Long‐term hematologic and clinical outcomes of splenectomy in children with hereditary spherocytosis and sickle cell disease

BackgroundTotal splenectomy (TS) and partial splenectomy (PS) are used for children with congenital hemolytic anemia (CHA), although the long‐term outcomes of these procedures are poorly defined. This report describes long‐term outcomes of children with CHA requiring TS or PS.ProcedureWe collected data from children ages 2‐17 with hereditary spherocytosis (HS) or sickle cell disease (SCD) requiring TS or PS from 1996 to 2016 from 14 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a prospective, observational patient registry. We summarized hematologic outcomes, clinical outcomes, and adverse events to 5 years after surgery. Hematologic outcomes were compared using mixed effects modeling.ResultsOver the study period, 110 children with HS and 97 children with SCD underwent TS or PS. From preoperatively compared to postoperatively, children with HS increased their mean hemoglobin level by 3.4 g/dL, decreased their mean reticulocyte percentage by 6.7%, and decreased their mean bilirubin by 2.4 mg/dL. Hematologic improvements and improved clinical outcomes were sustained over 5 years of follow‐up. For children with SCD, there was no change in hemoglobin after PS or TS following surgery, although all clinical outcomes were improved. Over 5 years, there was one child with HS and five children with SCD who developed postsplenectomy sepsis.ConclusionsFor children with HS, there are excellent long‐term hematologic and clinical outcomes following either PS or TS. Although hemoglobin levels do not change after TS or PS in SCD, the long‐term clinical outcomes for children with SCD are favorable.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/155920/1/pbc28290.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/155920/2/pbc28290_am.pd

The utility of 6-minute walk distance in predicting waitlist mortality for lung transplant candidates.

BACKGROUND The lung allocation score (LAS) has led to improved organ allocation for transplant candidates. At present, the 6-minute walk distance (6MWD) is treated as a binary categorical variable of whether or not a candidate can walk more than 150 feet in 6 minutes. In this study, we tested the hypothesis that 6MWD is presently under-utilized with respect to discriminatory power, and that, as a continuous variable, could better prognosticate risk of waitlist mortality. METHODS A retrospective cohort analysis was performed using the Organ Procurement and Transplantation Network/United Network for Organ Sharing (OPTN/UNOS) transplant database. Candidates listed for isolated lung transplant between May 2005 and December 2011 were included. The population was stratified by 6MWD quartiles and unadjusted survival rates were estimated. Multivariable Cox proportional hazards modeling was used to assess the effect of 6MWD on risk of death. The Scientific Registry of Transplant Recipients (SRTR) Waitlist Risk Model was used to adjust for confounders. The optimal 6MWD for discriminative accuracy in predicting waitlist mortality was assessed by receiver-operating characteristic (ROC) curves. RESULTS Analysis was performed on 12,298 recipients. Recipients were segregated into quartiles by distance walked. Waitlist mortality decreased as 6MWD increased. In the multivariable model, significant variables included 6MWD, male gender, non-white ethnicity and restrictive lung diseases. ROC curves discriminated 6-month mortality was best at 655 feet. CONCLUSIONS The 6MWD is a significant predictor of waitlist mortality. A cut-off of 150 feet sub-optimally identifies candidates with increased risk of mortality. A cut-off between 550 and 655 feet is more optimal if 6MWD is to be treated as a dichotomous variable. Utilization of the LAS as a continuous variable could further enhance predictive capabilities

Racial, Ethnic, and Insurance Status Disparities in Use of Posthospitalization Care after Trauma

Abstract available at publisher's web site.http://dx.doi.org/10.1016/j.jamcollsurg.2011.08.01

The utility of preoperative six-minute-walk distance in lung transplantation

RATIONALE The use of 6-minute-walk distance (6MWD) as an indicator of exercise capacity to predict postoperative survival in lung transplantation has not previously been well studied. OBJECTIVES To evaluate the association between 6MWD and postoperative survival following lung transplantation. METHODS Adult, first time, lung-only transplantations per the United Network for Organ Sharing database from May 2005 to December 2011 were analyzed. Kaplan-Meier methods and Cox proportional hazards modeling were used to determine the association between preoperative 6MWD and post-transplant survival after adjusting for potential confounders. A receiver operating characteristic curve was used to determine the 6MWD value that provided maximal separation in 1-year mortality. A subanalysis was performed to assess the association between 6MWD and post-transplant survival by disease category. MEASUREMENTS AND MAIN RESULTS A total of 9,526 patients were included for analysis. The median 6MWD was 787 ft (25th-75th percentiles = 450-1,082 ft). Increasing 6MWD was associated with significantly lower overall hazard of death (P < 0.001). Continuous increase in walk distance through 1,200-1,400 ft conferred an incremental survival advantage. Although 6MWD strongly correlated with survival, the impact of a single dichotomous value to predict outcomes was limited. All disease categories demonstrated significantly longer survival with increasing 6MWD (P ≤ 0.009) except pulmonary vascular disease (P = 0.74); however, the low volume in this category (n = 312; 3.3%) may limit the ability to detect an association. CONCLUSIONS 6MWD is significantly associated with post-transplant survival and is best incorporated into transplant evaluations on a continuous basis given limited ability of a single, dichotomous value to predict outcomes