CASO

Anamnesi | Un uomo di 30 anni si rivolge al medico curante perché da ormai troppo tempo avverte un prurito incoercibile. In anamnesi segnala che soffre di rettocolite ulcerosa che tratta ormai da anni. Il medico lo visita e annota che il paziente presenta clinicamente ittero, perdita di peso, dolorabilità addominale, alvo irregolare, astenia, affaticabilità e altri segni di malassorbimento. In particolare gli prescrive indagini sierologiche. Il paziente si presenta dal medico con i risultati delle analisi e il medico annota iper GammaGT, aumento fosfatasi alcalina, ipercolesterolemia, iperbilirubinemia coniugata. Non convinto chiede una colangiografia. Dopo numerose indagini di conferma non ha più dubbi e convoca il paziente per la poco felice diagnosi. Quale sarà ?


SOLUZIONE

Diagnosi | Colangite sclerosante primaria

La colangite sclerosante primaria è una patologia sclerotica delle vie biliari, probabilmente autoimmune, che clinicamente si associa spesso a malattie infiammatorie intestinali, specie la colite ulcerosa. Non è nota la ragione di questa associazione ma è probabile che il meccanismo patogenetico alla base della colangite sclerosante primaria veda coinvolta una reazione immuno mediata sia umorale che cellulare, forse a partenza delle cellule già coinvolte nell’autoimmunità della colite ulcerosa, le quali riconoscono specifici epitopi sui colangiociti delle vie biliari innestando un classico meccanismo di infiammazione sclerosante.  L’evento finale è che le vie biliari epatiche ed extraepatiche colpite vanno incontro a stenosi e fibrosi periportale dei dotti biliari. Quest’ultima è molto caratteristica all’istologico ed è chiamata ‘a buccia di cipolla’ per l’aspetto concentrico lamellare che assumono gli istiociti epitelioidi responsabili della reazione granulomatosa. La colangite sclerosante quindi è una malattia tipicamente colestatica. Tutti i sintomi sono infatti sintomi tipici della colestasi (es. il prurito, l’ittero colestatico etc.) così come i marker di laboratorio alterati (GGT, fosfatasi alcalina, colesterolo, bilirubina coniugata etc.). La colangite sclerosante inoltre si associa a maggior rischio di neoplasie, soprattutto i colangiocarcinomi e di pancreatiti croniche e carcinoma epatocellulare. Inoltre l’80% dei pazienti ha anticorpi anti-MPO ANCA che sono tipici ma non esclusivi della vasculite dei piccoli vasi detta sindrome di Churg-Strauss. Non si conosce ancora l’entità della patologia. Purtroppo ad oggi non c’è cura per la colangite sclerosante primaria ma una possibilità d’allievo dei sintomi e miglioria della colestasi è dato dalla somministrazione di acido ursodesossicolico responsabile della solubilizzazione del colesterolo e che abbassa la formazione di calcoli biliari (colelitiasi) molto comune nelle colestasi. Inoltre tale terapia abbassa anche il rischio di complicanze a lungo termine legate ad epatopatia cronica come ad es. l’ascite, l’ipertensione portale o la formazione di varici esofagee

Redazione | Nato a Torre del greco il 27/03/1992 5°anno facoltà di medicina e chirurgia Federico II Napoli Appassionato di molte discipline diverse la medicina rappresenta sicuramente la più importante. Non disdegno alcun settore ma le branche che più mi interessano sono quelle della diagnostica clinica, della dermatologia, della ricerca oncologica, della farmacologia, della pediatria e della medicina interna. La mia idea di medico è quella di un lavoro di gruppo che possa essere parte di un team multidisciplinare ben strutturato che garantisca ai pazienti un iter di cura completo ed efficace. Spero un giorno di poter fare esperienze anche in zone di guerra specialmente in medio oriente magari con MSF o Emergency

69 COMMENTI

  1. Good day! Do you know if they make any plugins to assist 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.

    Kudos!

  2. Can I just say what a comfort to uncover somebody who really understands what they’re talking about on the web.

    You actually know how to bring a problem to light and make it important.
    More people must look at this and understand this side of your story.

    I can’t believe you’re not more popular since you most
    certainly have the gift.

  3. Hi there, I found your web site via Google whilst searching for a similar subject, your
    web site got here up, it looks great. I have bookmarked it in my google
    bookmarks.
    Hello there, simply was alert to your weblog through Google, and located that it is truly informative.
    I am going to watch out for brussels. I will appreciate should you proceed this in future.
    Numerous people can be benefited from your writing.

    Cheers! plenty of fish natalielise

  4. May I just say what a comfort to discover somebody that truly
    understands what they’re discussing over the internet. You definitely understand how to
    bring an issue to light and make it important. A lot more people really need to
    check this out and understand this side of the story.
    I was surprised you are not more popular given that you definitely have the gift.

  5. I do accept as true with all the ideas you have presented on your post.
    They’re really convincing and will certainly work.
    Still, the posts are too quick for newbies. Could you please prolong them a little from subsequent time?

    Thanks for the post.

  6. Greetings from Colorado! I’m bored to death at work so
    I decided to check out your site on my iphone during lunch break.
    I love the knowledge you present here and can’t wait to take a look when I get
    home. I’m shocked at how fast your blog loaded on my mobile ..
    I’m not even using WIFI, just 3G .. Anyways, fantastic site!
    pof natalielise

  7. Please let me know if you’re looking for a writer for your weblog.
    You have some really good posts and I feel I would be a good asset.
    If you ever want to take some of the load off, I’d really
    like to write some articles for your blog in exchange for a link back to mine.
    Please blast me an e-mail if interested. Thanks!

  8. I think what you wrote was actually very logical. However,
    what about this? suppose you added a little content? I ain’t saying your content isn’t solid, however what if
    you added a title to possibly get a person’s attention? I mean Caso clinico #12 is a little boring.
    You could peek at Yahoo’s home page and watch how they write article
    titles to grab people to open the links. You might add a video
    or a related pic or two to grab readers interested about everything’ve got to say.
    Just my opinion, it could make your website a little livelier.

  9. Greetings from Los angeles! I’m bored to tears at work so I decided to check out your site on my iphone during lunch break. I love the info you present here and can’t wait to take a look when I get home. I’m surprised at how quick your blog loaded on my phone .. I’m not even using WIFI, just 3G .. Anyhow, fantastic blog!

  10. Do you mind if I quote a couple of your posts as long as I provide
    credit and sources back to your blog? My blog site is in the very same niche as yours and
    my visitors would certainly benefit from a lot of the information you provide here.

    Please let me know if this okay with you. Many thanks!

  11. Its like you read my mind! You appear to know a lot about this, like you wrote the book in it or something.
    I think that you could do with a few pics to drive the message home a little bit, but instead of that, this is wonderful blog.
    An excellent read. I’ll definitely be back.

  12. I just want to say I am very new to blogs and truly savored you’re web site. More than likely I’m likely to bookmark your website . You amazingly come with superb articles and reviews. Regards for sharing your webpage.

  13. Hello exceptional website! Does running a blog
    like this require a large amount of work? I’ve absolutely no knowledge of programming however I was hoping to start
    my own blog in the near future. Anyhow, should you have any ideas or tips for new blog owners please
    share. I understand this is off subject nevertheless I simply wanted to ask.
    Kudos!

  14. My spouse and I absolutely love your blog and find the
    majority of your post’s to be just what
    I’m looking for. Do you offer guest writers to write content for you personally?
    I wouldn’t mind publishing a post or elaborating on a few of the subjects you write in relation to here.
    Again, awesome website!

  15. It is perfect time to make a few plans for the future and it
    is time to be happy. I have learn this post and if I may just
    I desire to recommend you few fascinating things or tips.
    Perhaps you can write subsequent articles regarding this article.
    I desire to read more issues about it!

  16. Somebody necessarily help to make critically articles I would state.
    That is the very first time I frequented your web page and up to now?
    I amazed with the analysis you made to make this actual put up extraordinary.
    Excellent process!

LASCIA UN COMMENTO

Please enter your comment!
Please enter your name here