… The organic acidemias considered in this overview are inherited in an autosomal recessive
manner. The parents are obligate heterozygotes and, therefore, carry a single copy of a …

… Nutrition management of organic acidemias The goal of nutrition management for a
patient with an organic acidemia is to reduce the accumulation of toxic metabolites, provide …

… in the breakdown pathways of amino acids. Most organic acidemias become clinically
apparent during the … The more prevalent organic acidemias are also reviewed in detail here, …

… overview of all known organic acidemias that feature cardiac complications and a state-of-the-art
overview of the … a focused approach on PA, the organic acidemia that is most strongly …

… Historical overview … elucidated and MMA was among the first of the organic acid disorders
described with … In the reports of liver transplant for organic acidemias and urea cycle disorders, …

… Lactated Ringer’s solution is not recommended in patients with organic acidemias. In
patients with QT abnormalities, avoid medications that can prolong the QT interval. Neuroleptic …

… Organic acidemias (OAs) are inherited metabolic disorders caused by deficiency of enzymatic
activities in the … Besides providing an overview of MS-based approaches most frequently …

… , it appears helpful to consider two main types of clinical presentations: Systemic intoxication
or severe acute encephalopathy, which is the most common presentation (overview in [11]). …

… During the five-year period, 70 patients were diagnosed with organic acidemias at one medical
… Table 7 shows the number of cases of organic acidemias that were reported in our study …

… This article focuses on the two more common OAs, methylmalonic and propionic acidemias.
Literature search was done on PUBMED, CINAHL, … The organic acidemias: An overview …