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Weekly Updates in Pediatrics

March 2012 - Current Updates in Pediatrics

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March 2012 

1. How “conscious” are we?
In a study of levels of consciousness (a subjective experience) three distinct decreased stages have been described: “Coma” “the vegetative state” (VS) and “minimally conscious state” (MCS). Differentiation is based on behavioral criteria. “Coma” patients cannot be aroused while those in “VS” are awake without conscious awareness. (Both are considered to be unconscious). “MCS” patients are believed to have fluctuating consciousness and may have a minimal ability to understand and/or communicate.
In an intriguing publication patients who are behaviorally unresponsive have been found to generate EEG responses. Consciousness may therefore need to be subdivided as a subjective experience (eg. “feeling pain”) and an “ability to be aroused.”
Patients’ level of consciousness being defined by failure to “arouse” or “follow commands” (Coma and VS) may not in fact adequately define the ability of the brain to experience complex aspects of perception.
Lancet, Published Online Nov 2011
 
2. Diet and the prevention of neural tube defects (NTD)
While the beneficial effect of Folic acid supplementation and food fortification has been shown to be beneficial in reducing NTDs, it does not prevent all NTD’s.
A multicenter, population based case-control study of food-frequency data was used to calculate the “Mediterranean Diet Score” (a diet rich in olive oil, legumes, unrefined cereal, fruits, vegetable, fish; moderate consumption of wine and dairy products) and a “Diet Quality Index-DQI (with 10 dietary variables) to assess the effects of other nutritional factors which may be valuable in preventing NTDs.
Increasing maternal diet quality based on either index is associated with reduced NTD incidence. 
Improvements in maternal periconceptual diets may be of value to further reduce the risk of major birth defects.
 
3. Achondroplasia.
Achondroplasia in over 95% of cases is caused by mutations in the gene encoding fibroblast growth factor receptor type 3. While inherited in an autosomal dominant pattern, 80% of patients have averaged sized parents. (high new mutation rate).
Children with Achondroplasia have a different schedule of developmental milestone attainment and condition-specific charts should be used to monitor their growth. Weight gain is frequently a problem and appropriate eating and lifestyle habits should be encouraged.
As complications of this condition are broadly-based, a multidisciplinary team is needed to best manage their ongoing care.
Arch Dis Child 2012; 97:129-134
 
4. Encephalitis in children
Encephalitis is a relatively uncommon acute neurologic syndrome in children and has a diverse etiologic background. Frequently it affects previously healthy children. Signs and symptoms are often non-specific. Causes can be broadly divided into:
1. Those associated directly or indirectly with infectious agents, or
2. Those caused by other inflammatory pathologies which include immune mediated encephalitis and acute disseminated encephalomyelitis.
Frequently a cause is never identified. This reference is an excellent article on the subject, for those interested in an extensive review on encephalitis.
Arch Dis Child 2012; 97:150-161

5. Asthma and birth weight
Even though asthma is the most common chronic disease in children, etiological factors are still not clearly understood. It has previously been thought that perhaps fetomaternal health may play a role by affecting programming of immunological and pulmonary development; lower birth weight appears to increase the risk of the subsequent development of asthma.
A study of 687,194 live born singleton infants categorized by birth weight and followed for six years (in an integrated healthcare system) was undertaken to assess any association between birth weight and asthma.
Compared to normal birth weight infants babies born with birth weights 4.6kg to 6.5kg appear to have a lower risk for asthma, while a trend to increased incidence of asthma is observed in those babies with birth weight > 6.5kg. It appears therefore that the extremes of birth weight both increase risk of asthma.
Arch Dis Child 2012; 97:169-171
 
6. Microarray testing (genome analysis)
For children with non-syndromal global developmental delay (GDD)
microarray testing provides whole genome analysis superior to conventional karyotyping. For selected patients with GDD and negative clinical and laboratory testing, a positive diagnosis may be achieved in 16% with genome analysis.
Many clinicians believe microarray testing to be too expensive for initial routine use. This study performed on 114 Canadian children with GDD suggests otherwise.
Arch Dis Child 2012; 97:120
 
7. Obesity in childhood-implications for future health
Childhood obesity is a global epidemic. In the USA the prevalence has tripled since 1980 with approximately 12.5 million being obese. Obesity increases the risk for a variety of diseases. Obese adolescent children are now developing type II Diabetes, fatty liver disease and early stage atherosclerotic changes which may vary according to race/ethnicity.
While recent data suggests recent improvement in arterial blood pressure and lipid profile measurements, a rising fasting blood glucose noted, is of concern. Further studies are desperately needed.
 
8. Partial IgA deficiency and joint involvement
A small retrospective clinical study of 13 partial IgA deficient children who had a history of frequent infections were assessed to identify the frequency of joint involvement. Eleven of 13 patients complained of either joint pain, swelling or morning stiffness.
Six were diagnosed with definitive rheumatic disease. Partial IgA deficiency may present with joint signs and symptoms.

9. Treatment of Phimosis
A symptomatic tight foreskin is a common pediatric problem in boys which often requires circumcision.
A prospective study of 260 such boys (0-15 yrs of age) with phimosis were treated with betamethasone diproprionate (0.05%) application applied to a gently stretched prepuce, twice daily. Follow up occurred at weeks 1, 2 and 4, and at 6 months.
Successful treatment with betamethasone application occurred in 91% of boys by the end of 4 weeks. Most had a positive response by the end of the first week and very few after two weeks of treatment. 17.8% had a recurrence (mean 25.4 months) and 23% finally required a circumcision. 

10. Investigations following a first urinary tract infection (UTI)
Abnormal ultrasound (US) or Tc-dimercaptosuccinic acid (DMSA) scans has a sensitivity of 100% to detect high-grade reflux.
The routine value of identifying vesicoureteral reflux (VUR) by voiding cystourethrogram (VCUG) does not appear to be required after a first febrile UTI with a normal US or DMSA scan. (the use of routine nuclear renal scans exposes children to both higher radiation doses and imaging costs.) 
 
11. 2 year vs 5 year mental/motor assessment of very low birth weight (VLBW) (< 28 week/<1,000gms) infants
It appears that neurological and motor assessments performed at 2 years of age on VLBW infants, particularly in those without major impairments, maybe of limited value in predicting cognitive and motor function at 5 years of age. 
12 Lens abnormalities in children
A retrospective analysis of 1047 children, mean age 6.35 years seen in a “lens clinic” was undertaken to identify the causes of lens abnormalities found in young children.
“Development” and “post-traumatic” cataracts account for 75% of children with lens abnormalities, seen. Other abnormalities found include; cataract with retinal detachment, subluxated lens, micro/spherophakia etc. 
13. Tanning device use in male high school students
It appears that approximately 6.7% of male high school students place themselves at risk for developing melanoma by using an indoor tanning device. (25.4% of female students do the same!)
Adjusted analysis of cross-sectional male student data indicates associations between indoor tanning and;
1. Having ever taken steroids without prescription.
2. Unhealthy weight control practices.
3. Binge drinking.
4. Excessive fruit/vegetable consumption.
5. Attempted suicide.
6. Playing on at least one sport team.
Appearance and health related behavior identification may assist in reducing the practice of indoor male student tanning.

14. A cause of recurrent gross hematuria-“Nutcracker Syndrome”
Usually the assessment of gross hematuria includes an evaluation of a long list of glomerular and extraglomerular etiologies.
An interesting case report of a young 10 year old boy who presented with 6 bouts of painless gross hematuria with no other history or abnormality on physical examination reminds us of an easily missed and underreported condition.
Nutcracker Syndrome results from the compression of the left renal vein between the aorta and the superior mesenteric artery (other variants exist).
While there may be a wide variation in clinical manifestation, hematuria is a frequent presenting feature (stenting or reimplantation of the renal vein are therapeutic choices). 

15 “Plus Disease” diagnosis in Retinopathy of Prematurity (ROP)
The presence of “Plus Disease” in ROP appears to be important I deciding which patients require treatment. “Plus Disease” is usually defined as patients having at least 2 of their 4 retinal quadrants showing increased vascular blood flow (dilatation/tortuosity) around their optic nerve. “Plus Disease” is also defined by a standard published photograph selected 20 years ago. The diagnosis appears to be both subjective and qualitative.
Four main computer-based systems have recently been used to objectively analyze vessel dilatation and tortuosity in “Plus Disease” in ROP. Some show promise to improve the diagnosis. 

16 Serum lipid levels in childhood
The National Health and Nutrition Examination Survey (1999-2008) was utilized to estimate total cholesterol (TL), high-density lipoprotein (HDL), low-density lipoprotein (LDL) and triglyceride levels by age and sex in 7,681 healthy children.
In boys TL, HDL and LDL levels peaked at age 8-12 years and at 7-11 years for girls. Triglyceride do not appear to change across age groups for boys, while girls have a peak level.
 
17 Bilious vomiting does not rule out Hypertrophic Pyloric Stenosis (HPS).
Charts of 354 intraoperatively diagnosed infantile HPS patients (median age 4wks, 6 days) were retrospectively reviewed to determine the incidence of “bilious vomiting”
1.4% of infants with HPS have bilious vomiting. 
These babies have significantly smaller pyloric thickness measurements on ultrasound. 

18 Do State policies that regulate the sale of sugar-sweetened soda in schools reduce consumption?
Banning all sugar-sweetened beverages in school, adjusted for race/ethnicity, poverty status, locale, State obesity prevalence and State clustering appears to reduce in school access, BUT NOT overall consumption. 

19 “Mini-tablet” use in young children
A study of 60 children, 0.5-6 yrs of age enrolled in a prospective, open random, two-way exploratory pilot study was utilized to compare the ability of these children to swallow an uncoated drug free nini-tablet, 2mm in diameter (with a beverage of their choice) vs swallowing a liquid formulation.
Immediate swallowing or chewing of the mini-tablet followed by swallowing, was higher (or equal) to that of syrup. 
Even very young infants (6-12 months) appear fully capable of swallowing a mini-tablet.

20 Outcome in preterm infants infected via breast milk with human cytomegalovirus (HCMV)
A small group of 21 HCMV preterm infants was assessed neurologically at school age and compared to similar group of uninfected children to document the medium term effect on neurodevelopment and hearing. 
In both groups cognitive and motor function were within normal limits, however the HCMV infected children performed less well. No child in either group had hearing difficulties. HCMV infection via breast milk may result in nuanced neurodevelopmental changes.

21 Cardiac biomarkers in neonatal hypoxia
The cardiac dysfunction that occurs following a perinatal hypoxic-ischemic insult may be assessed by measuring Troponin-T, Troponin-I and brain natriuretic peptide. 
How these results reflect neurodevelopmental outcome remains to be determined. 

22. Outcome factors following Traumatic Brain Injury (TBI)
Two different and interesting prospective studies of the cognitive, functional, social and behavioral skills and IQ abilities of children who sustained an acute TBI at a young age (2-7 years) were recently reported. All children were subsequently assessed and compared to a comparable normal group at 12mths, 30mths, 40mths and 10yrs post-injury
It appears that increasing severity of brain injury results in increasing neurobehavioral deficits, with older and less severely injured children recovering normally. For the young mild/moderate TBI child, cognition, functional skills and IQ performance appears to be significantly affected with improvement in verbal skills occurring over a 10yr period.
A family with good social and behavioral functions
(Environmental factors!) positively influences outcome in TBI children. 

23 Bronchoscopic findings in children with a chronic wet cough
Chronic cough is variously defined as a persistent cough lasting more than 2-4 weeks. It is a symptom not a disease. The etiology varies to some extent, on the age of the child.
In an interesting retrospective review of the charts of 197 children who presented with a chronic wet cough, 109 were 0-3yrs of age and 30.3% had laryngo or trachea malacia. At bronchoscopy purulent bronchitis was found in 56% of children and bronchoalveolar lavage was positive for bacteria in 46%.
In the absence of an alternative diagnosis chronic cough due to bacterial bronchitis appears to be not as uncommon as often thought.  

24 Outcome in severe Intraventricular hemorrhage (IVH) with post-hemorrhage ventricular dilatation (PHVD)
Preterm infants (<30wks gestation) who have PHVD without parenchymal involvement may have a normal neurodevelopmental outcome. A study of 32 surgically treated PHVD infants was undertaken to assess neurodevelopmental outcome at 5-8yrs following neurosurgical intervention.
59% had no impairment with no child with a grade III IVH developing cerebral palsy. (53% with grade IV IVH developed cerebral palsy). Subtle motor abnormalities (39%) and IQ of <85 was found in 29% of children. 
The majority of infants with severe IVH/PHVD requiring surgery will have no subsequent neurodevelopmental problems. 

25 Does it matter is chemotherapy is delayed over a weekend?
The effect of a few days’ delay in treating acute lymphoblastic leukemia (ALL) in children has not been fully investigated.
A study of ALL children admitted and diagnosed on a Friday afternoon was retrospectively analyzed.
Friday admission did not significantly delay chemotherapy initiation, nor was there an association between treatment delay days, duration of hospitalization, time to relapse or death.http://journals.lww.com/jpho-