J.M. Ramos Fernández

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.

Encefalomielitis aguda diseminada: análisis epidemiológico, clínico, analítico y evolutivo en 16 pacientes

INTRODUCTION Acute disseminated encephalomyelitis (ADEM) is a rare paediatric disease of autoimmune origin. The aim of this study is to estimate its impact in our area over the past 10 years and to analyse the clinical, laboratory and radiological presentation, as well as study the response to treatment and outcome. PATIENTS AND METHODS A retrospective analysis of cases diagnosed with current ADEM criteria. The age and month of the year at onset, sex, family history of demyelination, initial symptoms, disease or previous vaccination, CSF features, serology, PCR for herpes virus group in blood and CSF, oligoclonal bands in CSF, EEG and neuroimaging features of the skull by MRI, the type of treatment received and the response were recorded on each of the patients diagnosed. RESULTS There were 16 patients, with a mean age of 5.6 years. Male/female: 11/5, with a dominant debut in the colder months of the year, predominantly gait disorder, changes in consciousness and seizures. The radiology by MRI showed a predominance of multiple lesions in the same T2 stage, in deep white matter and lesions in deep grey matter. There were no in blood parameters and the CSF was normal in half of the cases. The origin was determined in 7 cases. CONCLUSIONS The annual incidence in children under 14 years was estimated at 0.64/100,000. The clinical and radiological findings were the basis of diagnosis. The human herpes virus 6 was involved in 4 cases. Most patients recovered completely.

Situación de la pediatría hospitalaria en España: informe de la Sociedad Española de Pediatría Hospitalaria (SEPHO)

Few reports are available on the status of pediatric hospital medicine in Spain. This has prompted the Spanish Society of Hospital Pediatrics (SEPHO) to conduct a study to determine the status of pediatric hospital care received. Data released by the Ministry of Health, the National Institute of Statistics have been used in the study, and an analysis was made of the results of a computerized survey designed and developed by SEPHO and available on the Internet for completion from November 2011 to December 2012 among Spanish hospitals. The results of this survey are part of the beginning of our journey as an association, and the current status of child and family welfare during hospitalization needs to be determined in order to consider and, where appropriate, make recommendations for improvement and standardization of care. The study, still unpublished, is to determine the state of pediatric hospital care as seen and analyzed from the perspective of the professionals directly involved in pediatric general hospital care. We included hospitals of different size and complexity of care. The aim of this report is to present the results of the survey and relate it to demographic and health care data from official sources.

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.

PP09.2 – 2584: Spinal muscular atrophy: Review of clinical characteristics and treatment in the last 25 years

Objective Spinal muscular atrophy (SMA) include a group of disorders that produce disabilities in all patients. Our aim is to review the epidemiological and clinical characteristics, genetic distribution and to analyze the care and development of the SMA in our area. Methods Retrospective descriptive study of patients diagnosed in our hospital during the past 25 years (1987–2013), with clinical and neurophysiological diagnosis of SMA. Results 37 patients were found, representing an incidence of 1 case per 10,000 live births in our reference population. The male/female ratio was 1.6/1. The type of SMA diagnosed more frequently was type I (26 cases), followed by type II with 9 cases, 1 case of SMA type III, and 1 case of SMA with respiratory distress (SMARD1). The most frequent genetic alteration was homozygous deletion of exons 7 and 8 of the SMN1 gene (31 cases). 25 patients were followed in our centre, 7 by the Pediatric Home Hospitalisation Unit. Most of them received motor rehabilitation (15), breathing physiotherapy in 8 cases or basic respiratory care in 5. 2 cases received oxygen by low-flow nasal cannula, non-invasive ventilation in 1 case and invasive ventilation with tracheostomy in 2 cases. In 4 cases a nasogastric tube and in 1 case a gastrostomy was prescribed. The median survival for type I was 8.0 months and 15.8 years for type II. Conclusion The incidence in our population remains stable at around 1/10.000. Most cases presented a typical genetic alteration and were predominantly male. In approximately 1/10 patients the genetic alteration was different from the classical to the SMN gene. The caring level has increased in line with social and welfare demands in recent years. We improve our work with consensus statement for standard of care in spinal muscular atrophy.