Cryptographic cirrhosis

[Marwan76]

Any one here have Cryptographic cirrhosis , how it was diagnosed . which specific Gens apart of KRT18 -KRT8 . and what kind of treatment , diet you follow

[AyrshireK]

Only me again - cryptogenic just means they don't know what has caused your cirrhosis (it purely means of obscure or uncertain origin). They don't test for it and the diagnosis arrives because they have ruled out all other causes and labelled it cirrhosis of unknown cause. There is no treatment for any form of cirrhosis, yes it's easier when you know the cause then you can amend lifestyle (if it's lifestyle related) or take medication if it's illness related. Other than that all you can do is follow looking after your liver guidance, eating healthy, maintaining a healthy weight and dealing with (treating) any side effects that having cirrhosis brings.

As regards the KRT-18 & KRT-8 a quick search of Wiki reveals "Keratin 18 is a type I cytokeratin. It is, together with its filament partner keratin 8, perhaps the most commonly found products of the intermediate filament gene family. They are expressed in single layer epithelial tissues of the body. Mutations in this gene have been linked to cryptogenic cirrhosis. Two transcript variants encoding the same protein have been found for this gene."

I found this page too which goes into the condition. :- ncbi.nlm.nih.gov/books/NBK5...

Katie

[Marwan76 in reply to AyrshireK]

thanks always Katies , i do agree with all what you said except that there might be difference between cryptogenic liver cirrhosis and idiopathic liver cirrhosis .

the first one the responsible Gens is identified , but the second one the Reason of the liver cirrhosis is unknown so far

[Hidden]

Hi,

Here is our information on Cryptogenic cirrhosis;

britishlivertrust.org.uk/in...

For all diet guidance we would suggest to discuss with the liver specialist as advice will depend on stage.

Best wishes

Trust1

[Kristian]

Yes, I ended up with a diagnosis of cryptogenic cirrhosis. However, this was mainly because they couldn't identify the specific cause. As Katie has said, cryptogenic just means of unknown cause. In some cases a cause does eventually become apparent over time. That was the case for me and it was eventually determined that I had PSC. H

No idea about the gene thing. Not something I ever recall appearing on any of my test results.

[Marwan76 in reply to Kristian]

Thanks Kristian for the Reply , do you IBD like Crohn's disease ?

how they diagnosed PSC , through MRCP ? if through MRCP , did they inform that your bile duct is dilated ?

[Kristian in reply to Marwan76]

My diagnosis came post transplant. They diagnosed PSC when they did the pathology on the liver after they took it out. Normally, the tell tale signs would be picked up on MRCP, but in my case they weren't. After they considered it Cryptogenic Cirrhosis I stopped having the MRCPs. Had I done so it may have shown up in later scans. I'll just never know, lol. Those signs could include dilation of the bile ducts or they may refer to seeing a "beading" pattern of dilated and narrow sections of ducts.

I did have a colonoscopy early on but this didn't pick up any IBD. However, since getting the PSC diagnosis post transplant I entered the annual screening programme and it turns out that I actually do have inflammatory bowel disease. Its not entirely clear whether its Crohns or UC yet. They are currently favouring Crohns given the sections of bowel that are mainly affected. However, there is a colitis associated with PSC that mimics closer to Crohns so it may still end up being that. For the moment though we're sticking with Crohns until things suggest otherwise.

[Marwan76 in reply to Kristian]

thanks , i did MRCP twice , one before 7 years and one more recently before 6 Month and they said bile duct looks normal , however one doctor told me not necessarily will be shown up through MRCP , you need biopsy to roll out PSC ,

may i ask you about your GGT . ALP level and how many years take from Diagnosis to transplant.

i was diagnosed with light degree of IBD , Crohns , later on said it is not Crohns , it is some light degree of inflammation ( nothing important ) .

do you think PSC may reverse after transplant , do you have any treatment for this ?

i am having ursofalk since 7 years and it bring down my GGT from 400 to almost normal level

[Kristian]

If the MRCP is inconclusive they may do a biopsy to see if there are any small changes there. The biopsy will show the degree of fibrosis if there is any and could also pick up some of the microscopic signs of what's called small duct PSC. If they are suspecting PSC, or trying to rule it out, then a biopsy is a routine step for them to take. It's also useful to see if it may be something else too, like Autoimmune hepatitis for example. If your ALP is raised, I'm not surprised they may investigate PSC as a possibility. That's a sensible thing to do. I should though point out its not easy to pick up and can be missed even on biopsies. I had 2 and neither picked up the PSC, but they are only small samples that are taken.

I had to smile when I read your comment on your colonoscopy. It's very similar to what was found on my first one. A small amount of inflammation was all that was picked up. So not thought diagnostic of anything. A small amount of inflammation is not that unusual apparently. I do though have to wonder if it was just very early stages, lol. You can never really tell, but do ask them to continue monitoring to see if it does develop further.

Transplant is pretty much curative for PSC. There is a chance that it can return afterwards, i think it's around 30% but I could be wrong on that. However that does mean that in the majority of cases it wont.

My ALP levels were usually between 250 and 350, occaisionally higher sometimes slightly lower. GGT was anything from around 250 to 750ish or thereabouts. They didnt really measure GGT that often though.

I was on URSO too and it did bring those level down. They never really got near normal but usually kept the ALP below 300 and ALT below 100. It never caused me any problems and in the main I think it may have helped stave off some of the more troublesome symptoms. I never really suffered with an itch problem, and I put that down to the URSO. It could though just be coincidence. URSO is known to lower liver blood tests but it's not clear if that just masks the problem or is actually beneficial. It doesnt really delay the end point by all accounts, but on balance I was glad I was taking it for what benefits it may have given. My results were certainly much higher when I wasnt taking it.

Hope that's helpful again.

[Marwan76]

thanks for the clarfication . it seems we have many shared things . except that my ALP was always normal during all the past years before and after treatment with URSO .

MRCP say no extra or intra dialation and normal calipar of common bile duct is 4 mm . its distal end not visualized .