Trasplante hepático en hemofilia A y enfermedad de von Willebrand tipo 3. Manejo perioperatorio y evolución postrasplante / Liver transplantation in hemophilia A and von Willebrand disease type 3: perioperative management and post-transplant outcome

Introducción: la infección por virus hepatitis C (VHC) ha sido causa de importante morbi-mortalidad en hemofilia, planteándose el trasplante hepático (TH) por cirrosis y/o carcinoma hepatocelular (CHC). Caso clínico: presentamos los casos con coagulopatías congénitas e infección por VHC sometidos a TH en nuestro centro: tres pacientes con hemofilia A y uno con enfermedad de von Willebrand (EvW) tipo 3. Evaluamos el curso de la coagulopatía, el manejo perioperatorio, el consumo de factor y componentes sanguíneos y la supervivencia postrasplante. El factor deficitario se comenzó a administrar en bolo iv directo una hora antes del inicio de la cirugía para alcanzar un nivel deseado de dicho factor de 100 UI/dl, mantenido hasta conseguir el control estable de la hemostasia. Los tres pacientes con hemofilia A curaron su coagulopatía postrasplante. El factor VIII (FVIII) fue 93 UI/dl a los once años, 59 UI/dl a los 13 meses y 109 UI/dl a los nueve meses postrasplante en cada uno de los casos. El consumo medio perioperatorio de concentrados de FVIII fue 175 UI/kg, infundido hasta 36 h postrasplante de media. El paciente con EvW tipo 3 consiguió atenuar el curso natural de su sintomatología hemorrágica sin que se detectaran niveles hemostáticos del antígeno del factor von Willebrand (FVW:Ag) postrasplante. Discusión: tras el trasplante hepático, se produce la curación de la hemofilia A y la mejoría del fenotipo hemorrágico en la EvW tipo 3

Introduction: infection with the hepatitis C virus (HCV) causes significant morbidity and mortality in patients with hemophilia. Finally, patients are considered for a liver transplantation (LT) due to cirrhosis and/or hepatocellular carcinoma (HCC). Case report: we report the cases of congenital coagulopathy and HCV infection that underwent LT in our institution. There were three patients with hemophilia A and one patient with von Willebrand disease (vWD) type 3. The coagulopathy outcome, perioperative management, factor and blood product usage and post-transplant survival were assessed. The deficient factor was initially administered in a direct bolus one hour before surgery with a target level of 100 IU/dl, which was sustained until stable hemostasis was reached. All three patients with hemophilia A were cured of their coagulopathy following transplantation. Factor VIII (FVIII) was 93 IU/dl at eleven years, 59 IU/dl at 13 months and 109 IU/dl at nine months post-transplant, in each case. The mean perioperative usage of FVIII concentrates was 175 IU/kg; concentrates were infused for an average of 36 hours post-transplant. The natural course of the bleeding symptoms of the patient with type-3 vWD was attenuated, with no detectable hemostatic levels of von Willebrand factor antigen (vWF:Ag) after transplantation. Discussion: after transplantation, hemophilia A cure and improved bleeding phenotype of type-3 vWD reduced morbidity and mortality. However, potential graft reinfection with HCV and relapsing HCC cast a shadow over these optimum results

Liver transplantation in hemophilia A and von Willebrand disease type 3: perioperative management and post-transplant outcome.

INTRODUCTION: infection with the hepatitis C virus (HCV) causes significant morbidity and mortality in patients with hemophilia. Finally, patients are considered for a liver transplantation (LT) due to cirrhosis and/or hepatocellular carcinoma (HCC). CASE REPORT: we report the cases of congenital coagulopathy and HCV infection that underwent LT in our institution. There were three patients with hemophilia A and one patient with von Willebrand disease (vWD) type 3. The coagulopathy outcome, perioperative management, factor and blood product usage and post-transplant survival were assessed. The deficient factor was initially administered in a direct bolus one hour before surgery with a target level of 100 IU/dl, which was sustained until stable hemostasis was reached. All three patients with hemophilia A were cured of their coagulopathy following transplantation. Factor VIII (FVIII) was 93 IU/dl at eleven years, 59 IU/dl at 13 months and 109 IU/dl at nine months post-transplant, in each case. The mean perioperative usage of FVIII concentrates was 175 IU/kg; concentrates were infused for an average of 36 hours post-transplant. The natural course of the bleeding symptoms of the patient with type-3 vWD was attenuated, with no detectable hemostatic levels of von Willebrand factor antigen (vWF:Ag) after transplantation. DISCUSSION: after transplantation, hemophilia A cure and improved bleeding phenotype of type-3 vWD reduced morbidity and mortality. However, potential graft reinfection with HCV and relapsing HCC cast a shadow over these optimum results.

A Clinical Study Assessing the Influence of Anodized Titanium and Zirconium Dioxide Abutments and Peri-implant Soft Tissue Thickness on the Optical Outcome of Implant-Supported Lithium Disilicate Single Crowns.

PURPOSE: To assess the influence of anodized titanium and zirconium dioxide abutments and peri-implant soft tissue thickness on the optical outcome of implant-supported lithium disilicate single crowns. MATERIALS AND METHODS: Twenty patients with a missing maxillary single incisor, canine, or first premolar received an endosseous implant after a two-stage surgery protocol. After healing and soft tissue conditioning, peri-implant soft tissues were reproduced in the impression, and the thickness was measured. Customized abutments were made of titanium, gold-anodized titanium, pink-anodized titanium, and zirconium dioxide. The definitive prosthesis was a lithium disilicate crown stratified by feldsphatic porcelain. Customized abutments were screwed (35 Ncm), and the crown was temporarily placed on the abutment with a try-in paste. Color measurements were made using a spectrophotometer. CIELab color scale was employed following the formula: ΔE = (ΔL)² + (Δa) ² + (Δb) ². Data were analyzed using repeated-measures analysis of variance (ANOVA), Bonferroni and Pearson's correlation tests (α = .05). RESULTS: Abutment material type significantly affected the ΔE values at both the peri-implant soft tissue (P = .0001) and coronal level (P = .001). The lowest ΔE values were obtained with zirconia abutments at both soft tissue (6.06 ± 3.2) and coronal level (5.76 ± 2.9) compared with those of other abutments (soft tissue: 8.96 ± 3.1 to 11.56 ± 3.4; coronal: 8.66 ± 6.1 to 10.42 ± 6.3). Mean soft tissue thickness (1.63 ± 0.64 mm) affected the ΔE values at the peri-implant soft tissue level for only titanium and pink-anodized titanium abutments (P = .024 and P = .048, respectively). In all conditions, correlation coefficients between ΔE and the abutment materials were higher for titanium (r = -0.544; P = .024) and the least for zirconia (r = -0.313; P = .238) and gold-anodized titanium (r = -0.393; P = .119) abutments. CONCLUSION: All abutment types demonstrated noticeable color difference at both the soft tissue and coronal levels. Zirconia abutments showed the lowest ΔE values at both measurement zones. Soft tissue thickness did not affect the ΔE values at the peri-implant soft tissue level.

Valoracion densitometrica de los maxilares en pacientes desdentados mediante absorciometria dual de doble energia de rayos x (dexa) / Densitometric analysis of the jawbones of toothless patients using dual energy X-ray absorptiometry (DXA)

Objetivo: El propósito de este estudio es analizarla densidad mineral ósea de los maxilares des dentados in vivo mediante el uso de la absorciometría dual de doble energía de rayos X(DEXA). Con ello se quiere valorar las diferencias entre las distintas zonas de los maxilares, así como las diferencias según edad y sexo, elaborando un mapa de densidades. Material y método: El estudio se realizo en 40pacientes (22 mujeres, 18 varones) con zonas edentulas de mas de 9 meses de evolución realizándoseles una valoración densito métrica en el área desdentada mediante DEXA. El estudio estadístico comparativo de tablas de frecuencias de valores cualitativos se realizo mediante la prueba de Chi-cuadrado con la corrección de Yates. La de relaciones entre dos grupos mediante la Tde Student; se utilizo el Análisis de la Varianza para estudiar relaciones de uno o dos factores y el test de comparaciones múltiples asociado al de Duncan. Resultados: Entre los resultados destacan una media de densidad general de 0,914 gr/cm2(¦Ò = 0,195) y unas diferencias estadísticamente significativas entre las zonas superior inferior(p<0,01), anterior-posterior (p<0,05), anteroinferior-posteroinferior (p<0,01), anteroinferior-anterosuperior (p<0,01), inferiorderecho-superior derecho (p<0,01), inferiorizquierdo-superior izquierdo (p<0,05). No aparecieron diferencias significativas en cuanto al sexo ni a la edad. Conclusiones: Se ha realizado un mapa preliminar de densidades según aéreas de los maxilares y se comprueba que el DEXA es un método valido para la medida de densidad mineral ósea in vivo de los maxilares, aunque requiere adaptaciones en el dispositivo y el método para su utilización practica (AU)

Objective: The aim of this study is to analyse in vivo the bone mineral density of the toothless jawbones using dual energy X-ray absorptiometry (DXA). This method is used to attempt to assess the differences between the different parts of the jawbone, as well as differences between different ages and sexes, generating a density map. Materials & methods: The study group was made up of 40 patients (22 women, 18 men) who had had several teeth missing for over 9 months. Adensitometric measurement of the toothless area was carried out using DXA. The comparative statistical study of qualitative value frequency tables was carried out using the Chi-square Test with (..) ( AU)

Study of available bone for interforaminal implant treatment using cone-beam computed tomography.

OBJECTIVES: To analyze the availability of bone in the interforaminal region and to demonstrate the variation in diagnosis between panoramic x-ray and cone-beam computed tomography (CBCT). MATERIAL AND METHODS: In 50 patients and in 5 areas of each, a series of parameters concerning available bone were measured on both the panoramic and the CT images. RESULTS: Panoramic images underestimate the vertical and horizontal measurements when compared with CBCT. Regarding implant treatment, the interforaminal region presents the same remaining bone height throughout its full extent, the correlation between one side of the midline and the other was highly significant for all the parameters studied, and 20% of the sample showed some buccal and/or lingual concavity. CONCLUSION: Cone-beam CT is an advantageous system for interforaminal implant treatment planning, especially since the reported radiation dose is minimal and geometric accuracy is very high.

Study of available bone for interforaminal implant treatment using cone-beam computed tomography

Objectives: To analyze the availability of bone in the interforaminal region and to demonstrate the variation in diagnosis between panoramic x-ray and cone-beam computed tomography (CBCT). Material and methods: In 50 patients and in 5 areas of each, a series of parameters concerning available bone were measured on both the panoramic and the CT images. Results: Panoramic images underestimate the vertical and horizontal measurements when compared with CBCT. Regarding implant treatment, the interforaminal region presents the same remaining bone height throughout its full extent, the correlation between one side of the midline and the other was highly significant for all the parameters studied, and 20% of the sample showed some buccal and/or lingual concavity. Conclusion: Cone-beam CT is an advantageous system for interforaminal implant treatment planning, especially since the reported radiation dose is minimal and geometric accuracy is very high (AU)

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