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]

• Surgical Procedure

  procedures may be done: Inserting a long, thin tube with a balloon at the end to open up narrowing ( endoscopic balloon dilation of strictures) Placement of a drain or tube for major narrowing (strictures) of bile ducts Proctocolectomy (removal of colon [medlineplus.gov]

  The risk of infection is particularly high after you've had a surgical procedure to expand a badly scarred bile duct or remove a stone blocking a bile duct. Portal hypertension. [mayoclinic.org]

  On the fourth day following admission the patient suffered a haemorrhagic shock and underwent an emergency surgical procedure with a bleeding diverticulum on the posterior wall of the duodenum tightly adhering to the pancreas being found. [giornalechirurgia.it]

• Jaundice

  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]

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

  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]

• Liver Dysfunction

  However, chemotherapy was discontinued because of liver dysfunction. MRCP and ERCP demonstrated multiple stenoses of the hepatic ducts and the intrahepatic bile ducts. [jstage.jst.go.jp]

  Alteration or disturbance of mental status called hepatic encephalopathy caused by liver dysfunction that shunts blood away from the liver. Gallbladder cancer. Colon cancer when PSC occurs along with Inflammatory Bowel disorder. [medindia.net]

  In general, the more severe the skin, liver, or gut involvement, the less favorable the outcome. Total Parenteral Nutrition Total parenteral nutrition (TPN) is associated with liver dysfunction resulting in steatosis, cholestasis, and cirrhosis. [clevelandclinicmeded.com]

• 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]

• Arthralgia

  During the treatment, she was well and showed no major side effects except for alopecia and arthralgia. The tumor showed a tendency to reduction. However, chemotherapy was discontinued because of liver dysfunction. [jstage.jst.go.jp]

  There were no symptoms of sicca syndrome, mouth ulcers, photosensitivity, skin rashes, dysphagia, abdominal pain, diarrhoea, arthralgias, arthritis or eye problems.On examination she was icteric with presence of taut skin. [jdeonline.in]

  A trial of corticosteroids in patients with idiopathic granulomatous hepatitis who are symptomatic, with fever, myalgias, and arthralgias, may be helpful. Empirical antituberculous therapy should be considered before instituting corticosteroids. [clevelandclinicmeded.com]

• Alopecia

  During the treatment, she was well and showed no major side effects except for alopecia and arthralgia. The tumor showed a tendency to reduction. However, chemotherapy was discontinued because of liver dysfunction. [jstage.jst.go.jp]

  Langerhans cell histiocytosis choledocholithiasis sickle cell disease total parenteral nutrition Hodgkin disease primary intestinal T-cell lymphoma ( 9893108 ) cystic fibrosis postbiliary surgery ischemia (floxuridine) genetic diseases ichtyosis and scarring alopecia [humpath.com]

  Alopecia and skin rash may be present depending on the form (e.g. SCID due to gamma chain deficiency or SCID due to JAK3 deficiency; see this term). Patients are unable to produce specific antibodies after vaccination or natural infection. [orpha.net]

  Figure 2 Infant with Omenn's syndrome, showing alopecia, erythroderma, and varicella zoster lesions. Routine tests are often overlooked as an aid to diagnosing SCID. [jcp.bmj.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]

• Staphylococcus Aureus

  가장 흔한 원인균은 폐렴구균(Streptococcus pneumonia)과 포도상구균(Staphylococcus aureus)이다. 기타 폐 합병증 인플루엔자와 관련된 기타 폐 합병증으로는 만성 폐쇄성폐질환의 급성 악화 및 만성 기관지염과 천식의 악화 등의 합병증이 흔히 관찰된다. 소아에서 인플루엔자 감염이 크룹으로 나타날 수 있다[7]. [ekjm.org]

  Pseudomonas infection is associated with previous biliary manipulation while Staphylococcus aureus is associated with transcutaneous biliary procedures. [clinicaladvisor.com]

  In our cohort, organisms isolated included both gram- negative and gram-positive bacterium: Staphylococcus aureus ( n = 2), Klebsiella pneumonia ( n = 8), Pseudomonas aeruginosa ( n = 4), Burkholderia ( n = 1), Chryseobacterium ( n = 1). [frontiersin.org]

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]

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]

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]

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]

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

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]