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Caroli disease

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https://www.readbyqxmd.com/read/29159008/caroli-s-disease-as-a-cause-of-chronic-epigastric-abdominal-pain-two-case-reports-and-a-brief-review-of-the-literature
#1
REVIEW
Pedro Cabral Correia, Bruno Morgado
Caroli's disease is a very rare congenital malformation, currently included in cystic diseases of the biliary tract, and is characterized by ectasia and dilatation of the intrahepatic bile ducts. Two clinical entities can be distinguished, Caroli's disease in which congenital hepatic impairment is limited to cystic dilatation and Caroli's syndrome in which congenital hepatic fibrosis coexists. We present two cases of atypical presentations of Caroli's disease. Case one was a 76-year-old man who was referred to our hospital for chronic non-remitting epigastric pain prior to diagnosis...
September 20, 2017: Curēus
https://www.readbyqxmd.com/read/29108211/-a-case-of-caroli-disease-in-tibet-china
#2
J Wang
No abstract text is available yet for this article.
October 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29104033/oral-and-maxillofacial-manifestations-of-chronic-kidney-disease-mineral-and-bone-disorder-a-multicenter-retrospective-study
#3
Flávia Sirotheau Corrêa Pontes, Márcio Ajudarte Lopes, Lucas Lacerda de Souza, Diogo Dos Santos da Mata Rezende, Alan Roger Santos-Silva, Jacks Jorge, Wagner Gomes da Silva, Fábio Ramôa Pires, André Caroli Rocha, Wladimir Gushiken de Campos, Milena Coelho Fernandes Caldato, Regina Matsunaga Martin, Felipe Paiva Fonseca, Hélder Antônio Rebelo Pontes
OBJECTIVE: To describe the oral and maxillofacial manifestations of patients diagnosed with chronic kidney disease-mineral and bone disorders. STUDY DESIGN: Over a 13-year period, clinicopathologic data of patients diagnosed with CKD-MBD who had oral and maxillofacial alterations were retrieved from the files of 4 Brazilian institutions. Data included clinical, radiographic, microscopic, and biochemical findings; treatment employed; and follow-up status. RESULTS: Twenty-one cases were identified, with 13 patients diagnosed as brown tumor of hyperparathyroidism (BTH) and 8 as osteitis fibrosa/renal osteodystrophy (OF/RO) (4 of them clinically consistent with Sagliker syndrome)...
October 10, 2017: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
https://www.readbyqxmd.com/read/29091323/lower-levels-of-dehydroepiandrosterone-sulfate-are-associated-with-more-advanced-liver-fibrosis-in-chronic-hepatitis-c
#4
João Marcello de Araujo Neto, Henrique Sérgio Moraes Coelho, Maria Chiara Chindamo, Guilherme Ferreira da Motta Rezende, Vera Lúcia Nunes Pannain, Adriana Marques Caroli de Freitas Bottino, Luiz Fernando Bruzzi Porto, Ronir Raggio Luiz, Cristiane Alves Villela-Nogueira, Renata M Perez
Chronic infection with the hepatitis C virus induces liver fibrosis, but it is unknown why some patients progress to advanced fibrosis while others remain with mild disease. Recently, an inverse association between serum levels of dehydroepiandrosterone sulphate (DHEA-S) and liver fibrosis in patients with nonalcoholic fatty liver disease was described and it was postulated that dehydroepiandrosterone (DHEA) has anti-fibrotic effects. Our aim was to compare serum DHEA-S levels with liver fibrosis in hepatitis C patients...
November 1, 2017: Journal of Viral Hepatitis
https://www.readbyqxmd.com/read/29062909/congenital-hepatic-fibrosis-in-a-9-year-old-female-patient-a-case-report
#5
Kamil Janowski, Maria Goliszek, Joanna Cielecka-Kuszyk, Irena Jankowska, Joanna Pawłowska
Congenital hepatic fibrosis (CHF) is a rare, autosomal recessive disorder, clinically characterized by hepatic fibrosis and portal hypertension. CHF results from ductal plate malformation (DPM) of the intrahepatic bile ducts. Four clinical forms can be observed: portal hypertensive, cholangitic, mixed and latent. CHF is one of the "fibropolycystic diseases" which also include several conditions with a variety of intrahepatic bile duct dilatation and associated periportal fibrosis such as Caroli disease, autosomal recessive and dominant polycystic kidney disease (ARPKD or ADPKD), Ivemark, Jeune, Joubert, Bardet-Biedl, Meckel-Gruber and Arima syndromes...
September 2017: Clin Exp Hepatol
https://www.readbyqxmd.com/read/29043403/hepatic-morphology-abnormalities-beyond-cirrhosis
#6
REVIEW
Giuseppe Mamone, Kelvin Cortis, Aquilina Sarah, Settimo Caruso, Roberto Miraglia
The diagnosis of cirrhosis can be reached on the basis of established hepatic morphological changes. However, some other conditions can mimic cirrhosis. The aim of this pictorial essay is to review the CT and MRI appearances of hepatic morphology abnormalities in the cirrhotic liver and other diseases, describing pathologic conditions that can mimic cirrhosis, with useful tips for the differential diagnosis. Mimickers of cirrhosis include congenital hepatic fibrosis, Caroli disease, Budd-Chiari Syndrome, hepatoportal sclerosis, cavernous transformation of the portal vein, pseudocirrhosis from metastatic disease, acute liver failure, post-therapeutic morphologic changes in the liver, and infective conditions including schistosomiasis and oriental cholangiohepatitis...
October 17, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28939930/-upper-abdominal-pain-and-febrile-episodes-in-a-44-year-old-filipino-woman
#7
A Deimel, J Sturm, T Vielfort, T Zöpf
A 44-year-old Filipino woman presented with abdominal pain and fever. Clinical examination and blood tests revealed no pathological results; however, (cross-sectional) imaging showed saccular cystic bile duct dilatation in the right liver with solid intraductal masses. Due to the clinical presentation the patient was admitted for surgical intervention with the diagnosis of Caroli disease. During the surgical procedure histopathology showed an intraductal papillary neoplasm of the bile duct (IPNB). The planned segmentetomy was extended to hemihepatectomy...
September 22, 2017: Der Internist
https://www.readbyqxmd.com/read/28921746/identification-of-occult-active-infection-using-pet-ct-in-a-combined-liver-kidney-transplant-candidate
#8
Julien Al Shakarchi, Desley Neil, Graham Lipkin, Ahmed Elsharkawy, Nicholas Inston
This case describes a patient being considered for combined liver-kidney transplantation for Caroli's disease with a failed renal transplant. A chronic septic focus could not be located with standard imaging techniques, such as ultrasonography and computed tomography. This case report highlights the observation that a retained non-functioning transplant can be the cause of fever of unknown origin and PET-CT can be useful in diagnosing these challenging cases. This article is protected by copyright. All rights reserved...
September 16, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28912093/lentiviral-gene-delivery-to-plasmolipin-expressing-cells-using-mus-caroli-endogenous-retrovirus-envelope-protein
#9
REVIEW
M M Prokofjeva, G M Proshkina, T D Lebedev, A A Shulgin, P V Spirin, V S Prassolov, S M Deyev
Gene therapy is a promising method for treating malignant diseases. One of the main problems is target delivery of therapeutic genes. Here we show that lentiviral vector particles pseudotyped with Mus caroli endogenous retrovirus (McERV) envelope protein can be used for selective transduction of PLLP-expressing cells. As a therapeutic gene in McERV-pseudotyped vector particles we used miniSOG encoding the cytotoxic FMN-binding protein, which can generate reactive oxygen species under illumination. Significant cytotoxic effect (up to 80% of dead cells in population) was observed in PLLP-expressing cells transduced with McERV-pseudotyped vector particles and subjected to illumination...
November 2017: Biochimie
https://www.readbyqxmd.com/read/28892125/novel-spondyloepimetaphyseal-dysplasia-due-to-ufsp2-gene-mutation
#10
M Di Rocco, M Rusmini, F Caroli, A Madeo, M Bertamino, G Marre-Brunenghi, I Ceccherini
Beukes Hip Dysplasia is an autosomal dominant disease which has to date been described only in a large South African family of Dutch origin. The patients presented with progressive epiphyseal dysplasia limited to femoral capital epiphysis and their height was not significantly reduced. A unique variant of the ubiquitin-fold modifier 1 (Ufm1)-specific peptidase 2 (UFSP2) gene (c.868T>C) has been reported in all individuals from Beukes family with clinical and radiological diagnosis of Beukes Hip Dysplasia...
September 11, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28884036/severe-hepatopulmonary-syndrome-in-a-child-with-caroli-syndrome
#11
W De Jesus-Rojas, K McBeth, A Yadav, J M Stark, R A Mosquera, C Jon
Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that leads to portal hypertension. In children with liver disease, HPS should be considered in the differential diagnosis of prolonged, otherwise unexplained, hypoxemia. The presence of HPS can improve patient priority on the liver transplantation wait list, despite their Pediatric End-Stage Liver Disease (PELD) score...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28814334/abernethy-malformation-associated-with-caroli-s-syndrome-in-a-patient-with-a-pkhd1-mutation-a-case-report
#12
Xiao-Xiao Mi, Xiao-Guang Li, Zi-Rong Wang, Ling Lin, Chun-Hai Xu, Jun-Ping Shi
BACKGROUND: Abernethy malformation is a rare congenital anomaly characterised by the partial or complete absence of the portal vein and the subsequent development of an extrahepatic portosystemic shunt. Caroli's disease is a rare congenital condition characterised by non-obstructive saccular intrahepatic bile duct dilation. Caroli's disease combined with congenital hepatic fibrosis and/or renal cystic disease is referred to - Caroli's syndrome. The combination of Abernethy malformation and Caroli's syndrome has not been reported previously...
August 16, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28771916/vitamin-d-deficiency-in-an-italian-cohort-of-infertile-women
#13
Paola Triggianese, Abdulla Watad, Francesca Cedola, Carlo Perricone, Howard Amital, Ilio Giambini, Roberto Perricone, Yehuda Shoenfeld, Caterina De Carolis
PROBLEM: The purpose of this study was to explore whether vitamin D might be a marker of female primary infertility in association with the presence of autoimmune diseases (ADs). METHODS: The study was a cross-sectional descriptive study in consecutive outpatients of the Polymedical Center for Prevention of Recurrent Spontaneous Abortion (RSA), in Rome, Italy. Women were eligible if they received a diagnosis of primary infertility or RSA. Serum vitamin D, calcium, and PTH were analyzed...
August 3, 2017: American Journal of Reproductive Immunology: AJRI
https://www.readbyqxmd.com/read/28734697/prognostic-significance-of-hmsh2-hmsh3-and-hmsh6-expression-in-ameloblastoma
#14
Gleyson Kleber do Amaral-Silva, Celeste Sánchez-Romero, Vivian Petersen Wagner, Manoela Domingues Martins, Hélder Antônio Rebelo Pontes, Eduardo Rodrigues Fregnani, Fernando Augusto Soares, Oslei Paes de Almeida, André Caroli Rocha, Alan Roger Santos-Silva, Felipe Paiva Fonseca, Pablo Agustin Vargas
OBJECTIVE: The aim of this study was to investigate hMutS proteins in developing human tooth, ameloblastomas, and ameloblastic carcinoma and to determine whether the expression of these proteins has any prognostic potential. STUDY DESIGN: Ten cases of developing human tooth, 39 ameloblastomas, and 2 ameloblastic carcinomas were used to determine the distribution of the proteins during the process of carcinogenesis. Simultaneously, another sample of 73 ameloblastomas was arranged in tissue microarray, and their clinical, microscopic, and radiographic features; treatment outcome; presence of BRAF-V600E mutation; and follow-up data were assessed to determine the prognostic relevance of the expression of hMutS (hMSH2, hMSH3, hMSH6) and Ki-67...
June 7, 2017: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
https://www.readbyqxmd.com/read/28669735/prenatal-diagnosis-of-caroli-disease-associated-with-autosomal-recessive-polycystic-kidney-disease-by-3-d-ultrasound-and-magnetic-resonance-imaging
#15
Pedro Teixeira Castro, Ana Paula Pinho Matos, Heron Werner, Pedro Daltro, Tatiana Fazecas, Renata Nogueira, Edward Araujo Júnior
BACKGROUND: Caroli disease is a very rare congenital anomaly characterized by non-obstructive saccular or fusiform dilatation of the intrahepatic bile ducts. It is associated with bile stagnation and hepatolithiasis, which explain the recurrent cholangitis and portal hypertension as a consequence of congenital liver fibrosis. Although there are several reports of diagnosis in childhood and adult life, the prenatal diagnosis using conventional 2-D ultrasound is rare, with few reports in the literature...
June 29, 2017: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
https://www.readbyqxmd.com/read/28638817/a-challenging-case-of-hepatoblastoma-concomitant-with-autosomal-recessive-polycystic-kidney-disease-and-caroli-syndrome-review-of-the-literature
#16
Nevil Kadakia, Steven J Lobritto, Nadia Ovchinsky, Helen E Remotti, Darrell J Yamashiro, Jean C Emond, Mercedes Martinez
We report a rare case of an 18-month-old female with autosomal recessive polycystic kidney disease, Caroli syndrome, and pure fetal type hepatoblastoma. The liver tumor was surgically resected with no chemotherapy given. Now 9 years post resection she demonstrates no local or distant recurrence and stable renal function.
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28633199/diffuse-type-caroli-disease-with-characteristic-central-dot-sign-complicated-by-multiple-intrahepatic-and-common-bile-duct-stones
#17
Moon Joo Hwang, Tae Nyeun Kim
Caroli disease (CD) is a rare congenital malformation of the liver characterized by non-obstructive, segmental, cystic dilatation of the intrahepatic bile ducts (IHDs). The clinical course is usually asymptomatic for the first 5-20 years, and symptoms may seldom occur throughout the patient's life. Bile stagnation leads to recurrent episodes of cholangitis, stone formation, or liver abscesses, and biliary cirrhosis usually occurs years later. Here we report on a 42-year-old man diagnosed with diffuse-type CD with a characteristic central dot sign, who had multiple intrahepatic and common bile duct (CBD) stones...
July 2017: Clinical Endoscopy
https://www.readbyqxmd.com/read/28621010/diversity-of-renal-phenotypes-in-patients-with-wdr19-mutations-two-case-reports
#18
Takahisa Yoshikawa, Koichi Kamei, Hiroko Nagata, Ken Saida, Mai Sato, Masao Ogura, Shuichi Ito, Osamu Miyazaki, Maki Urushihara, Shuji Kondo, Noriko Sugawara, Kiyonobu Ishizuka, Yuko Hamasaki, Seiichiro Shishido, Naoya Morisada, Kazumoto Iijima, Michio Nagata, Takako Yoshioka, Kentaro Ogata, Kenji Ishikura
WDR19 has been reported as a causative gene of nephronophthisis-related ciliopathies. Patients with WDR19 mutations can show various extrarenal manifestations such as skeletal disorders, Caroli disease, and retinal dystrophy, and typically display nephronophthisis as a renal phenotype. However, there is limited information on the renal phenotypes of patients with WDR19 mutations. We report two Japanese infants with Sensenbrenner syndrome caused by WDR19 mutations who demonstrated different features in renal ultrasound and histopathological results, despite several common extrarenal manifestations...
July 2017: Nephrology
https://www.readbyqxmd.com/read/28606758/transjugular-intrahepatic-portosystemic-shunt-in-a-patient-with-caroli-s-disease-and-portal-cavernoma
#19
Roberto Miraglia, Luigi Maruzzelli, Sarah Degiorgio, Angelo Luca
No abstract text is available yet for this article.
May 25, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28579820/cannabis-and-intractable-chronic-pain-an-explorative-retrospective-analysis-of-italian-cohort-of-614-patients
#20
Guido Fanelli, Giuliano De Carolis, Claudio Leonardi, Adele Longobardi, Ennio Sarli, Massimo Allegri, Michael E Schatman
BACKGROUND: Despite growing interest in the therapeutic use of cannabis to manage chronic pain, only limited data that address these issues are available. In recent years, a number of nations have introduced specific laws to allow patients to use cannabis preparations to treat a variety of medical conditions. In 2015, the Italian government authorized the use of cannabis to treat several diseases, including chronic pain generally, spasticity in multiple sclerosis, cachexia and anorexia among AIDS and cancer patients, glaucoma, Tourette syndrome, and certain types of epilepsy...
2017: Journal of Pain Research
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