Changes in cerebrospinal fluid homovanillic acid in children with Ondine's curse.

The cerebrospinal fluid (CSF) concentrations of three acid monoamine metabolites, two purines, and a group of amino acids were determined in two children with chronic central alveolar hypoventilation (Ondine's curse). The levels of all assayed neuroactive substances, metabolites, and amino acids, with one exception, were normal compared to an age-matched group of neurologically healthy children. The levels of the dopamine metabolite homovanillic acid in the children with Ondine's curse were approximately 2.4 times higher than expected for age range. The present findings may indicate a link between central nervous system dopamine activity and chronic central alveolar hypoventilation. Among other possible explanations, the changes seen might represent a primary alteration in dopamine activity or may reflect a change in dopamine turnover resulting from the chronic hypoventilation.

Non-invasive imaging techniques in pediatric cardiology: impact on clinical decision-making.

To plan the treatment of congenital heart malformations, a detailed knowledge of anatomy and hemodynamics is essential. This has previously been obtained by heart catheterization and angiocardiography. During the past 20 years, several non-invasive techniques, such as echocardiography with Doppler, magnetic resonance imaging and isotope techniques have evolved. They have profoundly changed the way we practise pediatric cardiology. For the initial investigation of a patient with suspected heart disease, echocardiography is the routine method. For the less experienced, tele-transmission to a pediatric cardiology center can be used to complement to investigation. In the preoperative investigation, echocardiography with Doppler plays an important role. In some situations, such as aortic lesions or complex cardiac malformations, magnetic resonance imaging is useful. Isotope methods have been used mainly for quantification of the degree of left-to-right shunts. During a 5-year period, 695 infants and children were operated on for a heart malformation: 40% were operated on without previous heart catheterization. For patients needing surgery before 1 month of age, 58% could be operated on based on non-invasive data alone.

A postoperative follow-up study of infantile coarctation of the aorta.

Follow-up investigations were performed in 16 patients operated on for coarctation during infancy. The follow-up period ranged from 4.5 to 11 years (median 5.5 years). Four different surgical techniques were used: resection with end-to-end anastomosis (REE) (4 patients), subclavian flap aortoplasty (SFA) (10 patients), patch aortoplasty (1 patient) and resection and SFA (1 patient). One patient developed recoarctation (6%). She had been operated on by REE at 7 days of age. The other three patients operated on by REE had equal pulses in the arms and legs; none had hypertension and all had normal arm/leg pressure gradients at rest. Seven (58%) of the 12 patients operated on by SFA or aortoplasty had weak radial pulses in the left arm but no limitation of left arm function. The left a rm showed a normal bone age but was smaller and shorter than the right arm in 9 (81%) of the patients. None of the patients operated on by SFA had hypertension and the arm/leg gradient at rest was normal.