This report is based upon 2 cases of a disease entity characterised by the presence of excessive amounts of “lipochrome” like pigment in liver cells in patients with the clinical picture of “constitutional hyperbilirubinemia.”
We reported the clinical pictures, histological findings, histochemical views of this granule and lipofuscin and electromicroscopical findings of the liver cell.
The life span of these erythrocytes with tagged radioactive Cr
51 was lost as early as about sixty days in both 2 cases.
In the bone marrow and the kidney, the granules were histologically revealed. And in bile and urine sediment also present.
On the histological examination of these two cases, the liver was normal except for a striking amount of “lipochrome” like pigment in parenchymal cells. This pigment is histochemically positive with PAS, Schmorl, Alkaline Silver, PFAS, and Methylenblau and negative with Gmelin, so that, this resembles the group of lipofuscin.
On electromicloscopic examination, the arrangement of the granules is corresponded with mitochondria and the small granules in the mitohondria are thought to be corresondedp with the cristae mitochondriales.
These granules thought to be degenerative mitochondria are generally large, tense and some granules are coagulating each other contrary to the electromicroscopic findings of lipofuscin.
Dubin-Johnson's syndrome is a disease entity; although its granules histochemically resemble with lipofuscin, the formation of the granules microscopicary and electromicroscopically not resemples with lipofuscin.
Further, we discussed about the etiology of this disease and jaundice.
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