Clearance of amino acids by hemodialysis in argininosuccinate synthetase deficiency

McBryde, Kevin D.; Kudelka, Timothy; Kershaw, David B.; Brophy, Patrick D.; Gardner, John C.; Smoyer, William E.

doi:10.1016/j.jpeds.2004.01.037

Cited by 32 publications

(16 citation statements)

References 31 publications

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“…This is consistent with work performed in other laboratories [2,3] and indicates that use of these medications prior to the initiation of therapy may be beneficial and is certainly not detrimental, even though these medications will be cleared on dialysis [2,3].…”

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confidence: 86%

The complexity of dialytic therapy in hyperammonemic neonates

Bunchman

2015

Pediatr Nephrol

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The utilization of renal replacement therapy (RRT) in the setting of hyperammonia is a rare and complicated occurrence. Data demonstrate that the quicker the ammonia level is normalized, the better the neurological outcome. The optimal form of RRT is often decided by local practice. The recent work by Picca and colleagues details a larger series of children who underwent RRT for hyperammonia and adds some credence to the use of peritoneal dialysis (PD) in this population. While these authors conclude that PD is not optimal, they do note that the use of PD may be an option when other forms of RRT are not available. The results reinforce the general maxim that you should continue to do that which you do well and often, which in this context refers to continuing to use your form of RRT until alternative modalities are available. Keywords Infant . Inborn error of metabolism . Renal replacement therapyThe recent paper by Picca and colleagues identified a series of 45 neonates over a 21-year span who underwent extracorporeal therapy or peritoneal dialysis (PD) for hyperammonemia due to an inborn error of metabolism (IBM) [1]. One of the aim of the authors was to examine the question of whether PD can play a role in hyperammonemia therapy and if so, then how does PD compare to either hemodialysis (HD) or continuous renal replacement therapy (CRRT) in the form of continuous venovenous hemofiltration or continuous venoarterial hemofiltration. The latter modalities have been referred to as extracorporeal therapy.

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confidence: 86%

The complexity of dialytic therapy in hyperammonemic neonates

Bunchman

2015

Pediatr Nephrol

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“…Recently, it has been demonstrated that HD has significant clearance of citrulline but not arginine. 31 These authors also demonstrated that acute HD removes glutamine and glycine and may well inhibit further ammoniagenesis in children with urea cycle defects. 31 Limitations of this study are many.…”

Section: Discussionmentioning

confidence: 95%

Renal replacement therapy in the treatment of confirmed or suspected inborn errors of metabolism

McBryde

Kershaw

Bunchman

et al. 2006

The Journal of Pediatrics

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“…HD also enhances the efficiency of nitrogen removal through the clearance of amino acids such as citrulline, glycine, or glutamine. Current recommendations clearly point to HD as a primary therapy for patients with severe hyperammonemia (>1,500 mmol/L), and once serum ammonia levels are <200 µmol/L, treatment should be transitioned over to CRRT for rebound control [89,[91][92][93]. Whereas ammonia extraction via HD has been promoted as more efficient than other modalities, multiple dialysis sessions are often needed due to potential "rebound" in the circulation of toxic metabolites.…”

Section: Dialysismentioning

confidence: 99%

Hyperammonemia in review: pathophysiology, diagnosis, and treatment

2011

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Ammonia is an important source of nitrogen and is required for amino acid synthesis. It is also necessary for normal acid-base balance. When present in high concentrations, ammonia is toxic. Endogenous ammonia intoxication can occur when there is impaired capacity of the body to excrete nitrogenous waste, as seen with congenital enzymatic deficiencies. A variety of environmental causes and medications may also lead to ammonia toxicity. Hyperammonemia refers to a clinical condition associated with elevated ammonia levels manifested by a variety of symptoms and signs, including significant central nervous system (CNS) abnormalities. Appropriate and timely management requires a solid understanding of the fundamental pathophysiology, differential diagnosis, and treatment approaches available. The following review discusses the etiology, pathogenesis, differential diagnosis, and treatment of hyperammonemia.

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Clearance of amino acids by hemodialysis in argininosuccinate synthetase deficiency

Cited by 32 publications

References 31 publications

The complexity of dialytic therapy in hyperammonemic neonates

The complexity of dialytic therapy in hyperammonemic neonates

Renal replacement therapy in the treatment of confirmed or suspected inborn errors of metabolism

Hyperammonemia in review: pathophysiology, diagnosis, and treatment

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