[Psychomotor development in patients with severe congenital heart disease]. / Desarrollo psicomotor en pacientes con cardiopatia congenita grave.

AIM: Retrospective analysis of the neurodevelopment in the first two years of life in patients with severe congenital heart disease. PATIENTS AND METHODS: Out of 89 patients with severe congenital heart disease 19 were excluded due to a history of prematurity and/or chromosomopathy, four due to a history of ischemic stroke and two due to lack of medical history. Denver Test (DT) results at 2, 6, 12, 15 and 18 months, and results in motor, language and social interaction fields were achieved. RESULTS: 59.4% were male and 40.6% female. The mean age of patients undergoing extracorporeal membrane oxygenation with pathological DT at 18 months was 3 months, compared to 11.88 months in those with normal DT. DT at 2 months was normal in 98.4% of patients, 87.5% at 6 and 12 months, 81.3% at 15 months and 85% at 18 months. Two patients with abnormal neurodevelopment normalized the DT before 24 months. The field of neurodevelopment most affected was language (15.6%), followed by motor (10.9%) and social interaction (8%). CONCLUSIONS: Psychomotor development delay, especially in the area of language, is more frequent in patients with severe congenital heart disease. The presence of cyanosis and the need for extracorporeal membrane oxygenation were the variables that are most associated with this type of pathology.

TITLE: Desarrollo psicomotor en pacientes con cardiopatia congenita grave.Objetivo. Analizar los hitos motores alcanzados en los dos primeros años de vida en pacientes con cardiopatia congenita grave. Pacientes y metodos. De 89 pacientes con cardiopatia congenita grave, 19 fueron excluidos por antecedentes de prematuridad o cromosomopatia, cuatro por antecedente de ictus isquemico y dos por ausencia de historia clinica. Se obtuvieron resultados del test de Denver (TD) a los 2, 6, 12, 15 y 18 meses, y resultados en los campos motor, del lenguaje y de interaccion social. Resultados. El 59,4% fueron varones, y el 40,6%, mujeres. La edad media de los pacientes sometidos a oxigenacion con membrana extracorporea con TD patologico a los 18 meses fue de 3 meses, frente a 11,88 meses de los que presentaban un TD normal. El TD a los 2 meses resulto normal en el 98,4% de los pacientes, en el 87,5% a los 6 y 12 meses, en el 81,3% a los 15 meses, y en el 85% a los 18 meses. Dos de los pacientes con alteracion en el neurodesarrollo normalizaron el TD antes de los 24 meses. El campo del neurodesarrollo mas afectado fue el del lenguaje (15,6%), seguido del motor (10,9%) y de la interaccion social (8%). Conclusiones. El retraso en el desarrollo psicomotor, especialmente en el area del lenguaje, es mas frecuente en pacientes con cardiopatias congenitas graves, y la presencia de cianosis y la necesidad de circulacion con membrana extracorporea son las variables que mas se asocian con este tipo de patologia.

[Diagnostic test in emergency departments for bacterial infections in infants younger than 12 months]. / Orientación diagnóstica en la unidad de urgencias en lactantes menores de 12 meses con infección bacteriana.

OBJECTIVE: To evaluate clinical and analytic numeric data that may help the emergency departments to identify bacterial infections in infants. PATIENTS AND METHODS: A retrospective study of 430 infants with bacterial growth in cultures (culture from blood, 30; urine, 207; stools, 193, and/or cerebrospinal fluid, n 25) was performed. These patients were compared with a control group (n 430), randomly selected from patients aged less than 12 months with negative cultures who were hospitalized with suspected infection. Neonates and surgical patients were excluded from both groups. Statistical analysis was performed using Student's t-test for independent samples, Levene's test for the study of equality of variances, bivariate correlation and one-factor ANOVA, and receiver-operating characteristic (ROC) curves and odds ratios were calculated when statistically significant (p < 0.05) results were obtained. These analyses were performed using the SPSS 10.0 statistical software package. RESULTS: Of the infants admitted to the pediatric unit, 11.7 % had at least one positive bacterial culture. Temperature (p 0.005), leucocyte count (p 0.003), percentage of neutrophils (p < 0.0001) and C-reactive protein (p < 0.0001) were significantly higher in infants with positive cultures. In invasive infections significant differences were found in sex (more frequent in males) (p 0.03), heart rate (p < 0.0001) and respiratory rate (p 0.003). In the ROC curves, the best diagnostic yield was obtained for C-reactive protein (0.93 for a cutoff value of 29 mg/l, 86 % specificity and 91 % sensitivity). CONCLUSION: C-reactive protein is essential for diagnosis of bacterial infection in infants in the emergency department.

First experience of enzyme replacement therapy with idursulfase in Spanish patients with Hunter syndrome under 5 years of age: case observations from the Hunter Outcome Survey (HOS).

Hunter syndrome (mucopolysaccharidosis type II [MPS II], OMIM309900) is a rare X-linked lysosomal storage disorder caused by deficiency of the enzyme iduronate-2-sulphatase, resulting in accumulation of glycosaminoglycans (GAGs), multisystem organ failure and early death. Enzyme replacement therapy (ERT) with idursulfase is commercially available since 2007. Early access programs were established since 2005. However, limited information on the effects of ERT in young children is available to date. The aim of this analysis was therefore to determine the effects of ERT on patients younger than 5 years of age. We report data from six Spanish patients with confirmed Hunter syndrome who were younger than 5 years at the start of ERT, and had been treated with weekly intravenous infusions of idursulfase between 6 and 14 months. Baseline and treatment data were obtained from the Hunter Outcome Survey (HOS). HOS is an international database of MPS II patients on ERT or candidates to be treated, that collects data in a registry manner. HOS is supported by Shire Human Genetic Therapies, Inc. (Cambridge, MA, USA). At baseline, all patients showed neurological abnormalities, including ventriculomegaly, hydrocephaly, cerebral atrophy, perivascular changes and white matter lesions. Other signs and symptoms included thoracic deformity, otitis media, joint stiffness and hepatosplenomegaly, demonstrating that children under 5 years old can also be severely affected. ERT reduced urinary GAG levels, and reduced spleen (n = 2) and liver size (n = 1) after only 8 months. Height growth was maintained within the normal range during ERT. Joint mobility either stabilized or improved during ERT. In conclusion, this case series confirms the early onset of signs and symptoms of Hunter syndrome and provides the first evidence of ERT beneficial effects in patients less than 5 years of age. Similar efficacy and safety profiles to those seen in older children can be suggested, although further studies including a direct comparison with older patients would still be required.

[Benign convulsions with mild rotavirus gastroenteritis]. / Convulsiones benignas durante gastroenteritis leve por rotavirus.

INTRODUCTION: Benign convulsions with mild gastroenteritis (CwG) are a rare diagnosis in Western countries, and it is characterized by afebrile seizures, almost times more than once, during a gastroenteritis without severe hydroelectrolitic affection. The most frequent implied virus is rotavirus. This is a benign disease, in spite of cluster seizures, and neither complementary explorations nor antiepileptic treatment is required. PATIENTS AND METHODS: Seven cases between 6 and 27 months aged of CwG due to rotavirus are reported. RESULTS: Four patients had more than one seizure, and all convulsions were generalized hypermotor type. Rotavirus gastroenteritis was mild in all cases; only one patient was admitted for hospitalization before seizures appearance. There were no findings in all cases with lumbar punction or brain neuroimaging. Electroencephalogram was normal in all cases, except in one patient who showed frontal acute waves, which disappeared in a later examination. No patients have shown seizure recurrence or development delay. CONCLUSIONS: CwG due to rotavirus are possibly infradiagnosed, and perhaps classified as atypical febrile seizures, febrile status or early infancy benign partial epilepsy. This benign diagnosis should be considered for a correct management and prognosis.

[Acute Gastroenteritis in hospitalized children. 14-Year evolution]. / Gastroenteritis aguda en pacientes hospitalizados. Estudio evolutivo de 14 años.

OBJECTIVES: To analyze the etiology and evolution of patients with acute gastroenteritis hospitalized in our pediatric department and to study the clinical and laboratory differences between acute viral and bacterial gastroenteritis. PATIENTS AND METHODS: We studied the children with a diagnosis of acute gastroenteritis, aged between 0 and 14 years, who were consecutively admitted between 1987 and 2000. Differences were considered statistically significant if p < 0.05. RESULTS: A total of 2,613 patients diagnosed with acute gastroenteritis were hospitalized (10.4 % of hospital admissions). The most common pathogens isolated were rotaviruses (46.5 %), followed by Salmonella (32.6 %) and Campylobacter (19.3 %). Hospital admissions due to Salmonella (p < 0.0001), other bacteria (Escherichia coli and Shigella) (p < 0.002) and adenoviruses (p < 0.01) significantly decreased. Rotaviruses were the most frequently detected pathogens in winter and in children aged less than 1 year (p < 0.0001). The incidence of Salmonella spp was greater in summer and in children older than 2 years (p < 0.0001). The incidence of hyperthermia (rectal temperature higher than 38.5 degreeC) (p < 0.0001), dehydration (p < 0.0005) and fecal blood (p < 0.0001) was higher in bacterial diarrheas. Erythrocyte sedimentation rate (p < 0.001) and leukocyte counts were higher in bacterial gastroenteritis (p < 0.01). CONCLUSIONS: Rotaviruses were the most frequently isolated enteropathogens. The features that best distinguished between bacterial and viral diarrhea were hyperthermia and fecal blood. Hospital admissions due to Salmonella Shigella E. coli, and adenoviruses significantly decreased.