source: Neuromuscular Disorders
Doris TE, Bowron A, Armstrong A, Messer B
The longer survival in Duchenne dystrophy can be associated with previously unrecognised medical issues, in particular cardiac and nutritional problems, which provide new challenges in the management of these patients. We describe a series of patients who have DMD presenting with severe acid base abnormalities due to ketoacidosis. The cases described all developed the abnormalities in the context of decreased dietary intake. In all cases, the acid-base derangement resolved with intravenous glucose infusion. We conclude that decreased calorific intake for any reason can lead to very severe acid-base derangement in patients with DMD. The treatment is relatively simple once the problem has been identified. An awareness of this potential complication is important for all clinicians involved in the acute care of these patients.
Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle, UK
10.1016/j.nmd.2018.06.003 read more
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