Wednesday, October 27, 2010

Hyperammonemia - if not Liver...what is it?

I have an interesting 50ish  patient in the Clinic with a history of 3 -4 admissions in the last 6 months due to episodes of altered mental status, confusion, lethargy which has made her stop driving(school bus) on one occasion. Her imaging was normal and her EEG showed diffuse slowing suggestive of a metabolic cause. Her ammonia(NH3) level was 110 with a normal liver function test and no clinical signs of chronic liver disease. She improved in 24-48 hours with lactulose. She is not an alcoholic or smoker. Her BMI is 30, and she had bariatric surgery done 4 years ago. So what can we do for her? Well....first will continue on lactulose while we investigate....and advise to stop driving.


How to approach . Well it is tough when we have some one with symptomatic high ammonia levels with a normal Liver function. These are the differentials in this case ---


1.Medications - Sodium valproate ( by inhibiting carbamoyl phosphate synthetase in the urea cycle. can happen both in acute & chronic setting. Some of these patients do have an underlying enzyme defect which is now unmasked by valproate). Chemotherapy drugs are also implicated esp 5- Flurouracil. But the underlying malignancy can also cause high NH3 levels. So interpretation might be difficult in that situation.
2.Digestive & Urinary tract infections - Proteus, H.pylori, Corynebacterium, Klebsiella are urea splitting bacteria and can cause hyperammonemia.
3. Surgical procedures - Ureterosigmoidostomy(due to diffusion of NH3 into circulation), portosystemic shunts, lung & bone marrow transplant (mostly due to GI bleeding, total parenteral nutrition), and bariatric surgery( again due to unmasking of underlying enzyme defect due to a high protein diet)
4. Late onset enzymatic defeciency - The most common in adults would be Ornithine transcarbamoylase(OTCM) defeciency(X-linked), but other urea cycle defects can occur. Best way to start investigating is to get a Citrulline & arginine level. If citrulline is low.... its a defect of either Carbamoyl phosphate(CP) or Ornithine TCM. If high.....its a defect in arginosuccinate synthase. If normal go with arginine( check the diagram). 
5. Small bowel bacterial overgrowth(SBBO) -  more bacteria in the gut breaks down more protein and releases more NH3 . This is possible in patients with bariatric surgery(esp if its a bypass with a blind loop).


So...in our patient..we think it is related to her bariatric surgery. Going through the above list......2 possible mechanism could contribute. Underlying enzyme defect Vs SBBO. I have scheduled her for a Hydrogen breath test with Lactulose(for SBBO) and awaiting her citrulline levels. Hopefully we would be able to nail the culprit. In case its SBBO..she would need to go on antibiotics preferred one being Metronidazole or Rifaximin. Lets see!

26 comments:

  1. OTC deficiency is pretty unlikely unless she is markedly skewed in X chromosome lyonisation. Citrulline may be normal in CPS deficiency and the only real way of excluding it and NAGS deficiency is by sequencing. The best way to start investigating these is to order urine organic and amino acids whilst she is unwell (not blood citrulline and arginine). Best to manage her hyperammonemia with benzoate or phenylbutyrate in addition to lactulose, and don't be afraid to dialyse her.

    ReplyDelete
  2. Fasioliasis (Hepatica fasciola) excretes ammonia and blocks/degrades biliary channels. Early stages of Fasioliasis will show no/low liver compromise.

    Activated charcoal slurry will lower ammonia levels, without the adverse effects of Lactulose. Activated charcoal poultice over liver 4-8 hours will drastically reduce levels within minutes.

    ReplyDelete
  3. Carbaglu may be used to treat this.

    ReplyDelete
  4. I was curious if you ever thought of changing the page layout of your website?
    Its very well written; I love what youve got to say. But maybe you could a little more in the way of content so people could connect with it better.
    Youve got an awful lot of text for only having 1 or 2 images.
    Maybe you could space it out better?

    Also visit my web site: daily male enhancement

    ReplyDelete
  5. Great goods from you, man. I've be mindful your stuff previous to and you are just too fantastic. I really like what you have acquired right here, really like what you are saying and the best way wherein you are saying it. You make it entertaining and you continue to care for to stay it sensible. I can't wait
    to learn far more from you. That is actually a tremendous website.


    Have a look at my homepage men herbal supplements

    ReplyDelete
  6. This design is incredible! You definitely know how to keep a reader amused.
    Between your wit and your videos, I was almost moved to start my own blog (well, almost.
    ..HaHa!) Fantastic job. I really loved what you had to say, and more than that, how you
    presented it. Too cool!


    http://wiki.openwebfoundation.org/User:RamonaBel

    Also visit my blog ... Best Male Enhancement

    ReplyDelete
  7. Post writing is also a fun, if you know then you can write or else it is complex to write.


    Here is my page Side Effects Of Testosterone Supplements

    ReplyDelete
  8. You really make it seem so eaay with your presentation but I find this matter tto bee actually something whhich I think I would never understand.

    It seems too complicated and very broad foor me. I am looking
    forard for your next post, I'll try to get the hang of it!


    Here is my web blog; FarmVille 2 Escapada Rural Cheats

    ReplyDelete
  9. Cheap Hermes Bags Online

    Hey there would you mind stating which blog platform you're
    working with? I'm going to start my own blog soon but I'm
    having a hard time making a decision between BlogEngine/Wordpress/B2evolution and Drupal.
    The reason I ask is because your design and
    style seems different then most blogs and I'm looking for something completely unique.
    P.S My apologies for getting off-topic but I had to ask!

    ReplyDelete
  10. All of the jobs listed below can remain part time, supplement other work, or be parlayed
    into a full time venture. The clients that use the services of
    corporate banking include many major companies, financial institutions and also commercial state companies.

    In various online job opportunities there are jobs which are easily
    available.

    Take a look at my page ... jobs you can do from home

    ReplyDelete
  11. I blog often and I really thank you for your content.
    This great article has truly peaked my interest. I will take a
    note of your site and keep checking for new details about once
    per week. I opted in for your Feed as well.


    my webpage ... tips on saving mad money nvda

    ReplyDelete
  12. The antioxidant property in green tea has catechins which are known to be effective
    for various infection conditions and the associated damage caused by bacteria,
    etc. Doing a lot of small things, can make a huge difference in the quality of your
    life. The multi-packs of Naked Juice Superfood
    Smoothies are not available yet, but may definitely help cut back on the total cost if you develop
    a daily Superfood Smoothie habit.

    Look into my page :: Chris Ashenden click ()

    ReplyDelete
  13. Enhanced immunity, while always valued for preventing colds and infections is not the only
    benefit of deer velvet supplementation. I'm suspicious, so please provide details that correct procedures were
    followed regarding treatment of the foundation's finances.

    The effects of the spray are therefore felt much faster than with many other forms of administration such
    as tablets and capsules which must be absorbed through the digestive tract first.


    my website :: deer antler spray

    ReplyDelete
  14. Thank you for the good writeup. It in fact was a amusement account it.

    Look advanced to more delivered agreeable from
    you! By the way, how could we communicate?

    my web site - turku hotelli satamassa

    ReplyDelete
  15. Good ƿost. I learn something new and challenging on webѕites
    I stumbleupon everyday. It will аlways be eхciting to reawd through content from оthеr аuthors and use somethіng from other websites.

    ReplyDelete
  16. Creepy,frissons Grèce au lieu de accrocher à partir de seulement comment pirater
    compte facebook sans logiciel gratuitement. amuser pour
    vous-m�me pirater un compte facebook forum.



    my web-site: comment pirater un compte facebook gratuitement

    ReplyDelete
  17. Good day! Do you know if they make any plugins to help
    with SEO? I'm trying to get my blog to rank for some targeted keywords but I'm not seeing very good gains.
    If you know of any please share. Cheers!


    Visit my page :: citifinancialservicing.com (http://www.toplistar.com/)

    ReplyDelete
  18. Thank you for sharing your info. I really appreciate your efforts and I am waiting for your further write ups
    thanks once again.

    Have a look at my homepage; alta white teeth whitening reviews

    ReplyDelete
  19. Hi there, just wanted to tell you, I liked this article.
    It was funny. Keep on posting!

    Also visit my website - samurai siege hack

    ReplyDelete
  20. What's up to every one, the contents present at this website are in fact amazing
    for people knowledge, well, keep up the nice work fellows.


    Feel free to visit my web site; test port

    ReplyDelete
  21. Heya i am for the first time here. I found this board and I to find It really helpful & it helped me out a lot.
    I'm hoping to present one thing back and help others like you helped me.


    Also visit my page: where to buy twitter followers

    ReplyDelete
  22. It can be a blow to you as a new homeowner to find out that, after buying
    a house and taking it at face value, several expenses will have to
    be incurred, as you may have to replace the roof or have structural work done not long after you have moved in. You
    as a borrower will eventually be matched with a lender, if your mortgage broker is doing his or her work properly.
    They also offer financial advices to those who need it, especially to first time
    buyers.

    Here is my blog post - mortgage brokers calgary

    ReplyDelete
  23. A lot of people have commented that Frankfurt has the look and
    feel of an American city (hence the nicknames Bankfurt and Mainhattan); therefore a visit is not something you really want to prioritize.
    Although definitions may vary somewhat, reading can be defined as the act of interpreting visual symbols and deriving meaning
    from those symbols (letters, words, etc. The field
    of professional selling has changed dramatically since World War II.


    Also visit my page ... monster warlord cheats

    ReplyDelete
  24. Are you interested in acquiring the T-Mobile Samsung Vibrant
    Android Telephone? Vibration Down loadable polyphonic, MP3 ring tones are all the alert sorts that this gadget supports.
    So, you believe that you may have a Cheating Wife?


    Here is my web-site download dragon city game free

    ReplyDelete
  25. Fastidious response in return of this query with firm arguments and telling the whole thing on the
    topic of that.

    Also visit my blog post: George Washington book

    ReplyDelete