Progressive familial intrahepatic cholestasis in children

Sevinç, Eylem; Ağın, Mehmet; Doğan, Erkan

doi:10.33582/2637-4501/1003

Ann Gastroenterol Dig Syst

2018

DOI: 10.33582/2637-4501/1003

|View full text |Cite

Progressive familial intrahepatic cholestasis in children

Eylem Sevinç¹,

Mehmet Ağın²,

Erkan Doğan³

Abstract: Progressive familial intrahepatic cholestasis (PFIC) is a chronic condition that begin in childhood and leads to cirrhosis and liver failure in the first years of life. It can be difficult to distinguish from other forms of cholestatic liver diseases. Treatment of PFIC includes medical and surgical methods. This study aimed to review PFIC in light of the recent studies.

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...

Citation Types

Supporting

Mentioning

Contrasting

Year Published

2021

Publication Types

Select...

Other1

Relationship

Self Cite0

Independent1

Authors

Journals

Cited by 1 publication

References 25 publications

(29 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

Клинический Случай Синдрома Байлера У Ребенка

Сергиенко¹,

Романова²,

Ключарева³

et al. 2021

Педиатрия. Восточная Европа

View full text Add to dashboard Cite

Прогрессирующий семейный внутрипеченочный холестаз (ПСВХ) – это редкая форма наследственной патологии печени, которая обусловлена дефектами секреции желчи, непрерывно прогрессирует и способствует формированию фиброза и цирроза печени. Частота данной патологии составляет 1 на 50 000–100 000 новорожденных. Начинается заболевание, как правило, в младенчестве, но прогрессирование процесса может быть и в подростковом возрасте. Выделяют три типа ПСВХ: при ПСВХ 1-го и 2-го типа нарушается секреция солей желчных кислот, а при ПСВХ 3-го типа изменена экскреция фосфолипидов. Диагностика основана на клинических проявлениях, результатах холангиографии, УЗИ, гистологии печени и специфических тестах. Терапия урсодезоксихолевой кислотой должна быть начата во всех случаях для предотвращения прогрессирования повреждения печени. Тем не менее, большинство пациентов с ПСВХ в конечном итоге являются кандидатами на трансплантацию печени. Таким образом, ранняя диагностика ПСВХ позволяет своевременно определять тактику ведения пациентов с целью предотвращения необратимого исхода заболевания. Progressive familial intrahepatic cholestasis (PFIC) is a rare form of hereditary diseases of the liver, which (due to defects in bile secretion) constantly progresses and promotes the formation of liver fibrosis and cirrhosis. Its incidence is estimated as 1 to 50.000–100.000 newborns. The onset of the disease is generally in infancy, but the diagnosis may be delayed until adolescence. Three types of PFIC are distinguished: in PFIC types I, II, the secretion of bile salts deteriorates, whereas in PFIC type III, the excretion of phospholipids is altered. Diagnosis is based on clinical manifestations,cholangiography, liver ultrasonography, and histology, as well as on specific tests. Ursodeoxycholic acid therapy should be initiated in all patients to prevent the progression of liver damage. However, the majority of PFIC patients are ultimately candidates for liver transplantation. Thus, early diagnosis of PFIC allows timely determination of patient management tactics in order to prevent an irreversible outcome of the disease.

show abstract

Клинический Случай Синдрома Байлера У Ребенка

Сергиенко¹,

Романова²,

Ключарева³

et al. 2021

Педиатрия. Восточная Европа

View full text Add to dashboard Cite

show abstract

scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.

Contact Info

hi@scite.ai

10624 S. Eastern Ave., Ste. A-614

Henderson, NV 89052, USA

Blog Terms and Conditions API Terms Privacy Policy Contact Cookie Preferences Do Not Sell or Share My Personal Information

Made with 💙 for researchers

Part of the Research Solutions Family.

Progressive familial intrahepatic cholestasis in children

Cited by 1 publication

References 25 publications

Клинический Случай Синдрома Байлера У Ребенка

Клинический Случай Синдрома Байлера У Ребенка

Contact Info

Product

Resources

About