[Design and implementation of a transdisciplinary care model for patients with hereditary angioedema, in a Colombian health institution]. / Diseño e implementación de un modelo de atención transdisciplinaria para pacientes con angioedema hereditario, en una institución de salud colombiana.

OBJECTIVE: Describe the design and implementation of a transdisciplinary care model for patients with hereditary angioedema in Colombia. METHODS: Descriptive longitudinal observational study. 140 patients with hereditary angioedema were included in a transdisciplinary care model for one year. Seizure rates, hospitalizations, emergency room visits, quality of life, and pharmacological adherence were measured. RESULTS: The model was associated with reductions of 76% in seizures, 66% in hospitalizations, and 87% in emergency room visits. Pharmacological adherence increased 19% and was complete after four months. The quality of life increased significantly. CONCLUSIONS: Hereditary angioedema is an orphan disease that requires a comprehensive approach for effective care.

OBJETIVO: Describir el diseño e implementación de un modelo transdisciplinario de atención para pacientes con angioedema hereditario en Colombia. MÉTODOS: Estudio observacional longitudinal descriptivo. 140 pacientes con angioedema hereditario fueron incluidos en un modelo de atención transdisciplinario por un año. Se midieron tasas de crisis, hospitalizaciones, visitas a urgencias, calidad de vida y adherencia farmacológica. RESULTADOS: El modelo se asoció con reducciones del 76% en crisis, 66% en hospitalizaciones y 87% en visitas a urgencias. La adherencia farmacológica aumentó 19% y fue completa después de cuatro meses. La calidad de vida aumentó significativamente. CONCLUSIÓN: El angioedema hereditario es una enfermedad solitaria que requiere un abordaje integral para la atención eficaz.

The frequency of complications in a cohort of patients diagnosed with hemophilia A and hemophilia B receiving prophylactic treatment in Colombia: A retrospective noninterventional study.

INTRODUCTION: Hemophilia A and B are disorders associated with the deficit of coagulation factors VIII and IX. OBJECTIVE: Was to determine the incidence of complications in a cohort of patients diagnosed with moderate and severe hemophilia A or B under treatment in a specialized institution. METHODS: A retrospective study of a cohort of patients with replacement therapy for hemophilia A or B, evaluating treatment and complications between January/2012 and July/2019. Sociodemographic, clinical and disease management-related variables were extracted from the medical records. Time to inhibitor development and rate associated with bleeding and hospitalizations were evaluated. RESULTS: A total of 159 male patients were identified with hemophilia A (n = 140; 88.1%) and B (n = 19; 11.9%) with a mean follow-up of 5.9±2.3 years. The mean age was 23.6±16.1 years, hemophilia was reported as severe in 125 patients in hemophilia A (89.3%) and 13 patients in hemophilia B (68.4%). Primary prophylaxis was registered in 17.0% of patients, 44.7% secondary, and 38.3% tertiary, with recombinant factors (n = 84; 52.8%) followed by plasma derived factors (n = 75; 47.2%). The incidence of inhibitor development was 0.3 per 100 patients/year, with mean time to event of 509 days. The incidence of bleeding was 192 per 100 patients/year, especially at the joint (n = 99; 62.3%) and muscle (n = 25; 15.7%) level. The incidence of hospitalization was 3.7 per 100 patients/year. CONCLUSIONS: The most common complication was joint bleeding which was expected in this type of patients. Low proportion of patients developed factor inhibitors during the follow up.

Factors Involved in the Development of Inhibitory Antibodies in Patients with Hemophilia in Colombia: A Case-Control Study.

BACKGROUND: The appearance of inhibitory antibodies against antihemophilic factors is one of the most serious complications related to hemophilia. OBJECTIVE: The objective of this study was to identify variables and factors related to the development of inhibitory antibodies in a group of patients undergoing antihemophilic therapy in Colombia. METHODS: A case-control study in patients with hemophilia treated in Specialized Healthcare Provider Institutions (IPS-E) in 21 cities of Colombia of any age and with a diagnosis of inhibitory antibodies against factor VIII or IX during 2016. Four controls per case paired by age and type of hemophilia were used. Sociodemographic, clinical, and pharmacological variables were identified and analyzed. RESULTS: Seventeen patients with inhibitory antibodies and 68 controls with hemophilia were identified. The mean age was 28.3 ± 17.8 years. A total of 94.1% had hemophilia A, and 88.2% of the cases and 50.0% of the controls had severe hemophilia; 47.1% of the cases and 54.4% of the controls were receiving prophylaxis with coagulation factors. Multivariate analysis showed that having severe hemophilia (OR:17.0, 95%CI:1.32-219.60) and lack of knowledge of the coagulation factor with which the patient was treated before entering the care program in the IPS-E (OR:8.9, 95%CI:1.82-43.75) were significantly associated with a higher probability of developing inhibitory antibodies. CONCLUSION AND RELEVANCE: Coagulation factors associated with the development of inhibitory antibodies were severe hemophilia and lack of knowledge of the type of factor used prior to entering the follow-up cohort.