Fatal familial steatosis of myocardium, liver and kidneys in three siblings.
Publication/Presentation Date
3-1-1981
Abstract
A 2-day-old male and 3-day-old female child were born within the span of one year to two young and healthy unrelated parents with no history of any past illness. A third child was born two years later. After birth, the first born baby appeared well but became ill the following day with marked dyspnea, cyanosis, generalized flaccidity, bradycardia and finally total respiratory arrest. The second 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 third child at the age of seven months became apathic, cyanotic and dyspneic. It died ten minutes 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 three siblings suffered with and died of a type of fatal steatosis affecting some of the most important organs of the body.
Volume
70
Issue
2
First Page
247
Last Page
252
ISSN
0001-656X
Published In/Presented At
Suprun, H., & Freundlich, E. (1981). Fatal familial steatosis of myocardium, liver and kidneys in three siblings. Acta paediatrica Scandinavica, 70(2), 247–252. https://doi.org/10.1111/j.1651-2227.1981.tb05549.x
Disciplines
Medicine and Health Sciences
PubMedID
7234407
Department(s)
Department of Pathology and Laboratory Medicine
Document Type
Article