Guillermo Milano-Manso

Derrame pericárdico quiloso espontáneo: presentación de dos casos

Most cases of chylopericardium in childhood are secondary to thoracic surgery, especially after cardiac surgery. However, it can also be present in children without this history, as we show in this report. First, a nine year-old boy in whom chylopericardium was the first manifestation of a lymphangiomatosis, and the second one, a fifteen months old girl with a history of Downs syndrome and autoimmune enteropathy, which was classified as primary congenital chylopericardium. Also, a review of literature over the last 13 years is made, and management of this rare entity is discussed.

Déficit de vitamina D y morbimortalidad en pacientes críticos pediátricos

OBJECTIVES To determine the prevalence and risks factors of vitamin D deficiency, as well as its relationship with morbidity and mortality in a PICU. MATERIAL AND METHODS An observational prospective study in a tertiary childrens University Hospital PICU conducted in two phases: i: cohorts study, and ii: prevalence study. The study included 340 critically ill children with ages comprising 6 months to 16 years old. EXCLUSION CRITERIA Chronic kidney disease, known parathyroid disorders, and vitamin D supplementation. Total 25-hydroxyvitamin D [25(OH)D] was measured in the first 48hours of admission to a PICU. Parathormone, calcium, phosphate, blood gases, blood count, C-reactive protein, and procalcitonin were also analysed. A record was also made of demographic features, characteristics of the episode, and complications during the PICU stay. RESULTS The overall prevalence rate of vitamin D deficiency was 43.8%, with a mean of 22.28 (95% CI 21.15-23.41) ng/ml. Patients with vitamin D deficiency were older (61 vs 47 months, P=.039), had parents with a higher level of academic studies (36.5% vs 20%, P=.016), were admitted more often in winter and spring, had a higher PRISM-III (6.8 vs 5.1, P=.037), a longer PICU stay (3 vs 2 days, P=.001), and higher morbidity (61.1% vs 30.4%, P<001) than the patients with sufficient levels of 25(OH)D. Patients who died had lower levels of 25(OH)D (14±8.81ng/ml versus 22.53±10.53ng/ml, P=.012). Adjusted OR for morbidity was 5.44 (95%CI; 2.5-11.6). CONCLUSIONS Vitamin D deficiency is frequent in critically ill children, and it is related to both morbidity and mortality, although it remains unclear whether it is a causal relationship or it is simply a marker of severity in different clinical situations.

OC-64 Hemopahagocytic lymphohistiocytosis: is it really important to make a difference between primary and secondary cases?

Background and aims Despite HLH-04 diagnostic criteria and treatment protocol for hemophagocytic lymphohistiocytosis (HLH) were proposed for both primary (pHLH) and secondary (sHLH) cases, several experts have criticised they are not optimum for the latter. The aims of this investigation are to describe the features of HLH patients that can help to differenciate between primary and secondary cases. Methods Retrospective descriptive study in of HLH patients from 0 to 14 years, diagnosed according to HLH-04 criteria, in a tertiary Paediatric Hospital during the last 10 years (Jan2007-Jan2017). Clinical, analytical and therapeutic data were retrospectively collected from medical records. pHLH and sHLH features were compared in a bivariant analysis. Statistical significance was considered as p<0.1. Results We found 13 patients, 3pHLH and 10sHLH, whose features are described in Table 1. The median age was 1.5 years (IR: 0.75–3.3), earlier in primary cases (1.0y vs 1.8y), though not significantly. 2 of 3 pHLH had grey hair and neurological impairment and were part of a consanguineous Moroccan family, with cousins diagnosed with Griscelli syndrome, so pHLH was suspected since the begining. CNS injury was more frequent in pHLH (66%) than in sHLH (10%)(p=0.04). There were no significant differences between other organ involvement. At onset, ferritin (p=0.043), neutrophils (p=0.05) and platelets (p=0.28) values were higher in sHLH than in pHLH. Lowest number of neutrophils (p=0.028), highest LDH levels (p=0.036) and minimum platelets count (p=0.07) were higher in sHLH too. There were no differences in other analytical features. Regarding treatment, pHLH cases needed steroids more often (p=0.06), as well as agressive therapies as etoposide (p=0.05) and intrathecal methotrexate (p=0.05). Mortality was significantly higher in primary group (p=0.03). ConclutionS In our experience pHLH and sHLH have important disparities according to evolution, mortality and treatment. Although 2 of our pHLH had physical appereance and family background that helped with etiological diagnosis, it is not always that easy. In this study, we found pHLH and sHLH have important differences, according to clinical and laboratory findings, that may have implications in subsequent management. Larger multicentric More studies are required to establish specific diagnostic criteria and therapeutic schemes depending on the aetiology. Abstract OC64 Table 1 Features of primary and secondary HLH in a tertiary Paediatric Hospital pHLH (n=3) sHLH (n=10) pHLH (n=3) sHLH (n=10) Male, n (%) 2 (66.7) 3 (30) Age (years), median (IR) 1 (0.5–1) 1.8 (0.8–4) Aetiology, n (%) Griscelli syndrome: 2 (66.7) Viral infections:6 (60): 3 CMV, 2 EBV, 1 MUNC deficiency: 1 (33.3) Enterovirus Rheumatic diseases:2 (20) Length of fever at diagnosis (days), median (IR) 7 (2–7) 8 (2.7–11.7) Organ involvement  Liver 2 (66.7) 1 (10)  CNS 2 (66.7) 1 (10)  Kidney 0 (0) 1 (10)  Hemodinamics 0 (0) 3 (30)  Lung 0 (0) 1 (10) Hepatomegaly, n (%) 3 (100) 10 (100) Splenomegaly, n (%) 3 (100) 9 (90) Haemoglobin (mg/dl), median (IR)  aD 7.6 (6.9–7.6) 9 (8.4–9.9)  Lv 6.5 (5.6–6.7) 7.6 (6.5–8.7) Neutrophils/mm3, median (IR)  aD 560 (400-600) 2050 (775–6450)  Lv 300 (20-300) 941 (547–1480) Ferritin (µg/L), median (IR)  aD 1651 (1392–1720) 7222 (3140–14922)  Hv 2187 (564–2324) 7222 (3735–14636) Fibrinogen (mg/dL), median (IR)  aD 167.7 (84–172) 157.5 (92.2–344.5)  Lv 56.7 (28.9–61.2) 91.5 (51.9–91.5) ALT (U/l), median (IR)  aD 102 (78-105) 176.5 (121–300)  Hv 133 (105-142) 383.5 (135–529) Hemophagocytosis in bone marrow n (%) 2 (66.7) 2 (33.3)*1 Treatment, n (%)  Steroids 3 (100) 4 (40)  Cyclosporine A 1 (33.3) 2 (20)  Etoposide 2 (66.7) 0 (0)  Intrathecal methotrexate 1 (33.3) 0 (0)  Plasmapheresis/IVIG 1 (33.3) 3 (30)  Underlying disease therapy 1 (33.3) 7 (70) Deaths, n (%) 3 (100) 1 (10) *1: Bone marrow biopsy in only 6 patients. aD: At diagnosis; Lv: Lowest value; Hv: Highest value; IVIG: Intravenous immunoglobulins

Estudio de validez de una escala de gravedad de la bronquiolitis aguda para orientar el ingreso en UCIP

INTRODUCTION At present, there are few validated scoring tests for assessing acute bronchiolitis (AB) severity, and limited information on their test power. The aim of the present study is to evaluate the validity of an acute bronchiolitis severity score (ABSS) to help in deciding PICU admission. PATIENTS AND METHOD Prospective, descriptive, observational study of previously healthy infants under 1 year of age with AB, where the ABSS was used to compare severity as regards the need for PICU admission. The sample size was estimated as at least 175 patients. The research team was trained in the use of ABSS. All patients in the study were evaluated with ABSS daily, as well as in the case of clinical deterioration. The initial and maximum ABSS scores were contrasted to the need for PICU admission. A receiver operative curve was constructed, and the area under the curve was calculated, and the optimum point of sensitivity / specificity was estimated. RESULTS The study included a total of 190 patients (male / female: 58% / 42%). PICU was required in 11 (6%). The mean± SD ABSS-maximal score for patients who required and did not require PICU was 10.55± 1.12 and 6.35± 2.3, respectively (P<.001). The AUC for ABSS-maximal was 0.94 (P<.001, 95% CI: 0.90-0.98). The optimal cut-off point was set at ≥10 points for a sensitivity of 82% and a specificity of 91%. CONCLUSIONS ABSS estimates the severity of AB regarding the need for PICU admission, with a sensitivity and specificity of clinical usefulness.

Menor respuesta linfocitaria en casos graves de bronquiolitis aguda por virus respiratorio sincitial

INTRODUCTION Acute bronchiolitis (AB) of the infant has a serious outcome in 6-16% of the hospital admitted cases. Its pathogenesis and evolution is related to the response of the T lymphocytes. The objective of the present study is to determine if the lower systemic lymphocytic response is related to a worse outcome of AB in hospitalised infants. PATIENTS AND METHOD Retrospective observational-analytical study of cases-controls nested in a cohort of patients admitted due to RSV-AB between the period from October 2010 to March 2015. Those with a full blood count in the first 48hours of respiratory distress were included. Infants with underlying disease, bacterial superinfection, and premature infants <32 weeks of gestation were excluded. The main dichotomous variable was PICU admission. Other variables were: gender, age, post-menstrual age, gestational and post-natal tobacco exposure, admission month, type of lactation, and days of onset of respiratory distress. Lymphocyte counts were categorised by quartiles. Bivariate analysis was performed with the main variable and then by logistic regression to analyse confounding factors. RESULTS The study included 252 infants, of whom 6.6% (17) required PICU admission. The difference in mean±SD of lymphocytes for patients admitted to and not admitted to PICU was 4,044±1755 and 5,035±1786, respectively (Student-t test, P<.05). An association was found between PICU admission and lymphocyte count <3700/ml (Chi-squared, P=.019; OR: 3.2) and it was found to be maintained in the logistic regression, regardless of age and all other studied factors (Wald 4.191 P=.041, OR: 3.8). CONCLUSIONS A relationship was found between lymphocytosis <3700/ml in the first days of respiratory distress and a worse outcome in previously healthy infants <12 months and gestational age greater than 32 weeks with RSV-AB.Introduction: Acute bronchiolitis (AB) of the infant has a serious outcome in 6-16% of the hospital admitted cases. Its pathogenesis and evolution is related to the response of the T lymphocytes. The objective of the present study is to determine if the lower systemic lymphocytic response is related to a worse outcome of AB in hospitalised infants. Patients and method: Retrospective observational-analytical study of cases-controls nested in a cohort of patients admitted due to RSV-AB between the period from October 2010 to March 2015. Those with a full blood count in the first 48 hours of respiratory distress were included. Infants with underlying disease, bacterial superinfection, and premature infants <32 weeks of gestation were excluded. The main dichotomous variable was PICU admission. Other variables were: gender, age, post-menstrual age, gestational and post-natal tobacco exposure, admission month, type of lactation, and days of onset of respiratory distress. Lymphocyte counts were categorised by quartiles. Bivariate analysis was performed with the main variable and then by logistic regression to analyse confounding factors. Results: The study included 252 infants, of whom 6.6% (17) required PICU admission. The difference in mean ± SD of lymphocytes for patients admitted to and not admitted to PICU was 4,044 ± 1755 and 5,035 ± 1786, respectively (Student-t test, P<.05). An association was found between PICU admission and lymphocyte count <3700/ml (Chi-squared, P=.019; OR: 3.2) and it was found to be maintained in the logistic regression, regardless of age and all other studied factors (Wald 4.191 P=.041, OR: 3.8). Conclusions: A relationship was found between lymphocytosis <3700/ml in the first days of respiratory distress and a worse outcome in previously healthy infants <12 months and gestational age greater than 32 weeks with RSV-AB.