Comparison of two-step transient evoked otoacoustic emissions (TEOAE) and automated auditory brainstem response (AABR) for universal newborn hearing screening programs.

OBJECTIVE: Both transitory auditory otoemissions (TEOAE) and automated auditory brainstem responses (AABR) are considered adequate methods for universal hearing screening. The goal of this study was to compare the results obtained with each device, applying the same screening procedure. MATERIALS AND METHODS: From 2001 to 2003, all the newborns in our health area (2454 infants) were evaluated with TEOAE (ILO92, otodynamics) and all those born from 2004 to 2006 (3117) were evaluated with AABR (AccuScreen, Fischer-Zoth). The population studied included all well newborns and those admitted to neonatal intensive care units (NICU). The first screening was normally undertaken with well babies during the first 48h of life, before hospital discharge. Infants referred from this first step underwent a second screening after hospital discharge, before they were a month old. RESULTS: The results from each study group were compared and analyzed for significant differences. TEOAE screening yielded 10.2% fail results from the first screening step; AABR gave 2.6%. In the second screening step, 2% of the newborns screened with TEOAE were referred, whereas 0.32% of those screened with AABR were referred. These differences are statistically significant. CONCLUSIONS: Although AABR screening tests involve a slightly higher cost in time and money than TEOAE, the results obtained compensate this difference. AABR gives fewer false positives and a lower referral rate; the percent of infants lost during follow-up is consequently smaller. Therefore, in our environment, universal newborn auditory screening with AABR is more effective than that with TEOAE.

Hiperbilirrubinemia neonatal. Peculiar debut del déficit de alfa-1-antitripsina / Neonatal hyperbilirrubinemia: a peculiar presentation of alpha-1-antitrypsin deficiency

La deficiencia de alfa-1-antitripsina (AlTT) es una enfermedad hereditaria que predispone a afección hepática en la infancia y a enfisema pulmonar en la edad adulta. Constituye la causa genética más común en el niño y de enfisema en el adulto; también es la que motiva más trasplantes hepáticos en niños y adolescentes. En nuestro medio presenta una prevalencia de 1 / 1.600-4.000 y una incidencia de 1 / 200 nacidos vivos. La A1TT es una glucoproteína inhibidora de proteasas séricas, codificada en el cromosoma 14 y que se hereda de forma autosómica recesiva. El fenotipo normal es el MM, y las mutaciones más frecuentes son las que asocian S y Z. Las manifestaciones clinicas más frecuentes en neonatos son la hiperbilirrubinemia conjugada, la colestasis y la hepatomegalia. Presentamos el caso de un recién nacido del sexo masculino ingresado por prematuridad e hiperbilirrubinemia directa, lo que motiva diferentes supuestos etiopatogénicos. Realizadas las pruebas sistemáticas y el estudio hepático, se determinó descenso de AlTT, y se decidió efectuar un estudio genético, en el que presentó un genotipo SS para el alelo de AlTT. La madre era SS y el padre MS, y ambos eran asintomáticos. En controles posteriores, se confirmó descenso en la cifra de AlTT, asociado a elevaciones puntuales de ésta como reactante de fase aguda, en relación con diversos procesos agudos. El interés de este caso radica en que, compartiendo la alteración genética con familiares de primer grado y, al ser éstos aparentemente asintomáticos, nuestro paciente debutó en el periodo neonatal inmediato; este hecho es peculiar en la bibliografía médica (AU)

[Results of one year's application of a universal protocol for the early detection of hearing loss in neonates]. / Resultados de aplicar durante 1 año un protocolo universal de detección precoz de la hipoacusia en neonatos.

These are the results of the first year of the application of a universal programme for the early detection of neonatal deafness. Our objective is making a diagnosis of all the unilateral or bilateral cases of hearing loss above 35 dB HL, before the age of 6 months. The detection strategy has 2 screening phases with transient evoked otoacoustic emissions (TEOAE) and one diagnostic phase with auditory brain stem response (ABR). The ABR were carried out both on those patients that failed the TEOAE screening and on the neonates with hearing loss risk factors. Of the 1,277 living newborn babies (NB), we evaluated 94%. 3.3% of the NB showed hearing loss risk factors. 90% of the NB passed the first TEOAE in both ears and, after the second TEOAE, only 2.5% of the NB reached the diagnostic phase. We provided early detection and treatment of 0.58% of cases of bilateral severe-deep hearing loss. Finally, the results obtained are examined together with the importance of planning universal hearing screening.

[Results of the application of the protocol for the early detection of hearing loss in high-risk neonates]. / Resultados de la aplicación del protocolo de detección precoz de la hipoacusia en neonatos de alto riesgo.

In coordination with the Paediatrics Department, we drew up a protocol for the "Early Detection of Hearing Loss in High-Risk Neonates" based fundamentally on the sequential application of impedanciometry and the Auditory Brain Stem Response (ABR). These are the results obtained from October 1994 to March 2001. Of the 240 children examined, we found 36 cases (15%) of severe-to-profound bilateral sensorineural hearing loss (> 60 dBs HL). 55% was associated with risk factors. However, we diagnosed only 2 of these cases of hearing loss (5.5%) using the protocol (sent by the neonatology department due to the fact that they showed hearing loss risk factors), since the rest (94.5%) came as a result of suspected hearing loss, which resulted in an average age for diagnosis of 3 years. Although the procedure used is highly sensitive and is diagnostically specific, in the light of these results, we consider it necessary to extend hearing screening to all newborn infants in order to evaluate its effectiveness in the early detection of infant deafness.

Resultados de la aplicación del protocolo de detección precoz / Results of the application of the protocol for the early detection of hearing loss in high-risk neonates

En coordinación con el Servicio de Pediatría, elaboramos un protocolo de "Detección precoz de la hipoacusia en neonatos de alto riesgo" basado fundamentalmente en la aplicación secuencias de la impedanciometría y los potenciales evocados auditivos del tronco cerebral (PEATC). Presentamos los resultados obtenidos desde octubre de 1994 a marzo de 2001. De los 240 niños estudiados, hemos encontrado 36 hipoacusias neurosensoriales severas profundas (<60 dBs HL) bilaterales (15 por ciento). El 55 por ciento se asociaba con factores de riesgo. Sin embargo, sólo diagnosticado 2 de estas hipoacusias (5,5 por ciento) gracias al protocolo (enviados por el Servicio de Neonatología por presentar factores de riesgo de hipoacusia), pues el resto (94,5 por ciento) acudieron por sospecha de hipoacusia, con lo que la edad media de diagnóstico es de 3 años. Aunque el procedimiento utilizado presenta alta sensibilidad y especificidad diagnóstica, a la vista de estos resultados, creemos necesario universalizar la exploración auditiva a todos los recién nacidos, a fin de evaluar su efectividad en la detección precoz de la sordera infantil (AU)

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Corticoides antenatales en la amenaza de parto prematuro / Antenatal corticoids in the threat of premature birth

La prematuridad es uno de los problemas más importantesde la asistencia perinatal actual, y la prevención delparto prematuro y de sus secuelas, su principal reto.La inducción farmacológica de la madurez pulmonarfetal con corticoides es la intervención que más ha cambiado el pronóstico de estos niños, ya que desde su aplicación se ha conseguido disminuir de forma notable la incidencia del síndrome de distrés respiratorio y de la mortalidad perinatal. Aunque la maduración pulmonar fetal (a través de la regulación de distintos sistemas enzimáticos en los neumocitos tipo 2) es el efecto más importante de estos fármacos, poseen también otros efectos extrapulmonares que contribuyen a la maduración de los diferentes órganos y sistemas del recién nacido. Se han utilizado diversos corticoides pero, en base a los últimos estudios, la betametasona parece el más recomendable. Es preferible administrar un ciclo único (dos dosis), ya que la utilización de múltiples ciclos no parece disminuir la morbilidad global y se ha relacionado, en diversos trabajos experimentales, con efectos adversos y restricciones en el crecimiento cerebral. Quedan todavía numerosos aspectos por esclarecer con respecto a la administración prenatal de corticoides, fundamentalmente relacionados con sus efectos a largo plazo, pero, a la luz de los conocimientos actuales, los efectos beneficiosos están fuera de toda duda

[Early diagnosis of childhood deafness: detection protocol in high-risk neonates]. / Diagnóstico precoz de hipoacusia infantil: protocolo de detección en neonatos de alto riesgo.

The protocol developed at our hospital for auditory assessment of children with risk factors for deafness is presented. The results obtained with tympanometry and auditory brainstem response (ABR) audiometry from October 1994 to March 1998 were analyzed. In 122 children, 17 cases (14%) of severe-to-profound bilateral deafness were found. Only 50% of these cases were associated with risk factors present at birth. The average age at time of diagnosis of deafness was over 3 years. In view of these results, we recommend auditory screening for all newborns as the only effective procedure for early detection of childhood deafness.

Markers of pulmonary inflammation in tracheobronchial fluid of premature infants with respiratory distress syndrome.

To asses the role of pulmonary inflammation in the outcome of preterm neonates with respiratory distress syndrome (RDS) we measured soluble intercellular adhesion molecule-1 (ICAM-1), interleukin-8 (IL-8), eosinophil cationic protein (ECP) and transforming growth factor beta-1 (TGF beta 1) in tracheobronquial lavage (TBL) fluid of 15 preterm infants; 9/15 completely recovered from RDS and 6/15 developed chronic lung disease (CLD). ICAM-1 (p: 0.001) and TGF beta 1 (p: 0.04) levels increased in TBL fluid during the first days of life. The values of ICAM-1 were correlated to the days of 0(2) and mechanical ventilation dependency. At 3 days of age, ICAM-1 levels in TBL fluid were higher in infants who later developed CLD compared to infants without CLD (24.5 vs 8.3 micrograms/ml; p: 0.02). Thereafter no significant differences were found although the CLD group had higher values. IL-8 levels showed a fall, specially from 1 to 3 days of age in children without CLD (77.0 to 41.7 ng/ml) although not significant. No difference in TGF beta 1 values were found between both groups, but the TGF beta 1 levels were lower in patients with CLD and they showed undetectable values in 8 samples. ICAM-1 is a major factor associated with airways inflammation whereas IL-8 is not a good marker during the first days of life to predict the RDS outcome. A defect of TGF beta 1 in the smallest premature infants may delay the lung repair process which occurs after tissue injury. High ICAM-1 levels and low TGF beta 1 levels in lung fluid are related to oxygen dependency at 28 days of age.

Epidemiology and prevalence of seropositivity against Borrelia burgdorferi antigen in La Rioja, Spain.

The aim of this study was to acquire a better knowledge of the epidemiology of Lyme disease. A seroepidemiologic study was made by evaluating the different levels of antibodies to Borrelia burgdorferi in a five hundred serum representative sample of a population from the province of La Rioja, Spain. Samples were randomly selected and stratified to take into account sex, age, rural or urban residence, and geographical area. All subjects included in the study were asked to fill out a questionnaire containing this information along with data concerning leisure activities, occupation, contact with animals, tick-bite and antecedent features related to Lyme disease. No statistical differences were found in relation to sex or age. The seroprevalence was very high (31.3%) in mountain areas, where Ixodes ricinus and deer are present. The risk factors associated with seropositivity were forestry and cattle-raising activities, as well as contact with animals. The best titer of antibodies associated with clinical antecedents of Lyme disease and related disorders was 1/256.

[The prevalence of Borrelia burgdorferi antibodies in a population at risk]. / Prevalencia de anticuerpos frente a la Borrelia burgdorferi en una población de riesgo.

Given the increasing interest in Lyme borreliosis in our community, and the lack of epidemiological studies in our country, we have considered convenient to analyze the presence of anti-Borrelia burgdorferi antibodies in a group of workmen who we have considered at high risk since they carry out their everyday work in an environment infested by ticks. Significant antibody titers (= or greater than 1/128) are found in 38% of cases versus 0% in the control group. The significance of these results in relation to the presence or not of compatible clinical manifestations and tick bites are studied.

Seroepidemiology of the poliovirus in Monrovia (Liberia).

Neutralizing antibodies against poliovirus have been studied in 265 persons in Monrovia (Liberia). 34% of the individuals studied possessed antibodies against the three polioviruses, 23% against two, 26% against one; the remaining 17% showed none. The three poliovirus antibodies were evenly distributed amongst the population, although there was an indication that antibodies to polio 2 occurred more commonly in females than in males. The distribution of the antibodies did not seem to be influenced by place of residence, source of drinking water or social class. Significant variations in the immunological state according to age were noted. The study of concordance and discordance of antibody titre in the blood of neonates and their mothers revealed that the lower percentage of titre concordance for polio 3 has no statistical value. The low titre in the neonates (an average for each poliovirus scarcely above 1:16) is a sign of insufficient titre in the adults. The need for a massive antipoliomyelitis campaign among infants and adult women is stressed.