A good example is the organic acid disorder propionic acidemia. While elevation in propionic acid causes an anion gap acidosis, there are many other effects including a functional pyloric stenosis. Some patients have even gone to surgery for pyloromyotomy in the past. This results in a deficiency of acetyl CoA, the substrate for oxidative phosphorylation and an important regulator of the urea cycle. Secondary lactic acidosis and hyperammonemia result.
Patients often have hypoglycemia.
Propionic acid is also conjugated to carnitine and excreted in the urine depleting the body of carnitine. Carnitine depletion causes a block in the metabolism of fatty acids, further interrupting energy generation. Prevent catabolism - the dietary intake of offending substances is usually a small fraction of the amount contained within the body. Since cellular proteins turn over about every 24 hours, an increase in catabolism due to stress from infection, surgery, birth, etc. can rapidly overwhelm the compensatory mechanisms and result in clinical decompensation. Administration of calories is used in the treatment of acute episodes to try to slow down catabolism.