Patient-reported outcomes of Children with an Anorectal Malformation.

Objective: We aimed to study the impact of anorectal malformation (ARM) type and sacral ratio on continence outcomes in children. We secondarily aimed to compare continence outcomes by age group and determine quality of life (QoL) with different bowel regimens.

Summary Background Data: Children with ARM experience dysfunctional stooling into adulthood.

Anorectal Malformation Patients in Australia and Europe: Different Location, Same Problem? A Retrospective Comparative Registry-Based Study.

Anorectal malformations (ARM) encompass a spectrum of rare congenital defects of the rectum and anus, requiring specialized reconstructive surgery. To improve epidemiological and clinical research in rare diseases such as ARM, collaborative efforts and patient registries are key. This retrospective study pools clinical data over a 30-year period from two ARM patient registries (The Royal Children's Hospital (RCH) in Melbourne, Australia, and the ARM-Network Consortium in Europe).

Transition From Pediatric to Adult Healthcare for Colorectal Conditions: A Systematic Review.

Background: Despite surgical advances for complex congenital colorectal conditions, such as anorectal malformation (ARM) and Hirschsprung disease (HD), many adolescents require transfer from specialist pediatric to adult providers for ongoing care.

Methodology: A systematic review of PubMed, MEDLINE and Embase was conducted to identify what is known about the transitional care of patients with ARM and HD (PROSPERO # CRD42022281558). The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) framework guided our reporting of studies that focused on the transition care of 10-30-year-olds with ARM and HD.

Psychosocial Outcomes of Parents of Children with Hirschsprung Disease Beyond Early Childhood.

Purpose: The lifelong impact of Hirschsprung disease (HD) upon children and their families is increasingly well recognized. Parental psychosocial wellbeing and family functioning are determinants of psychological and health-related outcomes in children with chronic conditions. We performed a cross-sectional cohort study to evaluate the psychosocial functioning of parents/caregivers of children with HD, beyond early childhood.

The early years: hirschsprung disease and health-related quality of life.

Purpose: Chronic diseases are notorious in the way that they interfere with many aspects of a child's development, and this holds true for children with Hirschsprung disease (HD). The present research aims to (1) determine whether the health-related quality of life (HRQoL) of HD children differs from healthy paediatric populations; and (2) explore the relationship between HD children's HRQoL and psychosocial outcomes of parents.

Methods: Using a cross-sectional survey study design, children's HRQoL was assessed using the Pediatric Quality of Life Inventory (PedsQL), while parental psychosocial outcomes were measured using the Patient-Reported Outcomes Measurement Information System (PROMIS) anxiety and depression short-forms, Family Management Measure (FaMM), and Parent Experience of Child Illness.

A systematic overview of rare disease patient registries: challenges in design, quality management, and maintenance.

Patient registries serve to overcome the research limitations inherent in the study of rare diseases, where patient numbers are typically small. Despite the value of real-world data collected through registries, adequate design and maintenance are integral to data quality. We aimed to describe an overview of the challenges in design, quality management, and maintenance of rare disease registries.

Post-Operative Anorectal Manometry in Children following Anorectal Malformation Repair: A Systematic Review.

Despite surgical correction, children with anorectal malformations may experience long-term bowel dysfunction, including fecal incontinence and/or disorders of evacuation. Anorectal manometry is the most widely used test of anorectal function. Although considerable attention has been devoted to its application in the anorectal malformation cohort, there have been few attempts to consolidate the findings obtained.

A Quality Assessment of the ARM-Net Registry Design and Data Collection.

Background: Registries are important in rare disease research. The Anorectal Malformation Network (ARM-Net) registry is a well-established European patient registry collecting demographic, clinical, and functional outcome data. We assessed the quality of this registry through review of the structure, data elements, collected data, and user experience.

Post-operative colonic manometry in children with anorectal malformations: A systematic review.

Background: Children with anorectal malformations may experience constipation and fecal incontinence following repair. The contribution of altered anorectal function to these persistent symptoms is relatively intuitive; however, colonic motility in this cohort is less well understood. Manometry may be used to directly assess colonic motility.

Perioperative opioid use in paediatric inguinal hernia patients: A systematic review and retrospective audit of practice.

Background: Opioids play a major role in postoperative pain management in children, but their administration remains an under investigated topic. This study aimed to describe perioperative opioid prescribing practices for paediatric inguinal hernia patients in the literature and at The Royal Children's Hospital (RCH) in Melbourne, Australia.

Material/method: A systematic review of English articles (published from 2009 to 2019) was conducted on paediatric (0-18y) inguinal hernia patients who received a postoperative or discharge opioid prescription, or both.

Post-operative anorectal manometry in children with Hirschsprung disease: A systematic review.

Background: Hirschsprung disease is commonly encountered by pediatric surgeons. Despite advances in the surgical management, these children may experience symptoms of bowel dysfunction throughout adulthood. Anorectal manometry may be used to assess post-operative anorectal structure and function.

Quality of Life Outcomes in Primary Caregivers of Children with Esophageal Atresia.

Objective: To investigate the quality of life (QoL) impact on primary caregivers of children with esophageal atresia.

Study Design: We used a prospective cohort study design, inviting primary caregivers of children with esophageal atresia to complete the following questionnaires: Parent Experience of Child Illness (PECI), Patient-Reported Outcomes Measurement Information System (PROMIS) Anxiety, PROMIS Depression, 12-Item Short Form Survey (SF-12), and Pediatric Quality of Life Inventory (PedsQL). The PECI, PROMIS Anxiety and Depression, and SF-12 assessed caregiver QoL, and the PedsQL assessed patient QoL.

Post-operative colonic manometry in children with Hirschsprung disease: A systematic review.

Background: A significant proportion of children experience bowel dysfunction (including constipation and fecal incontinence) following surgical repair of Hirschsprung disease (HD). Persistent symptoms are thought to relate to underlying colonic and/or anorectal dysmotility. Manometry may be used to investigate the gastrointestinal motility patterns of this population.

Audit of enuresis referrals on the waiting list for a tertiary hospital outpatient clinic.

Aim: Enuresis, defined as intermittent incontinence occurring exclusively during sleep, affects 4-19% of children, but can be effectively treated using education and alarm-bell therapies. However, delays in treatment are likely to impact upon the quality of life of the child, parents and carers. Poor quality and incomplete referrals are thought to be a major driver of inefficiencies.

Polyethylene Glycol Dosing for Constipation in Children Younger Than 24 Months: A Systematic Review.

Objectives: Evaluate safety and effectiveness of Polyethylene glycol (PEG) for chronic constipation in children aged younger than 24 months. Identify the optimum dose of PEG to manage chronic constipation in children aged younger than 24 months.

Methods: In this systematic review, Embase, Medline Ovid, Pubmed, and the Cochrane Library were searched between January 1, 2000 and February 1, 2019.

Paediatric bladder dysfunction: A single centre experience of public hospital wait times.

Aim: Paediatric bladder dysfunction, including daytime urinary incontinence and enuresis, is a common and distressing condition. Unfortunately, children with these symptoms are often on waitlists for several months. This treatment delay may significantly impact upon the child and family unit.

Children who soil: A review of the assessment and management of faecal incontinence.

Soiling is a common and distressing condition affecting children. In the vast majority of patients, it is associated with constipation. Most constipation is functional and is best thought of as difficulty achieving adequate bowel emptying.

The long-term quality of life outcomes in adolescents with Hirschsprung disease.

Background: Postoperative outcomes for Hirschsprung disease (HD) remain variable, with many patients affected by constipation and/or fecal incontinence. The long-term impact upon quality of life (QoL) for HD patients is unclear. We measured long-term QoL outcomes in adolescents with HD using validated questionnaires.

Quality of life outcomes in children with Hirschsprung disease.

Background: Morbidity following repair of Hirschsprung disease (HD) is common. However, quality of life (QoL) results focused on HD children are contradictory. We aimed to measure QoL outcomes in HD children using validated questionnaires.

Enhancing the early home learning environment through a brief group parenting intervention: study protocol for a cluster randomised controlled trial.

Background: The quality of the home learning environment has a significant influence on children's language and communication skills during the early years with children from disadvantaged families disproportionately affected. This paper describes the protocol and participant baseline characteristics of a community-based effectiveness study. It evaluates the effects of 'smalltalk', a brief group parenting intervention (with or without home coaching) on the quality of the early childhood home learning environment.

Parental management of childhood complaints: over-the-counter medicine use and advice-seeking behaviours.

Aims And Objectives: To explore parental management of childhood complaints with respect to factors associated with the purchase of over-the-counter medicines and sources of information accessed by parents.

Background: The use of over-the-counter medicines is extensive, but this practice is not without risks. To ensure safe use, nurses and other healthcare providers need to understand parental reasons for purchase and sources of information they access regarding management practices.

Use of over-the-counter medicines for young children in Australia.

Aim: To describe over-the-counter (OTC) medicine use by Australian parents for children aged birth to 24 months; types of medicines used and indications for use.

Methods: A cross-sectional survey of parents was conducted using a self-administered over-the-counter medicine use questionnaire. A total of 640 questionnaires were distributed to parents of children who attended a hospital outpatient clinic, maternal and child health centre, or a childcare service in Melbourne, Australia.