RESUMO
Objective: To evaluate the clinical manifestations, metal metabolism, imaging characteristics and treatment response in patients with delayed Wilson disease (WD). Methods: Patients with untreated WD (40 with delayed onset and 40 with non-delayed onset) were enrolled. Twenty healthy people were included as normal controls. All patients were evaluated with modified Young scale neural symptom scores, grade of Child liver function and mental symptoms rating scale, magnetic resonance imaging (MRI) scan, magnetic sensitive imaging (susceptibility weighted imaging, SWI), metal metabolism. Corrected phase (CP) was measured at SWI. After 2 week treatment, neurologic symptoms, liver function, and metal metabolism were reviewed. Results: The total score of neurological symptoms in WD patients with delayed onset was lower than that of non-delayed onset (13.00±6.87 vs. 21.13±5.53, P=0.033). The scores of SCL-90 and HAMA depression scales in patients with delayed onset were lower than those of non-delayed onset. On T(2) weighted imaging, areas including substantia nigra and thalamus, the caudate nucleus, globus pallidus, putamen presented high signal rate in patients with delated onset than those with non-delayed (P=0.022, 0.037, 0.022, 0.037, 0.029 respectively). The SWI CP values of cangbai sphere and shell nucleus in patients with delayed onset were lower than those with non-delayed onset. Patients with delayed onset had higher urinary copper than those with non-delayed onset before and after treatment (P=0.040, 0.036). After treatment, the score of abnormal tremor and gait in patients with delayed onset was decreased (P=0.037, 0.044), while as the occurrence of neurological symptoms was increased by 10%, and the liver function level in patients with delayed WD was decreased in 3 cases. Conclusions: The brain of WD patients with delayed onset is mainly composed of metal deposits, however the cell damage is not apparent. Clinical symptoms are characterized by significant liver injury, but relatively mild neurological and psychiatric symptoms. Patients with delayed WD have higher urinary copper excretion than those with non-delayed WD. Chelating agents improves the neurological symptoms in patients with delayed onset.
Assuntos
Encéfalo/diagnóstico por imagem , Cobre/metabolismo , Degeneração Hepatolenticular/patologia , Encéfalo/metabolismo , Estudos de Casos e Controles , Criança , Cobre/urina , Degeneração Hepatolenticular/metabolismo , Humanos , Imageamento por Ressonância Magnética , TálamoRESUMO
We aimed to investigate the mechanism of shaking as a prenatal stressor impacting the development of the offspring and Chinese medicines correcting the alterations. Pregnant rats were randomized into earthquake simulation group (ESG), herbal group (HG) which received herbal supplements in feed after shaking, and control group (CG). Findings revealed body weight and open field test (OFT) score of ESG offspring were statistically inferior to the CG and HG offspring. The corticosterone levels of ESG were higher than those of CG but not than HG. The dopamine level of ESG was slightly lower than that of the CG and of HG was higher than that of ESG. The 5-HT of ESG was higher than CG and HG. The growth hormone level of the ESG was significantly lower than ESG but not than CG. Gene expression profile showed 81 genes upregulated and 39 genes downregulated in ESG versus CG, and 60 genes upregulated and 28 genes downregulated in ESG versus HG. Eighty-four genes were found differentially expressed in ESG versus CG comparison and were normalized in ESG versus HG. We conclude that maternal shaking negatively affected physical and nervous system development, with specific alterations in neurohormones and gene expression. Chinese herbal medicine reduced these negative outcomes.
RESUMO
AIM: To study the protective effect of Gn-3 (a stilbene polymer isolated from Gnetum parvifolium) against liver injury induced by CCl4, N-acetyl-p-aminophenol (APAP) and Bacillus Calmette-Guerin (BCG) plus bacterial lipopolysaccharide (LPS) in mice. METHODS: The experimental model of liver injury were induced by 0.1% CCl4 i.p. (10 mL.kg-1.d-1 for 3d), APAP i.p. (150 mg.kg-1) or BCG (5 mg) plus LPS (7.5 micrograms) in mice. The levels of ALT in serum, MDA and GSH in liver tissues were detected. The histopathologic changes were observed by light microscope. RESULTS: Gn-3 was shown to markedly reduce the elevated serum ALT levels, liver tissue MDA and improve the histopathological changes in all the three experimental liver injury models. No effect of Gn-3 was observed on the liver GSH level in liver injury mice. CONCLUSION: Gn-3 was found to inhibit the development of liver injury caused by CCl4, APAP, or BCG plus LPS. This means that Gn-3 has liver protective effects.
Assuntos
Doença Hepática Induzida por Substâncias e Drogas/patologia , Fígado/patologia , Substâncias Protetoras/farmacologia , Estilbenos/farmacologia , Acetaminofen , Alanina Transaminase/sangue , Animais , Intoxicação por Tetracloreto de Carbono , Doença Hepática Induzida por Substâncias e Drogas/metabolismo , Gnetum/química , Lipopolissacarídeos , Fígado/metabolismo , Masculino , Malondialdeído/metabolismo , Camundongos , Mycobacterium bovis , Plantas Medicinais/química , Distribuição Aleatória , Estilbenos/isolamento & purificaçãoRESUMO
OBJECTIVE: To explore the Syndrome pattern in insulin resistant model rats. METHODS: Eight Sprague-Dawley (SD) rats were induced to insulin resistance (IR) by 60% high-sucrose forage. RESULTS: The correlative co-efficient of some biological and internal changes were clusterly analyzed and divided into 3 groups, which was closely related with phleg tubidity, blood stasis and internal toxin types respectively. The phlegm turbidity type was characterized by high content of blood lipid (triglyceride, total cholesterol) and high concentration of glycated serum protein; the blood stasis type was characterized by tendency of high viscosity and high coagulant state, the blood pressure increased, prothrombin time shortened, fibrinogen content raised and RBC and platelet count increased; while the internal toxin type was characterized by high content of glucose, insulin and the elevation of tumor necrosis factor. CONCLUSION: The combined Syndrome of phlegm turbidity, blood stasis and internal toxin is the Syndrome-pattern in insulin resistant model rat. This observation provides theoretic basis for clinical and experimental studies of TCM.