Background

There is a large body of literature examining factors associated with children's preoperative anxiety; however, cultural variables such as ethnicity and language have not been included.

Aims

The purpose of this investigation was to examine the role of Latino ethnicity and Spanish-speaking families in pediatric preoperative anxiety.

Methods

Participants were 294 children aged 2-15 years of age undergoing outpatient, elective tonsillectomy and/or adenoidectomy surgery and general anesthesia. Participants were recruited and categorized into 3 groups: English-speaking non-Latino White (n = 139), English-speaking Latino (n = 88), and Spanish-speaking Latino (n = 67). Children's anxiety was rated at 2 time points before surgery: the time the child entered the threshold of the operating room (Induction 1) and the time when the anesthesia mask was placed (Induction 2).

Results

Results from separate linear regression models at Induction 1 and Induction 2, respectively, showed that being from a Spanish-speaking Latino family was associated with higher levels of preoperative anxiety compared with being from an English-speaking family. In addition, young age and low sociability was associated with higher preoperative anxiety in children.

Conclusion

Clinicians should be aware that younger, less sociable children of Spanish-speaking Latino parents are at higher risk of developing preoperative anxiety and manage these children based on this increased risk.

Objective

This study was conducted to determine the relationship of frontal lobe cortical thickness and basal ganglia volumes to measures of cognition in adults with sickle cell anemia (SCA).

Methods

Participants included 120 adults with SCA with no history of neurologic dysfunction and 33 healthy controls (HCs). Participants were enrolled at 12 medical center sites, and raters were blinded to diagnostic group. We hypothesized that individuals with SCA would exhibit reductions in frontal lobe cortex thickness and reduced basal ganglia and thalamus volumes compared with HCs and that these structural brain abnormalities would be associated with measures of cognitive functioning (Wechsler Adult Intelligence Scale, 3rd edition).

Results

After adjusting for age, sex, education level, and intracranial volume, participants with SCA exhibited thinner frontal lobe cortex (t = -2.99, p = 0.003) and reduced basal ganglia and thalamus volumes compared with HCs (t = -3.95, p < 0.001). Reduced volume of the basal ganglia and thalamus was significantly associated with lower Performance IQ (model estimate = 3.75, p = 0.004) as well as lower Perceptual Organization (model estimate = 1.44, p = 0.007) and Working Memory scores (model estimate = 1.37, p = 0.015). Frontal lobe cortex thickness was not significantly associated with any cognitive measures.

Conclusions

Our findings suggest that basal ganglia and thalamus abnormalities may represent a particularly salient contributor to cognitive dysfunction in adults with SCA.

Objectives

Using multiple well-validated measures and a large sample size, the goal of this paper was to describe the immediate clinical and behavioral recovery of children following tonsillectomy with or without an adenoidectomy (T&A) during the first two weeks following surgery.

Study design

Observational, longitudinal study.

Setting

Four major pediatric hospitals in the U.S. consisting of Children's Hospital of Orange County, Children's Hospital of Los Angeles, Lucile Packard Children's Hospital, and Children's Hospital Colorado.

Subjects

and Methods: Participants included 827 patients between 2 and 15 years of age who underwent tonsillectomy with or without adenoidectomy surgery. Baseline and demographic information were gathered prior to surgery, and measures of clinical, behavioral, and physical recovery were recorded immediately following and up through two weeks after surgery.

Results

Pain following T&A was clinically significant through the first post-operative week and nearly resolved by the end of the second week. Negative behavioral changes were highly prevalent after surgery (75.6% of children at Day 0) through the first week (63.9% at Week 1), and over 20% of children continued to evidence new onset negative behavioral changes at two weeks post-operatively. Children were rated as experiencing significant functional impairment in the immediate three days following surgery and most children returned to baseline functioning by the end of the second week.

Conclusions

Results of this study suggest that children show immediate impairment in functioning and experience clinically significant pain throughout the first week following T&A, and new onset maladaptive behavioral changes persisting even up to the two-week assessment period.

Objective

The purpose of this study is to compare postoperative pain scores between children undergoing tonsillectomy and adenoidectomy (T&A) surgery and their parents, identify potential predictors for this disagreement, and determine possible impact on analgesic administration.

Methods

This is a prospective longitudinal study conducted with children undergoing outpatient T&A in 4 major tertiary hospitals and their parents. Children and their parents were enrolled prior to surgery and completed baseline psychological instruments assessing parental anxiety (STAI), parental coping style (MBSS), child temperament (EAS) and parental medication administration attitude questionnaire (MAQ). Postoperatively, parents and children completed at-home pain severity ratings (Faces Pain Scale-Revised, children; Numeric Rating Scale, parents) on postoperative recovery days 1, 2, and 3, reflecting an overall pain level for the past 24 h. Parents also completed a log of analgesic administration. Based on postoperative pain scores, parent-child dyads were classified as overestimators (i.e., parents rated their child's pain higher than children rated their own pain), in agreement (i.e., rating in agreement), or underestimators (i.e., parents rated their child's pain lower than children rated their own pain).

Results

A significant proportion of parent-child pairs disagreed on pain ratings on postoperative days 1-3 (30.05%-35.95%). Of those pairs in disagreement, the majority of parents overestimated their child's pain on all three postoperative days, specifically such that a total of 24-26% parents overestimated their child's pain on postoperative days 1, 2, and 3. Repeated measures ANOVA demonstrated that parents in the overestimator group administered higher, though still within safe limits, amounts of ibuprofen and oxycodone (mg/day) than did the underestimator or agreement groups. Multiple regression models showed hospital site as the only independent predictor for postoperative pain rating disagreement between children and parents.

Conclusions

Since parents overestimate their child's postoperative pain and may administer more analgesics to their child, it is essential to develop a standardized method of child pain assessment and a tailored recommended postoperative analgesic regimen amongst medical providers for children undergoing T&A.

Background

Satisfaction in the hospital setting is an important component of both hospital funding and patient experience. When it comes to a child's hospital experience, parent satisfaction of their child's perioperative care is also necessary to understand. However, little research has been conducted on the predictors of this outcome. Therefore, the purpose of this current study was to validate a priori selected predictors for parental satisfaction in their child's perioperative process.

Methods

Eight hundred and ten pediatric patients who underwent tonsillectomy and adenoidectomy surgery and their parents were included in this study. The primary outcome was assessed using a 21-item parent satisfaction questionnaire resulting in three satisfaction scores: overall care satisfaction, OR/induction satisfaction, and total satisfaction.

Results

Descriptive statistics and correlational analysis found that sedative-premedication, parental presence at anesthesia induction, child social functioning, parental anxiety, and language were all significant predictors of various components of the satisfaction score. Regression models, however, revealed that only parent anxiety and child social functioning remained significant predictors such that parents who reported lower state anxiety (OR/induction satisfaction: OR = 0.975, 95% CI [0.957, 0.994]; total satisfaction: OR = 0.968, 95% CI [0.943, 0.993]) and who had higher socially functioning children (overall care satisfaction: OR = 1.019, 95% CI [1.005, 1.033]; OR/induction satisfaction: OR = 1.011, 95% CI [1.000, 1.022]) were significantly more satisfied with the perioperative care they received.

Conclusion

Lower parent anxiety and higher child social functioning were predictive of higher parental satisfaction scores.

Introduction

Mucopolysaccharidosis (MPS) VI or Maroteaux-Lamy syndrome (253200) is an autosomal recessive lysosomal storage disorder caused by deficiency in N-acetylgalactosamine-4-sulfatase (arylsulfatase B). The heterogeneity and progressive nature of MPS VI necessitates a multidisciplinary team approach and there is a need for robust guidance to achieve optimal management. This programme was convened to develop evidence-based, expert-agreed recommendations for the general principles of management, routine monitoring requirements and the use of medical and surgical interventions in patients with MPS VI.

Methods

26 international healthcare professionals from various disciplines, all with expertise in managing MPS VI, and three patient advocates formed the Steering Committee group (SC) and contributed to the development of this guidance. Members from six Patient Advocacy Groups (PAGs) acted as advisors and attended interviews to ensure representation of the patient perspective. A modified-Delphi methodology was used to demonstrate consensus among a wider group of healthcare professionals with expertise and experience managing patients with MPS VI and the manuscript has been evaluated against the validated Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument by three independent reviewers.

Results

A total of 93 guidance statements were developed covering five domains: (1) general management principles; (2) recommended routine monitoring and assessments; (3) enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT); (4) interventions to support respiratory and sleep disorders; (5) anaesthetics and surgical interventions. Consensus was reached on all statements after two rounds of voting. The greatest challenges faced by patients as relayed by consultation with PAGs were deficits in endurance, dexterity, hearing, vision and respiratory function. The overall guideline AGREE II assessment score obtained for the development of the guidance was 5.3/7 (where 1 represents the lowest quality and 7 represents the highest quality of guidance).

Conclusion

This manuscript provides evidence- and consensus-based recommendations for the management of patients with MPS VI and is for use by healthcare professionals that manage the holistic care of patients with the intention to improve clinical- and patient-reported outcomes and enhance patient quality of life. It is recognised that the guidance provided represents a point in time and further research is required to address current knowledge and evidence gaps.

Introduction

Mucopolysaccharidosis (MPS) IVA or Morquio A syndrome is an autosomal recessive lysosomal storage disorder (LSD) caused by deficiency of the N-acetylgalactosamine-6-sulfatase (GALNS) enzyme, which impairs lysosomal degradation of keratan sulphate and chondroitin-6-sulphate. The multiple clinical manifestations of MPS IVA present numerous challenges for management and necessitate the need for individualised treatment. Although treatment guidelines are available, the methodology used to develop this guidance has come under increased scrutiny. This programme was conducted to provide evidence-based, expert-agreed recommendations to optimise management of MPS IVA.

Methods

Twenty six international healthcare professionals across multiple disciplines, with expertise in managing MPS IVA, and three patient advocates formed the Steering Committee (SC) and contributed to the development of this guidance. Representatives from six Patient Advocacy Groups (PAGs) were interviewed to gain insights on patient perspectives. A modified-Delphi methodology was used to demonstrate consensus among a wider group of healthcare professionals with experience managing patients with MPS IVA and the manuscript was evaluated against the validated Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument by three independent reviewers.

Results

A total of 87 guidance statements were developed covering five domains: (1) general management principles; (2) recommended routine monitoring and assessments; (3) disease-modifying interventions (enzyme replacement therapy [ERT] and haematopoietic stem cell transplantation [HSCT]); (4) interventions to support respiratory and sleep disorders; (5) anaesthetics and surgical interventions (including spinal, limb, ophthalmic, cardio-thoracic and ear-nose-throat [ENT] surgeries). Consensus was reached on all statements after two rounds of voting. The overall guideline AGREE II assessment score obtained for the development of the guidance was 5.3/7 (where 1 represents the lowest quality and 7 represents the highest quality of guidance).

Conclusion

This manuscript provides evidence- and consensus-based recommendations for the management of patients with MPS IVA and is for use by healthcare professionals that manage the holistic care of patients with the intention to improve clinical- and patient-reported outcomes and enhance patient quality of life. It is recognised that the guidance provided represents a point in time and further research is required to address current knowledge and evidence gaps.