نتایج جستجو برای: type 1 tyrosinemia

تعداد نتایج: 3647237  

Journal: :Journal of the Chinese Medical Association : JCMA 2006
Chun-Pin Tsai Pei-Yu Lin Ni-Chung Lee Dau-Ming Niu Shui-Mei Lee Wen-Ming Hsu

Tyrosinemia type II (Richner-Hanhart syndrome) is a rare autosomal recessive disease with deficiency of tyrosine aminotransferase and subsequently increasing level of serum tyrosine. We report the case of a 2-year-old girl who was referred due to bilateral corneal lesions. Slit-lamp examination showed small granular white deposits arranged in a dendritic pattern in the superficial central corne...

2015
MICHAEL BLUNDELL

Inherited metabolic diseases (IMDs) are a large class of heterogeneous genetic disorders caused by dysfunction within a single pathway of intermediary metabolism. In many of these diseases, the dysfunction of metabolic enzymes leads to the accumulation of toxic metabolites which disrupts the normal development of the central nervous system. With the advent of treatments that positively influenc...

2006
Naoto SHIKANO Syuichi NAKAJIMA Takashi KOTANI Yusuke ITOH Ryuichi NISHII Mitsuyoshi YOSHIMOTO Leo Garcia FLORES Hideo SAJI Nobuyoshi ISHIKAWA Keiichi KAWAI

FANCONI SYNDROME is a renal dysfunction associated with a variety of metabolic disorders, including tyrosinemia, cystinosis, Wilson’s disease, glycogen storage disease,1 galactosemia and oculocerebrorenal syndrome of Lowe.2,3 Detection of maleate-induced Fanconi syndrome by decreasing accumulation of 125I-3-iodo-α-methyl-L-tyrosine in the proximal tubule segment-1 region of renal cortex in mice...

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