ABSTRACT
The indicators of quality for impartial quantitative assessment of postmortem examination have been suggested. These indicators were based on ratio of achievement and target indexes of pathologicoanatomic activity. The indicators are necessary for decision making for enhancement of pathologicoanatomic service quality in the health-care agencies.
Subject(s)
Needs Assessment/standards , Pathology, Clinical/standards , Standard of Care/standards , Humans , Needs Assessment/legislation & jurisprudence , Needs Assessment/organization & administration , Pathology, Clinical/legislation & jurisprudence , Pathology, Clinical/organization & administration , Standard of Care/legislation & jurisprudence , Standard of Care/organization & administrationABSTRACT
Clinical and morphologic manifestations of a rare disease developing, as a rule, in early life--an infantile variant of Niemann-Pick disease--are illustrated with a fatal case of a 6.5-month-old child. The diagnosis was established histochemically using, in particular, Smith-Dietrich method which provided differential diagnosis with cerebroside lipidosis (Gaucher's disease). In addition to typical enlargement of the liver, spleen and lymph nodes, cerebral and adrenal affection there appeared true porencephalia.
Subject(s)
Brain/pathology , Niemann-Pick Diseases/pathology , Diagnosis, Differential , Female , Gaucher Disease/complications , Gaucher Disease/pathology , Humans , Infant , Microcephaly/complications , Microcephaly/pathology , Niemann-Pick Diseases/complicationsABSTRACT
A case of nephropathic cystinosis (Abderhalden-Lignac-Kaufmann disease) in a 8-month girl is reported. Features of pathomorphologic changes in the viscera are described, in kidneys, in particular. The changes described occur not only in the renal tubular system, but also in the glomeruli with the development of sclerosis in them, are more common in older children and may be considered as an intermediate form of cystinosis.
Subject(s)
Cystinosis/pathology , Cystinosis/diagnosis , Female , Humans , Infant , Kidney/pathology , Viscera/pathologyABSTRACT
Krabbe's globoid leukodystrophy was observed in a girl of 6 months. The pathological diagnosis of the disease is based on the oligodendroglia cell damage, nerve fibre demyelinization and presence of giant multi-nuclear globoid cells.
