Brief Overview of Methylmalonic Acidemia | 合法藥品大搜索
2017年12月26日—Methylmalonicacidemiaisanautosomalrecessivedisorderofaminoacidmetabolism,involvingadefectintheconversionof ...
In an acute phase, identify and treat intercurrent infections that triggered the acidotic episode. Correct the acidosis; dialysis may be required in cases of severe ketoacidosis and hyperammonemia. A case report noted a decrease in ammonia levels with the use of carbamylglutamate in preference to dialysis alone. [31]
Dietary modifications must be made in a hospital setting. Outcomes are better in patients with cobalamin-responsive disease than in those with the cobalamin-nonresponsive disease, in association with dietary changes and supplementation of carnitine and cobalamin. [32, 33]
Immediately prescribe a protein-restricted diet when an acidemia is a diagnostic consideration. This modification decreases the key amino acids (eg, isoleucine, valine, threonine, methionine) that enter the metabolic pathway.
Try cyanocobalamin[1], even in patients whose dis...
Methylmalonic acidemia | 合法藥品大搜索
Methylmalonic acidemia | 合法藥品大搜索
Methylmalonic acidemia | 合法藥品大搜索
Methylmalonic acidemia | 合法藥品大搜索
About Methylmalonic Acidemia | 合法藥品大搜索
Brief Overview of Methylmalonic Acidemia | 合法藥品大搜索
Methylmalonic acidemia | 合法藥品大搜索
Isolated Methylmalonic Acidemia | 合法藥品大搜索
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