Successful extracorporeal treatment of a male with hyperammonaemic coma

Haller, Maria; Boulanger, Angelika Henzler-Le; Sass, Jörn Oliver; Brandis, M.; Zimmerhackl, Lothar Bernd

doi:10.1093/ndt/gfh495

Cited by 4 publications

(5 citation statements)

References 3 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“… Expanded information on the included studies is available online in Supplementary Table 2 (refs 31 , 37 – 39 , 46 , 48 – 53 , 56 – 67 ). ASL, argininosuccinate lyase; CAVHDF, continuous arteriovenous haemodiafiltration; CAVHF, continuous arteriovenous haemofiltration; CoA, coenzyme A; CPS, carbamoyl phosphate synthetase; CVVH, continuous venovenous haemofiltration; CVVHD, continuous venovenous haemodialysis; CVVHDF, continuous venovenous haemodiafiltration; ECMO, extracorporeal membrane oxygenation; HD, haemodialysis; HF, haemofiltration; HUS, haemolytic uraemic syndrome; IQR, interquartile range; KRT, kidney replacement therapy; MMA, methyl malonic acidaemia; MSUD, maple syrup urine disease; OTC, ornithine transcarbamylase; PA, propionic acidaemia; PD, peritoneal dialysis; UCD, urea cycle disorders.…”

Section: Methodsmentioning

confidence: 99%

See 1 more Smart Citation

Consensus guidelines for management of hyperammonaemia in paediatric patients receiving continuous kidney replacement therapy

et al. 2020

View full text Add to dashboard Cite

Hyperammonaemia in children can lead to grave consequences in the form of cerebral oedema, severe neurological impairment and even death. In infants and children, common causes of hyperammonaemia include urea cycle disorders or organic acidaemias. Few studies have assessed the role of extracorporeal therapies in the management of hyperammonaemia in neonates and children. Moreover, consensus guidelines are lacking for the use of non-kidney replacement therapy (NKRT) and kidney replacement therapies (KRTs, including peritoneal dialysis, continuous KRT, haemodialysis and hybrid therapy) to manage hyperammonaemia in neonates and children. Prompt treatment with KRT and/or NKRT, the choice of which depends on the ammonia concentrations and presenting symptoms of the patient, is crucial. This expert Consensus Statement presents recommendations for the management of hyperammonaemia requiring KRT in paediatric populations. Additional studies are required to strengthen these recommendations.

show abstract

Section: Methodsmentioning

confidence: 99%

“…In patients with inborn errors of metabolism, high HD infusion rates enable the administration of calories (along with ammonia clearance) to prevent catabolism 41 , 48 . An HD protocol is listed in Supplementary Information 3 .…”

Section: Consensus Panel Recommendationsmentioning

confidence: 99%

Consensus guidelines for management of hyperammonaemia in paediatric patients receiving continuous kidney replacement therapy

et al. 2020

View full text Add to dashboard Cite

show abstract

“…If the foregoing therapies fail to yield any appreciable change in the blood ammonia level within a few hours, hemodialysis or peritoneal dialysis should be used. Hemodialysis and HDF are more effective for reducing serum ammonia level as a result of higher dialysance 1,2 There are increasing reports about HDF use in the acute phase of hyperammonemia in infants [3][4][5][6][7] The advantage of continuous HDF over HD is that continuous HDF causes less hemodynamic instability during ultrafiltration. Furthermore, continuous HDF is reported to be superior to HD and PD in the aspect of ammonia clearance, because the pore size of the filter is more suitable for removal of ammonia compared with that of HD.…”

Section: Discussionmentioning

confidence: 99%

“…Different dialysis modalities, such as continuous hemofiltration, intermittent hemodialysis (HD), continuous hemodialysis and continuous hemodiafiltration (CHDF) are the most effective treatment methods for decreasing serum ammonia levels [1][2][3][4][5][6][7][8][9][10] . Continuous venovenous hemodiafiltration (CVVHDF) is a modality choice to treat acute decompensation in inborn error of metabolism.…”

Section: Introductionmentioning

confidence: 99%

Hiperamonemili Üç Yenidoğan Bebekte Devamlı Venovenöz Hemodiafiltrasyon

Yapıcıoğlu¹,

Yıldızdaş²,

Özlü³

et al. 2015

Cukurova Medical Journal

View full text Add to dashboard Cite

In newborns, hyperammonemia leads to encephalopathy which is usually characterized by vomiting, hypotonia, lethargy, seizures and coma. Continuous venovenous hemodiafiltration (CVVHDF) is a modality choice to treat acute decompensation in hyperammonemia. Here we report three newborn patients with hypotonia, convulsion and hyperammonaemia. In the first three days of life, their serum ammonia levels were 4609, 1023 and 1949 µg/ml. They were successfully treated with CVVHDF and serum ammonia levels subsequently decreased to 268, 164 and 65 µg/ml in the first 24 hours of treatment. The complications were mild hypothermia and anemia. Key words: Hemodiafiltration, Neonate, Hyperammonemia. ÖZETYenidoğan bebeklerde hiperamonemi, hipotoni, letarji, nöbetler ve koma ile karakterize ensefalopati tablosuna yol açabilir. Akut dekompanse olmuş hiperamonemi tedavisinde devamlı venovenöz hemodiafiltrasyon tedavi seçeneği olabilir. Burada hipotoni, konvülziyon ve hiperamonemi olan üç yenidoğan olgusu sunulmuştur.yaşamlarının ilk üç gününde amonyak değerleri 4609, 1023, 1949 microgram/ml idi. Devamlı venovenöz hemodiafiltrasyon ile başarılı bir şekilde tedavi edilerek tedavinin ilk 24 saatinde amonyak değerleri 268, 164 65 microgram/ml'ye azaltıldı.hafif hipotermi ve anemi komplikasyon olarak saptandı.

show abstract

“…The various alternatives for dialysis have been reviewed elsewhere [23]. ECMO/ haemodialysis and haemodiafiltration are the most effective [7,23] but they are technically more demanding. Although somewhat less effective, continuous veno-venous haemofiltration is widely available and less likely to cause serious complications [9].…”

Section: Dialysismentioning

confidence: 99%

Hypothesis: proposals for the management of a neonate at risk of hyperammonaemia due to a urea cycle disorder

2007

View full text Add to dashboard Cite

It is difficult to prevent hyperammonaemia in patients with urea cycle disorders that present in the newborn period. This is true, even if treatment is started prospectively because of an affected relative. We propose several additional measures that could be used in conjunction with conventional therapy to improve the metabolic control. Catabolism could be reduced by delivering the babies by elective caesarean section, by starting intravenous glucose immediately after delivery and, possibly, by using beta-blockers or octreotide and insulin. The effectiveness of sodium benzoate and sodium phenylbutyrate might be increased by giving phenobarbital to the mother before delivery and subsequently to the baby to induce the enzymes for conjugation. We would expect the proposed measures to reduce the risk of hyperammonaemia and to improve the outcome for these patients. They have not, however, previously been used in this context, so families would need to be counselled carefully and controlled studies should be undertaken.

show abstract

Successful extracorporeal treatment of a male with hyperammonaemic coma

Cited by 4 publications

References 3 publications

Consensus guidelines for management of hyperammonaemia in paediatric patients receiving continuous kidney replacement therapy

Consensus guidelines for management of hyperammonaemia in paediatric patients receiving continuous kidney replacement therapy

Hiperamonemili Üç Yenidoğan Bebekte Devamlı Venovenöz Hemodiafiltrasyon

Hypothesis: proposals for the management of a neonate at risk of hyperammonaemia due to a urea cycle disorder

Contact Info

Product

Resources

About