Measured versus self-reported physical function in adult survivors of childhood cancer.

PURPOSE: Childhood cancer survivors (CCS) experience late effects that interfere with physical function. Limitations in physical function can affect CCS abilities to actively participate in daily activities. The purpose of this investigation was to evaluate the concordance between self-reported physical performance and clinically evaluated physical performance among adult CCS. METHODS: CCS 18 yr or older and 10 yr or older from diagnosis who are participants in the St. Jude Lifetime cohort study responded to the physical function section of the Medical Outcome Survey Short Form (SF-36). Measured physical performance was evaluated using the Physical Performance Test and the 6-Minute Walk Test. RESULTS: Individuals (N = 1778, 50.8% female) with a median time since diagnosis of 24.9 yr (range = 10.9-48.2) and a median age of 32.4 yr (range = 19.1-48.2) completed testing. Limitations in physical performance were self-reported by 14.1% of participants. The accuracy of self-report physical performance was 0.87 when the SF-36 was compared with the 6-Minute Walk Test or the Physical Performance Test. Reporting inaccuracies most often involved reporting a physical performance limitation. Poor accuracy was associated with previous diagnosis of a bone or CNS tumor, lymphoma, older age, and large body size. CONCLUSIONS: These results suggest that self-report, using the physical performance subscale of the SF-36, correctly identifies CCS who do not have physical performance limitations. In contrast, this same measure is less able to identify individuals who have performance limitations.

A review of pathophysiology and management of fetuses and neonates with meconium ileus for the pediatric surgeon.

PURPOSE: Meconium ileus (MI) is the earliest clinical manifestation of cystic fibrosis (CF), occurring in up to 20% of patients with CF. Our aim was to review and integrate current knowledge about the diagnosis and management of fetuses and neonates with MI that may aid the pediatric surgeon in caring for these patients. METHODS: We identified areas of interest including pathophysiology, prenatal diagnosis, nonoperative and operative management, postoperative management, and prognosis. We performed a Medline search using the search term meconium ileus for English language articles published in the last 20 years. We reviewed reference lists to identify other articles of historical significance. RESULTS: Meconium ileus is primarily associated with CF transmembrane (conductance) regulator mutations F508del, G542X, W1282X, R553X, and G551D, and modifier genes have been found to explain approximately 17% of the phenotypic variability. Mouse, pig, and ferret models for CF demonstrate neonatal bowel obstruction mimicking MI. Sonographic findings of hyperechoic masses and dilated bowel in a high-risk fetus are suggestive of MI. Less than 7% of low-risk fetuses with hyperechoic bowel will have MI. Contemporary series of noninvasive management with Gastrografin enema report success rates of 36% to 39%, significantly lower than historical values. The optimal surgical technique remains controversial, although primary anastomosis results in surgical complication rates between 21% and 31%, higher than those noted with delayed anastomosis. Pulmonary function for patients with CF and MI at 15 and 25 years old is similar to those without MI, although height and weight percentiles may be lower. CONCLUSIONS: This review for pediatric surgeons presents an examination of the literature and synthesizes current information about the pathophysiology, prenatal diagnosis, nonoperative and operative management, postoperative management, and prognosis of the patient with CF and MI.