Anesthesia in a patient with arginase deficiency: implications and management

Kaul, Naresh; Khan, Rashid M.; Sharma, Pradeep; Sumant, Ashok

doi:10.1111/j.1460-9592.2008.02660.x

Cited by 6 publications

(8 citation statements)

References 5 publications

(6 reference statements)

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“…Spastic paralysis is one of the symptoms of hyperargininemia [3]. The administration of muscle relaxants to patients with spastic paralysis is considered unsafe because of its unpredictable effects [8]. However, in the present case, we used a muscle relaxant and performed endotracheal intubation, as there was no history of spastic paralysis.…”

Section: Discussionmentioning

confidence: 79%

“…Arginine is a precursor of nitric oxide, which is well known to be a strong vasodilator. Therefore, patients with arginase-1 deficiency may develop significant hypotension during anesthetic induction [8]. To minimize any changes in circulation, we chose gradual induction with sevoflurane.…”

Section: Discussionmentioning

confidence: 99%

“…Dehydration enhances protein catabolism and worsens hyperammonemia; on the other hand, hyperammonemia may cause cerebral edema, which can be worsened by excessive infusion [8,9]. Therefore, especially in cases where surgery is prolonged, highly invasive, or where severe blood loss is expected, it is necessary to monitor a dynamic index of preload such as pulse pressure variation or stroke volume variation.…”

Section: Discussionmentioning

confidence: 99%

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Anesthetic management of a pediatric patient with arginase-1 deficiency undergoing strabismus operation: a case report

2019

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Background: Urea cycle disorders are rare; arginase-1 deficiency is one of those extremely rare autosomal recessive metabolic disorders. Arginase-1 is one among the enzymes involved in the production of urea from ammonia in the liver, and its deficiency produces the characteristic feature, hyperargininemia. Case presentation: We report a case of a girl, aged 5 years and 10 months presenting with arginase-1 deficiency. The patient was scheduled to undergo strabismus surgery for intermittent exotropia under general anesthesia. Preoperative blood tests showed high serum arginine levels, but ammonia levels were within the normal range. Anesthesia was induced with sevoflurane and nitrous oxide via face mask and maintained with sevoflurane, fentanyl, and rocuronium. Vital signs were stable throughout the surgery. There was an intraoperative decrease in blood glucose levels (from 82 mg/dL to 42 mg/dL) that was treated with intravenous glucose. Arginine levels remained high after surgery; however, hyperammonemia did not develop. There were no complications and the patient was discharged on the following day. Conclusions: We successfully performed general anesthesia in a child with hyperargininemia. Only a few cases of arginase-1 deficiency had been reported and much remains unknown about its pathology. Therefore, information sharing among medical professionals is essential to customize the plan for the management of this disorder in patients.

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Section: Discussionmentioning

confidence: 79%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Anesthetic management of a pediatric patient with arginase-1 deficiency undergoing strabismus operation: a case report

2019

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“…Despite the enzyme stimulating effects of carbamazepine, antiepileptic drugs show additive effects with anesthetic agents. That's why, there is also some literature expressing that the titration induction of a single agent may be helpful in assessing the anesthetic requirement for maintenance of anesthesia (5).…”

Section: Discussionmentioning

confidence: 99%

“…Paucity of relevant information in the literature poses several challenges in the anesthetic management of these patients. Few cases of arginase-deficiency have been described in the literature, one of which was related to anesthetic management (3,5). In this report, we presented the anesthetic management of a pediatric patient with arginase deficiency.…”

Section: Introductionmentioning

confidence: 99%