Shaare Zedek Medical Center

To describe the clinical and laboratory findings, disease course, and treatment effectiveness of a large, genetically homogenous group of patients with deficiency of adenosine deaminase 2 (DADA2), and to identify factors associated with disease-related damage. We conducted a retrospective cohort study that included 45 patients with DADA2 vasculitis with long-term follow-up. Information was collected from patients' charts, including demographic information, clinical presentations, laboratory findings, and treatment. The extent of disease-related damage was determined based on the Vasculitis Damage Index. In this large homogenous cohort, we found that a young age at disease onset and periodic illness that included recurrent episodes of fever, myalgia, and abdominal pain were associated with disease-related damage. Patients with severe DADA2 disease responded favorably to tumor necrosis factor inhibitors (TNFi). Early age of onset and the periodic nature of the disease warrant prompt initiation of TNFi treatment to prevent later complications and severe disease progression.

Functional endoscopic sinus surgery (FESS) is the gold-standard surgical treatment for chronic rhinosinusitis (CRS) not responding to appropriate medical therapy. Identifying the natural maxillary ostium (NMO) during FESS is often challenging due to the lack of definitive landmarks. To aid in the identification of the NMO, we describe and assess the feasibility of using a new surgical landmark, the infra-bullar groove (IBG), and evaluate its visibility and reproducibility during FESS. Methods: Video recordings of 41 maxillary antrostomy procedures in patients with varying severity of CRS were reviewed. Surgeons of different experience levels assessed IBG visibility and its termination at the NMO. Results: In video recordings where the ethmoid bulla was preserved during uncinectomy, the IBG and its connection to the NMO were successfully identified by all reviewers in 100% of analyzable cases. The mean time to IBG identification did not significantly differ among surgeons. The IBG was consistently more pronounced in cases with well-pneumatized bullae. Conclusions: Under controlled surgical conditions where the ethmoid bulla is preserved during uncinectomy, the IBG demonstrates high visibility and reproducibility for locating the NMO. However, further prospective studies are needed to establish its real-world utility and impact on surgical outcomes.

Chronic pouchitis and Crohn's-like disease of the pouch are frequent complications following restorative total proctocolectomy with ileal pouch-anal anastomosis (IPAA) for ulcerative colitis (UC). Biologics are increasingly used in refractory cases, but head-to-head comparative effectiveness data between biologic therapies are limited. We aimed to compare week 52 clinical response and long-term treatment persistence among patients treated with vedolizumab, ustekinumab, or adalimumab. This retrospective bi-center cohort study included adult patients with chronic pouchitis or Crohn's-like disease of the pouch with mPDAI ≥ 5 at biologic initiation. The primary endpoint was week 52 clinical response based on cPDAI, defined as a ≥ 1-point reduction from baseline. Key secondary endpoints were treatment persistence and week 52 mPDAI response (≥ 2-point reduction). Additional secondary endpoints included cPDAI remission, mPDAI remission, ePDAI response and remission, and biochemical outcomes. Between-group comparisons were performed using chi-square tests for categorical variables and Kruskal-Wallis tests for continuous variables, with post hoc pairwise comparisons adjusted for multiple testing. Treatment persistence was analyzed using Kaplan-Meier survival curves with log-rank test. Seventy-seven patients were included (vedolizumab n = 36; ustekinumab n = 22; adalimumab n = 19). Baseline characteristics including distribution of chronic pouchitis and Crohn's-like disease of the pouch were broadly comparable across groups, although ustekinumab was more frequently used as third-line therapy (p = 0.001). At week 52, cPDAI response occurred in 88.9% of patients treated with ustekinumab, 44.8% treated with vedolizumab, and 56.3% treated with adalimumab (p = 0.01). cPDAI remission was achieved in 33.3% of ustekinumab-treated patients, 13.8% of vedolizumab-treated patients, and 6.3% of adalimumab-treated patients (p = 0.09). No statistically significant differences were observed in biochemical remission across groups. Three-year treatment persistence was highest in the ustekinumab group (81.5%) compared with adalimumab (52%) and vedolizumab (35%) (p = 0.01). In this real-world cohort, ustekinumab was associated with numerically higher long-term clinical response and treatment persistence compared with vedolizumab or adalimumab. These findings support the need for prospective trials to guide biologic selection in chronic pouchitis and Crohn's-like disease of the pouch.

Fetal growth restriction (FGR) is a major contributor to perinatal morbidity and mortality. While guidelines address timing of delivery, the optimal mode-induction of labor (IOL) versus planned cesarean delivery (CD)-remains uncertain. To evaluate the association between planned mode of delivery and neonatal outcomes in pregnancies complicated by late onset FGR (LOFGR). We conducted a retrospective cohort study at a tertiary Canadian center (2017-2022). Singleton pregnancies with LOFGR (> 34 weeks' gestation), defined by Society for Maternal-Fetal Medicine (SMFM) criteria, were eligible if the last ultrasound was within 14 days of delivery. Exclusions included spontaneous labor, delivery < 34 weeks, and contraindications to labor. Planned mode of delivery (IOL vs CD) was the exposure. Outcomes were classified as severe (perinatal death, 5-min Apgar < 4, umbilical arterial pH < 7.05, base deficit ≥ 12 mmol/L, hypoxic-ischemic encephalopathy/therapeutic hypothermia, grade III-IV intraventricular hemorrhage, necrotizing enterocolitis, sepsis, or invasive ventilation > 24 h) or moderate (NICU stay > 72 h, Apgar 4-6, pH 7.05-7.10, non-invasive respiratory support > 6-12 h, transient tachypnea, or brief resuscitation). Multivariable logistic regression adjusted for confounders. A prespecified subgroup applied the ISUOG criteria. Of 12,270 deliveries, 1,143 (9.3%) met SMFM criteria for LOFGR; 869 were eligible (192 planned CD, 677 IOL). Severe outcomes and moderate outcomes were more frequent after CD (23.4% vs 16.7%; p = 0.03 and 42.2% vs 31.2%; p < 0.01, respectively). IOL was associated with lower adjusted risk of severe outcomes (aOR 0.35; 95% CI 0.19-0.67) and moderate outcomes (aOR 0.43; 95% CI 0.24-0.76). Results were consistent using ISUOG criteria (aOR 0.33; 95% CI 0.17-0.62 and aOR 0.44; 95% CI 0.25-0.79, respectively) About 20% of induced patients required intrapartum CD. IOL was associated with reduced severe and moderate neonatal morbidity compared with planned CD. IOL represents a safe alternative when intrapartum surveillance and timely operative delivery are available.

To assess how inclusion criteria from major randomized controlled trials (RCTs) of extracorporeal cardiopulmonary resuscitation (ECPR) apply within a cannulated ECPR cohort and to benchmark observed outcomes against published trial results. We conducted a single-center retrospective cohort study at a 1,000-bed tertiary medical center of adults who underwent ECPR for out of hospital cardiac arrest. Inclusion criteria from the ARREST, PRAGUE, and INCEPTION trials were retrospectively applied. Analyses were restricted to cannulated patients, with survival evaluated overall and stratified by trial eligibility, and descriptively compared with published RCT outcomes. Sixty-six patients underwent ECPR, including 25 (38%) with a non-shockable initial rhythm. Overall survival to hospital discharge was 14% (9/66; 95% CI 6.4-24.3%), with favorable neurological outcome in 5/9 survivors. Survival was numerically higher among patients meeting trial inclusion criteria, but differences were not statistically significant. Survival was comparable to PRAGUE and INCEPTION and lower than ARREST. Within this program level, cannulated ECPR cohort, RCT-derived inclusion criteria did not clearly distinguish survivors from non-survivors, and survival occurred among patients not meeting one or more trial eligibility thresholds. Rigid application of trial criteria at the point of cannulation may therefore exclude some patients with potential for meaningful recovery.

Objective: Postpartum maternal readmission is a significant burden for patients as well as the health system. Postpartum readmission rate is a known factor in evaluating quality of care and in guiding potential beneficial interventions. Use of the Robson Group (RG) classification, initially used for analysis of cesarean section (CS) rates, has been recently expanded to evaluate other obstetrical outcomes. We aimed to describe the rates of postpartum maternal readmission across RG classification and to identify risk factors among the different maternity groups. Study Design: We carried out a retrospective register-based cohort study of all women who delivered >24 weeks gestation at a tertiary medical center over an 18-year period, with classification into the 10 RGs. Rates of postpartum readmission within 42 days of delivery were calculated for each group, as well as indications for readmission. The risk for maternal readmission was analyzed by univariate binary logistic regressions with comparison of results among RC groups, as well as by multivariate analysis models. Results: During the study period, 296,768 deliveries were classified according to Robson Group (RG) classification. The overall readmission rate for the study population was 0.5%. The following groups had a significant risk of readmission: RG 9 (transverse lie), 1.9%; RG 8 (multifetal pregnancies), 1.90=3%; RG 7 (multiparous breech pregnancies) 1.2% and RG2 (nulliparous pregnancies > 37 w, labor induction or prelabor cesarean), 1.2%. The most common indication for readmission among all RGs was fever (61.4%). Conclusions: Postpartum readmission rates varied among the RGs. The highest-risk groups were those with a higher risk of operative delivery, prolonged labor, or malpresentations. Interventions aimed to reduce the number of women in these groups; these included use of external cephalic version, vaginal delivery of breech, and multifetal pregnancies, all of which may be beneficial.