نتایج جستجو برای: ochronosis

تعداد نتایج: 209  

2014
Sreekanth Reddy Rajoli Vibhu Krishnan Vijay Goni Surabhi Subramanian

Alkaptonuria is a rare autosomal recessive, single-gene metabolic disease caused by the lack of the enzyme homogentisic acid oxidase and characterized by black pigmentation of cartilage and other connective tissues. 38 yr old male was admitted to the orthopedics ward with bilateral hip pain for the past 3 years and inability to walk for the past 1yr. He had also complained of brownish-black dis...

2017
Ottavia Spiga Vittoria Cicaloni Andrea Bernini Andrea Zatkova Annalisa Santucci

BACKGROUND Alkaptonuria (AKU; OMIM:203500) is a classic Mendelian genetic disorder described by Garrod already in 1902. It causes urine to turn black upon exposure to air and also leads to ochronosis as well as early osteoarthritis. Our objective is the implementation of a Precision Medicine (PM) approach to AKU. We present here a novel ApreciseKUre database facilitating the collection, integra...

Journal: :Caspian journal of internal medicine 2012
Aref Hosseinian Amiri Alireza Rafiei

BACKGROUND Alkaptonuria (AKU) or ochronosis is a rare progressive degenerative arthropathy that results from deficiency of enzyme homogentisate 1,2 dioxygenase (HGD). The features include arthritis of the spine and in larger peripheral joints, with chondrocalcinosis. In this paper, we present a case of alkaptonuria in a 54 year old woman in Tehran, Iran. CASE PRESENTATION A 54 year old woman ...

Journal: :Indian journal of biochemistry & biophysics 2013
Robert Aquaron

Alkaptonuria (AKU) is a very rare autosomal recessive disorder of tyrosine metabolism in the liver due to deficiency of homogentisate 1,2 dioxygenase (HGD) activity, resulting in the accumulation of homogentisic acid (HGA). Circulating HGA pass into various tissues through-out the body, mainly in cartilage and connective tissues, where its oxidation products polymerize and deposit as a melanin-...

2016
Emma K. T. Benn Andrew Alexis Nihal Mohamed Yan-Hong Wang Ikhlas A. Khan Bian Liu

Skin-bleaching practices, such as using skin creams and soaps to achieve a lighter skin tone, are common throughout the world and are triggered by cosmetic reasons that oftentimes have deep historical, economic, sociocultural, and psychosocial roots. Exposure to chemicals in the bleaching products, notably, mercury (Hg), hydroquinone, and steroids, has been associated with a variety of adverse ...

2014
Lia Millucci Lorenzo Ghezzi Eugenio Paccagnini Giovanna Giorgetti Cecilia Viti Daniela Braconi Marcella Laschi Michela Geminiani Patrizia Soldani Pietro Lupetti Maurizio Orlandini Chiara Benvenuti Federico Perfetto Adriano Spreafico Giulia Bernardini Annalisa Santucci

BACKGROUND Alkaptonuria, a rare autosomal recessive metabolic disorder caused by deficiency in homogentisate 1,2-dioxygenase activity, leads to accumulation of oxidised homogentisic acid in cartilage and collagenous structures present in all organs and tissues, especially joints and heart, causing a pigmentation called ochronosis. A secondary amyloidosis is associated with AKU. Here we report a...

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