An exploratory study of the physiological correlates of neurodevelopmental delay in infants with sickle cell anaemia

This study aimed to investigate whether infants with sickle cell anaemia (SCA) are at risk of neurodevelopmental delay, and whether any delay is associated with SCA pathology. Twenty-eight infants (14 SCA; 14 age- and ethnic-similar controls) were assessed longitudinally with the Bayley Infant Neurodevelopmental Screener (BINS) at 3, 9 and 12 months. Transcranial Doppler (TCD) and pulse oximetry (SpO2) measures were recorded longitudinally in SCA infants, and a subgroup of controls. Haemoglobin values were obtained from SCA infants. At each age, SCA infants obtained BINS scores indicative of greater risk of neurodevelopmental delay compared with controls. The number of moderate–high BINS risk scores increased significantly between 3 and 9 months. At 9 months BINS raw scores correlated negatively with TCD velocity and positively with haemoglobin. This exploratory study suggests that SCA infants may be at greater risk of neurodevelopmental delay than previously considered, and may provide the impetus for further research into the very early precursors of cognitive impairment

Pituitary function at long-term follow up of childhood traumatic brain injury

Pituitary dysfunction is a recognised sequel of traumatic brain injury (TBI), occurring in 10-83% of adult patients, but there are few data on the prevalence or natural history in childhood. Our objective was to determine pituitary function in children and young adults at least 4 years after TBI requiring paediatric intensive care unit (PICU) admission. The effects of TBI and hypopituitarism on height, adiposity and quality of life (QOL) were also evaluated. Unselected patients discharged from the regional PICU with TBI (age <18years at injury) from 1999-2004 were recruited. Blood and urine samples were collected for baseline pituitary function testing. Height and weight were measured. Adiposity was assessed by mid-upper arm and waist circumferences and body fat percentage estimation using four-site skinfold thickness and bioelectrical impedance. Auxology and adiposity data were compared to local age and sex matched healthy control data. QOL questionnaires (PedsQL 4.0 and QOL-AGHDA) were completed. Twenty subjects (median age 16.7 years (range 9.2 - 23.3 years), 13 male) of 127 eligible agreed to participate at a median of 6.8 years (range 4.2 - 10.3 years) since TBI; markers of injury were higher in those recruited than those who were not. Biochemical evidence of hypopituitarism was identified in only one case, possibly related to co-morbid pre-existing attention deficit hyperactivity disorder. Height, weight and adiposity were similar to healthy controls. Poor QOL was seen in patients with chronic functional deficits or co-morbidities. Overall, pituitary dysfunction was less prevalent than previous studies in adults and children. This study does not support the use of routine endocrine evaluation of children following TBI

Declaração da guarda para um futuro sustentável das áreas semi-naturais da europa

Item does not contain fulltex

Hypoxic adaptation during development: relation to pattern of neurological presentation and cognitive disability

Children with acute hypoxic-ischaemic events (e.g. stroke) and chronic neurological conditions associated with hypoxia frequently present to paediatric neurologists. Failure to adapt to hypoxia may be a common pathophysiological pathway linking a number of other conditions of childhood with cognitive deficit. There is evidence that congenital cardiac disease, asthma and sleep disordered breathing, for example, are associated with cognitive deficit, but little is known about the mechanism and whether there is any structural change. This review describes what is known about how the brain reacts and adapts to hypoxia, focusing on epilepsy and sickle cell disease (SCD). We prospectively recorded overnight oxyhaemoglobin saturation (SpO 2) in 18 children with intractable epilepsy, six of whom were currently or recently in minor status (MS). Children with MS were more likely to have an abnormal sleep study defined as either mean baseline SpO2 &lt;94 or &gt;4 dips of &gt;4 in SpO2/hour (p = .04). In our series of prospectively followed patients with SCD who subsequently developed acute neurological symptoms and signs, mean overnight SpO2 was lower in those with cerebrovascular disease on magnetic resonance angiography (Mann-Whitney, p = .01). Acute, intermittent and chronic hypoxia may have detrimental effects on the brain, the clinical manifestations perhaps depending on rapidity of presentation and prior exposure.</p