Caroli Disease (Clinical Observation)

Никитин, И. Г.; Карабиненко, А. А.; Никитин, А. В.; Дедов, Е. И.; Жукова, Д. Г.; Presnova, E D; Корвяков, С. А.; Азимов, Р. Х.; Резник, Е. В.

doi:10.22416/1382-4376-2018-28-6-77-83

Cited by 4 publications

(5 citation statements)

References 15 publications

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“…МРТ является наиболее специфическим и неинвазивным методом визуализации множественной дилатации протоков, наблюдаемой при болезни Кароли. ЭРХПГ является золотым стандартом в диагностике дилатации желчевыводящих путей [2,5,6].…”

Section: лабораторно-инструментальные исследованияunclassified

“…Лечение данной патологии зависит от клинических особенностей и локализации билиарной аномалии [2,4,5,6]. Основная цель терапии -поддерживающая.…”

Section: лабораторно-инструментальные исследованияunclassified

“…Диагноз болезни Кароли и синдрома Кароли устанавливается с помощью визуализирующих исследований, которые демонстрируют эктазию желчных протоков и неравномерное кистозное расширение крупных проксимальных внутрипеченочных желчных протоков при нормальном общем желчном протоке [2,3].…”

Section: заключениеunclassified

“…В данном наблюдении описывается Болезнь Кароли, выста вленна я на основа нии да нных компьютерной томографии и магнитнорезонансной холангиопанкреатографии, клинических данных [2].…”

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Clinical observation of the patient with monolobar form Caroli disease

Guryleva

2024

ECG

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Objective. To demonstrate a case of a rare congenital liver disease, Caroli disease, characterized by segmental non-obstructive fibrocystic dilatation of the intrahepatic bile ducts. Main points. Caroli disease was first described by the French doctor Jacques Caroli in 1958. There are 2 types of Caroli disease: True type with dilation of the intrahepatic bile ducts, usually affecting one segment and with a tendency to form stones and develop cholangitis. The second type, which is combined with congenital hepatic fibrosis, polycystic kidney disease and is more common than the true type. When the disease is accompanied by CHF, it is called Caroli syndrome or Grumbach disease. It is important to remember about Crowley’s disease and include it in the differential range of diseases of the biliary tract and liver, such as cholangitis, secondary biliary cirrhosis, portal hypertension of unknown etiology, cholangiocarcinoma. Conclusion.The diagnosis of Caroli disease and Caroli syndrome is made using imaging studies. Treatment is largely supportive and should be individualized, with prognosis depending on the severity of the disease and the presence of concomitant renal dysfunction.

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Section: лабораторно-инструментальные исследованияunclassified

Section: заключениеunclassified

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Clinical observation of the patient with monolobar form Caroli disease

Guryleva

2024

ECG

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“…Последнее десятилетие отмечен рост числа публикаций, посвященных исследованию болезни Кароли, что, безусловно, связано с развитием генетических и молекулярных технологий, а также с успехами трансплантологии [1][2][3][4]. Болезнь Кароли -редкое (1 случай на 1 млн человек) генетическое заболевание, характеризующееся необструктивными сегментарными кистозными (мешотчатыми или веретенообразными) расширениями внутрипеченочных желчных протоков.…”

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Caroli syndrome in children: a brief review and clinical observation

Gudkov

Dmitriev

Slobodyanyuk

2023

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A review of domestic and foreign literature on the disease and Caroli syndrome is presented. The review includes a historical background and a modern view on the etiology and pathogenesis of the disease, its place among fibrocystic diseases of the liver. Caroli’s disease is a rare genetic pathology, characterized by a wide clinical and morphological polymorphism from severe variants with fibrosis in the neonatal period to cases with manifestation at a late age. The genetic causes of pathology, in particular, mutations associated with autosomal recessive and autosomal dominant forms of polycystic kidney disease, have been studied. A large number of described mutations and the predominance of complex heterozygotes among patients explains the diversity of clinical forms of pathology. The relationship between the genotype and phenotypic variants of pathology has not been determined. In Caroli’s disease, in addition to cystic changes in the liver and kidneys, damage to other organs and systems, in particular cerebral vessels, can be observed. Extrahepatic and extrarenal symptoms of the disease are described, as well as combinations with various genetic diseases. A clinical case of Caroli syndrome in a child observed by the authors from birth is presented. The disease manifested as congenital cystic transformation of the kidney. Hepatic pathology was detected at the age of 5 years. Subsequently, there was a rapid progression of liver damage with the development of severe complications: cirrhosis of the liver, portal hypertension, edematous syndrome, hepatic encephalopathy. At the age of 11 years, the patient successfully underwent orthotopic liver transplantation. The presented case demonstrates the late detection of hepatic damage, its rapid progression. Despite the presence of severe complications, liver transplantation was a success and the patient is currently in a stable condition compensated for all functions.

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