Echocardiographic and clinical findings in patients with Fabry disease during long-term enzyme replacement therapy: a nationwide Danish cohort study

Objectives: In patients with Fabry disease (FD), left ventricular hypertrophy and arrhythmias are frequently observed and cardiac involvement is the leading cause of death. Long-term efficacy of enzyme replacement therapy (ERT) on cardiac involvement is unclear. We assessed and compared long-term progression of cardiac involvement according to ERT and non-ERT. Methods: We retrospectively assessed and compared long-term progression of cardiac involvement in adult patients with FD in the nationwide Danish cohort. We followed clinical signs, symptoms and findings by echocardiography, electrocardiography and Holter-monitoring. Results: We included 66 patients; 47 patients (27 women) received ERT (ERT group) and 19 patients (15 women) did not (non-ERT group). The groups were followed for a median of 8 [0–12] years and 6 [0–13] years, respectively. Comparison between ERT and non-ERT receiving patients by left ventricular mass (echocardiographic assessment) and Sokolow-Lyon voltage- and Cornell product criteria (electrocardiographic assessment) revealed no significant differences. In the ERT group, we observed no change in left ventricular mass but a decrease in Sokolow-Lyon voltage- and Cornell product criteria from baseline to follow-up; 30?mm [15–53] vs. 25?mm [3–44], p?p?Discussion: We raise concerns regarding the efficacy and benefit of ERT on cardiac involvement in Fabry disease and stress the need for further research.     

Glutamate infusion associated with reduced rises of p-Copeptin after coronary surgery: Substudy of GLUTAMICS II

Background

Glutamate plays a key role for post-ischaemic recovery of myocardial metabolism. According to post hoc analyses of the two GLUTAMICS trials, patients without diabetes benefit from glutamate with less myocardial dysfunction after coronary artery bypass surgery (CABG). Copeptin reflects activation of the Arginine Vasopressin system and is a reliable marker of heart failure but available studies in cardiac surgery are limited. We investigated whether glutamate infusion is associated with reduced postoperative rises of plasma Copeptin (p-Copeptin) after CABG.

Methods

A prespecified randomised double-blind substudy of GLUTAMICS II. Patients had left ventricular ejection fraction ≤0.30 or EuroSCORE II ≥3.0 and underwent CABG ± valve procedure. Intravenous infusion of 0.125 M L-glutamic acid or saline at 1.65 mL/kg/h was commenced 10–20 min before the release of the aortic cross-clamp and then continued for another 150 min P-Copeptin was measured preoperatively and postoperatively on day one (POD1) and day three. The primary endpoint was an increase in p-Copeptin from the preoperative level to POD1. Postoperative stroke ≤24 h and mortality ≤30 days were safety outcomes.

Results

We included 181 patients of whom 48% had diabetes. The incidence of postoperative mortality ≤30 days (0% vs. 2.1%; p = .50) and stroke ≤24 h (0% vs. 3.2%; p = .25) did not differ between the glutamate group and controls. P-Copeptin increased postoperatively with the highest values recorded on POD1 without significant inter-group differences. Among patients without diabetes, p-Copeptin did not differ preoperatively but postoperative rise from preoperative level to POD1 was significantly reduced in the glutamate group (73 ± 66 vs. 115 ± 102 pmol/L; p = .02). P-Copeptin was significantly lower in the Glutamate group on POD1 (p = .02) and POD 3 (p = .02).

Conclusions

Glutamate did not reduce rises of p-Copeptin significantly after moderate to high-risk CABG. However, glutamate was associated with reduced rises of p-Copeptin among patients without diabetes. These results agree with previous observations suggesting that glutamate mitigates myocardial dysfunction after CABG in patients without diabetes. Given the exploratory nature of these findings, they need to be confirmed in future studies.     

Open Arthrolysis for Chronic Elbow Dislocation with Extremely Severe Stiffness in Children

Objective

Studies on extremely severe elbow stiffness after chronic dislocation in children are scarce. This study aims to investigate the choice of surgical treatment modalities and to analyze their treatment efficacy in children with chronic elbow dislocation with extremely severe periarticular stiffness.

Methods

Data of 21 children with chronic elbow dislocation with extremely severe periarticular stiffness diagnosed and treated in our department between February 2015 and February 2021 were retrospectively analyzed. Twenty boys and one girl were included in the study, their mean age was 11 ± 2.5 years, and they had concomitant distal humerus fractures. For the treatment protocol, all children with extremely severe elbow stiffness were treated with open arthrolysis, and elbow joint stability was intraoperatively assessed. All children performed passive functional exercises the day after surgery. The elbow flexion and extension angles, range of motion (ROM), and Mayo score were evaluated preoperatively and at the final follow-up.

Results

Of the 21 children, only one had recurrent severe stiffness of the elbow joint after surgery; nevertheless, the function was still improved compared with that before surgery. Preoperatively, the mean elbow extension and flexion angles were 72.2° ± 12.7° and 93.6° ± 11.1°, respectively, and the range of motion (ROM) of the elbow joint was 17.8° ± 8.3°. At the final follow-up, the mean elbow extension and flexion angles were 22.7° ± 18.6° and 118.8° ± 15.4°, respectively, and the elbow joint ROM was 96.1° ± 17.4°. The differences in the preoperative and postoperative ROMs, flexion angles, and extension angles of the elbow joint were significant (p < 0.01). The MEPS at the final follow-up was 78.57 ± 14.24, which was significantly higher than preoperative (29.76 ± 10.89), and the excellent rate was 81%.

Conclusion

Open arthrolysis and open reduction and internal fixation of the elbow joint are effective in treating chronic elbow dislocation with extremely severe stiffness in children.     

The Fusion Rate of Cortical Bone Trajectory Screw Fixation and Pedicle Screw Fixations in L4-5 Interbody Fusion: A Retrospective Cohort Study

Objective

Although cortical bone trajectory (CBT) screw fixation has been used for several years, the number of studies on its fusion effects is limited. Furthermore, several studies report conflicting outcomes. We aimed to compare the fusion rates and clinical efficacy of CBT screw fixation and pedicle screw (PS) fixation for L4-L5 interbody fusion.

Methods

This study was a retrospective cohort control study. Patients with lumbar degenerative disease who underwent L4-L5 oblique lumbar interbody fusion (OLIF) or posterior decompression using CBT screws between February 2016 and February 2019 were included. Patients in whom PS was used were matched for age, sex, height, weight, and BMI. Record the operation time, blood loss. All enrolled patients underwent lumbar CT imaging at one-year follow-up to evaluate the fusion rate. At the two-year follow-up the visual analogue scale (VAS), Oswestry disability index (ODI), and Japanese Orthopaedic Association scores (JOA) were used to identify symptom improvement. Independent t-test was used for the comparison, and score data were analyzed using the χ² and exact probability tests.

Results

A total of 144 patients with were included. All patients were followed-up postoperatively for 25–36 months (average 32.42 ± 10.55 months). Twenty-eight patients underwent OLIF and CBT screw fixation, 36 underwent OLIF and PS fixation, 32 underwent posterior decompression and CBT screw fixation, and 48 underwent posterior decompression and PS fixation. The fusion rates following CBT screw and PS fixations in OLIF were 92.86% (26/28) and 91.67% (33/36), respectively (P = 1). The fusion rates following CBT screw and PS fixations in posterior decompression were 93.75% (30/32) and 93.75% (45/48), respectively (P > 0.05). Regardless of OLIF or posterior decompression, there were no significant differences in the VAS, ODI, and JOA scores between patients treated with CBT and PS (P > 0.05).

Conclusion

CBT screw fixation can achieve a satisfactory interbody fusion rate with a clinical efficacy similar to that of PS in patients with lumbar degenerative disease, regardless of whether OLIF or posterior decompression was performed.     

Detection of Common Anatomical Landmarks and Vertical Trajectories for Freehand Pedicle Screw Placement

Objective

It is clinically important for pedicle screws to be placed quickly and accurately. Misplacement of pedicle screws results in various complications. However, the incidence of complications varies greatly due to the different professional titles of physicians and surgical experience. Therefore, physicians must minimize pedicle screw dislocation. This study aims to compare the three nail placement methods in this study, and explore which method is the best for determining the anatomical landmarks and vertical trajectories.

Methods

This study involved 70 patients with moderate idiopathic scoliosis who had undergone deformity correction surgery between 2018 and 2021. Two spine surgeons used three techniques (preoperative computed tomography scan [CTS], visual inspection-X-freehand [XFH], and intraoperative detection [ID] of anatomical landmarks) to locate pedicle screws. The techniques used include visual inspection for 287 screws in 21 patients, preoperative planning for 346 screws in 26 patients, and intraoperative probing for 309 screws in 23 patients. Observers assessed screw conditions based on intraoperative CT scans (Grade A, B, C, D).

Results

There were no significant differences between the three groups in terms of age, sex, and degree of deformity. We found that 68.64% of screws in the XFH group, 67.63% in the CTS group, and 77.99% in the ID group were placed within the pedicle margins (grade A). On the other hand, 6.27% of screws in the XFH group, 4.33% in the CTS group, and 6.15% in the ID group were considered misplaced (grades C and D). The results show that the total amount of upper thoracic pedicle screws was fewer, meanwhile their placement accuracy was lower. The three methods used in this study had similar accuracy in intermediate physicians (P > 0.05). Compared with intermediate physicians, the placement accuracy of three techniques in senior physicians was higher. The intraoperative detection group was better than the other two groups in the good rate and accuracy of nail placement (P < 0.05).

Conclusion

Intraoperative common anatomical landmarks and vertical trajectories were beneficial to patients with moderate idiopathic scoliosis undergoing surgery. It is an optimal method for clinical application.     

Bone-to-Bone Ligament Preserving Laminoplasty with Ultrasonic Osteotome Assistance for Intraspinal Tumors: A Technical Note and Clinical Follow-Up

Objective

Laminectomy has been widely used for intraspinal tumor resection. However, the tilted spinous process and narrow lateral laminae of the thoracic spine along with the hypertrophic ligamentum flavum of the lumbar spine pose certain problems for the laminae removal of the traditional laminectomy. We improved the laminectomy method with ultrasonic osteotome to treat thoracolumbar tumors and assessed its safety and superiority.

Methods

A retrospective analysis was performed in 86 patients with thoracolumbar (T4–L5) spinal tumors treated by resection, including 44 with the lamina removed using the traditional method and 42 with the lamina removed using the bone-to-bone ligament preserving (BLP) laminoplasty, which preserves the posterior ligament complex. Age, sex, and tumor size, location, and depth were compared between the two groups. The length of incision and bone window, time to remove the vertebral lamina, and epidural effusion volume were recorded at 2 weeks after surgery in the two groups. Postoperative reexamination by magnetic resonance imaging (MRI) at 2 weeks and 3 months after surgery was compared with preoperative MRI to assess the change in vertebral lamina displacement.

Results

There were no statistical differences in age, sex, and tumor size, depth, or location between the two groups. The BLP laminectomy did not increase the risk of dural, spinal cord, or nerve injuries. The difference between the incision and tumor length, as well as the difference between the bone window and tumor length in the BLP laminectomy group, were smaller than those in the traditional laminectomy group, and the BLP laminectomy took less time compared to that of the traditional laminectomy (p < 0.05). There was no significant difference in the volume of epidural effusion between the two groups at 2 weeks postoperatively, or in the displacement of the returned vertebral plate observed in sagittal and axial positions. The same was true for the displacement at 3 months postoperatively in the axial position. However, the sagittal displacement in the BLP laminectomy group was smaller than that in the traditional laminectomy group (p < 0.05).

Conclusions

The BLP laminectomy is safe for the resection of thoracolumbar spinal canal tumors. It is less traumatic and faster, with less displacement of the returned lamina, resulting in a stable repair of the spine.     

A Comparison of Outcomes between the Wiltse Approach with Pedicle Screw Fixation and the Percutaneous Pedicle Screw Fixation for Multi-Segmental Thoracolumbar Fractures

Objective

Multi-segmental thoracolumbar fracture (MSF) generally refers to fractures occurring in two or more segments of the thoracolumbar spine. With the development of minimally invasive concept, there is little research on its application in the field of MSF. The purpose of this study is to compare two minimally invasive surgical techniques and determine which one is more suitable for treating patients with neurologically intact MSF.

Methods

We retrospectively analyzed the clinical data of 49 MSF patients with intact nerves who were admitted from January 2017 to February 2019. Among them, 25 cases underwent percutaneous pedicle screw fixation (PPSF), and 24 cases underwent Wiltse approach pedicle screw fixation (WAPSF). The operation time, number of fixed segments, blood loss, length of incision, postoperative ambulation time, accuracy of pedicle screw placement, facet joint violation (FJV), number of C-arm exposures, as well as pre- and postoperative visual analogue scale (VAS), Oswestry disability index (ODI), local Cobb's angle (LCA), and percentage of anterior vertebral body height (PAVBH) were recorded for both groups. Paired sample t-test was used for intra-group comparison before and after surgery while independent sample t-test was used for inter-group comparison.

Results

The differences in the number of fixed segments, intraoperative bleeding, postoperative bed time, accuracy rate of pedicle screw placement, VAS, and ODI between the two groups were not statistically significant (p > 0.05). However, the operative time and total surgical incision length were significantly shorter in the WAPSF group than in the PPSF group (p < 0.05), and the FJV was significantly higher in the PPSF group than in the WAPSF group (p < 0.05). Also, the PPSF group received more intraoperative fluoroscopy (p < 0.05). The result of LCA and PAVBH in the WAPSF group were significantly better than in the PPSF group (p < 0.05).

Conclusions

Both PPSF and WAPSF were found to be safe and effective in the treatment of MSF without neurological deficits through our study. However, considering radiation exposure, FJV, vertebral height restoration, correction of kyphosis, and learning curve, WAPSF may be a better choice for neurologically intact MSF.     

The associations between diode laser (810 nm) therapy and chronic wound healing and pain relief: Light into the chronic wound patient's life

Chronic wounds have become one of the major issues in medicine today, the treatments for which include dressing changes, negative pressure wound therapy, hyperbaric oxygen, light irradiation, surgery and so forth. Nevertheless, the application of diode lasers in chronic wounds has rarely been reported. This retrospective cohort study aimed to evaluate the therapeutic effect of diode laser (810 nm) irradiation on chronic wounds. Eighty-nine patients were enrolled in the study. The control group (41 patients) received traditional dressing change therapy, while the diode laser treatment group (48 patients) were patients received additional treatment with diode laser (810 nm) irradiation for 10 min at each dressing change. Wound healing time was compared between two groups, while the pain relief index was creatively introduced to evaluate the effect of relieving wound pain, which was calculated by the difference in pain scores between the first and last dressing changes divided by the number of treatment days. The wound healing time of the diode laser treatment group was 22.71 ± 8.99 days, which was significantly shorter than that of the control group (37.44 ± 23.42 days). The pain relief index of the diode laser treatment group was 0.081 ± 0.055, which was significantly increased compared with that of the control group (0.057 ± 0.033). Our findings suggest that diode laser irradiation has the potential to promote healing in chronic wounds and relieve wound pain.     

Incidence of and Risk Factors for Screw Loosening after Iliosacral Screw Fixation for Posterior Pelvic Ring Injury

Purposes

Screw loosening is a common complication of iliosacral screw fixation, with subsequent loss of stability and fracture re-displacement. This study aimed to investigate the incidence of and risk factors for screw loosening after iliosacral screw fixation for posterior pelvic ring injury.

Methods

A total of 135 patients with posterior pelvic ring injuries who were treated with iliosacral screw fixation in our department between July 2015 and April 2021 were selected for this retrospective analysis. The possible risk factors for screw loosening were investigated using univariate and multivariate logistic regression analyses of patient demographics and trauma-related and iatrogenic variables, including age, sex, body mass index, Osteoporosis Self-Assessment Tool for Asians (OSTA) index, mechanism of injury, Young–Burgess classification, site of injury, type of injury, type of screw, mode of fixation, numbers of guidewire adjustments, accuracy of screw position, and quality of fracture reduction.

Results

The incidence of screw loosening was 15.6% (n = 21). The mean duration for screw loosening was 3.2 ± 1.5 months after operation. Univariate analysis results showed that the Young–Burgess classification, type of injury, site of injury, type of screw, mode of fixation, and OSTA index might be related to screw loosening (p < 0.05). According to the multivariate logistic regression, vertical shear injuries (Odds ratios [OR] 9.80, 95% Confidence intervals [CI] [1.96–73.28], p = 0.008), type of injury (OR 0.25, 95% CI [0.13–0.79], p = 0.027), common screws (OR 6.94, 95% CI [1.53–31.40], p = 0.012), screws insertion only at the level of the first sacral segment (S1) (OR 8.79, 95% CI [1.18–65.46], p = 0.034), injury site located in the medial sacral foramina (OR 6.28, 95% CI [1.16–34.06], p = 0.033), and lower OSTA index [OR 0.41, 95% CI [0.24–0.71], p = 0.001] were significantly related to screw loosening.

Conclusions

Vertical shear injuries, sacral fractures, injury site located in the medial sacral foramina, and lower OSTA index are significantly associated with the postoperative occurrence of screw loosening. Transiliac–transsacral screw fixation and screws insertion both at the level of the S1 and second sacral segment can prevent screw loosening.