A. Urda Cardona

Validación de una escala clínica de severidad de la bronquiolitis aguda

INTRODUCTION Acute bronchiolitis (AB) is a very common disease, with a high rate of seasonal hospitalization. Its management requires homogeneous clinical interpretations for which there are different approaches depending on the scales, none of which are properly validated today. OBJECTIVE To create an AB severity scale (ABSS) and to validate it. MATERIAL AND METHOD The development of a parameterized construct with a gradual cumulative score of respiratory rate, heart rate, respiratory effort, auscultation of wheezing and crackles, and the inspiration/expiration ratio. Also, the validation of the ABSS performed on patients diagnosed with AB, the reliability measured by observing the behavior of internal consistency, test-retest, external validity and inter-observer agreement. RESULTS From a total of 290 measurements a Cronbachs reliability alpha of 0.83 was obtained; Kappa agreement index of 0.93 in the test-retest agreement, and Kappa index of 0.682 (α<0.05) for inter-observer agreement. CONCLUSIONS The ABSS can be a reliable tool for measuring the severity of AB.

Crisis convulsivas asociadas a gastroenteritis: estudio de incidencia y análisis clínico

INTRODUCTION Benign convulsions associated with gastroenteritis are now increasingly recognized as clinical condition to the extent that it has become an independent entity under the heading of non-epileptic situational seizures. The aim of this study is to determine the annual incidence in the reference population of our hospital and the clinical characterization of seizures associated to gastroenteritis, in the absence or presence of fever for comparison. PATIENTS AND METHOD All seizures associated with gastroenteritis treated in our hospital were prospectively collected over a period of two calendar years. The children included were aged 6 months to 6 years with seizures in the context of gastroenteritis without electrolyte abnormalities, and divided into two groups, with and without fever. RESULTS There were 14 cases from a reference population of 39,900 with a homogeneous semiological presentation. CONCLUSION The annual incidence was estimated at 1/10 000 children for afebrile seizures associated with gastroenteritis. The clinical behaviour and the incidence of seizures associated with fever and gastroenteritis was similar, but with an appearance somewhat earlier from the onset of symptoms, and at a slightly higher age.

Atrofia muscular espinal: revisión de nuestra casuística en los últimos 25 años

OBJECTIVES To determine the incidence of spinal muscular atrophy (SMA) in our study population and genetic distribution and epidemiological and clinical characteristics and to analyze the level of care and development. MATERIAL AND METHOD Retrospective descriptive study of patients treated in our hospital in the past 25 years (from 1987 to early 2013), with a clinical and neurophysiological diagnosis of SMA. RESULTS A total of 37 patients were found, representing an incidence for our reference population and year of 1 case per 10,000 live births. Males predominated (male/female ratio: 1.6/1). The type of SMA diagnosed more frequently was, type i (26 cases), followed by type ii (9 cases), one case with SMA type iii, and one case of spinal muscular atrophy with respiratory distress type 1 (SMARD1). The most frequent genetic alteration was homozygous deletion of exons 7 and 8 of SMN1 gene in 31 cases, while five patients had atypical genetics. The median survival for type i was 8.0 months and 15.8 years for type ii. CONCLUSIONS The incidence in our population remains stable at around 1/10.000. Most cases presented with, predominantly male, typical genetics. In approximately 1/10 patients the genetic alteration was different from the classical one to the SMN gene. The prevalence of AME unrelated SMN gene was 1/37. The level of care has increased in line with social and welfare demands in recent years.

Crisis febriles complejas: estudio de la patología asociada y utilidad de las pruebas complementarias

INTRODUCTION Although one third of febrile seizures are complex, a consensus has still not been reached on how to manage them, as is the case with simple febrile seizures. The objective of this study is to estimate the usefulness of complementary examinations and the risk of associated serious intracranial pathology. PATIENTS AND METHODS A retrospective review was conducted from 2003 until 2011 on patients from 6 months to 6 years presenting with a complex febrile seizure admitted to a tertiary care hospital, excluding the cases with previous neurological disease. Epidemiological and clinic variables were collected, as well as complementary tests and complications. RESULTS We found 65 patients (31 females and 34 males), of whom 44 had repeated seizures in the first 24 hours, with 15 having focal seizures. The vast majority (90%) of the recurrences occurred before 15 hours. The mean age was 20.7 months and temperature was 39.1 ± 0.12°C. None of the patients had severe intracranial pathology. The electroencephalogram gave no helpful information for the diagnosis. Neuroimaging was normal in all studied cases. CONCLUSIONS The incidence of complications in complex febrile seizure in our series did not justify the systematic admission or the systematic study with complementary tests when the neurological examination was normal. The routine electroencephalogram does not appear to be justified.INTRODUCTION Although one third of febrile seizures are complex, a consensus has still not been reached on how to manage them, as is the case with simple febrile seizures. The objective of this study is to estimate the usefulness of complementary examinations and the risk of associated serious intracranial pathology. PATIENTS AND METHODS A retrospective review was conducted from 2003 until 2011 on patients from 6 months to 6 years presenting with a complex febrile seizure admitted to a tertiary care hospital, excluding the cases with previous neurological disease. Epidemiological and clinic variables were collected, as well as complementary tests and complications. RESULTS We found 65 patients (31 females and 34 males), of whom 44 had repeated seizures in the first 24 hours, with 15 having focal seizures. The vast majority (90%) of the recurrences occurred before 15 hours. The mean age was 20.7 months and temperature was 39.1 ± 0.12°C. None of the patients had severe intracranial pathology. The electroencephalogram gave no helpful information for the diagnosis. Neuroimaging was normal in all studied cases. CONCLUSIONS The incidence of complications in complex febrile seizure in our series did not justify the systematic admission or the systematic study with complementary tests when the neurological examination was normal. The routine electroencephalogram does not appear to be justified.

Invasive disease caused by Haemophilus parainfluenzae III in a child with uropathy

Urinary tract infections (UTIs) caused by Haemophilus parainfluenzae represent a very small percentage of this kind of pathology in children, and it has scarcely been described in the medical literature. According to previous studies of over 800 urine samples in children under 15 years old, a decrease of 50% (from 0.13% to 0.07%) is estimated in its occurrence over the last two decades. This can be explained by the early detection of UTIs and their early empirical treatment, because this micro-organism shows high sensitivity to antibiotics. Also, the culture media in which this bacterium grows are not included in most current protocols. Here we report a case of a UTI caused by H. parainfluenzae in a 4-year-old boy.

Pamidronato: una alternativa terapéutica en osteomielitis crónica multifocal recurrente

1. Shepard CW, Rosenstein NE, Fischer M. Neonatal meningococcal disease in the United States, 1990 to 1999. Pediatr Infect Dis J. 2003;22:418—22. 2. Lo WT, Yuh YS, Wang CC, Chu ML. Early onset neonatal infection with Neisseria meningitidis serogroup C: case report and literature reviw. Eur J Pediatr. 2003;162:785—7. 3. Bhutta ZA, Khan IA, Agha Z. Fatal intrauterine meningococcal infection. Pediatr Infect Dis J. 1991;10:868—9. 4. Ellis M, Weindling AM, Davidson DC, Ho N, Damjanovic V. Neonatal meningococcal conjunctivitis associated with meningococcal meningitis. Arch Dis Child. 1992;67:1219—20. 5. Arango CA, Rathore MH. Neonatal meningococcal meningitis: case reports and review of literature. Pediatr Infect Dis J. 1996;15:1134—6. 6. Selander B, Schliamser S, Schalén C, Fredlund H, Unemo M, Johanson PJ. A premature infant with fulminant meningococcal septicaemia. Scand J Infect Dis. 2003;35:345—8. 7. Falero Gallego MP, Arroyos Plana A, Santillana Ferrer L, Cazorla Calleja R, Cobas Pazos J, Ureta Huertos A. Sepsis neonatal tardía con meningitis por meningococo del serogrupo C. An Esp Pediatr. 1999;50:639—40. 8. Clegg HW, Todres ID, Moylan FM, Keim DE, Shannon DC. Fulminant neonatal meningococcemia. Am J Dis Child. 1980;134:354—5. 9. Katier N, Traen M, De Dooy J, Geyskens L, Mahieu L. Neonatal purpura due to Neisseria meningitidis serogroup C infection. Eur J Pediatr. 2003;162:283—4. 10. Jones RN, Slepack J, Eades A. Fatal neonatal meningococcal meningitis: association with maternal cervical-vaginal colonization. JAMA. 1976;236:2652—3.

AB0968 Hypovitaminosis d in juvenile idiopathic arthritis: prevalence and related factors

Background 25hydroxy-vitamin D not only plays a key role in calcium homeostasis, but also has antiinflammatory and immunomodulatory properties. Hypovitaminosis D prevalence in children suffering from Juvenile Idiopathic Arthritis (JIA) ranges from 6% to 30% according to different publications. Objectives Evaluate hypovitaminosis D prevalence in JIA pediatric patients in Spain and assess involved factors. Methods Observational cross-sectional study in JIA Spanish patients from 4 to 15 years, monitored by a Pediatric Rheumatology Unit. Monoarticular forms and patients with other chronic diseases or receiving different treatments from those indicated for JIA were excluded. Anthropometric, clinical and treatment data were recorded. Bone metabolism parameters and validated diet (KIDMED) and exercise (PAQ-C/PAQ-A) questionnaires were obtained. Hypovitaminosis D was defined as 25hydroxy-vitamin D plasma levels lower than 30 ng/ml Results 76 children participated. Their characteristics are included in table 1. The populations prevalence estimation of hypovytaminosis D in children with JIA was 16 - 35% (CI 95%). We found no relationship between 25 hydroxy-vitamin D levels and sex, JIA subtype neither duration or dose of systemic glucocorticoids. In bivariate analysis we found direct association between hypovitaminosis D and Body Mass Index percentile (BMIp) (p=0,05), received dose of prednisone (p=0,03) and clinical activity duration (p=0,04); and an inverse relationship with physical activity level (p=0,04). In multivariate analysis, relationship between hypovitaminosis D and BMIp (B 0,024; p 0,016) and with disease activity (B 0,015; p 0,01) were maintained. Moreover, we found an inverse association with biological disease-modifiying antirheumatic drugs (B-4,69; p 0,048), specifically with anti-tumoral necrosis factor α (antiTNFα) (B -4,7; p 0,042) Conclusions Hypovitaminosis D prevalence in our population is similar to previously described. JIA patients with higher BMIp have more hypovitaminosis D, as it has been reported in other inflammatory diseases. A direct relationship exists between inflammatory activity and vitamin D, but we need more studies to asses if one is cause or consecuence of the other. Patients treated with antiTNF have better plasma levels of 25 hydroxy-vitamin D, this can be explained because these drugs may increase 25 hydroxy-vitamin D levels or due to a better response to antiTNF of those patient with higher plasma levels of 25 hydroxy-vitamin D. References Nisar MK, Masood F, Cookson P, Sansome A, Östör AJ. What do we know about juvenile idiopathic arthritis and vitamin D? A systematic literature review and meta-analysis of current evidence. Clin Rheumatol 2013; 32:729–734. Santos-Antunes J, Corte-Real Nunes A, Lopes S, Macedo G. The Relevance of Vitamin D and Antinuclear Antibodies in Patients with Inflammatory Bowel Disease Under Anti-TNF Treatment: A Prospective Study. Inflamm Bowel Dis 2016;22:1101–1106. Ham M, Longhi MS, Lahiff C, Cheifetz A, Robson S, Moss AC. Vitamin D levels in adults with Crohns disease are responsive to disease activity and treatment. Inflamm Bowel Dis. 2014; 20(5):856–60. Disclosure of Interest None declared

¿Son realmente útiles los anti-TNFα en vasculitis sistémicas? Experiencia en panarteritis nodosa

Polyarteritis nodosa (PAN) is a rare vasculitis that affects small and medium vessels. Its clinical manifestations are varied, ranging from mild skin changes to multisystemic forms.1 New diagnostic criteria were established in 2008,2 with diagnosis requiring histopathological abnormalities (necrotising vasculitis) or angiographic abnormalities (aneurism, stenosis or occlusion) with at least one of the following:

Seguridad hospitalaria en pediatría

Paediatrians are rendering care nowadays in environments that are increasingly complex and constantly changing. This results in multiple opportunities to cause unintended harm. In the last 15 years the world has been increasingly aware that care must be delivered in such a way as to ensure the maximum safety of the child. Since 1999, when the American Institute of Medicine published its report To Err is Human, professionals, as well as society, have been examining that care in order to identify the possible risks and implement safety solutions. The depth and breadth of harm incurred have prompted a constant stream of publications seeking to uncover avoidable errors. Patient safety (PS) is defined as ‘‘freedom from accidental injury’’ caused by medical care, such as harm or death attributable to adverse drug events, patient misidentification and health care-associated or health care-acquired infections. In recent decades it has been established that medical errors, previously considered on an individual level, have more far-reaching implications, and it has become clear that the design of health care systems plays a crucial role, since their complexity also leads to mistakes being made. In 2001 the American Academy of Pediatrics published the statement ‘‘Principles of patient safety in pediatrics’’, followed by ‘‘Prevention of medication errors in the pediatric inpatient setting’’ in 2007, ‘‘Patient safety in the

PO-0062 Technological Tools To Optimise Metabolic Control In Adolescents With Type 1 Diabetes

Introduction Flexible intensive therapy (FIT) emphasises in insulin administered before meals, adjusting the number of portions of carbohydrates and preprandialglycemic control. Accu - check expert (ACE) glucometer is a calculator with integrated data. It is necessary to introduce bolus insulin/carbohydrate ratio, factor sensitivity to insulin (FSI), ideal blood sugar and insulin duration adjusted by different time zones. It makes the calculation of insulin, managing in real-time the number of portions of carbohydrates and the glycemic control obtained. The patient must be trained how to change these parameters if it is required. Aims and methods Prospective randomised intervention to determine the effectiveness of bolus calculator ACE in type 1 diabetic (D1) adolescents with FIT and values of HbA1c higher than 7%. We measure glycosylated haemoglobin at 0, 3 and 6 months. Control group: 12 patients with D1 whose educational support involves counting portions and calculation of ratio and FSI before this intervention and at 3 months. Case group: 12 patients with D1 who have received equal educational support as control group and specific training on bolus calculator, before baseline and at 3 months. Results The groups are homogeneous in age, duration of diabetes and HbA1c before starting study. In both groups decreased HbA1c. There is a higher decrease in intervention group. The difference of HbA1c is significative statistically in case group at 6 months. Conclusions After the intervention both groups showed a decrease in HbA1c at 3 and 6 months, with higher difference in case group. This difference is clinically relevant and statistically significant in the case group when it is compared from baseline to 6 months. Abstract PO-0062 Table 1Introduction Flexible intensive therapy (FIT) emphasises in insulin administered before meals, adjusting the number of portions of carbohydrates and preprandialglycemic control. Accu - check expert (ACE) glucometer is a calculator with integrated data. It is necessary to introduce bolus insulin/carbohydrate ratio, factor sensitivity to insulin (FSI), ideal blood sugar and insulin duration adjusted by different time zones. It makes the calculation of insulin, managing in real-time the number of portions of carbohydrates and the glycemic control obtained. The patient must be trained how to change these parameters if it is required. Aims and methods Prospective randomised intervention to determine the effectiveness of bolus calculator ACE in type 1 diabetic (D1) adolescents with FIT and values of HbA1c higher than 7%. We measure glycosylated haemoglobin at 0, 3 and 6 months. Control group: 12 patients with D1 whose educational support involves counting portions and calculation of ratio and FSI before this intervention and at 3 months. Case group: 12 patients with D1 who have received equal educational support as control group and specific training on bolus calculator, before baseline and at 3 months. Results The groups are homogeneous in age, duration of diabetes and HbA1c before starting study. In both groups decreased HbA1c. There is a higher decrease in intervention group. The difference of HbA1c is significative statistically in case group at 6 months. Conclusions After the intervention both groups showed a decrease in HbA1c at 3 and 6 months, with higher difference in case group. This difference is clinically relevant and statistically significant in the case group when it is compared from baseline to 6 months.