Neuropsychological functioning in preschool-aged children undergoing evaluation for organ transplant.

Objective: The purpose of this study was to review the current literature on neuropsychological functioning in two groups of children requiring organ transplants (liver or heart) and present recent clinical data collected through the liver and cardiac transplantation programs at a large pediatric academic medical center.

Method: Data included in this study came from 18 patients who completed evaluations for heart transplant (n = 8) or liver transplant (n = 10) between the ages of 2 and 6 years (inclusive). Measures examining neurocognitive, emotional-behavioral, and adaptive functioning were collected as part of standard pre-transplant clinical neuropsychological evaluations.

Neurodevelopmental functioning in children being evaluated for heart transplant prior to 2 years of age.

Cardiac defects represent the most common type of birth defect and children with these abnormalities are known to experience a variety of developmental and neuropsychological delays. Children receiving heart transplants may be at the highest risk for delays as they tend to represent the most severely ill patients with heart disease. This study investigates neurocognitive and adaptive functioning in 20 children under 2 years of age who were undergoing evaluation for heart transplant, comparing their performance to that of normative samples.

Cognitive outcomes and familial stress after cochlear implantation in deaf children with and without developmental delays.

Objective: The benefits of cochlear implantation for children with developmental delays (DD) often are unclear. We compared cognition, adaptive behavior, familial stress, and communication in children with and without DD.

Study Design: Retrospective review.

Developmental assessment of infants with biliary atresia: differences between boys and girls.

Objective: The aim of the present study was to investigate whether male and female infants with biliary atresia (BA) differ cognitively and to confirm previously documented developmental lags in infants with BA before liver transplantation.

Methods: With the Mullen Scales of Early Learning, we examined 21 female and 12 male infants (ages 3-20 months) with BA, comparing scores across indices by sex and correlating Mullen Scales of Early Learning scores with standard clinical and biochemical parameters.

Results: Overall, both boys and girls were found to be vulnerable to developmental lags in the areas of expressive language (EL) and gross motor skills.

Nonverbal cognitive development in children with cochlear implants: relationship between the Mullen Scales of Early Learning and later performance on the Leiter International Performance Scales-Revised.

Methodologically, longitudinal assessment of cognitive development in young children has proven difficult because few measures span infancy through school age. This matter is further complicated when the child presents with a sensory deficit such as hearing loss. Few measures are validated in this population, and children who are evaluated for cochlear implantation are often reevaluated annually.

Language and motor skills are impaired in infants with biliary atresia before transplantation.

Objective: To test the hypothesis that in very young patients with biliary atresia, cognitive deficits are apparent before transplantation.

Study Design: With the Mullen Scales of Early Learning (Mullen), we examined 15 infants (mean age, 7.8 months) with biliary atresia, correlating Mullen scores with standard clinical and biochemical parameters.

Neurocognitive functioning of a child with partial trisomy 6 and monosomy 21.

This case study describes the neurocognitive presentation of a child with identified genetic abnormalities of trisomy 6 and monosomy 21 who was evaluated as part of a standard medical protocol for cochlear implantation following diagnosis of profound sensorineural hearing loss. This child received neurocognitive testing prior to cochlear implantation and approximately 12 months post-activation of his cochlear implant. While he has not fully developed oral language, his presentation suggested improvement in overall skills since the activation of the cochlear implant; however, less than would be expected for a typically developing child.

Cognition in children with sensorineural hearing loss: etiologic considerations.

Objectives: A considerable amount of literature has documented the impact of hearing impairment on spoken language skills in deaf children referred for cochlear implantation. Critical areas of neurocognitive development in the acquisition of visual (manual) language also appear to be impacted, although the evidence is less robust. The present study focused on the development of visual and fine motor skills in a sample of preschool-age children diagnosed with sensorineural hearing loss with no known neurologic conditions (n=36).