Primary Biliary Cirrhosis
童永贤博士
肝胆胰外科及普通外科医生
内外全科医学士 (MBBS) • 医学硕士 (MMed) • 爱尔兰皇家外科医师学会会员 (MRCS (IRE)) • 爱丁堡皇家外科医师学会会员 (FRCSED) • 印度医学科学院院士 (FAMS)
What is Biliary Cirrhosis?
Biliary cirrhosis is a chronic condition characterised by the progressive destruction of the bile ducts within the liver. This destruction hampers bile transport, a fluid essential for absorbing fats and fat-soluble vitamins in the small intestine.
The accumulation of bile leads to inflammation and scarring (fibrosis) in the liver, which can progress to cirrhosis, where normal liver tissue is replaced by scar tissue, severely impacting liver function.
Causes and Risk Factors
This condition is categorised into two main types: primary biliary cirrhosis (PBC) and secondary biliary cirrhosis (SBC). The causes and risk factors vary significantly between both types.
Primary Biliary Cirrhosis (PBC)
PBC is primarily considered an autoimmune condition, though its exact cause remains unclear. Key factors include:
- Genetic predisposition: A family history of PBC or other autoimmune diseases can increase the risk.
- Environmental triggers: Certain infections, smoking, and toxic substance exposure can be potential triggers for PBC.
- Immune system dysfunction: The body’s immune system mistakenly attacks the cells lining the bile ducts, leading to inflammation and damage.
Secondary Biliary Cirrhosis (SBC)
SBC results from conditions that cause prolonged obstruction of the bile ducts, leading to bile accumulation and liver damage. Common causes include:
- Bile duct obstructions: These can be due to gallstones, cysts, or tumours blocking the bile ducts.
- Chronic biliary infection: Infections that affect the bile ducts can lead to scarring and obstruction.
- Surgical complications: Procedures involving the bile ducts may inadvertently cause damage or strictures, leading to SBC.
风险因素
Apart from the direct causes, several risk factors increase the likelihood of developing biliary cirrhosis:
- Age: PBC more commonly affects people between the ages of 30 and 60.
- Gender: Women are more likely to develop PBC than men.
- History of autoimmune diseases: Individuals with other autoimmune conditions, such as rheumatoid arthritis or thyroiditis, have a higher risk of PBC.
症状
Biliary cirrhosis can progress asymptomatically, often presenting no symptoms until the condition is in an advanced stage. When symptoms do appear, they may include:
Early Symptoms
Fatigue: A pervasive sense of tiredness that is not relieved by rest
Itching (Pruritus): Unexplained, persistent itching, typically without a rash, affecting various body parts.
Dry eyes and mouth: Symptoms that are part of the sicca syndrome, which can be associated with autoimmune disorders.
As the Disease Progresses
- Jaundice: Yellowing of the skin and whites of the eyes, resulting from the accumulation of bilirubin in the blood.
- Dark urine and pale stools: Changes in urine and stool colour due to altered bilirubin processing.
- Right upper quadrant pain: Discomfort or pain in the upper right part of the abdomen, near the liver.
Advanced Symptoms
- Swelling in the legs and abdomen: Caused by fluid accumulation due to reduced protein production by the liver and increased pressure in the abdominal blood vessels.
- Easy bruising and bleeding: As liver function declines, the production of proteins necessary for blood clotting decreases.
- Bone weakness: Osteoporosis can develop, increasing the risk of fractures.
诊断
Diagnosing biliary cirrhosis involves a combination of clinical evaluation, laboratory tests, and imaging studies to assess liver function and structure.
Clinical Evaluation
- Medical history: To identify risk factors, symptoms, and any history of liver disease or autoimmune disorders.
- Physical examination: Focuses on signs such as jaundice, skin changes, or liver enlargement.
Laboratory Tests
- Blood tests: Measure liver enzymes, bilirubin levels, and specific markers of autoimmune activity against bile duct cells.
- Immunoglobulin levels: Elevated IgM levels can be indicative of PBC.
- Liver function tests: Assess the liver’s ability to produce proteins and process waste products.
Imaging Studies
- Ultrasound: A method to visualise the liver and bile ducts, looking for signs of damage or obstruction.
- Magnetic Resonance Imaging (MRI): Offer detailed images of the bile ducts and can identify blockages or structural changes.
附加测试
Additional diagnostic procedures may be recommended to rule out other conditions or confirm the diagnosis, such as endoscopic retrograde cholangiopancreatography (ERCP), which visualises the bile and pancreatic ducts more directly.
Understanding the available treatment options for biliary cirrhosis aids in managing the condition and improving patient outcomes.
Non-Surgical Treatment Options
Non-surgical treatments focus on medication and lifestyle adjustments to manage symptoms and slow disease progression.
Medical Therapies
- liver support medication: This bile acid is often the first line of treatment, aiming to slow disease progression by improving bile flow.
- OCA: For patients who do not respond adequately to UDCA, OCA offers an alternative to improve liver enzyme levels.
- Immunosuppressive medications: These are used to manage autoimmune components of the disease, reducing inflammation and immune system activity.
Symptomatic Treatments
- Bile-removing medication: Specifically targets itching associated with bile accumulation.
- Fat-soluble vitamin supplements: Supplements for vitamins D, E, A, and K to counteract deficiencies due to impaired bile flow.
- Bone health medications: Treatments to prevent or manage osteoporosis, including calcium and vitamin D supplements.
Lifestyle Adjustments
- Regular monitoring: Essential for adjusting treatment plans based on the progression of the disease.
- Diet and exercise: A balanced diet and regular exercise support overall liver health.
Surgical Treatment And Other Procedures
Surgical treatments and procedures, including biliary drainage and liver transplantation, are considered for advanced cases or when non-surgical options are insufficient.
- Biliary Drainage Procedures: For secondary biliary cirrhosis caused by obstruction, surgical intervention may be necessary to remove or bypass blockages.
- Liver Transplantation: In cases where liver function is severely compromised, a liver transplant may be the most effective treatment, offering a chance for prolonged survival and improved quality of life.
Possible Complications
As the disease progresses, several complications may arise.
Portal hypertension
Increased blood pressure in the portal vein system could lead to varices and an increased risk of bleeding.
Liver failure
A severe reduction in liver function can lead to life-threatening complications.
Increased risk of liver cancer
Cirrhosis elevates the risk of developing liver cancer.
Liver failure
Reduced bone density, making bones more fragile and prone to fractures.
童永贤博士
高级顾问
肝胆胰外科及普通外科医生
MBBS（新加坡）• MMed（外科）• MRCS（爱尔兰）• FRCSEd（普通）• FAMS（外科）
唐永贤医生是一位接受过专科培训的肝胆胰外科及普通外科医生，已完成超过6000例外科手术。.
- 医学学士和外科学士
- 新加坡国立大学
- 爱尔兰皇家外科医学院院士
- 新加坡医学硕士（普通外科）
- 爱丁堡皇家外科医学院院士
- 新加坡医学科学院院士
他首先在新加坡中央医院肝胆外科接受了肝胆外科的专科培训。之后，他在韩国首尔大学医院盆唐分院完成了微创肝胆外科的临床研究员培训。.
唐医生在其所在的医院创建并率先开展了微创手术项目，用于治疗复杂的肝脏和胰腺疾病。他的手术曾多次在国家电视台进行现场直播。他的手术视频也曾在国际外科会议上展出。此外，他还率先将胶囊内镜应用于结直肠癌筛查。.
Thng医生曾在医院、医疗集群和卫生部等多个委员会任职。Thng医生曾担任卫生部以价值为导向的医疗服务（VDC）腹腔镜胆囊切除术临床医生工作组成员。.
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