Oasthouse syndrome

The most Important use of body odors in disease diagnosis relates to the infant diseases involving errors in amino acid metabolism. Strong and unusual odors are manifest in the breath, sweat, and urine of these individuals. Table II summarizes several known acidurias, the amino acids that are not properly metabolized, and the odors associated with the compounds which accumulate and can be detected in the urine ( ). In the case of the Maple Syrup Urine and Oasthouse syndrome, the keto- and hydroxy- acids which have been identified may not be responsible for the observed maple and celery/yeast odors (.9). Alternatively, these odors could be the result of conversion of 2-keto-butyrlc acid to methyl-ethyl-tetronlc acid (Slusser s lactone) which is used as an extender in maple and celery flavors and has a maple syrup-like odor (R. Soukup, personal communication). With these acidurias it is imperative that an immediate diagnosis is made, since corrective diet can prevent the brain damage that results from these diseases. This is readily done on an olfactory basis which can subsequently be supported by gas chromatographic... 

Hyperaminoaciduria (oasthouse syndrome) Infant skin Dried malt or hops... 

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