scholarly journals Achondroplasia with seronegative spondyloarthropathy resulting in recurrent spinal stenosis : A case report

2021 ◽  
Vol 12 ◽  
pp. 354
Author(s):  
Rajendra Sakhrekar ◽  
Shailesh Hadgaonkar ◽  
Manisha Hadgaonkar ◽  
Parag Sancheti ◽  
Ashok Shyam
Keyword(s):  
Short Stature ◽  
Spinal Stenosis ◽  
Growth Factor Receptor ◽  
Gene Encoding ◽  
Seronegative Spondyloarthropathy ◽  
Canal Stenosis ◽  
Upper Cervical ◽  
One Year ◽  
Neurologically Intact ◽  
Dominant Condition

Background: Achondroplasia is an autosomal dominant condition caused by the G380 mutation of the gene encoding fibroblast growth factor receptor 3 on chromosome 4P. The classical findings include rhizomelic extremities, short stature, and spinal stenosis involving the upper cervical and distal lumbar spine. Rarely, achondroplasia coexisting with seronegative spondyloarthropathy can result in recurrent canal stenosis. Here, we report a 36-year-old male with symptomatic recurrent L3-L4 spinal stenosis 9 years following an original L2-S1 lumbar decompression for stenosis. Case Description: A 36-year-old male with achondroplasia (height of 113 cm and weight 43 kg [BMI-33.7]) presented with low back and right lower extremity sciatica (ODI 39). He had achondroplasia with a short stature. Nine years ago, he had an L2-S1 laminectomy for decompression of stenosis. When the new MRI revealed recurrent severe L3-4 stenosis, he underwent a repeated L3-L4 decompression with fusion. One year later, the patient was neurologically intact with radiographic confirmation of adequate L3-L4 arthrodesis. Conclusion: A 36-year-old male with achondroplasia and a history 9 years ago of an L2-S1 laminectomy for stenosis, presented with symptoms and signs of recurrent L3-L4 stenosis that responded to repeated decompression and fusion.

scholarly journals Advantages and Disadvantages of Different Treatment Methods in Achondroplasia: A Review

2021 ◽  
Vol 22 (11) ◽  
pp. 5573
Author(s):  
Wiktoria Wrobel ◽  
Emilia Pach ◽  
Iwona Ben-Skowronek
Keyword(s):  
Clinical Trials ◽  
Short Stature ◽  
Animal Studies ◽  
Recombinant Human Growth Hormone ◽  
Growth Factor Receptor ◽  
Human Growth ◽  
Foramen Magnum ◽  
Advantages And Disadvantages ◽  
Canal Stenosis

Achondroplasia (ACH) is a disease caused by a missense mutation in the FGFR3 (fibroblast growth factor receptor 3) gene, which is the most common cause of short stature in humans. The treatment of ACH is necessary and urgent because untreated achondroplasia has many complications, both orthopedic and neurological, which ultimately lead to disability. This review presents the current and potential pharmacological treatments for achondroplasia, highlighting the advantages and disadvantages of all the drugs that have been demonstrated in human and animal studies in different stages of clinical trials. The article includes the potential impacts of drugs on achondroplasia symptoms other than short stature, including their effects on spinal canal stenosis, the narrowing of the foramen magnum and the proportionality of body structure. Addressing these effects could significantly improve the quality of life of patients, possibly reducing the frequency and necessity of hospitalization and painful surgical procedures, which are currently the only therapeutic options used. The criteria for a good drug for achondroplasia are best met by recombinant human growth hormone at present and will potentially be met by vosoritide in the future, while the rest of the drugs are in the early stages of clinical trials.

Minimally Invasive versus Open Thoracolumbar Surgery for Lumbar Spinal Stenosis in Patients with Diabetes: A CSORN Study

2021 ◽  
pp. 13
Author(s):  
Kalpesh Hathi
Keyword(s):  
Back Pain ◽  
Minimally Invasive ◽  
Spinal Stenosis ◽  
Lumbar Spinal Stenosis ◽  
Research Network ◽  
Leg Pain ◽  
Number Of Patients ◽  
Patients With Diabetes ◽  
One Year ◽  
Lumbar Spinal

Introduction: This study was aimed at comparing outcomes of minimally invasive (MIS) versus OPEN surgery for lumbar spinal stenosis (LSS) in patients with diabetes. Methodology: This retrospective cohort study included patients with diabetes who underwent spinal decompression alone or with fusion for LSS within the Canadian Spine Outcomes and Research Network (CSORN) database. Outcomes of MIS and OPEN approaches were compared for two cohorts: (i) patients with diabetes who underwent decompression alone (N = 116; MIS, n = 58, OPEN, n = 58) and (ii) patients with diabetes who underwent decompression with fusion (N = 108; MIS, n = 54, OPEN, n = 54). Mixed measures analyses of covariance compared modified Oswestry Disability Index (mODI) and back and leg pain at one-year post operation. The number of patients meeting minimum clinically important difference (MCID) or minimum pain/disability at one year were compared. Result: MIS approaches had less blood loss (decompression alone difference 99.66 mL, p = 0.002; with fusion difference 244.23, p < 0.001) and shorter LOS (decompression alone difference 1.15 days, p = 0.008; with fusion difference 1.23 days, p = 0.026). MIS compared to OPEN decompression with fusion had less patients experience an adverse event (difference, 13 patients, p = 0.007). The MIS decompression with fusion group had lower one-year mODI (difference, 14.25, p < 0.001) and back pain (difference, 1.64, p = 0.002) compared to OPEN. More patients in the MIS decompression with fusion group exceeded MCID at one year for mODI (MIS 75.9% vs OPEN 53.7%, p = 0.028) and back pain (MIS 85.2% vs OPEN 70.4%, p = 0.017). Conclusion: MIS approaches were associated with more favorable outcomes for patients with diabetes undergoing decompression with fusion for LSS.

scholarly journals The Influence of Pain Sensitivity on the Symptom Severity in Patients with Lumbar Spinal Stenosis

2013 ◽  
Vol 2;16 (2;3) ◽  
pp. 135-144
Author(s):  
Jin S. JYeom
Keyword(s):  
Back Pain ◽  
Spinal Stenosis ◽  
Disc Degeneration ◽  
Lumbar Spinal Stenosis ◽  
Pain Sensitivity ◽  
Leg Pain ◽  
Canal Stenosis ◽  
Sf 36 ◽  
The Individual ◽  
Lumbar Spinal

Background: The symptom severity of back pain/leg pain is not correlated with the severity of degenerative changes and canal stenosis in lumbar stenosis. Considering the individual pain sensitivity might play an important role in pain perception, this discordance between the radiologic findings and clinical symptoms in degenerative lumbar stenosis might originate from the individual difference of pain sensitivity for back pain and/or leg pain. Objective: To determine the relationship among the clinical symptoms, radiologic findings, and the individual pain sensitivity in the patients with degenerative lumbar spinal stenosis. Study Design: Retrospective analysis of prospectively collected data. Setting: A spine center in the department of orthopedic surgery. Methods: In 94 patients who had chronic back pain and/or leg pain caused by degenerative lumbar spinal stenosis, a medical history, a physical examination, and completion of a series of questionnaires, including pain sensitivity questionnaire (PSQ) [total PSQ and PSQ-minor], Oswestry Disability Index (ODI), Visual Analog Pain Scale (VAS) for back pain, and Short Form36 (SF-36) were recorded on the first visit. Radiologic analysis was performed using the MRI findings. The grading of canal stenosis was based on the method by Schizas, and the degree of disc degeneration was graded from T2-weighted images with the Pfirrmann classification. The correlations among variables were statistically analyzed. Results: Total PSQ and PSQ-minor were not dependent on the grade of canal stenosis after gender adjustment. VAS for leg pain and back pain was highly associated with the total PSQ and the PSQ-minor. Total PSQ and PSQ-minor were also significantly associated with ODI. Among SF36 scales, the PSQ minor had significant correlations with SF-36 such as bodily pain (BP), Roleemotional (RE), and Mental Component Summary (MCS) after control of confounding variables such as body mass index (BMI), age, and the grade of canal stenosis/disc degeneration. Total PSQ was significantly associated with the SF-36 RP, BP, and RE. Furthermore, after adjustment for gender and pain sensitivity, there was no significant association between the grade of canal stenosis and VAS for back pain/leg pain and ODI, and no correlation was found between the grade of disc degeneration and VAS for back pain/leg pain and ODI, either. Limitations: The multiple lesions of canal stenosis and/or disc degeneration and the grade of facet degeneration were not considered as a variable. Conclusion: The current study suggests that the pain sensitivity could be a determining factor for symptom severity in the degenerative spinal disease. Key words: Pain sensitivity, pain sensitivity questionnaire, lumbar spinal stenosis, visual analog pain scale, Oswestry disability index, Short Form-36

scholarly journals DVL3 is over-expressed in the tumors of breast cancer patients treated with trastuzumab.

2020 ◽  
Author(s):  
Shahan Mamoor
Keyword(s):  
Breast Cancer ◽  
Cancer Patients ◽  
Growth Factor Receptor ◽  
Metastatic Breast ◽  
The United States ◽  
Breast Cancer Patients ◽  
Gene Encoding ◽  
Significant Differential Expression ◽  
Primary Tumors ◽  
Unbiased Manner

Trastuzumab (Herceptin) is a monoclonal antibody targeting the extracellular domain of the human epidermal growth factor receptor 2 (HER2) (1) utilized for the treatment of adjuvant and metastatic breast cancer (2) in the United States and worldwide. We mined published and public microarray and gene expression data (3, 4) to discover in an unbiased manner the most striking transcriptional features of trastuzumab treatment. We identified significant differential expression of the gene encoding the Wnt pathway molecule dishevelled-3, DVL3 (5-7), in the primary tumors of breast cancer patients treated with trastuzumab. DVL3 expression in primary tumors of the breast in patients treated with trastuzumab was significantly higher than in patients not treated with trastuzumab.

scholarly journals Anterior Controllable Antedisplacement and Fusion (ACAF) technique for the treatment of multilevel cervical spondylotic myelopathy with spinal stenosis (MCSMSS): a retrospective study of 45 cases

2019 ◽  
Author(s):  
Xi Luo ◽  
Kaiqiang Sun ◽  
Jingchuan Sun ◽  
Shunmin Wang ◽  
Yuan Wang ◽  
...  
Keyword(s):  
Spinal Stenosis ◽  
Cervical Spondylotic Myelopathy ◽  
Japanese Orthopaedic Association ◽  
Neck Disability Index ◽  
Operation Time ◽  
Significant Difference ◽  
Csf Leakage ◽  
One Year ◽  
Neck Disability ◽  
Multilevel Cervical Spondylotic Myelopathy

Abstract Background To investigate the clinical effect of anterior controllable antedisplacement and fusion (ACAF) technique for the treatment of multilevel cervical spondylotic myelopathy with spinal stenosis (MCSMSS), and compare ACAF with hybrid decompression fixation (HDF). Methods A retrospective analysis of 85 cases with MCSMSS was carried out. 45 patients were treated with ACAF, while 40 patients were treated with HDF. The operation time, intraoperative bleeding volume, postoperative complications, Japanese Orthopaedic Association (JOA) score, Neck Disability Index (NDI) score, Computed Tomography (CT) transverse measurement, cervical curvature and Kang's grade were compared between two groups. Results The patients were followed up for 12 to 17 months. Compared with HDF, ACAF group achieved better decompression according to CT measurement and Kang’s grade (P < 0.05), and recovered to a greater cervical Cobb’s angle (P < 0.05). However, JOA score and NDI index showed no significant difference one year after surgery (P＞0.05). Additionally, ACAF presented longer operation time and greater intraoperative blood loss (P < 0.05). As to complications, ACAF developed less incidences of cerebrospinal fluid examination (CSF) leakage, neurologic deterioration, epidural hematoma and C5 palsy by comparing with HDF. Conclusions ACAF is an effective method for the treatment of MCSMSS. Compared with HDF, ACAF has the advantages of significant decompression, increasing cervical curvature, and reducing the incidences of complications.

scholarly journals Post-operative Morbidity and Mortality of Lumbar Spinal Stenosis at one Teaching Hospital in North-eastern Benin.

2020 ◽  
Vol 6 (1) ◽  
pp. 13-20
Author(s):  
Holden Olatoundji Fatigba ◽  
◽  
Luphin Hode ◽  
Kisito Quenum ◽  
Thierry Alihonou ◽  
...  
Keyword(s):  
Spinal Stenosis ◽  
Surgical Management ◽  
Lumbar Spinal Stenosis ◽  
Teaching Hospital ◽  
Cauda Equina ◽  
Canal Stenosis ◽  
Republic Of Benin ◽  
The Republic ◽  
Lumbar Spinal ◽  
Selection Of

Background and Aim: Surgical management of Lumbar Spinal Stenosis (LSS) is a common practice. This study aimed to report the morbidities and mortalities observed during surgical treatment of LSS and the outcome of these patients after management over an 8-year study period. Methods and Materials/Patients: It was a retrospective, descriptive, and transversal study performed at the Departmental Teaching Hospital of Borgou in the Republic of Benin (West Africa) from January 2010 to December 2018. This study included the patients who underwent surgical management for LSS. Type of complication, its management, and the patient’s outcomes was registered. Results: During the study period and based on the selection criteria, 239 patients were selected. These patients were divided into 135 men (56.5%) and 104 women (43.5%). The mean age of the patients was 52.3±10.9 years. Laminectomy was performed on one, two, and three lumbar spinal segments in 17.6%, 55.6% and 26.8% of cases, respectively. Laminectomy was associated with discectomy in 6.3% of the cases. No arthrodesis with spinal fixation was performed. Ninety percent of patients had no post-operative complications. Functional post-operative results were considered excellent, good, acceptable and poor in 32.1%, 52.1%, 10.9% and 4.9% of cases, respectively. Five types of complications were observed in 22 patients (9.2%). A reoperation was performed in 1.6% of cases. These different complications included dural tear (4.6%), wound infection (3.3%), stroke (0.8%), cauda equina syndrome (0.4%) and pseudomeningocele (0.4%). The mortality rate was 0.8% (n=2). Conclusion: Lumbar canal stenosis surgery is not without complications. Careful selection of patients, consideration of risk factors, and selection of an appropriate surgical strategy can reduce or avoid these complications.

Treatment of Short Stature in Aggrecan Deficient Patients with Recombinant Human Growth Hormone: One-Year Response

2021 ◽  
Author(s):  
Gajanthan Muthuvel ◽  
Andrew Dauber ◽  
Eirene Alexandrou ◽  
Leah Tyzinski ◽  
Melissa Andrew ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Short Stature ◽  
Human Growth Hormone ◽  
Linear Growth ◽  
Recombinant Human Growth Hormone ◽  
Human Growth ◽  
Skeletal Maturation ◽  
Height Velocity ◽  
Heterozygous Mutation ◽  
One Year

Abstract Context Patients with aggrecan (ACAN) deficiency present with dominantly inherited short stature, often with advanced skeletal maturation and premature growth cessation. There is a paucity of information on the effects of growth-promoting interventions. Objective The aim of this study was to evaluate the efficacy and safety of recombinant human growth hormone (rhGH) therapy on linear growth in children with ACAN deficiency. Design and Setting Open-label, single-arm, prospective study at Cincinnati Children’s Hospital Medical Center. Patients Ten treatment-naïve patients were recruited. Inclusion criteria were: a confirmed heterozygous mutation in ACAN, age ≥ 2 years, pre-pubertal, bone age (BA) ≥ chronological age (CA), and normal IGF-I concentration. Intervention Treatment with rhGH (50 mcg/kg/day) over one year. Main Outcome Measure(s) Main outcomes measured were height velocity (HV) and change in (Δ) height SD (HtSDS). Results Ten patients (six females) were enrolled with median CA of 5.6 yrs (range 2.4 to 9.7). Baseline median HtSDS was -2.5 (range -4.3 to -1.1). Median baseline BA was 6.9 yrs (range 2.5 to 10.0), with median BA/CA of 1.2 (range 0.9 to 1.5). Median pre-treatment HV was 5.2 cm/y (range 3.8 to 7.1), increased to 8.3 cm/y (range 7.3 to 11.2) after one year of therapy (p=0.004). Median ΔHtSDS after one year was +0.62 (range +0.35 to +1.39) (p=0.002). Skeletal maturation did not advance inappropriately (median Δ BA/CA -0.1, p=0.09). No adverse events related to rhGH were observed. Conclusion Treatment with rhGH improved linear growth in a cohort of patients with short stature due to ACAN deficiency.

scholarly journals Intraoperative Myelography in Transpsoas Lateral Lumbar Interbody Fusion for Degenerative Lumbar Spinal Stenosis: A Preliminary Prospective Study

2017 ◽  
Vol 2017 ◽  
pp. 1-8 ◽  
Author(s):  
Yang Yang ◽  
Liangming Zhang ◽  
Jianwen Dong ◽  
Zihao Chen ◽  
Peigen Xie ◽  
...  
Keyword(s):  
Spinal Stenosis ◽  
Lumbar Spinal Stenosis ◽  
Spinal Canal ◽  
Interbody Fusion ◽  
Lumbar Interbody Fusion ◽  
Degenerative Lumbar Spinal Stenosis ◽  
Lateral Lumbar Interbody Fusion ◽  
Radiological Parameters ◽  
One Year ◽  
Lumbar Spinal

Aim. To investigate the feasibility and effectiveness of intraoperative myelography in determining adequacy of indirect spinal canal decompression during transpsoas lateral lumbar interbody fusion (LLIF). Methods. Seven patients diagnosed with degenerative lumbar spinal stenosis (DLSS) were prospectively included to this study. All patients underwent LLIF and subsequently received intraoperative myelography to determine the effect of indirect spinal canal decompression, which was visualized in both anterior-posterior and lateral images. Those patients with insufficient indirect canal decompression were further resolved by microendoscopic canal decompression (MECD). Radiological parameters, including stenosis ratio and dural sac area of operated levels, were measured and compared before and after operation. Besides, all patients were followed up for at least one year using visual analogue scale (VAS) for back and leg, Japanese Orthopaedic Association score (JOA), and Oswestry disability index (ODI). Results. Seven patients with 8 operated levels underwent LLIF safely and demonstrated significant symptom relief postoperatively. Five operated levels showed adequate indirect canal decompression intraoperatively, while the remaining three levels did not achieve the adequacy, and their residual stenosis was resolved following MECD. Radiological parameters were improved statistically when compared with preoperation (P<0.05). Furthermore, neurological symptoms of all patients were also improved significantly (P<0.05), shown by improved VAS (back and leg), JOA, and ODI at both two-week and one-year follow-up. Conclusions. Intraoperative myelography during LLIF is able to assess adequacy of indirect canal decompression for DLSS, thus promising favorable clinical outcomes.

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