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MCH's 49th Annual Pediatric Postgraduate Course
February 27- March 2
2014
Register today!
www.ppgcpip.com
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Congenital heart disease (CHD) and neurologic impairment.
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Infants with severe CHD (i.e. hypoplastic left heart syndrome, transposition of the great arteries, etc.) requiring surgery in early infancy have a significant risk for a wide range of neurological impairments including cognition, language, behavior, visuo-spatial and motor function abnormalities. The functions are associated with the orbitofrontal region of the brain and magnetic resonance imaging (MRI) studies have given insight into some of the mechanisms involved. Preoperative delays in neural and axonal development, smaller brain volumes, immature structures and a delay in cortical development to outline a few.
Cortical folding and sulcation are an important part of brain development. In a study of 15 infants with severe CHD, preoperative reduction in cortical surface area and gyration is seen in 46% of patients, particularly involving the orbital frontal region.
Journal of Pediatrics
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Intraventricular hemorrhage (IVH) in the preterm newborn (Gene analysis).
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"IVH is a disorder of complex etiology". Recent data have suggested a number of risk genes implicated in coagulation, inflammation and vascular pathways are associated with Grade 3-4 IVH in preterm infants. 11 polymorphic genetics variants in 9 genes were identified following a literature search which had associations with preterm IVH. 7 genotypes from 224 preterm infants with normal cranial ultrasound were then prospectively genotyped and genomic profiles established for those with and without Grade 3-4 IVH.
The MTHFR 1298 > C polymorphism appears to be the only gene variant of the 9 published so far, to be associated with IVH. This gene polymorphism is implicated in endothelial cell injury and alterations in coagulation.
Journal of Pediatrics
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Adenotonsillectomy and pediatric obstructive sleep apnea syndrome (OSAS).
Adenotonsillectomy is thought to be effective ("common knowledge") in OSAS children, improving quality of life, behavior, cognition and polysomnographic measures of OSAS.
The first randomized control trial comparing adenotonsillectomy to "watchful waiting" (in OSAS children 5-9 years of age) indicates that while early surgery does not improve attention or executive function outcomes, it does reduce symptoms and improve behavior, quality of life and polysomnographic findings.
Journal of Pediatrics
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Video Feature
 | | Sleep Apnea in Children |
Good Morning Texas via YouTube
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Treatment of head lice- treatment with a non-toxic, non-traditional method.
Apparently head lice are the second most common problem afflicting children after the common cold. Over-the counter medicated shampoos and the use and re-use of traditional lice killing treatments cause anxiety among parents fearing their child's exposure to essentially pesticides and substances with potentially serious side-effects.
Homeopathic remedies like dimeticone salts (a silicone oil which forms a slick lubricating coating on each strand of hair), herbs and other essential oils are appealing because they are non-pesticides. Dimeticone "smothers" lice to death and facilitates their removal using a fine toothed comb.
A study comparing the single application of 4% dimeticone liquid gel to two applications of 1 % permethrin cr�me rinse shows that a one 15 minute application of dimeticone gives an approximate 70% success rate, compared to 15% for the permethrin rinse.
Journal of Pediatrics
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What is the benefit/risks of higher vs. lower oxygen saturation (O2sats) for retinopathy of prematurity (ROP) in preterm infants <28 weeks gestation?
Among 2,448 preterm infants recruited for a "high" (91-95%) vs. "low" (85-89%) O2sat trial, 1,187 belonged to the "low" O2sat group. These infants had a decreased rate of ROP, an increased rate of necrotizing enterocolitis and significantly higher death rate.
Journal of Pediatrics
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Tongue fasciculations in the new born.
Spinal muscular atrophy (SMA) is a term used for both the autosomal recessive gene disorder (SMN1 gene) on chromosome 5, the deficiency of which results in neuronal death, and a general label for a large number of genetic disorders which result in a slow progression of weakness without sensory loss. There are 4 types:
1. Infantile (Werdnig-Hoffman Disease - onset 0 to 6 months)
2. Intermediate (Dubowitz disease - onset 6 to 18 months)
3. Juvenile (Kugelberg Welander - onset > 18 months)
4. Adult onset (onset in adulthood)
A recent case report of a baby born to non-related parents reminds us of some of the molecular genetic and clinical features of the most severe Infantile form of SMA. These include polyhydramnios, late decreased fetal movements, breathing difficulties with a low 1 minute Apgar score requiring ventilator support, progressive general weakness, profound hypotonia with absent deep tendon reflexes, tongue fasciculations (which may also occur in hypoxic-ischemic injuries, M�bius syndrome and Pompe disease) and early death (usually within the first 3 months of life.
Journal of Pediatrics
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