I recently started bloging on medical conditons patients present with and how they may or may not interfere with podiatric issues the patients complain of.

This week a young female (30's) patient presented with Primary Biliary Cirrhosis. Her chief complaint to me was bunion pain.

What is primary biliary cirrhosis? it is a chronic disease of the liver thought to be autoimmune in nature. The disease slowly destroys the bile ducts within the liver. It damages the liver and replaces it with scar tissue, the more scar tissue the more the liver is damaged and can't function properly.

There are 4 phases of the disease:

1) Preclinical lasts about 2-10 years. No symptoms, normal liver tests

2) Asymptomatic : could be indefinate, sometimes 2-20 years. This stage has abnormal liver tests. Incidental finding of elevated alkaline phosphatase is what commonly leads to the diagnosis of PBC in this phase. 40% of aymptomatic patients will develop symptpms in the next 6 years.

3) Symptomatic: abnormal liver tests, symptoms are present lasts 3-11 years.

4) Advanced: abnormal liver tests will live 0-2 years without a liver transplant.

What are some of the symptoms of the disease:

Fatigue, itching, metabolic bone disease, xanthomas, fat and vitamin malabsorption, jaundice, hperpigmentation.

Granted this is a very brief overview of the condtion, and it was the first visit for the bunion pain complaint.

I am not even sure at this point what stage of the disease process the patient is in.

For the purpose of discussion, Would anyone contemplate a bunion correction if the patient was a stage 2 or beyond? 

Any relevant concerns should be shared as this is for learning purposes.

Some might respond, I would call the MD and ask if this patient is a candidate for surgery and that would be an acceptable answer as well.

Dr. Bates no doubt will first consult his trusted Zaeir - please let us know what you found.

If there are any three year residents out there - please show off the knowledge you have learned in your medical rotations.

  • Comments (9)
  • bunion 6 days post opQuote:

    Very good thought because the lesions are caused by the same thing - iron! His condition was actually hemochromatosis, production of too much iron. It causes terrible end organ damage as well. The treatment is remarkably simple - blood draws to reduce the iron levels. Dr. Bates, you are always sharp. Do you have one to offer, a medical stumper?


    This is post- op day 3.  No oral temperature.    1/5 pain on  protected weight bearing.  

  • Very good thought because the lesions are caused by the same thing - iron! His condition was actually hemochromatosis, production of too much iron. It causes terrible end organ damage as well.

    The treatment is remarkably simple - blood draws to reduce the iron levels.

    Dr. Bates, you are always sharp. Do you have one to offer, a medical stumper?



  • Quote:

    The biggest organ in the body is the skin!

    But back to the PBC....I'm a bit far out of residency but I am known as a real medicine nerd so you hit on a subject I enjoy, Jeff.

    A patient with PBC has hypercholesterolemia. This is a different kind of high cholesterol than in what we see in the dietary-induced kind. These patients don’t run the same risks for atherosclerosis as the others (high fat diet ones.) BUT you have to watch out for cardiovascular problems in the PBC group. And I agree - be careful of the meds. No antifungals for them!

    There are some lower extremity clues that a patient has PBC.....anyone have any ideas?

    They can have xanthomata on their soles (also on tendon sheaths, peripheral nerves, knees,  …..lots of places.) The ones around the nerves can actually produce neuropathy!  Do you remember xanthelasmata? Those are the ones around the eyes. They can get pretty profound and look like yellow hanging drapes around the inner eyelids.

    Here's another one for you: I had a patient, a nurse, who came in with the skin of his legs and thighs looking like he had sprinkled cayenne pepper over himself quite liberally. He said he had several relatives who had the same thing and they didn't live to ripe old ages either.

    Any idea about what he might have and how to treat it?

    1.  Edema    2. Bilateral Shamberg's Purpura

  • My copy of Zier has LOTS of underlining with pencil in the Gastroenterology chapter.  And lots of poorly drawn mnemonic hieroglyphs/pictographs all made with pencil.  Stickman anatomy/pathology is best drawn with pencil.  I read this entire chapter word- for- word on several occasions.  Two of those occasions were at the times of my own personal gut issues.  I annotated McGlamry's texts over the past 2 decades for my own several classical foot/ankle pathologies in the same personal fashion.    My wife tells me I am narcissistic about it.

  • I just got off the phone with my residency partner from my first year of residency. I asked him if there was any concern with performing a bunionectomy with a patient with "PBC". Frank, who is now a gastroenterologist told me there really would be no problem unless "her numbers were messed up". He stated that PBC is generally a very slow onst and that it was unusual for my 32 year old patient to be diagnosed at her age.
    He said the concern is more in a patient with HIV+ He also stated there are specific medications that are to be looked out for Tylenol being one of them, but many of the others there was not a specific concern with regards to PBC.

    Still - I only saw the patient one time for this complaint, and its always a good idea to have a second visit before booking a surgery. Idn't that what PICA preaches?

    I must say the articles that I read had a much more serious tone than Frank did. Incidently, Frank the MD is doing very well. He just told me he was opening an Italian Restaurant.

    They better make good cannoli's. I love a good cannoli.

    Dr. Satterfield perhaps Zeir meant internal organ?
  • The biggest organ in the body is the skin!

    But back to the PBC....I'm a bit far out of residency but I am known as a real medicine nerd so you hit on a subject I enjoy, Jeff.

    A patient with PBC has hypercholesterolemia. This is a different kind of high cholesterol than in what we see in the dietary-induced kind. These patients don’t run the same risks for atherosclerosis as the others (high fat diet ones.) BUT you have to watch out for cardiovascular problems in the PBC group. And I agree - be careful of the meds. No antifungals for them!

    There are some lower extremity clues that a patient has PBC.....anyone have any ideas?

    They can have xanthomata on their soles (also on tendon sheaths, peripheral nerves, knees,  …..lots of places.) The ones around the nerves can actually produce neuropathy!  Do you remember xanthelasmata? Those are the ones around the eyes. They can get pretty profound and look like yellow hanging drapes around the inner eyelids.

    Here's another one for you: I had a patient, a nurse, who came in with the skin of his legs and thighs looking like he had sprinkled cayenne pepper over himself quite liberally. He said he had several relatives who had the same thing and they didn't live to ripe old ages either.

    Any idea about what he might have and how to treat it?

  • I found my Zier - its interesting that many of the chapters I highlighted certain parts. The Gastroenterolgy chapter is little to no high lighting.
    Since the book is open: Here is the trivia of the day: What is the largest organ in the body?
    According to my Zier - that would be the Liver. Makes you wonder where the expression "you have a big heart" came from....
  • Marc - all excellent points!!! Pretty much any medication that the patient would be given could also potentially worsen the condition. NSAIDS, pain killers, anesthesia.....
    Vitamin D levels need to be checked in these patients as well, but since I do not have any intention at this point of proceeding with surgical intervention the point is moot for the purpose of the surgery. (They still should be checked for the patients own health.)
  • Don't operate on a hepatically impaired patient!!!!!

    Don't take a clearance from the PCP!!!

    If the patient has a gastroenterologist or better yet a hepatologist then I might trust a clearence.  But Liver disease is not as easily partitioned into stages and MELD scores as literature would suggest.  These patients may be normal on Monday and in ICU on Wednesday.  Propofol, Tylenol, ethanol, a high protein diet or just a fluctuation in the disease process may cause very unfortunate effects.  Healing can be compromised by increased toxins (primarily but not exclusively ammonia), splenic congestion and chronic or acute hepatopulmonary syndrome and this type of crisis can then lead to a renal failure.  Even if labs are normal these values can fluctuate widely and cascade from one crisis to the next.

    You may want to consider a Pulmonary consult and/or pulmonary function testing to ensure that there is no developing hepato-pulmonary disease developing.