Annals of Diagnostic Paediatric Pathology

91
Fig. 2 Liver biopsy of patient no 2 shows severe diffuse macrovesicular steatosis.
Liver biopsy of patient no 3 shows diffuse, moderate, mixed steatosis.
Mild intralobular inflamatory infiltrates of lymphoid cells and single necrotic
hepatocytes are observed.
Case No 3
The liver tissue obtained during biopsy showed fatty changes
in approximately 60 % of hepatocytes. They revealed diffuse
distribution. Lipid droplets in hepatocytes were of mixed pattern:
macrovacuoles and microvacuoles were present. The mild
intralobular and portal inflamatory infiltrates of lymphoid cells
were found. Single necrotic hepatocytes were observed. Fibrosis
and cholestasis were not detected (Fig. 2).
Case No 4
The liver biopsy specimen obtained prior to autopsy revealed
precirrhotic pattern of liver injury. Macrovesicular steatotis were
seen in 30 % of hepatocytes. Their localization was rather focal,
within some of the nodules. Severe portal inflammatory
infiltrates were present. Cholestasis was not found (Fig. 3).
Case No 5 (the same patient as above)
Subsequent liver specimen obtained during autopsy revealed
cirrhosis. Severe macrovesicular fatty changes concerned all
hepatocytes. Mild portal inflammatory infiltrates remained
similar to those of the biopsy specimen. No cholestasis was
present (Fig. 3).
Fig. 3 Precihrrotic pattern of moderate, focal, macrovesicular steatosis of
patient 4. Subsequent, post mortem examination of the same patient reveals
severe, macrovesicular fatty changes.
Case No 6
The liver biopsy differed substantially from other patients.
Diffuse, mixed steatotic changes were seen only in about 5-
10% of hepatocytes. No inflammatory infiltrates, fibrosis or
cholestasis were detected in the liver.
Case No 7
The girl was referred to autopsy examination with diagnosis
of cardiac insufficiency due to hypertrophied cardiomyopathy.
Appropriate diagnosis was established retrospectively,
some years after death on the basis on genetic family study. It
was performed after her younger brother was diagnosed to
have LCHAD deficiency. On autopsy examination features of
excentric hypertrophy of left cardiac ventricle with accompanying
signs of mitral insufficiency and endocardial thickening
was observed. Steatosis of the liver was also reported in
autopsy protocol, but no morphological characteristic was
made. Microscopically, macrovesicular steatosis of diffuse localization
consisted of 10 % of hepatocytes accompanied by
mild fibrosis was found. No inflammation and cholestasis was
observed.