Homocystinuria, a disorder originating in defects in the methionine metabolism, is characterized by an elevated plasma concentration of homocysteine. Most patients have a defect in the cystathionine-beta-synthase, the key enzyme in the conversion of homocysteine to cysteine. Many abnormalities in the connective tissue of patients with homocystinuria resemble those seen in Marfan syndrome, cause...

This article aims to comprehensively describe a particular case of bilateral intraocular subluxated lens with pseudoexfoliation syndrome in Moroccan woman homocystinuria. We report 56-year-old patient who has cataractous along whitish deposits. The association PEX hyperhomocysteinemia rarely been found. It worsens the lenses' zonular stability and causes multiple complications.

Wilcken, B., and Turner, B. (1973). Archives of Disease in Childhood, 48, 58. Homocystinuria: reduced folate levels during pyridoxine treatment. Nine patients with homocystinuria due to cystathionine synthase deficiency were treated with pyridoxine: 6 responded biochemically and 5 of these showed marked clinical improvement. Full biochemical response was only obtained slowly in some patients. R...

Homocystinuria follows an autosomal recessive pattern and transpires due to Cystathionine beta-synthase enzyme deficiency. This condition is associated with thrombotic thromboembolic complications increase in homocysteine methionine levels the plasma urine. We present a case of Indian adolescent boy who presented us generalized tonic clinic seizure Broca's aphasia he was diagnosed as having hom...

Homocystinuria is of particular interest to ophthalmologists, associated as it is with sub-luxated or dislocated lenses in over go per cent. of cases. It was first described by Carson and Neill (I962) and well over I00 cases have since been reported. The subject has recently been reviewed by Cusworth and Dent (I969) and by FranSois (1970). It is our purpose to discuss the management of anterior...