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- W2080322450 abstract "Hyperammonemia in urea cycle defects is a consequence of impaired waste nitrogen excretion (WNE).In contrast to other defects, ASAemia is not necessarily associated with impaired WNE. ASA contains 2 N atoms normally excreted as urea and 2 N atoms as ornithine (orn).If these are replenished stoichemetrically,either by arginine (arg) or by de novo orn synthesis, WNE could be normal and hyperammonemia prevented. This hypothesis was tested in a full-term 3 kg.black female who became lethargic at 24 hrs of age and comatose at 48 hrs.At 7 days of age, the diagnosis of ASAemia was made based on a plasma NH4+ of 800 μM, and identification of peaks of ASA and its anhydrides in plasma and urine.Over a period of 24 hrs., she received two intravenous doses of Arg.HCl 5 mmol/kg. Twenty-four hours after the first infusion, there was a fall in NH4+(837→112 μM), glutamine (1799→1081) and alanine (1566→188) There were increases in arg (40→883), orn (62→170) and ASA (466→1558).Subsequently the NH4+ fell to 62.Arg was then discontinued and the NH4+ rose and ASA fell.Thus, provision of orn skeletons as arg permitted ASA synthesis and a calculated 3-fold increase in WNE as ASA.As a consequence, NH4+ and its precursors (alanine and glutamine) fell to near-normal.Although ASA levels were increased, they may not be toxic (Clin.Res.,24:186A, 1976).Although this child had irreversible brain damage and died at 17 days, future cases, if diagnosed early, should respond to supplemental arg or orn with normal NH4+ and survival." @default.
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- W2080322450 date "1978-04-01" @default.
- W2080322450 modified "2023-09-26" @default.
- W2080322450 title "507 CORRECTION OF HYEPAMMONEMIA IN ARGININOSUCCINIC ACID EMIA BY STIMULATING ARGNINOSUCCINIC ACID (ASA) SYNTHESIS" @default.
- W2080322450 doi "https://doi.org/10.1203/00006450-197804001-00512" @default.
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