The MELD Score Is Superior to the Maddrey Discriminant Function Score to Predict Short-Term Mortality in Alcohol-Associated Hepatitis: A Global Study.

INTRODUCTION: Several scoring systems predict mortality in alcohol-associated hepatitis (AH), including the Maddrey discriminant function (mDF) and model for end-stage liver disease (MELD) score developed in the United States, Glasgow alcoholic hepatitis score in the United Kingdom, and age, bilirubin, international normalized ratio, and creatinine score in Spain. To date, no global studies have examined the utility of these scores, nor has the MELD-sodium been evaluated for outcome prediction in AH. In this study, we assessed the accuracy of different scores to predict short-term mortality in AH and investigated additional factors to improve mortality prediction. METHODS: Patients admitted to hospital with a definite or probable AH were recruited by 85 tertiary centers in 11 countries and across 3 continents. Baseline demographic and laboratory variables were obtained. The primary outcome was all-cause mortality at 28 and 90 days. RESULTS: In total, 3,101 patients were eligible for inclusion. After exclusions (n = 520), 2,581 patients were enrolled (74.4% male, median age 48 years, interquartile range 40.9-55.0 years). The median MELD score was 23.5 (interquartile range 20.5-27.8). Mortality at 28 and 90 days was 20% and 30.9%, respectively. The area under the receiver operating characteristic curve for 28-day mortality ranged from 0.776 for MELD-sodium to 0.701 for mDF, and for 90-day mortality, it ranged from 0.773 for MELD to 0.709 for mDF. The area under the receiver operating characteristic curve for mDF to predict death was significantly lower than all other scores. Age added to MELD obtained only a small improvement of AUC. DISCUSSION: These results suggest that the mDF score should no longer be used to assess AH's prognosis. The MELD score has the best performance in predicting short-term mortality.

Obstructive sleep apnea syndrome and growth failure.

Obstructive sleep apnea syndrome is a common problem among children and is recognized as a cause of significant medical morbidity. Since the 1980s, it has been suggested that obstructive sleep apnea syndrome is a risk factor for growth failure in children. In many cases, it has been shown that growth failure is reversible once the obstructive sleep apnea syndrome is resolved. The objectives of this study were to analyze and compare growth failure prevalence in a Mediterranean population of children with obstructive sleep apnea syndrome and healthy children matched in age and sex, and to assess the effectiveness of tonsillectomy and adenoidectomy in resolving growth retardation. We compared 172 children with obstructive sleep apnea syndrome (apnea-hypopnea index ≥ 3) who had undergone tonsillectomy and adenoidectomy with 172 healthy controls in terms of key anthropometric parameters. Most of the criteria used for growth failure were higher to a statistically significant degree in the study group vs the control group: height-for-age ≤ 3rd percentile (7.56% vs 2.91%; p = 0.044), weight-for-age ≤ 5th percentile (9.30% vs 2.33%; p = 0.005), weight-for-age ≤ 3rd percentile (8.14% vs 2.33%; p = 0.013) and height and/or weight for-age ≤ 5th percentile (13.95% vs 5.81%; p = 0.009). The height-for-age ≤ 5th percentile was almost at the limit of statistical significance (8.72% for the study group vs 4.65% for the control group; p = 0.097). At one-year post-surgery follow-up, 10 of 15 children with height-for-age ≤ 5th percentile had achieved catch-up growth (66.6%), and 14 of 24 children with height- and/or weight-for-age ≤ 5th percentile had normalized growth (58.33%). For children with failure to thrive or who have growth failure, physicians should consider the possibility of obstructive sleep apnea. A significant number of children with obstructive sleep apnea concurrent with growth failure could benefit from tonsillectomy and adenoidectomy to recover and normalize their growth rate.

[X-linked adrenoleukodystrophy with an atypical radiological pattern]. / Adrenoleucodistrofia ligada al X con patron radiologico atipico.

INTRODUCTION: X-linked adrenoleukodystrophy (X-ALD) is the most frequent peroxisomal disease. It is due to a mutation in the ABCD1 gene. The loss of functioning of ABCD1 triggers ineffective beta oxidation of very long-chain fatty acids, which gives rise to an accumulation of these fatty acids. The typical alteration revealed in neuroimaging scans in the cerebral form is symmetrical periventricular demyelination with posterior location. CASE REPORT: We report the case of a 10-year-old boy with right spastic hemiparesis and subacute cognitive impairment. Magnetic resonance imaging of the brain showed symmetrical involvement of the white matter in the left frontoparietotemporal region, and calcifications were observed in the computerised axial tomography scan. X-ALD was confirmed by means of the elevated levels of very long-chain fatty acids, and a pathogenic variant was found in the ABCD1 gene. CONCLUSIONS: Symmetrical demyelination with calcifications has rarely been reported in X-ALD, and these findings could delay diagnosis. This exceptional presentation should always be taken into consideration in children with subacute onset of motor symptoms and cognitive or behavioural regression.

TITLE: Adrenoleucodistrofia ligada al X con patron radiologico atipico.Introduccion. La adrenoleucodistrofia ligada al X (ALD-X) es la enfermedad peroxisomica mas frecuente. Se debe a una mutacion en el gen ABCD1. La perdida de la funcion de ABCD1 provoca una betaoxidacion inefectiva de los acidos grasos de cadena muy larga, lo que provoca la acumulacion de estos acidos grasos. La alteracion tipica en la neuroimagen en la forma cerebral es la desmielinizacion periventricular simetrica y de localizacion posterior. Caso clinico. Niño de 10 anos, con hemiparesia espastica derecha y deterioro cognitivo subagudo. La resonancia magnetica cerebral mostro afectacion asimetrica de la sustancia blanca en la region frontoparietotemporal izquierda, y en la tomografia axial computarizada se visualizaban calcificaciones. Se confirmo ALD-X mediante la elevacion de los niveles de acidos grasos de cadena muy larga, y se encontro una variante patogenica en el gen ABCD1. Conclusiones. La desmielinizacion asimetrica con calcificaciones raramente se ha descrito en la ALD-X, y estos hallazgos podrian retrasar el diagnostico. Esta presentacion excepcional se deberia considerar siempre en niños con inicio subagudo de sintomas motores y regresion cognitiva o del comportamiento.

Diagnóstico diferencial de parálisis flácida en pediatría / Differential diagnosis of flaccid paralysis in paediatric medicine

No disponible

Resultados de la colocación del estimulador del nervio vago en epilepsia y calidad de vida en un hospital pediátrico / Vagus nerve stimulator implantation for epilepsy in a paediatric hospital: outcomes and effect on quality of life

Introducción: La epilepsia es uno de los trastornos neurológicos más frecuentes de la infancia, presentándose en un 0,5-1%. Aproximadamente un 20-30% de los pacientes son farmacorresistentes. El objetivo de este trabajo es describir en 30 pacientes el impacto sobre las crisis y la calidad de vida del estimulador del nervio vago (ENV). Métodos: Se trata de un estudio descriptivo, retrospectivo, mediante revisión de las historias clínicas de todos los pacientes a quienes se les colocó el ENV entre el 2008 y 2013 en nuestro centro. La calidad de vida fue valorada mediante la escala de calidad de vida en el niño con epilepsia (CAVE), obtenida por medio de una entrevista telefónica. Resultados: Se incluyeron 19 niños (64%) y 11 niñas (36%) con una mediana de comienzo de las crisis de 21 meses (1-144 meses). La edad promedio de colocación del ENV fue de 11,89 años. El tiempo de seguimiento fue de 6-36 meses. A los 6 meses la reducción de las crisis en promedio fue del 38%, a los 12 meses del 43%, a los 24 meses del 42% y a los 36 meses del 54%. De todos los pacientes evaluados al menos un 50% se catalogaron como respondedores. Según la CAVE un 54% de las familias encontró el efecto del ENV como bueno o muy bueno y un 39% como regular. Conclusiones: El ENV es un tratamiento paliativo, generalmente bien tolerado, parcialmente efectivo para el control de la epilepsia refractaria en pediatría y con repercusiones positivas sobre la calidad de vida

Introduction: Epilepsy, which is present in 0.5% to 1% of the paediatric population, is one of the most frequent childhood neurological disorders. Approximately 20% to 30% of these cases will be drug-resistant. The objective of this study is to describe the impact of vagal nerve stimulation (VNS) on seizures and quality of life in a sample of 30 patients. Methods: Descriptive, retrospective study of all patients with a VNS device implanted between 2008 and 2013 in a single paediatric hospital, based on patients’ medical records. Quality of life was assessed using the Spanish scale for quality of life in children with epilepsy, completed by means of a telephone interview. Results: We describe a population of 19 boys (64%) and 11 girls (36%) with a mean age at seizure onset of 21 months (1-144 months). The mean age of VNS implantation was 11.89 years. Follow-up periods ranged from 6 to 36 months. Mean reduction in seizures at 6 months was 38%, with a reduction of 43% at 12 months, 42% at 24 months, and 54% at 36 months. At least half of all patients were classified as responders. According to the quality of life scale, 54% of the families rated the effect of VNS as either very good or good while 39% rated it as fair. Conclusions: VNS is a safe palliative treatment that is generally well tolerated. It is partially effective for controlling drug-resistant epilepsy and exerts a positive effect on quality of life

Vagus nerve stimulator implantation for epilepsy in a paediatric hospital: outcomes and effect on quality of life.

INTRODUCTION: Epilepsy, which is present in 0.5% to 1% of the paediatric population, is one of the most frequent childhood neurological disorders. Approximately 20% to 30% of these cases will be drug-resistant. The objective of this study is to describe the impact of vagal nerve stimulation (VNS) on seizures and quality of life in a sample of 30 patients. METHODS: Descriptive, retrospective study of all patients with a VNS device implanted between 2008 and 2013 in a single paediatric hospital, based on patients' medical records. Quality of life was assessed using the Spanish scale for quality of life in children with epilepsy, completed by means of a telephone interview. RESULTS: We describe a population of 19 boys (64%) and 11 girls (36%) with a mean age at seizure onset of 21 months (1-144 months). The mean age of VNS implantation was 11.89 years. Follow-up periods ranged from 6 to 36 months. Mean reduction in seizures at 6 months was 38%, with a reduction of 43% at 12 months, 42% at 24 months, and 54% at 36 months. At least half of all patients were classified as responders. According to the quality of life scale, 54% of the families rated the effect of VNS as either very good or good while 39% rated it as fair. CONCLUSIONS: VNS is a safe palliative treatment that is generally well tolerated. It is partially effective for controlling drug-resistant epilepsy and exerts a positive effect on quality of life.

Macroamilasemia en una niña de 13 años / Macroamylasemia in a thirteen-year-old girl

La Macroamilasemia es una alteración bioquímica caracterizada por la presencia en la sangre de un complejo macromolecular formado por amilasa ligada a las inmunoglobulinas séricas. Suele cursar con hiperamilasemia, lo que plantea el diagnóstico diferencial con la pancreatitis. Aunque no es excepcional en la edad adulta, se han descrito muy pocos casos en la edad pediátrica. Reportamos un caso clínico de una paciente de 13 años de edad, con antecedentes de agenesia renal izquierda congénita y un cuadro clínico de dolor abdominal recidivante. Los exámenes complementarios evidenciaron hiperamilasemia persistente, con valores normales de amilasuria, así como de tripsina inmunoreactiva y lipasa séricas. El cociente de aclaramiento amilasa-creatinina no estaba elevado. Otros exámenes complementarios para investigar las posibles causas del dolor abdominal, incluida la pancreatitis, fueron normales o negativos. La ultrasonografía y TAC abdominales no evidenciaron alteraciones pancreáticas ni otras lesiones, a excepción de la agenesia renal izquierda y la hiperplasia compensadora del riñón derecho. El diagnóstico de macroamilasemia se confirmó por electroforesis en gel de agarosa y mediante precipitación con polietilenglicol. Al ser la macroamilasemia un trastorno benigno, su identificación como la causa de la hiperamilasemia es esencial para evitar un diagnóstico y tratamientos erróneos (AU)

A new computerised method for the assessment of skeletal maturity in the newborn infant.

Of the existing methods for assessment of skeletal maturity in children over 1 year of age none is particularly suited to the newborn infant. We describe a computerised method by which area, perimeter and progression in the shape of ossification centres of talus and calcaneus are evaluated separately. From single lateral radiographs of the left ankle of 302 normal term and preterm infants whose birth weights were appropriate for gestational age we constructed reference curves of areas and perimeters at different gestational ages, as well as frequency distributions of each morphological maturity stage. This method may be applicable in assessing skeletal maturity in pathological conditions, such as intrauterine growth retardation and congenital hypothyroidism.

Evaluation of serum zinc-binding capacity during childbirth, in newborn infants and during the menstrual cycle.

This is a study of the value of total serum zinc, serum zinc per g of protein, serum zinc-binding capacity and serum zinc-binding capacity per g of protein in 102 pregnant women during childbirth and in the cord blood of their newborn infants. A control group is established by analyzing these parameters in 10 healthy non-pregnant women on the first day of menstruation and on the day of ovulation during two consecutive menstrual cycles. The total serum zinc and serum zinc per g of protein values found were significantly higher in cord blood than in maternal blood, and the binding capacity of zinc and of zinc per g protein were significantly higher in maternal blood. There is a negative correlation between total zinc and binding capacity and total zinc and binding capacity per g protein both in maternal and in cord blood. In the control group, the values of total zinc and zinc per g protein vary from one menstrual cycle to the next and between the different phases of each cycle. Zinc-binding capacity per g protein during ovulation is significantly lower than during menstruation. These results allow us to conclude that the rise of zinc-binding capacity and zinc-binding capacity per g protein in maternal blood during childbirth, and during menstruation in the control group, can be due to the effect of progesterone. The fact that zinc-binding capacity and zinc-binding capacity per g of protein are higher in maternal blood than in cord blood would confirm the existence of mechanisms which tend to increase binding of zinc in the fetus.

Serum zinc binding capacity in pregnant women.

Zinc is an important element during the prenatal period. Since plasma zinc levels are not directly related to the nutritional status of this metal in pregnant women, we designed this study to find out if plasma protein zinc binding capacity is a more reliable parameter than total serum zinc to establish zinc nutritional status during pregnancy. We analyzed total serum zinc and zinc binding capacity in 84 normal pregnant women and in 14 nonpregnant women. Within pregnant women, samples were classified according to the gestational age. Total zinc serum was significantly decreased in pregnant women as compared to control ones. The decrease in zinc occurred slowly during the whole period of pregnancy; a negative correlation was found between serum zinc and gestational time. Protein zinc binding capacity was found to be significantly higher in pregnant women than in the control ones. This parameter was positively correlated to total serum zinc in pregnancy. We conclude that since the protein zinc binding capacity increases with advancing gestation, pregnant women take advantage of this to maintain the levels of serum-free zinc, transferable to the fetus.

[Cardiorespirography of the newborn infant at 12 hours of life]. / Cardiorrespirografía en el recién nacido a las doce horas de vida.

The introduction of the cardiorespirogram more than twenty years ago provided a useful continous noninvasive monitoring technique of heart and respiratory functions. However, there are only few studies about recording of these parameters and the topic is often partially discussed. Thus, the aim of this paper is to analyze the cardiorespirograms from newborn infants, divided into three groups: 50 normal fullterm infants, 28 preterm and 16 fullterm low birth weight infants. The age at monitoring was 12 hours. The 50 normal newborns reveal patterns similar to those described by other authors. The preterm and fullterm low birth weight infants show a significantly higher baseline with less variability and reactivity, data which lead us to bear in mind gestational age and birth weight to evaluate the cardiorespirogram.