ABSTRACT
BACKGROUND: Batten disease (juvenile neuronal ceroid lipofuscinosis [JNCL]) is an autosomal recessive neurodegenerative disorder characterized by blindness, seizures, and relentless decline in cognitive, motor, and behavioral function. Onset is in the early school years, with progression to death typically by late adolescence. Development of a clinical instrument to quantify severity of illness is a prerequisite to eventual assessment of experimental therapeutic interventions. OBJECTIVE: To develop a clinical rating instrument to assess motor, behavioral, and functional capability in JNCL. METHODS: A clinical rating instrument, the Unified Batten Disease Rating Scale (UBDRS), was developed by the authors to assess motor, behavioral, and functional capability in JNCL. Children with verified JNCL were evaluated independently by three neurologists. Intraclass correlation coefficients (ICCs) were used to estimate the interrater reliability for total scores in each domain. Interrater reliability for scale items was assessed with weighted kappa statistics. RESULTS: Thirty-one children with confirmed JNCL (10 boys, 21 girls) were evaluated. The mean age at symptom onset was 6.1 +/- 1.6 years, and the mean duration of illness was 9.0 +/- 4.4 years. The ICCs for the domains were as follows: motor = 0.83, behavioral = 0.68, and functional capability = 0.85. CONCLUSIONS: The Unified Batten Disease Rating Scale (UBDRS) is a reliable instrument that effectively tests for neurologic function in blind and demented patients. In its current form, the UBDRS is useful for monitoring the diverse clinical findings seen in Batten disease.
Subject(s)
Neuronal Ceroid-Lipofuscinoses/diagnosis , Neuropsychological Tests/standards , Personality Tests/standards , Severity of Illness Index , Adolescent , Adult , Child , Child, Preschool , Clinical Trials as Topic/standards , Disease Progression , Female , Humans , Male , Neurologic Examination/methods , Neurologic Examination/standards , Neuronal Ceroid-Lipofuscinoses/physiopathology , Neuronal Ceroid-Lipofuscinoses/psychology , Predictive Value of Tests , Prognosis , Treatment OutcomeABSTRACT
BACKGROUND: To prevent breastfeeding problems, cup-feeding has been recommended as a method of providing medically necessary supplemental feedings to breastfed infants. OBJECTIVES: To compare amounts ingested, administration time, and infant physiologic stability during cup-, bottle-, and breastfeeding. DESIGN/METHODS: A total of 98 term, healthy newborns were randomized to either cup-feeding (n = 51) or bottle-feeding (n = 47). The heart (HR), respiratory (RR), and oxygen (O(2)) saturation rates were monitored on these infants and 25 breastfed newborns during 1 feeding. Differences in amounts ingested and administration times were evaluated with t tests and physiologic data with repeat measures analysis of variance. RESULTS: There were no significant differences in administration time, amounts ingested or overall HR, RR, and (O(2)) saturation rates, between cup and bottle groups. Breastfed infants had longer administration times and lower overall HR, RR, and higher O(2) saturation as compared with cup- and bottle-fed infants. CONCLUSIONS: Administration times, amounts ingested, and infant physiologic stability do not differ with cup- and bottle-feeding. Breastfeeding takes longer than cup- or bottle-feeding, but infants experience less physiologic variability. These data support cup-feeding as an alternative to bottle-feeding for supplying supplements to breastfed infants.
Subject(s)
Bottle Feeding , Breast Feeding , Feeding Methods , Female , Heart Rate , Humans , Infant, Newborn , Male , Oxygen/bloodABSTRACT
OBJECTIVE: We conducted a national survey of pediatric, family practice, and obstetrics and gynecology residency program directors to determine the curriculum content and predominant practices in US training programs with regard to neonatal circumcision and anesthesia/analgesia for the procedure. METHODS: Residency directors of accredited programs were surveyed in two mailings of a forced response and short answer survey (response rate: 680/914, 74%; pediatrics 83%; family practice 72%; obstetrics 71%). RESULTS: Pediatric residents were less likely than family practice [odds ratio (OR), 0.04; 95% confidence interval (CI), 0.02-0.08] or obstetrical (OR, 0.14; 95% CI, 0.08-0.23) residents to be taught circumcision. Training and local custom were rated as important determinants of medical responsibility for neonatal circumcision. Pediatric residents training in programs in which community pediatricians perform circumcisions were more likely to learn circumcision (OR, 39.0; 95% CI, 14.3-110.6) as were obstetric residents (OR, 79.0; 95% CI, 22.4-306.4) training in programs in which community obstetricians perform circumcision. In programs that teach circumcision, pediatric (84%; OR, 3.4; 95% CI, 1.7-7.1) and family practice (80%; OR, 2.7; 95% CI, 1.7-4.2) programs were more likely than obstetric programs (60%) to teach analgesia/anesthesia techniques to relieve procedural pain. Overall, 26% of programs that taught circumcision failed to provide instruction in anesthesia/analgesia for the procedure. Significant regional variations in training in circumcision and analgesia/anesthesia techniques were noted within and across medical specialties. CONCLUSIONS: Residency training standards are not consistent for pediatric, family practice, and obstetrical residents with regard to neonatal circumcision or instruction in analgesia/anesthesia for the procedure. Training with regard to pain relief is clearly inadequate for what remains a common surgical procedure in the United States. Given the overwhelming evidence that neonatal circumcision is painful and the existence of safe and effective anesthesia/analgesia methods, residency training in neonatal circumcision should include instruction in pain relief techniques.
