| Smell
| # of Reports
in PWS
| Significance
|
acidic, acid-like
(vinegar ?)
| 1
(vinegary)
| methylmalonic acidemia
|
cabbage-like, boiled or rotten cabbage
"rotten mushrooms"
| -
| tyrosinemia type I,
methionine adenosyltransferase deficiency [ OMIM ]
|
| tomcat urine
| -
| 3-methylcrotonylglycinuria,
multiple carboxylase deficiency,
propionyl-CoA carboxylase deficiency
|
| fishy
| 1
| trimethylaminuria
|
| musty, mousy
| -
| classical phenylketonuria (PKU)
|
| maple sugar, burnt sugar or caramel, sweet, curry
| 1
| maple sugar urine disease (MSUD) aka branched-chain amino aciduria
|
| rancid butter
| -
| tyrosinemia type I
|
| sulfurous
| -
| cystinuria
|
| sweaty feet, rancid urine
| -
| - isovaleric acidemia (inborn error of short-chain fatty acid metabolism and metabolic pathways closely related to MSUD, unusual odor is result of butyric and hexanoic acids)
- multiple acyl-CoA dehydrogenase deficiency (glutaric acidemia type II)
|
| "like a swimming pool" (chlorine?)
| -
| hawkinsinuria
|
| (sickly) sweet, fruity or acetone (nail polish remover)
| -
| diabetes, diabetic ketoacidosis
|
