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Intrahepatic Cholestasis

Biliary Stases Intrahepatic


Presentation

  • We report a kindred of Anglo-Celtic descent, among whom many members present with ICP, gallstones or cholestasis related to use of oral contraception.[ncbi.nlm.nih.gov]
Increased Energy
  • CONCLUSIONS: Increased levels of vitamins B1 and B2 may indicate a need for increased energy metabolism at the fetus.[ncbi.nlm.nih.gov]
High Fever
  • A 63-year-old man had a week-long history of intermittent high fever and rapidly progressive jaundice with atypical erythema. The results of liver function tests were recorded.[ncbi.nlm.nih.gov]
Clay-Colored Stool
  • Jaundice and Intrahepatic cholestasis of pregnancy (ICP) The increased build-up of bilirubin leads to jaundice, dark colored urine and pale white clay colored stool. This may be seen in very few severe cases of ICP.[news-medical.net]
Left Ventricular Dysfunction
  • CONCLUSIONS: Fetuses of ICP cases have significant left ventricular dysfunction. Mod-MPI can be used in the prediction of adverse perinatal outcomes in ICP.[ncbi.nlm.nih.gov]
Jaundice
  • Painless jaundice is usually the first sign of tumor. METHOD: We present a patient with a CC (Klatskin tumor) with a complete biliary drainage by an aberrant bile duct without jaundice.[ncbi.nlm.nih.gov]
  • Jaundice and Intrahepatic cholestasis of pregnancy (ICP) The increased build-up of bilirubin leads to jaundice, dark colored urine and pale white clay colored stool. This may be seen in very few severe cases of ICP.[news-medical.net]
  • Intrahepatic cholestasis of pregnancy Also known as recurrent cholestasis of pregnancy, obstetric cholestasis, cholestasis of pregnancy, recurrent jaundice of pregnancy, cholestatic jaundice of pregnancy, idiopathic jaundice of pregnancy, prurigo gravidarum[dermcoll.edu.au]
Recurrent Jaundice
  • Intrahepatic cholestasis of pregnancy Also known as recurrent cholestasis of pregnancy, obstetric cholestasis, cholestasis of pregnancy, recurrent jaundice of pregnancy, cholestatic jaundice of pregnancy, idiopathic jaundice of pregnancy, prurigo gravidarum[dermcoll.edu.au]
  • Description DEFINITION Intrahepatic cholestasis of pregnancy ICP also known as Obstretic cholestasis or recurrent jaundice of pregnancy or Pruritus gravidarum, is a cholestatic disorder characterized by pruritus,elevated serum aminotransferases and bile[flipper.diff.org]
  • Keywords Bile Acid Primary Biliary Cirrhosis Primary Sclerosing Cholangitis Intrahepatic Cholestasis Serum Bile Acid Disease name and synonyms Intrahepatic cholestasis of pregnancy Obstetric cholestasis Recurrent jaundice of pregnancy Pruritus gravidarum[ojrd.biomedcentral.com]
  • Ben-hamou Recurrent jaundice caused by recurrent hypere-mesis gravidarum Gut, 25 (1984), pp. 1414-1415 [26.] AFG De Patger, Henegouwen GP Van Berge, Bokkel Huinink JA Ten, K.H. Brandt Familial bening recurrent intrahe-patic cholestasis.[elsevier.es]
Scleral Icterus
  • Scleral icterus and jaundice were observed at 27 weeks. Elevated bile acid levels confirmed a diagnosis of intrahepatic cholestasis of pregnancy, and ursodeoxycholic acid therapy was initiated.[ncbi.nlm.nih.gov]
Scleral Icterus
  • Scleral icterus and jaundice were observed at 27 weeks. Elevated bile acid levels confirmed a diagnosis of intrahepatic cholestasis of pregnancy, and ursodeoxycholic acid therapy was initiated.[ncbi.nlm.nih.gov]
Fetal Disorder
  • Thus, anti-inflammation therapy targeting Gpbar1/NF-κB pathway could be effective in suppressing bile acid-induced inflammation and alleviating ICP-associated fetal disorders. The Author (2016).[ncbi.nlm.nih.gov]
Pruritus
  • Abstract Intrahepatic cholestasis of pregnancy (ICP) is an uncommon disorder, which generally occurs in the second and third trimester of pregnancy with symptoms of pruritus.[ncbi.nlm.nih.gov]
Excoriation
  • There is no rash but the skin may show scratch marks (excoriations) or become yellow (jaundiced). This condition typically develops late in pregnancy and resolves within days after the baby is born.[dermcoll.edu.au]
  • These are called excoriations and may be complicated with bacterial infections as well. The itching sites may sometimes bleed due to excessive scratching. Due to chronic itching the nails of the patient may also appear shiny and smooth.[news-medical.net]
  • Related Signs and Symptoms In addition to severe itching, the signs and symptoms of intrahepatic cholestasis of pregnancy may include: Jaundice Rash due to scratching (excoriations) Loss of appetite (anorexia) Fatigue Light colored and greasy stools that[dermatology.about.com]
  • Diagnostic criteria included the following: 1) clinical presentation of severe itching without a rash (except for excoriation), with a pattern of distribution characteristic of ICP, which includes the hands and feet (mainly palms and soles) [ 24 ]; 2)[journals.plos.org]
Papule
  • […] delivery beginning at 36 weeks and the risk of expectant management continues to rise each week following 36 weeks gestation. [22] See also [ edit ] ICP Care ICP Support Cholestasis Cholestatic pruritus List of cutaneous conditions Pruritic urticarial papules[en.wikipedia.org]
Dark Urine
  • Associated manifestations include fatigue, loss of appetite, dark urine and pale stools. Hepatomegaly is also a common finding. Between episodes patients show no symptoms and the interval between attacks varies from months to years.[orpha.net]
  • Less common symptoms include dark urine, pale stools, pain under your ribs or shoulder blade, and some women get jaundiced.[community.babycenter.com]
  • Although itching, which may be mild or severe and is typically on hands and feet (although it can be anywhere on the body) is the main symptom of ICP, other things to look out for are dark urine and pale stools.[goodtoknow.co.uk]
  • Dark urine and pale stools can also be an indicator, as well as feeling unwell and not having much appetite. Some women develop jaundice (yellowing of the whites of the eyes and skin) but this is not common.[thenaturalparentmagazine.com]
Vaginal Bleeding
  • Initial contractions and vaginal bleeding subsided spontaneously, and corticosteroids were administered for fetal lung maturation. However, in utero fetal death occurred 9 hours after normal cardiotocography.[ncbi.nlm.nih.gov]

Workup

Anisocytosis
  • Red blood cell distribution width, an anisocytosis marker in a complete blood count, has been used as an inflammation marker in various diseases.[ncbi.nlm.nih.gov]
Erythrocytosis
  • Clinical investigation disclosed severe intrahepatic cholestasis, nephrotic syndrome, erythrocytosis and hypoglycemia. Diagnosis of systemic AL amyloidosis was established by percutaneous liver biopsy. Bone marrow biopsy showed 32% of myeloma cells.[ncbi.nlm.nih.gov]
First-Degree Atrioventricular Block
  • Moreover, four fetuses from the ICP group had a mechanical PR interval 150 ms, which is compatible with a first-degree atrioventricular block.[ncbi.nlm.nih.gov]

Treatment

  • After 2-3 weeks of treatment, ALT levels decreased by more than 50% of base line in 67/86 (77.9%) patients and normalized in 34/86 (39.5%), and TBA concentrations decreased in 28/81 (34.6%). Only one patient stopped the treatment before delivery.[ncbi.nlm.nih.gov]

Prognosis

  • Data were compared between good (n 21) (disease-free before 1 year old) and poor prognosis groups (n 19). Good prognosis patients had a higher urinary THBA proportion than poor prognosis patients [25.89% (3.45-76.73%) vs. 1.93% (0.05-48.90%)].[ncbi.nlm.nih.gov]
  • Prognosis The prognosis is generally good with a tendency for a reduction in the frequency of attacks with age. However, progression from BRIC to PFIC and cirrhosis has been reported in the literature, indicative of a clinical continuum.[orpha.net]
  • However, it may worsen malabsorption of vitamin K and does not alter liver function or fetal prognosis [12].[peridocs.com]
  • Maternal prognosis is usually good but can result in adverse fetal outcome.[e-ijd.org]

Etiology

  • OBJECTIVES: The etiology of intrahepatic cholestasis of pregnancy (ICP) involves environmental, hormonal and genetic factors.[ncbi.nlm.nih.gov]
  • The etiology is unclear, but it believed to have genetic, hormonal, and environmental components. Possible fetal complications include meconium staining of amniotic fluid, preterm labor, and unexplained sudden intrauterine demise.[benthamscience.com]

Epidemiology

  • This article will review the epidemiology, clinical features, pregnancy complications, and treatment for ICP.[benthamscience.com]
  • Epidemiology/Incidence : 0.3-0.5% among the general population with up to 15% incidence in Latin American countries. Incidence also increased in Hepatitis C infection (6-16%).[smfm.org]
  • Summary Epidemiology The prevalence of BRIC is unknown. Clinical description The first cholestatic episode can occur at any age but onset within the first two decades of life is most common.[orpha.net]
  • Epidemiology ICP varies geographically world wide but high incidence has been reported in the following countries: Araucanian Indian descent in Chile and Boliva - 6%-27.6% Sweden 1%-1.5% Women of Indian-Asian/Pakistain-Asian Orign - 1-2-1.5% Lower elsewhere[slideshare.net]
Sex distribution
Age distribution

Pathophysiology

  • In this review, we will describe recent advances in the understanding of this condition with a particular emphasis on how aspects of genetic and reproductive hormone involvement in pathophysiology have been elucidated.[ncbi.nlm.nih.gov]

Prevention

  • PRESENTATION: Here, we report for the first time a 24-year Japanese case of severe ICP diagnosed by typical symptoms, serum biochemical analysis, and treated with the administration of ursodeoxycholic acid which improved cholestasis and liver injury and prevented[ncbi.nlm.nih.gov]
  • Prevention : There are no preventative therapies or interventions available. Management: Preconception counseling: Risk of recurrence is between 50-60% in subsequent pregnancies after an affected index pregnancy.[smfm.org]
  • The difficulty in predicting and preventing unanticipated fetal death near term drives the obstetrician's desire to deliver infants before 38 weeks.[neoreviews.aappublications.org]

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