This study demonstrates that peanut allergy is outgrown in about 21.5% of patients. Patients with low PN-IgE levels should be offered a peanut challenge in a medical setting to demonstrate whether they can now tolerate peanuts.
Obese adults have an increased prevalence of pulmonary disorders. Although childhood obesity is a common problem, few studies have evaluated the pulmonary complications of obesity in the pediatric population. We, therefore, performed pulmonary function tests (PFTs), polysomnography, and multiple sleep latency tests (MSLTs) in 22 obese children and adolescents [mean age, 10 2 5 (SD) years; 73% female; 184 5 36% ideal body weight], none of whom presented because of sleep or respiratory complaints. PFTs were normal in all but two subjects. Ten (46%) subjects had abnormal polysomnograms. There was a positive correlation between the degree of obesity and the apnea index (r = 0.47, P < 0.05), and an inverse correlation between the degree of obesity and the S,02 nadir (r = -0.60, P < 0.01). The degree of sleepiness on MSLT correlated with the degree of obesity (r = -0.50, P < 0.05). We conclude that obese children and adolescents have a high prevalence of sleep-disordered breathing, although in many cases it is mild. Obstructive sleep apnea syndrome (OSAS) improved following tonsillectomy and adenoidectomy. We recommend that pediatricians have a high index of suspicion for OSAS when evaluating obese patients, and that polysomnography be considered for these patients.
Sapropterin dihydrochloride, a synthetic, stable form of the tetrahydrobiopterin cofactor of phenylalanine hydroxylase, has been shown to reduce plasma phenylalanine (Phe) levels in a significant portion of patients with phenylketonuria (PKU). When we undertook introducing this medication to our PKU clinic population, the challenges of recalling and reconnecting with a variably treated and variably compliant patient population became apparent. We offered a trial of sapropterin to all of our clinic patients with PKU. In order to determine responsiveness, we used a 2 tier dose escalation protocol. After diet records were taken, and baseline plasma Phe levels were established, a 7-day trial of sapropterin at 10 mg/kg/day was started. At day 8, plasma phenylalanine levels were measured. Patients were considered to be responders if they had a 30% reduction in plasma Phe. If they did not respond, the dose of sapropterin was increased to 20 mg/ kg/day, and levels were rechecked again in 8 days. Patients who were not responders at this time continued sapropterin for a total of 30 days and had Phe levels checked one last time. Patients who were responders and who were on a Phe restricted diet underwent gradual liberalization of their diet to the maximum tolerated natural protein intake while still maintaining plasma levels in the acceptable treatment range of 120-360 µmol/L. In our population, 36/39 patients with hyperphenylalaninemia (HPA) who were offered a trial of sapropterin elected to start sapropterin. Five of 36 patients were non-adherent with diet records and/or medication doses and we were unable to determine if they were responders. We were unable to categorize 2 of 31 of the patients who completed the trial as responders due to dietary issues, though they were probably responders. Of the 29 patients who completed the sapropterin trial and we could categorize, 18/29 (62%) were determined to be responders. Patients were classified based on their off-diet diagnostic plasma phenylalanine levels as classical PKU (>1200 µmol/L) and variant PKU (>400and <1200 µmol/L). The group with variant PKU had a 100% response rate, and patients with classical PKU had a 27% response rate. For the patients in the responder group who were on Phe restricted diet, we were able to liberalize most diets, in two cases to unrestricted protein intake. We also had unexpected beneficial findings in our clinic experience, including positive behavioral improvements in an adult severely affected by untreated PKU. Even in patients who were not considered to be responders, the introduction of sapropterin provided a tool to reconnect with patients and re-introduce beneficial dietary measures.
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