IntroductionNutritional support in the care of children with threatening or limiting diseases is based on improving their nutritional status and quality of life. Malnutrition is common in these patients due to swallowing disorders, maldigestion or malabsorption, and to chronic diseases with increased energy expenditure, losses or requirements.MethodsA descriptive, retrospective analysis of patients with life-threatening or life-limiting diseases receiving Enteral Nutrition (EN) from 2000 to 2016, admitted to a Paediatric Palliative Care/Home Hospitalisation Unit, has been performed.Results188 patients have been treated. The mean age was 2.8 years (IR: 24 days-15.8 years). The most frequent indications were: swallowing disorders (42%), malnutrition (13.8%) and failure to thrive (12.2%).According to the classification of the Association for children with life threatening situation or terminal situation and their families:• Group 1 (Diseases for which curative treatment may be feasible but may fail): 23.9% (heart disease 75.5%, cancer 17.8%)• Group 2 (Diseases for which premature death is anticipated, but intensive treatment may prolong good-quality life and participation in normal childhood activities): 26.6% (Gastrointestinal (GI) diseases and malformations 48%)• Group 3 (progressive diseases for which treatment is exclusively palliative): 21.3% (57.5% advanced or metastatic cancer, 20% neuromuscular diseases)• Group 4 (severe, non-progressive neurological impairment resulting in vulnerability and complications that may lead to premature death): 28.2% (39.6% hypoxic encephalopathy)Enteral feeding was performed through a nasogastric tube in most patients (83%), requiring gastrostomy in 23.4%.During this time period, 49 patients (26%) died in relation to their underlying disease except for one due to surgical complication of the gastrostomy.ConclusionsNeurological and oncological patients represent the most frequent pathologies requiring EN. In our study, children with congenital heart disease represent the third pathology in frequency, above GI diseases. Nutrition, along with symptom control, is one of the most important aspects of treating patients with limiting or life-threatening situations, half of which will require specialised palliative care.

Background and aimsDespite 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.MethodsRetrospective 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.ResultsWe 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).ConclutionSIn 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 1Features 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, 1MUNC 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 Liver2 (66.7)1 (10) CNS2 (66.7)1 (10) Kidney0 (0)1 (10) Hemodinamics0 (0)3 (30) Lung0 (0)1 (10)Hepatomegaly, n (%)3 (100)10 (100)Splenomegaly, n (%)3 (100)9 (90)Haemoglobin (mg/dl), median (IR) aD7.6 (6.9–7.6)9 (8.4–...

Introductionobesity (OB) is one of the most serious public health problems of the 21 st century, especially associated with complications, among which the predisposition to hypertension. There are conflicting data on the relationship between arginine levels and the presence of hypertension in adults, with almost no data on children.Material and methodsA prospective longitudinal observational study of cohorts. Case = high weight (overweight (BMI>p85) and obese (OB) (BMI>p95)); control = normal weight (BMI p95 respectively Children 6–11 years old, prepubertal (Tanner 1–2) were selected. Exclusion criteria: secondary obesity, other organic disease, chronic intake of drugs or diabetes. Plasma arginine levels were measured by tandem mass spectrometry (mmol/L) at initial recruitment and after 12–15 months. One year follow-up of patients repeating clinical measurements of blood pressure, anthropometry and analytical data (arginine).Results100 children, 52% male, with a mean age of 8.6±1.6 years; 71% overweight and overweight (63% obese); 79 children with waist circumference above p90. Arginine has no correlation with any of the analytical parameters or with the percentiles of BP and there is no statistically significant difference between baseline arginine levels according to the presence of insulin resistance, overweight or obesity Patients with high blood pressure at initial moment and after one year follow up have lower levels of plasma arginine (p<0.05). Among patients with a valid measure of BP after one year, 55% have a baseline arginine less than 20 mmol/L and, among them, 59% presented a rise in blood pressure above the 90th percentile (p 0.006), with an odds ratio of 7.22 (1.60–32.46), p 0.006.DiscussionOur work provides evidence that there is an already valuable arginine deficit a few months before a tensional increase above the 90th percentile. Biochemical data are provided that can serve as predictors at an age where there may be reversibility of the ”hypertensive gait” process. These findings may elucidate new pathogenic mechanisms in adult hypertension begins in childhood, especially in the case of overweight patients.

Background and aimsInherited metabolic diseases (IMD) have an increasing prevalence, and can be detected by neonatal screening, at their onset as an acute decompensation or a chronic disease. The heart is a target organ in these disorders and can compromise the patient life if no early treatment is employed. The aim of this study is to describe the cases occurred in a tertiary children hospital and to analyse the heart involvement.MethodsRetrospective study of 14 cases diagnosed with cardiomyopathy associated to IMD in a tertiary children’s hospital from January 2010 to January 2017.Resultsamong the 14 cases described, 4 were lysosomal storage disorders (2 Pompe diseases, 1 Hunter disease (HD), 1 mucopolysaccharidosis type IX (Sly syndrome), 7 β-oxidation disorders (3 CPT2 deficiency, 2 carnitine transporter deficiency, 2 VLCAD), 1 glycosylation defect, 1 atypical progeroid syndrome (APS) and 1 glycine encephalopathy. Regarding cardiac involvement, hypertrophic cardiomyopathy was the most frequent one (8 cases), followed by rhythm disorders (1 VLCAD deficiency with paroxysmal supraventricular tachycardia, 3 CPT II deficiency with lethal neonatal form), 1 case of dilated cardiomyopathy and 1 severe pulmonary hypertension with secondary cardiac failure. 11 cases were diagnosed at disease onset and only in 3 cases the cardiac involvement emerged during the course of the disease, having been previously diagnosed with the underlying disease (HD, VLCADD, APS). 6 patients died and 4 developed sequelae from the underlying diseases or the heart involvement. Only 4 remain healthy, with remission of the cardiomyopathy. Treatment was pharmacological for all patients except for the HD one, who required surgery because of broken mitral valve, and the APS who needed a heart transplantation because of severe dilated cardiomyopathy. Symptomatic treatment in all patients with 3 patients under enzymatic replacement treatment (HD and Pompe).Conclusionscardiomyopathy is commonly associated with IMD, with the hyperthophic cardiomyopathy as the most frecuent one. Other heart involvement can be found (arrhythmia, progressive heart failure o postransplant complications), because of the evolution of the underlying disease or clínical deteroration after acute decompensations.We would like to highlight that neonatal screening facilitates an early diagnosis of some of these diseases (4 cases in our series) and can be the diagnostic key before or after cardiomyopathy manifestation, helping with therapeutic management or genetic counselling, taking into account that these entities have a high mortality (46% in our series).