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Biomedical subjects

Robert Listernick

Publications and source records attributed to Robert Listernick.

At least 55 records · Page 3Linked to original sources
PubMed2003

Optic pathway gliomas in neurofibromatosis type 1: the effect of presenting symptoms on outcome.

Children with neurofibromatosis type 1 (NF1) may present with optic pathway gliomas (OPG) that can progress to visual loss or other neurologic symptoms. These tumors may become evident either as a result of patient signs or symptoms or as an incidental finding on "baseline" neuroimaging studies. In an attempt to determine if there were differences between symptomatic and asymptomatic children with OPG, a retrospective cohort study of ninety children with NF1 and OPG was performed using data from two large NF1 referral centers. Age at diagnosis, presenting symptoms, tumor location, associated features, and clinical response were assessed for children who were initially symptomatic from their OPG (n = 51) and compared to similar data of asymptomatic children whose tumors were incidentally discovered by MRI (n = 39). There were no differences in age at presentation, tumor location, NF1-associated symptoms, or clinical response between the groups. Initially symptomatic children were much more likely to require treatment (OR: 14.8, 95% CI [1.9-116.7]) than those with incidentally discovered, asymptomatic OPG. Although 36% of OPG were diagnosed in children over the age of 6 years, none received prior neuroimaging and only two children had previously normal eye examinations, suggesting that the vast majority of OPG in this group were longstanding, undiagnosed tumors. Based on these findings, we do not advocate "baseline" MRI in children with NF1, but strongly recommend that all children of the age 10 years and younger with NF1 have complete annual ophthalmologic evaluations.

Child↗
PubMed2003

Segmental neurofibromatosis in childhood.

Segmental neurofibromatosis refers to individuals who have manifestations of neurofibromatosis type 1 (NF-1) limited to one area of the body. It results from a post-conceptional mutation in the NF-1 gene leading to somatic mosaicism. Although it is generally considered a rare condition, this report of 39 children with segmental NF-1 demonstrates that it is commonly seen in a pediatric NF-1 referral center. The mean age at diagnosis was 7.8 years (range: 2-25 years). Twenty-nine patients had only pigmentary manifestations of segmental NF-1, including seven who had only café-au-lait macules and 22 who had café-au-lait macules and freckling. Two patients had isolated plexiform neurofibromas; a third patient had a plexiform neurofibroma of the eyelid in addition to ipsilateral dysplasia of the sphenoid wing and Lisch nodules. A 12-year-old girl had an isolated tibial pseudarthrosis. An 8-year-old boy had an isolated optic pathway tumor, which behaved both biologically and radiographically as an NF1-associated tumor. While most children with segmental NF-1 have only localized pigmentary changes, some children will have isolated plexiform neurofibromas, pseudarthroses, or optic pathway tumors. Accurate diagnosis of segmental NF-1 is crucial for both management and genetic counseling.

Adolescent↗
PubMed2002

Persistence and emergence of anemia in children during participation in the Special Supplemental Nutrition Program for Women, Infants, and Children.

CONTEXT: The prevalence of iron-deficiency anemia in children has decreased owing to the provision of iron-containing infant formula and cereal and food vouchers to children enrolled in the Special Supplemental Nutrition Program for Women, Infants, and Children (WIC). OBJECTIVE: To determine the prevalence of anemia and changes in anemia status in children receiving WIC supplementation. DESIGN: Retrospective cross-sectional and longitudinal analysis of information on WIC participants. Two definitions of anemia were considered separately: Anemia1 and Anemia2, the latter using a more stringent definition of anemia to avoid misclassification. PARTICIPANTS: Consecutive cohort of 7053 infants and children aged 6 to 59 months. MAIN OUTCOME MEASURES: Prevalence of anemia by age and race or ethnicity and relationship between anemia and sex, birth weight, and weight-for-height z score. RESULTS: Infants aged 6 to 8 months were 3.3 times more likely to be anemic than children aged 36 to 59 months. There was no association between anemia and race, birth weight, sex, or weight-for-height z score. Anemia rates were approximately halved in the more stringently defined Anemia2 group. Among children seen for at least 3 visits (n = 2926), 8.5% developed anemia and 19.1% of initially anemic children remained anemic; an additional 6.6% developed anemia at a third visit after having had 2 normal hemoglobin measurements. CONCLUSIONS: Anemia was common in WIC participants, with infants at highest risk. The diagnosis of anemia in black children depends on the cutoff value used. Despite ongoing receipt of WIC benefits, many children develop anemia or remain anemic. Implementation of mandatory follow-up of all anemic infants by WIC or health care providers may be warranted.

Adult↗