No abstract

Settings Hemophilia is a coagulation disorder that occurs in one in 5000 male births. Patients with untreated severe hemophilia A have hemorrhagic complications, including joint bleeds and decreased survival. Emicizumab is a monoclonal antibody approved by the United States for routine prophylaxis of pediatric and adult patients with severe hemophilia A with factor VIII inhibitors. Objectives To perform a cost-effectiveness study of emicizumab prophylaxis for children and adults with severe hemophilia A compared with the current disease management in the Peruvian Ministry of Health and Social Security Health Insurance. Methods The patient transition between medical states was modeled with Markov methodology, and the lifetime costs and incremental effects of emicizumab compared to current management were estimated. The budgetary impact of emicizumab was estimated by projecting annual net costs and its five-year present value. Results In the Ministry of Health, emicizumab would generate savings between 14.6 and 16.0 per child and 11.8 per adult, in current US$ million. Social Security Health Insurance savings would be 12.8 to 14.9 per child and 40.1 per adult. In addition, this strategy would generate effectiveness gains, measured in quality-adjusted life-years, of 0.36 per child and 0.56 per adult and 0.25 per child, and 0.36 per adult in those respective institutions. The budgetary impact would be a net annual saving of 12.8 and 15.0 US$ million in those entities. Conclusions The current management of hemophilia A is very costly and has health outcomes inferior to those possible with emicizumab. This drug would produce significant savings and better patient health. The Ministry of Health and Social Health Insurance should implement hemophilia prophylaxis and treatment protocols and finance this drug.

Las manifestaciones musculoesqueléticas son comunes en las hemoglobinopatías. Se describen dos pacientes con anemia de células falciformes homocigota (HbSS) que desarrollan compromiso osteoarticular asociado a crisis hemolítica. Una mujer mestiza de 22 años de edad con necrosis de la cabeza femoral derecha y un varón negro de 21 años con derrame articular de ambas rodillas.

Presentamos el estudio de 58 pacientes con el diagnóstico de brucellosis, internados en el Hospital Nacional «Dos de Mayo» entre,Junio de 1994 y Junio 1996. Cuarenta pacientes fueron varones (69%) y 18 mujeres (31 %), la edad promedio fue de 33,2 años (14-81 años) y 41 pacientes fueron menores de 40 años (70,7%). Los síntomas más frecuentemente encontrados fueron fiebre (56 pacientes, 96,6%), compromiso articular en 35 pacientes (60,3%), esplenomegalia en 32 (55,2%), linfadenopatía en 30 (51,7%), hepatomegalia en 28 (48,3%), cefalea en 24 (41,4%), palidez en 20 (34,5) y complicaciones hemorrágicas en 11 (19%). Hubo compromiso hepático en 5 pacientes. Los cambios hematológicos encontrados fueron anemia en 43 pacientes (74,1 %), linfopenia en 13 (22,4 %), trombocitopenia en 9 (15,5%), leucopenia en 6 (10,3%) y pancitopenia en 2 pacientes (3,5). La médula ósea en 36 pacientes evaluados fue normocelular o hiperplásica, con incremento en el número de histiocitos y leve a moderada citofagocitosis.