title: Upshaw-Schulman Syndrome.
abstract: A13 years girl presented with history of sudden onset fits, altered sensorium and anuria for 2 days. There was also history of right sided weakness of the whole body. Past history revealed repeated episodes of similar complaints since early childhood. On the basis of history, physical examination and extensive investigations, patient was diagnosed as Upshaw-Schulman syndrome, a rare case of congenital Thrombotic Thrombocytopenic Purpura (TTP). She is now in remission and being maintained on twice weekly transfusions of Fresh Frozen Plasma (FFP).
authors.affiliation: Department of Pathology, Allama Iqbal Medical College, Lahore.
authors.forename: Rabia
authors.initials: R
authors.lastname: Ahmad
authors.affiliation: Department of Pathology, Allama Iqbal Medical College, Lahore.
authors.forename: Muneeza
authors.initials: M
authors.lastname: Natiq
authors.affiliation: Department of Haematology, Sheikh Zayed Hospital/FPGMI, Lahore.
authors.forename: Mona
authors.initials: M
authors.lastname: Aziz
mesh_terms: D000293:Adolescent; D005260:Female; D006801:Humans; D010949:Plasma; D010956:Plasmapheresis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D016896:Treatment Outcome