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SITRDMF syndrome

Situs Inversus Totalis, Renal Dysplasia and Multisystem Fibrosis, situs inversus totalis with reno-pancreatic dysplasia, Pinar syndrome

 

Synopsis

-  renal dysplasia
-  situs inversus totalis
-  multisystem fibrosis
-  hydrops fetalis
-  uretreal duplication

-  ureteral fibrosis
-  pancreatic fibrosis
-  thyroid fibrosis
-  uterine fibrosis
-  pericardial fibrosis (extensive fibrous pericarditis)

-  small lungs with large irregular pulmonary lobules bounded by fibrotic septa

References

-  Pinar H, Rogers BB. Renal dysplasia, situs inversus totalis, and multisystem fibrosis: a new syndrome. Pediatr Pathol. 1992 Mar-Apr;12(2):215-21. PMID: #1570238#


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