Research and Innovations

Vitamin C May Help Prevent Long-Term Complications of Type 1 Diabetes

Diabetic Adolescents Who Received Vitamin C During Infusion Didn’t Experience Endothelial Impairment Caused by Hyperglycemia 

Adenotonsillectomy May Not Resolve Sleep Disordered Breathing in Children with Prader-Willi Syndrome

Not all children with Prader-Willi syndrome receive relief from sleep disorders after undergoing an adenotonsillectomy, suggests a study from Nationwide Children’s Hospital.

Patients with Prader-Willi syndrome are at risk for sleep disordered breathing as a result of their hypotonia, obesity, and/or growth hormone therapy which can cause the tonsils and adenoids to enlarge.  

At Nationwide Children’s Hospital, Prader-Willi patients undergo an annual sleep study and are evaluated for potential adenotonsillectomy if obstructive apnea events are present.  However, there is limited and conflicting data regarding the benefit of adenotonsillectomy in children with Prader-Willi syndrome.

To evaluate the efficacy of adenotonsillectomy in the treatment of sleep apnea in Prader-Willi syndrome, investigators at Nationwide Children’s performed a retrospective chart review.   Thirteen patients met the study criteria and were categorized based on severe, moderate or mild apnea/hypopnea indexes and obstructive hypoxia.

Findings showed that the 11 patients with mild-to-moderate obstructive sleep apnea or obstructive hypoventilation improved or normalized after receiving adenotonsillectomy.  But of the four children with severe obstructive sleep apnea, two normalized after surgery and two continued to have severe apneas.  Unexpectedly, these patients initially had obstructive sleep apnea and after surgery a central sleep apnea was also unmasked. 

These findings suggest that adenotonsillectomy is effective in most children with Prader-Willi syndrome who demonstrate mild to moderate obstructive sleep apnea, but may not be curative in children with severe forms of the condition.

Investigators stress that patients should receive a repeat sleep study six-to-eight weeks postoperatively to detect central apneas that can occur in some Prader-Willi children after upper airway surgical intervention. 

These findings were presented at the Pediatric Academic Societies (PAS) annual meeting April 28 – May 1, 2012. 

Health Literacy and Willingness to Use Online Health Information by Teens with Asthma and Diabetes

This study measured health literacy in a population of teens in treatment for asthma or diabetes.  Finding showed that teens with lower health literacy searched online for health information as often as peers with higher literacy, but were less likely to express the intent to use recommended sites. Belief in the usefulness of a Web site is the strongest attitudinal predictor of intended future use.

Access an abstract of this study: Health Literacy and Willingness to Use Online Health Information by Teens with Asthma and Diabetes. Telemed J E Health. 2011 Sep 23. [Epub ahead of print]

Early Introduction of Growth Hormone Therapy in Prader Willi Syndrome Yields Positive Results

Recent research from Nationwide Children’s Hospital is showing that early introduction of growth hormone (GH) therapy as early as 3-4 months of age in the Prader-Willi Syndrome (PWS) population is beneficial.  Not only has it resulted in improving body composition and motor skills, but is also showing subsequent improvement in cognition and language development. It is hypothesized that infants with earlier motor development (such as sitting upright) develop improved cognitive and communicative skills due to increased stimulatory interaction with surroundings.

Both gross motor and language developmental delay are prevalent in children with PWS. Historically, early milestones in children with PWS are achieved on average of double the normal age (e.g., sitting at 12 months, walking at 24 months, and first words at 2 years) 1. Since growth hormone therapy has consistently demonstrated increased muscle tone, muscle strength, and improved body composition in patients with PWS, it can be surmised that these physical changes may precede improvements in cognitive developmental progress.  Although growth hormone therapy is approved for the treatment of PWS, there are no clear guidelines at what age to initiate GH therapy in these children.

The multi-disciplinary PWS clinic at Nationwide Children’s developed a protocol for early initiation of growth hormone therapy in children with PWS.  Four of the five subjects in the early intervention group had formal developmental assessment screening using the Bayley Scales of Infant and Toddler Development 3rd edition (Bayley-3). These formal developmental assessments indicate that early initiation of GH therapy may contribute to much earlier cognitive and language development than what has been historically demonstrated in patients with PWS.
Findings showed that:

• Patients in the early GH initiation group demonstrated significantly earlier attainment of developmental milestones when compared to the other groups
• Patients who were started on GH therapy prior to 6 months of age had an earlier age of sitting up, talking  and g-tube removal (indicating improved oral muscle strength, coordination, and improved weight gain).
• Bayley developmental scores were nearly normal in cognitive, communication, and fine motor categories. This is in contrast to the mild-moderate global developmental delay generally seen in the Prader-Willi population.

The protocol for early initiation of GH therapy has demonstrated that growth hormone can be safe and effective when initiated in early infancy. In addition, our clinic ensures these patients are receiving aggressive developmental therapies (speech, PT/OT) which presumably has contributed to such superb developmental outcomes.