Liver cirrhosis in patients newly diagnosed with neurological phenotype of Wilson�s disease

Przybyłkowski, Adam

doi:10.11138/fneur/2014.29.1.023

Cited by 20 publications

(17 citation statements)

References 20 publications

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“…Its principal causative factors in developing countries are viral infection with hepatitis B and C and parasitic infection with Schistosoma Mansoni , while it is excessive alcohol consumption in developed countries [ 2 ]. Some drugs, autoimmune diseases and genetic disorders are also contributed to liver fibrosis [ 3 - 5 ]. Persistent inflammation caused by chronic liver injury, resulted in aberrant wound healing response with excessive deposition of extracellular matrix (ECM) in the space of Disse that leads to disruption of normal microanatomy of liver sinusoids [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

Lactoferrin Enhanced Apoptosis and Protected Against Thioacetamide-Induced Liver Fibrosis in Rats

Hessin¹,

Hegazy²,

Hassan

et al. 2015

Open Access Maced J Med Sci

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BACKGROUND:Liver fibrosis is the common pathologic consequence of all chronic liver diseases.AIM:Lactoferrin (Lf) was investigated for its possible hepatoprotective effect against thioacetamide (TAA)-induced liver fibrosis rat model.MATERIAL AND METHODS:Rats received TAA (200 mg/kg/biweekly, ip) for four successive weeks. Lf (200 mg/kg/day, p.o.) or vehicle (VHC) was administered for one month before and another month during TAA injection. Body weight and mortality rate were assessed during the month of TAA-intoxication. Thereafter, serum and liver tissues were analyzed for liver function, oxidative, fibrotic and apoptotic markers.RESULTS:Lf conserved rats against TAA-induced body weight-loss and mortality. Preservation of serum albumin, alkaline phosphatase and total bilirubin levels was also observed. Lf also protected rats against TAA-induced decrease in reduced glutathione and increase in malondialdehyde liver contents. Normal liver contents of hydroxyproline, nuclear factor kappa B and alpha fetoprotein; as markers of fibrosis; were increased with TAA and conserved with Lf-TAA. Lf maintained the normal architecture of the liver and immunohistochemical findings revealed increase in apoptotic bodies compared to TAA that favored necrosis.CONCLUSION:In conclusion, Lf improved liver function, reduced oxidative stress and liver fibrosis, and enhanced apoptosis in rats with liver fibrosis, suggesting it to have useful therapeutic potential in patients with liver fibrosis.

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Section: Introductionmentioning

confidence: 99%

Lactoferrin Enhanced Apoptosis and Protected Against Thioacetamide-Induced Liver Fibrosis in Rats

Hessin¹,

Hegazy²,

Hassan

et al. 2015

Open Access Maced J Med Sci

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“…9,10 Non-ceruloplasmin-bound copper accumulates increasingly in the brain of WD patients along with the progression of copper-induced liver disease 4–6. This process may also be age-dependent in affected siblings.…”

Section: Discussionmentioning

confidence: 99%

Phenotypes and Chronic Organ Damage may be Different among Siblings with Wilson’s Disease

Yahata¹,

Yung²,

Mandai

et al. 2017

Journal of Clinical and Translational Hepatology

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Background and Aims: Cloning of ATP7B provided evidence that Wilson's disease is a hepatic copper toxicosis with a variety of extrahepatic complications. Affected siblings with the same genetic background and exposure to similar environmental factors may be a good model for the study of genotype-phenotype correlation. Methods: Twenty-three affected siblings in 11 families were selected from a database. The first phenotypes were determined according to the international proposal. The final types of chronic organ damage were re-evaluated for life-long management. Results: Phenotypes were identical in 5 of the families and different in 6 of the families. The acute hepatic phenotype H1 was found in 3 younger siblings and 1 older sibling. All survived an acute episode of hemolysis with underlying chronic liver disease. One also presented complication with neurological disease. The neurological phenotype N1 with neuropsychiatric symptoms and hepatic disease was found in 2 aged siblings of 1 family, in an older sibling in 3 families and in the oldest sibling in 1 family. Phenotypes in siblings were mainly split by either H1 occurring in random order or age-dependent N1. Types of chronic organ damage were identical in 8 of the families and different in 3 of the families. The same combination of chronic liver disease was found in 6 families and chronic liver disease complicated with neurological disease in 2 families. Split organ damage in siblings was found when an

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“…Într-un studiu pe 156 pacienţi cu BW efectuat în Polonia, s-a evidenţiat faptul că 94,23% dintre cazurile de pacienţi cu BW s-au prezentat cu manifestări hepatice, dintre care 16,23% cu insuficienţă hepatică (15). Ciroza hepatică poate fi prezentă în unele cazuri de la diagnostic (16,17).…”

Section: Manifestările Clinice îN Boala Wilson La Copilunclassified

Boala Wilson La Copil – Criterii Şi Scoruri De Diagnostic

Coroleuca¹,

Alexandrescu“²,

Păcurar³

et al. 2019

Ro J Pediatr.

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Boala Wilson este o afecţiune genetică, transmisă autozomal recesiv, ce determină tulburări ale metabolismului cuprului, ducând la acumularea acestuia în ţesuturile ţintă. Este o afecţiune multisistemică şi are tablou clinic foarte polimorf. Stabilirea diagnosticului pozitiv poate fi dificilă, nefiind disponibil un singur test care să confirme sau să infirme cu certitudine această afecţiune. Au fost stabilite criterii de diagnostic pentru boala Wilson iniţial pentru pacienţii adulţi, ulterior au fost reevaluate pentru copil. Stabilirea diagnosticului precoce permite iniţierea terapiei chelatoare la timp, ceea ce oferă un prognostic bun, încetinind progresia bolii. De aceea, se pune foarte mult accentul pe importanţa screening-ului familial.

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Liver cirrhosis in patients newly diagnosed with neurological phenotype of Wilson�s disease

Cited by 20 publications

References 20 publications

Lactoferrin Enhanced Apoptosis and Protected Against Thioacetamide-Induced Liver Fibrosis in Rats

Lactoferrin Enhanced Apoptosis and Protected Against Thioacetamide-Induced Liver Fibrosis in Rats

Phenotypes and Chronic Organ Damage may be Different among Siblings with Wilson’s Disease

Boala Wilson La Copil – Criterii Şi Scoruri De Diagnostic

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