Correction to: Neurocognitive functioning and health‑related quality of life of children after pediatric intensive care admission: a systematic review

Quality of Life Research -     

Endocrine-metabolic response to abdominal aortic surgery: A randomized trial of general anesthesia versus general plus epidural anesthesia

The influence of epidural anesthesia on the endocrine-metabolic response following abdominal aortic reconstruction was studied in a prospective randomized trial. Cortisol and catecholamine responses and nitrogen balance were measured in two groups of five patients receiving general anesthesia only (group 1) or general anesthesia combined with epidural bupivacaine (group 2). The study lasted from preoperatively until the first postoperative day. At 2100 hours on the day of surgery serum cortisol concentrations were higher in group 1 than in group 2 (1.41 versus 0.82 mol/L; p<0.01). Likewise the total perioperative hypercortisolemia, expressed as the area under the curve, was significantly higher in group 1 (11.7 versus 5.7 mol/L/hr, p<0.01). Intraoperative urinary excretion of epinephrine and postoperative norepinephrine excretion were significantly higher in group 1 than in group 2. Urinary excretion of free cortisol and cumulative nitrogen balance were not different between the groups. Although the number of patients was limited and the sensory nerve block level was not measured perioperatively, this study suggests that epidural anesthesia attenuates the stress response to aortic surgery.

---

Resumen Se diseñó un ensayo clínico prospectivo y randomizado con el fin de estudiar el efecto de la anestesia epidural sobre la respuesta endocrino-metabolica luego de una reconstrucción aórtica abdominal. Se determinaron la respuesta de cortisol y catecolaminas y el balance de nitrógeno en dos grupos de 5 pacientes que recibieron anestesia general solamente (Grupo 1) o anestesia general combinada con bupivacaína epidural (Grupo 2). El estudio se realizó en el periodo comprendido entre la fase preoperatoria hasta el primer día postoperatorio. A las 21:00 horas del mismo día de la cirugía las concentraciones séricas de cortisol resultaron más altas en el Grupo 1 que en el Grupo 2 (1.41 versus 0.82 micromol/l, p<0.01). Igualmente, la hipercortisolemia total perioperatoria apareció más alta en el Grupo 1 (11.7 versus 5.7 micromol/l^* hora, p<0.01). La excreción urinaria intraoperatoria de epinefrina y la excreción postoperatoria de norepinefrina aparecieron significativamente más altas en el Grupo 1 que en el Grupo 2. No se registraron diferencias en la excreción urinaria de cortisol libre ni en el balance acumulado de nitrógeno. Aunque el número de pacientes fue limitado y el nivel del bloqueo sensorial no fue determinado perioperatoriamente, el presente estudio sugiere que la anestesia epidural atenúa la respuesta de estrés en la cirugia aórtica.

---

Résumé Dans une étude prospective et randomisée, l'influence de l'anesthésie péridurale sur la résponse métabolique endocrinienne a été étudiée dans la période préopératoire immédiate jusqu'au premier jour postopératoire après la chirurgie de l'aorte abdominale. On a mesuré les résponses en catécholamines et l'équilibre azoté chez cinq patients ayant eu une anesthésie générale seule (groupe 1) et cinq patients ayant une anesthésie générale combinée avec une anesthésie péridurale par la bupivacaïne (groupe 2). A 21H00 le jour de l'intervention,. la concentration en cortisol était plus haute dans le groupe 1 par rapport au groupe 2 (1.41 vs 0.82 micromol/l, p<0.01). De même, l'hypercortisolémie périopératoire totale, exprimée par la surface sous la courbe, était significativement plus élevée dans le groupe 1 (11.7 vs 5.7 micromol/l heure, p<0.01). L'excrétion urinaire peropératoire d'épinéphrine et l'excrétion postopératoire de la norépinéphrine étaient significativement plus élevées dans le groupe 1 par rapport au groupe 2. L'excrétion urinaire du cortisol libre et l'équilibre azoté tout au long de l'étude n'étaient pas très différents. Bien que le nombre de patients était limité et que le niveau supérieur de l'anesthésie n'ait pas été mesuré en périopératoire, les résultats de cette étude suggèrent que l'anesthésie péridurale atténue la réponse au stress dans la chirurgie de l'aorte abdominale.

     

Recognizing the tenascin‐X deficient type of Ehlers–Danlos syndrome: a cross‐sectional study in 17 patients

The tenascin‐X (TNX) deficient type Ehlers–Danlos syndrome (EDS) is similar to the classical type of EDS. Because of the limited awareness among geneticists and the challenge of the molecular analysis of the TNXB gene, the TNX‐deficient type EDS is probably to be under diagnosed. We therefore performed an observational, cross‐sectional study. History and physical examination were performed. Results of serum TNX measurements were collected and mutation analysis was performed by a combination of next‐generation sequencing (NGS), Sanger sequencing and multiplex ligation‐dependent probe amplification (MLPA). Included were 17 patients of 11 families with autosomal recessive inheritance and childhood onset. All patients had hyperextensible skin without atrophic scarring. Hypermobility of the joints was observed in 16 of 17 patients. Deformities of the hands and feet were observed frequently. TNX serum level was tested and absent in 11 patients (seven families). Genetic testing was performed in all families; 12 different mutations were detected, most of which are suspected to lead to non‐sense mRNA mediated decay. In short, patients with the TNX‐deficient type EDS typically have generalized joint hypermobility, skin hyperextensibility and easy bruising. In contrast to the classical type, the inheritance pattern is autosomal recessive and atrophic scarring is absent. Molecular analysis of TNXB in a diagnostic setting is challenging.     

Systematic review of the effects of physical exercise training programmes in children and young adults with congenital heart disease

Background

Most patients with congenital heart disease (ConHD) do not perform regular physical exercise. Consensus reports have stated that exercise should be encouraged and regularly performed in these patients, but this is not common practise. We reviewed the literature on actual evidence for either negative or positive effects of physical exercise training programmes in children and young adults with ConHD.

Methods

Using the Medline database, we systematically searched for articles on physical exercise training programmes in ConHD.

Results

A total of 31 articles met all inclusion criteria; in total, 621 subjects (age range 4 to 45 years) were included.Most studies used training programmes with a duration of 12 weeks. On average, the number of training sessions was 3 times per week. In 12 studies, training intensity was set at a percentage of peak heart rate.Outcome measures reported were PeakVO₂, activity levels and muscle strength. Twenty-three studies (72%) found a significant positive change in the main outcome measure after the physical exercise training period. None of the studies reported negative findings related to physical exercise training in ConHD. Cardiac effects have hardly been studied.

Conclusion

In most studies, participation in a physical exercise training programme was safe and improved fitness in children and young adults with ConHD. We recommend that patients with ConHD participate in physical exercise training. Cardiac effects need to be studied more extensively.     

Association between nutritional status and subjective health status in chronically ill children attending special schools

Purpose

In hospitalized children with a chronic disease, malnutrition was associated with a lower subjective health status. In outpatient children with a chronic disease attending special schools, this association has never been studied. The aim of this study was to assess the association between nutritional status and subjective health status in chronically ill children attending special schools.

Methods

Overall, 642 children, median age 9.8 years (IQR 7.7–11.5), 60 % male, 72 % Caucasian, were included in this prospective study in nine special schools for chronically ill children in the Netherlands. Overall malnutrition was assessed as: acute malnutrition (<?2 SDS for weight for height (WFH)) and chronic malnutrition (<?2 SDS for height for age). The malnutrition risk was assessed with the nutritional risk-screening tool STRONG_kids. Subjective health status was assessed with EQ-5D.

Results

Overall, 16 % of the children had overall malnutrition: 3 % acute and 13 % chronic malnutrition. Nurses reported ‘some/severe problems’ on the health status dimensions mobility (15 %), self-care (17 %), usual activities (19 %), pain/discomfort (22 %), and anxiety/depression (22 %) in chronically ill children. Their mean visual analogue scale score (VAS) was 73.0 (SD 11.1). Malnutrition, medication usage, and younger age explained 38 % of the variance of the VAS score.

Conclusions

The presence of overall malnutrition in chronically ill children attending special schools was associated with lower subjective health status, especially in younger children and in those with chronic medication usage. Therefore, it is important to develop and use profile-screening tools to identify these children.