Quantifying physical decline in juvenile neuronal ceroid lipofuscinosis (Batten disease).

OBJECTIVE: To use the Unified Batten Disease Rating Scale (UBDRS) to measure the rate of decline in physical and functional capability domains in patients with juvenile neuronal ceroid lipofuscinosis (JNCL) or Batten disease, a neurodegenerative lysosomal storage disorder. We have evaluated the UBDRS in subjects with JNCL since 2002; during that time, the scale has been refined to improve reliability and validity. Now that therapies are being proposed to prevent, slow, or reverse the course of JNCL, the UBDRS will play an important role in quantitatively assessing clinical outcomes in research trials. METHODS: We administered the UBDRS to 82 subjects with JNCL genetically confirmed by CLN3 mutational analysis. Forty-four subjects were seen for more than one annual visit. From these data, the rate of physical impairment over time was quantified using multivariate linear regression and repeated-measures analysis. RESULTS: The UBDRS Physical Impairment subscale shows worsening over time that proceeds at a quantifiable linear rate in the years following initial onset of clinical symptoms. This deterioration correlates with functional capability and is not influenced by CLN3 genotype. CONCLUSION: The UBDRS is a reliable and valid instrument that measures clinical progression in JNCL. Our data support the use of the UBDRS to quantify the rate of progression of physical impairment in subjects with JNCL in clinical trials.

Office prenatal formula advertising and its effect on breast-feeding patterns.

OBJECTIVE: To compare the effect of formula company-produced materials about infant feeding to breast-feeding promotion materials without formula advertising on breast-feeding initiation and duration. METHODS: Five hundred forty-seven pregnant women were randomized to receive either formula company (commercial; n = 277) or specially designed (research; n = 270) educational packs about infant feeding at their first prenatal visit. Feeding method was determined at delivery. Breast-feeding duration of the 294 women who chose to breast-feed was ascertained at 2, 6, 12, and 24 weeks. Survival analyses were used to evaluate continuous outcomes, and chi2 and logistic regression analyses were used to evaluate discrete outcomes. RESULTS: Breast-feeding initiation (relative risk [RR] 0.93, 95% confidence interval [CI] 0.61, 1.43) and duration after 2 weeks (hazard ratio 1.19, 95% CI 0.86, 1.64) were not affected. Women in the commercial group were more likely to cease breast-feeding before hospital discharge (RR 5.80, 95% CI 1.25, 54.01) and before 2 weeks (adjusted odds ratio [OR] 1.91, 95% CI 1.02, 3.55). In subgroup analyses, women with uncertain goals for breast-feeding or goals of 12 weeks or less experienced shortened exclusive (hazard ratio 1.53, 95% CI 1.06, 2.21), full (hazard ratio 1.70, 95% CI 1.18, 2.48), and overall (hazard ratio 1.75, 95% CI 1.16, 2.64) breast-feeding duration when exposed to the commercial intervention. CONCLUSION: Although breast-feeding initiation and long-term duration were not affected, exposure to formula promotion materials increased significantly breast-feeding cessation in the first 2 weeks. Additionally, among women with uncertain goals or breast-feeding goals of 12 weeks or less, exclusive, full, and overall breast-feeding duration were shortened. Educational materials about infant feeding should support unequivocally breast-feeding as optimal nutrition for infants; formula promotion products should be eliminated from prenatal settings.

A randomized, controlled trial of a eutectic mixture of local anesthetic cream (lidocaine and prilocaine) versus penile nerve block for pain relief during circumcision.

OBJECTIVE: We set out to compare a eutectic mixture of local anesthetic cream (lidocaine and prilocaine) to dorsal penile nerve block with lidocaine for anesthesia during circumcision. STUDY DESIGN: In a double-blind study, term newborns were randomized to local anesthetic cream and sodium chloride solution dorsal penile nerve block (n = 31) or to placebo cream and lidocaine dorsal penile nerve block (n = 29). Pain was assessed by determination of heart rate, respiratory rate, and behavioral distress scoring. Group differences were evaluated with repeat-measures analyses of variance. RESULTS: Distress scores and heart rates were significantly higher in the eutectic mixture group than in the lidocaine group. Respiratory rates were higher in the eutectic mixture group but did not reach statistical significance. CONCLUSIONS: Distress scores and heart rates were significantly higher in infants treated with the anesthetic mixture than in infants treated with lidocaine. Dorsal penile nerve block with lidocaine is a more efficacious means of providing anesthesia for neonatal circumcision than the mixture of local anesthetics.

The effects of early pacifier use on breastfeeding duration.

OBJECTIVE: To evaluate the effects of pacifier use and the timing of pacifier introduction on breastfeeding duration, problems, and frequency. METHODS: A cohort of 265 breastfeeding mother-infant dyads was followed prospectively. Maternal interviews were conducted at delivery, 2, 6, 12, and 24 weeks, and thereafter every 90 days until breastfeeding ended. Information was obtained regarding pacifier use, infant feeding, use of supplemental foods and breastfeeding frequency, duration, and problems. The effect of pacifier introduction by 6 weeks of age on breastfeeding duration was evaluated with Kaplan-Meier and Cox proportional hazards models. The effect of the timing of pacifier introduction (