WILSON'S DISEASE (A report of two cases).
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ABSTRACT:
SUBMITTER: Kanitkar M
PROVIDER: S-EPMC5529699 | biostudies-other | 1994 Jan
REPOSITORIES: biostudies-other
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Project description:Background and aimLiver transplantation is a potential cure for liver damage from Wilson's disease but the course of neuropsychiatric manifestations after transplantation remains undetermined.Material and methodsIn this study, data on all patients who'd received a liver transplant for Wilson's disease at the Shiraz Organ Transplantation Center between December 2000 and March 2014 were reviewed and compared to data on a control group who'd received a liver transplant over the same period but due to other causes.ResultsOut of 2198 patients who'd received a liver transplant in the period; 107 patients were diagnosed with Wilson's disease (21 with fulminant hepatic failure); age of patient ranged from 5 to 59 years; 56.07% of patients in this series had some type of neuropsychiatric manifestation before transplantation, of which 66.67% showed improvement after the procedure. 18 patients had aggravation of neuropsychiatric symptoms after transplantation. These neuropsychiatric symptoms were mostly for anxiety, tremor and depression but there were four cases of new onset dysarthria, rigidity and ataxia in various combinations. Survival rates of 1-month, 1-year, and 5-years for patients with Wilson's disease were 88%, 86%, 82%, respectively, evaluations were not statistically different from that of the control group.ConclusionsLiver transplantation showed good long-term results in patients with Wilson's disease, even in those presenting fulminant hepatic failure. Neuropsychiatric manifestations normally show improvement after transplantation but in some cases new onset of manifestations occurred after successful liver transplantation.
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Project description:Wilson's disease betides due to mutation in ATP-7B that leads to snagging in copper transport by the hepatic lysosomes resulted in the deposition of copper in the brain, liver, kidney or skeletal system. The symptoms are jaundice, edema in legs, ascites, Kayser-Fleischer rings, dysarthria, dysphagia, ataxia, dyskinesia, and muscle spasticity. Current therapeutic modalities for the management of Wilson's disease include zinc, trientine, penicillamine and ammonium tetrathiomolybdate. A 12 year old child diagnosed with Wilson's disease came with complaints of inability to speak, difficulty in swallowing and generalized stiffness for 6 months. His investigations showed SGPT 43 U/L, Ceruloplasmin 0.03 g/L, urine copper level 225.03 μg per 24 h, a chronic parenchymal disease in the liver and splenomegaly. According to Ayurveda, this case of generalized stiffness with hepatomegaly and splenomegaly was correlated with Agnimandya at the Dhatu level that led to Vatavyadhi, Yakrutodara, and Plihodara. The treatment mentioned for Vatavyadhi is Snehana (oleation), MruduSwedana (mild sudation), Anuvasana Basti (oil enema) and for Yakrutodara and Plihodara is Niruha Basti (Decoction Enema) and Anuvasana Basti (oil enema). The case was treated with Abhyanga, Swedana, Basti and oral medication. After treatment, the symptoms were reduced and he was able to extend both lower limbs completely. His urinary copper level came to normal (47.01 μg per 24 h), so, it can be concluded that the Ayurvedic approach and diet modifications in such patients may help in providing supportive care and improving the quality of life.
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