|1.||Ranganath, Lakshminarayan R: 4 articles (09/2015 - 01/2014)|
|2.||Gallagher, James A: 3 articles (09/2015 - 01/2014)|
|3.||McKiernan, P J: 3 articles (08/2015 - 02/2006)|
|4.||Gahl, William A: 3 articles (10/2011 - 06/2005)|
|5.||Hughes, Andrew T: 2 articles (09/2015 - 01/2015)|
|6.||Milan, Anna M: 2 articles (09/2015 - 01/2015)|
|7.||Bussières, Jean-François: 2 articles (05/2015 - 09/2012)|
|8.||Barshop, Bruce A: 2 articles (03/2015 - 01/2015)|
|9.||Gertsman, Ilya: 2 articles (03/2015 - 01/2015)|
|10.||Nyhan, William L: 2 articles (03/2015 - 01/2015)|
05/01/2015 - "To estimate the direct medical costs of health care services related to the treatment of tyrosinemia type I, taking into consideration the real-life efficacy of nitisinone. "
03/15/2010 - "2-(2-Nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) is a relatively new drug which is used to prevent the accumulation of toxic metabolites in patients with hereditary tyrosinemia type I. In the present study, we have developed a new, simple, fast, and cost-effective capillary electrophoresis method for the quantitative monitoring of this drug in serum samples. "
07/09/1999 - "The chemical substance 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) is in clinical use for the treatment of hereditary tyrosinemia type 1. In the present study, the plasma concentration of NTBC was determined by a coupled column liquid chromatographic method. "
05/01/2015 - "Cost-Consequence Analysis of Nitisinone for Treatment of Tyrosinemia Type I."
02/01/2015 - "In tyrosinemia type I, dietary therapy and nitisinone (Orfandin®) are effective. "
06/01/2005 - "We investigated the safety and the HGA-depleting efficacy of nitisinone in an open-label, single-center study of 9 alkaptonuria patients (5 women, 4 men; 35-69 years of age) over the course of 3 to 4 months. "
01/01/2015 - "The Suitability of Nitisinone in Alkaptonuria 1 (SONIA 1) study is a dose-finding trial for nitisinone treatment of AKU patients. "
08/01/2011 - "A 3-year randomized therapeutic trial of nitisinone in alkaptonuria."
09/01/2015 - "The method developed and validated shows good precision, accuracy and linearity appropriate for the monitoring of alkaptonuria patients, pre- and post-nitisinone therapy."
01/01/2015 - "Metabolic Effects of Increasing Doses of Nitisinone in the Treatment of Alkaptonuria."
|3.||Hepatocellular Carcinoma (Hepatoma)
08/01/2015 - "All patients with hepatocellular carcinoma received pretransplant nitisinone treatment. "
04/01/2015 - "Despite nitisinone treatment HT1 still carries the risks of hepatocellular carcinoma (HCC) and neuropsychological outcome. "
12/01/2011 - "Only one patient required liver transplantation after nitisinone and none had hepatocellular carcinoma. "
05/01/2008 - "This review examines recent pharmacological and clinical literature on nitisinone, and assesses its impact as a pharmacological treatment for hereditary tyrosinaemia type 1. English language literature from MedLine and EmBase for nitisinone was searched from 1990 to 2008 for all papers relevant to the use of nitisinone in hereditary tyrosinaemia type 1. Nitisinone can prevent the development of liver disease and significantly reduce the risk of developing hepatocellular carcinoma; however, vigorous surveillance for the development of HCC needs to be continued lifelong."
12/01/2003 - "Administration of 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) corrects the tyrosinemia phenotype, but does not prevent development of hepatocellular carcinoma. "
|4.||Photophobia (Light Sensitivity)
01/01/2012 - "Corneal deposits and photophobia are cardinal features of untreated tyrosinaemia type II, but not of type I. Novel treatment strategies (with nitisinone) for type I tyrosinaemia lead to a phenotype comparable with type II, including these corneal deposits. "
09/01/2012 - "One probable nitisinone-related event occurred, transient corneal crystals with photophobia. "
|10.||Biological Markers (Surrogate Marker)
|2.||Protein-Restricted Diet (Diet, Protein Restricted)