Short Cilia, Immunodeficiency, and Cystic Fibrosis in a Mother-Daughter Pair.

Patients with chronic rhinosinusitis (CRS) that is refractory to maximal medical and surgical therapy should be evaluated for other primary conditions. Cystic fibrosis (CF), primary immunodeficiency (PID), and primary ciliary dyskinesia (PCD) are potential risk factors for refractory CRS. These conditions present with variable disease severity and diagnosis may be delayed into adulthood. We report a case of a mother-daughter pair with CRS refractory to maximal medical management. The patients were further evaluated and found to have features consistent with CF, PID, and PCD. All 3 are rare disorders and thought to cause CRS in isolation. Patients with refractory CRS should be further evaluated to identify alternative diagnoses and ensure proper management. Refractory CRS may be multifactorial, with different risk factors simultaneously contributing to its persistence.

Retrospective Cohort Study on the Impact of the COVID-19 Pandemic on Pregnancy Outcomes for Women Living With HIV in British Columbia.

BACKGROUND: For pregnant women living with HIV (WLWH), engagement in care is crucial to maternal health and reducing the risk of perinatal transmission. To date, there have been no studies in Canada examining the impact of the COVID-19 pandemic on pregnant WLWH. METHODS: This was a retrospective cohort study assessing the impact of the pandemic on perinatal outcomes for pregnant WLWH using data from the Perinatal HIV Surveillance Program in British Columbia, Canada. We compared maternal characteristics, pregnancy outcomes, and clinical indicators related to engagement with care between a prepandemic (January 2017-March 2020) and pandemic cohort (March 2020-December 2022). We investigated preterm birth rates with explanatory variables using logistic regression analysis. RESULTS: The prepandemic cohort (n = 87) had a significantly (P < 0.05) lower gestational age at the first antenatal encounter (9.0 vs 11.8) and lower rates of preterm births compared with the pandemic cohort (n = 56; 15% vs 37%). Adjusted odds of preterm birth increased with the presence of substance use in pregnancy (aOR = 10.45, 95% confidence interval: 2.19 to 49.94) in WLWH. There were 2 cases of perinatal transmission of HIV in the pandemic cohort, whereas the prepandemic cohort had none. CONCLUSIONS: The pandemic had pronounced effects on pregnant WLWH and their infants in British Columbia including higher rates of preterm birth and higher gestational age at the first antenatal encounter. The nonstatistically significant increase in perinatal transmission rates is of high clinical importance.

Real-World Adverse Events After Type 2 Biologic use in Chronic Rhinosinusitis with Nasal Polyps.

OBJECTIVE: To investigate the frequency and nature of adverse events related to type 2 biologic use in patients with chronic rhinosinusitis with nasal polyps (CRSwNP), including dupilumab and mepolizumab. METHODS: This is a single-institution retrospective study of real-world patient data. Patients were included if they have a diagnosis of CRSwNP, have undergone at least one endoscopic sinus surgery, and have taken at least two doses of dupilumab or mepolizumab between January 1, 2016, and July 1, 2023. Data collected include clinicodemographic information and past medical/surgical history. The primary outcomes are the incidence and types of adverse events. Adjusted odds ratio was calculated to compare the biologics using logistic regression. Risk factors for developing adverse events were investigated. RESULTS: Eighty-seven patients on dupilumab and 51 patients on mepolizumab were included. Thirty-nine (45%) and 10 (20%) patients respectively encountered adverse events, which differed from phase 3 trial data. The adjusted odds ratio for adverse event rates between these two treatment groups was 3.8 (95% CI, 1.5-10.5). The most common adverse events for dupilumab were arthralgia (16%), rash (14%), and conjunctivitis or xerophthalmia (10%). The main adverse events for mepolizumab were headache (6%) and fatigue (6%). Seven dupilumab and three mepolizumab patients discontinued therapy due to adverse events. The average duration of treatment was 22.5 (range: 1-77) months for mepolizumab and 15.9 (range: 1-35) months for dupilumab. CONCLUSION: Dupilumab and mepolizumab have distinct adverse event profiles. This study contributes to informing clinicians' decisions regarding the use of type 2 biologics in CRSwNP patients. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:3054-3059, 2024.

Endoscopic treatment in pediatric patients with recurrent and H-type tracheoesophageal fistulas - A systematic review and meta-analysis.

OBJECTIVES: Endoscopic treatments for managing recurrent tracheoesophageal fistula (rTEF) and H-type TEF are being utilized lately; however, the preferred technique is yet to be determined. We aimed to systematically review existing publications on endoscopic treatment of rTEF and H-type TEF to analyze their success and complication rates. METHODS: PRISMA guidelines were followed. MEDLINE, EMBASE, CINAHL and the Cochrane Central Register of Controlled Trials were comprehensively searched in accordance to a priori developed protocol, from 1975 until 2020. English, Spanish and German papers were included. Studies were independently screened and analyzed by two reviewers. RESULTS: 84 full texts were assessed for eligibility out of 581 screened studies, of these, 39 studies with 127 patients were eligible for inclusion (115 rTEF and 12 H-type TEF). All included studies were cases reports or case series. Overall success rate was 45% with sealant injection, 87% with de-epithelialization and 80% with sealant injection and de-epithelialization combined. The mean number of required treatments for success was 1.9 (range 1-6). Mixed effect model meta-analysis of case series with n > 1 showed that sealant injection had a significantly lower success rate of 50% (95% CI 1-99%, I2 72%) compared to de-epithelialization 90% (95% CI 72-99%, I2 27%), p = 0.007 and the combination of both techniques 87% (95% CI 68-99%, I2 11%), p = 0.02. Nine patients (7%) had transient respiratory distress. No mortalities reported. CONCLUSION: Endoscopic treatment for rTEF and H-type fistula is a minimally invasive technique with favorable outcome and considerably less morbidity compared to open surgery, suggesting it as a safe and effective first line treatment option. Repeated endoscopic treatment attempts can be expected to obtain complete closure. De-epithelization techniques with or without combined tissue adhesive injection had significantly better results than sealant injection techniques alone.