Guest guest Posted October 1, 2004 Report Share Posted October 1, 2004 Dear Sharon; You might try running the idea by your GI that it is possible that Mayo might consider that you have a version of PSC called " small-duct PSC " , in which the ERCP often looks quite normal. Here's a reference describing this version: Hepatology. 2002 Jun;35(6):1494-500. Small-duct primary sclerosing cholangitis: a long-term follow-up study. Angulo P, Maor-Kendler Y, Lindor KD. Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, MN 55905, USA. Some patients with inflammatory bowel disease (IBD) have chronic cholestasis and hepatic histology compatible with primary sclerosing cholangitis (PSC) but normal findings on cholangiography. These patients with small-duct PSC have remained largely unstudied. Our aim was to determine the prevalence and long-term outcomes of patients with small-duct PSC. Eighteen patients with small-duct PSC (7 female and 11 male patients; mean age, 39.9 +/- 15.3 years [range, 13-68 years]) seen over a 4-year period were matched blindly by age and sex to 36 patients with classic PSC and followed up for 32.5 years. Small- duct PSC represented 5.8% of patients (18 of 309) with sclerosing cholangitis. Subsequent endoscopic retrograde cholangiography (ERC) performed in 5 patients with small-duct PSC showed progression to typical PSC in 3 patients at 4, 5.5, and 21 years of follow-up. None of the patients with small-duct PSC but 4 of the patients with classic PSC developed hepatobiliary malignancy. There were 3 deaths (17%) or liver transplantations in patients with small-duct PSC (2 after progressing to classic PSC) and 15 (42%) in the classic PSC group. Survival free of liver transplantation was significantly greater in the small-duct than in the classic PSC group (P =.04). Compared with the general U.S. population, survival in patients with small-duct PSC was similar (P =.4) but significantly lower in patients with classic PSC (P <.001). In conclusion, small-duct PSC may represent an earlier stage of PSC associated with a significantly better long-term prognosis. Some patients, however, progress to classic PSC and/or end-stage liver disease with the consequent necessity of liver transplantation. PMID: 12029635 Best regards, Dave (father of (19); PSC 07/03; UC 08/03) Quote Link to comment Share on other sites More sharing options...
Guest guest Posted October 1, 2004 Report Share Posted October 1, 2004 Dear Sharon; You might try running the idea by your GI that it is possible that Mayo might consider that you have a version of PSC called " small-duct PSC " , in which the ERCP often looks quite normal. Here's a reference describing this version: Hepatology. 2002 Jun;35(6):1494-500. Small-duct primary sclerosing cholangitis: a long-term follow-up study. Angulo P, Maor-Kendler Y, Lindor KD. Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, MN 55905, USA. Some patients with inflammatory bowel disease (IBD) have chronic cholestasis and hepatic histology compatible with primary sclerosing cholangitis (PSC) but normal findings on cholangiography. These patients with small-duct PSC have remained largely unstudied. Our aim was to determine the prevalence and long-term outcomes of patients with small-duct PSC. Eighteen patients with small-duct PSC (7 female and 11 male patients; mean age, 39.9 +/- 15.3 years [range, 13-68 years]) seen over a 4-year period were matched blindly by age and sex to 36 patients with classic PSC and followed up for 32.5 years. Small- duct PSC represented 5.8% of patients (18 of 309) with sclerosing cholangitis. Subsequent endoscopic retrograde cholangiography (ERC) performed in 5 patients with small-duct PSC showed progression to typical PSC in 3 patients at 4, 5.5, and 21 years of follow-up. None of the patients with small-duct PSC but 4 of the patients with classic PSC developed hepatobiliary malignancy. There were 3 deaths (17%) or liver transplantations in patients with small-duct PSC (2 after progressing to classic PSC) and 15 (42%) in the classic PSC group. Survival free of liver transplantation was significantly greater in the small-duct than in the classic PSC group (P =.04). Compared with the general U.S. population, survival in patients with small-duct PSC was similar (P =.4) but significantly lower in patients with classic PSC (P <.001). In conclusion, small-duct PSC may represent an earlier stage of PSC associated with a significantly better long-term prognosis. Some patients, however, progress to classic PSC and/or end-stage liver disease with the consequent necessity of liver transplantation. PMID: 12029635 Best regards, Dave (father of (19); PSC 07/03; UC 08/03) Quote Link to comment Share on other sites More sharing options...
Guest guest Posted October 2, 2004 Report Share Posted October 2, 2004 Sharon - here's my responses: Yes, I take Ursodiol - 7, 300 mg capsules/day (I'm about 175 lbs). I've been taking it since mid-2001. I've been asymptomatic since mid 2001 (I was wrong about 2000). I was diagnosed in May 2000 via ERCP after a bout of chills, fever, URQ pain, dark urine, pale stools, jaundice and ER admission. For a couple of years I had had bouts of URQ pain (actually, a little more central). LFTs were significantly elevated (I don't have the results). The ERCP clearly showed my CBD to be severely strictured with typical (of PSC) beaded and corkscrew appearance. This was at a local Minneapolis hospital. The GI who did the ERCP was unable to dilate my CBD (too narrow). I did get pancreatitis from the ERCP. I don't recall being put on Urso at the time. Almost a year later I was admitted to the same ER, same ER Dr., same GI on call with the same symptoms (message 22220). LFTs were significantly elevated (total bili ~6, AST 170, ALT 220). This time the GI on call said I was " too complicated a patient " and sent me to Mayo (a fun midnight 2-hour ambulance ride). I told them they were unable to dilate my CBD and the specialist said " We can do it - we've got every tool every manufactured and have developed our own too " . The ERCP was successful this time and a stent was installed to keep the duct open. I did get pancreatitis again. The stent was removed 2 weeks later (another midnight ride to Mayo) due to severe URQ pain (no pancreatitis this time!). LFTs were normal. At that time, it was identified that my gallbladder was severely inflamed and enlarged. A couple of months later (another midnight ride to the ER - message 26079), my gallbladder was removed. I've been asymptomatic since. URQ pain without the fever and chills is what we usually refer to as " sludge blockage " , where the bile thickens in the narrow passages and causes the pain. It frequently resolves itself. You should make sure you're getting plenty of fluids. Urso thins the bile and eases passage through narrowed ducts and appears to have some protective action for colon cancer. Whether it (permanently) improves LFTs or not, this is a good effect. It's unlikely Mayo would misdiagnose you. They have extensive experience with PSC. is correct about the possibility of small-duct PSC. Hope this helps. Arne 53 - UC 9/77 - PSC 4/00 Alive and (mostly) well in Minnesota -----Original Message----- From: Sharon Lee Botch Hello, and Arne Arne in Minnesota wrote that his numbers are normal and he has been asymptomatic since mid 2000. Okay, Arne, are you on Urso? When you were diagnosed (Mayo?) did you have symptoms? High LFT's? Did you have an ERCP that showed blockage? This is what is throwing my new gastro doctor. I should be thrilled about what is happening, but it came as such a shock to hear that this doctor didn't think I have PSC after four years of being told at Mayo that I did. A couple of weeks ago I had what I call a " flare up " . I went through the night sweats, the pain in the upper right side, and lots of fatigue. Today, I'm feeling better. This has been happening since I was diagnosed at Mayo and now this doctor is telling me I don't even have PSC. After all that, I do want to thank everyone for answering my questions and the input. It's not like one can bring up PSC in normal conversation. It's good to have others who understand. Thanks, Sharon (60) PSC 06/00 Quote Link to comment Share on other sites More sharing options...
Guest guest Posted October 2, 2004 Report Share Posted October 2, 2004 Arne, am I reading this right? Seven thousand 300 mg of URSO? I ask because my son is on a supposed high dose of ONE thousand mg a day. [4 - 250 mg tablets] I don't want to assume that the 7 was a typo, with this disease I wouldn't be surprised to hear that you were taking a vastly different amount than my son, it just seems way out there. Lee > > Sharon - here's my responses: > > Yes, I take Ursodiol - 7, 300 mg capsules/day (I'm about 175 lbs). > I've > been taking it since mid-2001. I've been asymptomatic since mid 2001 > (I was > wrong about 2000). > > I was diagnosed in May 2000 via ERCP after a bout of chills, fever, URQ > pain, dark urine, pale stools, jaundice and ER admission. For a > couple of > years I had had bouts of URQ pain (actually, a little more central). > LFTs > were significantly elevated (I don't have the results). The ERCP > clearly > showed my CBD to be severely strictured with typical (of PSC) beaded > and > corkscrew appearance. This was at a local Minneapolis hospital. The > GI who > did the ERCP was unable to dilate my CBD (too narrow). I did get > pancreatitis from the ERCP. I don't recall being put on Urso at the > time. > > Almost a year later I was admitted to the same ER, same ER Dr., same > GI on > call with the same symptoms (message 22220). LFTs were significantly > elevated (total bili ~6, AST 170, ALT 220). This time the GI on call > said I > was " too complicated a patient " and sent me to Mayo (a fun midnight > 2-hour > ambulance ride). I told them they were unable to dilate my CBD and the > specialist said " We can do it - we've got every tool every > manufactured and > have developed our own too " . The ERCP was successful this time and a > stent > was installed to keep the duct open. I did get pancreatitis again. > > The stent was removed 2 weeks later (another midnight ride to Mayo) > due to > severe URQ pain (no pancreatitis this time!). LFTs were normal. At > that > time, it was identified that my gallbladder was severely inflamed and > enlarged. > > A couple of months later (another midnight ride to the ER - message > 26079), > my gallbladder was removed. I've been asymptomatic since. > > URQ pain without the fever and chills is what we usually refer to as > " sludge > blockage " , where the bile thickens in the narrow passages and causes > the > pain. It frequently resolves itself. You should make sure you're > getting > plenty of fluids. Urso thins the bile and eases passage through > narrowed > ducts and appears to have some protective action for colon cancer. > Whether > it (permanently) improves LFTs or not, this is a good effect. > > It's unlikely Mayo would misdiagnose you. They have extensive > experience > with PSC. is correct about the possibility of small-duct PSC. > > Hope this helps. > > > Arne > 53 - UC 9/77 - PSC 4/00 > Alive and (mostly) well in Minnesota > > > -----Original Message----- > From: Sharon Lee Botch > > > Hello, and Arne > > Arne in Minnesota wrote that his numbers are normal and he has been > asymptomatic since mid 2000. Okay, Arne, are you on Urso? When you > were > diagnosed (Mayo?) did you have symptoms? High LFT's? Did you have an > ERCP > that showed blockage? This is what is throwing my new gastro doctor. I > should be thrilled about what is happening, but it came as such a > shock to > hear that this doctor didn't think I have PSC after four years of > being told > at Mayo that I did. A couple of weeks ago I had what I call a " flare > up " . > I went through the night sweats, the pain in the upper right side, and > lots > of fatigue. Today, I'm feeling better. This has been happening since > I was > diagnosed at Mayo and now this doctor is telling me I don't even have > PSC. > After all that, I do want to thank everyone for answering my questions > and > the input. It's not like one can bring up PSC in normal conversation. > It's > good to have others who understand. Thanks, Sharon (60) PSC 06/00 > > > > > > > > Quote Link to comment Share on other sites More sharing options...
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