NEW CONGNITION ON FOCAL SEGMENTAL
GLOMERULOSCLEROSIS (FSGS) PROGRESSION OF PATHOGENESIS, REPORT TWO AUTOPSY
CASES
Pang
Ning1, Zhou Xiang1, Ding Jinghong1, Wang
Tailin2, Zhang Jing2
1 Department of Pediatrics
2 Department of Pathology, China-Japan Friendship Hospital,
Beijing, China
Objective: Probe into
new congnition on FSGS
Methods: Review the
autopsy data of two children FSGS cases. According to the pathologic
characteristics go further into the pathogenesis, causes of death &
treatment.
Results: (1) The
lesion of FSGS are mainly located at juxta medullar glomeruli; account for
approximately 20-30% glomeruli. The size of the FSGS glomeruli are bigger
than the remainder which had no light microscopic changes; except for the
glomerular tuft epithelial cells are swelling (that shows increased
permeability). (2) The above renal pathologic changes sustained the recent
observation that the circulating permeability factor (CPF)etc.implicated in
the pathogenesis of FSGS. (3) In minimal change nephrotic syndrome (MCNS)
the initial bigger size glomeruli is a highly sensitive predictor to
develop to FSGS. (4) Clinical manifestations of the cases were: heavy
proteinuria , edema. no severe renal function deteriorated. Causes of death
in case 1 were cardiac, cerebral & laryngeal severe edema. Case 2 were
complicated with mycotic pneumonia,electrolytic disturbance. (5) Undergone
the 6 months’ course in case 2 the aorta showed lipid spots & streaks.
(6) FSGS may sensitive to steroid or combined immunosuppresive agents, But
considerably of FSGS do not respond to these therapy as our two patients.
Conclusion: According
to the clinical & pathologic data of these two cases,sustained that the
CPF etc implicated in the pathogenesis of FSGS. It is necessary to attach
importance for study the CPF and focus treatment on it, as performed
plasmapheresis, immunabsorbtion. Prophylaxis, treatment for complications
are important. Study on the pathogenesis& treatment of FSGS would probe
deeply in the future.