ABSTRACT
Although pain is a near-universal experience, pain expression and beliefs are highly variable and can affect assessment and management of pain. This systematic review seeks to synthesize the research findings regarding pain management for Australian Aboriginal and Torres Strait Islander peoples addressing variation as voiced by patients, clinicians, and researchers alike. A systematic review was performed across 7 research databases for all articles related to pain within Indigenous Australian peoples. Additional literature was identified by hand-searching reference lists. Articles were restricted to literature which addressed pain within Indigenous Australians as the primary focus of the article. Thematic analysis was performed to group articles according to those which focussed on the experience, expression, assessment, or management of pain. A total of 294 articles were identified on initial search of literature, of which 20 met inclusion criteria for this study. This review captured gross heterogeneity in cohorts, research methodologies, and conditions studied, making generalized assumptions impossible and inappropriate. Studies suggest that the beliefs of both patients and practitioners are important considerations in approaching effective assessment and management of pain. Health practitioners should appreciate how our own beliefs influence the management of patients and must ensure community consultation is undertaken in order to improve pain assessment and management.
ABSTRACT
OBJECTIVES: To evaluate the clinical features of erythromelalgia in childhood associated with gain-of-function SCN9A mutations that increase activity of the Nav1.7 voltage-gated sodium channel, we conducted a systematic review of pediatric presentations of erythromelalgia related to SCN9A mutations, and compared pediatric clinical presentations of symptomatic erythromelalgia, with or without SCN9A mutations. STUDY DESIGN: PubMed, Embase, and PsycINFO Databases were searched for reports of inherited erythromelalgia in childhood. Clinical features, management, and genotype were extracted. Case notes of pediatric patients with erythromelalgia from the Great Ormond Street Hospital Pain Service were reviewed for clinical features, patient-reported outcomes, and treatments. Children aged over 10 years were recruited for quantitative sensory testing. RESULTS: Twenty-eight publications described erythromelalgia associated with 15 different SCN9A gene variants in 25 children. Pain was severe and often refractory to multiple treatments, including nonspecific sodium channel blockers. Skin damage or other complications of cold immersion for symptomatic relief were common (60%). SCN9A mutations resulting in greater hyperpolarizing shifts in Nav1.7 sodium channels correlated with symptom onset at younger ages (P = .016). Variability in reporting, and potential publication bias toward severe cases, limit any estimations of overall prevalence. In our case series, symptoms were similar but comorbidities were more common in children with SCN9A mutations. Quantitative sensory testing revealed marked dynamic warm allodynia. CONCLUSIONS: Inherited erythromelalgia in children is associated with difficult-to-manage pain and significant morbidity. Standardized reporting of outcome and management in larger series will strengthen identification of genotype-phenotype relationships. More effective long-term therapies are a significant unmet clinical need.
