To save content items to your account,
please confirm that you agree to abide by our usage policies.
If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account.
Find out more about saving content to .
To save content items to your Kindle, first ensure firstname.lastname@example.org
is added to your Approved Personal Document E-mail List under your Personal Document Settings
on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part
of your Kindle email address below.
Find out more about saving to your Kindle.
Note you can select to save to either the @free.kindle.com or @kindle.com variations.
‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi.
‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.
The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) lacks a rigorous enrollment audit process, unlike other collaborative networks. Most centers require individual families to consent to participate. It is unknown whether there is variation across centers or biases in enrollment.
We used the Pediatric Cardiac Critical Care Consortium (PC4) registry to assess enrollment rates in NPC-QIC for those centers participating in both registries using indirect identifiers (date of birth, date of admission, gender, and center) to match patient records. All infants born 1/1/2018–12/31/2020 and admitted 30 days of life were eligible. In PC4, all infants with a fundamental diagnosis of hypoplastic left heart or variant or who underwent a surgical or hybrid Norwood or variant were eligible. Standard descriptive statistics were used to describe the cohort and center match rates were plotted on a funnel chart.
Of 898 eligible NPC-QIC patients, 841 were linked to 1,114 eligible PC4 patients (match rate 75.5%) in 32 centers. Match rates were lower in patients of Hispanic/Latino ethnicity (66.1%, p = 0.005), and those with any specified chromosomal abnormality (57.4%, p = 0.002), noncardiac abnormality (67.8%, p = 0.005), or any specified syndrome (66.5%, p = 0.001). Match rates were lower for patients who transferred to another hospital or died prior to discharge. Match rates varied from 0 to 100% across centers.
It is feasible to match patients between the NPC-QIC and PC4 registries. Variation in match rates suggests opportunities for improvement in NPC-QIC patient enrollment.
To evaluate the short-term and long-term clinical effectiveness and safety of subthalamic nucleus deep brain stimulation (STN-DBS) for medically intractable pediatric isolated dystonia.
Using a longitudinal retrospective design, we assessed the clinical outcomes of nine patients who underwent STN-DBS for treatment-refractory pediatric isolated dystonia one decade ago (mean age at surgery: 15.9 ± 4.5 years). The primary clinical outcome used was assessed by retrospective video analyses of patients’ dystonia symptoms using the Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS). Clinical assessments were performed at baseline, 1-year follow-up (1-yr FU), and 10-year follow-up (10-yr FU). Adverse side effects, including surgery-related, device-related, and stimulation-related effects, were also documented.
After STN-DBS surgery, the mean improvement in the BFMDRS motor score was 77.1 ± 26.6% at 1-yr FU and 90.4 ± 10.4% at 10-yr FU. Similarly, the mean BFMDRS disability score was improved by 69.5 ± 13.6% at 1-yr FU and by 86.5 ± 13.9% at 10-yr FU. The clinical improvements gained at 10-yr FU were significantly larger than those observed at 1-yr FU. Negative correlations were found between the duration of disease to age at surgery ratio (DD/AS) and the improvements in the BFMDRS motor score and total score at 1-yr FU and 10-yr FU.
To our knowledge, this study provides the first clinical evidence for the short- and long-term effectiveness and safety of STN-DBS for pediatric isolated dystonia. Additionally, putative evidence is provided that earlier STN-DBS intervention in patients with refractory pediatric isolated dystonia may improve short- and long-term clinical outcomes.
Chylothorax after paediatric cardiac surgery incurs significant morbidity; however, a detailed understanding that does not rely on single-centre or administrative data is lacking. We described the present clinical epidemiology of postoperative chylothorax and evaluated variation in rates among centres with a multicentre cohort of patients treated in cardiac ICU.
This was a retrospective cohort study using prospectively collected clinical data from the Pediatric Cardiac Critical Care Consortium registry. All postoperative paediatric cardiac surgical patients admitted from October, 2013 to September, 2015 were included. Risk factors for chylothorax and association with outcomes were evaluated using multivariable logistic or linear regression models, as appropriate, accounting for within-centre clustering using generalised estimating equations.
A total of 4864 surgical hospitalisations from 15 centres were included. Chylothorax occurred in 3.8% (n=185) of hospitalisations. Case-mix-adjusted chylothorax rates varied from 1.5 to 7.6% and were not associated with centre volume. Independent risk factors for chylothorax included age <1 year, non-Caucasian race, single-ventricle physiology, extracardiac anomalies, longer cardiopulmonary bypass time, and thrombosis associated with an upper-extremity central venous line (all p<0.05). Chylothorax was associated with significantly longer duration of postoperative mechanical ventilation, cardiac ICU and hospital length of stay, and higher in-hospital mortality (all p<0.001).
Chylothorax after cardiac surgery in children is associated with significant morbidity and mortality. A five-fold variation in chylothorax rates was observed across centres. Future investigations should identify centres most adept at preventing and managing chylothorax and disseminate best practices.
To evaluate the prevalence of vitamin D deficiency in pregnant women and their newborns in Beijing, China and the influence of vitamin D deficiency on birth size.
A cross-sectional study.
Data were collected from pregnant women who delivered during April to May 2010 at 306 Hospital of PLA in Beijing, China.
Participants in the study were seventy healthy nulliparous pregnant women with singleton pregnancies who delivered healthy babies at full term and their newborns.
Severe vitamin D deficiency (25-hydroxyvitamin D (25(OH)D) < 25 nmol/l) was detected in 54·5 % of mothers and 46·6 % of newborns. Neither mothers nor newborns had serum 25(OH)D concentrations that reached the normal level (>75 nmol/l). The concentration of 25(OH)D in mothers was positively correlated with that in cord blood (r = 0·89, P < 0·001). Newborns of mothers with severe vitamin D deficiency had lower birth length and birth weight. The head circumference and birth weight were lower in vitamin D-deficient newborns.
The study indicates that pregnant women and neonates residing in Beijing are at high risk of vitamin D deficiency. Neonatal 25(OH)D concentrations are dependently related to maternal 25(OH)D levels. Maternal and neonatal vitamin D status influences newborn size.
Email your librarian or administrator to recommend adding this to your organisation's collection.