Infections in Infants with SCID: Isolation, Infection Screening, and Prophylaxis in PIDTC Centers.

PURPOSE: The Primary Immune Deficiency Treatment Consortium (PIDTC) enrolled children with severe combined immunodeficiency (SCID) in a prospective natural history study of hematopoietic stem cell transplant (HSCT) outcomes over the last decade. Despite newborn screening (NBS) for SCID, infections occurred prior to HSCT. This study's objectives were to define the types and timing of infection prior to HSCT in patients diagnosed via NBS or by family history (FH) and to understand the breadth of strategies employed at PIDTC centers for infection prevention. METHODS: We analyzed retrospective data on infections and pre-transplant management in patients with SCID diagnosed by NBS and/or FH and treated with HSCT between 2010 and 2014. PIDTC centers were surveyed in 2018 to understand their practices and protocols for pre-HSCT management. RESULTS: Infections were more common in patients diagnosed via NBS (55%) versus those diagnosed via FH (19%) (p = 0.012). Outpatient versus inpatient management did not impact infections (47% vs 35%, respectively; p = 0.423). There was no consensus among PIDTC survey respondents as to the best setting (inpatient vs outpatient) for pre-HSCT management. While isolation practices varied, immunoglobulin replacement and antimicrobial prophylaxis were more uniformly implemented. CONCLUSION: Infants with SCID diagnosed due to FH had lower rates of infection and proceeded to HSCT more quickly than did those diagnosed via NBS. Pre-HSCT management practices were highly variable between centers, although uses of prophylaxis and immunoglobulin support were more consistent. This study demonstrates a critical need for development of evidence-based guidelines for the pre-HSCT management of infants with SCID following an abnormal NBS. TRIAL REGISTRATION: NCT01186913.

Renal dysfunction in cardiac surgery: identifying potential risk factors.

Aprotinin has been associated with increased renal failure and mortality when used in cardiac surgery. The purpose of this retrospective study was to compare the incidence of renal failure and mortality in our patient population to the published rates, accounting for risk factors associated with renal failure. After IRB approval; using the STS Database and cardiopulmonary bypass pump records, a total of 2292 cardiac patients were identified from January 2004 through June 2008. Forty-nine patients were excluded because they were on renal dialysis preoperatively. There were 1226 coronary artery bypass patients. Patients were separated into groups, according to which antifibrinolytic agent was used. This study included a total of 716 patients, divided into three groups; aprotinin (n = 436), tranexamic acid (n = 61), and off-pump coronary artery bypass (OPCAB) (n = 219). Epsilon aminocaproic acid (AMICAR) was given by the anesthesiologist to the majority of the remaining 510 patients and was not recorded on the bypass record. Therefore, patients given AMICAR were not included in this study. Outcomes included renal dialysis after surgery and mortality. Risk factors were identified and compared to patients in a study published by Mangano in the New England Journal of Medicine (N Engl J Med 2006; 354: 353-365). Aprotinin vs. control group showed no significant difference in risk factors for diabetes mellitus, hypertension, creatinine level above 1.3 mg/dl, or low ejection fraction. The percentage of patients requiring renal dialysis and mortality was less in Medical University of South Carolina (MUSC) patients than the other published study. Overall, the patients in the MUSC study had greater risk factors for renal failure, with the exception of patients with preoperative serum creatinine of >1.3 mg/dl (8.3 vs. 15.1%). This study does not show the same risk for renal failure associated with aprotinin that has been published elsewhere.