[vc_row row_type=”row” use_row_as_full_screen_section=”no” type=”full_width” oblique_section=”no” text_align=”left” css_animation=””][vc_column][ultimate_heading main_heading=”Candidate brief” main_heading_color=”#000000″ sub_heading_color=”#000000″ main_heading_style=”font-weight:bold;” main_heading_font_size=”desktop:36px;”]

“This lady has presented to the acute medical unit feeling generally unwell.  Please examine her abdomen and then discuss your findings with the examiners.”

[/ultimate_heading][vc_empty_space image_repeat=”no-repeat”][vc_video link=”https://vimeo.com/138191653″][vc_empty_space image_repeat=”no-repeat”][vc_row_inner row_type=”row” type=”full_width” use_row_as_full_screen_section_slide=”no” text_align=”left” css_animation=””][vc_column_inner width=”1/4″][stat_counter icon_size=”32″ counter_title=”Station Time” counter_value=”20″ counter_suffix=” minutes” speed=”3″][/vc_column_inner][vc_column_inner width=”1/4″][stat_counter icon_size=”32″ counter_title=”Time for this encounter” counter_value=”10″ counter_suffix=” minutes” speed=”3″][/vc_column_inner][vc_column_inner width=”1/4″][stat_counter icon_size=”32″ counter_title=”Maximum time to examine your patient” counter_value=”6″ counter_suffix=” minutes” speed=”3″][/vc_column_inner][vc_column_inner width=”1/4″][stat_counter icon_size=”32″ counter_title=”Minimum time for discussion and questions” counter_value=”4″ counter_suffix=” minutes” speed=”3″][/vc_column_inner][/vc_row_inner][vc_empty_space image_repeat=”no-repeat”][/vc_column][/vc_row][vc_row row_type=”row” use_row_as_full_screen_section=”no” type=”full_width” oblique_section=”no” text_align=”left” css_animation=””][vc_column][vc_accordion style=”accordion”][vc_accordion_tab title=”Common examiner questions”][vc_column_text]Common examiner questions include the following:

  1. What do you think this patient has?
  2. How would you like to investigate this patient next?
  3. What do you think the underlying cause of this patient’s signs is?

[/vc_column_text][/vc_accordion_tab][vc_accordion_tab title=”Diagnosis and clinical signs”][vc_column_text]This patient has primary biliary cirrhosis.

The clinical signs exhibited in this video include:

  • Excoriations in the right antecubital fossa in keeping with pruritis, a feature of PBC;
  • Subtle evidence of jaundice;
  • Spider naevi;
  • 2cm hepatomegaly

Note is also made of subtle abdominal striae and an appendicectomy scar.[/vc_column_text][/vc_accordion_tab][vc_accordion_tab title=”Discussion”][vc_column_text]

Clinical Picture:

Primary biliary cirrhosis (PBC) is a chronic disease of the liver, likely autoimmune in nature that leads to progressive cholestasis and often end-stage liver disease. PBC is most frequently a disease of women and occurs between the fourth and sixth decades of life.

Pruritis is the most common complain accompanied by fatigue, right upper quadrant discomfort, jaundice, hepatomegaly, splenomegaly (late symptom), hyperpigmentation, Sicca Syndrome, osteomalacia due to fat-soluble vitamin deficiency and osteoporosis. Patients may present with skin xanthomas due to elevated serum cholesterol. In advanced disease state spider nevi, palmar eythema, ascites, temporal and proximal muscle wasting and oedema develop.

Initial Investigation:

Liver function tests are the initial investigation which shows elevated conjugated bilirubin, ALP and GGT suggesting cholestatic jaundice. Primary biliary cirrhosis also presents with increased serum IgM. Anti-mitochondrial antibody (AMA) is raised in 95% cases. Elevated bilirubin level, prolonged prothrombin time and decreased albumin level indicates progression of disease to cirrhosis. Thrombocytopenia indicates portal hypertension.
Definitive diagnosis is obtained by liver biopsy which shows lymphocytic infiltrate in portal tracts and granulomatous destruction of bile ducts.

Underlying Pathology:

PBC is a progressive, cholestatic liver disease of unknown cause, possibly autoimmune. It is characterized by destruction of intra-hepatic bile ducts and impaired regulation of both B and T lymphocytes. It is associated with some autoimmune diseases like Sjogren’s syndrome, Raynaud’s Phenomemon, coeliac disease and systemic sclerosis suggesting it’s an autoimmune-mediated disease.

Treatment:

Ursodeoxycholic acid slows down the progression of the disease hence it is the mainstay of treatment. Cholestyramine is the drug of choice for pruritis. For treating osteomalacia fat soluble vitamin supplementation is administered. Calcium and Vitamin D are advised for osteoporosis. Liver Transplantation is the definitive treatment once liver failure has developed.

Most common cause of death in patients with Primary Biliary Cirrhosis is liver failure.

[/vc_column_text][/vc_accordion_tab][vc_accordion_tab title=”Specimen case presentation”][vc_column_text]”This lady has stigmata of chronic liver disease including jaundice, hepatomegaly and spider naevi.  She also has excoriations in her antecubital fossa, implying pruritis.  Because of this I would speculate that she may have primary biliary cirrhosis as an underlying cause, although of course other causes need to be considered also.  I would like to investigate further with routine venous bloods and an abdominal ultrasound.”[/vc_column_text][/vc_accordion_tab][/vc_accordion][/vc_column][/vc_row][vc_row row_type=”row” use_row_as_full_screen_section=”no” type=”full_width” oblique_section=”no” text_align=”left” css_animation=””][vc_column][/vc_column][/vc_row]