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Idiopathic Copper-Associated Cirrhosis

Non-Wilsonian Hepatic Copper Toxicosis of Infancy and Childhood


Presentation

  • 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]
  • Increased arsenic levels are present in drinking water in some countries and may contribute to regional differences in incidence.[rarediseases.org]
  • 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]
Malnutrition
  • Complications of Cirrhosis on health Cirrhosis is highly detrimental to a person’s health causing internal bleeding, organ failure, malnutrition and the malfunction of metabolic processes needed for health and living.[medic8.com]
  • […] incompletely understood, it is believed that their presence in men is associated with an increase in the estradiol to free testosterone ratio. 12 Vascular spiders are not specific for cirrhosis: they also occur during pregnancy, in patients with severe malnutrition[aafp.org]
Italian
  • […] degeneratie; lever, vettig, degeneratie; vettig, lever, infiltraat; lever, vettig, lever; degeneratie, vettig, lever; infiltraat, vettig, lever; vet, vet; lever, vettig; degeneratie lever, vettig; leverdegeneratie, Leversteatose, Leververvetting, Vetlever Italian[fpnotebook.com]
  • TNF-alpha polymorphisms in primary biliary cirrhosis: a northern and southern Italian experience. Ann N Y Acad Sci. 2009 Sep. 1173:557-63. [Medline]. Drebber U, Mueller JJ, Klein E, et al.[emedicine.medscape.com]
Xerostomia
  • […] the disease advances, the following signs may be noted: Hepatomegaly (25%) Hyperpigmentation (25%) Splenomegaly (15%) Jaundice (10%) Xanthomas and Xanthelasmas (10%): In late stages of the disease Sicca syndrome (50-75%): Xerophthalmia (ie, dry eyes), xerostomia[emedicine.medscape.com]
Fracture
  • 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]
Dysarthria
  • Affected individuals present with insulin-dependent diabetes and neurologic symptoms, including dysarthria, dystonia, and dementia as a direct result of iron accumulation in affected tissues.[ommbid.mhmedical.com]

Workup

  • […] 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

  • 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]
  • 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]
  • 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]
  • "Treatment of Wilson's disease with trientine (triethylene tetramine) dihydrochloride". Lancet. 1 (8273): 643–7. doi : 10.1016/S0140-6736(82)92201-2. PMID 6121964. Harper PL, Walshe JM (December 1986).[en.wikipedia.org]
  • Treatment is supportive. Resources In This Article[msdmanuals.com]

Prognosis

  • 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]

Etiology

  • 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]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • Pathophysiology The exact mechanism of the liver damage is unknown, although evidence indicates that it can be of autoimmune origin.[emedicine.medscape.com]

Prevention

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

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