Citomegalovirus congénito: ¿El diagnóstico y el tratamiento precoz pueden revertir una discapacidad permanente? / Congenital cytomegalovirus: Can early diagnosis and treatment revert permanent disability?

Introducción: La infección congénita por citomegalovirus (CMV) es la causa más frecuente de hipoacusia neurosensorial (HNS) no genética en países desarrollados. La incidencia de HNS en los lactantes sintomáticos oscila entre el 30 y el 65%. Objetivos: Describir las formas de presentación clínica de la infección por CMV congénita en pacientes sintomáticos y la evolución auditiva en los pacientes tratados con antivirales y aquellos sin tratamiento. Diseño: Estudio retrospectivo, descriptivo, observacional y longitudinal. Población: Se incluyeron niños, menores de 2 meses, con CMV congénito (confirmado por viruria positiva con método de PCR), sintomáticos, internados en la Unidad de Neonatología, desde el año 2005 al 2013. Método: Diagnóstico y seguimiento auditivo utilizando otoemisiones acústicas (OEA), potenciales evocados auditivos de tronco cerebral (PEAT) y audiometría (AT) según edad e indicación en cada caso en particular. El tratamiento antiviral se realizó con ganciclovir (GCV) y/o valganciclovir (VGCV). Resultados: Clínicamente se estudiaron 16 pacientes con diagnóstico de CMV congénito sintomáticos. Se excluyeron tres. Se describen los motivos de internación más frecuentes. Para el estudio y seguimiento audiológico los pacientes se dividieron en dos grupos de acuerdo a que recibieran o no tratamiento: A: no recibieron tratamiento antiviral (n: 5) y B: recibieron tratamiento antiviral (n: 8). En los pacientes que recibieron tratamiento, las secuelas auditivas fueron menores y en dos de los casos se produjo una mejoría importante en la audición. Conclusiones: El tratamiento de neonatos con infección congénita por CMV con GCV y/o VGCV ofrece resultados alentadores en la prevención de la hipoacusia (AU)

Introduction: Congenital cytomegalovirus (CMV) infection is the most common cause of non-genetic sensorineural hearing loss (SNH) in developed countries. The incidence of SNH in symptomatic infants ranges between 30% and 65%. Objectives: To describe different forms of clinical presentation of congenital CMV infection in symptomatic patients as well as outcome in patients treated with antiviral drugs and those in whom treatment was withheld. Study design: A retrospective, longitudinal, observational, descriptive study. Population: Symptomatic infants younger than 2 months of life with congenital CMV infection (confirmed by positive viruria using PCR), admitted to the Neonatology Unit between 2005 and 2013. Method: Diagnosis and audiological follow-up with otoacoustic emissions (OAE), brainstem auditory evoked potentials (BAEP) and audiometry (AT) according to age and indication for the individual patient. Antiviral treatment consisted of ganciclovir (GCV) and/or valganciclovir (VGCV). Results: Sixteen patients with symptomatic congenital CMV infection were clinically studied. Three were excluded. Main reasons for admission are described. For evaluation and audiological follow-up the patients were divided into two groups according to whether or not they received treatment. Group A: did not receive antiviral treatment (n: 5) and B: received antiviral treatment (n: 8). In patients that received treatment hearing sequelae were less severe and in two patients significant hearing improvement was observed. Conclusions: Treatment with GCV and/or VGCV of neonates with congenital CMV was found to have promising results for the prevention of hearing loss (AU)

Evaluación del cumplimiento de la pesquisa auditiva en pacientes ingresados al Hospital Garrahan entre marzo de 2012-2013 / Evaluation of compliance with hearing screening of patients admitted to Garrahan Hospital from March 2012-2013

Objetivos: 1) Evaluar el cumplimiento del Programa Nacional de Detección Temprana y Atención de la Hipoacusia. 2) Correlacionar los resultados con la presencia o ausencia de cobertura social. 3) Evaluar la información brindada a los padres en contenido y la demora en efectuar la prueba. Métodos: estudio observacional y transversal. Se incluyeron todos los pacientes entre 1/03/12 y 31/03/13 que consultaron espontáneamente en el Hospital Garrahan y requirieron hospitalización en la sala de internación conjunta del Área de Neonatología. Se analizaron los datos obtenidos por interrogatorio, historia clínica y encuesta realizada a los padres. Se empleó estadística descriptiva y Chi2 según necesidad. Resultados: Ingresaron 153 pacientes. Mediana de edad: 21 días (16-30). Cobertura social 25% (38/153). 30% (46/153) tenían efectuada OEA. 30 tenían turno emitido, 44 habían recibido la orden médica y 33 de los 107 no evaluados no tenían ningún tipo de vinculación con la prueba. En el 34% (13/38) de los casos con obra social vs el 17% (20/115) de los casos sin cobertura se observó incumplimiento total (p 0,02).Con respecto a la información de los padres 35 /153 pacientes (23%) habían sido informados sobre la importancia de esta prueba. Discusión: Son escasos los pacientes evaluados previo al egreso de la maternidad o dentro del primer mes de vida. Poseer cobertura social no facilita el cumplimiento de la ley. Los padres carecen de información sobre la importancia de la misma (AU)

Aims: 1) To evaluate compliance with the National Program for Early Detection and Care of Hearing Loss. 2) To correlate results with presence or absence of health insurance coverage. 3) To evaluate the quality of information provided to the parents and the delay in taking the test. Methods: An observational and cross-sectional study. All patients who consulted spontaneously at Hospital Garrahan between 1/03/12 and 31/03/13 and who required admission to the neonatology ward were included. Data obtained from the clinical chart and from an interview and questionnaire administered to the parents were analyzed. Depending on the data descriptive statistics and chi-square test were used. Results: 153 patients were admitted. Mean age was 21 days (16-30). Health care coverage: 25% (38/153). Acoustic otoemissions (AOE) were performed in 30% (46/153). Overall, 30 had been called for an appointment, 44 had received the medical indication, and 33 of 107 infants who were not evaluated had not been in touch with the test. In 34% (13/38) of the cases with health insurance coverage vs 17% (20/115) without total incompliance was observed (p 0.02). Regarding information for parents, 35/153 (23%) had been informed on the importance of the test. Conclusion: Few patients are screened on discharge from the maternity clinic or within the first month of life. Health insurance coverage does not facilitate compliance with the law. Parents do not receive information on the importance of the test (AU)

Programa de diagnóstico y tratamiento fetal. Estudio de cohorte sobre su efectividad en la evolución de neonatos con patologías quirúrgicas / Program for prenatal diagnosis and treatment: A cohort study on effectivity on the outcome of newborns with surgical pathologies

Introducción. El diagnóstico prenatal de las malformaciones congénitas (MC) permite optimizar el cuidado perinatal. Al Hospital Garrahan (HG) ingresan recién nacidos (RN) con MC para tratamiento quirúrgico. Desde el año 2008 funciona el programa de diagnostico y tratamiento fetal (PDTF) para optimizar el cuidado prenatal y perinatal de RN con MC. El objetivo del estudio es evaluar el impacto de la derivación prenatal en RN que ingresan a la unidad de cuidados intensivos neonatales (UCIN) del HG por MC seleccionadas. Población y Métodos: estudio observacional analítico y comparativo entre grupos, sobre condición de ingreso y evolución de RN con gastrosquisis (GTQ), mielomeningocele (MMC) y hernia diafragmática (HD) y grado de stress parental, según ingreso a UCIN por derivación prenatal o postnatal. Se realizó análisis bivariado, descriptivo y comparativo de indicadores generales y especiales de cuidado. Resultados: Se incluyeron 164 RN (44 con derivación prenatal a través del PDTF). Este grupo presentó: mejor control del embarazo (93% vs 66%, p: 0.04), menor edad gestacional al diagnóstico (24s vs 33s p=0.0006) y mayor tasa de cesárea electiva (95 vs 47%, p=0.0001). Los RN tuvieron menor necesidad de reanimación e ingresaron más tempranamente a la Unidad (mediana 4hs vs 10hs, p=0,004). Hubo menor stress parental en relación al hijo (17 vs 40%). Los RN con GTQ ingresaron mejor curados y se alimentaron por vía enteral más rápidamente. Conclusión: Los RN con MC derivados prenatalmente a través del PDTF, ingresan precozmente y presentaron mejores estrategias de cuidado que los ingresados por derivación habitual (AU)

Introduction. Prenatal diagnosis of congenital malformations (CM) improve perinatal care. At the Garrahan Hospital (GH) newborns (NB) with CM are admitted for surgical treatment. Since 2008 a program for prenatal diagnosis and treatment (PDT) has been in place to optimize prenatal and perinatal care of NB with CM. The aim of this study was to assess the impact of prenatal referral of NB that are admitted to the GH for selected CM. Population and Methods: An observational, analytical, and between-group comparative study was conducted on the status on admission and outcome in NB with gastroschisis (GS), myelomeningocele (MMC), and diaphragmatic hernia (DH) and degree of parental stress, according to NICU admission after prenatal or postnatal referral. Bivariate, descriptive and comparative analysis of general and specific markers of care was performed. Results: 164 NB were included (44 were prenatally referred through the PDT program). The latter group presented with better pregnancy control (93% vs 66%, p: 0.04), younger gestational age at diagnosis (24w vs 33w p=0.0006), and higher rate of elective cesarean section (95 vs 47%, p=0.0001). This group of NB needed less reanimation and were admitted to the NICU earlier (∑4hs vs 10hs, p=0.004). Parental child-related stress was less (17 vs 40%). NB with GS had a better surgical outcome and were started on enteral feeding earlier. Conclusion: NB with CM that were prenatally referred through the PDT program, were admitted earlier and could be managed with better strategies than those who were conventionally referred (AU)

[Hydrocortisone for the treatment of refractory hypotension: a randomized controlled trial]. / Hidrocortisona para el tratamiento de hipotensión refractaria: ensayo clínico controlado y aleatorizado.

INTRODUCTION: Systemic hypotension is a common sign in critically sick infants. Several studies have suggested that the use of short series of corticosteroids increases arterial blood pressure and reduces the inotropic support needs in preterm neonates with hypotension. There are a small number of reports on the use of hydrocortisone (HC) for the treatment of refractory hypotension in infants. MATERIAL AND METHODS: To assess the effectiveness of hydrocortisone in the reduction of inotropic support in infants with refractory hypotension. POPULATION: infants who required dopamine ≥ 14 µg/kg/min and/or epinephrine. DESIGN: prospective, controlled, randomized, double blind trial with placebo. INTERVENTION HC: 2.5mg/kg every 12 hours, for 48 hours intravenously (intervention group [IG]); placebo: isotonic saline 1.25 ml/kg/doses intravenously (placebo group [PG]) every 12 hours, for 48 hours. Randomization was performed in blocks with blind assignment. RESULTS: A total of 50 infants with refractory systemic hypotension were prospectively recruited. Patient characteristics were similar in both groups. Requirements for inotropic support at 48 hrs were achieved in 60%, of the IG versus 24% of the PG (P=.009, RR: 2.5, 95% CI, 1.16-5.38). A significant association was observed between the administration of HC in infants treated with epinephrine and the presence of hyperglycemia (P =.008). CONCLUSION: In patients with refractory hypotension hydrocortisone administration reduced the need for inotropic support. Further studies with a greater number of patients are needed to confirm the effectiveness of HC as a therapeutic tool in these infants.

Neonatology in the emerging countries: the strategies and health-economics challenges related to prevention of neonatal and infant infections.

The prevalence of neonatal and infant infections is higher in emerging countries when compared to the developed world. Major factors associated to this increased frequency include the scarcity of trained health personnel, overcrowding of the neonatal units, late onset and slow advance of feeding, use of formula instead of breastfeeding, failure to comply with handwashing recommendations, and excessive use of antibiotics, resulting in the emergence of resistant strains. Infants discharged home frequently share rooms with a large number of siblings and other cohabitants, increasing the risk of infection by respiratory viruses. Several strategies are described that could decrease these serious problems which impact increasing significantly neonatal and infant mortality rates in developing countries.

Impacto de la inmunoprofilaxis en una población de lactantes prematuros con y sin displasia broncopulmonar / Impact of immunoprophylaxis in a population of preterm infants with and without bronchopulmonary dysplasia

Con el fin de objetivar el impacto de la infección por Virus Sincicial Respiratorio (VSR) se analizó una cohorte de prematuros (PT) atendidos en nuestro consultorio de seguimiento de alto riesgo (CSAR) entre los años 1998 y 2004. A par tir del año 2006 el Hospital incorpora el anticuerpo monoclonal Palivizumab (PVZ) para la profilaxis de VSR en esta población vulnerable. Objetivo: analizar el impacto de la profilaxis con PVZ en la población de lactantes PT con y sin displasia broncopulmonar (DBP). Población: PT atendidos en el CSAR del Hospital Garrahan entre los años 1998- 2004 y 2006-2010. Diseño y Método: estudio longitudinal y observacional mediante comparación de riesgo de hospitalización por VSR entre la cohor te retrospectiva que no recibió PVZ (SP) vs una cohor te prospectiva que recibió profilaxis (CP) Resultados: SP: 154 pacientes, CP: 99 pacientes. La tasa de internación en el grupo SP fue 26% (21% en el grupo sin DBP y 28% para el grupo con DBP); la tasa de internación en el grupo CP disminuyó a 6% (5% y 6,3% para los PT sin y con DBP). Estas diferencias resultaron significativas (RR 0,22 IC95% 0,10 a 0,51); el NNT (número necesario a tratar) fue de 5. Conclusiones: la incorporación de inmunoprofilaxis en este grupo de riesgo produjo un impor tante descenso de la tasa de internación. El impacto en nuestra población fue mayor que el repor tado, incluso en la población de pacientes mas graves como los lactantes con DBP...

[Supernumerary clavicle: a case report]. / Clavicula supernumeraria: reporte de un caso.

We report a case of left sided supernumerary clavicle in a male child, 3 years old with impossibility to the flexion and extension of the neck and lateralization since birth, in the 3D CAT SCAN we could appreciate the presence of bone tissue with italic S shape that was articulated with the jaw and the left sternun clavicle region. Previous evaluation from the rehabilitation service the patient underwent the removal of the lesion. The pathology study confirmed that this piece was osseous tissue with distal joints surfaces and central bone marrow.

Indice de ventilación oxigenación (IVO) como predictor de mortalidad en la hernia diafragmática congénita en recién nacidos / Ventilation-oxygenation index as apredictor for outcome in newborns with congenital diaphragmatic hernias)

Con el objetivo de buscar predictores de gravedad en los recién nacidos (RN) con hernia diafragmática congénita(HDC) de alto riesgo, se evaluaron en 25 recién nacidos los siguientes índices : 1) diferencia alveoloarterial de oxígeno (AaDO2), 2) índice ventilatorio(IV), 3) mejor Pa02 postductal,4)mejor PaCO2 postductal, 5) índice de ventilación oxigenación (IVO). Durante el período pre operatorio, la mejor PO2, la mejor POC2 y la AaDO2 no permitieron diferenciar la población de RN que luego vivieron de los que fallecieron.En cambio, el IV y el IVO fueron significativamente diferentes entre ambas poblaciones (p 0.001). El IVO: PO2/IV x 100 contiene en su fórmula datos del respirador(IV=frecuencia respiratoria y presión en la vía aérea), y del paciente (PO2). Por lo tanto permite obtener datos independientes de la modalidad ventilatoria de cada institución y podría contribuir a determinar el mejor momento quirúrgico, luego del período de estabilización preoperatoria

Indice de ventilación oxigenación (IVO) como predictor de mortalidad en la hernia diafragmática congénita en recién nacidos / Ventilation-oxygenation index as apredictor for outcome in newborns with congenital diaphragmatic hernias)

Con el objetivo de buscar predictores de gravedad en los recién nacidos (RN) con hernia diafragmática congénita(HDC) de alto riesgo, se evaluaron en 25 recién nacidos los siguientes índices : 1) diferencia alveoloarterial de oxígeno (AaDO2), 2) índice ventilatorio(IV), 3) mejor Pa02 postductal,4)mejor PaCO2 postductal, 5) índice de ventilación oxigenación (IVO). Durante el período pre operatorio, la mejor PO2, la mejor POC2 y la AaDO2 no permitieron diferenciar la población de RN que luego vivieron de los que fallecieron.En cambio, el IV y el IVO fueron significativamente diferentes entre ambas poblaciones (p 0.001). El IVO: PO2/IV x 100 contiene en su fórmula datos del respirador(IV=frecuencia respiratoria y presión en la vía aérea), y del paciente (PO2). Por lo tanto permite obtener datos independientes de la modalidad ventilatoria de cada institución y podría contribuir a determinar el mejor momento quirúrgico, luego del período de estabilización preoperatoria

Imperforate anus, delayed presentation in a 7-year-old girl.

Most of the cases of imperforate anus should be discovered at birth, during the initial physical examination. This case represents the status of Third World rural perinatal care, wherein delivery of an infant by a midwife in poverty stricken area of mountainous Jipijapa, Ecuador, failed to reveal the anomaly during the cursory examination. Moreover, the cultural taboos of the Jipijapan agricultural community further delayed the diagnosis. A 7-year-old girl with imperforate anus presenting with a rectovaginal fistula is reported. Specific findings in the case, therapy, and an approach to the diagnosis are discussed.

Lack of relationship between the true airway pressure and the pressure displayed with an infant ventilator.

OBJECTIVE: To establish if the pressure indicated in the manometer of an infant ventilator (IV 100B, Sechrist, Anaheim, CA) reflects the true pressure delivered to the proximal airway during mechanical ventilation in the neonatal ICU. DESIGN: With approval of our Institutional Research Board, data were collected prospectively. Peak inspiratory pressure and end-expiratory pressure were measured at the "Y" piece of the breathing tubing. Pressure readings from the conventional ventilator's manometer were compared with simultaneously obtained measurements using an electronic monitor. SETTING: This study was conducted in a 45-bed neonatal ICU, admitting 700 to 750 newborns per year. PATIENTS: Twelve neonates who required mechanical ventilation were included in the study. INTERVENTIONS: Specific interventions were not made by study design. Measurements routinely obtained were compared. MEASUREMENTS AND MAIN RESULTS: Two hundred seventy-five simultaneous measurements of peak inspiratory pressure and positive end-expiratory pressure were compared. Peak inspiratory pressure values were higher with the electronic monitor in 273 (99%) of 275 measurements and the mean of the differences between the electronic monitor and ventilator's manometer was statistically significant (p less than .001). For positive end-expiratory pressure measurements, values indicated by the electronic monitor were lower in 152 (55%) of 275 determinations, equal in 65 (23%), and higher in 58 (21%) determinations. Percent variations between methods ranged from 0% to 140% for peak inspiratory pressures and from 0% to 500% for positive end-expiratory pressure. CONCLUSIONS: These data demonstrate that it is impossible to know the true pressure delivered to the proximal airway of a neonate during mechanical ventilation by observing the ventilator pressure manometer. The manometer readings consistently underestimate the true peak inspiratory pressure values and are very unpredictable regarding positive end-expiratory pressure values. These findings support the use of other methods to monitor the proximal airway pressure besides the ventilator's manometer in the neonatal ICU. Furthermore, mean airway pressure should not be calculated from the pressure readings obtained from the tested ventilator's manometer.

Early diagnosis of ABO haemolytic disease of the newborn.

To assess the usefulness of cord blood tests in diagnosing ABO-haemolytic disease of the newborn (ABO-HDN), 132 term, adequate for gestational age (AGA) neonates were evaluated. The tests studied and their significant results were: quantitative elution test (greater than or equal to 1/16), direct Coombs test (positive), bilirubin concentration (greater than or equal to 4 mg/dl). In none of the 56 O+ newborn infants delivered by O+ women were the results of any test positive. Of the 76 A+ and B+ newborn infants delivered by O+ women, 17 (22%) developed ABO-HDN. When the combined result of any two tests was positive, the sensitivity, the specificity and the positive predictive accuracy for the diagnosis of ABO-HDN was higher than for any one of the isolated tests. The probability that ABO-HDN was present when the results of at least two cord blood tests were positive was 70%, and the probability that ABO-HDN was not present when less than two cord blood tests gave positive results was 93%. It is suggested that the combination of quantitative elution test, bilirubin concentration and direct Coombs test in the cord blood is useful for an early diagnosis of ABO-HDN.