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Deficiency of Adenosine Deaminase 2 (DADA2) patients presenting with primary immunodeficiency are at risk of uncontrolled EBV infection and secondary malignancies including EBV-related lymphoproliferative disorders (LPD). This paper describes the first case of EBV related diffuse large B-cell lymphoma in a patient with DADA2 and uncontrolled EBV infection. Consideration should be given to monitoring for EBV viraemia and to preventative EBV specific therapy in DADA2 and patients with at risk primary immunodeficiencies. A type I interferon (IFN) gene signature is associated with DADA2 though its association with immune dysregulation is unclear., (© 2024. The Author(s).)

Introduction: The adoption of new technology should be supported by improvements in patient-reported outcomes (PROMs). The purpose of this study was to assess the one-year PROMs of patients who underwent total hip arthroplasty (THA) using a novel, fluoroscopy-based, robotic-assisted (RA-THA) system when compared to a manual, fluoroscopic-assisted technique (mTHA)., Materials and Methods: A review of 91 consecutive mTHA and 85 consecutive RA-THA via a direct anterior approach was conducted. All cases were performed by the same surgeon at the same institution, for a pre-operative diagnosis of osteoarthritis, avascular necrosis, or rheumatoid arthritis. Outcomes included one-year Veterans RAND-12 (VR-12) Physical/Mental, Hip Disability and Osteoarthritis Outcome (HOOS) Pain/Physical Function/Joint Replacement, and University of California Los Angeles (UCLA) Activity scores, as well as the difference between pre-operative and one-year post-operative PROMs., Results: Patients in the RA-THA cohort had lower pre-operative HOOS-JR scores compared to patients in the mTHA cohort (37.0 vs. 43.1; p = 0.031). Cohorts experienced similar one-year post-operative VR-12, HOOS, and UCLA Activity scores. Patients in the RA-THA cohort experienced greater improvements across all pre- and post-operative HOOS scores compared to patients in the mTHA cohort: Pain (+ 54.7 vs. +42.1; p = 0.009), Physical Function (-41.6 vs. -28.7; p = 0.007), and Joint Replacement (+ 46.6 vs. +33.0; p = 0.002). These differences exceeded minimum clinically important difference (MCID)., Conclusions: Both manual and robotic cohorts experienced benefit from THA at one-year post-operative. Importantly, the use of a novel, fluoroscopy-based robotic assistance system for primary THA resulted in greater improvements in PROMs at one-year relative to manual technique., (© 2024. The Author(s).)

Background: Idiopathic Anaphylaxis (IA) is the most common anaphylactic syndrome in adults. Mental health problems associated with IA are not well recognised. We aimed to assess if patients diagnosed with IA were more likely to experience mental health problems compared to a normative Australian population. We additionally hypothesised that the number of anaphylactic episodes would correlate with symptoms of anxiety., Methods: A total of 34 patients with at least one episode of IA were recruited from an adult immunology clinic. Patients were recruited as part of a separate study evaluating alternative aetiologies in IA. Mental health problems were measured using the Depression, Anxiety and Stress Scale (DASS-21). An extension of the survey included questions specifically focused on the psychological impact of IA., Results: Compared to population norms, those with IA had significantly higher levels of mental health problems. Statistically significant DASS-21 scores were identified for depression 4.24 vs. 2.57 (p < 0.001), anxiety 4.76 vs. 1.74 (p < 0.012), stress 7.35 vs. 3.95 (p < 0.001) and total score 16.35 vs. 8.00 (p < 0.001). There was no association between two or more episodes of anaphylaxis and increased anxiety levels (β = 0.52, CI -2.59-3.62, p = 0.74)., Conclusions: This is the first paper to demonstrate that patients living with idiopathic anaphylaxis are more symptomatic for mental illness than those in the community. Screening for mental illness and referral for psychological support should be undertaken in people with IA., (© 2023. Canadian Society of Allergy & Clinical Immunology.)

Introduction: The personality traits of those who become orthopaedic surgeons may also lead to overwork, work-life balance issues, and burnout. Health and wellness practices of orthopaedic surgeons have not been widely explored. This study evaluated the personal health habits, wellness, and burnout of practicing orthopaedic surgeons in the United States., Methods: An anonymous self-assessment survey was completed by 234 practicing orthopaedic surgeon alumni from two large residency programs. The survey assessed exercise habits according to Centers for Disease Control and Prevention recommendations, compliance with preventive medical care practices according to the United States Preventive Services Task Force, prioritization of occupational wellness strategies, and the presence of burnout via an adapted Maslach Burnout Inventory. Survey responders' mean age was 52 years, 88% were male, and 93% had a body mass index <30 kg/m2. Surgeons were stratified according to practice type, years in practice, and subspecialty., Results: Among orthopaedic surgeons, compliance with aerobic and strength exercise recommendations was 31%. Surgeons in academic practice were significantly (P = 0.007) less compliant with exercise recommendations (18%) compared with private (34%) or employed (43%) practicing surgeons. Most (71%) had seen their primary care provider within 2 years and were up to date on age-appropriate health care screening including a cholesterol check within 5 years (79%), colonoscopy (89%), and mammogram (92%). Protecting time away from work for family/friends and finding meaning in work were the most important wellness strategies. The overall burnout rate was 15% and remained not significantly different (P > 0.3) regardless of years in practice, practice type, or subspecialty., Conclusion: This survey study identifies practicing orthopaedic surgeons' health habits and wellness strategies, including limited compliance with aerobic and strength exercise recommendations. Orthopaedic surgeons should be aware of areas of diminished personal wellness to improve quality of life and avoid burnout., (Copyright © 2023 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Orthopaedic Surgeons.)

Background: For patients who have a history of cerebrovascular accident (CVA) with neurological sequelae undergoing primary total hip arthroplasty (THA) and total knee arthroplasty (TKA), we sought to determine mortality rate, implant survivorship, complications, and clinical outcomes., Methods: Our total joint registry identified CVA sequelae patients undergoing primary THA (n = 42 with 25 on affected hip) and TKA (n = 56 with 34 on affected knee). Patients were 1:2 matched based upon age, sex, body mass index, and surgical year to a non-CVA cohort. Mortality and implant survivorship were evaluated via Kaplan-Meier methods. Clinical outcomes were assessed via Harris Hip scores or Knee Society scores . Mean follow-up was 5 years (range, 2-12)., Results: For CVA sequelae and non-CVA patients, respectively, the 5-year patient survivorship was 69 versus 89% after THA (HR = 2.5; P = .006) and 56 versus 90% after TKA (HR = 2.4, P = .003). No significant difference was noted between groups in implant survivorship free from any reoperation after THA (P > .2) and TKA (P > .6). Postoperative CVA occurred at an equal rate in CVA sequelae and non-CVA patients after TKA (1.8%); none after THA in either group. The magnitude of change in Harris Hip scores (P = .7) and Knee Society scores (P = .7) were similar for CVA sequelae and non-CVA patients., Conclusion: Complications, including the risk of postoperative CVA, implant survivorship, and outcome score improvement are similar for CVA sequelae and non-CVA patients. A 2.5-fold increased risk of death at a mean of 5 years after primary THA or TKA exist for CVA sequelae patients., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Solid organ transplant (SOT) patients have increased risk of complications, infection, and mortality after elective total hip arthroplasty (THA). The study aims to compare SOT recipients' clinical outcomes to a matched group of nontransplant patients after nonelective THA and hemiarthroplasty for acute femoral neck fracture (FNF)., Methods: A retrospective review identified 31 SOT patients undergoing hip arthroplasty (24 hemiarthroplasty and 7 THA) for FNF and were matched 1:1 to non-SOT patients based on age, sex, body mass index, surgical procedure, and year of surgery. Patient survivorship, perioperative outcomes, complications, and reoperations were compared. The mean follow-up was 3 years., Results: The estimated survivorship free from mortality for SOT and non-SOT patients at 1- year was not different (77% and 84%, respectively, P  = .52). The 90-day readmission rate was significantly higher with 8 (26%) in the SOT cohort and none in the non-SOT group ( P < .01). Major medical complications occurred in 16% of SOT patients compared to 5% in controls ( P  = .21). Three (10%) reoperations/revisions were required for SOT patients and none in non-SOT group ( P  = .24)., Conclusion: SOT recipients undergoing nonelective hip arthroplasty for FNF demonstrated increased readmission rates compared to matched controls. For this rare clinical scenario, diligent perioperative care by surgeons and multidisciplinary transplant specialists is necessary to mitigate increased risk of SOT patients., (© 2021 The Authors.)

Background: Kawasaki Disease (KD) is the most common paediatric vasculitis affecting small to medium arteries. Although the average age of diagnosis is 3.4 years with a well-defined clinical presentation, older patients with KD including adolescent and adult patients demonstrate a less classical presentation with prominent findings including hepatitis, cervical lymphadenopathy, and arthralgia. We describe a case of an adolescent presentation of Kawasaki Disease presenting with a predominantly cholestatic hepatic picture., Case Presentation: We describe a case of KD in a 16-year-old Caucasian female with predominately hepatic disease that showed resistance to intravenous immunoglobulin (IVIG). The formal diagnosis of KD was made on her 8th day of symptoms. She displayed classical symptoms commencing with fever, followed by peripheral desquamation, strawberry tongue, cervical lymphadenopathy. She became clinically jaundiced with evidence of hepatic artery narrowing on ultrasound that resolved with treatment. Her disease was biphasic and required further IVIG for non-hepatic symptoms. She did not develop coronary aneurysms., Conclusion: Significant hepatic dysfunction with clinical jaundice is rare in KD without associated gall bladder hydrops and tends to occur in older patients. We describe such a case and review the five described cases in the literature. Diagnostic delay is more common in adolescent patients and given that the prognosis of KD is closely correlated to diagnostic timing and provision of care, it is important to consider Kawasaki Disease in older demographics especially with undiagnosed hepatic disease.

Background: Open and arthroscopic approaches have been described to address femoroacetabular impingement (FAI). Despite good outcomes, there is a subset of patients who subsequently require total hip arthroplasty (THA). However, there is a paucity of data on the outcomes of THA after surgery for FAI. The purpose of this study was to determine whether clinical outcomes of THA are affected by prior open or arthroscopic treatment of FAI., Methods: This case-matched retrospective review included 23 patients (24 hips) that underwent THA after previous surgery for FAI (14 arthroscopic and 10 open) and compared them to 24 matched controls with no history of prior surgery on the operative hip. The controls were matched for age, sex, surgical approach, implants used, and preoperative modified Harris hip score (mHHS) did not differ between groups. The primary outcome measure was the mHHS. Operative time, blood loss, and the presence of heterotopic ossification after THA were also compared between groups., Results: There was no significant difference in mean mHHS between the FAI treatment group 92.9 ± 12.7 and controls 95.2 ± 6.6 (P = .43) at a mean follow-up after THA of 33 (24-70) months. Increased operative times were noted for THA after surgical hip dislocation (SHD; mean 109.3 ± 29.8) compared to controls (mean 88.0 ± 24.2; P < .05). There was no significant difference in blood loss between groups. The occurrence of heterotopic ossification was significantly higher after SHD compared to controls (P < .05)., Conclusions: Clinical outcomes after THA are not affected by prior open or arthroscopic procedures for FAI. However, increased operative times and an increased risk of heterotopic ossification were noted after SHD., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Painful hip snapping can present as a rare sequela of proximal hamstring injury. We present a case of painful snapping of the conjoint tendon of the semitendinosus and biceps femoris over the ischial tuberosity in the setting of a partial proximal hamstring disruption. Dynamic ultrasonography identified the source of snapping and a persistent attachment of the conjoint tendon to the sacrotuberous ligament, which prevented retraction and allowed subluxation of the tendons across the ischial tuberosity. Following surgical transection of the persistent sacrotuberous ligament attachment to the conjoint tendon and tendon reattachment, the patient's symptoms resolved with full return of function., (Copyright © 2015 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.)

Background: This study aims to evaluate admission blood glucose level (BGL) in patients presenting to the emergency department (ED) as a risk factor for a major adverse cardiac event (MACE) on presentation and up to 30 days post discharge. Admission BGL is a prognostic indicator in patients with confirmed acute coronary syndrome (ACS). It is unclear if admission BGL improves the diagnosis and stratification of patients presenting to the ED with suspected ACS., Methods: This study is an analysis of data collected from a prospective observational study. The study population consisted of ED patients from Brisbane, Australia and Christchurch, New Zealand. Patients were enrolled between November 2007 and February 2011. Admission BGL was taken as part of routine admission blood with fasting status unknown. The primary end point for this study was a MACE at presentation and up to 30 days post discharge. Logistic regression analyses examined the relationship between admission hyperglycaemia and MACE. A hyperglycaemic threshold of 7 mmol/L was chosen based on WHO standards., Results: A total of 1708 patients were eligible. A MACE was identified in 336 patients (19.7%) within 30 days. Of these 98 had confirmed unstable angina and 232 had non-ST elevation myocardial infarction. Hyperglycaemia was identified in 476 (27.9%) patients with 147 (30.9%) having a MACE. Admission BGL >7 mmol/L was demonstrated as an independent predictor of a MACE (OR1.51 CI 1.06 to 2.14). Gender, age, hypertension, dyslipidaemia, family history, ischaemic ECG and positive troponin remained important factors., Conclusions: Admission BGL is an independent risk factor for a MACE in patients with suspected ACS. Hyperglycaemia should be considered a risk factor for MACEs and consideration be given to its inclusion in existing diagnostic tools., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Background: Some patients with psoriasis may require hospital admission to stabilize their condition, although the role of inpatient management is changing given recent advances in therapeutic options, emphasis on community-based care for chronic conditions and limited healthcare resources. There is a need for evidence-based national standards for inpatient management of psoriasis taking account of factors that predict length of stay., Objectives: To determine which factors predict length of stay for patients with psoriasis requiring inpatient hospital care with a view to setting evidence-based standards for inpatient psoriasis management., Methods: A multicentre service review was conducted on all psoriasis admissions over a 9-month period in four dermatology centres in the U.K. We collected data on admission, at discharge and, where possible, at 3 months following discharge. Psoriasis severity was assessed using four validated scoring systems, including Psoriasis Area and Severity Index (PASI) and Dermatology Life Quality Index. We also recorded length of stay and treatment details., Results: Length of stay varied widely between the four centres, but was similar in the two centres which received a high proportion of tertiary referrals for severe psoriasis (mean 19.7 days, range 1-78, analysis of variance P=0.002). Disease severity, measured by PASI, on admission (mean 15.7, interquartile range 8.3-20.8) was significantly higher in the tertiary centres (P<0.0001). However, there was no significant difference in PASI between centres on discharge. The admission PASI was significantly associated with length of stay (r=0.2, P=0.02). There was no significant correlation between other measures of disease severity and length of stay., Conclusions: Disease severity on admission for patients with psoriasis is greater in tertiary referral centres for psoriasis and is directly associated with length of stay. Length of stay should be used in conjunction with clinical measures such as PASI improvement to set national standards for quality in secondary care.

The endogenous lipid signaling agent oleoylethanolamide (OEA) has recently been described as a peripherally acting agent that reduces food intake and body weight gain in rat feeding models. This paper presents evidence that OEA is an endogenous ligand of the orphan receptor GPR119, a G protein-coupled receptor (GPCR) expressed predominantly in the human and rodent pancreas and gastrointestinal tract and also in rodent brain, suggesting that the reported effects of OEA on food intake may be mediated, at least in part, via the GPR119 receptor. Furthermore, we have used the recombinant receptor to discover novel selective small-molecule GPR119 agonists, typified by PSN632408, which suppress food intake in rats and reduce body weight gain and white adipose tissue deposition upon subchronic oral administration to high-fat-fed rats. GPR119 therefore represents a novel and attractive potential target for the therapy of obesity and related metabolic disorders.

The synthesis, SAR and biological evaluation of a series of ureas that activate glucokinase, a target for diabetes therapy as a result of its critical role in the regulation of whole-body glucose homeostasis, are described. Some of the urea-containing glucokinase activators lowered blood glucose levels in vivo following oral dosing to C57BL/6J mice.