Pharmacoepidemiology of Drug Exposure in Intubated and Non-Intubated Preterm Infants With Severe Bronchopulmonary Dysplasia.

Background: Infants with severe bronchopulmonary dysplasia (BPD) are commonly treated with off-label drugs due to lack of approved therapies. To prioritize drugs for rigorous efficacy and safety testing, it is important to describe exposure patterns in this population. Objective: Our objective was to compare rates of drug exposure between preterm infants with severe bronchopulmonary dysplasia based on respiratory support status at or beyond 36 weeks post-menstrual age. Methods: A cross-sectional cohort study was performed on October 29, 2019. Preterm infants with severe BPD were eligible and details of respiratory support and drug therapy were recorded. Wilcoxon paired signed rank test was used to compare continuous variables between the invasive and non-invasive groups. Fisher's exact test was used to compare binary variables by respiratory support status. Results: 187 infants were eligible for the study at 16 sites. Diuretics were the drug class that most subjects were receiving on the day of study comprising 54% of the entire cohort, followed by inhaled steroids (47%) and short-acting bronchodilators (42%). Infants who were invasively ventilated (verses on non-invasive support) were significantly more likely to be receiving diuretics (p 0.013), short-acting bronchodilators (p < 0.01), long-acting bronchodilators (p < 0.01), systemic steroids (p < 0.01), systemic pulmonary hypertension drugs (p < 0.01), and inhaled nitric oxide (p < 0.01). Conclusion: Infant with severe BPD, especially those who remain on invasive ventilation at 36 weeks, are routinely exposed to multiple drug classes despite insufficient pharmacokinetic, safety, and efficacy evaluations. This study helps prioritize sub-populations, drugs and drug classes for future study.

Pseudo-glutarylcarnitinaemia in medium-chain acyl-CoA dehydrogenase deficiency detected by tandem mass spectrometry newborn screening.

As well as characteristic increases in C(8) carnitine, dried blood spot samples from 11 newborns with medium-chain acyl-CoA dehydrogenase deficiency detected by tandem mass spectrometry screening using butyl esters showed apparent increases in glutarylcarnitine (m / z 388 signals). In four of the newborns in which it was measured, apparent increases in malonylcarnitine (m / z 360) were also detected. It was shown that the apparent increases were caused by interfering acylcarnitines, putatively identified as hydroxyoctanoylcarnitine and hydroxydecanoylcarnitine, respectively, using alternative derivatives for tandem mass spectrometry. Levels of the two abnormal carnitines correlated with C(8) carnitine levels and normalized with repeat testing in 10 cases. These results indicated that the abnormal carnitines were significantly elevated only during periods of increased fatty acid catabolism, as may occur in the immediate postnatal period.

Monitorización electroencefalográfica mediante video prolongado: experiencia del Hospital Militar de Santiago: 1991-1996 / Prolonged electroencephalography and video monitoring: an experience at Hospital Militar de Santiago: 1991-1996

Background: Cerebral activity must be registered for prolonged periods in certain clinical situations that are not resolved with conventional electroencephalography. Aim: To report the experience with prolonged electroencephalographic and video monitoring at a Neurology Department, of a Military hospital in Santiago. Patients and methods: A retrospective analysis of patients referred for continuous electroencephalographic and video monitoring between 1991 and 1996. Three hundred thirty six patients, aged 3 months to 60 years old, were studied in the period and in 244, there was information about the diagnosis, treatment and evolution. Results: Monitoring was performed in an outpatient basis in 84 percent of subjects and lasted between 2 and 7 hours. One hundred ten patients were epileptics, 77 patients had a suspicion of epilepsy, 13 patients had possible pseudoseizures and 33 patients had miscellaneous diagnoses. In 154 patients, electroencephalographs recorded during wakefulness, somnolence and spontaneous dream, were normal. Intercritical recordings with epileptic activity were obtained in 76 patients and in 30 of these, critical epileptic activity was also recorded, not always associated to clinical manifestations. Unspecific electroencephalographic alterations were recorded in 14 patients. Conclusions: Prolonged electroencephalographic and video monitoring can be useful for patients with complex neurological problems

Evaluación del paciente en como en UCI / Evaluation of the coma patient in ICU

El paciente con alteración de conciencia o en coma constituye una urgencia médica y obliga al médico tratante a seguir un procedimiento lógico y sistemático de estudio, el cual debe estar basado en principios anatómicos y fisiológicos. En la primera parte de esta exposición se analizan los elementos básicos fisiológicos que mantienen el estado de conciencia normal, y se comentan los elementos más destacados del metabolismo cerebral. A continuación se analizan desde el punto de vista clínico las diferentes formas en que se puede alterar la conciencia, para luego comentar los elementos más relevantes del examen clínico neurológico de un paciente en coma, y los diversos procesos patológicos que pueden inducir este estado. Para finalizar se describen las diferentes formas que pueden utilizarse en un paciente en la UCI para monitorizar la función cerebral, detallando la utilidad del examen neurológico y de algunos procedimientos neurofisiológicos, como son el electroencefalograma y los potenciales evocados en un grupo de pacientes estudiados en la UCI del Hospital Militar, portadores de Encefalopatía hipóxica e isquémica

Use of the argon laser in the treatment of malleus fixation.

This case demonstrates the use of the argon laser for ossicular mobilization. A preoperative audiologic evaluation revealed a severe conductive hearing loss, with a maximum air-bone gap. Since normal drilling procedures would result in a sensorineural hearing loss, the argon laser was chosen to remove a bony spur connecting the malleus to the posterior canal wall. When using the argon laser, no disarticulation of the incus and stapes is required. Postoperative audiologic evaluation revealed normal hearing sensitivity bilaterally.

Actividad lenta posterior en el electroencefalograma / Posterior slow rhythmic activity in the EEG

Se analizan 7000 EEG anormales efectuados en nuestro laboratorio entre los años 1968 y 1986, encontrando actividad lenta posterior en 4,95% de estos registros. Se reconocieron dos tipos de actividad lenta posterior, uno constituido por ritmos monomorfos posteriores de frecuencias entre 2 y 5 cps y otro por grafoelementos aislados hipersincrónicos con la configuración de puntas lentas o puntas lentas-ondas lentas. Ambos tipos de actividad lenta se observaron sólo en individuos menores de 15 años, por lo que se trata de un tipo de actividad dependiente de la edad. Ninguno de estos tipos de actividad lenta posterior es específica, asociándose a muy diversas manifestaciones clínicas inclusive, de acuerdo a la literatura , podrían observarse en sujetos normales. Sin embargo, la actividad lenta monomorfa a 3-3.5 cps se observa mas en pacientes con antecedentes de crisis convulsivas epilépticas y el grupo entre 4 y 5 cps es el que mas se correlaciona con trastornos conductuales o cognitivos. Cuando estos dos tipos de actividad lenta monomorfa posterior, cuya presencia supondría una disposición convulsivante, se asocian a crisis convulsivas epilépticas, se supone un buen pronóstico, ya que en general se la observa en pacientes que han dejado de convulsionar o que muestran ataques muy aislados

Atrofias olivopontocerebelosas: análisis clínico de 11 casos / Olivopontocerebellar atrophies: clinical analysis of 11 cases

Se presentan 11 pacientes portadores de una atrofia olivo ponto cerebelosa, describiéndose en detalle la sintomatología neurológica y su evolución. Las diversas formas sindromáticas se analizan en relación a los hallazgos de la Tomografía Axial Computada de cerebro, apreciándose que todos nuestros casos tienen evidencia tomográfica de atrofia del vermis cerebeloso, hemisferios cerebelosos y protuberancia, independientemente de la forma sindromática que presentan. Finalmente se comenta la relación de este cuadro con el Síndrome de Shy-Drager y la degeneración striato-nigral.