Une alimentation pauvre en cuivre peut aider à gérer la maladie et réduire les symptômes.
Régime alimentaireCuivre
#5
Les traitements sont-ils à vie ?
Oui, un traitement continu est souvent nécessaire pour contrôler la maladie.
Traitement à long termeDégénérescence hépatolenticulaire
Complications
5
#1
Quelles sont les complications possibles de la maladie ?
Les complications incluent des lésions hépatiques, des troubles neurologiques et psychiatriques.
ComplicationsDégénérescence hépatolenticulaire
#2
La cirrhose est-elle une complication fréquente ?
Oui, la cirrhose peut se développer en raison de l'accumulation de cuivre dans le foie.
CirrhoseDégénérescence hépatolenticulaire
#3
Les troubles mentaux peuvent-ils s'aggraver ?
Oui, sans traitement, les troubles mentaux peuvent s'aggraver et affecter la qualité de vie.
Troubles mentauxDégénérescence hépatolenticulaire
#4
Y a-t-il un risque accru de cancer du foie ?
Oui, les patients atteints de cirrhose ont un risque accru de développer un cancer du foie.
Cancer du foieCirrhose
#5
Les complications peuvent-elles être évitées ?
Un traitement précoce et régulier peut réduire le risque de complications graves.
Prévention des complicationsDégénérescence hépatolenticulaire
Facteurs de risque
5
#1
Quels sont les facteurs de risque de la maladie ?
Les antécédents familiaux et les mutations génétiques sont des facteurs de risque majeurs.
Facteurs de risqueDégénérescence hépatolenticulaire
#2
La consanguinité augmente-t-elle le risque ?
Oui, la consanguinité peut augmenter la probabilité de transmettre la maladie génétique.
ConsanguinitéDégénérescence hépatolenticulaire
#3
Les personnes d'ascendance spécifique sont-elles plus à risque ?
Oui, certaines populations, comme les Caucasiens, ont un risque plus élevé.
AscendanceDégénérescence hépatolenticulaire
#4
Les femmes sont-elles plus à risque que les hommes ?
Non, les deux sexes sont également affectés par la maladie de Wilson.
SexeDégénérescence hépatolenticulaire
#5
Les jeunes adultes sont-ils plus touchés ?
Oui, les symptômes apparaissent souvent entre 5 et 35 ans, mais peuvent survenir plus tôt.
Jeunes adultesDégénérescence hépatolenticulaire
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"name": "Comment diagnostiquer la dégénérescence hépatolenticulaire ?",
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"text": "Le diagnostic repose sur des tests sanguins, des analyses d'urine et des examens d'imagerie."
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"@type": "Question",
"name": "Quels tests sanguins sont utilisés pour le diagnostic ?",
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"name": "L'IRM est-elle utile dans le diagnostic ?",
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"position": 8,
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"position": 9,
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"@type": "Question",
"name": "Les tests génétiques sont-ils recommandés ?",
"position": 12,
"acceptedAnswer": {
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"position": 13,
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"@type": "Question",
"name": "Les conseils diététiques peuvent-ils prévenir la maladie ?",
"position": 14,
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"@type": "Question",
"name": "Les personnes à risque doivent-elles être surveillées ?",
"position": 15,
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"name": "Quels traitements sont disponibles pour cette maladie ?",
"position": 16,
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"@type": "Question",
"name": "Comment les chélateurs de cuivre fonctionnent-ils ?",
"position": 17,
"acceptedAnswer": {
"@type": "Answer",
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"@type": "Question",
"name": "La transplantation hépatique est-elle une option ?",
"position": 18,
"acceptedAnswer": {
"@type": "Answer",
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{
"@type": "Question",
"name": "Quel rôle joue la diététique dans le traitement ?",
"position": 19,
"acceptedAnswer": {
"@type": "Answer",
"text": "Une alimentation pauvre en cuivre peut aider à gérer la maladie et réduire les symptômes."
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"@type": "Question",
"name": "Les traitements sont-ils à vie ?",
"position": 20,
"acceptedAnswer": {
"@type": "Answer",
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{
"@type": "Question",
"name": "Quelles sont les complications possibles de la maladie ?",
"position": 21,
"acceptedAnswer": {
"@type": "Answer",
"text": "Les complications incluent des lésions hépatiques, des troubles neurologiques et psychiatriques."
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},
{
"@type": "Question",
"name": "La cirrhose est-elle une complication fréquente ?",
"position": 22,
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"@type": "Question",
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"position": 23,
"acceptedAnswer": {
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"@type": "Question",
"name": "Y a-t-il un risque accru de cancer du foie ?",
"position": 24,
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"position": 26,
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"text": "Les antécédents familiaux et les mutations génétiques sont des facteurs de risque majeurs."
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},
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"@type": "Question",
"name": "La consanguinité augmente-t-elle le risque ?",
"position": 27,
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},
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"@type": "Question",
"name": "Les personnes d'ascendance spécifique sont-elles plus à risque ?",
"position": 28,
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{
"@type": "Question",
"name": "Les femmes sont-elles plus à risque que les hommes ?",
"position": 29,
"acceptedAnswer": {
"@type": "Answer",
"text": "Non, les deux sexes sont également affectés par la maladie de Wilson."
}
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{
"@type": "Question",
"name": "Les jeunes adultes sont-ils plus touchés ?",
"position": 30,
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}
}
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}
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}
School of Pharmacy, Anhui University of Chinese Medicine, Hefei, China.
Anhui Province Key Laboratory of Research and Development of Chinese Medicine, Anhui Province Key Laboratory of Chinese Medicinal Formula, Hefei, China.
Publications dans "Dégénérescence hépatolenticulaire" :
The Experimental Research Center, Anhui University of Chinese Medicine, Hefei, China.
Anhui Province Key Laboratory of Research and Development of Chinese Medicine, Anhui Province Key Laboratory of Chinese Medicinal Formula, Hefei, China.
Publications dans "Dégénérescence hépatolenticulaire" :
Department of Gastroenterology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai Key Lab of Pediatric Gastroenterology and Nutrition, Shanghai 200092, China.
Publications dans "Dégénérescence hépatolenticulaire" :
Department of Gastroenterology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai Key Lab of Pediatric Gastroenterology and Nutrition, Shanghai 200092, China.
Publications dans "Dégénérescence hépatolenticulaire" :
School of Pharmacy, Anhui University of Chinese Medicine, Hefei, China.
Anhui Province Key Laboratory of Research and Development of Chinese Medicine, Anhui Province Key Laboratory of Chinese Medicinal Formula, Hefei, China.
Publications dans "Dégénérescence hépatolenticulaire" :
Difficult & Complicated Liver Diseases and Artificial Liver Center, Beijing You An Hospital, Capital Medical University & Beijing Municipal Key Laboratory of Liver Failure and Artificial Liver Treatment Research, Beijing 100069, China.
Publications dans "Dégénérescence hépatolenticulaire" :
Encephalopathy Center, the First Affiliated Hospital of Anhui University of Chinese Medicine, No 117 Meishan Road, Shushan District, Hefei, 230031, People's Republic of China.
Publications dans "Dégénérescence hépatolenticulaire" :
Encephalopathy Center, the First Affiliated Hospital of Anhui University of Chinese Medicine, No 117 Meishan Road, Shushan District, Hefei, 230031, People's Republic of China.
Publications dans "Dégénérescence hépatolenticulaire" :
Encephalopathy Center, the First Affiliated Hospital of Anhui University of Chinese Medicine, No 117 Meishan Road, Shushan District, Hefei, 230031, People's Republic of China.
Publications dans "Dégénérescence hépatolenticulaire" :
Patients with hepatic reticulum degeneration (HLD) may eventually develop complications of cirrhosis with splenomegaly and hypersplenism, requiring splenectomy to alleviate hypersplenism and complete ...
A retrospective systematic analysis was conducted on the liver function indicators of 220 HLD patients who underwent splenectomy from January 2015 to January 2018 before surgery and on days 1, 3, 5, 7...
The Child score increased on the 1st day after surgery and gradually decreased after the 1st day. The level of alanine aminotransferase (ALT), aspartate aminotransferase (AST), and total bilirubin (TB...
Splenectomy is proved to be beneficial for the improvement of liver function in HLD patients combined with hypersplenism, which realize a lifelong anti-copper treatment....
Hepatolenticular degeneration (HLD) is an autosomal recessive disorder that manifests as multiorgan damage due to impaired copper (Cu) metabolism. Female patients with HLD often experience reproductiv...
Mice were categorized into control group, HLD TX group (HLD group), penicillamine (Cu chelator)-treated TX group and berberine-treated TX group. Body weight, ovary weight and the number of ovulated eg...
Ovarian tissue damage was evident in the HLD group, with a significant increase in ferroptosis and ER stress compared to the control group. This damage was inhibited by treatment with penicillamine, a...
Ferroptosis and ER stress are involved in Cu-induced ovarian damage in TX mice. Berberine ameliorates ovarian damage in HLD TX mice by inhibiting ferroptosis and ER stress. Please cite this article as...
Splenectomy is a vital treatment method for hypersplenism with portal hypertension. However, portal venous system thrombosis (PVST) is a serious problem after splenectomy. Therefore, constructing an e...
Between January 2016 and December 2021, 309 HLD patients were selected. The data were split into a development set (215 cases from January 2016 to December 2019) and a validation set (94 cases from Ja...
Seven predictive variables were screened out using LASSO regression analysis, including grade, POD14D-dimer (Postoperative day 14 D-dimer), POD7PLT (Postoperative day 7 platelet), PVD (portal vein dia...
The proposed nomogram-based prediction model can predict postoperative PVST. Meanwhile, an earlier intervention should be performed on three sites of PVST....
Long non-coding RNAs (lncRNAs) are a recently discovered group of non-coding RNAs that play a crucial role in the regulation of various human diseases, especially in the study of nervous system diseas...
Traditional Chinese medicine (TCM) is widely used in the clinical treatment of hepatolenticular degeneration (HLD) and liver fibrosis (LF). In the present study, the curative effect was assessed using...
For literature collection, we searched several databases, including PubMed, Embase, Cochrane Library, Web of Science, Chinese National Knowledge Infrastructure (CNKI), VIP Database for Chinese Technic...
The results of the meta-analysis revealed that the addition of Chinese herbal medicine (CHM) in treating HLD resulted in a higher total clinical effective rate than western medicine alone [RR 1.25, 95...
Meta-analysis indicates that TCM is beneficial in treating HLD patients and improving LF. The present study successfully predicts the effective components and potential targets and pathways involved i...
https://www.crd.york.ac.uk/PROSPERO, identifier: CRD42022302374....
Wilson disease (WD) is a rare disorder of copper metabolism, causing copper accumulation mainly in the liver and the brain. The prevalence of WD was previously estimated around 20 to 33.3 patients per...
To describe patient characteristics and to assess prevalence of WD in Germany using a representative claims database....
WD patients were identified in the WIG2 (Wissenschaftliches Institut für Gesundheitsökonomie und Gesundheitssystemforschung; Scientific Institute for Health Economics and Health Systems Research) benc...
Seventy unique patients were identified. Most patients (86%) were between 18 and 64 years of age and more often male (60%) than female. Two patients (3%) younger than 18 years were included, as well a...
This study adds valuable real-world data on the prevalence and patient characteristics of WD in Germany. Generally, our findings align with other reports and contribute to the global understanding of ...
Wilson disease (WD) is a rare genetic disease affecting copper metabolism and the biliary tract's copper excretion. Lifelong medication is necessary to prevent liver failure, neurological complication...
Ninety out of 92 patients were included in the analysis, excluding two patients who underwent liver transplantation without drug treatment due to an acute liver failure diagnosis. Treatment outcomes a...
The median age at diagnosis was 8.3 years. There was a statistically significant difference in drug changes due to treatment ineffectiveness among the three drugs: trientine (22/50, 44%), zinc (15/61,...
In pediatric WD, DPA, zinc, and trientine have therapeutic effects in that order. However, DPA and zinc are associated with more adverse effects compared to trientine....
Noninvasive and accurate biomarkers of neurologic Wilson disease (NWD), a rare inherited disorder, could reduce diagnostic error or delay. Excessive subcortical metal deposition seen on susceptibility...
Patients with WD, healthy participants with monoallelic...
We identified a linear signal intensity change consisting of a hyperintense strip at the lateral border of the globus pallidus in patients with NWD. We termed this feature "hyperintense globus pallidu...
The hyperintense globus pallidus rim sign showed high sensitivity and excellent specificity for diagnosis and differential diagnosis of NWD. It is related to a special metal deposition pattern in the ...
The study provides Class II evidence that the hyperintense globus pallidus rim sign on 7T SWI MRI can accurately diagnose neurologic WD....
Wilson disease (WD) is an autosomal recessive disorder that leads to organ toxicity due to copper overload. Early diagnosis is complicated by the rarity and diversity of manifestations....
To describe the diagnostic features and response to treatment in our cohort of WD patients....
This was a retrospective analysis of 262 WD patients stratified by clinical presentation, complementary exams,...
Symptoms occurred at an average age of 17.4 (7-49) years, and patients were followed up for an average of 9.6 (0-45) years. Patients presented mainly with hepatic (36.3%), neurologic (34.7%), and neur...
Wilson disease is diagnosed at a late stage, and therapeutic options are limited. In people under 40 years of age with compatible manifestations, WD could be considered earlier in the differential dia...
Long-term D-penicillamine (D-pen) therapy in Wilson disease (WD) has numerous adverse effects which advocates its withdrawal, but with an inherent risk of relapse. This prospective observational study...
Hepatic WD patients <18 years of age and on combination therapy for >2 years with 6 months of biochemical remission were included. Biochemical remission was defined as achievement of (i) aspartate ami...
Forty-five patients enrolled with median combination therapy duration of 36 months. Sixty percent of them had their index presentation as decompensated cirrhosis. Fourteen patients (31.8%) relapsed (c...
Incidence of relapse after withdrawal of D-pen from combination therapy is 31.8% in hepatic WD. ALT ≥40 U/L, at the time of D-pen stoppage, predicts future relapse....