Sleep Disturbance in Children with Fetal Alcohol Spectrum Disorder and the Relationship to the Neurodevelopmental Profile.

OBJECTIVE: Sleep disturbance is an important feature of fetal alcohol spectrum disorder (FASD). We sought to describe sleep patterns in school-aged children with FASD, in comparison with a typically developing community group, and investigate the relationship between sleep and neurodevelopmental profiles. METHOD: The FASD cohort (N = 36) was recruited from a tertiary Australian FASD diagnostic center, and the typically developing group (N = 36) was previously recruited as a control cohort for a separate study. Sleep disturbance was assessed with the caregiver-completed Sleep Disturbance Scale for Children (SDSC) questionnaire. Neurodevelopmental assessment results for the 10 domains impaired in FASD were used for correlations with sleep disturbance. RESULTS: In the FASD group, 80% of children scored above the SDSC cutoff, compared with 22% of the control group (p < 0.001). Statistically significant group differences were seen for all 6 subscales of the SDSC (p < 0.05). The most frequently affected domains in the FASD group related to difficulties with initiating and maintaining sleep (58%), sleep-wake transition disorders (44%), and disorders of arousal (42%). A statistically significant relationship was not found between sleep and the severity of neurodevelopmental impairment or impairment of a particular domain, acknowledging the limitations of our small sample size. Half of the FASD sample (52%) were taking a pharmaceutical agent to support sleep, which was not associated with lower SDSC scores. CONCLUSION: In this small study, sleep disturbances were frequently reported by carers of children with FASD, independent of the severity of their neurodevelopmental impairments. Persistent sleep disturbance despite the use of sleep medications highlights the need for prospective studies exploring sleep interventions in this population. Integration of behavioral sleep medicine into management is recommended for all children with FASD.

Hip surveillance in Tasmanian children with cerebral palsy.

BACKGROUND: Children with cerebral palsy (CP) are at risk of hip subluxation. Over time, subluxation can lead to dislocation, deformity and pain. Hip surveillance in the form of an X-ray and clinical examination of this 'at risk group' can identify early subluxation. Early subluxation can be treated, preventing hip dislocation in many cases. Hip surveillance in CP commenced in Tasmania in 1992. AIMS: To audit the hip surveillance data to date, perform a literature review to understand the emerging evidence for prevention and management of hip subluxation in CP and update hip surveillance guidelines. METHODS: New guidelines were written and distributed, and an audit of the previous 12 years performed by review of medical files and X-rays. RESULTS: Two hundred and eighteen children had been involved in the hip surveillance programme. Fifteen cases of dislocation were recorded in this time. The incidence of subluxation and surgery, as well as the gross motor function classification system (GMFCS) level, was recorded. CONCLUSION: Data from Tasmania showed a similar incidence of hip subluxation according to GMFCS level, and frequency of different surgical interventions as other recent audits. Some children with minor subluxation improved without orthopaedic intervention once weight bearing occurred, which had not before been appreciated. Migration percentage alone is not adequate to fully describe the outcome of hip subluxation. More appropriate measures of outcome in terms of quality of life for children with CP need to be developed.

Vincristine treatment of nephrotic syndrome complicated by Kimura disease.

Kimura disease is a rare inflammatory condition of unknown aetiology. It typically presents in young Asian males with the triad of non-tender subcutaneous swellings in the head and neck region, peripheral eosinophilia and raised serum IgE. About 16% of cases have associated renal disease. We present the case of a 10-year-old boy with a past history of steroid responsive, frequently relapsing nephrotic syndrome who developed a right submandibular swelling and eosinophilia. Kimura disease was diagnosed on the basis of clinical and histological findings. The condition recurred during relapses of nephrotic syndrome. Because of poor adherence with oral medication, our patient was treated with intravenous vincristine with synchronous remissions of his nephrotic syndrome and Kimura swellings on each occasion.