Post-transplant lymphoproliferative disorders (PTLDs) are lymphoid or plasmacytic proliferations ranging from polyclonal reactive proliferations to overt lymphomas that develop as consequence of immunosuppression in recipients of solid organ transplantation (SOT) or allogeneic bone marrow/hematopoietic stem cell transplantation. Immunosuppression and Epstein–Barr virus (EBV) infection are known risk factors for PTLD. Patients with documented histopathologic diagnosis of primary PTLD at our institution between January 2000 and October 2019 were studied. Sixty-six patients with PTLD following SOT were followed for a median of 9.0 years. The overall median time from transplant to PTLD diagnosis was 5.5 years, with infant transplants showing the longest time to diagnosis at 12.0 years, compared to pediatric and adolescent transplants at 4.0 years and adult transplants at 4.5 years. The median overall survival (OS) was 19.0 years. In the monomorphic diffuse large B-cell (M-DLBCL-PTLD) subtype, median OS was 10.7 years, while median OS for polymorphic subtype was not yet reached. There was no significant difference in OS in patients with M-DLBCL-PTLD stratified by quantitative EBV viral load over and under 100,000 copies/mL at time of diagnosis, although there was a trend towards worse prognosis in those with higher copies.

Post-transplant lymphoproliferative disorders (PTLD) are heterogenous lymphoproliferative disorders that develop as a consequence of immunosuppression in transplant recipients. We sought to determine if subtypes of PTLD correlated with different outcomes. We performed a retrospective review of PTLD occurring in pediatric heart transplant recipients. A total of 558 children and infants underwent cardiac transplantation at our institution between 1985 and 2019 and were followed until March 2021. Forty-nine of 558 patients developed PTLD (8.8%). As compared to older children (>one year of age), infant recipients (<three months of age) were less likely to develop PTLD. Monomorphic PTLDs (M-PTLD, 61%) was the most common subtype at initial diagnosis, followed by non-destructive (21%), polymorphic (14%), and classic Hodgkin lymphoma (cHL, 4%). Patients who underwent transplantation at a young age (<three months) had significantly lower rates of M-PTLD or cHL and a longer time from transplant to PTLD diagnosis as compared to children older than one year at transplant (p = 0.04). Although not reaching statistical significance, patients with a shorter time to PTLD diagnosis showed a trend toward higher rates of M-PTLD or cHL. As expected, the overall survival (OS) of patients with M-PTLD or cHL was significantly lower than patients with non-destructive or polymorphic PTLD.

To characterize the clinical course and outcomes of nasal intermittent mandatory ventilation (NIMV) use in acute pediatric respiratory failure. We identified all patients treated with NIMV in the pediatric intensive care unit (PICU) or inpatient general pediatrics between January 2013 and December 2015 at two academic centers. Patients who utilized NIMV with other modes of noninvasive ventilation during the same admission were included. Data included demographics, vital signs on admission and prior to initiation of NIMV, pediatric risk of mortality III (PRISM-III) scores, complications, respiratory support characteristics, PICU and hospital length of stays, duration of respiratory support, and complications. Patients who did not require escalation to mechanical ventilation were defined as NIMV responders; those who required escalation to mechanical ventilation (MV) were defined as NIMV non-responders. NIMV responders were compared to NIMV non-responders. Forty-two patients met study criteria. Six (14%) failed treatment and required MV. The majority of the patients (74%) had a primary diagnosis of bronchiolitis. The median age of these 42 patients was 4 mo (range 0.5-28.1 mo, IQR 7, P = 0.69). No significant difference was measured in other baseline demographics and vitals on initiation of NIMV; these included age, temperature, respiratory rate, O2 saturation, heart rate, systolic blood pressure, diastolic blood pressure, and PRISM-III scores. The duration of NIMV was shorter in the NIMV non-responder vs NIMV responder group (6.5 h vs 65 h, P < 0.0005). Otherwise, NIMV failure was not associated with significant differences in PICU length of stay (LOS), hospital LOS, or total duration of respiratory support. No patients had aspiration pneumonia, pneumothorax, or skin breakdown. Most of our patients responded to NIMV. NIMV failure is not associated with differences in hospital LOS, PICU LOS, or duration of respiratory support.

Objective: The purpose of this experiment was to evaluate the use of RPL and LAHSHAL coding systems by individuals not specialized in craniofacial abnormalities to code cleft lip and palate (CLP). The effectiveness of system use by referring services and the electronic medical record (EMR) applicability of other CLP systems was evaluated by literature review and testing data. Design and Participants: The RPL and LAHSHAL systems were presented together to a sample of medical students ( n = 28) and neonatal intensive care unit nurses ( n = 24) from Loma Linda University. Following the presentation, a test assessing the application of each system was administered. A second assessment of the medical students ( n = 23) 2 weeks after the initial presentation evaluated system retention. Scores were compared using t test ( P ≤ .05). Results: Both the medical students and nurses used RPL more accurately than LAHSHAL in the first assessment (76.9% versus 45.2%, P < .001; 46.6% versus 22.5%, P < .001). Medical students again used RPL more accurately at the 2-week assessment (72.2% versus 43.7%, P < .001). Accuracy of use within each system was not significantly different between the two assessments. Conclusion: Our test results and literature review indicate that, when compared with LAHSHAL, RPL is more easily and accurately used by representatives not specialized in abnormalities in CLP; therefore, RPL may be a more effective system for nonspecialist health care providers to improve the accuracy of referrals and simple EMR documentation.

The impact of noninvasive ventilation (NIV) on local and systemic inflammation is poorly characterized, particularly when compared with invasive mechanical ventilation (IMV). We sought to quantify the local and systemic inflammatory response of these 2 respiratory treatments in rats with lipopolysaccharide (LPS)-induced lung injury (LPS-injured) and healthy rats. Animals were subjected to 4 h of NIV or IMV treatments at noninjurious settings, or 4 h of control treatment in which healthy or LPS-injured animals remained spontaneously breathing under isoflurane anesthesia with no respiratory support. Cytokines were then quantified in the serum and lung tissue by multiplex enzyme-linked immunosorbent assay. Contrary to our hypothesis, there were no significant differences in cytokine levels in serum or lung when comparing the NIV- and IMV-treated groups; this was true in both LPS-injured and healthy rats. However, within the LPS-injured group, pulmonary levels of interleukin (IL)-1α, IL-6, and tumor necrosis factor α were significantly lower in the NIV-treated group than in control but not in the IMV-treated group compared with control. We conclude that NIV, unlike IMV, could attenuate local inflammation.

Acute kidney injury following orthotopic heart transplantation in pediatric recipients is often multifactorial, requiring balance of immune suppression, nephrotoxic medication exposure, nutrition, and fluid status. Therapeutic options are often limited by patient size and hemodynamic stability. We describe a four-month, 4.9-kg female bridged by mechanical circulatory support to transplant after failed stage 1 palliation secondary to recurrent aortic stenosis and severe ventricular dysfunction. Posttransplant, kidney injury was managed by transcatheter relief of central obstruction from an anastomotic stricture and continuous renal replacement therapy, allowing uninterrupted immune suppression, medication, and nutrition delivery until sufficient recovery of renal function.