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AimsWe aimed to compare the implant survival, complications, readmissions, and mortality of Vancouver B2 periprosthetic femoral fractures (PFFs) treated with internal fixation with that of B1 PFFs treated with internal fixation and B2 fractures treated with revision arthroplasty.MethodsWe retrospectively reviewed the data of 112 PFFs, of which 47 (42%) B1 and 27 (24%) B2 PFFs were treated with internal fixation, whereas 38 (34%) B2 fractures underwent revision arthroplasty. Decision to perform internal fixation for B2 PFFs was based on specific radiological (polished femoral components, intact bone-cement interface) and clinical criteria (low-demand patient). Median follow-up was 36.4 months (24 to 60). Implant survival and mortality over time were estimated with the Kaplan-Meier method. Adverse events (measured with a modified Dindo-Clavien classification) and 90-day readmissions were additionally compared between groups.ResultsIn all, nine (8.01%) surgical failures were detected. All failures occurred within the first 24 months following surgery. The 24-month implant survival was 95.4% (95% confidence interval (CI) 89.13 to 100) for B1 fractures treated with internal fixation, 90% (95% CI 76.86 to 100) for B2 PFFs treated with osteosynthesis-only, and 85.8% (95% CI 74.24 to 97.36) for B2 fractures treated with revision THA, without significant differences between groups (p = 0.296). Readmissions and major adverse events including mortality were overall high, but similar between groups (p > 0.05). The two-year patient survival rate was 87.1% (95% CI 77.49 to 95.76), 66.7% (95% CI 48.86 to 84.53), and 84.2% (95% CI 72.63 to 95.76), for the B1 group, B2 osteosynthesis group, and B2 revision group, respectively (p = 0.102).ConclusionImplant survival in Vancouver B2 PFFs treated with internal fixation was similar to that of B1 fractures treated with the same method and to B2 PFFs treated with revision arthroplasty. Low-demand, elderly patients with B2 fractures around well-cemented polished femoral components with an intact bone-cement interface can be safely treated with internal fixation.Cite this article: Bone Joint J2021;103-B(7):1222–1230.

Objectives: Compare the clinical and radiological outcome of CFP stem with the MiniHip design in a prospective series, with special interest in intraoperative periprosthetic fracture (IPPF).Methods: We prospectively followed 101 cases treated with the MiniHip stem (Group 1) and 89 with the CFP stem (Group 2) operated between 2010 and 2014. No significant demographic differences were observed between both groups. Median follow-up was 72 months. Average stem length was 41% shorter in the MiniHip group (p<0.001). Radiological parameters were measured and a logistcic regression model was created to evaluate factors associated with IPPF.Results: Mean mHHS improved from 54 to 95 in the MiniHip group (p<0.001) and from 64 to 98 in the CFP group (p<0.001). No significant differences were observed in terms of loosening, infection or instability. We observed 7 IPPFs (3.68%), 3 in group 1 and 4 in group 2. After adjusting for confounders, CFP was not associated with a greater risk of IPPF (OR 3.23; 95% CI, 0.250–42.034, p=0.368), however, a more complex fracture pattern was observed with this stem design. Prior acetabular fractures were associated with IPPF (OR 66.85; 95% CI, 1.142–3911, p=0.043). Compared to Dorr A femurs, type Dorr B appeared protective against IPPF (OR 0.039; 95% CI, 0.001–1.109, p=0.058). Valgus alignment tended to increase the risk of IPPF (OR 20.59; 95% CI 0.870–487.221, p=0.061).Conclusions: MiniHip showed similar radiological outcomes to CFP at short- to mid-term follow-up without increasing IPPFs with a shorter stem length. Given that CFP produced a more complex IPPF pattern, surgeons should be cautious with alignment of this particular design, especially in Dorr A femur.

AimsWe aimed to report the mid- to long-term rates of septic and aseptic failure after two-stage revision surgery for periprosthetic joint infection (PJI) following total hip arthroplasty (THA).MethodsWe retrospectively reviewed 96 cases which met the Musculoskeletal Infection Society criteria for PJI. The mean follow-up was 90 months (SD 32). Septic failure was assessed using a Delphi-based consensus definition. Any further surgery undertaken for aseptic mechanical causes was considered as aseptic failure. The cumulative incidence with competing risk analysis was used to predict the risk of septic failure. A regression model was used to evaluate factors associated with septic failure. The cumulative incidence of aseptic failure was also analyzed.ResultsThere were 23 septic failures at final follow-up, with a cumulative incidence of 14% (95% confidence interval (CI) 8% to 22%) at one year, 18% (95% CI 11% to 27%) at two years, 22% (95% CI 14% to 31%) at five years, and 23% (95% CI 15% to 33%) at ten years. Having at least one positive culture (hazard ratio (HR) 2.38 (interquartile range (IQR) 1.19 to 4.74); p = 0.013), or a positive intraoperative frozen section (HR 2.55 (IQR 1.06 to 6.15); p = 0.037) was significantly associated with septic failure after reimplantation. With dislocation being the most common cause of aseptic revision (5.2%), the cumulative incidence of aseptic failure was 1% (95% CI 0% to 5%) at one year, 6% (95% CI 1% to 8%) at five years, and 8% (95%CI 3% to 17%) at ten years.ConclusionIf there is no recurrent infection in the five years following reimplantation, the chances of further infection thereafter are remote. While the results of a frozen section may be a reliable guide to the timing of reimplantation, intraoperative culture has, currently, only prognostic value. Surgeons should be aware that instability remains a potential indication for further revision surgery.Cite this article: Bone Joint J2021;103-B(1):79–86.

En este estudio se explora la forma en que el envejecimiento afecta a la producción del habla. Indudablemente, son muchos los cambios que se producen a edades avanzadas en los procesos de control motor, así como en la integridad de los músculos laríngeos y supralaríngeos, y que pueden afectar a la producción del habla. Sin embargo, hay pocos trabajos que examinen estas capacidades en el marco del envejecimiento saludable, especialmente en personas de habla francesa.

Purpose: There is little known about investigating the importance of all proximity dimensions simultaneously as a result of geographical proximity on university-industry collaborative innovation. This paper aims to answer the question of how geographically proximate university and industry influence cognitive, social, organizational, institutional and cultural proximity within university-industry joint laboratories and finally, what is the outcome of these interplays on collaborative innovation. Design/methodology/approach: The study uses an exploratory multiple-case study approach. The results are derived from 53 in-depth, semistructured interviews with laboratory directors and representatives from both the company and the university within 8 joint laboratories of Telecom Italia (TIM). The data collection was carried out in 2014 and 2015. The analysis follows a multi-grounded theory approach and relies on a mix of deductive and inductive reasoning with the final goal of theoretical elaboration. Findings: This study finds the role of social and cultural proximity at the individual level as a result of geographical proximity as an enabler of collaborative innovation by triggering mutual learning, trust formation and frequent interactions. Cognitive proximity at the interface level could systematically influence collaborative innovation, while organizational and institutional proximity has marginal roles in facilitating collaborative innovation. The qualitative analysis offers a conceptual framework for proximity dimensions and collaborative innovation within university-industry joint laboratories. Practical implications: The framework not only advances state-of-the-art university-industry collaboration and proximity dimension but also offers guidance for managers in designing collaborative innovation settings between university and industry. Originality/value: With this study, the paper advances the understanding beyond solely the relationship between proximity and collaboration and shed light on the interplay between geographical proximity and other proximity dimensions in this context, which has received limited scholarly attention.

Tosedostat is an orally administered metalloenzyme inhibitor with antiproliferative and antiangiogenic activity against hematological and solid human cancers. Clinical activity has been demonstrated in relapsed acute myeloid leukemia (AML). Thirty-three elderly patients with AML (median age, 75 years) received 120 mg tosedostat orally once daily combined with subcutaneous low-dose cytarabine (20 mg twice per day for 10 days, up to 8 cycles), until disease progression. Induction mortality was 12%. According to an intention-to-treat analysis, the complete remission (CR) rate was 48.5%, and thus the primary end point of the study was reached (expected CR, 25%). The partial remission rate was 6.1%, with an overall response rate of 54.5%. Furthermore, 4 of 33 patients had stable disease (median: 286 days). The median progression-free survival and overall survival (OS) were 203 days and 222 days, respectively. Responding patients had a longer median OS than nonresponding patients (P = .001). A microarray analysis performed in 29 of 33 patients identified 188 genes associated with clinical response (CR vs no CR). Three of them (CD93, GORASP1, CXCL16) were validated by quantitative polymerase chain reaction, which correctly classified 83% of the patients. Specifically, CR achievement was efficiently predicted by the gene expression patterns, with an overall accuracy exceeding 90%. Finally, a negative predictive value of 100% was validated in an independent series, thus representing the first molecular predictor for clinical response to a specific combination drug treatment for AML. This trial has been registered at the European Medicines Agency and on the European Clinical Trials Database (https://www.clinicaltrialsregister.eu) as #2012-000334-19.

Puesto que la artroscopia de cadera es controversial en el tratamiento de la displasia, nuestro objetivo fue analizar sus resultados clínicos y radiológicos en una cohorte de pacientes con displasia limítrofe y compararlos con controles con choque femoroacetabular (CFA).

Background: The goals of intertrochanteric hip fracture (IHF) treatment are stable fixation, early mobilisation and function restoration. If the attempt to reduce, stabilise and fracture healing utilising a femoral cephalomedullar nail (CMN) fails, options for subsequent attempts are limited.Purpose: Evaluate the clinical and radiographic outcomes of conversion total hip arthroplasty (THA) using a modular stem following a CMN failure.Materials and methods: We retrospectively reviewed a consecutive series of patients with an IHF between 2012 and 2014 to identify CMN patients that went on to the subsequent failure and conversion to THA utilising a modular femoral stem (MFS). In all cases, MP Reconstruction Prosthesis (Waldemar Link, Hamburg, Germany) was implanted. Primary clinical outcomes were assessed using Harris Hip Score (HSS) before conversion procedure, 3 months, 6 months and recent office visit post-conversion THA thereafter. The secondary outcome was to analyse intra and postoperative complications. Serial radiographs at each follow-up interval were assessed for clinical success or to confirm adverse events.Results: 28 patients were included in the study; 17 were females. The average age was 72.7 years (SD ± 10.5); the average time from the index procedure to conversion THA was 12.6 months (SD ± 3.5). At baseline, average HHS was 42.1 (SD ± 3.6), improved to 80.7 (SD ± 5.1) at 3 months, 86.0 (SD ± 3.9) at 6-months which levelled off to 86.1 (SD ± 4.0) at final follow-up. There were 4 (14%) post-conversion complications: 2 dislocations, 1 superficial wound infection, 1 patient with symptomatic abductor deficiency. All 4 cases were conservatively treated successfully, the implants were retained, and the patients progressed without further issue.Conclusions: MFSs allow to successfully treat failed CMN and adverse variations in femoral anatomy with a device that will permit simultaneous correction of leg length, offset and version to relieve pain, restore function and create a durable prosthetic to host composite.

Realizar una comparación funcional y radiográfica de los resultados iniciales obtenidos con la utilización de los abordajes anterior directo (AAD) y posterolateral (PL) en la artroplastia total de cadera (ATC).

Laryngopharyngeal reflux (LPR) is an inflammatory condition suspected to be associated with dysphonia. In this study, we investigated multidimensional perceptual, aerodynamic, and acoustic voice changes in patients with clinically diagnosed LPR compared to healthy participants. We prospectively included 80 outpatients with Reflux Finding Score (RFS) >7 and Reflux Symptom Index (RSI) >13 from September 2013 to April 2016 and we compared clinical and voice quality assessments of these patients with 80 healthy participants. Statistically significant differences were found between groups with regard to Voice Handicap Index, perceptual voice quality (grades of dysphonia, roughness, strain, breathiness, asthenia, and instability), phonatory quotient, percentage jitter, percentage shimmer, peak-to-peak amplitude variation, standard deviation of fundamental frequency, and noise to harmonic ratio. Granulation score of RFS was found to affect the highest number of acoustic parameters. We did not identify significant correlation between vocal fold edema and objective voice quality measurements. This study supports that patients with LPR have significant deterioration of both subjective and objective voice quality compared to healthy participants.

Aims Ionizing radiation may lead to mitochondrial DNA (mtDNA) mutations and changes in mtDNA content in cells, major driving mechanisms for carcinogenesis, vascular aging and neurodegeneration. The aim of this study was to investigate the possible induction of common mitochondrial deletion (mtDNA4977) and mtDNA copy number (mtDNA-CN) changes in peripheral blood of personnel working in high-volume cardiac catheterization laboratories (Cath Labs).Methods A group of 147 Cath Lab workers (median individual effective dose = 16.8 mSv, for the 41 with lifetime dosimetric record) and 74 unexposed individuals were evaluated. The occupational radiological risk score was computed for each subject on the basis of the length of employment, individual caseload and proximity to the radiation source. mtDNA4977 deletion and mtDNA-CN were assessed by using quantitative real-time polymerase chain reaction.Results Increased levels of mtDNA4977 deletion were observed in high-exposure Cath Lab workers compared with unexposed individuals (p< 0.0001). Conversely, mtDNA-CN was significantly greater in the low-exposure workers (p= 0.003). Occupational radiological risk score was positively correlated with mtDNA4977 deletion (Spearman's r= 0.172, p= 0.03) and inversely correlated with mtDNA-CN (Spearman's r= –0.202, p= 0.01). In multiple regression model, occupational radiological risk score emerged as significant predictor of high levels of mtDNA4977 deletion (ß coefficient = 0.236, p= 0.04).Conclusion mtDNA4977 deletion is significantly high in Cath Lab personnel. Beyond the well-recognized nuclear DNA, mtDNA damage might deserve attention as a pathogenetic molecular pathway and a potential therapeutic target of ionizing radiation damage.

Hodgkin lymphoma (HL) is an uncommon malignancy that is characterized by Hodgkin or Reed–Sternberg cells. Cardiac implications of HL remain one of the least investigated subjects. There are few case reports in the literature of cardiac tamponade in HL patients. We describe a case of a 21-year-old female patient who presented with cardiac tamponade as an initial presentation of HL. Any pericardial effusion significant for tamponade requires immediate drainage and fluid analysis for thorough investigation. Prompt identification and timely intervention are crucial in effectively addressing these complex situations. Therefore, clinicians should maintain heightened awareness in such cases.

We present a patient at risk of misdiagnosis with multiple myeloma due to pseudohypercalcemia. Examinations showed monoclonal protein, 50% monoclonal plasma cells in bone marrow, and hypercalcemia but no osteolytic bone lesions. Follow-up tests revealed pseudohypercalcemia, with elevated total calcium, but normal ionized calcium: a discrepancy due to calcium binding to monoclonal paraprotein (confirmed by laboratory experiments). Accordingly, the patient was diagnosed with smouldering myeloma. After 900?days, the presence of bone lesions prompted the start of treatment for myeloma. Consequently, monoclonal paraprotein levels declined and pseudohypercalcemia dissolved. Hence, ionized calcium should be measured in monoclonal gammopathies to avoid misdiagnosis.

Down syndrome (DS) is defined by an extra copy of chromosome 21 and confers an increased susceptibility to hematological disorders. Transient abnormal myelopoiesis (TAM) and myeloid-leukemia associated with Down syndrome (ML-DS) are two conditions that need to be accurately diagnosed to provide appropriate management. Both TAM and ML-DS are characterized by proliferation of megakaryoblasts carrying a mutation in the GATA1 gene. Here, we report four cases with educational significance, highlighting typical diagnostic features that facilitate the differentiation between these two conditions, thereby assisting clinicians and medical laboratory professionals in effectively managing and monitoring these patients.

We describe the case of a 27-year-old male, previously healthy though with a social history notable for recreational cocaine use, who developed bone marrow failure due to aplastic anemia (AA) with associated serous fat atrophy (SFA). After the SFA was corrected with nutritional supplementation, the patient underwent successful allogeneic, haploidentical stem cell transplantation with a regimen designed to treat AA. To our knowledge, this is the first case of hematopoietic stem cell transplantation (HSCT) performed following correction of SFA. Herein we propose our novel hypothesis that SFA, once resolved, is not a contraindication to stem cell transplantation, which we believe adds valuable insight toward an improved understanding of nutrition’s role in HSCT. Additionally, the AA is thought to be toxin-induced and specifically levamisole-mediated after exposure to levamisole-adulterated cocaine. We highlight potential connections between levamisole, AA, and SFA and call for further efforts to understand these relationships—especially as the use of levamisole as a cocaine adulterant continues to rise across the globe.

EBV-positive primary nodal T-cell/NK cell lymphoma (TNKL) is a rare diagnosis with a poor prognosis. No relationship with follicular lymphoma (FL), classic Hodgkin lymphoma (cHL), or other non-Hodgkin lymphomas is established. We describe a case of Epstein–Barr virus (EBV)-positive cHL and EBV-positive primary nodal TNKL in the background of an antecedent FL, with all 3 subtypes identified in a single lymph node biopsy from an immunocompetent patient. Intensive frontline therapy achieved only a temporary response, with subsequent rapid progression associated with hemophagocytic lymphohistiocytosis (HLH). We discuss the relationship of the three lymphoma subtypes and the potential roles of EBV and immune dysregulation as contributing factors to this previously undescribed composite lymphoma.

Epstein–Barr virus (EBV) may cause a wide spectrum of symptomatology in humans ranging from asymptomatic upper respiratory tract infection to infectious mononucleosis and in more severe cases lymphoproliferative disorders or hemophagocytic lymphohistiocytosis. Its neoplastic potential is higher in immunocompromised individuals. We describe a case of EBV-positive mucocutaneous ulcer, a more indolent clinical entity on the spectrum of EBV-driven lymphoproliferative disorders, and are one of the first to put sulfasalazine, an immunomodulatory agent, forward as the possible culprit.

T-cell prolymphocytic leukemia (T-PLL) is a rare, mature T-cell leukemia which usually presents with aggressive behavior. We report an asymptomatic T-PLL patient diagnosed by clinical features, lymphocyte morphology, and flow cytometry. Incidentally, she was found to have lymphocytosis and lymphadenopathy. Flow cytometry from blood revealed an abnormally increased CD4+ T-cell population. T-cell receptor clonality assessment by next-generation sequencing revealed a dominant clone in the ß-chain constant region. No pathogenic mutations in 25 lymphoma-related genes were found. Due to her asymptomatic T-PLL disease, we observed her clinical situation and blood count every three months for at least one year.

Castleman disease (CD) is a rare lymphoproliferative disorder characterized by benign lymph node enlargement. We present the case of a 43-year-old male with a complex medical history, including Crohn’s disease treated with Adalimumab and later complicated with tuberculosis (TB) infection. Subsequently, in May 2021, he was diagnosed with human immunodeficiency virus (HIV) and started on antiretroviral therapy (efavirez, emricitabine, and tenofovir). Despite stropping adalimumab, anti-Tb, and antiviral therapy, he experienced persistent fever, neurological symptoms, and lymphadenopathy. Toxoplasmosis, Cytomegalovirus (CMV), and Human Herpesvirus-8 (HHV-8) were diagnosed and then treated. Furthermore, the patient displayed intermittent febrile episodes, pancytopenia, altered coagulation parameters, hypoalbuminemia, edema, and generalized abdominal pain, as well as radiological evidence of hepatosplenomegaly and pulmonary infiltrates. Left axillary lymph node biopsy (ALNB) was done and confirmed multicentric castleman disease (MCD). Moreover, the bone marrow aspirate showed plasmocytes. His treatment included chemotherapy with doxorubicin and rituximab while continuing his anti-Tb and antiretroviral therapy. This complex case highlights the diagnostic challenges of managing CD in the presence of multiple coexisting conditions, emphasizing the need for comprehensive evaluation in complex clinical presentations.

We describe a 10-month-old female with Diamond-Blackfan anemia (DBA) who presented with macrocytic anemia and reticulocytopenia. Whole exome sequencing revealed a de novointronic variant in RPL27(NM_000988.3:c.-2-1G?>?A p.?) previously reported in one individual with DBA. The existing literature suggests the RPL27gene encodes for a ribosomal protein involved in pre-rRNA processing and erythropoiesis. Further research is needed to assess the functional significance of this variant and its implications for genetic testing and therapeutic strategies. This case expands the clinical spectrum of RPL27-associated DBA and highlights the importance of reclassifying this gene to likely pathogenic.

Mantle cell lymphoma (MCL) is a mature B-cell lymphoma associated with cyclin D family rearrangements and typically expresses CD5 and cyclin D1. Epstein–Barr virus- (EBV-) positive MCL is rare, and the role of EBV infection and its transformation in MCL remains unclear. We present a case of CD5-negative classic MCL that progressed to an EBV?+?pleomorphic MCL six years after the initial diagnosis. Molecular studies confirmed the same clonal origin. To the best of our knowledge, the EBV-positive transformation of CD5-negative MCL into a pleomorphic variant has rarely been reported, and its recognition is important for the diagnosis and the management of patients with MCL.

The unique histiocytic entity of indeterminate dendritic cell tumor (IDCT) is known to cause diagnostic conundrum and treatment dilemmas with no published consensus on either. We report a rare case of cutaneous IDCT with ETV3::NOAC2 rearrangement providing further evidence to its association with this condition. With its ease of administration and minimal side effects, PUVA therapy can be successfully used to treat cutaneous forms of IDCT.

We present a rare case of pseudohyponatremia in a 20-year-old male patient with adult T-cell acute lymphoblastic leukemia (ATLL). The patient was admitted for a mediastinal mass with superior vena cava syndrome and was receiving pegaspargase therapy. The pseudohyponatremia was found to be secondary to hypertriglyceridemia associated with the pegaspargase treatment. In this case report, we discuss the important considerations in the management of adult ATLL patients receiving pegaspargase therapy, and the specific approaches taken to care for the patient described in this case.

The coexistence of chronic myeloid leukemia (CML) and chronic lymphocytic leukemia (CLL) in the same patient is exceedingly rare, with only a few cases reported in the literature. Here, we report a patient with CML who, having achieved a major molecular response with imatinib, subsequently developed CLL, which necessitated the concomitant administration of ibrutinib.

Hemolytic disease of the fetus and newborn (HDFN) is the development of anemia, hyperbilirubinemia, and finally hydrops fetalis in the fetus when antibodies to antigens on the surface of erythrocytes are transferred from the placenta to the fetus. The most common cause is D-HDFN. K (KEL1) from the Kell blood group system is the most potent immunogenic antigen after D among all blood group antigens. K-HDFN occurs in 0.1–0.3% of pregnant women. It accounts for 10% of cases of antibody-mediated severe fetal anemia. We present a successful management of Kell alloimmunization in a pregnant woman who had 3 times pregnancy loss with hydrops fetalis due to K-HDFN and who was proven to have K-HDFN in the postnatal period in her last pregnancy.

Most B cell lymphomas are positive for one or more B cell markers including CD19, CD20, CD79a, or PAX5. However, rare cases of mature B cell lymphoma not expressing any B cell markers have been characterized and recognized as distinct diagnostic entities by current classification guidelines, including plasmablastic lymphoma, primary effusion lymphoma, and ALK-positive large B cell lymphoma. We present a case of pan-B cell marker negative, EBV positive diffuse large B cell lymphoma that is positive for OCT2, BOB1, and clonal immunoglobulin gene rearrangement that does not meet diagnostic criteria for any B cell lymphoma by current 4thand 5thEd beta version WHO Hematolymphoid Tumors classification. In challenging cases like the one presented, utilizing OCT2 and BOB1 immunohistochemical stains can assist in determining B cell lineage. The WHO tumor classification system should consider adding OCT2 and BOB1 as alternative B cell lineage markers into their corresponding categories.