Abstract Background Intraosseous access is used in emergency medicine as an alternative when intravenous access is difficult to obtain. Intraosseous samples can be used for laboratory testing to ...guide treatment. Many laboratories are reluctant to analyse intraosseous samples, as they frequently block conventional laboratory equipment. We aimed to evaluate the feasibility and accuracy of analysis of intraosseous samples using an i-STAT® point-of-care analyser. Methods Intravenous and intraosseous samples of twenty children presenting for scheduled diagnostic bone marrow aspiration were analysed using an i-STAT® point-of-care analyser. Sample types were compared using Bland Altman plots and by calculating intraclass correlation coefficients and coefficients of variance. Results The handheld i-STAT® point-of-care analyser proved suitable for analysing intraosseous samples without technical difficulties. Differences between venous and intraosseous samples were clinically acceptable for pH, base excess, sodium, ionised calcium and glucose in these haemodynamically stable patients. The intraclass correlation coefficient was excellent (>0.8) for comparison of intraosseous and intravenous base excess, and moderate (around 0.6) for bicarbonate, sodium and glucose. The coefficient of variance of intraosseous samples was smaller than that of venous samples for most variables. Conclusion Analysis of intraosseous samples with a bedside, single-use cartridge-based analyser is feasible and avoids the problem of bone marrow contents damaging conventional laboratory equipment. In an emergency situation point-of-care analysis of intraosseous aspirates may be a useful guide to treatment.
Introduction
Spirometry plays an important role in the assessment of possible respiratory failure in children with neuromuscular diseases (NMDs). However, obtaining reliable spirometry results is a ...major challenge. We studied the relation between oscillometry and spirometry results. Oscillometry is an easy, noninvasive method to measure respiratory resistance R and reactance X. We hypothesized an increased R and reduced X in patients with more reduced lung function.
Methods
In this prospective single‐center study, we included all children with NMDs able to perform spirometry. We consecutively measured R and X at 5, 11, and 19 Hz and (forced) vital capacity, peak expiratory flow. Spearman correlation coefficients and positive and negative predictive values were calculated. Regression curves were estimated.
Results
We included 148 patients, median age 13 years (interquartile range: 8–16). A negative correlation was found between R and spirometry outcomes (Spearman correlation coefficient ρ: −0.5 to −0.6, p < 0.001). A positive correlation was found between X (i.e., less negative outcomes) and spirometry outcomes (ρ: 0.4–0.6, p < 0.001). Highest correlation was found at lower frequencies. Regression analysis showed a nonlinear relation. Measurement of inspiratory and expiratory R and X did not provide added value. Positive predictive values of 80%–85% were found for z‐scores of R measured at 5 Hz versus (F)VC ≤ 60%.
Conclusion
We found a nonlinear relation between oscillometry and spirometry results with increased R and reduced X in patients with more restrictive lung function decline. Given the difficulties with performing spirometry, oscillometry may be a promising substitute.
Introduction
Children with neuromuscular diseases develop cough impairment. Airway clearance techniques (ACTs) may help to prevent recurrent respiratory tract infections (RTIs). A commonly used ACT ...is mechanical insufflation‐exsufflation (MI‐E), but evidence for efficacy is limited. We hypothesize that MI‐E has beneficial effect on RTI related hospital admission rate.
Methods
In this single‐center retrospective study, we reviewed all children who used daily MI‐E between 2005 till June 2019. Primary outcome studied was the number of RTIs requiring hospital admission. Patient satisfaction and burden experienced by MI‐E use were explored by questionnaires using a Likert scale. The relative number of RTIs requiring admission and the number of admission days per eligible period before and after the introduction of MI‐E were compared using the Friedman test and the Wilcoxon signed‐rank test.
Results
Thirty‐seven children were included.
The median number of RTI related hospital admissions per 1000 eligible days after the introduction of MI‐E was 0.9 (interquartile range IQR 0.0‐3.1) compared to the 3 preceding years (median 3.7; IQR 1.4‐5.9; P = .006). The median number of RTI related admission days per 1000 eligible days after the introduction of MI‐E was significantly lower with a median of 2.7 (IQR 0.0‐17.4) compared to the 3 preceding years (median 33.6; IQR 15.0‐51.1; P = .001). Patient satisfaction was high with low burden, even in patients who discontinued treatment.
Conclusion
A significantly lower number of RTIs requiring hospital admission and shorter admission duration after the introduction of MI‐E was found, with high patient satisfaction and low burden.
Introduction
Understanding the impact of scoliosis surgery on lung function is important for counseling patients about risks and benefits of surgery. We prospectively compared the trends in lung ...function test (LFT) results before and after scoliosis surgery in children with neuromuscular diseases or dysmorphic syndromes. We hypothesized a stabilization.
Methods
We prospectively included children with neuromuscular or syndromic scoliosis able to perform LFTs. We studied (forced) vital capacity (FVC), ratio of forced expiratory volume in 1 s (FEV1) and FVC, and peak expiratory flow (PEF). Preoperative LFT results were compared with results 3–4 months after surgery. The mean monthly change in LFT results up to 2 years after surgery was compared with the preoperative natural history using linear mixed‐effects models.
Results
We included 43 patients. No significant change was observed in absolute values of (F)VC, FEV1/FVC, and PEF before and after surgery. In 23 neuromuscular patients median standardized VC, FVC, and PEF decreased significantly after surgery from 43% to 33%, 42% to 31%, and 51% to 40%, respectively. In 20 syndromic patients, median FVC decreased from 68% to 65%. The monthly rate of change in FVC did not change significantly in both groups with a mean difference of 0.18% (95% CI: −0.27, −0.61) and −0.44% (95% CI: −1.05, 0.16).
Conclusion
No stabilization of lung function 3–4 months after scoliosis surgery was observed in children with neuromuscular and syndromic scoliosis with restrictive lung function disease. The effect on the rate of lung function decline remains inconclusive.
Background
Citrate is preferred over heparin as an anticoagulant in adult continuous renal replacement therapy (CRRT). However, its potential adverse effects and data on use in CRRT in infants and ...toddlers is limited. We conducted a prospective study on using citrate in CRRT in critically ill small children.
Methods
Children who underwent CRRT with the smallest filter in our PICU between November 2011 and November 2016 were included. Both heparin and citrate were applied according to a strict protocol. Our primary outcome was circuit survival time. Secondary outcomes were alkalosis, citrate toxicity, and number of red blood cell transfusions.
Results
Heparin was used in six patients (121 circuits, total CRRT time 3723 h). Citrate was used in 14 patients (105 circuits, total CRRT time 4530 h). Median circuit survival time with heparin was 21 h (IQR 14.5–27.5) compared to 45.2 h (IQR 37.5–52.8) with citrate (
p
< 0.001). Actual administered effluent dose compared to prescribed dose was 85% (IQR 69–98%) with heparin compared to 92% (IQR 88–98%) with citrate (
p
= 0.31). No patient treated with citrate developed citrate toxicity. No other differences in electrolytes were found between the two CRRT regimes. In the heparin group, a median of 6.5 units of red blood cells (IQR 1.5–23.8) were given during CRRT, compared to three in the citrate group (IQR 2.0–5.0,
p
= 0.12).
Conclusions
Use of regional citrate significantly prolongs circuit survival time and thereby should increase CRRT efficiency when compared to heparin. In addition, citrate appears safe for CRRT in critically ill small children.
Air stacking (AS) and mechanical insufflation–exsufflation (MI-E) aim to increase cough efficacy by augmenting inspiratory lung volumes in patients with neuromuscular diseases (NMDs). We studied the ...short-term effect of AS and MI-E on lung function. We prospectively included NMD patients familiar with daily AS or MI-E use. Studied outcomes were forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and peak expiratory flow (PEF) prior to, immediately after, and up to 2 h after treatment. Paired sample T-test and Wilcoxon signed-rank test was used. Sixty-seven patients participated. We observed increased FVC and FEV1 immediately after AS with a mean difference of respectively 0.090 L (95% CI 0.045; 0.135, p < .001) and 0.073 L (95% CI 0.017; 0.128, p = .012). Increased FVC immediately after MI-E (mean difference 0.059 L (95% CI 0.010; 0.109, p = .021) persisted 1 hour (mean difference 0.079 L (95% CI 0.034; 0.125, p = .003). The effect of treatment was more pronounced in patients diagnosed with Spinal Muscular Atrophy, compared to patients with Duchenne muscular dystrophy. AS and MI-E improved FVC immediately after treatment, which persisted 1 h after MI-E. There is insufficient evidence that short-lasting increases in FVC would explain the possible beneficial effect of AS and MI-E.
Objectives
In addition to breathing problems, patients with Robin sequence (RS) often encounter feeding difficulties (FD). Data regarding the occurrence of FD and possible influencing factors are ...scarce. The study aim was to elucidate these factors to improve treatment strategies.
Material and methods
A retrospective comparative cohort study was conducted, consisting of 69 infants diagnosed with both RS and a cleft palate and 64 isolated cleft palate only (iCPO) infants. Data regarding FD, growth, and airway intervention were collected during the first 2 years of life. A systematic review of the literature was conducted to identify reported FD in RS patients.
Results
RS patients had more FD (91 %) than iCPO patients (72 %;
p
= 0.004). Also, nasogastric (NG)-tube feeding was necessary more frequently and for a longer period (both
p
< 0.001). Growth was lower in RS than iCPO infants (
p
= 0.008) and was not affected by the kind of airway management (conservative/surgical;
p
= 0.178), cleft palate grade (
p
= 0.308), or associated disorders (
p
= 0.785). By contrast, surgical intervention subtype did significantly affect growth. Mean reported FD for RS in the literature is 80 % (range = 47–100 %), and 55 % (range = 11–100 %) of infants need NG-tube feeding.
Conclusions
FD is present in a large proportion of infants with RS, which indicates the need for early recognition and proper treatment to ensure optimal growth. Growth during the first 2 years of life is significantly lower in RS patients than iCPO patients, which indicates the need for careful attention and long-term follow-up.
Clinical relevance
This study indicates the need for early recognition and proper treatment of FD in RS to ensure optimal growth. In addition, growth needs careful attention and long-term follow-up.
Abstract
Nusinersen (Spinraza®) improves survival of infants with hereditary proximal spinal muscular atrophy and motor function in children up to 12 years. Population-based assessments of treatment ...efficacy are limited and confined to select cohorts of patients. We performed a nationwide, population-based, single-centre cohort study in children with spinal muscular atrophy younger than 9.5 years at start of treatment in line with reimbursement criteria in the Netherlands. We assessed age-relevant motor function scores, the need for tube feeding, hours of ventilatory support and documented adverse events. We used linear mixed modelling to assess treatment effects. We compared motor function during treatment with natural history data and to individual trajectories of muscle strength and motor function before the start of treatment. We included 71 out of 72 Dutch children who were treated (median age 54 months; range 0–117) and followed them for a median of 38 months (range 5–52). We observed improvement of motor function in 72% and stabilization in another 18% of the symptomatic children, which differed from the natural disease course in a matched cohort of which we had previously collected natural history data. Longitudinal analysis showed that motor function improved up to a median of 24 months (range 12–30) of treatment after which it stabilized. Shorter disease duration at start of treatment resulted in better treatment efficacy (P < 0.01). Sixteen children (23%) achieved new motor milestones. Bulbar and respiratory function did not improve significantly during treatment. In 15 patients from whom treatment-naïve data were available, the pre-treatment trajectory of motor function decline changed to stabilization or improvement after the start of treatment. We documented 82 adverse events after 934 injections (9%) in 45 patients. None of the adverse events led to treatment discontinuation. Intrathecal nusinersen treatment is safe and improves or stabilizes motor function in 90% of young children with spinal muscular atrophy types 1c–3a. We did not observe improvement of respiratory and bulbar functions.
Objectives
Initial approaches to and treatments of infants with Robin sequence (RS) is diverse and inconsistent. The care of these sometimes critically ill infants involves many different medical ...specialties, which can make the decision process complex and difficult. To optimize the care of infants with RS, we present our institution’s approach and a review of the current literature.
Material and methods
A retrospective cohort study was conducted among 75 infants diagnosed with RS and managed at our institution in the 1996–2012 period. Additionally, the conducted treatment regimen in this paper was discussed with recent literature describing the approach of infants with RS.
Results
Forty-four infants (59 %) were found to have been treated conservatively. A significant larger proportion of nonisolated RS infants than isolated RS infants needed surgical intervention (53 vs. 25 %,
p
= .014). A mandibular distraction was conducted in 24 % (
n
= 18) of cases, a tracheotomy in 9 % (
n
= 7), and a tongue–lip adhesion in 8 % (
n
= 6). Seventy-seven percent of all infants had received temporary nasogastric tube feeding. The literature review of 31 studies showed that initial examinations and the indications to perform a surgical intervention varied and were often not clearly described.
Conclusions
RS is a heterogenic group with a wide spectrum of associated anomalies. As a result, the decisional process is challenging, and a multidisciplinary approach to treatment is desirable. Current treatment options in literature vary, and a more uniform approach is recommended.
Clinical Relevance
We provide a comprehensive and pragmatic approach to the analysis and treatment of infants with RS, which could serve as useful guidance in other clinics.
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