gulsen

yes you're right some of them are associated with hypoketotic hypoglycemia which was more logical for these pathway blocks. But most of them make ketosis.

thank you for the answer but ketosis is seen in some organic acidemias such as propionic acidemia. while i was looking for a reference for this information, luckily i found the answer.

organic acids can inhibit gluconeogenesis therefore fatty acid oxidation increases to meet body's energy requirement. Also organic acidemias cause hyperammonemia by inhibiting urea cycle. But thanks again, i searched a lot, but couldn't find the answer till now...

reference: https://books.google.com.tr/books?id=a7CqcE1ZrFkC&pg=RA3-PA987&dq=organic+acidemia+ketosis&hl=tr&sa=X&redir_esc=y#v=onepage&q=organic%20acidemia%20ketosis&f=false

Maybe the questoin is simple but i couldn't find an answer in clinical textbooks. organic acidemias are related with amino acid catabolism and there's a block in catabolism pathway. so if the amino acids can't degrade to their end products such as pyruvate or acetyl coA, where the ketone bodies seen in these disorders come from?