Phase 1 Study of Quercetin, a Natural Antioxidant in Children and Young Adults with Fanconi Anemia (FA).

Fanconi anemia (FA) is a rare inherited disorder characterized by progressive bone marrow failure (BMF) and a predisposition to malignancy. Systemic reactive-oxygen species (ROS) and increased sensitivity of FA hematopoietic progenitors to ROS play a key role in the pathogenesis of BMF. Treatment with antioxidants improve hematopoietic function in Fancc-/- mice.

Revision of metal-on-metal hip replacements with dual-mobility bearings and acetabular component retention.

Aims: In metal-on-metal (MoM) hip arthroplasties and resurfacings, mechanically induced corrosion can lead to elevated serum metal ions, a local inflammatory response, and formation of pseudotumours, ultimately requiring revision. The size and diametral clearance of anatomical (ADM) and modular (MDM) dual-mobility polyethylene bearings match those of Birmingham hip MoM components. If the acetabular component is satisfactorily positioned, well integrated into the bone, and has no surface damage, this presents the opportunity for revision with exchange of the metal head for ADM/MDM polyethylene bearings without removal of the acetabular component.

Multifocal Insulinoma as the Unique Presenting Feature of Multiple Endocrine Neoplasia Type 1 in an Adolescent.

Introduction: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant inherited disorder defined by the presence of two of the following endocrinopathies: primary hyperparathyroidism, anterior pituitary tumors, and duodenopancreatic neuroendocrine tumors (NETs). NETs, which can secrete hormones including insulin, gastrin, and glucagon, among others, are common in patients with MEN1 and are a major cause of morbidity and premature death. NETs are more common later in life, with very few cases described in children.

Mixed manufacturer dual mobility bearing and the Exeter V40 Stem: is it safe? Short-term results in primary and revision hip replacement.

The aim is to review clinical and radiological outcomes for all cases of primary and revision THA, combining a cemented stem (Exeter V40) with a dual mobility component from a different manufacturer (SERF Novae), to evaluate whether concerns regarding mixing components from different manufacturers are justified. We identified 72 hip replacements performed between May 2010 and December 2015 using the SERF Novae dual mobility cup with an Exeter V40 stem, the majority of which were cemented (90%) and revisions (58%). Patients were evaluated clinically and radiologically at a minimum of two years.

Intraoperative cell salvage in revision hip arthroplasty.

Aims: The aim of this study was to perform a systematic review of the evidence for the use of intraoperative cell salvage in patients undergoing revision hip arthroplasty, and specifically to analyze the available data in order to quantify any associated reduction in the use of allogenic blood transfusion, and the volume which is used.

Methods: An electronic search of MEDLINE (PubMed), Embase, Scopus, and the Cochrane Library was completed from the date of their inception to 24 February 2022, using a search strategy and protocol created in conjunction with the PRISMA statement. Inclusion criteria were patients aged > 18 years who underwent revision hip arthroplasty when cell salvage was used.

The Exeter-Trident THA with ceramic-on-ceramic-bearings: 10-year outcomes in 275 total hip arthroplasties.

Aims: This study reports on the Exeter-Trident total hip arthroplasty (THA) using an alumina ceramic-on-ceramic bearing with 10-year clinical and radiographic follow-up.

Patients And Methods: Between January 2001 and January 2006, 275 THAs were performed. Mean age at surgery was 52.

Male pediatric, adolescent, and young adult reproductive survivorship.

As pediatric, adolescent, and young adult cancer survival rates increase, emphasis is placed on reducing late effects, including reproductive complications and potential impact to fertility. Male survivors are at risk of abnormalities in sperm, hormone deficiencies, and sexual dysfunction. This can impact one's progression into puberty and ability to have a biological child and impacts quality of life following treatment.

Incidence of Symptomatic Venous Thromboembolism (VTE) in 8,885 Elective Total Hip Arthroplasty Patients Receiving Post-operative Aspirin VTE Prophylaxis.

Background: Venous thromboembolism (VTE) is a potentially reducible cause of morbidity and mortality in patients undergoing elective hip arthroplasty surgery. The balance of post-operative VTE prophylaxis and risk of post-operative haemorrhage remains at the forefront of surgeon's mind. The National Institute for Health and Care Excellence (NICE) published updated guidelines in 2018 which recommend the use of both mechanical and pharmacological methods in patients undergoing elective total hip arthroplasty (THA).

Cemented Femoral Stem Fixation: Back to the Future.

Background: Periprosthetic fractures following elective and nonelective hip arthroplasty remain one of the most common modes of early failure.

Methods: This symposium will explore the current role of cemented fixation and periprosthetic fracture, focusing on history and rationale for cemented stem fixation, registry data, and other potential advantages of cemented stem fixation. A meticulous and methodical surgical technique of cemented stem fixation is paramount to the success and will be thoroughly discussed.

Gonadal function in pediatric Fanconi anemia patients treated with hematopoietic stem cell transplant.

Gonadal dysfunction and reduced fertility are clinical manifestations well described in patients with Fanconi anemia (FA) and following hematopoietic stem cell transplantation (HSCT). It is difficult to differentiate gonadal dysfunction from the primary disease itself or from HSCT procedures. Therefore, it is important to manage expectations about gonadal failure and infertility for all patients with FA, regardless of the HSCT status.

Reduced-Intensity Conditioning Mitigates Risk for Primary Ovarian Insufficiency but Does Not Decrease Risk for Infertility in Pediatric and Young Adult Survivors of Hematopoietic Stem Cell Transplantation.

Hematopoietic stem cell transplantation (HSCT) is a curative therapy for many pediatric malignant and nonmalignant conditions. Gonadal insufficiency or infertility is present in almost all HSCT survivors who received a myeloablative conditioning (MAC) regimen. Reduced-intensity conditioning (RIC) regimens are being increasingly used in medically fragile patients or in patients with nonmalignant diagnoses to limit the toxicities associated with HSCT; however, the short-term and long-term gonadal toxicity of RIC regimens in pediatric and young adult survivors remains unknown.

Development of the Revision Hip Complexity Classification using a modified Delphi technique.

Aims: The aim of this modified Delphi process was to create a structured Revision Hip Complexity Classification (RHCC) which can be used as a tool to help direct multidisciplinary team (MDT) discussions of complex cases in local or regional revision networks.

Methods: The RHCC was developed with the help of a steering group and an invitation through the British Hip Society (BHS) to members to apply, forming an expert panel of 35. We ran a mixed-method modified Delphi process (three rounds of questionnaires and one virtual meeting).

Is rationing of total hip arthroplasty justified? Working to optimize patient accessibility to surgery using long-term patient-reported outcome data.

Aims: The aim of this study was to assess medium-term improvements following total hip arthroplasty (THA), and to evaluate what effect different preoperative Oxford Hip Score (OHS) thresholds for treatment may have on patients' access to THA and outcomes.

Methods: Patients undergoing primary THA at our institution with an OHS both preoperatively and at least four years postoperatively were included. Rationing thresholds were explored to identify possible deprivation of OHS improvement.

Bone Health Outcomes at 1 Year after Hematopoietic Stem Cell Transplantation in a Heterogeneous Pediatric Population.

Impaired bone mineral density (BMD) is a known complication of hematopoietic stem cell transplantation (HSCT) in adults and may lead to increased fracture risk. Less is known in children about the risks for impaired BMD and fragility (low trauma) fractures after HSCT. In this study, we evaluated the incidence of fragility fractures in a large diverse pediatric HSCT recipient population and identified risk factors for both fracture and impaired BMD.

Incidence of thyroid dysfunction in children after HSCT with reduced intensity conditioning (RIC) or myeloablative conditioning (MAC).

We have previously demonstrated a 11% incidence of post-transplant de novo thyroid disease, even with a radiation-free RIC regimen. Following the enactment of a universal late effects screening program at our institution, we compared the outcomes of 108 pediatric hematopoietic stem cell transplant recipients after a RIC regimen (n = 33) to those after a MAC regimen (n = 75) during the same time period. Overall, 10% of subjects developed thyroid dysfunction after HSCT, with a median follow-up of 669 days.

B2 or not B2? That is the question: a review of periprosthetic fractures around cemented taper-slip femoral components.

Aims: Periprosthetic fractures (PPFs) around cemented taper-slip femoral prostheses often result in a femoral component that is loose at the prosthesis-cement interface, but where the cement-bone interface remains well-fixed and bone stock is good. We aim to understand how best to classify and manage these fractures by using a modification of the Vancouver classification.

Methods: We reviewed 87 PPFs.

Long-Term Survival of the Cemented Exeter Universal Stem in Patients 50 Years and Younger: An Update on 130 Hips.

Background: There is variable evidence regarding survivorship beyond 20 years of total hip arthroplasties in young patients. We report the long-term results of the Exeter cemented hip system in patients ≤50 years at minimum of 20 years.

Methods: Clinical and radiological outcomes of 130 consecutive total hip arthroplasties in 107 patients aged 50 years or younger at primary operation were reviewed; 77% had a diagnosis other than osteoarthritis.

Pituitary disease in pediatric brain tumor survivors.

Introduction: Treatment of childhood brain tumors, including surgical resection and especially external beam radiation, often results in endocrine complications manifested by hypopituitarism, which can involve growth hormone deficiency, hypothyroidism, adrenal insufficiency, disorders of puberty, diabetes insipidus, and hypothalamic obesity.

Areas Covered: A comprehensive literature search was conducted on Medline (publications from the 1990s to 01/2019) including systematic reviews, meta-analyses, longitudinal controlled studies, retrospective cohort studies, and case reports. Herein, we present an up-to-date review of the current literature regarding endocrine sequellae of childhood brain tumor survivors.

The Exeter Short Revision Stem for Cement-in-Cement Femoral Revision: A Five to Twelve Year Review.

Background: Cement-in-cement femoral revision is a proven technique in revision total hip arthroplasty, with excellent results reported using standard-sized Exeter stems. The Exeter 44/00/125 short revision stem was introduced in 2004 to facilitate cement-in-cement revision. The stem is 25-mm shorter and has a slimmer body than standard stems to facilitate adjustment of depth of insertion and stem version.

Not all cemented hips are the same: a register-based (NJR) comparison of taper-slip and composite beam femoral stems.

Background and purpose - No difference in outcome has been demonstrated comparing cemented taper-slip and composite beam designs in short-term randomised trials; we assessed outcome differences using a registry analysis. Patients and methods - All cemented stems with > 100 implantations were identified in the National Joint Registry of England and Wales from April 1, 2003 to September 31, 2013 and categorised as taper-slip or composite beam. Survival analyses using Kaplan-Meier and Cox regression were performed.

Single Ultra-High-Dose Cholecalciferol to Prevent Vitamin D Deficiency in Pediatric Hematopoietic Stem Cell Transplantation.

Vitamin D deficiency is prevalent among childhood hematopoietic stem cell transplantation (HSCT) recipients and associated with inferior survival at 100 days after transplantation. Achieving and maintaining therapeutic vitamin D levels in HSCT recipients is extremely challenging in the first 3 to 6 months after transplantation due to poor compliance in the setting of mucositis and the concomitant use of critical transplantation drugs that interfere with vitamin D absorption. We sought to evaluate the safety and efficacy of a single, ultra-high-dose of vitamin D given before childhood HSCT to maintain levels in a therapeutic range during the peritransplantation period.

Complication of intraprosthetic dislocation of dual-mobility hip implant following closed reduction.

Total hip replacement is a successful operation for the management of hip pain but there are potential complications, of which dislocation is one of the most common. The management of recurrent dislocation is a challenging problem that requires a multimodal approach and the use of dual-mobility implants is one option. We present a patient who was previously revised with a dual-mobility implant for recurrent dislocation, who had a complication after closed reduction of a subsequent intraprosthetic dislocation.

3-phase Technicium-99m bone scanning in patients with pain in the hip region after cemented total hip replacement: a multicentre series of 100 cases.

Introduction: The aim of this study was to assess the benefit of a Technetium-99M (HDP) 3-phase bone scan (TPBS) as an additional diagnostic test in the evaluation of pain in the hip region following cemented total hip replacement (THR) surgery.

Methods: A retrospective study over a 24-month period was performed comprising 100 patients investigated with a TPBS. Investigations were summarised and analysed, and were classified as entirely normal, possibly abnormal, and definitely abnormal.