Recent tPA administration can cause pseudo‐hyperargininemia and may mimic arginase deficiency or arginine supplementation
Abstract Individuals suspected of or diagnosed with a rare disorder, including inherited metabolic disorders (IMD), often need frequent and/or urgent vascular access for blood draws and treatment, making central indwelling catheters commonly used devices in this patient population. These indwelling...
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2022-11-01
|
| Series: | JIMD Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/jmd2.12328 |
| _version_ | 1798046320773038080 |
|---|---|
| author | Kristina P. Cusmano‐Ozog Alicia K. Renck Christina G. Tise |
| author_facet | Kristina P. Cusmano‐Ozog Alicia K. Renck Christina G. Tise |
| author_sort | Kristina P. Cusmano‐Ozog |
| collection | DOAJ |
| description | Abstract Individuals suspected of or diagnosed with a rare disorder, including inherited metabolic disorders (IMD), often need frequent and/or urgent vascular access for blood draws and treatment, making central indwelling catheters commonly used devices in this patient population. These indwelling catheters are prone to thrombosis, limiting vascular access. This complication is frequently resolved with the use of altepase, a recombinant tissue plasminogen activator (tPA). This report describes two individuals, one with a known IMD and one undergoing evaluation for an IMD, who were found to have hyperargininemia (>500 μM; reference 10–140 μM) by plasma amino acid (PAA) analysis of a specimen collected ~1.5–3 h after clearance of an indwelling catheter with tPA. In both cases, hyperargininemia resolved with repeat testing, suggesting pseudo‐hyperargininemia secondary to tPA administration. Quantitative amino acid analysis of the administered tPA demonstrated an arginine level of ~200 mM, supporting tPA as the cause of pseudo‐hyperargininemia. Certain formulations of tPA contain high concentrations of arginine, which if not cleared properly can result in marked elevations of arginine, mimicking arginase deficiency or suggesting arginine supplementation. Thus, the possibility of pseudohyperargininemia due to tPA administration should be considered when obtaining PAAs from an indwelling catheter in any individual being evaluated or managed for an IMD. |
| first_indexed | 2024-04-11T23:35:47Z |
| format | Article |
| id | doaj.art-7a0658fdb1104d8b9fa061309e3a0ec5 |
| institution | Directory Open Access Journal |
| issn | 2192-8312 |
| language | English |
| last_indexed | 2024-04-11T23:35:47Z |
| publishDate | 2022-11-01 |
| publisher | Wiley |
| record_format | Article |
| series | JIMD Reports |
| spelling | doaj.art-7a0658fdb1104d8b9fa061309e3a0ec52022-12-22T03:56:57ZengWileyJIMD Reports2192-83122022-11-0163656356710.1002/jmd2.12328Recent tPA administration can cause pseudo‐hyperargininemia and may mimic arginase deficiency or arginine supplementationKristina P. Cusmano‐Ozog0Alicia K. Renck1Christina G. Tise2Department of Pathology Stanford University Stanford California USADivision of Medical Genetics Stanford University Stanford California USADivision of Medical Genetics Stanford University Stanford California USAAbstract Individuals suspected of or diagnosed with a rare disorder, including inherited metabolic disorders (IMD), often need frequent and/or urgent vascular access for blood draws and treatment, making central indwelling catheters commonly used devices in this patient population. These indwelling catheters are prone to thrombosis, limiting vascular access. This complication is frequently resolved with the use of altepase, a recombinant tissue plasminogen activator (tPA). This report describes two individuals, one with a known IMD and one undergoing evaluation for an IMD, who were found to have hyperargininemia (>500 μM; reference 10–140 μM) by plasma amino acid (PAA) analysis of a specimen collected ~1.5–3 h after clearance of an indwelling catheter with tPA. In both cases, hyperargininemia resolved with repeat testing, suggesting pseudo‐hyperargininemia secondary to tPA administration. Quantitative amino acid analysis of the administered tPA demonstrated an arginine level of ~200 mM, supporting tPA as the cause of pseudo‐hyperargininemia. Certain formulations of tPA contain high concentrations of arginine, which if not cleared properly can result in marked elevations of arginine, mimicking arginase deficiency or suggesting arginine supplementation. Thus, the possibility of pseudohyperargininemia due to tPA administration should be considered when obtaining PAAs from an indwelling catheter in any individual being evaluated or managed for an IMD.https://doi.org/10.1002/jmd2.12328altepasecentral indwelling catheterhyperargininemiaplasma amino acidspropionic acidemiapseudo‐hyperargininemia |
| spellingShingle | Kristina P. Cusmano‐Ozog Alicia K. Renck Christina G. Tise Recent tPA administration can cause pseudo‐hyperargininemia and may mimic arginase deficiency or arginine supplementation JIMD Reports altepase central indwelling catheter hyperargininemia plasma amino acids propionic acidemia pseudo‐hyperargininemia |
| title | Recent tPA administration can cause pseudo‐hyperargininemia and may mimic arginase deficiency or arginine supplementation |
| title_full | Recent tPA administration can cause pseudo‐hyperargininemia and may mimic arginase deficiency or arginine supplementation |
| title_fullStr | Recent tPA administration can cause pseudo‐hyperargininemia and may mimic arginase deficiency or arginine supplementation |
| title_full_unstemmed | Recent tPA administration can cause pseudo‐hyperargininemia and may mimic arginase deficiency or arginine supplementation |
| title_short | Recent tPA administration can cause pseudo‐hyperargininemia and may mimic arginase deficiency or arginine supplementation |
| title_sort | recent tpa administration can cause pseudo hyperargininemia and may mimic arginase deficiency or arginine supplementation |
| topic | altepase central indwelling catheter hyperargininemia plasma amino acids propionic acidemia pseudo‐hyperargininemia |
| url | https://doi.org/10.1002/jmd2.12328 |
| work_keys_str_mv | AT kristinapcusmanoozog recenttpaadministrationcancausepseudohyperargininemiaandmaymimicarginasedeficiencyorargininesupplementation AT aliciakrenck recenttpaadministrationcancausepseudohyperargininemiaandmaymimicarginasedeficiencyorargininesupplementation AT christinagtise recenttpaadministrationcancausepseudohyperargininemiaandmaymimicarginasedeficiencyorargininesupplementation |