AIRE mutation in an elderly Caroli's patient with cholangitis and sepsis: a case report.

Background: Caroli's disease, an autosomal recessive, hereditary-related disorder, is a rare disease, in which the diagnosis is based primarily on medical imaging and pathophysiological examinations. It is characterized by intrahepatic cystic dilation or cysts. Hepatic resection of diseased lobes can cure or avoid the risk of malignancy.

Alveolar Echinococcosis - Diagnostic challenges of a parasitic disease on the rise in Europe - a case report.

A 45-year-old male presented with jaundice, abdominal discomfort, and weight loss. Abdominal ultrasound revealed intrahepatic cholestasis and cholelithiasis indicative of Caroli-syndrome. Subsequent magnetic resonance imaging demonstrated a corresponding 5 × 4 cm polycyclic, calcified mass and a distant 12 mm subhilar stenosis of the common bile duct resembling cholangiocarcinoma.

Gas exchange abnormalities in Long COVID are driven by the alteration of the vascular component.

Background: There are uncertainties whether the impairment of lung diffusing capacity in COVID-19 is due to an alteration in the diffusive conductance of the alveolar membrane (Dm), or an alteration of the alveolar capillary volume (Vc), or a combination of both. The combined measurement DLNO and DLCO diffusion, owing to NO higher affinity and faster reaction rate with haemoglobin compared to CO, enables the simultaneous and rapid determination of both Vc and Dm. The aim of the present study was to better identify the precise cause of post-COVID-19 diffusion impairment.

Caroli's disease misdiagnosed as bile duct cystadenoma: A case report.

Caroli's disease is also known as Congenital intrahepatic bile duct dilatation, and previously known as a congenital intrahepatic bile duct cyst; it is characterized by single or multiple intrahepatic cystic dilatations. In this article, we report a case of Caroli's disease (CT size 21.2 × 16.

Caroli's Disease Associated with Autosomal Dominant Polycystic Kidney Disease with Acute Pancreatitis: A Case Report.

A rare congenital hepatobiliary disorder called Caroli's disease is characterized by multifocal segmental dilatation of intrahepatic bile ducts that can affect the entire liver or only specific areas of it. Coexisting conditions with Caroli's disease include autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD). ADPKD results in the development of cysts, which are tiny fluid-filled sacs, in the kidneys.

An Artificial Intelligence Generated Automated Algorithm to Measure Total Kidney Volume in ADPKD.

Introduction: Accurate tools to inform individual prognosis in patients with autosomal dominant polycystic kidney disease (ADPKD) are lacking. Here, we report an artificial intelligence (AI)-generated method for routinely measuring total kidney volume (TKV).

Methods: An ensemble U-net algorithm was created using the nnUNet approach.

Caroli's Syndrome: A Case Report and Literature Review.

Synonymous with congenital non-obstructive saccular or fusiform intra-hepatic duct dilatation and congenital communicating cavernous ectasia of the intra-hepatic biliary tract, Caroli's syndrome (CS) is an extremely rare fibro-polycystic liver disorder characterized by ductal plate malformation and consequent peri-portal fibrosis due to segmental intra-hepatic duct dilatation. No more than 200 cases of the syndrome have been reported since 1958. CS may affect one or both lobes of the liver, but more commonly it affects the left hepatic lobe.

A Unique Case of Caroli Disease in Kenya's Medical Landscape.

BACKGROUND If a young patient presents with fever, abdominal pain, jaundice and significant imaging abnormalities, especially dilation of the biliary system, it is usually due to obstruction from stones or strictures. However, on very rare occasions, it can be due to complications of congenital cystic dilatation of the biliary system, known as Caroli disease. We present such a patient and discuss the differential diagnosis and implications for long-term management.

Caroli disease combined with Banti syndrome in a woman: a case report.

Caroli disease is a rare congenital malformation that predisposes to segmental cystic dilatation of the intrahepatic bile ducts. Banti syndrome is characterized by persistent splenomegaly due to chronic congestion, resulting in a low hematocrit and ultimately leading to pancytopenia. In this report, we describe a 29-year-old woman who presented with a >20-year history of hepatitis B surface antigen positivity and a >1-year history of recurrent fatigue and malaise.

Caroli disease with subcutaneous hemorrhage as the sole clinical manifestation: A case report.

Rational: The disease of Caroli is a rare congenital disorder, characterized by the dilated intrahepatic bile ducts, resulting from mutations in the PKHD1 gene. Caroli syndrome, characterized by dilated intrahepatic bile ducts with congenital hepatic fibrosis, is linked to autosomal recessive polycystic kidney disease. The clinical manifestations of Caroli disease are not typical, and Caroli disease is easy to be missed and misdiagnosed.

Primary Squamous Cell Biliary Carcinoma With Liver Metastasis Is Rare but Malicious.

Primary squamous cell carcinoma (SCC) of the liver is quite rare, and to our knowledge, very few cases have been reported in the literature. The exact pathogenesis of the disease is unestablished; however, it is mostly reported to be associated with hepatic cyst, Caroli's disease, hepatolithiasis, hepatic cirrhosis, and hepatic teratoma. We report a case of a 50-year-old woman with no prior medical history initially, who presented with postprandial epigastric and right upper quadrant pain that continued to worsen and was associated with early satiety, nausea, and weight loss of 25 pounds over 2 months, which prompted further evaluation by her primary care physician.

Functional Magnetic Resonance Imaging to Monitor Disease Progression: A Prospective Study in Patients with Primary Membranoproliferative Glomerulonephritis.

Introduction: Primary membranoproliferative glomerulonephritis (MPGN) is a rare kidney disease with poor prognosis and no specific therapies. The disease heterogeneity and the difficulty of performing repeated kidney biopsies pose big challenges. This study investigates the correlation between non-contrast enhanced magnetic resonance imaging (MRI) and histologic and clinical findings in patients with primary MPGN.

Automated prognosis of renal function decline in ADPKD patients using deep learning.

An accurate prognosis of renal function decline in Autosomal Dominant Polycystic Kidney Disease (ADPKD) is crucial for early intervention. Current biomarkers used are height-adjusted total kidney volume (HtTKV), estimated glomerular filtration rate (eGFR), and patient age. However, manually measuring kidney volume is time-consuming and subject to observer variability.

Abdominal Imaging in ADPKD: Beyond Total Kidney Volume.

In the context of autosomal dominant polycystic kidney disease (ADPKD), measurement of the total kidney volume (TKV) is crucial. It acts as a marker for tracking disease progression, and evaluating the effectiveness of treatment strategies. The TKV has also been recognized as an enrichment biomarker and a possible surrogate endpoint in clinical trials.

Caroli syndrome associated with atrial septal defect and polydactyly: a case report.

Introduction: Caroli disease is multifocal segmental dilatation of the large intrahepatic bile ducts that connect to the main duct. It is considered a rare disease with an incidence rate of 1 in 1,000,000 births. There are two types of Caroli: the first type is the simple type, Caroli disease, which includes only cystic dilatation of the intrahepatic bile ducts.

Hydatid Disease: A Radiological Pictorial Review of a Great Neoplasms Mimicker.

Hydatid cyst is a common name for the larval stage of a tapeworm species of , which is transmitted from animals to humans via the fecal-oral route. Hydatid cysts predominantly affect the liver (75%), followed by the lung (15%), and they can affect many organs in the human body. Medical imaging modalities are the keystone for the diagnosis of hydatid cysts with high sensitivity and specificity.

Findings of cholangitis in a kidney transplant patient with Caroli disease on FDG PET/CT and MRI.

Caroli disease is a rare congenital disorder of segmental cystic dilatations of the intrahepatic biliary ducts that maintain communication with the rest of the biliary tree. Its typical clinical presentation is recurrent episodes of cholangitis. The diagnosis is usually made using abdominal imaging modalities.

Flank pain has a significant adverse impact on quality of life in ADPKD: the CYSTic-QoL study.

Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disorder and a major cause of kidney failure worldwide. However, its impact on quality-of-life has not been systematically explored.

Methods: The CYSTic-QoL study was an observational study designed to study quality-of-life in adult European ADPKD patients with an estimated glomerular filtration rate (eGFR) ≥30 mL/min/1.

Effects of Octreotide-Long-Acting Release Added-on Tolvaptan in Patients with Autosomal Dominant Polycystic Kidney Disease: Pilot, Randomized, Placebo-Controlled, Cross-Over Trial.

Background: Tolvaptan and octreotide-long-acting release (LAR) have renoprotective effects in autosomal dominant polycystic kidney disease (ADPKD) that are partially mediated by amelioration of compensatory glomerular hyperfiltration. We compared the effects of tolvaptan and octreotide-LAR combination therapy versus those of tolvaptan monotherapy in patients with ADPKD.

Methods: This pilot, randomized, placebo-controlled, cross-over trial primarily compared the effects of 1- and 4-week treatments with octreotide-LAR (two 20-mg i.

Brain diffusion alterations in patients with COVID-19 pathology and neurological manifestations.

Background And Objective: COVID-19 neurological manifestations have been progressively recognized. Among available MRI techniques, diffusion weighted imaging (DWI) shows promise to study microstructure, inflammation, and edema. Previous DWI studies reported alterations in brain diffusivity in COVID-19 patients, as assessed by morphologic evaluation of brain DWI scans only.