McLeod, Daryl J.

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.

OBJECTIVES: Posterior urethral valves predispose children to renal replacement therapy (RRT) and bladder dysfunction. Researchers of single-institutional series were unable to refine risk stratification because of rarity of the disease. We aimed to identify clinical variables associated with the risk of RRT and clean intermittent catheterization (CIC) in a large multicenter cohort study. METHODS: Children with posterior urethral valves born between 1995 and 2005 who were treated before 90 days of life at 5 children’s hospitals were retrospectively reviewed. Outcomes included RRT and recommendation for CIC. Predictors and outcomes were assessed by using survival analysis. RESULTS: A total of 274 patients were managed for a median of 6.3 years, and 42 progressed to RRT. On survival analysis, 16% progressed to RRT by 10 years of age. RRT varied by the serum nadir creatinine level in the first year of life (SNC1) (log-rank P &lt; .001). After stratifying by the SNC1, the estimated risk of progressing to RRT by 10 years of age was 0%, 2%, 27%, and 100% for an SNC1 of &lt;0.4, an SNC1 of 0.4 to 0.69, an SNC1 of 0.7 to 0.99, and an SNC1 of ≥1.0 mg/dL, respectively. CIC was recommended in 60 patients, which translated on survival analysis to a risk of 26% by 10 years of age. CONCLUSIONS: Risk of RRT and CIC recommendation increased with age. The SNC1 strongly predicted need for RRT. These results allow for both improved family counseling and the potential for more appropriate screening and intervention strategies for those identified in higher-risk groups.