[Multi-organ failure as first clinical sign of macrophage activation syndrome in childhood Still's disease]. / Fracaso multiorgánico como forma de presentación del síndrome de activación macrofágica en la enfermedad de Still infantil.

Macrophage activation syndrome is a form of secondary haemophagocytic lymphohistiocytosis seen in the context of rheumatic diseases. It is seen most frequently in association with systemic onset juvenile arthritis or childhood Still's disease. Hemophagocytosis is part of a sepsis-like clinical syndrome caused by hypercytokinemia due to a highly stimulated but ineffective immune response. Coagulopathy and hemorrhages, decreased white cell count, elevated levels of aspartate aminotransferase, fever, rash, hepatosplenomegaly and central nervous system dysfunction are some of diagnostic criteria of macrophage activation syndrome, but it is very difficult to diagnose due to the lack of specific clinical signs. We report a 8-year-old child who was admitted to the ICU with lethargy, fever, acute respiratory failure, coagulopathy, metabolic acidosis and multiorgan failure. Septic shock was suspected, but he was diagnosed with macrophage activation syndrome and treated with corticosteroids and intravenous immunoglobulin and later discharged from the ICU.

[The evaluation of autopsy in the pediatric intensive unit]. / Evaluación de la autopsia en la Unidad de Cuidados Intensivos Pediátricos.

OBJECTIVE: Because of concerns about the declining autopsy rate, an attempt was made to evaluate the contributions from the postmortem examination in children. PATIENTS AND METHODS: We carried out a retrospective comparison analysis between clinical and pathological diagnosis of 56 consecutive autopsies performed on children who died in the PICU during the period 1983-1995. RESULTS: The autopsy rate was 60%. Autopsy provided valuable clinical information in 50% of the cases. There were major diagnostic errors in three patients (5%), that if detected before death would probably have improved survival. Another 14 cases (25%) showed missed clinical diagnoses related to the basic illness and the cause of death, whose premortem diagnosis would not have prolonged survival. There were no diagnostic discrepancies in 28 cases (50%). The most unexpected findings revealed by the autopsies were iatrogenics (10 cases), metabolic diseases (4 cases), congenital immunodeficiency syndromes (4 cases) and pulmonary opportunistic infections (3 cases). Eight of these diseases were genetic. An age < 12 months or and ICU stay < 24 hours were not predicting factors of a higher incidence of major diagnostic errors. CONCLUSIONS: The value of the autopsy as quality assurance and to detect iatrogenics and occult genetic diseases is unquestionable. New strategies have to be designed to increase the rate of autopsies.

[Definitions of sepsis in severe juvenile meningococcal infection. A review of 80 cases]. / Definiciones de sepsis en la infección meningocócica infantil grave. Una revisión de 80 casos.

OBJECTIVE: To describe the definitions for sepsis proposed by ACCP/SCCM Consensus Conference and to evaluate its capacity to classify children with severe meningococcal infection in homogeneous risk groups. METHODS: Eighty children with acute meningococcal infection and severe sepsis or septic shock, admitted to the pediatric ICU during a ten years period were reviewed. Mean age: 38 months (1,3 mo-14 yrs). RESULTS: N. meningitidis was isolated in 84%. Sixty-four percent of the patients were bacteremic and 39% showed a positive culture in CSF. Overall mortality was 19%. Fifty-two patients (65%) were in severe sepsis on admission, fifteen of them (29%) developed shock, mortality for this group was 4%. Twenty-eight patients (35%) were in septic shock on admission, mortality was 44%. Overall mortality of the shock group was 35%, mortality of shock on admission was higher than mortality of shock postadmission (44% vs 13%, p = 0.0001). Major complications were: DIC (28%), ARDS (26%), purpura fulminans (21%). There were not major complications or deaths in patients who did not develop shock. Bacteremia was not significant associated with shock or death. Meningitis was more frequent in severe sepsis group but 62% of deaths got it. Univariant analysis showed significant differences between both groups relative to tissular perfusion variables, coagulation and meningeal involvement. Multivariate analysis allowed us to establish a predictive model of survival feasible on admission to the ICU. For its determination three parameters are used: blood pressure, platelets and base excess. CONCLUSION: Definitions proposed for severe sepsis and septic shock are a valuable tool to classify children with acute meningococcal infection in homogeneous risk groups.