Noninvasive measurement of cardiac output in critically ill children.

This study was performed to evaluate the hemodynamic status of children admitted to the intensive care unit, using suprasternal and transesophageal Doppler ultrasound, and to establish a suitable noninvasive technique to monitor trends in cardiac output in critically ill children. Twenty children were studied over a period of 6 months. The median age was 32.5 months and weight 14.5 kg. Minute distance (MD), which is a linear cardiac output parameter, was assessed. Seven simultaneous pairs of measurements of MD were made using transesophageal Doppler (TED) and suprasternal Doppler (SSD) by the same operator. Following a fluid challenge, seven repeat pairs of measurements were made. The mean percentage changes for MD by TED and SSD were 21.84 (SD 9.97) and 5.75 (SD 7.32). The average coefficients of variation for measurements of MD by TED and SSD were 2.34% and 15.98%, respectively. The mean difference in percentage change between MD, measured by TED and SSD, was 27.59 with a 95% confidence interval and wide limits of agreement. The repeatability of TED measurements was good, but the measurements by SSD were wide and erratic with poor reproducibility. Our study shows that TED is easy to use, reliable, and very useful for monitoring hemodynamic changes in critically ill children.

Reduction in case fatality rate from meningococcal disease associated with improved healthcare delivery.

BACKGROUND AND AIMS: The case fatality rate from meningococcal disease (MD) has remained relatively unchanged in the post antibiotic era, with 20-50% of patients who develop shock still dying. In 1992 a new paediatric intensive care unit (PICU) specialising in MD was opened. Educational information was disseminated to local hospitals, and a specialist transport service was established which delivered mobile intensive care. The influence of these changes on mortality of children with MD was investigated. METHODS: A total of 331 consecutive children with meningococcal disease admitted to the PICU between 1992 and 1997 were studied. Severity of the disease on admission was assessed using the paediatric risk of mortality (PRISM) score. Logistic regression analysis was used to correct for clinical severity, age, and sex; death was the outcome, and year of admission, a temporal trend variable, was the primary exposure. RESULTS: The case fatality rate fell year on year (from 23% in 1992/93 to 2% in 1997) despite disease severity remaining largely unchanged. After adjustment for age, sex, and disease severity, the overall estimate for improvement in the odds of death was 59% per year (odds ratio for the yearly trend 0.41). CONCLUSIONS: A significant improvement in outcome for children admitted with MD to a PICU has occurred in association with improvements in initial management of patients with MD at referring hospitals, use of a mobile intensive care service, and centralisation of care in a specialist unit.

The Keutel syndrome. Report of a case and review of the literature.

A 2-month-old boy presented with cartilage calcification, brachytelephalangism, peripheral pulmonary stenosis, hearing loss, short stature and slight psychomotor delay. This case is similar to the two cases described by Keutel in 1972. Since then, four other cases have been reported; we report the seventh case and discuss the clinical findings and the incidence of the disease.

Nonimmune hydrops fetalis associated with genetic abnormalities.

The purpose of this review of the literature on nonimmune hydrops fetalis was to evaluate whether recent clinicopathologic studies have modified the relative incidence of the different associated conditions and the management of these pregnancies. We found 600 cases of nonimmune hydrops fetalis published since 1982. These cases were reviewed with particular attention to genetic causes and were compared with a literature review of 298 cases published before 1982. The mean gestational age at diagnosis varied from 24-29 weeks in the recent series, compared with 31-33 weeks in the earlier series. Genetically transmitted conditions accounted for more than 35% of the fetal and maternal disorders associated with nonimmune hydrops fetalis in the recent series, compared with 21% before 1982. The most frequently identified genetic abnormalities in our review were chromosomal disorders (15.7%), alpha-thalassemia (10.3%), skeletal dysplasia (4%), arthrogryposis multiplex syndromes (1.8%), multiple pterygium syndrome (1.5%), and lysosomal storage disorders (1.0%). These results confirm the need for systematic chromosome analysis in fetuses with nonimmune hydrops. From this review, we conclude that prenatal noninvasive and invasive techniques combined with detailed pathologic studies have improved the accuracy of diagnosis of the underlying causes of nonimmune hydrops fetalis and have influenced the management of these pregnancies.

Embryonic remnants of the umbilical cord: morphologic and clinical aspects.

Embryonic vestiges of the umbilical cord are classic findings in routine morphologic examination of the placenta. In order to evaluate their clinicopathologic significance, we examined samples from the fetal and placental ends of 1,000 umbilical cords and collected the principal clinical findings of the corresponding newborns. Microscopic evidence of embryonic remnants were found in 231 cases (23.1%) divided into remnants of the allantoic duct (63%), the omphalomesenteric duct (6.6%), and the embryonic vessels (30.4%), including one case of hemangioma and an accessory small artery. There were no significant clinical differences between the three vestigial groups, and no particular association with congenital malformations or perinatal complications. In 70.9% of the cases, the embryonic remnants were found at the fetal end of the umbilical cord, where most tumors of the cord develop.