The clinical presentation and outcome (mean follow-up 62.7 months) of the 16 identified SSC-CIP cases were reviewed.Cholestasis was the first sign of SSC-CIP. GGT was the predominant enzyme of cholestasis. [ncbi.nlm.nih.gov]

Case presentation A 48-year-old woman presented with an episode of acute biliary pancreatitis that was complicated by pancreatic abcess formation. After 3 months she had an episode of severe pyogenic (E. [doi.org]

Here we present a rare case of SSC due to gallbladder adenocarcinoma. [karger.com]

Later presentation includes abdominal pain, jaundice, pruritus, fever and bacterial cholangitis from ascending infection. [orpha.net]

• Regurgitation

  Regurgitation of bile acids from leaky bile ducts causes sclerosing cholangitis in Mdr2 ( Abcb4 ) knockout mice. Gastroenterology 127, 261–274 (2004). Download references Acknowledgements Charles P. [nature.com]

  The absence of the murine bile transporter, Mdr2 (Abcb4), caused regurgitation of toxic bile through leaky cholangiocyte tight junctions, resulting in PSC-like lesions. [abdominalkey.com]

• Jaundice

  Cholestatic jaundice as a paraneoplastic feature of secondary sclerosing cholangitis is rare in Hodgkin’s lymphoma. [jamc.ayubmed.edu.pk]

  Overall 70% of patients with sclerosing cholangitis had jaundice vs. 25% without sclerosing cholangitis (p CONCLUSIONS: Our results indicate that drugs can lead to bile duct injury visualized on imaging. [ncbi.nlm.nih.gov]

  Later presentation includes abdominal pain, jaundice, pruritus, fever and bacterial cholangitis from ascending infection. [orpha.net]

• Hepatomegaly

  Hepatomegaly (may also have splenomegaly at presentation). Later stages: signs of cirrhosis, portal hypertension or hepatic failure. [patient.info]

  ERCP) Hepatomegaly / splenomegaly – approx 50% of patients Investigations P-ANCA – a serum antibody is found in 60% of cases LFT’s: ↑ALP, ↑ bilirubin MRCP / ERCP – are able to visualise bile ductal abnormalities. [almostadoctor.co.uk]

  Additional symptoms include a general feeling of ill health (malaise); abdominal pain, especially the upper right portion of the abdomen; nausea; dark urine; light-colored stools; unintended weight loss, and/or abnormal enlargement of the liver (hepatomegaly [rarediseases.org]

  In an early multivariable analysis, hepatomegaly and a serum bilirubin level > 1.5 mg/dL were found to be independently associated with a poor prognosis in PSC. [clinicalgate.com]

  Hepatomegaly and splenomegaly are the most frequent findings at clinical examination at the time of diagnosis in PSC. Osteopenic bone disease is a complication of advanced PSC, although less frequent than that reported in PBC. [easl.eu]

• Scleral Icterus

  A 55-year-old morbidly obese male presented electively with a 2-week history of low back pain and scleral icterus for 2 days. He also described severe epigastric pain that worsened postprandially and a 13 kg weight loss over the previous month. [karger.com]

• Excoriation

  Skin findings are common and include cutaneous hyperpigmentation, excoriations resulting from pruritus, and xanthomata. [clinicalgate.com]

Taylor and Jinxing Yu, MRI in the workup of biliary tract filling defects, Journal of Magnetic Resonance Imaging, 37, 5, (1020-1034), (2013). Christoph Jüngst, Thomas Berg, Jun Cheng, Richard M. Green, Jidong Jia, Andrew L. [doi.org]

Patients presenting with elevated liver enzymes require a full diagnostic workup and the differential diagnosis of potential hepatopathies is wide. [journals.plos.org]

Immunological Workup Initial investigations involved a complete blood cell count (CBC) on a Sysmex XS-800i (Sysmex Co., Cobe, Japan) 5-part automated hematological analyzer, lymphocyte subset analysis by flow cytometry using BD Multitest 6-color TBNK [frontiersin.org]

• Escherichia Coli O157:H7

  The authors experienced an extremely rare case of secondary sclerosing cholangitis and portal hypertension developed as late complications of hemolytic uremic syndrome (HUS) owing to Escherichia coli O157:H7 in a 2-year-old boy. [ncbi.nlm.nih.gov]

• Ischemic Changes

  Discussion Compared with PSC, the natural history and prognosis of SSC maybe more favorable if recognition is prompt and appropriate therapy timely. 1 ; 2 Conservative treatment often fails in patients with advanced SSC, generally because of ischemic [scipedia.com]

BACKGROUND AND STUDY AIMS: We present ten patients who developed secondary sclerosing cholangitis following long-term treatment in an intensive care unit (ICU) between 1999 and 2004. [ncbi.nlm.nih.gov]

These abnormalities were considered not to be amenable for endoscopic or percutaneous transhepatic treatment. Antibiotic treatment was continued and cholangitis did not recur during 14 months of follow-up. [doi.org]

SSC-CIP was associated with worse prognosis; transplant-free survival was ∼40 months (mean).Because of its high rate of serious complications and unfavorable prognosis, it is imperative to diagnose SSC-CIP early and to differentiate SSC-CIP from other [ncbi.nlm.nih.gov]

] PSC can be classified into small-duct or large-duct types, which seems to affect prognosis: [ 14 ] Small-duct PSC has a better prognosis, with longer transplant-free survival. [patient.info]

Characteristic features and prognosis. Hepatobil Pancreat Dis Int. 2009; 8 : 312-5 [hepatmon.com]

Increased awareness and early detection of the disease and its complications is considered to be crucial to improve the poor prognosis. [dovepress.com]

He was diagnosed as definite eosinophilic cholecystitis with sclerosing cholangitis with unknown etiology. The possible etiology of sclerosing cholangitis was consequent fibrosis from previous eosinophilic infiltration in the bile duct. [ncbi.nlm.nih.gov]

Liver biopsy, performed to exclude other etiologies of liver failure, demonstrated biliary cholestasis. [karger.com]

Definition / general Much more common than primary sclerosing cholangitis Etiology Biliary obstruction (choledochal cyst, choledocholithiasis, chronic pancreatitis, extrahepatic biliary atresia, postoperative), infection (immunodeficiency states), ischemia [pathologyoutlines.com]

The volume focuses on new data about risk factors for PSC, its natural history of the disease, and the epidemiology and genetics of PSC, while providing a comprehensive overview on current and future therapies for PSC, management of cholangiocarcinoma [books.google.com]

Therapy (122) Genetics (116) Gastroenterology and Hepatology (115) Pharmacology (105) Cardiology, Angiology (100) Endocrinology, Metabolism (84) Respiratory Medicine (84) Dermatology (82) Psychiatry, Psychology, Sexology (65) Obstetrics, Gynaecology (63) Epidemiology [portal.mefanet.cz]

Epidemiology 75% of cases are associated with IBD (usually ulcerative collitis ). [almostadoctor.co.uk]

Summary Epidemiology Overall incidence is estimated at about 1/50,000 live births, with regional differences and higher incidences among populations with a higher consanguinity rate. [orpha.net]

Bullous diseases in Malaysia: epidemiology and natural history / B. A. Adam // Int. J. Dermatol. - 1992. -Vol. 31. - P. 42-45 3. Brenner, S. Drug-induced pemphigus / S. Brenner, A. Bialy-Golan, V. Ruocco // Clin. Dermatol. - 1998. - Vol. 3. [medmag.bsmu.by]

CONCLUSIONS: The development of secondary sclerosing cholangitis in patients who have received long-term treatment in an ICU is a rare event of unknown pathophysiology, but patients demonstrate characteristic findings on ERCP. [ncbi.nlm.nih.gov]

Sclerosing cholangitis following severe trauma: description of a remarkable disease entity with emphasis on possible pathophysiologic mechanisms. World J. Gastroenterol. 11, 4199–4205 (2005). 46. Jaeger, C. et al. [nature.com]

Internal Medicine (392) Paediatrics, Neonatology (367) Pathology and Forensic Medicine (316) Infectology (243) Surgery, Traumatology and Orthopaedics (241) Microbiology (214) Neurology (169) Physiology and Pathophysiology (164) Medical Chemistry and Biochemistry [portal.mefanet.cz]

Approximately 80% of patients with PSC have inflammatory bowel disease, more commonly ulcerative colitis than Crohn’s disease. 21 Pathophysiology and Natural History The pathophysiology of PSC is unclear, but there is evidence suggesting an autoimmune [clevelandclinicmeded.com]

Besides medicines and therapies to help prevent infections, other precautions are used. Children with SCID: are placed in isolation to help prevent the spread of infection should not get live vaccines. [kidshealth.org]

In most cases, treatment will include: Antibiotics to treat any current infections and prevent new infections Immunoglobulin replacement Restricted contact with other people to prevent exposure to new infections Bone marrow transplant to correct the immunodeficiency [chop.edu]

Can SCID be prevented in children? Since this disorder is inherited, parents of children with SCID should think about talking with a genetic counselor. The counselor can talk with you about genetic testing and family planning. [stanfordchildrens.org]

The aim of the study is to assess the efficacy of rituximab to prevent recurrence of PSC. [upload.umin.ac.jp]

Genetics (116) Gastroenterology and Hepatology (115) Pharmacology (105) Cardiology, Angiology (100) Endocrinology, Metabolism (84) Respiratory Medicine (84) Dermatology (82) Psychiatry, Psychology, Sexology (65) Obstetrics, Gynaecology (63) Epidemiology, Preventive [portal.mefanet.cz]