Amino acid clearance during acute metabolic decompensation in maple syrup urine disease treated with continuous venovenous hemodialysis with filtration

Hmiel, S. Paul; Martin, Rick A.; Landt, Michael; Levy, Fiona H.; Grange, Dorothy K.

doi:10.1097/01.pcc.0000113265.92664.91

Cited by 16 publications

(12 citation statements)

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“…Should medical therapy fail to reduce the toxic metabolites, the use of RRT allows for the rapid removal of the accumulated plasma toxins, such as ammonia. [9][10][11][12][13][14][15][16][17][18][19][20] Early initiation of RRT should be the standard of care, as continuing with medical therapy that has failed to control the metabolic derangement would be expected to increase the risks for neurologic and developmental morbidity and mortality. Additional support for earlier initiation of RRT may be found in the relationship between the plasma ammonia concentration and outcome in our study population.…”

Section: Discussionmentioning

confidence: 99%

“…7,8 Renal replacement therapy (RRT), such as PD, HD, and CRRT, allows for the efficient removal of toxic metabolites to safer levels while diagnostic studies are being performed and minimizes the duration of the metabolic derangements shown to adversely affect long-term outcome. [9][10][11][12][13][14][15][16][17][18][19][20] The application of RRT to metabolic disturbances has changed dramatically over the past 20 years. Initial studies by Donn et al 7 compared exchange transfusion, PD, and HD and concluded that HD is the preferred modality for treating hyperammonemia caused by urea cycle defects.…”

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confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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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“…Given that a newborn has an approximate 0.2 m 2 body surface area, it can be expected that leucine plasma levels in older children will decrease at a rate similar to that shown in Figure 1 if a leucine clearance of at least 35 mL/min · 1.73 m 2 (4 mL/min ϫ 1.73/0.2) is done. In a 10-y-old child with acute-onset MSUD, Hmiel et al (7) reported a leucine clearance of 22.7 mL/min · 1.73 m 2 with plasma levels dropping from an initial value of 2585 M to a level below 1000 M after 24 h of CECRT (61% decrease). According to our kinetic modeling and with a leucine clearance of 35 mL/min · 1.73 m 2 , 6 -8 h would probably have been sufficient to attain a 60% decrease.…”

Section: Discussionmentioning

confidence: 99%

“…If these settings are not used during CECRT, it can still be ascertained that, when the leucine clearance reaches values Ͼ4 mL/min, the remain- ing duration of the session will be Ͻ7 h regardless of the method (hemofiltration, hemodialysis, or hemodiafiltration) or hemofilter used. In fact, because creatinine and leucine are neutral molecules without protein binding and with a similar molecular weight (113 D and 131 D, respectively) and mass transfer coefficient, creatinine clearance may be used in clinical practice to estimate leucine clearance if rapid chromatography analysis is not available (7).…”

Section: Discussionmentioning

confidence: 99%

Kinetic Modeling of Plasma Leucine Levels during Continuous Venovenous Extracorporeal Removal Therapy in Neonates with Maple Syrup Urine Disease

et al. 2005

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A kinetic modeling of leucine plasma concentration changes is proposed to describe the plasma leucine reduction rate during continuous extracorporeal removal therapy (CECRT) in neonates with maple syrup urine disease. Data were obtained from seven neonates using a bicompartmental model for the best fitted curve of plasma leucine decrease during CECRT. During the first 3 h, leucine plasma levels decreased according to an exponential curve: [Leu] -0.05t . Plasma leucine levels obtained from three other neonates were similar to those predicted by the model. The apparent distribution volumes for leucine that correspond to the two exponential equations obtained were calculated from the leucine mass removal collected in the spent dialysate and ultrafiltrate. The distribution volume was 34 Ϯ 3% of body weight during the first 3 h of CECRT and 72 Ϯ 7% from h 4 to the end of CECRT. These figures are similar to known values for the extracellular water compartment and for total body water in the newborn. The findings suggest that leucine handling during CECRT is similar to that of nonprotein-bound small-molecularweight solutes such as urea. MSUD is an inherited metabolic disease due to a deficiency in branched chain ketoacid dehydrogenase that leads to the accumulation of BCAA (leucine, isoleucine, and valine) and their keto-acid derivatives (1). Because of this impaired enzyme activity, leucine accumulates in cells and body fluids when the load exceeds leucine metabolism capacity and/or when a increased protein catabolism occurs. Given that renal clearance of BCAA and its derivatives is poor and due to the fact that their acute elevation causes neurologic deterioration, CECRT is indicated in severe cases and/or when nutritional support free of BCAA is not possible (2).Because assessment of leucine plasma levels by amino acid chromatography is not easily available and is time consuming, the aim of this study was to design a kinetic model that predicts leucine plasma level changes over time during the acute phase of the illness in neonates on CECRT. METHODS Patients.Between January 1991 and April 2000, seven neonates with MSUD presented with a severe acute deterioration of their status. After a 6-h period of conservative management that included correction of dehydration and increased caloric intake, all seven patients presented two of the three following criteria that are associated with a high risk of severe brain damage: comatose state, gastrointestinal intolerance, and leucine plasma levels Ն1700 M. The patients were referred to pediatric and neonatal intensive care for alternative therapies, in particular CECRT (3). Blood and spent dialysate samples were collected to monitor treatment efficiency. We retrospectively used these biologic analyses to elaborate the parameters involved in leucine kinetic modeling during CECRT (Table 1). Subsequently, between May 2000 and December 2001, three other neonates were treated with the same therapeutic strategy and were retrospectively studied to validate the leucine kinetic model...

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“…Most publications pertaining to PICU management of IEM describe small cohorts of patients and focus on specific aspects of diagnosis and treatment [1,3,9,11,17]. There are no studies that accurately describe the resources and training required to care for these patients in PICUs.…”

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confidence: 98%

Impact of inborn errors of metabolism on admission and mortality in a pediatric intensive care unit

et al. 2006

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The authors conducted a retrospective analysis of the patients admitted to a pediatric intensive care unit (PICU) during a five-year period, with specific focus on those with a suspected or confirmed diagnosis of inborn errors of metabolism (IEM), in order to ascertain the resources required to care for these patients. Medical records were reviewed for all admissions between January 1998 and December 2002 in a single metabolic referral center, and a subset of patients were identified with suspected IEM at admission or diagnosed IEM at hospital discharge. These patient charts were then further reviewed and the following information was extracted: IEM diagnosis, demographic data, biochemical characteristics at admission, need for mechanical ventilation, use of extracorporeal removal therapy, and outcome at PICU discharge. The study population comprised 70 patients (2.2% of all admissions during the study period) and included 33 neonates and 37 children aged >28 days. IEM diagnosis was known at the time of admission to the PICU in 9/33 of the neonates and 23/37 of the older children. Forty-three of the patients required invasive mechanical ventilation, while continuous extracorporeal removal therapy was used in 27 children. The median length of PICU stay was 3 days (range, 1 to 13 days) and 20 patients (28.6%) died. In conclusion, these observations show that inherited metabolic disease may be as frequent a primary diagnosis as septic shock in some PICUs. In neonates, these diseases are not usually diagnosed prior to PICU admission. Patients with IEM admitted to a PICU require aggressive support (including mechanical ventilation and extracorporeal removal therapies), and consume significant resources for relatively short PICU stays. These patients constitute a significant diagnostic and therapeutic challenge for pediatric intensivists.

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Amino acid clearance during acute metabolic decompensation in maple syrup urine disease treated with continuous venovenous hemodialysis with filtration

Abstract: Continuous venovenous hemodialysis with filtration provides an effective therapeutic alternative to intermittent hemodialysis during acute metabolic decompensation in maple syrup urine disease.

Cited by 16 publications

References 11 publications

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

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

Kinetic Modeling of Plasma Leucine Levels during Continuous Venovenous Extracorporeal Removal Therapy in Neonates with Maple Syrup Urine Disease

Impact of inborn errors of metabolism on admission and mortality in a pediatric intensive care unit

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