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Speciality Spotlight
Paediatrics
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O Dammann, EN Allred, N Veelken (Children’s Hosp, Boston; Harvard Med School, Boston; Gen Hosp Heidelberg, Germany)
Increased Risk of Spastic Diplegia among Very Low Birth Weight Children After Preterm Labor or Prelabor Rupture of Membranes.
J Pediatr 132: 531-535, 1998.
Among very preterm and very low birth weight (VLBW) children, spastic diplegia is the most prevalent subtype of cerebral palsy.
A previous study concluded that preterm birth and white matter damage were caused by maternal infection during pregnancy and that this may have resulted in spastic diplegia. It was also found that microbial invasion of the amniotic cavity was detected in 20% women who gave birth after preterm onset of labour and in 50% who had prelabour rupture of membranes.
The authors conclude that the risk of diplegia among VLBW babies is increased in children born after preterm onset of labour and prelabour rupture of membranes.
The infectious processes leading to both these conditions may be related to spastic diplegia.
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SR Wiener-Vacher, K Mazda (Hopital Robert Debre, Paris)
Asymmetric Otolith Vestibulo-ocular Responses in Children with Idiopathic Scoliosis.
J Pediatr 132: 1028-1032, 1998
About 3% children have idiopathic scoliosis (IS). Disequilibrium of the vestibulospinal pathways controlling the neck and trunk may be involved. Past studies tested this hypothesis based only on semicircular canal function.
In this study of 30 patients with IS, evaluation of otolith function was also included. MR images of the brain, brainstem and medulla were normal. Each individual underwent complete vestibular testing and off-vertical axis rotation to assess otolith vestibular function.
Two thirds of the patients with IS had greater asymmetry in otolith responses than controls. The authors conclude that peripheral dysfunction of otolith receptors seems a likely explanation for IS. They postulate that patients with IS have an imbalance of the central otolith system and this imbalance creates asymmetry in the tonic activity of the spinal muscles during rapid growth such as at puberty.
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FG Sheikh, K Pahan, M Khan, et al (Med Univ of South Carolina, Charleston)
Abnormality in Catalase Import Into Peroxisomes Leads to Severe Neurological Disorder.
Proc Natl Acad Sci U S A 95:2961-2966, 1998.
Functional abnormality of peroxisomes leads to progressive neurologic disorders.
Human skin fibroblast cell lines from patients with multiple peroxisomal dysfunctions and normal transport and location of PTS1- and 2-containing proteins (PTS= peroxisomal targeting signal sequence 1 & 2) but with abnormal localization of catalase were studied.
There is a PTS-1 and 2-independent pathway in fibroblasts that is used to transport catalase into human peroxisomes. Impairment of this pathway causes catalase to be improperly localized in the cytosol, instead of being localised in peroxisomes. Without catalase, H2O2 accumulates and suppresses the activities of other peroxisomal proteins, which leads to peroxisomal disease with severe neurologic malfunction.
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JM Freeman, EPG Vining, DJ Pillas, et al (Johns Hopkins Med Institutions, Baltimore, Md)
The Efficacy of the Ketogenic Diet – 1998: A Prospective Evaluation of Intervention in 150 children.
Pediatrics 102: 1358-1363, 1998.
The Ketogenic diet (high fat low protein, low CHO diet) first used in the 1920s, is designed for use in children, with difficult-to-control seizures.
The current role of this diet for children with refractory seizures (to modern antiepileptic drugs and surgery) was evaluated in a prospective study of 150 children.
The children were followed for at least one year or until they went off the diet.The authors conclude that the ketogenic diet appears to reduce seizure frequency for children with refractory seizures.
