Killer immunoglobulin-like receptor (KIR) and KIR ligand (KIRL) interactions play an important role in natural killer (NK) cell-mediated graft-versus-leukemia effect following hematopoietic cell transplantation (HCT). However, there is considerable heterogeneity in the KIR gene and KIRL content in individuals, making it difficult to estimate the full clinical impact of NK cell reconstitution following HCT. Here we present a novel adaptive mathematical model designed to quantify these interactions to better assess the influence of NK cell-mediated alloreactivity on transplant outcomes. Ninety-eight HLA- matched unrelated donor (URD) HCT recipients were studied retrospectively. The KIR-KIRL interactions were quantified using a system of matrix equations. Unit values were ascribed to each KIR-KIRL interaction, and the directionality of interactions was denoted by either a positive (activating) or negative (inhibition) symbol; these interactions were then summed. The absolute values of both the missing KIRL and inhibitory KIR-KIRL interactions were significantly associated with overall survival and relapse. These score components were initially used to develop a weighted score (w-KIR score) and subsequently a simplified, nonweighted KIR-KIRL interaction score (IM-KIR score). Increased w-KIR score and IM-KIR score were predictive of all-cause mortality and relapse (w-KIR score: hazard ratio [HR], .37 [P = .001] and .44 [P = .044], respectively; IM-KIR score: HR, .5 [P = .049] and .44 [P = .002], respectively). IM-KIR score was also associated with NK cell reconstitution post-HCT. KIR-KIRL interactions as reflected by the w-KIR and IM-KIR scores influence both relapse risk and survival in recipients of HLA-matched URD HCT with hematologic malignancies.

Background: Open window thoracostomy (OWT) is indicated for patients with bronchopleural fistula (BPF) or trapped lung in the setting of empyema refractory to non-surgical interventions. We investigated the role of OWT in the era of minimally invasive surgeries, endobronchial valves and fibrinolytic therapy.Methods: A retrospective chart review of all patients who underwent OWT at a single institution from 2010 to 2020 was performed. Indications for the procedure as well as operative details and morbidity and mortality were evaluated to determine patient outcomes for OWT.Results: Eighteen patients were identified for the study. The most common indication for OWT was post-resectional BPF (n = 9). Prior to OWT,n = 11 patients failed other surgical or minimally invasive interventions. Patient comorbidities were quantified with the Charlson Comorbidity index (n = 11 score ≥ 5, 10-year survival ≤21%). Three (16.7%) patients died < 30 days post-operatively and 12 (66%) patients were deceased by the study’s end (overall survival 24.0 ± 32.2 months). Mean number of ribs resected were 2.5 ± 1.2 (range 1–6) with one patient having 6 ribs removed. Patients were managed with negative pressure wound therapy (n = 9) or Kerlix packing (n = 9). Eleven patients (61.6%) underwent delayed closure (mean time from index surgery to closure 4.8 ± 6.7 months).Conclusions: Our study illustrates the significant comorbidities of patients undergoing OWT, the poor outcomes therein, and pitfalls associated with this procedure. We show that negative pressure wound therapy can be utilized as potential way to obliterate the pleural space and manage an open chest in the absence of an airleak; however, OWT procedures continue to be extremely morbid.

Previous research suggests that children and adolescents with acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL) often have difficulty adhering to complex treatment regimens during the maintenance phase of therapy. Measurement of treatment adherence can be done via objective (e.g., electronic monitoring (EM), pharmacological assays) or subjective methods (patient, parent, or physician reports). This paper provides an illustration of recommended strategies for comparing discrepancies between two objective measures of medication adherence (e.g., behavioral adherence using electronic monitoring versus pharmacological adherence using 6-mercaptopurine (6MP) metabolite data) within a relatively large cohort of pediatric patients with ALL or LBL (N = 139) who had longitudinal data for both measures of medication adherence over a 15-month period. Additionally, individual- and family-level factors such as gender, socioeconomic status, household environment, and dose intensity will be examined to identify possible sources of discrepancies between adherence measures. This information will provide practical advice for physicians, healthcare providers, and psychologists in identifying nonadherence and the caveats therein so patients achieve the best possible health outcomes.

Right sided congenital diaphragmatic hernia (CDH) associated with hepatopulmonary fusion (HPF) is a rare congenital anomaly in which the herniated liver is fused with lung parenchyma. We discuss the case of an infant with right-sided CDH and HPF found on index operation for repair of right-sided CDH. Due to the high incidence of vascular anomalies associated with HPF the decision was made to close the patient and get further imaging to characterize the HPF before returning to the operating room for definitive repair. We describe a novel and successful operative approach where the liver was left entirely fused to the lung and the liver was partially brought down from the chest and was plicated to the diaphragm form a seal between the parietal and pleural cavities to repair the CDH.

Pediatric extracorporeal membrane oxygenation (ECMO) decannulation practices vary widely regarding ligation versus repair of the common carotid artery (CCA) and internal jugular vein (IJV) with potential implications for neurologic outcomes. We examined rates of ischemic cerebral vascular accident (CVA) and vessel patency in pediatric ECMO patients following decannulation. A retrospective review of pediatric ECMO patients (2009–2023) cannulated via cervical approach was conducted, excluding palliative decannulations. Clinical outcome measures were evaluated using descriptive and inferential statistics. Of 140 patients, 49 (35.0%) underwent vessel repair. These patients were more likely to have a cardiac indication for ECMO (57.1% vs . 19.8%; p < 0.001). Median time from cannulation to vessel repair was 97 hours (interquartile range [IQR]: 73–170). Rates of ischemic CVA were not different between groups (8.2% repaired vs. 8.8% ligated; p = 0.899). Surveillance imaging was performed in 49% of repaired patients, with a 95.8% CCA and 55.5% IJV patency rate. High CCA patency rates were observed following cervical vessel repair, with no difference in ischemic CVA compared with ligation. Vessel repair may be beneficial in populations at risk for future mechanical support needs, such as those with complex cardiac disease, as it preserves the option for subsequent ECMO cannulation.