The Effect of Type of Femoral Component Fixation on Mortality and Morbidity after Hip Hemiarthroplasty: A Systematic Review and Meta-Analysis

HSS Journal ® - Hip hemiarthroplasty is a well-established treatment of displaced femoral neck fracture, although debate exists over whether cemented or uncemented fixation is superior....     

Letter to the Editor concerning “Age- and sex-related changes in facet orientation and tropism in lower lumbar spine: an MRI study of 600 patients” by Degulmadi D,et al. (Eur Spine J [2019] 28:961–966, https://doi.org/10.1007/s00586-019-05953-y)

European Spine Journal -     

Risk of second primary malignancy in patients with sinonasal tumors: a population‐based cohort study

Background

The 5‐year overall survival rate for patients with sinonasal cancers has remained around 50% for the last 3 decades. Prior studies on head and neck cancers have suggested that 1 reason for poor survival is the frequent development of second primary malignancies (SPMs). The purpose of this study is to assess overall and site‐specific risks of SPM following treatment of sinonasal malignancy.

Methods

A retrospective, population‐based cohort study was performed on 2614 patients in the Surveillance, Epidemiology, and End Results (SEER) database who were diagnosed with primary sinonasal malignancy between 1973 and 2014. Standardized incidence ratios (SIRs) and absolute excess risks (AERs) were calculated to assess risk of SPM relative to incidence in the general population.

Results

A total of 422 (16.1%) patients with primary sinonasal malignancies developed a total of 480 SPMs. This cohort had a significantly higher frequency of SPMs than expected in the general population (SIR 1.32; 95% confidence interval [CI], 1.20 to 1.44; AER 53.41). Site‐specific analyses of SIRs suggested highest risk of malignancy in the sinonasal tract (SIR 75.64; 95% CI, 53.53 to 103.83; AER 17.22), followed by bone, eye and orbit, oral cavity and pharynx, and lung and mediastinum.

Conclusion

Patients with history of sinonasal cancer are at significantly increased risk of developing an SPM. Careful monitoring for development of additional tumors may be warranted.

     

Human autoantibodies specific for the α1A calcium channel subunit reduce both P-type and Q-type calcium currents in cerebellar neurons

The pharmacological properties of voltage-dependent calcium channel (VDCC) subtypes appear mainly to be determined by the α₁ pore-forming subunit but, whether P-and Q-type VDCCs are encoded by the same α₁ gene presently is unresolved. To investigate this, we used IgG antibodies to presynaptic VDCCs at motor nerve terminals that underlie muscle weakness in the autoimmune Lambert–Eaton myasthenic syndrome (LEMS). We first studied their action on changes in intracellular free Ca²⁺ concentration [Ca²⁺]_i in human embryonic kidney (HEK293) cell lines expressing different combinations of human recombinant VDCC subunits. Incubation for 18 h with LEMS IgG (2 mg/ml) caused a significant dose-dependent reduction in the K⁺-stimulated [Ca²⁺]_i increase in the α_1A cell line but not in the α_1B, α_1C, α_1D, and α_1E cell lines, establishing the α_1A subunit as the target for these autoantibodies. Exploiting this specificity, we incubated cultured rat cerebellar neurones with LEMS IgG and observed a reduction in P-type current in Purkinje cells and both P- and Q-type currents in granule cells. These data are consistent with the hypothesis that the α_1A gene encodes for the pore-forming subunit of both P-type and Q-type VDCCs.     

Oral manifestations of 17 patients affected with mucopolysaccharidosis type VI

Objective

To assess oral manifestations of 17 patients with mucopolysaccharidosis type VI (MPS VI) or Maroteaux-Lamy syndrome.

Methods

We performed comprehensive oral examinations in 17 patients with MPS VI. Panoramic radiographs was performed only in 14 patients. All patients were of Thai, Turkish, and Indian origins. Ten of 17 patients had enzyme replacement therapy (ERT) (Naglazyme). Most Turkish patients (10/11) were on ERT. The Thai and Indian patients have never had ERT.

Results

Oral and radiographic examinations showed that hypoplastic mandibular condyles (93.3 %), malposition of unerupted teeth (92.9 %), large dental follicles (92.3 %), anterior open bite (86.7 %), maxillary constriction (56.3 %), and taurodontism (53.8 %) were common among patients with MPS VI. Newly recognized oral findings found in our study included taurodontism, long tooth roots, abnormal frenum, missing teeth, supernumerary teeth, and microdontia. Two patients who started ERT prior to 3 years old did not develope anterior open bite and one of them had mildly affected mandibular condyles.

Conclusion

Our study provides the most comprehensive study of oral manifestations in patients with MPS VI. Receiving ERT at very young ages may lessen craniofacial malformations including hypoplasic mandibular condyles and anterior open bite. Oral manifestations can be used as diagnostic features for MPS VI prior to assessing leukocyte ARSB activity or urinary excretion of dermatan sulfate.     

Extended experience with a non-cytotoxic DNMT1-targeting regimen of decitabine to treat myeloid malignancies

The nucleoside analogue decitabine can deplete the epigenetic regulator DNA methyltransferase 1 (DNMT1), an effect that occurs, and is saturated at, low concentrations/doses. A reason to pursue this molecular-targeted effect instead of the DNA damage/cytotoxicity produced with high concentrations/doses, is that non-cytotoxic DNMT1-depletion can cytoreduce even p53-null myeloid malignancies while sparing normal haematopoiesis. We thus identified minimum doses of decitabine (0·1–0·2 mg/kg) that deplete DNMT1 without off-target anti-metabolite effects/cytotoxicity, and then administered these well-tolerated doses frequently 1–2X/week to increase S-phase dependent DNMT1-depletion, and used a Myeloid Malignancy Registry to evaluate long-term outcomes in 69 patients treated this way. Consistent with the scientific rationale, treatment was well-tolerated and durable responses were produced (~40%) in genetically heterogeneous disease and the very elderly.     

Large granular lymphocytic leukaemia after solid organ and haematopoietic stem cell transplantation

T-cell large granular lymphocytic leukaemia (T-LGLL) is a chronic clonal lymphoproliferative disorder of cytotoxic T lymphocytes which commonly occurs in older patients and is often associated with autoimmune diseases. Among 246 patients with T-LGLL seen at our institution over the last 10 years, we encountered 15 cases following solid organ or haematopoietic stem cell transplantation. Here, we studied the clinical characterization of these cases and compared them to de novo T-LGLL. This experience represented a clear picture of the intricate nature of the disease manifestation and the complexities of several immune mechanisms triggering the clonal expansion.