Telangiectasia hemorrágica hereditaria Síndrome de Osler Weber Rendú y manejo con bevacizumab / Hereditary hemorrhagic telangiectasia Osler Weber Rendu syndrome and management with bevacizumab

Presentamos un caso clínico de una paciente con diagnóstico de telangiectasia hemorrágica hereditaria con múltiples manifestaciones sistémicas debidas a sangrados profusos, anemia severa y malformaciones arteriovenosas pulmonares, hepáticas y falla cardiaca de alto débito, con adecuada respuesta al uso de bevacizumab. (Acta Med Colomb 2015; 40: 66-68).

The case of a patient diagnosed with hereditary hemorrhagic telangiectasia with multiple systemic manifestations due to profuse bleeding, severe anemia and pulmonary arteriovenous malformations, liver and heart failure high debit adequate response to the use of bevacizumab, is presented. (Acta Med Colomb 2015; 40: 66-68).

Association of Blood Group O with Gastrointestinal Bleeding in Continuous-Flow LVAD Patients.

Background: Increasing use of mechanical circulatory devices for advanced heart failure therapy has led to challenges in managing complications that arise from device implantation for long term support either as a bridge to transplant or as destination therapy. Patients with continuous flow left ventricular assist devices (CF-LVADS) show increasing incidence of gastrointestinal (GI) bleeds. Lack of pulsatality and narrow pulse pressure result in initiation and progression of arteriovenous malformations. High shear forces generated lead to destruction of von Willebrand factor (vWF) making it functionally inactive predisposing patients to increasing bleeding. Literature suggests that patients with blood type O have lower baseline levels of von Willebrand factor. It therefore seemed relevant to study the role of blood groups in this population. Methodology: Retrospective data analysis of 119 CF-LVADS patients discharged between 01/2005 and 07/2014. This study was approved by the Institutional Review Board on 08/01/2014. Results: Blood type (p=0.23), gender (p=0.16) and the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) profile (p=0.86) were not associated with significant incidence of GI bleeding. Blood group O was not associated with a higher incidence of GI bleeding (correlation coefficient =0.07) Patients supported on the HeartMate II (HMII) LVAD experienced more GI bleeding than patients supported on the HeartWare LVAD (HVAD) (p=0.009). HMII LVAD patients who experienced GI bleeding had a mean age of 59.8 years vs. 55.7 years for HMII LVAD patients in the Non-GI bleed group. Age was a significant factor for GI bleeding (p=0.016). Conclusion: Blood group and GI bleeding did not show any significant association in our study. Patients with blood group O did not show increased incidence of GI bleeding despite reported lower baseline levels of von Willebrand factor suggesting contribution of additional factors leading to this complication. The HVAD had a decreased incidence of GI bleeding as compared to Heartmate II. This study is limited by the fact that it was a retrospective analysis in a small population.

Malformaciones arteriovenosas. Revisión y análisis estadístico de 52 MAVs tratadas durante el período de 2000-2010. Tercera parte / Arteriovenous malformations. Review and statistical analysis of 52 MAVs treated during the period 2000-2010. Third part

INTRODUCCIÓN: describir nuestra experiencia en el tratamiento de las MAVs analizando: procedimientos endovasculares utilizados, resultados postoperatorios y complicaciones asociadas, estadificación según escalas de Barthel y Rankin modificadas. MATERIAL Y MÉTODOS: la población de estudio se constituyó por 52 pacientes con MAVs la cual fue analizada y estudiada mediante examen neurológico pre y postoperatorio, TC cerebral, RM cerebral y arteriografía de 4 vasos de cuello, la totalidad fue tratada mediante cirugía convencional en nuestro Servicio durante el período comprendido entre los años 2000 a 2010. RESULTADOS: recibieron tratamiento endovascular previo a la cirugía 16 MAVs (30,76 %). Todas fueron operadas. Doce pacientes mejoraron en el postoperatorio (23,07 %), 30 pacientes (57,69%) no sufrieron modificaciones y 10 de ellos (19,23%) empeoraron durante el postoperatorio. La mortalidad fue de 7 casos (13,46 %). CONCLUSIÓN: consideramos a los procedimientos endovasculares y la radiocirugía una herramienta de indudable valor terapéutico. Creemos que el subgrupo de MAVs grados III, IV y V representa una entidad singular que las distingue del resto, como una subtipo que requiere más aun de una compleja toma de decisiones. Tuvimos las mayores complicaciones postoperatorias en MAVs grados III y IV. Nuestra mortalidad postoperatoria coincide con la bibliografía consultada

INTRODUCTION: to describe our experience in treating AVMs based on the endovascular procedures used, postoperative results and associated complications, staging according to Barthel Index and modified Rankin Scale. PATIENTS AND METHOD: we present 52 patients with AVMs which were analysed and studied by Pre and Post-Surgery Neurological exam, brain CT, Brain IRM and four Neck vessels arteriography. All the patients were treated by conventional Surgery at our Department of Neurosurgery for the 2000­2010 period. RESULTS: Sixteen patients with AVMs (30.76%) underwent endovascular treatment prior to surgery. 100% were operated. 12 patients (23.07%) improved their clinical condition in the postoperative period, 30 patients (57.69%) showed no changes, and 10 of them (19.23%) experienced deterioration during the postoperative period. There were 7 mortal cases (13.46%). CONCLUSION: we believe that endovascular procedures and radiosurgery are tools of immense therapeutic value. We also consider that the subgroup of AVMs grade III, IV and V have unique features that distinguish them among the rest as a subtype, thus requiring extreme care when making decisions. Most postoperative complications occurred with AVMs grade III and IV. The mortal cases in the postoperative period coincided with those mentioned in the bibliography consulted