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OBJECTIVE: To noninvasively measure arterial wall thickness in a group of patients with Williams syndrome (WS). METHODS: High-resolution, real-time B-mode ultrasonography was used to examine the carotid arteries of 20 patients with WS (ages 7 months to 24.9 years) and 25 control subjects (ages 2.5 years to 25.5 years). RESULTS: The mean combined intimal-medial wall thickness of the patients in the WS group was 0.86 mm +/- 0.08 mm compared with a mean of 0.54 mm +/- 0.05 mm in the control subjects (p < 0.0001). Within the WS group, arterial wall thickness did not vary significantly with gender, patient age, the presence or absence of stenotic cardiac disease, or the presence or absence of hypertension. CONCLUSIONS: The ultrasonographic finding of increased carotid arterial wall thickness across a wide range of patients with WS demonstrates the pervasive nature of the arteriopathy of this disorder. That increased arterial wall thickness was observed in all patients studied suggests that the arteriopathy of WS is related to haploinsufficiency for the elastin gene.

Assuntos

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OBJECTIVE: To compare the efficacy and safety of two surfactant preparations in the treatment of respiratory distress syndrome (RDS). METHODS: We conducted a randomized, masked comparison trial at 21 centers. Infants with RDS who were undergoing mechanical ventilation were eligible for treatment with two doses of either a synthetic (Exosurf) or natural (Infasurf) surfactant if the ratio of arterial to alveolar partial pressure of oxygen was less than or equal to 0.22. Crossover treatment was allowed within 96 hours of age if severe respiratory failure (defined as two consecutive arterial/alveolar oxygen tension ratios < or = 0.10) persisted after two doses of the randomly assigned surfactant. Four primary outcome measures of efficacy (the incidence of pulmonary air leak (< or = 7 days); the severity of RDS; the incidence of death from RDS; and the incidence of survival without bronchopulmonary dysplasia (BPD) at 28 days after birth) were compared by means of linear regression techniques. RESULTS: The primary analysis of efficacy was performed in 1033 eligible infants and an analysis of safety outcomes in the 1126 infants who received study surfactant. Preentry demographic characteristics and respiratory status were similar for the two treatment groups, except for a small but significant difference in mean gestational age (0.5 week) that favored the infasurf treatment group. Pulmonary air leak (< or = 7 days) occurred in 21% of Exosurf- and 11% of infasurf-treated infants (adjusted relative risk, 0.53; 95% confidence interval, 0.40 to 0.71; p < or = 0.0001). During the 72 hours after the initial surfactant treatment, the average fraction of inspired oxygen (+/-SEM) was 0.47 +/- 0.01 for Exosurf- and 0.39 +/- 0.01 for infasurf-treated infants (difference, 0.08; 95% confidence interval, 0.06 to 0.10; p < 0.0001); the average mean airway pressure (+/-SEM) was 8.6 +/- 0.1 cm H2O; for Exosurf- and 7.2 +/- 0.1 cm H2O for Infasurf-treated infants (difference, 1.4 cm H2O; 95% confidence interval, 1.0 to 1.8 cm H2O; p < 0.0001). The incidences of RDS-related death, total respiratory death, death to discharge, and survival without bronchopulmonary dysplasia at 28 days after birth did not differ. The number of days of more than 30% inspired oxygen and of assisted ventilation, but not the duration of hospitalization, were significantly lower in Infasurf-treated infants. CONCLUSION: Compared with Exosurf, Infasurf provided more effective therapy for RDS as assessed by significant reductions in the severity of respiratory disease and in the incidence of air leak complications.

Assuntos

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The objective of our prospective study was to compare the sensitivity and specificity of ultrasonography, intravenous pyelography, and dimercaptosuccinic acid scan in detecting scarred kidneys. Twenty-seven consecutive subjects with recurrent urinary tract infections, vesicoureteral reflux, scarred kidneys, or a combination of these problems had all three imaging procedures performed. With the total number of scars serving as the gold standard, the sensitivity (94%) and specificity (100%) in identifying renal scars in children were highest for the DMSA scan. Intraobserver (95%) and interobserver (90%) reliability were also high for the DMSA scan. However, the clinical interpretation of the increased sensitivity of the DMSA scan is unknown. Changes on the scan not identified by intravenous urography may not represent true scars. Research into the long-term significance of these scars is indicated.

Assuntos

Assuntos

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Ultrasound brain scans sometimes demonstrate increased echogenicity or cysts, or both, in the periventricular white matter, superolateral to the ventricle, in the most common site of periventricular infarction. Over 33 months, 23 preterm infants dying after 20 or more days of life were entered into this study. Superolateral echogenicity or cysts were found in 13 (57%) cases. Periventricular infarction was present at autopsy in 12 (52%) cases. Ultrasound accurately diagnosed the size, site, and extent of periventricular infarction in 78% of scans. Interpretive errors were made with poor-quality scans and with early and late studies. We conclude that sector ultrasound brain scans accurately diagnose major periventricular infarction. Hemorrhage into the site of infarction is not a prerequisite for diagnosis of periventricular infarction by ultrasound.

Assuntos

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Thyroid ultrasound findings in 55 patients aged 6 days to 19 years were reviewed to assess the importance of this technique in evaluating childhood thyroid disorders. Findings were correlated with the available clinical, radionuclide, and pathologic data. In 25 patients with diffuse thyroid lesions (thyroiditis, Graves disease, euthyroid goiter, iodine-induced goiter, goitrous cretinism), ultrasound revealed only homogeneous thyroid enlargement or a nonspecific patchy echo pattern. In two infants with poorly visualized glands on radionuclide scans, ultrasound confirmed the presence of anatomically normal thyroid tissue. Twenty patients had at least one focal thyroid lesion seen by ultrasound, including nodules not detected by palpation in one child or by technetium scan in three. Thyroid malignancies were found in four of 13 patients with solitary thyroid nodules, occurring in two of four patients with echogenic nodules, two of five children with complex lesions, and none of four with echofree nodules. Thyroid ultrasound is a sensitive, noninvasive means of evaluating thyroid anatomy. Because it can detect thyroid tissue in the neck not seen on radionuclide scan in patients at all ages and can define the number and consistency of focal lesions, this technique offers definite advantages in assessing a variety of childhood thyroid disorders.

Assuntos

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The aim of this study was to validate brain imaging techniques in the preterm infant. A homogeneous group of very immature (less than 32 week) neonates dying in the neonatal period were sequentially scanned with linear-array real-time ultrasound scans, and after death with compound B static sector ultrasound and high-resolution computed tomography (CT) scans. All three imaging techniques were correlated with the autopsy results. All germinal matrix bleeds greater than 5mm in size and intraventricular hemorrhages associated with ventricular dilation or distortion were accurately diagnosed. In the immature infant it was difficult to distinguish the normal highly vascular germinal matrix and choroid plexus from hemorrhage into the brain or ventricles, respectively. Further studies that address the questions of accurate timing and incidence of bleeds must consider the spatial resolution of the individual scanner, the maturity of the brain, the site and size of the lesion, and the evolution of the lesion. For the diagnosis of major hemorrhagic lesions in the preterm infant, either ultrasound or CT scans may be used with confidence.

Assuntos