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Biliary Atresia
Published in Charles Theisler, Adjuvant Medical Care, 2023
Biliary atresia is also known as extrahepatic ductopenia and progressive obliterative cholangiopathy. It is a childhood disease of the liver in which one or more bile ducts are inflamed and blocked or abnormally narrow. This prevents bile from draining out of the liver into the intestines. It is one of the leading causes of cholestasis in a newborn, the foremost reason for cirrhosis and liver-related death in children. Biliary atresia first manifests during the first two to eight weeks of life with jaundice and pale (clay-colored) stools. Additional symptoms such as dark urine along with irritability and weight loss may also be present. Surgical intervention via Kasai portoenterostomy is the medical treatment of choice.1
Chronic Liver Disease
Published in Praveen S. Goday, Cassandra L. S. Walia, Pediatric Nutrition for Dietitians, 2022
Julia M. Boster, Kelly A. Klaczkiewicz, Shikha S. Sundaram
The term progressive familial intrahepatic cholestasis (PFIC) encompasses a group of rare genetic disorders characterized by abnormal bile synthesis or transport, leading to cholestasis. There are at least eight separate disorders classified as PFICs, several of which have been recently discovered and can be diagnosed by genetic testing. Treatment is supportive to manage complications of cholestasis, such as fat-soluble vitamin deficiency, pruritus, and growth failure. When medical management is inadequate, however, liver transplantation may be needed.
Disorders of the digestive tract
Published in Judy Bothamley, Maureen Boyle, Medical Conditions Affecting Pregnancy and Childbirth, 2020
Bile salts are a constituent of bile (see description of gall bladder). Cholestasis refers to reduced bile flow and excretion. In ICP there is a slowing down of the transport or recycling of bile acids that results in an accumulation of bile acids in the blood. In normal pregnancies there are low levels of bile acids in the fetal blood; however, when the woman has ICP, the levels of maternal bile acids rise and these cross the placenta and affect the fetus64.
Kuhuang injection exerts a protective effect by activating PPAR-γ in an in vitro model of chlorpromazine-induced cholestatic liver injury constructed by tissue engineering
Published in Pharmaceutical Biology, 2022
Qiao Wu, Zhongping Duan, Long Huang, Zhijie Li
Drug-induced liver injury (DILI) is a common clinical adverse drug reaction and an important factor in drug development failure and withdrawal (Kullak-Ublick et al. 2017). Cholestatic injury and mixed hepatocytic/cholestatic injury constitute the two main subtypes of DILI and may account for 50% of all DILI cases (Shen et al. 2019). Drug-induced cholestasis (DRIC) may manifest clinically as pruritus, malaise, darkened urine, and jaundice and is often asymptomatic in the early stages, eventually manifesting as elevated serum alkaline phosphatase (ALP) and γ-glutamyl transpeptidase (GGT) levels and possibly progressing to hyperbilirubinemia, which can lead to liver failure or even death in severe cases (Padda et al. 2011). At present, effective drugs for the clinical treatment of cholestasis are lacking. Ursodeoxycholic acid (UDCA) and obeticholic acid (OCA) are the clinically approved drugs for cholestasis and are recognized to have certain curative effects, but their therapeutic effect is limited, as there are large individual differences in their effects (Santiago et al. 2018). Thus, the treatment strategies for DRIC are not ideal. Therefore, it is necessary to develop new drugs for the treatment of cholestatic liver injury. Furthermore, traditional Chinese medicine has received increasing attention in the treatment of DRIC (Li et al. 2019; Wang et al. 2020; Hua et al. 2021).
Effect of the increase rate of blood lipid concentration during pregnancy on the adverse pregnancy outcomes: a cohort study of 1051 singleton pregnancy
Published in Gynecological Endocrinology, 2022
Xiaoping Yu, Jinfeng Gao, Yan Huang, Yufei Zou, Ying Huang, Tao Du, Ju Zhang
The study showed that the TG concentration in pregnant women with GDM, hypertension during pregnancy, and LGA in the early and late trimesters was higher than those in non-patients, and blood lipid concentration was generally higher among pregnant women with complications during pregnancy. It suggested that blood lipid concentration during pregnancy was associated with various pregnancy complications. A previous study has reported the risk of GDM in the high TG group in the first trimester is 3.86 times that of the low TG group [14]. Other studies have also shown that TG in the first, second, and third trimesters of pregnancy is closely related to GDM and hypertension in pregnancy; TC, HDL-C, and LDL-C in the third trimester are closely related to the risk of intrahepatic cholestasis of pregnancy [5]. Unlike other adverse pregnancy outcomes, the pathogenesis of intrahepatic cholestasis of pregnancy is unclear. Moreover, there is no unified international opinion on diagnosis and treatment, and it is difficult to implement effective preventive measures. It is helpful to play an early warning role through the correlation study of other biochemical examination indexes. In addition, Wu et al. [15] demonstrated that the TG concentration in the second trimester of non-advanced pregnant women aged 20–34 are also related to the occurrence of premature babies.
Intrahepatic cholestasis of pregnancy: from an obstetrician point of view
Published in Journal of Obstetrics and Gynaecology, 2022
Mohsen M. A. Abdelhafez, Karim A. M. Ahmed, Win Win Than, Dg Marshitah Pg Baharuddin, Fairrul Kadir, Saffree Jeffree, Mohammad Firdaus Hayati, Mohd Nazri Bin Mohd Daud, Aya M. Eldiastey, Kai Xin Tay
Physical examination will show lack of any primary skin lesions, which questioned including ICP among pregnancy dermatoses, only excoriation marks due to severe scratching, are detected. The mechanism behind the pruritus-induced cholestasis is not clearly understood, it was hypothesised to be due to direct pruritogenic effects of bile acids on the skin, others attribute it to an unknown substance released from damaged liver cells by the effect of accumulated bile into general circulation (Ghent et al. 1977). Other ICP symptoms may include nausea, anorexia and right hypochondrial pain. A tinge of jaundice will be observed in 10–15% of patients, usually following pruritus by 4weeks (Kondrackiene and Kupcinskas 2008), steatorrhoea, usually mild, can occur secondary to malabsorption of fat, very seldom for steatorrhoea to be that severe, to result in vitamin K deficiency. Some women may present with systemic manifestations of cholestasis as pale stool and dark urine in addition to becoming jaundiced (Williamson and Geenes 2014b).