небольшая поправка, в отрезанном фрагменте из биопсии ребенка внизу имеется больше данных за 3-ий тип, a не первый - тогда и фиброз понятен, выделю аналогию на английском:
By contrast, in most patients with GSD III, the liver biopsy demonstrates a vacuolar accumulation of non–membrane bound glycogen primarily located in the cytoplasm. Lipid vacuoles are far less numerous in GSD III than in GSD I. The presence of fibrosis, ranging from minimal periportal fibrosis to micronodular cirrhosis, occurs in GSD III, but not in GSD I. Periportal fibrosis is a very early finding in GSD III. In GSD III, the stored material is within the cytoplasm, periodic acid schiff positive, and diastase sensitive.
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