FATAL FAMILIAL STEATOSIS OF MYOCARDIUM, LIVER AND KIDNEYS IN THREE SIBLINGS

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Abstract
A 2 day old male and 3 day old female child were born within the span of 1 yr to 2 young and healthy unrelated parents with no history of any past illness. A 3rd child was born 2 yr later. After birth, the 1st born baby appeared well but became ill the following day with marked dyspnea, cyanosis, generalized flaccidity, bradycardia and finally total respiratory arrest. The 2nd child suffered with an almost identical clinical picture and succumbed to its illness in a manner quite similar to that of its sibling brother. Likewise, the 3rd child at the age of 7 mo. became apathic, cyanotic and dyspneic. It died 10 min after arrival in the hospital in a state of unconsciousness. Autopsies of all siblings demonstrated very similar macroscopic and microscopic findings of a peculiar diffuse intense fatty degeneration of the liver and myocardium with focal renal tubular epithelial involvement. Apparently, all 3 siblings suffered with and died of a type of fetal steatosis affecting some of the most important organs of the body.