0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Article |

A Dermatitis Secondary to Amino-Acid Deficiency in Treated Maple Syrup Urine Disease-Reply

George P. Giacoia, MD; Gerard T. Berry, MD
Arch Pediatr Adolesc Med. 1994;148(9):993-994. doi:10.1001/archpedi.1994.02170090107026.
Text Size: A A A
Published online

The comments of Koch et al are well taken. They further remind us of the rarity of acrodermatitis enteropathica—like illness as a complication of amino-acid imbalance in maple syrup urine disease and the fact that the more common complication is a periorificial dermatitis.

Since the skin is one of the tissues that turns over most rapidly, it is not surprising that it would be one of the first tissues to be affected by an amino-acid imbalance. It certainly seems possible that amino-acid imbalance as a consequence of secondary effects on cellular transport may be as important as a frank amino-acid deficiency. We believe that our patient probably represented the far spectrum of severe involvement with amino-acid imbalance.

Different biochemical abnormalities in patients with maple syrup urine disease with inadequate dietary intake (ie, isolated deficiency of isoleucine or of the three branched-chain amino acids1 or an abnormal leucine-isoleucine ratio),

Topics

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

First Page Preview

View Large
First page PDF preview

Figures

Tables

References

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

Related Content

Customize your page view by dragging & repositioning the boxes below.

Jobs
brightcove.createExperiences();