ABSTRACT
AIM: To determine whether heart rate variability parameters vary between in-utero drug-exposed infants and controls. To determine correlations between Finnegan score and heart rate variability parameters. To differentiate those drug-exposed infants who require treatment from those infants who do not. METHODS: A total of 24 jaundiced control subjects and 25 in-utero drug-exposed infants were enrolled. The Finnegan score and an electrocardiographic rhythm strip were obtained at 4-h intervals. RR intervals (time between consecutive R waves) were manually tabulated from the rhythm strip and analyzed. Time-domain heart rate variability parameters were calculated and analyzed for both groups. RESULTS: Heart rate variability parameters were cumulatively lower over 3 days in in-utero drug-exposed infants compared with controls (p < 0.05). Root mean square of differences of standard deviation of RR intervals on first day of life, and standard deviation of RR intervals, percentage of consecutive RR intervals greater than 50 ms, and root mean square of differences of standard deviation of RR intervals on the second day of life were significantly lower between in-utero drug-exposed infants and control infants. Three out of five parameters were significantly lower in in-utero drug-exposed infants pre-treatment versus post-treatment (p = 0.001, p = 0.0001, and p = 0.021, respectively). Root mean square of differences of standard deviation of RR intervals was able to differentiate in-utero drug-exposed infants requiring opiate therapy and in-utero drug-exposed infants that did not (p = 0.02). CONCLUSION: Heart rate variability analysis can contribute to the management of in-utero drug-exposed infants. Heart rate variability could be used in dose titration.
ABSTRACT
BACKGROUND: Pompe disease is a lysosomal glycogen storage disease (GSDII) characterized by deficiency of acid glucosidase, resulting in lysosomal glycogen accumulation in multiple tissues, with cardiac and skeletal muscles being the most seriously affected. It manifests itself as a spectrum in multiple age groups including infancy, childhood and adulthood. CASE REPORT: We present a case of infantile Pompe disease that was detected at a four month well visit in the presence of hypotonia and failure to thrive. CONCLUSIONS: Pompe disease can be diagnosed clinically by plotting growth parameters and performing developmental screening accurately. Enzyme replacement is the only available medical treatment for Pompe disease. High index of suspicion is necessary in diagnosing Pompe disease.
