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Maple Syrup Urine Disease

Maple Syrup Urinal
Picmonic
Maple syrup urine disease, also called branched-chain ketoaciduria, is an autosomal recessive metabolic disorder caused by a deficiency of the alpha ketoacid dehydrogenase complex. This complex of enzymes is responsible for the degradation of branched chain amino acids including isoleucine, leucine, and valine. Defects in this enzyme cause the presence of sweet-smelling urine, similar to that of maple syrup leading to the name maple syrup urine disease. Infants with this metabolic disorder initially seem healthy at birth but if left untreated can suffer from seizures, CNS defects, and mental retardation. Other nonspecific symptoms include poor feeding, vomiting, dehydration, lethargy and hypotonia. Treatment includes diet with minimal levels of branched chain amino acids necessary for metabolic functions. 
7 KEY FACTS
PATHOPHYSIOLOGY
Defect in alpha ketoacid dehydrogenase
Broken Afro Key with Acidic-lemon Dehydrator

Maple syrup urine disease, also called branched-chain ketoaciduria, is a metabolic disorder caused by a deficiency of the alpha ketoacid dehydrogenase complex. This enzyme complex structure is analogous to alpha ketoglutarate dehydrogenase and pyruvate dehydrogenase, requiring the same cofactors thiamine diphosphate, FAD, NAD , lipoate, and coenzyme A. Alpha ketoacid dehydrogenase is responsible for degradation of branched chain amino acids.

Blocked degradation of branched chain amino acids
Branched Chains

Alpha ketoacid dehydrogenase is responsible for degradation of branched chain amino acids isoleucine, leucine, and valine.

Leucine
Lucy

Leucine is a branched chain amino acid that accumulates in the blood and urine in maple syrup urine disease.

Isoleucine
Ice-Lucy

Isoleucine is a branched chain amino acid that accumulates in the blood and urine in maple syrup urine disease.

Valine
Veil

Valine is a branched chain amino acid that accumulates in the blood and urine in maple syrup urine disease.

SIGNS AND SYMPTOMS
Seizures
Caesar

A seizure is defined as a transient episode of abnormal, excessive neuronal activity. Seizures can be a symptom of maple syrup urine disease.

Intellectual Disability (Mental Retardation)
Read-tarred-book

Infants with this metabolic disorder initially seem healthy at birth but if left untreated can suffer from CNS defects and mental retardation.

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