The only other Australian case, reported more than 20 years previously, had identical presentation, pathology and circumstances of occurrence. [unboundmedicine.com]

Clinical featuresClinical presentation is extremely variable : 16. [slideshare.net]

Thus, individuals with this autosomal-recessive disease present with signs and symptoms arising from impaired biliary copper excretion. [ommbid.mhmedical.com]

INR Sodium Childs-Pugh – click here to calulate the Childs-Pugh score Ascites Bilirubin Albumin PT Encephalopathy Initial management of chronic liver disease (CLD) Bloods FBC: normocytic normochromic anaemia with leukopenia and thrombocytopenia often present [oxfordmedicaleducation.com]

Increased arsenic levels are present in drinking water in some countries and may contribute to regional differences in incidence. [rarediseases.org]

• Movement Disorder

  Peripheral OedemaHepatic Encephalopathy 19. neurological presentationTends to occur in 2nd & 3rd decades or later3 main movements disorders : DYSTONIA 20. TREMOR AND INCOORDINATION 21. [slideshare.net]

  (twisting and repetitive movements of part of the body). [psychology.wikia.com]

• Pathologist

  Chicago: American Society of Clinical Pathologists Press, 1992:203-32. 12. Vazquez JJ, Moreno A. Liver biopsy and elementary hepatic lesions. In: Prieto J, Rodes J, Shafritz DA, eds. Hepatobiliary diseases. [nejm.org]

  Hence, rhodanine-stained slides were reviewed and graded by 2 pathologists (T.M. and T.C.S.) as follows: negative, no rusty red cytoplasmic granules in hepatocytes; grade 1, isolated periportal cells containing small sparse granules (not seen at low magnification [academic.oup.com]

• Joint Dislocation

  Yes, I have been reviewed by a dietician who said I was doing everything right, and I do physiotherapy and hydrotherapy but I have a chronic illness that makes most exercise dangerous for me because my joints dislocate (EDS). [healthunlocked.com]

• Excitement

  My docs were pretty excited about that one but I persistently test exactly middle of the good normal range, even of the free T3 and T4s. [healthunlocked.com]

[…] with seemingly intact, newly formed limiting plates Stage 3 (septal stage): Septal fibrosis with active inflammatory, passive paucicellular septa, or both are present Stage 4 (cirrhosis): Nodules with various degrees of inflammation are present See Workup [emedicine.medscape.com]

Treatment for Cirrhosis Cirrhosis cannot be cured but it can be managed. Treatments for Cirrhosis will depend on cause and complications, such as Wilson's disease which may be treated through chelation therapy to remove excess copper from the body. [medic8.com]

Edit Medical treatment Edit Various treatments are available for Wilson's disease. [psychology.wikia.com]

Treatments aimed at reversing the fibrosis are usually too toxic for long-term use (eg, corticosteroids, penicillamine ) or have no proven efficacy (eg, colchicine ). Other antifibrotic treatments are under study. [merckmanuals.com]

With early detection and treatment, most of those affected can live relatively normal lives. [en.wikipedia.org]

B6 & dimercaprolUsed as part of treatment 35. Evolution of Wilson disease 36. prevention GENETIC COUNSELLING recommended for people with family history of Wilson’s Disease 37. OTHER COPPER DISEASES1. Idiopathic Copper Toxicosis2. [slideshare.net]

Cienfuegos Maite Herraiz Jesus Prieto idiopathic adulthood ductopenia liver transplantation chronic cholestasis prognosis This is a preview of subscription content, log in to check access. Preview Unable to display preview. Download preview PDF. [springerlink.com]

[…] of disease Can be used for staging prior to consideration of liver transplantation Liver transplantation Complications of chronic liver disease (CLD) Variceal bleeding Ascites Spontaneous bacterial peritonitis Encephalopathy Hepatorenal syndrome HCC Prognosis [oxfordmedicaleducation.com]

Prognosis The most reliable determinants of patient prognosis in primary biliary cholangitis are the height of the serum bilirubin level and the Mayo risk score. [emedicine.medscape.com]

Assessment of the degree of hepatic fibrosis helps assess the prognosis of patients with chronic viral hepatitis. [merckmanuals.com]

Although the prognosis of the disease is apparently benign, further studies are needed to determine whether patients should be treated. In summary, we have observed idiopathic biliary ductopenia in adults without symptoms of liver disease. [nejm.org]

Physical examination of patients with cirrhosis may reveal a variety of findings that necessitate a hepatic- or gastrointestinal-based work-up to determine the etiology. [aafp.org]

[…] unknown etiology, but various factors have been implicated as the causes of this illness, such as the following: Genetic factors: First-degree relatives have a 570- to 1000-fold increased chance of developing primary biliary cholangitis. [emedicine.medscape.com]

[…] unclear why copper accumulates in cirrhosis, but architectural remodeling may lead to distortion of bile flow, hindering copper excretion in bile. 11, 21 This, however, signifies that a positive stain for copper has no significance in revealing the etiology [academic.oup.com]

A retrospective analysis of the epidemiology, an evaluation of its clinicopathologic characteristics, a definition of the pedigrees of affected families, and an investigation of nutritional habits was performed to uncover possible aetiologic factors of [journals.lww.com]

Definition of chronic liver disease (CLD) Progressive destruction of the liver parenchyma over a period greater than 6 months leading to fibrosis and cirrhosis Epidemiology of chronic liver disease (CLD) Literature on the incidence and prevalence of liver [oxfordmedicaleducation.com]

Epidemiology United States statistics Although the epidemiology of primary biliary cholangitis has not been studied systematically, the published prevalence is 65.4 cases for women and 12.1 cases for men (40.2 cases overall) per 100,000 population. [emedicine.medscape.com]

Relation of hemochromatosis with hepatocellular carcinoma: epidemiology, natural history, pathophysiology, screening, treatment, and prevention. Med Clin North Am. 2005;89:391–409. 19. Neimark E, Schilsky ML, Shneider BL. [aafp.org]

TABLE OF CONTENTS Wilson Disease - Epidemology Copper Metabolism Wilson Disease – Genetic Link Molecular Metabolism(Normal & Abnormal) Wilson Disease – Pathophysiology Clinical Features Laboratory Diagnosis Treatment Prevention References 4. [slideshare.net]

Pathophysiology Normal copper transportation and metabolism Copper is one of the essential elements that is required daily in diet in a range of 1.5 to 2 mg. [1] Copper is incorporated in number of important functions in the body. [wikidoc.org]

[…] with idiopathic non-cirrhotic portal hypertension, ascites and hepatorenal syndrome although the incidence of portal hypertension as well as any causal relationship or the benefit of haematological treatment remains uncertain. [2] [5] [6] Different pathophysiological [en.wikiversity.org]

Although numerous pathophysiologic mechanisms of injury exist, the final common pathway is persistent wound healing resulting in hepatic parenchymal fibrosis. [aafp.org]

These are not related to ATP7B mutations: for example, ICC has been linked to mutations in the KRT8 and the KRT18 gene. [7] Pathophysiology Edit File:Copper metabolism.png Copper is needed by the body for a number of functions, predominantly as a cofactor [psychology.wikia.com]

People can prevent liver disease in some cases or prevent deterioration by: Early intervention for any symptoms that may indicate liver damage. [medic8.com]

Page 312 - Chapuy MC, Arlot ME, Duboeuf F, et al: Vitamin D3 and calcium to prevent hip fractures in the elderly women. ‎ [books.google.com]

TABLE OF CONTENTS Wilson Disease - Epidemology Copper Metabolism Wilson Disease – Genetic Link Molecular Metabolism(Normal & Abnormal) Wilson Disease – Pathophysiology Clinical Features Laboratory Diagnosis Treatment Prevention References 4. [slideshare.net]

Centers for Disease Control and Prevention. Alcohol-attributable deaths and years of potential life lost—United States, 2001. MMWR Morb Mortal Wkly Rep. 2004;53:866–70. 8. [aafp.org]

Zinc stimulates metallothionein, a protein in gut cells that binds copper and prevents their absorption and transport to the liver. [en.wikipedia.org]