Longitudinal evaluation of cardiopulmonary performance during exercise after bone marrow transplantation in children.

OBJECTIVE: Abnormalities in cardiopulmonary performance during exercise have been reported in children after bone marrow transplantation (BMT). We sought to study changes in exercise performance over time in pediatric BMT survivors. STUDY DESIGN: We retrospectively reviewed the results of serial cardiopulmonary exercise tests performed by patients who had undergone BMT at our institution. Four measurements of cardiopulmonary function are reported: maximum cardiac index (MCI), maximal oxygen consumption (Max VO(2)), oxygen consumption at ventilatory threshold (VO(2) at VT), and maximum work (Max Work) performed. A linear mixed-effects model was fitted to assess changes in these parameters over time. RESULTS: Thirty-three patients performed 96 cardiopulmonary exercise tests. MCI and VO(2) at VT were depressed at initial testing and did not change over time. Max VO(2) increased by 4% per year to 69% predicted, and Max Work increased to 77% predicted at 6 years after BMT. CONCLUSIONS: In spite of an impaired cardiovascular response to exercise as indicated by the persistently low MCI, aerobic and physical working capacity increase. Improved Max VO(2) suggests that oxygen extraction at the musculoskeletal level becomes more efficient with recovery from BMT. This may represent a compensatory response to an impaired ability to increase cardiac output.

Short interpregnancy interval and the risk of disabling cerebral palsy in a low birth weight population.

OBJECTIVE: The objective of this study was to investigate the relation of short interpregnancy interval and cerebral palsy (CP) in a low birth weight population in whom neonatal brain injury was systematically ascertained in the newborn period. STUDY DESIGN: We used data from a prospective cohort study of 1105 infants born < or = 2000 gm, designed to study the cause and consequences of neonatal brain injury. Of the 777 children with data on brain injury and CP, 382 were born to multiparous women, and 375 had information available to calculate interpregnancy interval, defined as the number of days from the end of the last pregnancy to conception of the index pregnancy. RESULTS: In the presence of the strong explanatory power of neonatal brain injury, short interpregnancy interval maintains an independent risk for disabling CP. CONCLUSION: In this cohort of low birth weight infants, short interpregnancy interval appears to represent a significant risk for the development of disabling CP.

Prognostic signs in the surgical management of plexiform neurofibroma: the Children's Hospital of Philadelphia experience, 1974-1994.

OBJECTIVES: To estimate the rate of progression of plexiform neurofibroma after surgery and to identify prognostic factors that predict progression. STUDY DESIGN: A retrospective review of the inpatient and outpatient records of 121 patients, who had 302 procedures on 168 tumors over a 20-year period at a single large pediatric referral center. Data on age, location, indication for surgery, and extent of resection was analyzed for prognostic significance. RESULTS: The overall freedom from progression was 54%. Children < 10 years old had a shorter interval of tumor control than older children (p = 0.0004). Tumors of the head/neck/face fared worse than tumors of the extremities (p = 0.0003). Less extensive resection predicted shorter interval to progression (p < 0.0001). Indication for surgery was not of prognostic importance. In multivariable analysis older age and location in the extremities were predictors of a better outcome. CONCLUSIONS: Tumor progression is a serious problem for children with plexiform neurofibroma. Younger children, children with tumors of the head/neck/face, and tumors that cannot be nearly completely removed are at particular risk. These data may be useful in helping clinicians decide which patients and which tumors are most likely to benefit from surgical intervention.

Topical application of lidocaine-prilocaine (EMLA) cream reduces the pain of intramuscular infiltration of saline solution.

OBJECTIVE: Intramuscular injections may be painful. Some of this pain may be caused by the infiltration of medication into the muscle, separate from the pain of skin puncture. We hypothesized that topical application of lidocaine/prilocaine (EMLA) cream would reduce the pain of intramuscular infiltration. METHODS: A double-blinded, placebo-controlled study was performed in 40 adult volunteers to compare the pain of needle puncture and of infiltration of saline into the deltoid muscle after application of EMLA cream or placebo. Each subject served as his or her own control. Pain scores were obtained by using a 100 mm visual analog scale (VAS). RESULTS: Pain associated with needle puncture was significantly reduced by EMLA cream as compared with placebo (median VAS score, 7.5 vs 19.5; p = 0.0043), as was pain associated with intramuscular infiltration (median VAS score, 2.5 vs 11; p < 0.00005). CONCLUSIONS: Our results suggest that further clinical studies of EMLA cream for modifying perceived pain from intramuscular injection in children are warranted.

Longitudinal, prospective analysis of dietary intake in children with cystic fibrosis.

OBJECTIVES: Prospective, 3-year longitudinal dietary intakes of 25 prepubertal, pancreatic-insufficient children with cystic fibrosis (CF) and mild lung disease, and the intakes of 26 control children were compared, and relationships among energy intake, nutritional status, and pulmonary function were determined. STUDY DESIGN: Intakes from 3-day weighed food records were compared with CF recommendations, recommended dietary allowances (RDA), and the recommendations of the Third National Health and Nutrition Examination Survey (NHANES III). Energy and nutrient intakes were analyzed by repeated-measures analysis of variance. RESULTS: Children with CF consumed more energy than control children (p = 0.025) in terms of calories per day, percentage of RDA by age and by age and weight, energy adjusted for fat malabsorption, and percentage of NHANES III recommendations. Energy intake was similar between boys and girls with CF. The percentage of energy from fat was greater (p = 0.0004) in the CF group (3-year mean, 33%) than in the control group. Height and weight z scores declined in the CF group (p <0.05) with time. Vitamin and mineral intakes were generally adequate in the CF group. CONCLUSIONS: The children in this sample did not consume the CF recommended intakes of 120% RDA for energy or a high-fat (40% of energy) diet. Energy intakes may be insufficient in this group to meet requirements for optimal growth.