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OBJECTIVES: To prospectively assess the value of serum total bilirubin (TB) within 3 months of hepatoportoenterostomy (HPE) in infants with biliary atresia as a biomarker predictive of clinical sequelae of liver disease in the first 2 years of life. STUDY DESIGN: Infants with biliary atresia undergoing HPE between June 2004 and January 2011 were enrolled in a prospective, multicenter study. Complications were monitored until 2 years of age or the earliest of liver transplantation (LT), death, or study withdrawal. TB below 2 mg/dL (34.2 µM) at any time in the first 3 months (TB <2.0, all others TB ≥ 2) after HPE was examined as a biomarker, using Kaplan-Meier survival and logistic regression. RESULTS: Fifty percent (68/137) of infants had TB < 2.0 in the first 3 months after HPE. Transplant-free survival at 2 years was significantly higher in the TB < 2.0 group vs TB ≥ 2 (86% vs 20%, P < .0001). Infants with TB ≥ 2 had diminished weight gain (P < .0001), greater probability of developing ascites (OR 6.4, 95% CI 2.9-14.1, P < .0001), hypoalbuminemia (OR 7.6, 95% CI 3.2-17.7, P < .0001), coagulopathy (OR 10.8, 95% CI 3.1-38.2, P = .0002), LT (OR 12.4, 95% CI 5.3-28.7, P < .0001), or LT or death (OR 16.8, 95% CI 7.2-39.2, P < .0001). CONCLUSIONS: Infants whose TB does not fall below 2.0 mg/dL within 3 months of HPE were at high risk for early disease progression, suggesting they should be considered for LT in a timely fashion. Interventions increasing the likelihood of achieving TB <2.0 mg/dL within 3 months of HPE may enhance early outcomes. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00061828 and NCT00294684.

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BACKGROUND: Disseminated intravascular coagulation is an entity characterized by activation of the coagulation cascade and endogenous fibrinolysis, which can cause death. Our objectives were to identify the incidence of disseminated intravascular coagulation, its etiologic agents and the correlation between the Apache II score and the one proposed by the International Society on Thrombosis and Haemostasis for the diagnosis of this entity. METHODS: Retrospective, descriptive, observational study of patients treated in an intensive care unit over a 17-month period. Etiology, age, sex, platelet count, coagulation tests, serum fibrinogen levels and D-dimer quantification were analyzed. The score on the scale proposed by the International Society on Thrombosis and Haemostasis and the Apache II score were calculated. RESULTS: 11 patients (7.18 % of the total number treated subjects at the intensive care unit) had a diagnosis of disseminated intravascular coagulation; six were females. Sepsis was the main etiologic agent (four cases). The most affected age group was the 51-60 years group (four cases). The prognosis was bad in seven subjects. Patients with five points or more in the DIC system, but with a low Apache II score had a good prognosis. CONCLUSIONS: The combination of the DIC and the Apache II scores serves for predicting the outcome of patients with severe organ injuries.

INTRODUCCIÓN: la coagulación intravascular diseminada es una entidad caracteriza por activación de la cascada de la coagulación y fibrinólisis endógena, que puede provocar la muerte. Nuestros objetivos fueron identificar la incidencia de coagulación intravascular diseminada, sus agentes etiológicos y la correlación entre la puntuación de la escala Apache II y la propuesta por la Sociedad Internacional de Trombosis y Hemostasia para el diagnóstico de esta entidad. MÉTODOS: estudio retrospectivo, observacional y descriptivo de pacientes atendidos en una unidad de cuidados intensivos en un periodo de 17 meses. Se analizó etiología, edad, sexo, conteo de plaquetas, coagulograma, niveles de fibrinógeno sérico y cuantificación del dímero D. Se calculó la puntuación de la escala propuesta por la Sociedad Internacional de Trombosis y Hemostasia y de la escala APACHE II. RESULTADOS: 11 pacientes (7.18 % del total atendido en la unidad de cuidados intensivos) tuvieron diagnóstico de coagulación intravascular diseminada; seis eran mujeres. La sepsis fue el principal agente etiológico (cuatro casos). El grupo etario más afectado fue el de 51 a 60 años (cuatro casos). El pronóstico fue malo en siete. Los pacientes con cinco o más puntos en el sistema CID pero con puntuación baja en la escala Apache II tuvieron buen pronóstico. CONCLUSIONES: la combinación de la puntuación CID y de la escala Apache II sirve para pronosticar el desenlace de los pacientes con lesiones orgánicas severas.

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Se revisaron 96 casos de autopsia de carcinoma de páncreas con objeto de determinar la relación entre el tipo histológico y el pronóstico. Se estudió además la frecuencia de la coagulación intravascular ddiseminada en este grupo de casos y se comparó con la de un grupo testigo de noplasmas malignas no pancreáticas apareado por edad y sexo. La mayoría de los casos (86.4%) correspondió a adenocarcionomas de los conductos. El tiempo promedio de evoluación fue de 2.8 meses. Con excepción de algunos tumores raros, como el cistadenocarcinoma mucinoso, la clasificación histológica tiene poco valor poruqe el pronóstico es semejante en las distintas variedades. La coagulación intravascular diseminada y las tromboembolias nenosas y de arterias pulmonares fueron más comunes en casos de carcinoma de páncreas que en otras neoplasias malignas (p<0.035 y p>0.001 respectivamente). Esta diferencia se debe problablemente a la mayor producción de substancias tromboplásticas por el carcinoma de páncreas.

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Severe deficiencies of protein C, a pivotal coagulation-regulatory protein, have been reported in neonates as an apparently transient condition. In this prospective study, cord blood was collected at 193 deliveries and assays of protein C were correlated with clinical status, other coagulation results, and outcome. Protein C levels of less than 0.1 unit/ml were found most frequently in preterm infants with respiratory distress, infants of diabetic mothers, and infants of twin gestations. Levels of protein C correlated with levels of factor VIII activity but did not correlate with markers of consumptive coagulopathy. A protein C level less than 0.1 unit/ml was significantly correlated with the subsequent onset of thrombosis, even when the effects of gestational age and birth weight were excluded. Low cord blood levels of protein C may reflect delayed maturation or increased turnover in certain infants and appear to convey an independent risk of thrombosis, but the critical concentration of protein C necessary to maintain neonatal hemostasis is not known.

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Eight-seven consecutive children with head injury were evaluated within two hours of injury by clinical examination, by computed tomographic brain images, and for systemic blood clotting disorders. All were treated by a standard regimen and survival rates calculated according to the initial neurologic abnormalities and pathology of the injury. Patients with the more severe neurologic abnormalities and those with more brain tissue destruction had poorer survival rates. However, 71% of all patients had one or more abnormal clotting tests and 32% had the disseminated intravascular coagulation and fibrinolysis syndrome by laboratory criteria. The mortality was over four times greater in those patients with DIC compared to those with normal clotting values. Our findings indicate that minor hemostatic abnormalities are the rule in head-injured children, that DIC occurs in nearly one-third of cases, and that DIC is associated with a marked increase in the mortality after brain injury: DIC may be a treatable secondary effect of head trauma that could decrease the mortality.

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Between 1964 and 1971, 113 children with the syndrome of disseminated intravascular coagulation were studied at the Hospital Infantil de México, including 17 cases with the diagnosis of fulminant purpura. Comparison was established with reports from foreign institutions. The following conclusions are offered: Fulminant purpura appears after a period of latency following the causal disease. It is not caused by septicemia; shows a clear picture in infants, preschool and school children, with letality index of 17.7%. In Mexico, DIC was caused by infection in 88% of the cases and in 68% of them, the infection started in the digestive tract. The diagnosis of fulminant purpura is based on the clinical picture, while in DIC, the diagnosis must also be based on laboratory tests unless one half of the diagnoses be missed.

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