A case of split notochord syndrome: a child with a neuroenteric fistula presenting with meningitis

The authors describe a case of split notochord syndrome with a neuroenteric fistula in a newborn presenting with meningitis. Associated anomalies included agenesis of the corpus callosum, short colon, malrotation, epispadias, and an abnormally high bifurcation of the abdominal aorta and inferior vena cava. The embryological mechanisms and etiologic theories are discussed in short

Conversion to below-elbow cast after 3 weeks is safe for diaphyseal both-bone forearm fractures in children

Background It is unclear whether it is safe to convert above-elbow cast (AEC) to below-elbow cast (BEC) in a child who has sustained a displaced diaphyseal both-bone forearm fracture that is stable after reduction. In this multicenter study, we wanted to answer the question: does early conversion to BEC cause similar forearm rotation to that after treatment with AEC alone? Children and methods Children were randomly allocated to 6 weeks of AEC, or 3 weeks of AEC followed by 3 weeks of BEC. The primary outcome was limitation of pronation/supination after 6 months. The secondary outcomes were re-displacement of the fracture, limitation of flexion/extension of the wrist and elbow, complication rate, cast comfort, complaints in daily life, and cosmetics of the fractured arm. Results 62 children were treated with 6 weeks of AEC, and 65 children were treated with 3 weeks of AEC plus 3 weeks of BEC. The follow-up rate was 60/62 and 64/65, respectively with a mean time of 6.9 (4.7-13) months. The limitation of pronation/supination was similar in both groups (18 degrees for the AEC group and 11 degrees for the AEC/BEC group). The secondary outcomes were similar in both groups, with the exception of cast comfort, which was in favor of the AEC/BEC group. Interpretation Early conversion to BEC cast is safe and results in greater cast comfort

Antihypertensive drug concentration measurement combined with personalized feedback in resistant hypertension:a randomized controlled trial

Background:Adherence to antihypertensive drugs (AHDs) is crucial for controlling blood pressure (BP). We aimed to determine the effectiveness of measuring AHD concentrations using a dried blood spot (DBS) sampling method to identify nonadherence, combined with personalized feedback, in reducing resistant hypertension.Methods:We conducted a multicenter, randomized, controlled trial (RHYME-RCT, ICTRP NTR6914) in patients with established resistant hypertension. Patients were randomized to receive either an intervention with standard of care (SoC) or SoC alone. SoC consisted of BP measurement and DBS sampling at baseline, 3 months (t3), 6 months (t6), and 12 months (t12); AHD concentrations were measured but not reported in this arm. In the intervention arm, results on AHD concentrations were discussed during a personalized feedback conversation at baseline and t3. Study endpoints included the proportion of patients with RH and AHD adherence at t12.Results:Forty-nine patients were randomized to receive the intervention+SoC, and 51 were randomized to receive SoC alone. The proportion of adherent patients improved from 70.0 to 92.5% in the intervention+SoC arm (P = 0.008, n = 40) and remained the same in the SoC arm (71.4%, n = 42). The difference in adherence between the arms was statistically significant (P = 0.014). The prevalence of resistant hypertension decreased to 75.0% in the intervention+SoC arm (P &lt; 0.001, n = 40) and 59.5% in the SoC arm (P &lt; 0.001, n = 42) at t12; the difference between the arms was statistically nonsignificant (P = 0.14).Conclusion:Personalized feedback conversations based on DBS-derived AHD concentrations improved AHD adherence but did not reduce the prevalence of RH.</p

Altered left atrial 4D flow characteristics in patients with paroxysmal atrial fibrillation in the absence of apparent remodeling

The pathophysiology behind thrombus formation in paroxysmal atrial fibrillation (AF) patients is very complex. This can be due to left atrial (LA) flow changes, remodeling, or both. We investigated differences for cardiovascular magnetic resonance (CMR)-derived LA 4D flow and remodeling characteristics between paroxysmal AF patients and patients without cardiac disease. In this proof-of-concept study, the 4D flow data were acquired in 10 patients with paroxysmal AF (age=61 +/- 8 years) and 5 age/gender matched controls (age=56 +/- 1 years) during sinus rhythm. The following LA and LA appendage flow parameters were obtained: flow velocity (mean, peak), stasis defined as the relative volume with velocities<10 cm/s, and kinetic energy (KE). Furthermore, LA global strain values were derived from b-SSFP cine images using dedicated CMR feature-tracking software. Even in sinus rhythm, LA mean and peak flow velocities over the entire cardiac cycle were significantly lower in paroxysmal AF patients compared to controls [(13.12.4 cm/s vs. 16.7 +/- 2.1 cm/s, p=0.01) and (19.3 +/- 4.7 cm/s vs. 26.8 +/- 5.5 cm/s, p=0.02), respectively]. Moreover, paroxysmal AF patients expressed more stasis of blood than controls both in the LA (43.2 +/- 10.8% vs. 27.8 +/- 7.9%, p=0.01) and in the LA appendage (73.3 +/- 5.7% vs. 52.8 +/- 16.2%, p=0.04). With respect to energetics, paroxysmal AF patients demonstrated lower mean and peak KE values (indexed to maximum LA volume) than controls. No significant differences were observed for LA volume, function, and strain parameters between the groups. Global LA flow dynamics in paroxysmal AF patients appear to be impaired including mean/peak flow velocity, stasis fraction, and KE, partly independent of LA remodeling. This pathophysiological flow pattern may be of clinical value to explain the increased incidence of thromboembolic events in paroxysmal AF patients, in the absence of actual AF or LA remodeling.Cardiovascular Aspects of Radiolog

Neuroblastoma between 1990 and 2014 in the Netherlands: Increased incidence and improved survival of high-risk neuroblastoma

Purpose: Long-term trends in neuroblastoma incidence and survival in unscreened populations are unknown. We explored trends in incidence, stage at diagnosis, treatment and survival of neuroblastoma in the Netherlands from 1990 to 2014. Methods: The Netherlands Cancer Registry provided data on all patients aged <18 years diagnosed with a neuroblastoma. Trends in incidence and stage were evaluated by calculating the average annual percentage change (AAPC). Univariate and multivariable survival analyses were performed for stage 4 disease to test whether changes in treatment are associated with survival. Results: Of the 593 newly diagnosed neuroblastoma cases, 45% was <18 months of age at diagnosis and 52% had stage 4 disease. The age-standardized incidence rate for stage 4 disease increased at all ages from 3.2 to 5.3 per million children per year (AAPC + 2.9%, p <. 01). This increase was solely for patients ≥18 months old (3.0–5.4; AAPC +3.3%, p =. 01). Five-year OS of all patients increased from 44 ± 5% to 61 ± 4% from 1990 to 2014 (p <. 01) and from 19 ± 6% to 44 ± 6% (p <. 01) for patients with stage 4 disease. Multivariable analysis revealed that high-dose chemotherapy followed by autologous stem cell rescue and anti-GD2-based immunotherapy were associated with this survival increase (HR 0.46, p <. 01 and HR 0.37, p <. 01, respectively). Conclusion: Incidence of stage 4 neuroblastoma increased exclusively in patients aged ≥18 months since 1990, whereas the incidence of other stages remained stable. The 5-year OS of stage 4 patients improved, mostly due to the introduction of high-dose chemotherapy followed by stem cell rescue and immunotherapy

Longitudinal associations between asthma control, medication adherence, and quality of life among adolescents: results from a cross-lagged analysis

Item does not contain fulltextPURPOSE: It is not completely clear whether and how medication adherence, asthma control, and quality of life (QOL) predict each other over time. Therefore, the aim of the present study was to examine the longitudinal associations between asthma control, medication adherence, and quality of life among adolescents. METHODS: In this 3-wave longitudinal study, adolescents (N=139, Mean age=11.8) completed questionnaires about their medication adherence (Medication Adherence Report Scale for Asthma), asthma control (Asthma Control Questionnaire), and QOL (Adolescent Asthma Quality of Life Questionnaire) during home visits in the spring/summer of 2011, 2012, and 2013. Cross-lagged analyses examined temporal interrelations between the three variables over the course of 3years. RESULTS: Higher QOL at baseline predicted increased medication adherence at follow-up (T2), but did not predict changes in asthma control over time. Medication adherence and asthma control did not predict changes in QOL over time. There were no associations between asthma control and medication adherence over time. CONCLUSIONS: Interventions could focus on increasing QOL to increase medication adherence in adolescents with asthma

Moderating effect of gender on the prospective relation of physical activity with psychosocial outcomes and asthma control in adolescents: a longitudinal study

Item does not contain fulltextObjective: Adolescents with asthma experience more psychosocial and physiological problems compared to their healthy peers. Physical activity (PA) might decrease these problems. This study was the first observational longitudinal study to examine whether habitual PA could predict changes in psychosocial outcomes (i.e. symptoms of anxiety and depression, quality of life [QOL] and stress) and asthma control over time in adolescents with asthma and whether gender moderated these relationships. Methods: Adolescents with asthma (N = 253; aged 10-14 years at baseline) were visited at home in the spring/summer of 2012 and 2013. They completed questionnaires assessing their habitual PA, symptoms of anxiety and depression, QOL, perceived stress and asthma control. Path analyses using Mplus were conducted to examine longitudinal relationships among habitual PA, psychosocial outcomes and asthma control (controlled for body mass index, age and gender). Using multi-group analyses, we examined whether gender moderated these relationships. Results: Path analyses in the total group showed that habitual PA did not predict changes in psychosocial outcomes or asthma control over time. Multi-group analyses showed that gender moderated the relation of habitual PA with anxiety and depression. Habitual PA only significantly predicted a decrease in anxiety and depression over time for girls but not for boys. Conclusions: Increasing habitual PA in girls with asthma might decrease their symptoms of anxiety and depression

Congenital diaphragmatic hernia: still a moving target

Item does not contain fulltextThe primary therapeutic target for congenital diaphragmatic hernia (CDH) patients has shifted from emergency surgical repair towards a non-operative emergency of the newborn treated by interdisciplinary teams. The increased understanding of the epidemiological and pathophysiological aspects of CDH have led to an improved knowledge and application of prenatal diagnosis, postnatal ventilation strategies, treatment of associated pulmonary hypertension and the role of extracorporeal membrane oxygenation therapy. In the surgical field, the perspectives have changed with delayed CDH repair, the introduction of minimally invasive surgery and use of prosthetic material for closure of large defects. With decreased mortality, long term multi-organ morbidity has increased in some survivors. In the near future, randomized controlled trials on different aspects of therapy will determine evidence-based optimal care

Sport club participation of adolescents with asthma: maternal factors and adolescent cognitions

Item does not contain fulltextSport participation is especially important for patients with asthma in that it decreases psychosocial and physiological problems associated with inactivity. However, adolescents with asthma seem to participate less in sports compared to their non-asthmatic peers. The current study tested the direct associations between maternal sport-specific factors and sport club participation of early adolescents with asthma and the indirect effect through adolescent's sport-specific cognitions. METHODS: During home visits, 261 adolescents (aged 10-15) completed questionnaires about self-efficacy, beliefs regarding sport participation, and their actual sport club participation. Their mothers reported their sport-specific support, beliefs about offspring's and own sport participation, their own levels of physical activity, and their self-efficacy to stimulate offspring to participate in sports. Path analyses were used to examine the direct and indirect associations of maternal sport-specific factors with adolescents sport club participation via adolescent sport-specific cognitions. RESULTS: Analyses showed that maternal sport-specific support (β = 0.20, P = 0.007) and self-efficacy to stimulate offspring to participate in sports (β = 0.20, P = 0.027) related positively to adolescents' sport club participation. Adolescents' self-efficacy (indirect effect = 0.09, SE = 3.01, P < 0.001) mediated the positive relation between maternal self-efficacy to stimulate offspring to participate in sport and adolescents' participation in sport clubs. CONCLUSION: Maternal sport-specific factors related to adolescents' sport club participation directly and indirectly through adolescents' sport-specific cognitions. Intervention programs should focus on maternal sport-specific support and self-efficacy and adolescents' self-efficacy to increase sport participation of adolescents with asthma