RESUMEN
INTRODUCTION: ADAMTS13 modulates shear-dependent platelet thrombus formation (PTF) by limited proteolysis of von Willebrand factor (VWF). A high-plasma-ratio of VWF antigen to ADAMTS13 activity (VWF:Ag/ADAMTS13:AC) promotes PTF and aggravates shear-induced inflammation mediated by VWF. A role of ADAMTS13 in Kawasaki disease (KD) remains unknown, however. We investigated the involvement of ADAMTS13-VWF axis in the acute-phase of KD (acute-KD). METHODS: VWF:Ag and ADAMTS13:AC in 77 KD infants were measured at three time-points; immediately before (Pre), one-week (1â¯W) and one-month (1â¯M) after intravenous-immunoglobulin (IVIG) treatment. VWF multimer (VWFM) distribution and ADAMTS13-isoelectrofocusing (IEF) patterns were compared between the responders and non-responders to IVIG. RESULTS: A high VWF:Ag (195.7⯱â¯85.6%, pâ¯<â¯0.05), low ADAMTS13:AC (60.3⯱â¯23.8%, pâ¯<â¯0.05) and high VWF:Ag/ADAMTS13:AC ratio (3.70⯱â¯2.12, pâ¯<â¯0.05) at Pre were seen compared to control plasmas. These parameters returned to normal levels time-dependently after IVIG treatment. Non-responders to IVIG demonstrated high VWF:Ag and low ADAMTS13:AC at Pre, and high VWF:Ag/ADAMTS13:AC ratio at 1â¯W compared to responders, but there were no significant differences in VWFM distribution between both groups. IEF analyses revealed the decreased free form of ADAMTS13 and increased complex form with ADAMTS13 and high-molecular-weight-VWFM at Pre in non-responders. A high VWF:Ag/ADAMTS13:AC ratio was associated with increased white blood cell counts, together with decreased serum albumin and sodium at Pre and 1â¯W. CONCLUSIONS: A high VWF:Ag/ADAMTS13:AC ratio in acute-KD persisted after primary treatment in non-responders, and unbalanced substrate-to-enzyme ratio appeared to associate with vascular endothelial damage. Analysis of existing mode of ADAMTS13 may help to clarify pathogenesis of IVIG resistance in acute-KD.
Asunto(s)
Proteína ADAMTS13/sangre , Inmunoglobulinas Intravenosas/administración & dosificación , Síndrome Mucocutáneo Linfonodular/sangre , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Factor de von Willebrand/metabolismo , Enfermedad Aguda , Aspirina/administración & dosificación , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Inhibidores de Agregación Plaquetaria/administración & dosificación , Transducción de Señal/efectos de los fármacosRESUMEN
SUBJECT: Along with the promotion of "home medical care", an increasing number of clinics provide home medical care as part of their services or are dedicated to home medical care partly as a result of the reform of the healthcare system. This is considered to be the manifestation of the desire of patients to live their own lives and the intention of the healthcare professionals to satisfy patient needs. We examine what insurance pharmacies should and must do to provide higher-quality services to satisfy such patient needs, this time focusing on the community coordination for the purpose of providing the services which can give sense of security to patients. METHOD: Cases of HPN during the period from July 2002 to June 2003 were subdivided into those in which we were involved before and after the discharge from the hospital and those in which we were involved only after the discharge from the hospital to home medical care and comparatively studied from various perspectives. Existing coordination systems are introduced and discussed to achieve the desirable coordination systems. PRESENT AND FUTURE: In the home guidance service, some patients consult with us on the issues on which they hesitate to consult with doctors or nurses. This suggests that we can contribute to the mental care for patients and their care givers. In order to make the best use of our position, the coordination with hospitals is essential for insurance pharmacies. The degree of contribution to the improvement of QOL or the mitigation of anxiety over inexperienced issues is considered to differ depending on the preparatory period between the cases in which we were involved before and after the discharge from the hospital and those in which we were involved only after the discharge from the hospital to home medical care. The analysis of the differences in the actual cases is considered to be significant to seek for the ideal coordination.
Asunto(s)
Servicios de Atención a Domicilio Provisto por Hospital/tendencias , Servicios de Atención de Salud a Domicilio/tendencias , Hospitales/tendencias , Farmacias/tendencias , Anciano , Predicción , Humanos , Nutrición Parenteral en el Domicilio , Alta del PacienteRESUMEN
SUBJECT: Along with the promotion of "home medical care", an increasing number of clinics provide home medical care as part of their services or are dedicated to home medical care partly as a result of the reform of the healthcare system. This is considered to be the manifestation of the desire of patients to live their own lives and the intention of the healthcare professionals to satisfy patient needs. We examine what insurance pharmacies should and must do to provide higher-quality services to satisfy such patient needs, this time focusing on the community coordination for the purpose of providing the services which can give sense of security to patients. METHOD: Cases of HPN during the period from July 2002 to June 2003 were subdivided into those in which we were involved before and after the discharge from the hospital and those in which we were involved only after the discharge from the hospital to home medical care and comparatively studied from various perspectives. Existing coordination systems are introduced and discussed to achieve the desirable coordination systems. PRESENT AND FUTURE: In the home guidance service, some patients consult with us on the issues on which they hesitate to consult with doctors or nurses. This suggests that we can contribute to the mental care for patients and their care givers. In order to make the best use of our position, the coordination with hospitals is essential for insurance pharmacies. The degree of contribution to the improvement of QOL or the mitigation of anxiety over inexperienced issues is considered to differ depending on the preparatory period between the cases in which we were involved before and after the discharge from the hospital and those in which we were involved only after the discharge from the hospital to home medical care. The analysis of the differences in the actual cases is considered to be significant to seek for the ideal coordination.