The Distinctiveness of Liver Disease Care Managing PBC

 

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The Uniqueness of Liver Disease Care: Managing PBC. April Morris has spent 13 years working with liver disease patients. From being a sub-investigator on several clinical trials, to developing medications to treat and improve various liver conditions, she’s immersed in everything liver health. When you step into a career as a health care professional, there are many paths you can take.

Abstract. Primary biliary cholangitis (PBC) is a chronic, cholestatic, autoimmune disease with a variable progressive course. PBC can cause debilitating symptoms including fatigue and pruritus and, if left untreated, is associated with a high risk of cirrhosis and related complications, liver failure, and death. Although there’s no cure for PBC, treatments can improve your symptoms and protect your liver from further damage. The first treatment doctors usually try is ursodeoxycholic acid (UDCA) or.

The management of liver-related complications in PBC is the same as the management for patients with advanced liver disease related to other types of chronic liver diseases. For example, screening for esophageal varices and HCC as well as their management in cirrhotic patients with PBC should be carried out according to the AASLD and ACG guidelines ( 5 ). A new staging system for PBC was recently proposed, based on the assessment of chronic cholangitis and hepatitis activity [58–60]. This approach identifies four different stages by attributing a score of 0–3 to three histologic components: fibrosis, bile duct loss and deposition of orcein-positive granules. Primary Biliary Cholangitis (PBC; formerly known as primary biliary cirrhosis) is an important but uncommon disease that predominantly affects women.

It is a globally recognized autoimmune cholestatic liver disease with several characteristics. Most importantly: cholestasis, serologic reactivity to antimitochondrial. PBC is a chronic cholestatic disease with a pro-gressive course that may extend over many decades.

The rate of progression varies greatly among individ-ual patients. Over the past decades, there have been many changes in the diagnosis and management of PBC. More patients are being recognized with earli Primary biliary cirrhosis (PBC) is a disease of the liver in which the bile ducts slowly become destroyed. A fluid produced by the liver, bile aids in digestion and helps rid the body of worn-out.

In November 2018, the American Association for the Study of Liver Diseases (AASLD) published an update to the 2009 practice guideline on the Diagnosis, Staging, and Management of Primary Biliary Cholangitis (PBC). 1 The 2018 updated guidance on PBC includes updates on etiology and diagnosis, the role of imaging, clinical manifestations, and treatment of PBC since 2009. The AASLD 2018 PBC Guidance. People with PBC are at elevated risk of developing osteoporosis as compared to the general population and others with liver disease.

Screening and treatment of this complication is an important part of the management of PBC. As in all liver diseases, consumption of alcohol should be restricted or eliminated.

List of related literature:

Approximately 60% to 70% of patients with PBC respond to UDCA with improvement in serum AP and bilirubin levels and a delay in the progression to cirrhosis and liver transplantation.

“Miller's Anesthesia, 2-Volume Set E-Book” by Michael A. Gropper, Ronald D. Miller, Lars I. Eriksson, Lee A Fleisher, Jeanine P. Wiener-Kronish, Neal H Cohen, Kate Leslie
from Miller’s Anesthesia, 2-Volume Set E-Book
by Michael A. Gropper, Ronald D. Miller, et. al.
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These latter findings provide a mechanism to understand the biliary specificity of PBC, the recurrence of disease following liver transplantation, the success of ursodiol in treating PBC, and emphasize a critical role of the innate immune system in the perpetuation of this autoimmune disease.

“Liver Immunology: Principles and Practice” by M. Eric Gershwin, John M. Vierling, Michael P. Manns
from Liver Immunology: Principles and Practice
by M. Eric Gershwin, John M. Vierling, Michael P. Manns
Springer International Publishing, 2013

Increased body mass index, visceral adiposity, and coexisting IR and hepatic steatosis may also increase the risk of fibrosis progression, and reduce response to IFN.

“Desk Encyclopedia of Microbiology” by Moselio Schaechter
from Desk Encyclopedia of Microbiology
by Moselio Schaechter
Elsevier Science, 2010

Hepatic steatosis has also been associated with the use of parenteral nutrition as exhibited by elevation of serum aminotransferase values, alkaline phosphatase, and bilirubin levels.

“Nutrition in the Prevention and Treatment of Disease” by Carol J. Boushey, Ann M. Coulston, Cheryl L. Rock, Elaine Monsen
from Nutrition in the Prevention and Treatment of Disease
by Carol J. Boushey, Ann M. Coulston, et. al.
Elsevier Science, 2001

Lin47 observed that all 20 patients who were treated with IFN underwent remission, whereas 50% of patients in the control group had persistent nephrotic syndrome and 50% had mild proteinuria.48 (Patients in both groups were HBV positive.)

“Comprehensive Pediatric Nephrology E-Book” by Denis F. Geary, Franz Schaefer
from Comprehensive Pediatric Nephrology E-Book
by Denis F. Geary, Franz Schaefer
Elsevier Health Sciences, 2008

Approximately 10% of PBC patients have an AIHPBC overlap syndrome with elevation of serum aminotransferases together with markers of cholestasis, increased both of IgG and IgM levels, presence of AMA-M2 and histological findings compatible with AIH, including moderate to severe interface hepatitis.

“Liver: A Complete Book on Hepato-Pancreato-Biliary Diseases E-Book” by Mamun-Al Mahtab
from Liver: A Complete Book on Hepato-Pancreato-Biliary Diseases E-Book
by Mamun-Al Mahtab
Elsevier Health Sciences, 2012

Prevention of hepatitis B virus recurrence after liver transplantation in cirrhotic patients treated with lamivudine and passive immunoprophylaxis.

“Transplantation of the Liver E-Book” by Ronald W. Busuttil, Goran B. Klintmalm
from Transplantation of the Liver E-Book
by Ronald W. Busuttil, Goran B. Klintmalm
Elsevier Health Sciences, 2014

In the last cirrhotic stage of PBC features of advanced liver disease, such as portal hypertension, jaundice, ascites, porto-systemic encephalopathy, and upper digestive bleeding, are predominant as with liver cirrhosis from any cause.

“The Autoimmune Diseases” by Noel R. Rose, Ian R. Mackay
from The Autoimmune Diseases
by Noel R. Rose, Ian R. Mackay
Elsevier Science, 2013

Longterm survivors of HCT with chronic liver disease may progress to cirrhosis given the interplay of cancer therapy, immunosuppression, and liver disease etiologies [2].

“Schiff's Diseases of the Liver” by Eugene R. Schiff, Willis C. Maddrey, Michael F. Sorrell
from Schiff’s Diseases of the Liver
by Eugene R. Schiff, Willis C. Maddrey, Michael F. Sorrell
Wiley, 2011

Because patients with CN have good hepatic function other than conjugating bilirubin, they are ideal candidates for auxiliary liver transplantation.

“Nathan and Oski's Hematology of Infancy and Childhood E-Book” by Stuart H. Orkin, David G. Nathan, David Ginsburg, A. Thomas Look, David E. Fisher, Samuel Lux
from Nathan and Oski’s Hematology of Infancy and Childhood E-Book
by Stuart H. Orkin, David G. Nathan, et. al.
Elsevier Health Sciences, 2008

Oktay Kutluk

Kutluk Oktay, MD, FACOG is one of the world's foremost experts in fertility preservation as well as ovarian stimulation and in vitro fertilization for infertility treatments. He developed and performed the world's first ovarian transplantation procedures as well as pioneered new ovarian stimulation protocols for embryo and oocyte freezing for breast and endometrial cancer patients.

Mail: [email protected]
Telephone: +1 (877) 492-3666

Biography: https://medicine.yale.edu/profile/kutluk_oktay/
Bibliography: oktay_bibliography

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