915-25, 2014 Oct.
[Is] ISSN:1432-198X
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:Autosomal recessive polycystic kidney disease (ARPKD), although less frequent than the dominant form, is a common, inherited ciliopathy of childhood that is caused by mutations in the PKHD1-gene on chromosome 6. The characteristic dilatation of the renal collecting ducts starts in utero and can present at any stage from infancy to adulthood. Renal insufficiency may already begin in utero and may lead to early abortion or oligohydramnios and lung hypoplasia in the newborn. However, there are also affected children who have no evidence of renal dysfunction in utero and who are born with normal renal function. Up to 30 % of patients die in the perinatal period, and those surviving the neonatal period reach end stage renal disease (ESRD) in infancy, early childhood or adolescence. In contrast, some affected patients have been diagnosed as adults with renal function ranging from normal to moderate renal insufficiency to ESRD. The clinical spectrum of ARPKD is broader than previously recognized. While bilateral renal enlargement with microcystic dilatation is the predominant clinical feature, arterial hypertension, intrahepatic biliary dysgenesis remain important manifestations that affect approximately 45 % of infants. All patients with ARPKD develop clinical findings of congenital hepatic fibrosis (CHF); however, non-obstructive dilation of the intrahepatic bile ducts in the liver (Caroli's disease) is seen at the histological level in only a subset of patients. Cholangitis and variceal bleeding, sequelae of portal hypertension, are life-threatening complications that may occur more often in advanced cases of liver disease. In this review we focus on common and uncommon kidney-related and non-kidney-related phenotypes. Clinical management of ARPKD patients should include consideration of potential problems related to these manifestations.
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Em] Entry month:1409
[Js] Journal subset:IM
[St] Status:In-Process
[do] DOI:10.1007/s00467-013-2634-1


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