Caroli syndrome: a clinical case with detailed histopathological analysis
| العنوان: | Caroli syndrome: a clinical case with detailed histopathological analysis |
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| المؤلفون: | Nasima Gizzatullina, Ilyas Sayfutdinov, Angelina Titova, Renata Saitburkhanova, Ilya N. Kotov, Isaev Artur Aleksandrovich, M O Mavlikeev, Igor Plaksa, Roman V. Deev, Sayar Abdulkhakov, Maria Abyzova, Andrey P. Kiyasov |
| المصدر: | Clinical Journal of Gastroenterology. 12:106-111 |
| بيانات النشر: | Springer Science and Business Media LLC, 2018. |
| سنة النشر: | 2018 |
| مصطلحات موضوعية: | Adult, Male, medicine.medical_specialty, Pathology, Biliary cirrhosis, Mutation, Missense, Connective tissue, Lumen (anatomy), Receptors, Cell Surface, Epithelium, Desquamation, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, business.industry, Gastroenterology, Granulation tissue, General Medicine, medicine.disease, Caroli Disease, Bile Ducts, Intrahepatic, Kidney Tubules, medicine.anatomical_structure, Liver, 030220 oncology & carcinogenesis, 030211 gastroenterology & hepatology, Histopathology, Atrophy, medicine.symptom, business, Infiltration (medical), Dilatation, Pathologic |
| الوصف: | Herein we present a clinical case of the Caroli syndrome caused by the compound heterozygous mutation in the PKHD1 gene. Histopathological assessment of liver detected biliary cirrhosis, numerous dilated bile ducts of various sizes, hyperplastic cholangiocytes containing a large amount of acid mucopolysaccharides, decreased s-tubulin expression and increased proliferation of cholangiocytes. A significant proportion of hepatic tissue was composed of giant cysts lined with a single layer of cholangiocytes, containing pus and bile in its lumen and surrounded by granulation tissue. An accumulation of neutrophils in the lumen of the bile ducts was observed, as well as an infiltration of the ducts and cysts surrounding connective tissue by CD4+ and to a lesser extent CD8+ lymphocytes. This may be caused by the expression of HLA-DR by cholangiocytes. Atrophy and desquamation of the epithelium of collecting tubules with the formation of microcysts were detected in the kidneys without a clinically significant loss of renal function. Morphopathogenetic mechanisms of the Caroli syndrome can be targets for a potential pathogenetic therapy and prevention of its manifestations and complications. |
| تدمد: | 1865-7265 1865-7257 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8992b5770e45d11bd4bf6dbb258870f1 https://doi.org/10.1007/s12328-018-0917-6 |
| حقوق: | CLOSED |
| رقم الأكسشن: | edsair.doi.dedup.....8992b5770e45d11bd4bf6dbb258870f1 |
| قاعدة البيانات: | OpenAIRE |
Find this article in full text from Springer Verlag. | تدمد: | 18657265 18657257 |
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