RESUMEN
Ataxia-telangiectasia (AT) is a rare genetic disorder leading to neurological defects, telangiectasias, and immunodeficiency. We aimed to study the clinical and immunological features of Latin American patients with AT and analyze factors associated with mortality. Referral centers from 9 Latin American countries participated in this retrospective cohort study, and 218 patients were included. Median (IQR) ages at symptom onset and diagnosis were 1.0 (1.0-2.0) and 5.0 (3.0-8.0) years, respectively. Most patients presented recurrent airway infections, which was significantly associated with IgA deficiency. IgA deficiency was observed in 60.8% of patients and IgG deficiency in 28.6%. T- and B-lymphopenias were also present in most cases. Mean survival was 24.2 years, and Kaplan-Meier 20-year-survival rate was 52.6%, with higher mortality associated with female gender and low IgG levels. These findings suggest that immunologic status should be investigated in all patients with AT.
RESUMEN
Background: Nebulized epinephrine and hypertonic saline have been extensively studied in infants with acute bronchiolitis, with conflicting results. Aims: To evaluate the efficacy on length of stay (LOS), clinical severity scores (CSS), oxygen saturation (SaO2), and safety profile of nebulized epinephrine plus hypertonic saline (HS) in infants with acute bronchiolitis. Materials & Methods: This is a systematic review and meta-analysis. Outcomes were represented by mean differences (MD) or standard mean differences (SMD) and 95% confidence intervals (CIs) were utilized. Results: Eighteen trials were systematically selected and 16 of them contributed to the meta-analysis (1756 patients). Overall, a modest but significant positive impact was observed of the combination therapy on LOS (MD of -0.35 days, 95% CI -0.62 to -0.08, p = 0.01, I 2 = 91%). Stratification by time of CSS assessment unveiled positive results in favor of the combination therapy in CSS assessed 48 and 72 h after the admission (SMD of -0.35, 95% CI -0.62 to -0.09, p = 0.008, I 2 = 41% and SMD of -0.27, 95% CI -0.50 to -0.04, p = 0.02, I 2 = 0%, respectively). No difference in SaO2 was observed. Additional data showed a consistent safety profile, with a low rate of adverse events (1%), most of them mild and transient. Conclusion: Low-quality evidence from this systematic review suggests that nebulized epinephrine plus HS may be considered as a safe and efficient therapy for decreasing LOS and CSS in infants with acute bronchiolitis, especially in those who require hospitalization for more than 48 h.
RESUMEN
We aimed to compare Gleason score and tumor laterality between transrectal ultrasound-guided biopsy of the prostate (TRUSBX) and radical prostatectomy (RP). Some factors that could cause a discrepancy in results between these two procedures were also evaluated. Among the 318 cases reviewed, 191 cases were selected for inclusion in this comparative study. We divided the patients into two groups using the Gleason score: an intermediate/high-grade group (≥7) and a low-grade group (<6). Exploratory analyses were conducted for comparisons between groups. We also performed comparisons between TRUSBX and RP for tumor laterality. TRUSBX overestimated 6% and underestimated 24% cases in comparison with RP for Gleason score, and overestimated 2.6% and underestimated 46% cases compared with RP for tumor laterality. Biopsy specimens were slightly smaller in TRUSBX cases with underestimated tumor laterality (P < 0.05), and no relationship between the biopsy specimen size and underestimated Gleason score in TRUSBX was found. Prostatic volume showed no statistical correlation with the likelihood of under or overestimation (P > 0.05). Thus, our study showed that TRUSBX has a high likelihood of underestimating both the Gleason score and tumor laterality in prostate cancer (PCa). The size of the fragment appears to be an important factor influencing the likelihood of laterality underestimation and Gleason score overestimation via TRUSBX. Due to the high likelihood of underestimation of the Gleason score and tumor laterality by 12-core prostate biopsy, we conclude that this type of biopsy should not be used alone to guide therapy in PCa.