<prompt> In a child with cerebrohepatorenal syndrome and with hypotonia and hepatomegaly, the probable biochemical defect is accumulation of:PyruvateShort chain fatty acidsVery long chain fatty acidsAcetyl CoA  <response>: Ans. C. Very long chain fatty acids VCLFAsCerebrohepatorenal syndrome is also known as Zellweger syndrome, a rare congenital disorder. It is caused due to the reduction or absence of the peroxisomes in the cell. Peroxisomes are the site of oxidation of VCLFAs.Deficiency of peroxisomes leads to increased concentration of VLCFAs. 