Postpyloric nutrition to prevent emergencies - a step away from repeat inpatient care in children with methylmalonic acidaemia and propionic acidaemia - a case report of four cases

dc.contributor.authorSchumann, Stefan
dc.contributor.authorRommel, Frank Risto
dc.contributor.authorCantez, Serdar
dc.contributor.authorAlexanidou, Evdokia
dc.contributor.authorKamrath, Clemens
dc.contributor.authorde Laffolie, Jan
dc.date.accessioned2023-04-17T07:53:24Z
dc.date.available2023-04-17T07:53:24Z
dc.date.issued2023
dc.description.abstractMethylmalonic acidaemia (MMA) and propionic acidaemia (PA) are very rare autosomal recessive inherited metabolic diseases from the group of organoacidopathies. Katabolism due to minor infections can lead to metabolic decompensation including hyperammonemia and ketoacidosis, especially in small children. We present data from a small cohort to clarify whether placement of a percutaneous endoscopic gastrostomy with jejunal tube (J-PEG) reduce metabolic imbalances and hospital stays. The aim is to prevent emergencies from occurring by preventing metabolic derailments at an early stage. 4 patients with MMA (N = 3) or PA (N = 1) were included. Data were collected at every investigation, in particular pH value, pCO2, bicarbonate, base excess, ammonia and lactate. Due to repeated metabolic derailments, a percutaneous endoscopic gastrostomy was placed for postpyloric nutrition. In conclusion, placement of a percutaneous endoscopic gastrostomy with postpyloric tube appears to reduce the rate of metabolic decompensations. In addition, hospital stays and especially the number of treatment days can be reduced. This method, especially the placement of a postpyloric tube could enable parents to prevent catabolism when vomiting begins by continuously feeding through the jejunal part, as a step to prevent a metabolic emergency from occurring.
dc.identifier.urihttps://jlupub.ub.uni-giessen.de//handle/jlupub/16239
dc.identifier.urihttp://dx.doi.org/10.22029/jlupub-15622
dc.language.isoen
dc.rightsNamensnennung 4.0 International
dc.rights.urihttps://creativecommons.org/licenses/by/4.0/
dc.subjectpostpyloric nutrition
dc.subjectJ-PEG
dc.subjectprevention of emergencies
dc.subjectmethylmalonic acidaemia
dc.subjectMMA
dc.subjectpropionic acidaemia
dc.subjectPA
dc.subjectmetabolic decompensation
dc.subject.ddcddc:610
dc.titlePostpyloric nutrition to prevent emergencies - a step away from repeat inpatient care in children with methylmalonic acidaemia and propionic acidaemia - a case report of four cases
dc.typearticle
local.affiliationFB 11 - Medizin
local.source.articlenumber1078425
local.source.epage4
local.source.journaltitleFrontiers in Pediatrics
local.source.spage1
local.source.urihttps://doi.org/10.3389/fped.2023.1078425
local.source.volume11

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