Whole Body, Whole Life, Whole Family: Patients' Perspectives on X-Linked Hypophosphatemia.

The rare genetic disorder X-linked hypophosphatemia (XLH) is often exclusively considered to impact children, and, as such, adult patients with XLH may receive inadequate care because their symptoms are not associated with XLH. However, studies have shown that XLH has long-term adverse health consequences that continue throughout adulthood requiring comprehensive lifelong care. Indeed, XLH impacts patients' whole body, whole life, and whole family. XLH does not just affect the bones; symptoms are chronic and progressive, worsening throughout adulthood, and the burden of XLH overflows into the lives of a patient's family, friends, peers, and colleagues. To ensure early recognition, comprehensive care, and adequate management of XLH, there are key steps that clinicians can incorporate into their daily practice. These include education, a multidisciplinary approach, open communication, and support. Clinician education on rare disorders such as XLH is critical, and healthcare professionals (HCPs) should ensure that patients and their caregivers have access to XLH-related information. As a whole-body disorder, XLH requires a coordinated approach to treatment across specialties. Frequent open communication among members of the healthcare team is needed to increase HCPs' knowledge about XLH, and open communication must extend to the patient as well to ensure the patient's concerns and needs are addressed and treatment is tailored to their specific individual needs. Multiple networks of support, including social and psychological support, should be offered to patients and their families. A basic understanding that XLH affects patients' whole bodies, whole lives, and whole families is the first step toward accomplishing improved patient care.

The Role of Prophylactic Peroneal Nerve Decompression in Patients with Severe Valgus Deformity at the Time of Primary Total Knee Arthroplasty.

Background: Common peroneal nerve (PN) palsy after total knee arthroplasty (TKA) is a serious complication. Although many authors suggest delayed or immediate PN decompression after TKA in these patients, little is known about the role of prophylactic peroneal nerve decompression (PPND) at the time of TKA. The aim is to report the results of PPND in high-risk patients at the time of TKA. Materials and methods: A multi-institutional retrospective study review of nine patients (10 knees) who underwent PPND at the time of TKA was conducted. Patients who had severe valgus deformities (≥15° of femorotibial angle and not fully correctable by examination under anaesthesia) with or without flexion contractures were included. PPND was performed through a separate 3-4-cm incision at the time of TKA. The demographics, preoperative and postoperative anatomical and mechanical alignments, range of motion, operation time, postoperative neurological function and complications were recorded. Results: All patients had a completely normal motor and sensory neurological function postoperatively and no complications related to PPND were reported. All patients followed the standard physical therapy protocol after TKA without modifications.The mean preoperative femorotibial angle was 20° (range 15-33°) and the mean postoperative femorotibial angle was 6.3° (range 5-9°) (p = 0.005). The mean preoperative flexion contracture was 9 (range 0-20) and the mean residual contracture was 1.2° (range 2-5°) (p = 0.006). Conclusion: PPND at the time of TKA is an option to minimise the risk of PN palsy in high-risk patients. This approach can be considered for patients undergoing TKA in selected high-risk patients with a severe valgus deformity. How to cite this article: Makhdom A, Hamilton AA, Rozbruch SR. The Role of Prophylactic Peroneal Nerve Decompression in Patients with Severe Valgus Deformity at the Time of Primary Total Knee Arthroplasty. Strategies Trauma Limb Reconstr 2022;17(1):38-43.

Guidelines for the management of psychosis in the context of mood disorders.

Psychotic episodes occur in a substantial proportion of patients suffering from major mood disorders (both unipolar and bipolar) at some point in their lives. The nature of these episodes is less well understood than the more common, non-psychotic periods of illness and hence their management is also less sophisticated. This is a concern because the risk of suicide is particularly high in this subtype of mood disorder and comorbidity is far more common. In some cases psychotic symptoms may be signs of a comorbid illness but the relationship of psychotic mood to other forms of psychosis and in particular its interactions with schizophrenia is poorly understood. Therefore, our targeted review draws upon extant research and our combined experience to provide clinical context and a framework for the management of these disorders in real-world practice - taking into consideration both biological and psychological interventions.

STRYDE versus PRECICE magnetic internal lengthening nail for femur lengthening.

INTRODUCTION: Magnetic internal lengthening nails (MILNs) have been used for femoral lengthening to avoid complications associated with external fixation. The titanium version of the MILN (PRECICE®) has been in use since 2011 but had limitations (50-75 lb) in post-operative weight bearing. A new stainless-steel version of the MILN (STRYDE®) allows 150-250 lb of post-operative weight bearing. The aim is to compare the outcomes of using these two different MILNs for both unilateral and bilateral femoral lengthening. METHODS: A single-center, retrospective cohort study was conducted in which patients' records were reviewed from the period from January 2017 to March 2020. A total of 66 femoral lengthening procedures were included in the study and were divided into two groups: STRYDE® group (30 femora) and PRECICE® group (36 femora). Outcomes assessed were the 6-months post-operative Limb Deformity-Scoliosis Research Society (LD-SRS) Score, adjacent joint range of motion (ROM), average distraction rate, bone healing index (BHI), and complications. RESULTS: No statistically significant difference was found between the two groups in regard to the (LD-SRS) score, hip ROM, or knee ROM. Statistically significant differences were found between the two groups in regard to BHI (average of 0.84 months/cm and 0.67 months/cm for STRYDE® and PRECICE®, respectively) and distraction rate (average of 0.6 mm/day and 0.9 mm/day for STRYDE® and PRECICE®, respectively). No mechanical nail complications were reported in the STRYDE® group compared to three events of nail failure in the PRECICE® group. One femur in the PRECICE® group needed BMAC injection for delayed healing compared to four femurs in the STRYDE® group. CONCLUSION: The STRYDE® MILN yields comparable functional results to those of PRECICE® MILN and shows fewer mechanical nail complications. However, STRYDE® MILN requires a slower distraction rate and yields slower healing (larger BHI). LEVEL OF EVIDENCE: Level III, Therapeutic study.

Understanding treatment delay: Perceived barriers preventing treatment-seeking for eating disorders.

OBJECTIVE: Only a small proportion of individuals with an eating disorder will receive targeted treatment for their illness. The aim of this study was to examine the length of delay to treatment-seeking and determine the barriers preventing earlier access and utilisation of eating disorder treatment for each diagnostic group - anorexia nervosa, bulimia nervosa, binge eating disorder and other specified feeding or eating disorder. METHOD: Participants were recruited as part of the TrEAT multi-phase consortium study. One hundred and nineteen Australians (13-60 years; 96.9% female) with eating disorders currently accessing outpatient treatment for their illness completed an online survey comprised of self-report measures of eating disorder severity, treatment delay and perceived barriers to treatment-seeking. The treating clinician for each participant also provided additional information (e.g. body mass index and diagnosis). RESULTS: Overall, the average length of delay between onset of eating disorder symptoms and treatment-seeking was 5.28 years. Controlling for age, latency to treatment-seeking was significantly longer for individuals with bulimia nervosa and binge eating disorder compared to anorexia nervosa. However, when perceived barriers to treatment-seeking were investigated, there were no significant differences between the diagnostic groups in regard to the perceived barriers they experienced. Stigma was rated as the most impactful barrier for each diagnostic group. CONCLUSION: Findings suggest that individuals with eating disorders face substantial delays in accessing appropriate treatment and that latency to treatment-seeking is often magnified for counter-stereotypical eating disorder presentations. Further research is required to investigate other factors contributing to this delay.

Humerus Lengthening: A Comparison of the Internal Lengthening Nail to External Fixation.

Introduction: Magnetic internal lengthening nails (MILNs) have been used for humeral lengthening to avoid complications associated with external fixation. Purpose/Questions: We compared the 1-year Disabilities of the Arm, Shoulder and Hand (DASH) score, adjacent joint range of motion (ROM), bone healing index (BHI), length achieved, distraction rate, and complications when lengthening the humerus using MILN vs using external fixation. Methods: We conducted a retrospective cohort study of 18 patients (22 humeri) from January 2001 to March 2020 divided into 2 groups, the MILN group (7 patients, 7 humeri) and the mono-lateral fixator group (11 patients, 15 humeri). Results: The MILN group showed larger improvement of DASH scores (average 26.8 and 8 for MILN and fixator groups, respectively), less loss of elbow ROM (average 5° and 7° for MILN and fixator groups, respectively), and shorter time to full recovery of elbow ROM (average 39 days and 122 days for MILN and fixator groups, respectively). In the MILN group, there was slower distraction rate (average 0.66 mm/day and 0.86 mm/day for MILN and fixator groups, respectively), less lengthening achieved (average 5.2 cm and 7 cm for MILN and fixator group, respectively), and a lower lengthening percentage (average 19% and 41% for MILN and fixator group, respectively). Bone healing index (BHI) of 0.94 and 0.99 months/cm for the MILN and the fixator groups were similar. Conclusion: Humeral lengthening using the MILN allowed for early full recovery of joint ROM with comparable functional and radiographic outcomes compared with using external fixators.

Core psychosocial issues for children and adolescents in the context of limb lengthening and reconstruction surgery treatment.

PURPOSE: Addressing the psychosocial needs of adolescents can improve surgical outcomes. The aim of this retrospective comparative study was to understand the core psychosocial factors that shaped the experiences of adolescents who underwent multiple limb lengthening/reconstruction surgeries (LLRS). METHODS: A novel 62-question survey was developed and administered to 31 patients from the study institution. Data was obtained using a self-report inventory assessing medical care, communication/connection to doctor, peer relations, physical space, self-esteem, counselling/clergy, physical/emotional support, school issues and concerns about future. This survey and demographic questions were administered to young adults (now aged 18 to 30 years) who underwent LLRS treatment between the ages of 11 to 20 years. RESULTS: Psychosocial needs were determined to be within the categories of body image/self-esteem, subjective perception of treatment, patient-physician relationship, role of parents, peer interactions, academic performance and hospital experience. Patients valued parental involvement while also wanting their surgeon to speak directly to them. They preferred to be in private rooms on the paediatric floor and to not socialize while in the hospital. They were indifferent to keeping up with friends, speaking to a counsellor and having their surgeon inquire about their emotions. They expressed concern about pain, carrying out activities and the financial impact of surgery. CONCLUSIONS: Adolescent LLRS patients value focused psychosocial support from their surgeon and caregivers. This perceived level of support influences their ability to cope with their condition. These findings helped us understand the psychological issues and preferences of adolescents who underwent LLRS and can assist orthopaedic surgeons in providing holistic care. LEVEL OF EVIDENCE: IV.

The 2020 Royal Australian and New Zealand College of Psychiatrists clinical practice guidelines for mood disorders: Major depression summary.

OBJECTIVES: To provide a succinct, clinically useful summary of the management of major depression, based on the 2020 Royal Australian and New Zealand College of Psychiatrists clinical practice guidelines for mood disorders (MDcpg2020 ). METHODS: To develop the MDcpg2020 , the mood disorders committee conducted an extensive review of the available literature to develop evidence-based recommendations (EBR) based on National Health and Medical Research Council (NHMRC) guidelines. In the MDcpg2020 , these recommendations sit alongside consensus-based recommendations (CBR) that were derived from extensive deliberations of the mood disorders committee, drawing on their expertise and clinical experience. This guideline summary is an abridged version that focuses on major depression. In collaboration with international experts in the field, it synthesises the key recommendations made in relation to the diagnosis and management of major depression. RESULTS: The depression summary provides a systematic approach to diagnosis, and a logical clinical framework for management. The latter begins with Actions, which include important strategies that should be implemented from the outset. These include lifestyle changes, psychoeducation and psychological interventions. The summary advocates the use of antidepressants in the management of depression as Choices and nominates seven medications that can be trialled as clinically indicated before moving to Alternatives for managing depression. Subsequent strategies regarding Medication include Increasing Dose, Augmenting and Switching (MIDAS). The summary also recommends the use of electroconvulsive therapy (ECT), and discusses how to approach non-response. CONCLUSIONS: The major depression summary provides up to date guidance regarding the management of major depressive disorder, as set out in the MDcpg2020 . The recommendations are informed by research evidence in conjunction with clinical expertise and experience. The summary is intended for use by psychiatrists, psychologists and primary care physicians, but will be of interest to all clinicians and carers involved in the management of patients with depressive disorders.

The 2020 Royal Australian and New Zealand College of psychiatrists clinical practice guidelines for mood disorders: Bipolar disorder summary.

OBJECTIVES: To provide a succinct, clinically useful summary of the management of bipolar disorder, based on the 2020 Royal Australian and New Zealand College of Psychiatrists clinical practice guidelines for mood disorders (MDcpg2020 ). METHODS: To develop the MDcpg2020 , the mood disorders committee conducted an extensive review of the available literature to develop evidence-based recommendations (EBR) based on National Health and Medical Research Council (NHMRC) guidelines. In the MDcpg2020 , these recommendations sit alongside consensus-based recommendations (CBR) that were derived from extensive deliberations of the mood disorders committee, drawing on their expertise and clinical experience. This guideline summary is an abridged version that focuses on bipolar disorder. In collaboration with international experts in the field, it synthesises the key recommendations made in relation to the diagnosis and management of bipolar disorder. RESULTS: The bipolar disorder summary provides a systematic approach to diagnosis, and a logical clinical framework for management. It addresses the acute phases of bipolar disorder (mania, depression and mixed states) and its longer-term management (maintenance and prophylaxis). For each phase it begins with Actions, which include important strategies that should be implemented from the outset wherever possible. These include for example, lifestyle changes, psychoeducation and psychological interventions. In each phase, the summary advocates the use of Choice medications for pharmacotherapy, which are then used in combinations along with additional Alternatives to manage acute symptoms or maintain mood stability and provide prophylaxis. The summary also recommends the use of electroconvulsive therapy (ECT) for each of the acute phases but not for maintenance therapy. Finally, it briefly considers bipolar disorder in children and its overlap in adults with borderline personality disorder. CONCLUSIONS: The bipolar disorder summary provides up to date guidance regarding the management of bipolar disorder, as set out in the MDcpg2020 . The recommendations are informed by evidence and clinical expertise and experience. The summary is intended for use by psychiatrists, psychologists and primary care physicians but will be of interest to anyone involved in the management of patients with bipolar disorder.

Cognitive side-effects of electroconvulsive therapy: what are they, how to monitor them and what to tell patients.

BACKGROUND: Electroconvulsive therapy (ECT) is recommended in treatment guidelines as an efficacious therapy for treatment-resistant depression. However, it has been associated with loss of autobiographical memory and short-term reduction in new learning. AIMS: To provide clinically useful guidelines to aid clinicians in informing patients regarding the cognitive side-effects of ECT and in monitoring these during a course of ECT, using complex data. METHOD: A Committee of clinical and academic experts from Australia and New Zealand met to the discuss the key issues pertaining to ECT and cognitive side-effects. Evidence regarding cognitive side-effects was reviewed, as was the limited evidence regarding how to monitor them. Both issues were supplemented by the clinical experience of the authors. RESULTS: Meta-analyses suggest that new learning is impaired immediately following ECT but that group mean scores return at least to baseline by 14 days after ECT. Other cognitive functions are generally unaffected. However, the finding of a mean score that is not reduced from baseline cannot be taken to indicate that impairment, particularly of new learning, cannot occur in individuals, particularly those who are at greater risk. Therefore, monitoring is still important. Evidence suggests that ECT does cause deficits in autobiographical memory. The evidence for schedules of testing to monitor cognitive side-effects is currently limited. We therefore make practical recommendations based on clinical experience. CONCLUSIONS: Despite modern ECT techniques, cognitive side-effects remain an important issue, although their nature and degree remains to be clarified fully. In these circumstances it is useful for clinicians to have guidance regarding what to tell patients and how to monitor these side-effects clinically.