Clinical Course and Nutritional Management of Propionic and Methylmalonic Acidemias
Joint Authors
Mobarak, Amira
Dawoud, Heba
Nofal, Hanaa
Zoair, Amr
Source
Journal of Nutrition and Metabolism
Issue
Vol. 2020, Issue 2020 (31 Dec. 2020), pp.1-9, 9 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2020-09-16
Country of Publication
Egypt
No. of Pages
9
Main Subjects
Abstract EN
Propionic and methylmalonic acidemias result in multiple health problems including increased risk for neurological and intellectual disabilities.
Knowledge regarding factors that correlate to poor prognosis and long-term outcomes is still limited.
In this study, we aim to provide insight concerning clinical course and long-term complications by identifying possible correlating factors to complications.
Results.
This is a retrospective review of 20 Egyptian patients diagnosed with PA (n = 10) and MMA (n = 10) in the years 2014–2018.
PA patients had lower DQ/IQ and were more liable to hypotonia and developmental delay.
The DQ/IQ had a strong negative correlation with length of hospital stay, frequency of PICU admissions, time delay until diagnosis, and the mode ammonia level.
However, DQ/IQ did not correlate with age of onset of symptoms or the peak ammonia level at presentation.
Both the growth percentiles and albumin levels had a positive correlation with natural protein intake and did not correlate with the total protein intake.
Additionally, patients on higher amounts of medical formula did not necessarily show an improvement in the frequency of decompensation episodes.
Conclusion.
Our findings indicate that implementation of NBS, vigilant and proactive management of decompensation episodes, and pursuing normal ammonia levels during monitoring can help patients achieve a better neurological prognosis.
Furthermore, patients can have a better outcome on mainly natural protein; medical formula should only be used in cases where patients do not meet 100–120% of their DRI from natural protein.
American Psychological Association (APA)
Mobarak, Amira& Dawoud, Heba& Nofal, Hanaa& Zoair, Amr. 2020. Clinical Course and Nutritional Management of Propionic and Methylmalonic Acidemias. Journal of Nutrition and Metabolism،Vol. 2020, no. 2020, pp.1-9.
https://search.emarefa.net/detail/BIM-1188750
Modern Language Association (MLA)
Mobarak, Amira…[et al.]. Clinical Course and Nutritional Management of Propionic and Methylmalonic Acidemias. Journal of Nutrition and Metabolism No. 2020 (2020), pp.1-9.
https://search.emarefa.net/detail/BIM-1188750
American Medical Association (AMA)
Mobarak, Amira& Dawoud, Heba& Nofal, Hanaa& Zoair, Amr. Clinical Course and Nutritional Management of Propionic and Methylmalonic Acidemias. Journal of Nutrition and Metabolism. 2020. Vol. 2020, no. 2020, pp.1-9.
https://search.emarefa.net/detail/BIM-1188750
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1188750