Skin Lesions Associated with Nutritional Management of Maple Syrup Urine Disease
Joint Authors
Uaariyapanichkul, Jaraspong
Saengpanit, Puthita
Damrongphol, Ponghatai
Suphapeetiporn, Kanya
Chomtho, Sirinuch
Source
Case Reports in Dermatological Medicine
Issue
Vol. 2017, Issue 2017 (31 Dec. 2017), pp.1-4, 4 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2017-10-25
Country of Publication
Egypt
No. of Pages
4
Main Subjects
Abstract EN
Introduction.
Maple syrup urine disease (MSUD) is an inborn error of branched chain amino acids (BCAAs) metabolism.
We report an infant with MSUD who developed 2 episodes of cutaneous lesions as a result of isoleucine deficiency and zinc deficiency, respectively.
Case Presentation.
A 12-day-old male infant was presented with poor milk intake and lethargy.
The diagnosis of MSUD was made based on clinical and biochemical data.
Management and Outcome.
Specific dietary restriction of BCAAs was given.
Subsequently, natural protein was stopped as the patient developed hospital-acquired infections which resulted in an elevation of BCAAs.
Acrodermatitis dysmetabolica developed and was confirmed to be from isoleucine deficiency.
At the age of 6 months, the patient developed severe lethargy and was on natural protein exclusion for an extended period.
Despite enteral supplementation of zinc sulfate, cutaneous manifestations due to zinc deficiency occurred.
Discussion.
Skin lesions in MSUD patients could arise from multiple causes.
Nutritional deficiency including isoleucine and zinc deficiencies can occur and could complicate the treatment course as a result of malabsorption, even while on enteral supplementation.
Parenteral nutrition should be considered and initiated accordingly.
Clinical status, as well as BCAA levels, should be closely monitored in MSUD patients.
American Psychological Association (APA)
Uaariyapanichkul, Jaraspong& Saengpanit, Puthita& Damrongphol, Ponghatai& Suphapeetiporn, Kanya& Chomtho, Sirinuch. 2017. Skin Lesions Associated with Nutritional Management of Maple Syrup Urine Disease. Case Reports in Dermatological Medicine،Vol. 2017, no. 2017, pp.1-4.
https://search.emarefa.net/detail/BIM-1144979
Modern Language Association (MLA)
Uaariyapanichkul, Jaraspong…[et al.]. Skin Lesions Associated with Nutritional Management of Maple Syrup Urine Disease. Case Reports in Dermatological Medicine No. 2017 (2017), pp.1-4.
https://search.emarefa.net/detail/BIM-1144979
American Medical Association (AMA)
Uaariyapanichkul, Jaraspong& Saengpanit, Puthita& Damrongphol, Ponghatai& Suphapeetiporn, Kanya& Chomtho, Sirinuch. Skin Lesions Associated with Nutritional Management of Maple Syrup Urine Disease. Case Reports in Dermatological Medicine. 2017. Vol. 2017, no. 2017, pp.1-4.
https://search.emarefa.net/detail/BIM-1144979
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1144979