Caroli Disease and Caroli Syndrome

Harjai, MM; Bal, Rabindra Kumar; Mohanty, Sasmita; Bhalla, V P; Singh, Balwinder; Maudar, K K

doi:10.1016/s0377-1237(17)30276-9

Cited by 4 publications

(5 citation statements)

References 3 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…В период обострения отмечаются кожный зуд, ахоличный стул, нарушение всасывания жиров, стеаторрея, гиповитаминоз, болезненность и незначительное увеличение печени, а также изменение биохимических показателей крови: повышение билирубина и его фракций, аспарагиновой и аланиновой аминотрансферазы (АСТ, АЛТ), гаммаглютамилтранспептидазы (ГГТП) и щелочной фосфатазы (ЩФ). Нередко желтуха связана с выходом мелких конкрементов из кист в общий желчный проток [6,8,13,15,17,20,21]. У данной пациентки на первый план в клинической картине выступали осложнения, с которыми пришлось бороться преимущественно методами хирургических паллиативных коррекций и трансплантацией печени.…”

Section: Discussionunclassified

“…The ultrasound picture of the enlarged spleen, patient N.: The spleen is enlarged to 20 × 10 × 8 cm, the structure of the parenchyma is homogeneous. The splenic vein is expanded to 13 mm, passable www.gastro-j.ru Рос журн гастроэнтерол гепатол колопроктол 2018; 28(6) / Rus J Gastroenterol Hepatol Coloproctol 2018; 28 (6) спленомегалией, подтвержденной при УЗИ. По лабораторным данным была доказана нормальная функция печени, без повышения холестатических маркеров.…”

Section: Discussionunclassified

“…www.gastro-j.ru Рос журн гастроэнтерол гепатол колопроктол 2018; 28(6) / Rus J Gastroenterol Hepatol Coloproctol 2018; 28 (6) For citation: Nikitin I.G., Karabinenko A.A., Nikitin A.E., Dedov E.I., Zhukova D.G., Presnova E.D., Korvyakov S.A., Azimov R.H., Reznik E.V. Caroli Disease (Clinical Observation).Russian Journal of Gastroenterology, Hepatology, Coloproctology.…”

unclassified

“…Клинические наблюдения / Clinical cases www.gastro-j.ru Рос журн гастроэнтерол гепатол колопроктол 2018; 28(6) / Rus J Gastroenterol Hepatol Coloproctol 2018; 28(6) …”

unclassified

See 3 more Smart Citations

Caroli Disease (Clinical Observation)

Никитин

Карабиненко

Никитин

et al. 2019

Rossijskij žurnal gastroènterologii, gepatologii, koloproktolog

View full text Add to dashboard Cite

The incidence of Caroli disease is estimated to be 1 case per 1 million people; as a result, there are very few available clinical observations. At the moment, the treatment of Caroli disease is limited to symptomatic therapy and the prevention of complications, as well as to corrective surgery and liver transplantation.Aim. The aim of this observation is to describe one case of a rare congenital liver disease – Caroli disease – characterised by a segmented non-obstructive fibrocystic dilation of the intrahepatic bile ducts.Key findings. A 21-year-old woman was hospitalised with the signs of a systemic inflammatory reaction, hepatosplenomegaly, jaundice, portal hypertension, hepatocellular insufficiency, as well as with the manifestations of cytolytic and cholestatic syndromes. At the age of 8, she was diagnosed with a cyst of the bile ducts, which was treated with cystoenteroanastomosis. At the age of 20, in connection with high portal hypertension, portocaval shunting was performed and a mesenteric-caval anastomosis was applied. During the present hospitalisation, an expansion of the intrahepatic bile ducts was revealed by ultrasound and MSCT of the abdominal cavity with contrast, which made it possible to diagnose Caroli disease.Conclusion. A case of Caroli disease is described, which resulted in continuously recurrent cholangitis and biliary cirrhosis. This state required liver transplantation. Caroli disease should be included in differential diagnosis in patients suffering from the fever of unknown origin and cholestasis syndrome. An early diagnosis of Caroli disease is important for preventing complications, improving the quality of life and increasing the life expectancy of patients suffering from this rare disease.

show abstract

Section: Discussionunclassified

unclassified

See 2 more Smart Citations

Caroli Disease (Clinical Observation)

Никитин

Карабиненко

Никитин

et al. 2019

Rossijskij žurnal gastroènterologii, gepatologii, koloproktolog

View full text Add to dashboard Cite

show abstract

“…Выделяют две формы заболевания [10]. Первая (простая, истинная или изолированная) -собственно болезнь Кароли, описываемая преимущественно у взрослых, характеризуется мешкообразным расширением главных внутрипечёночных протоков с более частым вовлечением отдельной доли или сегмента печени.…”

unclassified

Caroli syndrome in children: a brief review and clinical observation

Gudkov

Dmitriev

Slobodyanyuk

2023

jour

View full text Add to dashboard Cite

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.

show abstract