Abstract | OBJECTIVE: METHODS: A 4-month-old female patient weighing 3.9 kg presented with decompensation with PA and initial hyperammonemia of 451 microg/dL. The patient was receiving a protein-restricted diet supplemented with L-carnitine at a dosage of 100 mg/kg/d during 2 consecutive months. Treatment with N-carbamoyl-L-glutamate was administered during 4 days on an outpatient basis at a dosage of 100 mg/kg/d, representing a dosage of 200 mg BID. The patient's hyperammonemia was monitored for 1 week. RESULTS: The patient's ammonia concentration started to decrease on the first day of the initiation of the treatment with N-carbamoyl-L-glutamate, from 451 to 68 microg/dL (normal range, 10-80 microg/dL) at day 6 of follow-up. The patient did not require intravenous fluids, invasive procedures, or hospitalization. CONCLUSION:
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Authors | Erdogan Soyucen, Esra Demirci, Ahmet Aydin |
Journal | Clinical therapeutics
(Clin Ther)
Vol. 32
Issue 4
Pg. 710-3
(Apr 2010)
ISSN: 1879-114X [Electronic] United States |
PMID | 20435240
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Glutamates
- N-carbamylglutamate
- Carnitine
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Topics |
- Carnitine
(therapeutic use)
- Diet
- Female
- Glutamates
(therapeutic use)
- Humans
- Hyperammonemia
(drug therapy, etiology)
- Infant
- Propionic Acidemia
(complications, drug therapy)
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