The findings in a male infant who died at 9 days of age of an illness characterized by jaundice, kernicterus, and evidence of hepatosteatosis are described. Chemical analyis of hepatic tissue obtained at autopsy showed the hepatosteatosis to be due to a striking increase in the concentration of total lipids and fatty acids. No known toxins, infections, blood group incompatibilities, or concurrent metabolic aberrations could be identified as playing an etiologic role. Four male siblings died previously within the first two weeks of life with clinical manifestations identical to those of the patient who underwent necropsy. Since all the patients were males, a sex-linked entity seems likely. It is believed that these patients may represent a previously undescribed genetic disorder of metabolism, the principal features of which are jaundice, kernicterus, and hepatosteatosis.

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