An unusual cause of hyperammonemia in a critically ill patient

Brasseur, Alexandre; Khattar, Lina; Bailly, Benjamin; Vincent, Jean‐Louis; Taccone, Fabio Silvio

doi:10.1007/s00134-012-2736-4

Cited by 4 publications

(4 citation statements)

References 4 publications

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“…Frequency of multiple organ failure (MOF) is significantly increasing in critically ill patients within the last decades with a prevalence of more than 30% [ 1 ]. Despite significant progress in management, mortality in patients with advanced stages of MOF is still excessively high.…”

Section: Introductionmentioning

confidence: 99%

“…Despite significant progress in management, mortality in patients with advanced stages of MOF is still excessively high. Recent publications indicate that more than 60% of these patients did not survive their stay at the ICU [ 1 , 2 ]. Liver failure is present in 20% of the ICU patients [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

“…Recent publications indicate that more than 60% of these patients did not survive their stay at the ICU [ 1 , 2 ]. Liver failure is present in 20% of the ICU patients [ 1 ]. Acute kidney injury (AKI) occurs in > 50% of critically ill patients [ 3 ] and is more common in association with various forms of liver disease [ 4 – 6 ].…”

Section: Introductionmentioning

confidence: 99%

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Advanced organ support (ADVOS) in the critically ill: first clinical experience in patients with multiple organ failure

Fuhrmann

Weber

Roedl

et al. 2020

Ann. Intensive Care

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Background: Prevalence of multiple organ failure (MOF) in critically ill patients is increasing and associated mortality remains high. Extracorporeal organ support is a cornerstone in the management of MOF. We report data of an advanced hemodialysis system based on albumin dialysis (ADVOS multi device) that can regulate acid-base balance in addition to the established properties of renal replacement therapy and albumin dialysis systems in critically ill patients with MOF. Methods: 34 critically ill patients with MOF received 102 ADVOS treatment sessions in the Department of Intensive Care Medicine of the University Medical Center Hamburg-Eppendorf. Markers of metabolic detoxification and acidbase regulation were collected and blood gas analyses were performed. A subgroup analyses were performed in patients with severe acidemia (pH < 7.2). Results: Median number of treatment sessions was 2 (range 1-9) per patient. Median duration of treatment was 17.5 (IQR 11-23) hours per session. Treatment with the ADVOS multi-albumin dialysis device caused a significant decrease in bilirubin levels, serum creatinine, BUN and ammonia levels. The relative elimination rate of bilirubin was concentration dependent. Furthermore, a significant improvement in blood pH, HCO 3 − and PaCO 2 , was achieved during ADVOS treatment including six patients that suffered from severe metabolic acidosis refractory to continuous renal replacement therapy. Delta pH, HCO 3 − and PaCO 2 were significantly affected by the ADVOS blood flow rate and pH settings. This improvement in the clinical course during ADVOS treatments allowed a reduction in norepinephrine during ADVOS therapy. Treatments were well tolerated. Mortality rates were 50% and 62% for 28 and 90 days, respectively. Conclusions: In this case series in patients with MOF, ADVOS was able to eliminate water-soluble and albuminbound substances. Furthermore, the device corrected severe metabolic and respiratory acid-base disequilibrium. No major adverse events associated with the ADVOS treatments were observed.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Advanced organ support (ADVOS) in the critically ill: first clinical experience in patients with multiple organ failure

Fuhrmann

Weber

Roedl

et al. 2020

Ann. Intensive Care

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“…Brain damage in OTCD patients has been reported to correlate with the severity and duration of acute hyperammonemia, especially in neonatal patients, although this has not been confirmed by all studies . Findings of cognitive impairment and behavioral as well as emotional problems are consistent with a pattern of global cerebral atrophy, indicating an impact of NH 4 + ‐induced neurotoxicity affecting the whole brain.…”

Section: Introductionmentioning

confidence: 87%

Impairment of cognitive function in ornithine transcarbamylase deficiency is global rather than domain‐specific and is associated with disease onset, sex, maximum ammonium, and number of hyperammonemic events

Buerger

Garbade

Alber

et al. 2019

J of Inher Metab Disea

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Beginning in 2006, the Urea Cycle Disorders Consortium (UCDC) has conducted a longitudinal study of eight inherited deficiencies of enzymes and transporters of the urea cycle, including 444 individuals with ornithine transcarbamylase deficiency (OTCD), of whom 300 (67 males, 233 females) received psychological evaluation. In a cross‐sectional study (age range, 3‐71 years), analysis of covariance (ANCOVA) determined the association between outcomes in five cognitive domains (global intelligence, executive functions, memory, visuomotor integration, visual perception) and sex, age at testing and timing of disease onset defined as early onset (≤28 days; EO), late onset (LO), or asymptomatic (AS). The dataset of 183 subjects with complete datasets (31 males, 152 females) revealed underrepresentation of EO subjects (2 males, 4 females), who were excluded from the ANCOVA. Although mean scores of LO and AS individuals were within 1 SD of the population norm, AS subjects attained significantly higher scores than LO subjects and males higher scores than females. Correlations between cognitive domains were high, particularly intelligence proved to be a distinguished indicator for cognitive functioning. Maximum plasma ammonium concentration and intelligence correlated significantly higher in EO (r = −0.47) than in LO subjects (r = 0.04). Correlation between the number of hyperammonemic events and intelligence scores were similar for EO (r = −0.30) and LO (r = −0.26) individuals. The number of clinical symptoms was significantly associated with intelligence (r = −0.28) but not with scores in other domains. Results suggest that OTCD has a global impact on cognitive functioning rather than a specific effect on distinct cognitive domains.

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An unusual cause of hyperammonemia in a critically ill patient

Cited by 4 publications

References 4 publications

Advanced organ support (ADVOS) in the critically ill: first clinical experience in patients with multiple organ failure

Advanced organ support (ADVOS) in the critically ill: first clinical experience in patients with multiple organ failure

Impairment of cognitive function in ornithine transcarbamylase deficiency is global rather than domain‐specific and is associated with disease onset, sex, maximum ammonium, and number of hyperammonemic events

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