311 results match your criteria Caroli Disease Imaging


Cerebral Microbleeds Assessment and Quantification in COVID-19 Patients With Neurological Manifestations.

Front Neurol 2022 23;13:884449. Epub 2022 May 23.

Department of Neuroradiology, ASST Papa Giovanni XXIII, Bergamo, Italy.

It is increasingly acknowledged that Coronavirus Disease 2019 (COVID-19) can have neurological manifestations, and cerebral microbleeds (CMBs) have been observed in this setting. The aim of this study was to characterize CMBs patterns on susceptibility-weighted imaging (SWI) in hospitalized patients with COVID-19 with neurological manifestations. CMBs volume was quantified and correlated with clinical and laboratory parameters. Read More

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Imaging of fibropolycystic liver disease.

Abdom Radiol (NY) 2022 Jul 7;47(7):2356-2370. Epub 2022 Jun 7.

Department of Abdominal Imaging, Virginia Commonwealth University, Richmond, VA, USA.

Fibropolycystic liver diseases (FLDs) make up a rare spectrum of heritable hepatobiliary diseases resulting from congenital ductal plate malformations (DPMs) due to the dysfunction of proteins expressed on the primary cilia of cholangiocytes. The embryonic development of the ductal plate is key to understanding this spectrum of diseases. In particular, DPMs can result in various degrees of intrahepatic duct involvement and a wide spectrum of cholangiopathies, including congenital hepatic fibrosis, Caroli disease, polycystic liver disease, and Von Meyenberg complexes. Read More

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Basic principles and new advances in kidney imaging.

Kidney Int 2021 11 11;100(5):1001-1011. Epub 2021 May 11.

Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota, USA.

Over the past few years, clinical renal imaging has seen great advances, allowing assessments of kidney structure and morphology, perfusion, function and metabolism, and oxygenation, as well as microstructure and the interstitium. Medical imaging is becoming increasingly important in the evaluation of kidney physiology and pathophysiology, showing promise in management of patients with renal disease, in particular with regard to diagnosis, classification, and prediction of disease development and progression, monitoring response to therapy, detection of drug toxicity, and patient selection for clinical trials. A variety of imaging modalities, ranging from routine to advanced tools, are currently available to probe the kidney both spatially and temporally, particularly ultrasonography, computed tomography, positron emission tomography, renal scintigraphy, and multiparametric magnetic resonance imaging. Read More

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November 2021

Diffuse Versus Localized Caroli Disease: A Comparative MRCP Study.

AJR Am J Roentgenol 2021 06 21;216(6):1530-1538. Epub 2021 Apr 21.

Université Paris Diderot, Paris, France.

The purpose of this multicenter retrospective study was to assess the MRCP features of Caroli disease (CD). Sixty-six patients were identified from 2000 to 2019. The inclusion criteria were diagnosis of diffuse or localized CD mentioned in an imaging report, presence of intrahepatic bile duct (IHBD) dilatation, and having undergone an MRCP examination. Read More

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Imaging manifestations of Caroli disease with autosomal recessive polycystic kidney disease: a case report and literature review.

BMC Pregnancy Childbirth 2021 Apr 12;21(1):294. Epub 2021 Apr 12.

Department of Ultrasound, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.

Background: Both Caroli disease (CD) and autosomal recessive polycystic kidney disease (ARPKD) are autosomal recessive disorders, which are more commonly found in infants and children, for whom surviving to adulthood is rare. Early diagnosis and intervention can improve the survival rate to some extent. This study adopted the case of a 26-year-old pregnant woman to explore the clinical and imaging manifestations and progress of CD concomitant with ARPKD to enable a better understanding of the disease. Read More

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Post-discharge chest CT findings and pulmonary function tests in severe COVID-19 patients.

Eur J Radiol 2021 May 20;138:109676. Epub 2021 Mar 20.

Department of Radiology, ASST Papa Giovanni XXIII, Piazza OMS 1, Bergamo, BG, 24127, Italy; Post Graduate School of Diagnostic Radiology, University of Milan-Bicocca, Piazza dell'Ateneo Nuovo 1, Milano, MI, 20126, Italy.

Purpose: To evaluate chest computed tomography (CT) and pulmonary function test (PFT) findings in severe COVID-19 patients after discharge and correlate CT pulmonary involvement with PFT results.

Methods: COVID-19 patients admitted to our hospital between February 25 and May 2, 2020, were retrospectively included according to the following criteria: (a) COVID-19 defined as severe based on the WHO interim guidance (i.e. Read More

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Flare phenomenon in prostate cancer: recent evidence on new drugs and next generation imaging.

Ther Adv Med Oncol 2021 24;13:1758835920987654. Epub 2021 Feb 24.

Department of Medical Oncology, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori "Dino Amadori" (IRST), IRCCS, Meldola, Emilia-Romagna, Italy.

Over the years, an increasing proportion of metastatic prostate cancer patients has been found to experience an initial bone flare phenomenon under both standard therapies (androgen deprivation therapy, chemotherapy, radiotherapy, abiraterone, enzalutamide) and novel agents (immunotherapy, bone-targeting radioisotopes). The underlying biological mechanisms of the flare phenomenon are still elusive and need further clarification, particularly in relation to different types of treatment and their treatment response assessment. Flare phenomenon is often underestimated and, in some cases, can negatively affect clinical outcome. Read More

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February 2021

Combined use of 177Lu-DOTATATE and metronomic capecitabine (Lu-X) in FDG-positive gastro-entero-pancreatic neuroendocrine tumors.

Eur J Nucl Med Mol Imaging 2021 09 18;48(10):3260-3267. Epub 2021 Feb 18.

Nuclear Medicine and Radiometabolic Unit, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Via P. Maroncelli 40, 47014, Meldola, Italy.

Purpose: FDG-positive neuroendocrine tumors (NETs) have a poorer prognosis and exhibit shorter response duration to peptide receptor radionuclide therapy (PRRT). The aim of this prospective phase II study was to evaluate the efficacy and toxicity of PRRT with Lu-DOTATATE associated with metronomic capecitabine as a radiosensitizer agent in patients with advanced progressive FDG-positive gastro-entero-pancreatic (GEP) NETs.

Patients And Methods: Patients with advanced somatostatin receptor- and FDG-positive G1-G3 GEP-NETs (Ki67 < 55%) were treated with a cumulative activity of 27. Read More

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September 2021

Incorporating radiomics into clinical trials: expert consensus endorsed by the European Society of Radiology on considerations for data-driven compared to biologically driven quantitative biomarkers.

Eur Radiol 2021 Aug 25;31(8):6001-6012. Epub 2021 Jan 25.

European Imaging Biomarkers Alliance (EIBALL), European Society of Radiology, Vienna, Austria.

Existing quantitative imaging biomarkers (QIBs) are associated with known biological tissue characteristics and follow a well-understood path of technical, biological and clinical validation before incorporation into clinical trials. In radiomics, novel data-driven processes extract numerous visually imperceptible statistical features from the imaging data with no a priori assumptions on their correlation with biological processes. The selection of relevant features (radiomic signature) and incorporation into clinical trials therefore requires additional considerations to ensure meaningful imaging endpoints. Read More

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Renal Diffusion-Weighted Imaging (DWI) for Apparent Diffusion Coefficient (ADC), Intravoxel Incoherent Motion (IVIM), and Diffusion Tensor Imaging (DTI): Basic Concepts.

Methods Mol Biol 2021 ;2216:187-204

Department of Diagnostic and Interventional Radiology, Medical Faculty, University Dusseldorf, Dusseldorf, Germany.

The specialized function of the kidney is reflected in its unique structure, characterized by juxtaposition of disorganized and ordered elements, including renal glomerula, capillaries, and tubules. The key role of the kidney in blood filtration, and changes in filtration rate and blood flow associated with pathological conditions, make it possible to investigate kidney function using the motion of water molecules in renal tissue. Diffusion-weighted imaging (DWI) is a versatile modality that sensitizes observable signal to water motion, and can inform on the complexity of the tissue microstructure. Read More

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Portal hypertension: An uncommon presentation of Caroli's syndrome.

Clin Case Rep 2020 Dec 24;8(12):3134-3138. Epub 2020 Sep 24.

Department of Gastrointestinal Surgery Tribhuvan University Teaching Hospital Kathmandu Nepal.

Portal hypertension is not a classical presentation of Caroli's syndrome. However, some young children can present with overt signs and symptoms indicative of advanced disease state despite the improvement in imaging technology which has decreased its diagnostic age. High index of clinical suspicion can help in timely diagnosis and management. Read More

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December 2020

Functional Magnetic Resonance Imaging Versus Kidney Biopsy to Assess Response to Therapy in Nephrotic Syndrome: A Case Report.

Kidney Med 2020 Nov-Dec;2(6):804-809. Epub 2020 Oct 23.

Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Bergamo, Italy.

In recent years, kidney functional magnetic resonance imaging (MRI) has seen great advances, with several cross-sectional studies demonstrating correlations between MRI biomarkers and glomerular filtration rate. However, the potential of MRI to monitor response to therapy in kidney disease remains undescribed. In this case report, a man in his 40s with drug-resistant membranous nephropathy was addressed to ofatumumab therapy. Read More

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October 2020

Consensus-Based Technical Recommendations for Clinical Translation of Renal Phase Contrast MRI.

J Magn Reson Imaging 2022 02 2;55(2):323-335. Epub 2020 Nov 2.

Department of Bioengineering, Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Bergamo, Italy.

Background: Phase-contrast (PC) MRI is a feasible and valid noninvasive technique to measure renal artery blood flow, showing potential to support diagnosis and monitoring of renal diseases. However, the variability in measured renal blood flow values across studies is large, most likely due to differences in PC-MRI acquisition and processing. Standardized acquisition and processing protocols are therefore needed to minimize this variability and maximize the potential of renal PC-MRI as a clinically useful tool. Read More

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February 2022

[Clinicopathological features of Caroli disease/Caroli syndrome: an analysis of 21 cases].

Zhonghua Yi Xue Za Zhi 2020 Oct;100(38):3005-3009

Liver Disease Center, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.

To summarize and compare clinicopathological features of Caroli disease and Caroli syndrome. A total of 21 patients diagnosed with Caroli disease or Caroli syndrome in Beijing Friendship Hospital, Capital Medical University, from January 2015 to December 2018 were included. Through the clinical manifestations and comparative analysis of the differences between different clinical types, the liver pathological features of these patients were described. Read More

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October 2020

Factors contributing to diagnostic delay of Caroli syndrome: a single-center, retrospective study.

BMC Gastroenterol 2020 Sep 29;20(1):317. Epub 2020 Sep 29.

Department of General Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1, Shuaifuyuan, Wangfujing St., Beijing, 100730, China.

Background: Caroli syndrome (CS) is a rare congenital disorder without pathognomonic clinical symptoms or laboratory findings; therefore, the diagnosis is often delayed. The objective of this study was to investigate the diagnostic delay and associated risk factors in CS patients.

Methods: This was a retrospective analysis of 16 CS patients admitted to a single tertiary medical center on mainland China. Read More

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September 2020

Type IV choledochal cyst with polycystic kidney disease: a case report.

BMC Gastroenterol 2020 Sep 21;20(1):306. Epub 2020 Sep 21.

Department of Gastroenterology, The First Affiliated Hospital of Soochow University, Soochow University, 188 Shizi Road, Suzhou, Jiangsu Province, China.

Background: Choledochal cysts are divided into 5 types. Physicians believe that Caroli disease (which refers to type V biliary cysts) is a special type of biliary cyst caused by a mutation in the PKHD1 gene and is associated with autosomal recessive polycystic kidney disease (ARPKD). There is currently no clear association between other types of choledochal cysts and polycystic kidney disease. Read More

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September 2020

Risk of malignancy in Caroli disease and syndrome: A systematic review.

World J Gastroenterol 2020 Aug;26(31):4718-4728

Department of Surgery, Bürgerspital Solothurn, Solothurn 4500, Switzerland.

Background: Congenital intrahepatic bile duct dilatation without fibrosis is called Caroli disease (CD), and is called Caroli syndrome (CS) when it has fibrotic and cirrhotic liver morphology. The development of intrahepatic carcinoma is described in both conditions, but the reported incidence varies extensively. Potential risk factors for the malignant transformation were not described. Read More

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A case report of a novel compound heterozygous mutation in a Brazilian patient with deficiency of Interleukin-1 receptor antagonist (DIRA).

Pediatr Rheumatol Online J 2020 Aug 20;18(1):67. Epub 2020 Aug 20.

Center for Autoinflammatory Diseases and Immunodeficiencies, IRCCS Giannina Gaslini, Genoa, Italy.

Background: Deficiency of the natural antagonist of interleukin-1 was first described in 2009 and so far 20 patients has been reported. In Brazil just two cases have been reported both carrying the same homozygous 15 bp deletion. Blocking interleukin-1 has changed rate survival for DIRA patients. Read More

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Pituitary stalk interruption syndrome broadens the clinical spectrum of the TTC26 ciliopathy.

Clin Genet 2020 09 3;98(3):303-307. Epub 2020 Aug 3.

Saban Pediatric Medical Center for Israel, Soroka University Medical Center, Beer-Sheva, Israel.

Ciliopathies are a heterogeneous group of disorders, related to abnormal ciliary function. Severe biliary ciliopathy, caused by bi-allelic mutations in TTC26, has been recently described in the context of a syndrome of polydactyly and severe neonatal cholestasis, with brain, kidney and heart involvement. Pituitary involvement has not been previously reported for patients with this condition. Read More

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September 2020

Clinical value of negative Ga-PSMA PET/CT in the management of biochemical recurrent prostate cancer patients.

Eur J Nucl Med Mol Imaging 2021 01 26;48(1):87-94. Epub 2020 Jun 26.

Nuclear Medicine Unit, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy.

Purpose: To evaluate the clinical value of Ga-PSMA PET/CT negativity in patients with biochemical recurrent prostate cancer (BCR).

Methods: One hundred three BCR patients (median age, 70 years; median PSA, 0.47 ng/mL) with negative Ga-PSMA PET/CT, followed up for at least 1 year, were retrospectively identified in a database of 1003 consecutive patients undergoing Ga-PSMA PET/CT for BCR. Read More

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January 2021

Is It Time to Introduce PET/CT in Rectal Cancer Guidelines?

Clin Nucl Med 2020 Aug;45(8):611-617

Department of Nuclear Medicine, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori, (IRST), IRCCS, Meldola, Italy.

At the moment, international guidelines for rectal cancer suggest to consider F-FDG PET/CT scan in a few conditions: (1) at disease presentation in case of suspected or proven metastatic synchronous adenocarcinoma with potentially curable M1 disease; (2) in the recurrence workup for serial carcinoembryonic antigen level elevation; (3) in the recurrence workup with metachronous metastases documented by CT, MRI, or biopsy; (4) in case of strong contraindication to IV contrast agent administration; and (5) to evaluate an equivocal finding on a contrast-enhanced CT or MRI. PET/CT is not indicated in the follow-up or surveillance of rectal cancer. On the other hand, an attentive evaluation of the literature shows that PET/CT may also be used in some circumstances with significant levels of diagnostic accuracy. Read More

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Is It Time to Introduce PET/CT in Colon Cancer Guidelines?

Clin Nucl Med 2020 Jul;45(7):525-530

Nuclear Medicine Department, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori, IRCCS, Meldola, Italy.

International colon cancer guidelines suggest F-FDG PET/CT in a few circumstances: (1) at disease presentation in case of suspected or proven metastatic synchronous adenocarcinoma; (2) in the workup of recurrent colon cancer with metachronous metastases documented by CT, MRI, or biopsy and in case of serial CEA elevation with negative colonoscopy and negative CT; and (3) in case of contraindication to iodine- and gadolinium-based contrast agents. However, review of the literature has shown that PET/CT can also be used in other scenarios with significant levels of diagnostic advantage. This review aims to emphasize differences between guidelines and scientific literature for the use of PET/CT in colon cancer. Read More

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Caroli's syndrome evaluated by ultrasound and magnetic resonance imaging during pregnancy.

Ultrasound Obstet Gynecol 2020 07;56(1):125-127

Department of Obstetrics, Paulista School of Medicine, Federal University of São Paulo (EPM-UNIFESP), São Paulo, Brazil.

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[Caroli's syndrome].

Rofo 2020 Feb 28;192(2):119-122. Epub 2019 Nov 28.

Diagnostische und Interventionelle Radiologie, Universitätsklinikum Tübingen, Tübingen, Germany.

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February 2020

Consensus-based technical recommendations for clinical translation of renal diffusion-weighted MRI.

MAGMA 2020 Feb 1;33(1):177-195. Epub 2019 Nov 1.

Department of Radiology, Center for Biomedical Imaging (CBI), Center for Advanced Imaging Innovation and Research (CAI2R), NYU Langone Health, New York, NY, USA.

Objectives: Standardization is an important milestone in the validation of DWI-based parameters as imaging biomarkers for renal disease. Here, we propose technical recommendations on three variants of renal DWI, monoexponential DWI, IVIM and DTI, as well as associated MRI biomarkers (ADC, D, D*, f, FA and MD) to aid ongoing international efforts on methodological harmonization.

Materials And Methods: Reported DWI biomarkers from 194 prior renal DWI studies were extracted and Pearson correlations between diffusion biomarkers and protocol parameters were computed. Read More

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February 2020

Endoscopic therapy for gastro-oesophageal varices of Caroli's syndrome: a case report.

J Int Med Res 2020 Feb 8;48(2):300060519877993. Epub 2019 Oct 8.

Department of Gastroenterology, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, Anhui Province, China.

Caroli's disease (CD) is a very rare congenital disorder that is characterized by non-obstructive, segmental and cystic dilatation of intrahepatic ducts. Most patients with CD are asymptomatic, but some patients may suffer from hepatic fibrosis, liver cirrhosis or/and portal hypertension. In complex CD, cystic dilatations of the intrahepatic bile ducts can be present with congenital hepatic fibrosis, liver cirrhosis, portal hypertension, oesophageal varices and autosomal recessive polycystic kidney disease; a condition known as Caroli's syndrome. Read More

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February 2020

Giant choledochal cyst and infantile polycystic kidneys as prenatal sonographic features of Caroli syndrome.

J Clin Ultrasound 2020 Jan 4;48(1):45-47. Epub 2019 Oct 4.

Department of Obstetrics and Gynecology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.

Caroli syndrome is a developmental disorder caused by complete or partial arrest of ductal plate remodeling, leading to dilated bile ducts along with fibrosis surrounding the portal tracts. It is most commonly associated with autosomal recessive polycystic kidney (ARPKD). We report a unique case of Caroli syndrome, diagnosed prenatally at 24 weeks of gestation in a 29-year-old Thai woman. Read More

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January 2020

Hepatobiliary and Pancreatic: Caroli syndrome: Egg-like sign on CT.

Authors:
Y Zhong S Peng Y Yang

J Gastroenterol Hepatol 2020 Feb 1;35(2):180. Epub 2019 Sep 1.

Department of Liver Disease, The Second Hospital of Nanjing, Nanjing University of Chinese Medicine, Nanjing, China.

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February 2020

Congenital hepatic fibrosis and coexistent retinal macular degeneration: A case report.

Medicine (Baltimore) 2019 Aug;98(35):e16909

Department of Hepatology, the First Hospital of Jilin University, Changchun, China, Jilin Province, China.

Rationale: Congenital hepatic fibrosis (CHF) is an autosomal recessive disease characterized by periportal fibrosis, portal hypertension, and renal cystic disease. Essentially, CHF is a variant of fibrocystic disorder in which liver and kidney are commonly affected. Other frequently associated conditions include Caroli syndrome and polycystic kidney disease. Read More

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Phase-contrast magnetic resonance imaging to assess renal perfusion: a systematic review and statement paper.

MAGMA 2020 Feb 17;33(1):3-21. Epub 2019 Aug 17.

Department of Biomedical Engineering, Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Bergamo, Italy.

Objective: Phase-contrast magnetic resonance imaging (PC-MRI) is a non-invasive method used to compute blood flow velocity and volume. This systematic review aims to discuss the current status of renal PC-MRI and provide practical recommendations which could inform future clinical studies and its adoption in clinical practice.

Methodology: A comprehensive search of all the PC-MRI studies in human healthy subjects or patients related to the kidneys was performed. Read More

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February 2020