Coagulation Profile in Neonates with Congenital Heart Disease: A Pilot Study.

Background and Objectives: congenital heart disease (CHD), cyanotic and, to a lesser degree, acyanotic, often are accompanied by coagulation abnormalities, impacting substantially morbidity and mortality. Until now, no consistent hemostatic patterns have been demonstrated in neonates and children with CHD because they represent a variable and heterogenous population. The aim of the present study is to investigate the hemostatic profile, as well as the role of ADAMTS-13 (a disintegrin and metalloprotease with thrombospondin type-1 motives), the cleaving protein of von Willebrand factor (VWF) in neonates with CHD and compare them to healthy age-matched controls. Materials and Methods: twenty neonates with a mean gestational age of 37.1 ± 2.5 weeks were included in the CHD group, and 18 healthy neonates with a mean gestational age of 38.2 ± 1.5 weeks were in the control group. Results: prothrombin time was significantly prolonged, and accordingly, factor VII (FVII) levels were significantly decreased in the CHD group in comparison to controls. Factor VIII (FVIII), VWF, and ristocetin cofactor activity (Rcof) levels were significantly higher in the study vs. control group. Concentrations of ADAMTS-13 were decreased in the CHD vs. control group, but the difference was not statistically significant. Our results, in combination, indicate a balanced hemostatic mechanism, although with greater variability in neonates with CHD, while developmental aspects of coagulation are evident in the specific patient population. Conclusions: the coagulation profile is moderately impaired early in the course of CHD, though increased thrombogenicity is already present and should not be ignored.

A Global Assessment of Coagulation Profile and a Novel Insight into Adamts-13 Implication in Neonatal Sepsis.

Neonatal sepsis is a life-threatening condition associated with significant morbidity and mortality. Sepsis-induced coagulopathy is a well-recognized entity, signifying the strong cross-talk between inflammation and coagulation. The aim of the present study was to compare the coagulation profile between the acute phase of sepsis and recovery in term and preterm neonates. Additional comparisons to healthy neonates were undertaken. Levels of clotting, anti-clotting factors and ADAMTS-13 (A disintegrin and metalloprotease with thrombospondin type-1 motives), the cleaving protein of von Willebrand factor (VWF), were measured in 16 term and preterm neonates in the acute phase of infection and following recovery, as well as in 18 healthy neonates. Clotting times were prolonged, while levels of particular clotting factors were lower in the acute phase of infection compared to controls and recovery. On the other hand, levels of fibrinogen, factor VIII (FVIII) and VWF were significantly higher in the acute phase in comparison to controls and recovery, while they remained persistently higher in the infection group compared to controls. In regard to the anticlotting mechanism, a clear suppression was observed in septic neonates. ADAMTS-13 levels were significantly lower in the acute phase of infection in comparison to controls and recovery (p = 0.015 and 0.004, respectively), while a trend toward superimposed normalization was demonstrated post infection, as higher ADAMTS-13 levels were measured in recovered neonates compared to controls (p = 0.002). The coagulation profile is considerably deranged in neonatal sepsis. ADAMTS-13 deficiency in septic neonates is a novel finding with promising future implications, as ADAMTS-13 substitution may serve as a useful therapeutic option in neonatal sepsis, prompting further investigation in future studies.

Evolución a largo plazo y factores influyentes de los pacientes que ingresan con FANV en los servicios de Medicina Interna de Galicia / Long-term evolution and influential factors of patients admitted with NVAF in the Internal Medicine services of Galicia

Objectives: The objective of this study was to investigate the longterm evolution and influencing factors of patients with non-valvular atrial fibrillation (NVAF) admitted to internal medicine services. Patients and method: This is an observational and retrospective study of the evolution during five years of the patients admitted, between January-2016 and January-2017, with FANV in the Galician Internal Medicine services. For this end, it was quantified the emergency room visits, hospital admissions and survival. The factors with more influence over these variables were studied. Results: It was included 1.342 patients and followed for 5 years. There were 3.691 hospital admissions, and 8.687 visits to the emergency department (ED). They had a survival of 66,6%, with a median survival of 1.034,57 days. The univariate analysis found that age, antithrombotic treatment at discharge and Barthel's index influenced survival, but not sex. However, in the multivariate analysis only Barthel’s index was found to be independent variable that influence survival. Conclusions: Patients with NVAF admitted to internal medicine services constitute a subpopulation at high risk of hospital readmission and visits to the ED. A change in the model of transition to discharge and outpatient follow-up is necessary, through adapted proactive programs, capable of reducing hospital events and improving the quality of life of these patients and their caregivers. (AU)

Objetivos: Estudiar la evolución a lo largo plazo, así como los factores que influyen en la misma, de la población con fibrilación auricular no valvular (FANV) que ingresa en los servicios de Medicina Interna. Pacientes y método: Estudio observacional y retrospectivo de la evolución durante cinco años, de los pacientes ingresados con FANV en los servicios de Medicina Interna gallegos, entre enero-2016 y enero-2017. Para este fin se cuantificaron las visitas a urgencias, los ingresos hospitalarios y la supervivencia. Se estudiaron los factores que más influyeron en estas variables. Resultados: Se incluyeron 1.342 pacientes y se realizó un seguimiento durante 5 años. Se contabilizaron 3.691 ingresos hospitalarios, y 8.687 visitas al servicio de urgencias (SU). Tuvieron una supervivencia del 66,6%, con una mediana de supervivencia de 1.034,57 días. En el análisis univariante, la edad, el tratamiento antitrombótico al alta y el índice de Barthel influyeron en la supervivencia, no así el sexo. En el análisis multivariante, el índice de Barthel fue la única variable independiente que influyó en la supervivencia. Conclusiones: Los pacientes con FANV ingresados en los servicios de Medicina Interna, suponen una subpoblación de alto riesgo de reingreso hospitalario y visitas al SU. Es necesario un cambio en el modelo de transición al alta y de seguimiento ambulatorio, mediante programas proactivos adaptados, capaces de reducir eventos hospitalarios y mejorar la calidad de vida de estos pacientes y sus cuidadores. (AU)

Abordaje multidisciplinar de un tumor phyllodes maligno de mama en una mujer de 16 años / Multidisciplinary approach to a malignant phyllodes tumour of the breast in a 16-year-old woman

INTRODUCCIÓN: El tumor phyllodes (TP) es una tumoración fibroepitelial que representa el 0,3-0,9% del total de tumores de mama. Si bien puede aparecer a cualquier edad, acostumbra a presentarse en mujeres jóvenes (edad media 40-45 años) como una masa palpable no dolorosa, de rápido crecimiento y de gran tamaño. Los TP presentan una elevada tasa de recurrencia local y raramente metastatizan. CASO CLÍNICO: Se presenta el caso de una paciente de 16 años, con antecedente de exéresis de un fibroadenoma de 70mm. A los 15 meses presentó reaparición de una masa ipsilateral filiada como TP maligno de mama. Fue sometida a tumorectomía amplia, pero dada la afectación de varios márgenes de resección finalmente se realizó mastectomía con reconstrucción inmediata. RESULTADOS: Los TP suponen un reto en cuanto al diagnóstico, a veces complejo, tanto mediante pruebas de imagen como histológico. El tratamiento principal es quirúrgico mediante resección amplia (cirugía conservadora o mastectomía) de la lesión sin abordaje axilar. La recurrencia local se asocia mayoritariamente a afectación de márgenes de resección. Con frecuencia es necesario recurrir a técnicas de oncoplastia dado el elevado volumen de mama afectado. CONCLUSIONES: El manejo de este tipo de tumores requiere un abordaje completo y multidisciplinar

INTRODUCTION: Phyllodes tumour of the breast (PT) is a fibroepithelial mass representing 0.3-0.9% of all breast tumours. Although it can appear at any age, it usually develops in young women (mean age 40-45 years) as a painless, palpable, large, rapidly-growing mass. These tumours have a high local recurrence rate and rarely metastasize. CASE REPORT: We present the case of a 16-year-old female patient who had undergone extirpation of a 70mm fibroadenoma. Fifteen months later, an ipsilateral mass developed, which was diagnosed as a malignant PT of the breast. She underwent wide local excision, but given the involvement of various resection margins, mastectomy with immediate reconstruction was performed. RESULTS: PT are a diagnostic, and sometimes complex, challenge both through imaging and histological tests. The main treatment is surgical, through wide (breast-conserving surgery or mastectomy) resection of the lesion without axillary clearance. Local recurrence is mainly associated with involvement of the resection margins. It is often necessary to resort to oncoplastic techniques due to the volume of affected breast tissue. CONCLUSIONS: Management of this type of tumour requires a comprehensive and multidisciplinary approach