Severe Fat Accumulation in Multiple Organs in Pediatric Autopsies

Author:

Bleeker Jeannette C12,Visser Gepke12,Wijburg Frits A2,Ferdinandusse Sacha3,Waterham Hans R3,Nikkels Peter G J4

Affiliation:

1. Department of Metabolic Diseases, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, The Netherlands

2. Department of Metabolic Diseases, Emma Children’s Hospital, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

3. Laboratory Genetic Metabolic Diseases, Departments of Clinical Chemistry and Pediatrics, Emma Children’s Hospital, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

4. Department of Pathology, University Medical Center Utrecht, Utrecht, The Netherlands

Abstract

Background The observation of fat accumulation in different organs at pediatric autopsy may help determine the cause of death. However, a comprehensive study on fat accumulation and related etiologies is still lacking. Aim To investigate the incidence of fat accumulation in different organs in pediatric autopsies and their relevance to the cause of death. Methods From February 2004 until March 2015, fat accumulation was assessed in 561 routinely performed pediatric autopsies in our center. Age at death ranged from 14 weeks of gestation for fetuses to 16 years. Samples of liver, heart, kidney, and muscle were stained with Oil-Red-O and scored for fat accumulation by one pediatric pathologist. Results Fat accumulation in ≥1 organ(s) was present in 132 (39.8%) of 332 prenatal cases. Only 3 (0.9%) had fat accumulation in all 4 organs. For postnatal cases, fat accumulation in ≥1 organ(s) was present in 106 (46.3%) of 229 postnatal cases. Only 12 (5.2%) had fat accumulation in all 4 organs. Fat accumulation was mostly seen in liver, both in prenatal and postnatal cases. Fatty acid oxidation disorders were the only cause of death associated with severe fat accumulation in all 4 organs. No other etiologies could be linked directly to distribution or severity of fat accumulation. Conclusion Severe fat accumulation in at least 4 organs is a rare finding in pediatric autopsies, whereas fat accumulation in only the liver is relatively common. Severe fat accumulation in both liver, muscle, kidney, and heart was only seen in cases with fatty acid oxidation disorders.

Publisher

SAGE Publications

Subject

General Medicine,Pathology and Forensic Medicine,Pediatrics, Perinatology and Child Health

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