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]
Entire Body System
- Inflammation
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]
ductular proliferation and fibrosis Canalicular cholestasis, absence of true ductular proliferation, periportal biliary metaplasia of hepatocytes, pronounced portal / lobular fibrosis, pronounced portal / lobular inflammation, hepatocellular necrosis [pathologyoutlines.com]
The histological features of acute alcoholic hepatitis—hepatocytonecrosis, hyaline degeneration, and acute inflammation—were present in 14 cases (group A) and absent in 19 (group B). [annals.org]
- Falling
“It is plausible that the mechanism underlying our findings is attenuated activity of the bile acid receptors FXR and TGR5, which resolves following the fall in sulfated progesterones and/or estrogens at delivery. [healio.com]
Once the ICP approaches the level of the mean systemic pressure, cerebral perfusion falls. The body’s response to a fall in CPP is to raise systemic blood pressure and dilate cerebral blood vessels. [en.m.wikipedia.org]
These measures may give some temporary relief, particularly before going to bed when the itch may ease enough to allow you to fall asleep. A bland moisturising cream may also give some temporary relief from itch. [patient.info]
This report covers the two severe and two mild forms of low GGT familial intrahepatic cholestasis that have been generally recognized, although researchers have begun to identify cases that fall in between these two extremes. [rarediseases.org]
Gastrointestinal
- Loss of Appetite
Other symptoms of Intrahepatic cholestasis of pregnancy (ICP) In addition to itching and jaundice, other symptoms include: general weakness, feeling of unwell and tiredness and easy fatigability there may be loss of appetite - some women complain of nausea [news-medical.net]
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]
They include consumption of large amounts of alcohol, loss of appetite, nausea, and vomiting. [merckmanuals.com]
- Failure to Thrive
The other more variable features included failure to thrive, bleeding diathesis, hypoproteinaemia, abnormal liver enzymes, prolonged coagulation profile, hyperammonaemia, hypergalactosaemia, multiple aminoacidaemia, elevated α-feto protein and urinary [ncbi.nlm.nih.gov]
[…] to thrive Cirrhosis within five to 10 years, with liver failure Liver cancer Gallstones in the gallbladder To tell if a patient has PFIC, some tests need to be done. [cincinnatichildrens.org]
Malabsorption leads to vitamin deficiency and eventually results in failure to thrive, growth deficiency, bleeding episodes such as repeated nosebleeds, an abnormal susceptibility to bruising, and rickets. [rarediseases.org]
PFIC1 is notorious for its extrahepatic symptoms and therefore is considered a systemic disease. [14] These manifestations include sensorineural hearing loss, persistent diarrhea, cholecystitis, pancreatitis, failure to thrive, short stature, elevated [ijpmonline.org]
- 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]
- Abdominal Distension
Besides intense itching on your feet and hands, less common symptoms can include liver enlargement and abdominal distension (an outward belly extension that's noticeably beyond your normal baby bump), yellowing of the skin and eyes (jaundice), nausea, [whattoexpect.com]
Liver, Gall & Pancreas
- Jaundice
Intrahepatic cholestatic jaundice of pregnancy presents as pruritus, abnormal liver biochemistry and increased serum bile acids. CASE PRESENTATION: A Sri Lankan girl presented with recurrent episodes of 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]
- 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]
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]
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]
- 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]
Skin
- Pruritus
These are deposited within the skin, causing intense pruritus. [flipper.diff.org]
[…] on pruritus as compared to UDCA monotherapy [ 42 ]. [ojrd.biomedcentral.com]
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]
The clinical features are maternal pruritus in the absence of a rash and deranged liver function tests, including raised serum bile acids. [journals.lww.com]
- Skin Lesion
Secondary skin lesions like excoriation marks may be observed, narrating a pruritic tale. [e-ijd.org]
* Clinical features * Pruritus :particulary on the palms of the hands and soles of the feet, is the primary clinical symptom of ICP and is not associated with any specific skin lesions. [flipper.diff.org]
Signs and Symptoms ICP has been linked to adverse maternal and fetal outcomes Main symptoms: Puritus - without evidence of skin lesions (Itching, particularly on the hands and feet (often is the only symptom noticed ) Dark urine color,steatorrhoea,Fatigue [slideshare.net]
For more on the inheritance of cholestasis of pregnancy see OMIM The differential for generalized itching without a primary skin lesion source includes [6,7] X erosis (dry skin) A lmost any drug Numerous systemic diseases including: Uremia Iron deficiency [peridocs.com]
lesions, which resolves completely in the following few days after delivery. [worldgastroenterology.org]
- 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]
Secondary skin lesions like excoriation marks may be observed, narrating a pruritic tale. [e-ijd.org]
- Skin Disease
Interleukin (IL)-31 is known to be involved in the pathogenesis of pruritic inflammatory skin diseases. [ncbi.nlm.nih.gov]
For more on the inheritance of cholestasis of pregnancy see OMIM The differential for generalized itching without a primary skin lesion source includes [6,7] X erosis (dry skin) A lmost any drug Numerous systemic diseases including: Uremia Iron deficiency [peridocs.com]
The rash of ICP is caused by scratching the intensely itchy skin. Jaundice, a yellowish discoloration of the skin and the whites of the eyes occurs in 10 percent to 15 percent of women with the disease. [dermatology.about.com]
The differential diagnosis of ICP with pruritus (and elevation of serum bile acid levels) only includes skin diseases and specific dermatoses of pregnancy, allergic reactions, renal pruritus and hematological disorders such as Hodgkin's disease and polycythemia [ojrd.biomedcentral.com]
- Skin Rash
You don't get a skin rash but you may get marks on your skin from where you have scratched. The itch can be all over, although it is often particularly bad on your hands and feet. It is often worst at night. Usually itch is the only symptom. [patient.info]
IHCP is a cholestatic disorder characterized by pruritus in the absence of a skin rash, with elevated aminotransferases and bile acid levels, occurring in the second or third trimester of pregnancy, and spontaneous relief of signs and symptoms two to [e-ijd.org]
Patients with ICP typically present in the third trimester with a characteristic pruritus pattern that involves the palms and soles in the absence of a skin rash. [journals.plos.org]
Neurologic
- Confusion
Various other names have been used for these disorders such as FIC1 deficiency for PFIC1 and BSEP deficiency for PFIC2, adding to the confusion. [rarediseases.org]
Unnecessary confusion about family planning after intrahepatic cholestasis of pregnancy. Contraception. 2012 Dec. 86(6):639-44. [Medline]. Hämäläinen ST, Turunen K, Mattila KJ, Sumanen M. [emedicine.medscape.com]
It is often accompanied by fever, and the clinical presentation can be confused with that of cholangitis. A careful medical history is essential to confirm a history of ethanol abuse or dependency. [clevelandclinicmeded.com]
Urogenital
- 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]
Symptoms Jaundice, dark urine, light-colored stools, and generalized itchiness are characteristic symptoms of cholestasis. [merckmanuals.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]
Treatment
TREATMENT The primary objective of pharmacologic treatment in ICP is to alleviate maternal symptoms and improve fetal outcome. Many drug agents have been used in the treatment of ICP. [flipper.diff.org]
Patients with intrahepatic cholestasis of pregnancy (ICP) benefit from ursodeoxycholic acid (UDCA) treatment. [ncbi.nlm.nih.gov]
Treatment of intrahepatic cholestasis of pregnancy (ICP). News-Medical, viewed 04 November 2022, https://www.news-medical.net/health/Treatment-of-intrahepatic-cholestasis-of-pregnancy-(ICP).aspx. [news-medical.net]
This article will review the epidemiology, clinical features, pregnancy complications, and treatment for ICP. [benthamscience.com]
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]
Maternal prognosis is usually good but can result in adverse fetal outcome. [e-ijd.org]
Maternal prognosis is good and symptoms resolve rapidly after delivery,accompanied by normalization of serum liver test. ICP recurs during subsequent pregnancies in 45-70% with varying severity of recurrent episodes. [flipper.diff.org]
Etiology
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]
We review the etiology, diagnosis, and management of this intriguing condition. [journals.lww.com]
OBJECTIVES: The etiology of intrahepatic cholestasis of pregnancy (ICP) involves environmental, hormonal and genetic factors. [ncbi.nlm.nih.gov]
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]
EPIDEMIOLOGY The incidence of ICP varies widely with geographical location and ethnicity. 1 to 2 pregnancies in 1000 are affected by cholestasis. it is most common in South America, particularly in Chile, where the early reports described an overall incidence [flipper.diff.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]
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]
None of these above mentioned pharmacotherapy has definitive protective role when it comes to preventing adverse fetal outcomes. [e-ijd.org]