Peritoneal Dialysis in the Treatment of Metabolic Crises Caused by Inherited Disorders of Organic and Amino Acid Metabolism

Gortner, Ludwig; Leupold, D.; Pohlandt, Frank; Bartmann, Peter

doi:10.1111/j.1651-2227.1989.tb11130.x

Cited by 40 publications

(20 citation statements)

References 20 publications

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“…The BCAA and a-ketoisocaproate blood levels obtained in our experimental model were close to those observed in neonates with MSUD when extracorporeal blood purification was required (Wendel et al 1980;Gortner et al 1989;Thompson et al 1991;Ring and Zobel 1992;Falk et al 1994). The epurative procedures were performed with a haemo(dia)filter and operational parameters already used in newborn infants.…”

Section: Discussionmentioning

confidence: 73%

“…The a-ketoisocaproate renal clearance is very low (Langenbeck et al 1979) and data about removal by peritoneal dialysis are scarce. Peritoneal clearance of a-ketoisocaproate in MSUD has been reported to be 0.62 ml/min in one neonate (Gortner et al 1989) and 1.1-1.9ml/min in an 18-month-old infant (Wendel et al 1980). The present study demonstrated that VVHDF gave higher a-ketoisocaproate clearances as compared with VVHE The a-ketoisocaproate clearances obtained with VVHDF at a 2L/h dialysate flow rate were 3.1 +0.7 and 5.0 + 1.2ml/min when Qb was 8.3 and 16.6 ml/min, respectively.…”

Section: Discussionmentioning

confidence: 98%

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Removal of branched‐chain amino acids andα‐ketoisocaproate by haemofiltration and haemodiafiltration

Semama

Prevot

et al. 1995

J of Inher Metab Disea

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Venovenous haemofiltration (VVHF) and haemodiafiltration (VVHDF) were performed with a neonatal haemo(dia)filter (Miniflow 10, Hospal) on 8 anaesthetized rabbits infused with branched-chain amino acids (leucine, isoleucine and valine) and alpha-ketoisocaproate. The branched-chain amino acids (BCAA) and alpha-ketoisocaproate blood levels were close to those previously observed in neonates with maple syrup urine disease when extracorporeal blood purification was required. VVHF and VVHDF performances were assessed with two different blood flows (Qb = 8.3 and 16.6 ml/min). VVHDF was performed with four dialysate flow rates (Qd = 0.5, 1.0, 2.0 and 3.0 L/h). Within each period, clearances of the three BCAA were strictly similar. BCAA clearances obtained by VVHF were similar to ultrafiltration rates (respectively, 0.78 +/- 0.14 and 1.79 +/- 0.28 ml/min at high and low Qb; p < 0.05). The alpha-ketoisocaproate clearances obtained by VVHF were 0.39 +/- 0.17 and 0.92 +/- 0.43 ml/min at low and high Qb (not significantly different). Whatever the Qd value, the VVHDF procedures always allowed higher BCAA and alpha-ketoisocaproate clearances as compared with the corresponding VVHF period with similar Qb. BCAA clearances obtained by VVHDF with a 0.5 L/h dialysate flow were 4.1 +/- 0.5 and 5.4 +/- 0.5 mL/min at low and high Qb, respectively. The concurrent alpha-ketoisocaproate clearances were 2.5 +/- 0.8 and 2.9 +/- 1.0 ml/min.

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Section: Discussionmentioning

confidence: 73%

Section: Discussionmentioning

confidence: 98%

Removal of branched‐chain amino acids andα‐ketoisocaproate by haemofiltration and haemodiafiltration

Semama

Prevot

et al. 1995

J of Inher Metab Disea

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“…However, peritoneal dialysis, at present, is not a preferred modality for the removal of ammonia because of slow rates of removal and unpredicted complications [21,22,23].…”

Section: Discussionmentioning

confidence: 99%

Acute hemodialysis for hyperammonemia in small neonates

Rajpoot

Gargus

2004

Pediatric Nephrology

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Neonatal metabolic emergencies require a multidisciplinary team approach for supportive management that has increasingly come to feature renal replacement therapies in addition to nutritional support, the use of pharmaceutical agents, and testing to guide management and provide a definitive diagnosis. An increased appreciation for the mechanisms involved in ammonia neurotoxicity has placed greater emphasis on the need for its rapid yet safe resolution to optimize long-term prognosis. We examined our experience of intermittent hemodialysis (HD) and considered (1) the feasibility of HD in low-weight neonates, (2) the rate of decrease in ammonia, (3) complications during HD in small neonates weighing <4 kg presenting at University Children's Hospital between 1999 and 2002. Additionally, we review the current cellular and molecular mechanism of ammonia-induced brain injury. All patients tolerated intermittent HD and all required pressor agents. We primed all our patients with 20 U/kg of heparin and there was no subsequent need for further heparinization. We also noted that hemodynamic instability persisted during the first 1-2 h of the procedure and improved thereafter, as indicated by a decreased need for pressor agents. All neonates are alive to date. In conclusion, HD remains an effective and practical mode of renal replacement therapy having readily managed complications in preterm neonates weighing <4 kg with metabolic disorders.

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“…If it is the only available modality, it can be prescribed using 1.5% standard dextrose fluid, exchange volumes of 40-50 ml/kg, with hourly cycles for up to 36 h (to allow removal of most of the ammonia burden), or longer in UCD. Potential complications of PD include obstruction, leakage, infection, and mechanical failure [13,77,96].…”

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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Peritoneal Dialysis in the Treatment of Metabolic Crises Caused by Inherited Disorders of Organic and Amino Acid Metabolism

Cited by 40 publications

References 20 publications

Removal of branched‐chain amino acids andα‐ketoisocaproate by haemofiltration and haemodiafiltration

Removal of branched‐chain amino acids andα‐ketoisocaproate by haemofiltration and haemodiafiltration

Acute hemodialysis for hyperammonemia in small neonates

Hyperammonemia in review: pathophysiology, diagnosis, and treatment

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