Influence of implementing a protocol for an intravenously administered ammonia scavenger on the management of acute hyperammonemia in a pediatric intensive care unit

Brossier, David; Goyer, Isabelle; Ziani, Lydia; Marquis, Christopher; Mitchell, Grant A.; Ozanne, Bruno; Jouvet, Philippe

doi:10.1002/jimd.12029

Cited by 7 publications

(6 citation statements)

References 37 publications

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“…57 Unfortunately, this study did not show a benefit to patient morbidity and mortality. 57 Non-pharmacological recommendations-detailed in Table 3 -in addition to hemodialysis, include adjustments to diet. Decreasing meat protein from the diet, whether by restriction of protein or replacement with dairy-based or vegetable protein, has been suggested as a method to limit substrates for ammoniagenesis.…”

Section: Treatmentmentioning

confidence: 62%

“…Additionally, the institution of set protocols for treating these patients may prove beneficial, as one study investigating a protocol for determining when to administer combined intravenous sodium benzoate and sodium phenylacetate was shown to significantly reduce the length of time to administration of these scavengers. 57 Unfortunately, this study did not show a benefit to patient morbidity and mortality. 57 …”

Section: Treatmentmentioning

confidence: 62%

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Hyperammonemia Post Lung Transplantation: A Review

Leger

Silverman

Hauck

et al. 2020

Clin Med Insights Circ Respir Pulm Med

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Hyperammonemia is the pathological accumulation of ammonia in the blood, which can occur in many different clinical settings. Most commonly in adults, hyperammonemia occurs secondary to hepatic dysfunction; however, it is also known to be associated with other pathologies, surgeries, and medications. Although less common, hyperammonemia has been described as a rare, but consistent complication of solid organ transplantation. Lung transplantation is increasingly recognized as a unique risk factor for the development of this condition, which can pose grave health risks—including long-term neurological sequelae and even death. Recent clinical findings have suggested that patients receiving lung transplantations may experience postoperative hyperammonemia at rates as high as 4.1%. A wide array of etiologies has been attributed to this condition. A growing number of case studies and investigations suggest disseminated opportunistic infection with Ureaplasma or Mycoplasma species may drive this metabolic disturbance in lung transplant recipients. Regardless of the etiology, hyperammonemia presents a severe clinical problem with reported mortality rates as high as 75%. Typical treatment regimens are multimodal and focus on 3 main avenues of management: (1) the reduction of impact on the brain through the use of neuroprotective medications and decreasing cerebral edema, (2) augmentation of mechanisms for the elimination of ammonia from the blood via hemodialysis, and (3) the diminishment of processes producing predominantly using antibiotics. The aim of this review is to detail the pathophysiology of hyperammonemia in the setting of orthotopic lung transplantation and discuss methods of identifying and managing patients with this condition.

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Section: Treatmentmentioning

confidence: 62%

Section: Treatmentmentioning

confidence: 62%

Hyperammonemia Post Lung Transplantation: A Review

Leger

Silverman

Hauck

et al. 2020

Clin Med Insights Circ Respir Pulm Med

View full text Add to dashboard Cite

show abstract

“…Sodium phenylacetate and sodium benzoate are available as a combined intravenous product. It facilitates faster nitrogen removal than sodium benzoate alone through the use of two conjugation pathways [39]. It is available as a brand name product, Ammonul ® , and as a generic.…”

Section: Nitrogen Scavengersmentioning

confidence: 99%

Treatment of Hyperammonemia Syndrome in Lung Transplant Recipients

Yun,

Scalia,

Farghaly

2023

JCM

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Hyperammonemia syndrome is a complication that has been reported to occur in 1–4% of lung transplant patients with mortality rates as high as 60–80%, making detection and management crucial components of post-transplant care. Patients are treated with a multimodal strategy that may include renal replacement therapy, bowel decontamination, supplementation of urea cycle intermediates, nitrogen scavengers, antibiotics against Mollicutes, protein restriction, and restriction of parenteral nutrition. In this review we provide a framework of pharmacologic mechanisms, medication doses, adverse effects, and available evidence for commonly used treatments to consider when initiating therapy. In the absence of evidence for individual strategies and conclusive knowledge of the causes of hyperammonemia syndrome, clinicians should continue to design multimodal regimens based on suspected etiologies, institutional drug availability, patient ability to tolerate enteral medications and nutrition, and availability of intravenous access.

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“…Прогностически неблагоприятными для жизни ребёнка, несмотря на оптимальную фармакологическую и диетическую терапию, являются метаболические нарушения, дебютирующие в раннем неонатальном периоде, сопровождающиеся рвотой, респираторным дистресс-синдромом, выраженной гипотермией, судорогами, комой на фоне респиpатоpного алкалоза и гипераммониемией [9][10][11]. Если манифестация заболевания пришлась на более поздние сроки (первые 3 года жизни и от 10 до 20 лет), шанс на сохранение жизни возрастает, хотя клиническая картина достаточно тяжела и весьма сходна с неонатальной формой проявления болезни [12,13]. Выделяют еще один вариант нарушения синтеза мочевины с частичной недостаточностью фермента и манифестацией заболевания на третьем десятке лет жизни, которое может проявляться как тяжёлыми метаболическими кризами, вплоть до летального исхода, так и бессимптомным течением, лишь с эпизодическими повышениями уровня печеночных ферментов [14].…”

Section: Introductionunclassified

Difficulties in diagnostics of urea synthesis cycle disturbance

Kolomeets,

Averyanova,

Khlynova

et al. 2023

jour

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Introduction. Hereditary metabolic diseases include a large group of diseases caused by genetic mutations with a high potential risk of transmitting this disorder to future offspring. Manifesting at any age and accompanied by recurrent and progressive clinical symptoms, some of these mutations become incompatible with life, while most lead to gross violations of the normal physiological process of child development. The paper presents a clinical case of hereditary urea synthesis disorder caused by deficiency of ornithine transcarbamylase of mitochondrial matrix in liver (hyperammonemia type 2) as a result of mutation of the OTC gene (MIM 300461). The disease recessive, linked with the X-chromosome and is associated with a high risk of mortality due to accumulation of toxic concentrations of ammonia. Materials and methods. The boy E., born in 2015. Introduction of protein-based complimentary foods and increase in its portion volume is associated with decrease in appetite, regurgitation, nausea, vomiting, flatulence, dyspepsia appear, and sharp weakness progresses. There is a significant increase in the blood concentration of intracellular liver enzymes. Physical development is slowed due to poor weight gain, and neuropsychiatric development lags significantly. Repeatedly examined in the hospital at the place of residence. Results obtained. A total of 47 genes with mutations causing hereditary diseases with predominant liver damage were studied by mass parallel sequencing. Substitution of c.523G>A (p.Asn175Asp) in the OTC gene in hemizygous state was detected. Clinical diagnosis was established: urea cycle metabolism disorder, hyperammonemia, ornithine transcarbamylase deficiency, E 72.2. Dietary recommendations were given. Carbaglu, Recordati, France at the rate of 100 mg/kg/24 h was prescribed on vital signs. Against the background of the therapy and dietary recommendations the condition of the child had a pronounced positive trend. Conclusions. Since the true incidence of this pathology in Russia has not been established (many cases of metabolic disorders of the urea cycle remain undiagnosed), the presented clinical case will allow clinicians, using modern possibilities of structural and functional analysis of the human genome, to make a timely diagnosis and prescribe adequate therapy, thereby not only prolonging the life of a young patient but also significantly improving its quality.

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Influence of implementing a protocol for an intravenously administered ammonia scavenger on the management of acute hyperammonemia in a pediatric intensive care unit

Cited by 7 publications

References 37 publications

Hyperammonemia Post Lung Transplantation: A Review

Hyperammonemia Post Lung Transplantation: A Review

Treatment of Hyperammonemia Syndrome in Lung Transplant Recipients

Difficulties in diagnostics of urea synthesis cycle disturbance

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