Vulvar embryonal rhabdomyosarcoma: A case report

Embryonal rhabdomyosarcoma (ERMS) is a rare malignant neoplasm found in both male and female children. It typically presents as a protruding vaginal mass with vaginal bleeding in girls less than 8 years of age. It is often seen as the sarcoma botryoides histologic sub type. This report represents the first case study of ERMS presenting as a vulvar mass. The article further explores the etiologies of pediatric vulvar masses, including ERMS, and the suggested diagnostic and treatment modalities.     

The effect of rate stress on the auditory brainstem response in autism: A preliminary report

Abstract

Efforts to correlate peripheral neurophysiologic function with perceptional deficits seen in autistic disorder (AD) have resulted in mixed findings, reflecting the high degree of heterogeneity observed in these individuals. We used the auditory brainstem response to study the effect of stress (high click presentation rate) on the auditory system in 20 children with AD (7–13 years) and 20 age-matched typically developing (TD) children. We report latency prolongations in children with AD vs. TD at waves I, III, and V that differed by ear of presentation: overall, left ear showed significant prolongations by group while right ear did not. The ‘stressed’ condition produced prolongations for both groups at each wave. At wave V, children with AD showed significant prolongations vs. TD, particularly for the right ear. For children with AD, wave V latency prolongations corresponded to language outcome as measured by VIQ, with longer prolongations associating with lower VIQ. Preliminary results provide evidence for reduced synaptic efficiency in auditory pathways in children with AD, which may form the neural bases for sensory reactivity and language impairment.

Sumario

Los esfuerzos para correlacionar la función neurofisiológica periférica con los déficits de percepción que se observan en el trastorno autístico (AD) han producido hallazgos mixtos que reflejan el alto grado de heterogeneidad que se aprecia en estos individuos. Usamos la respuesta auditiva de tallo cerebral para estudiar el efecto de tasas elevadas de presentación de clicks al sistema auditivo de 20 niños con AD (7-13 años) y de otros 20, pareados en edad, con desarrollo típico (TD). Reportamos latencias prolongadas en niños con AD vs. TD en las ondas I, III y V que difirieron según el oído estimulado. Globalmente, el oído izquierdo mostró prolongaciones significativas por grupo, mientras que no fue así con el oído derecho. La condición de “alta estimulación” produjo prolongaciones en ambos grupos y con cada una de las ondas. En la onda V, los niños con AD mostraron prolongaciones significativas vs. los niños TD, particularmente con el oído derecho. En niños con AD, las prolongaciones de latencia de la onda V, correspondieron a resultados lingüísticos medidos con VIQ, con prolongaciones aun mayores que se asociaron con niveles VIQ más bajos. Los resultados preliminares proporcionan evidencias de eficiencia sináptica reducida en las vías auditivas de niños con AD, que pueden ser el fundamento neural de la reactividad sensorial y de los impedimentos del lenguaje.     

Congenital aural atresia and stenosis: Surgery strategies and long-term results

Abstract

Objective: To compare the patients who underwent surgery for congenital aural atresia (CAA) with congenital aural stenosis (CAS) for the stability of hearing results and complications during long-term follow-up. Design: Retrospective review. Study sample: Seventy-five CAA patients and fifty CAS patients who underwent congenital meatoplasty with canalplasty and tympanoplasty between 2007 and 2012. Results: Paired comparison analyses detected no significant difference in preoperative ABG but significant changes in postoperative ABG, ΔABG, the number of ABG < 30 dB and ABG < 10 dB between CAA and CAS. Complications such as postoperative stenosis, bony regrowth, external aural canal (EAC) infection, EAC eczema, total deaf, and lateralization of the tympanic membrane (TM) were observed in 61.3% of patients with CAA and 20% of patients with CAS. Chi square test detected significant differences in complications between patients with CAA and CAS (χ² = 20.73, p < 0.01). Conclusion: Meatoplasty with canalplasty and tympanoplasty in individuals with CAS can yield reliable and lasting positive hearing results with a low incidence of severe complications. The existence and preoperative condition of patients’ TM and EAC skin helped improve hearing results and decrease the incidence of complications. However, the final hearing results and complications required stricter indications for CAA patients.     

Silencing of DNA repair sensitizes pediatric brain tumor cells to γ-irradiation using gold nanoparticles

We present a nanoparticle (NP)-mediated delivery vehicle that effectively carries and protects siRNA in pediatric ependymoma (EP) and medulloblastoma (MB) cells. The delivery vehicle consists of gold NPs coated with a polymeric shell comprising polyethylene glycol (PG), chitosan and polyethyleneimine (Au-CP-PEI). NPs loaded with siRNA knocked down Ape1 expression by over 75% in both MB and EP cells. Further, this reduction in Ape1 expression is associated with an increase in DNA damage after irradiation. The results indicate that NP-associated delivery of siApe1 is a feasible approach to circumventing pediatric brain tumor resistance to radiation therapy.