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http://www.ncbi.nlm.nih.gov/pubmed/24345828 Hussain E, Grimason M, Goldstein J, Smith CM, Alonso E, Whitington PF, Wainwright MS. EEG abnormalities are associated with increased risk of transplant or poor outcome in children with acute liver failure. J Pediatr Gastroenterol Nutr. 2014 Apr; 58(4): 449-56. Abstract OBJECTIVES: There are limited data on the incidence of seizures and utility of brain imaging and electroencephalogram (EEG) to predict outcome of children with acute liver failure (ALF). We investigated the association between hepatic encephalopathy (HE) scores, abnormal EEG or neuroimaging, and short-term outcome. METHODS: Published on:
Apr-2014
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Treatment guidelines for virus highlight challenge of paying for expensive drugs in low-income countries.
Campaigners in India have been calling for access to cheap hepatitis C treatments. The publication last week of the first treatment guidelines for hepatitis C virus (HCV), and the advent of drugs that can cure most infections of the virus, have left public-health researchers with a touch of déjà vu.
Three decades after wrestling to lower the cost of AIDS drugs (prices fell from about US$10,000 per patient per year in the 1990s to less than $100 in the mid-2000s), they are once again asking how expensive life-saving medicines can be made affordable for patients.
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American Hepatitis C drug on the market in SeptemberDr. Wahid Doss, head of the National Institute for Liver Diseases, said on Monday said the Health Ministry and the registry of the Central Administration of Pharmaceuticals met to complete the registration of the American Hepatitis C drug. He added that the ministry is buying the drug at 1 percent of its global price.
“Registration will be completed within two months, the fastest period in the history of drug registration in Egypt,” he said. “It will be available on the market in September.”
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Gaucher's disease is a rare human genetic condition caused by hereditary deficiency of that enzyme. People with Gaucher's -- which can manifest itself with fatigue, bruising, anaemia, low blood platelets and an enlarged liver and spleen -- often are treated with drugs and bone marrow transplants but still face pain and often poor long-range health prospects. Scientists in Brazil have genetically modified a goat to produce milk with an enzyme to treat a rare genetic disorder, O Estado de Sao Paulo newspaper reported Tuesday. The goat, named "Gluca," is the first of its kind in South America. It has been genetically modified to produce the enzyme glucocerebrosidase. |
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What is hepatitis C?Hepatitis C is the disease of the liver caused due to infection by hepatitis C virus (HCV). It is one of the common causes of chronic liver disease and may to progressive liver disease. It is also the leading indication for liver transplantation. How common is hepatitis C infection?It has been estimated that the globally around 2% are infected with HCV. 170 million people are chronically infected the virus. Also 3 to 4 million people get newly infected with the virus each year. In India around 1-2% of people are infected with this virus. The disease is common among children who require multiple transfusions like those suffering from Thalassemia and hemophilia. |
http://7thspace.com/headlines/455326/ Wilson's disease (WD) evolves rapidly and is fatal if untreated. The treatment of WD patients with mild liver disease is not clearly defined. To address this issue, we evaluated long-term outcomes of three treatment regimens (D-penicillamine, zinc or both) in patients diagnosed in childhood. Methods: We retrospectively evaluated efficacy, compliance and reasons for treatment discontinuation in 42 WD patients (median age at diagnosis: 6 years; median follow-up: 12 years) with mild liver disease. Treatment duration for each treatment block until a medication change or completion of follow-up was analyzed. Events of change of treatment were evaluated using Kaplan-Meier analysis. Published on:
Mar-2014
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What is Budd Chiari syndrome?Budd chiari syndrome (BCS) is a disease characterized by obstruction of the hepatic venous outflow tract which is the main channel taking blood from the liver to the heart. Liver receives the blood supply from the portal vein and hepatic artery. After circulating through the liver, the blood then flows out via the hepatic veins to the inferior vena cava (large blood vessel emptying into the heart). There are total three hepatic veins. The obstruction to the blood flow can be in the hepatic veins or in the inferior vena cava. If the obstruction is in the smaller veins of liver (hepatic venules), then it is called as sinusoidal obstruction syndrome. |
http://www.indianpediatrics.net/mar2014/mar-203-210.htm Justification: Neonatal cholestasis is an important cause of chronic liver disease in young children. Late referral and lack of precise etiological diagnosis are reasons for poor outcome in substantial number of cases in India. There is a need to create better awareness among the pediatricians, obstetricians and primary care physicians on early recognition, prompt evaluation and referral to regional centers. Published on:
Mar-2014
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Enzyme therapy proves effective in treating LSDs, whilst gene therapy is an upcoming contender. Lysosomes are membrane-bound organelles found in most animal cells. They are responsible for treating cellular waste. Genetic mutations in lysosomal enzymes lead to lysosome malfunction and waste accumulation. And this leads to a whole range of complex metabolic disorders, collectively called Lysosomal Storage Diseases (LSD). There are two kinds of LDSs: those that affect the brain (neuropathic) and those that do not (non-neuropathic). The EU-funded EUCLYD project, completed in 2011, studied four non-neuropathic LSDs out of the 50 currently known. These were Gaucher disease, Pompe disease, the mucopolysaccharidosis (MPS) VI and the multiple sulfatase deficiency. |
http://www.ncbi.nlm.nih.gov/pubmed/24345835 Wen JW, Haber BA. Maternal-fetal transmission of hepatitis C infection: what is so special about babies? J PediatrGastroenterolNutr. 2014; 58(3): 278-82. Published on:
Mar-2014
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