Citations

Children with pediatric-onset multiple sclerosis and pediatric controls were enrolled across 16 pediatric multiple sclerosis centers in the United States and completed questionnaires that addressed time of first unaided walking and acquisition of 2-word phrases. A total of 467 (308 female) cases and 428 (209 female) controls were enrolled. Pediatric multiple sclerosis (n = 467) were not delayed in walking or using 2-word phrases compared to healthy controls (n = 428) (2.2% vs 5.7%, respectively). Children with disease onset before age 11 versus onset at 11 years or after were more likely to need an individualized education plan ( P = .002), reading assistance ( P = .0003), and math assistance ( P = .001). Children with multiple sclerosis onset prior to age 18 are not delayed in meeting the 2 major early developmental milestones but do have a significantly increased use of special services or learning assistance at school. Further research will need to address whether other measures of development (eg, rate of language acquisition or fine motor skills) differ between pediatric multiple sclerosis and controls.

Hedgehog (HH) signaling participates in hepatobiliary repair after injury and is activated in patients with cholangiopathies. Cholangiopathies are associated with bile duct (BD) hyperplasia, including expansion of peribiliary glands, the niche for biliary progenitor cells. The inflammation‐associated cytokine interleukin (IL)‐33 is also up‐regulated in cholangiopathies, including cholangiocarcinoma. We hypothesized that HH signaling synergizes with IL‐33 in acute inflammation‐induced BD hyperplasia. We measured extrahepatic BD (EHBD) thickness and cell proliferation with and without an IL‐33 challenge in wild‐type mice, mice overexpressing Sonic HH ( pCMV‐Shh ), and mice with loss of the HH pathway effector glioma‐associated oncogene 1 ( Gli1lacZ/lacZ ). LacZ reporter mice were used to map the expression of HH effector genes in mouse EHBDs. An EHBD organoid (BDO) system was developed to study biliary progenitor cells in vitro . EHBDs from the HH overexpressing pCMV‐Shh mice showed increased epithelial cell proliferation and hyperplasia when challenged with IL‐33. In Gli1lacZ/lacZ mice, we observed a decreased proliferative response to IL‐33 and decreased expression of Il6 . The HH ligands Shh and Indian HH ( Ihh ) were expressed in epithelial cells, whereas the transcriptional effectors Gli1 , Gli2 , and Gli3 and the HH receptor Patched1 ( Ptch1 ) were expressed in stromal cells, as assessed by in situ hybridization and lacZ reporter mice. Although BDO cells lacked canonical HH signaling, they expressed the IL‐33 receptor suppression of tumorigenicity 2. Accordingly, IL‐33 treatment directly induced BDO cell proliferation in a nuclear factor κB‐dependent manner. Conclusion: HH ligand overexpression enhances EHBD epithelial cell proliferation induced by IL‐33. This proproliferative synergism of HH and IL‐33 involves crosstalk between HH ligand‐producing epithelial cells and HH‐responding stromal cells.

Background The specificity of the aquaporin-4 antibody to predict recurrent inflammatory central nervous system disease has led to the design of the 2015 neuromyelitis optica spectrum disorder criteria which capture all aquaporin-4 antibody seropositive patients. Objective The purpose of this study was to compare treatment outcomes in aquaporin-4 antibody seropositive patients who met the previous 2006 clinical criteria for neuromyelitis optica with patients who meet the 2015 neuromyelitis optica spectrum disorder criteria. Methods The study involved a three-center retrospective chart review of clinical outcomes among aquaporin-4 patients diagnosed with neuromyelitis optica and neuromyelitis optica spectrum disorder. Results Hazard ratios of relapse during immunosuppressive therapy, relative to pre-therapy, were not significantly different for patients who met the 2006 criteria of neuromyelitis optica versus the 2015 neuromyelitis optica spectrum disorder criteria among those treated with azathioprine ( p = 0.24), mycophenolate mofetil ( p = 0.63), or rituximab ( p = 0.97). Conclusion Reductions in the hazard of relapse during treatment with immunosuppressive therapies, relative to average pre-treatment, were not different for aquaporin-4 antibody seropositive patients categorized using the 2006 criteria of neuromyelitis optica and the 2015 neuromyelitis optica spectrum disorder criteria. These therapeutic findings support the design of the 2015 neuromyelitis optica spectrum disorder criteria which capture all aquaporin-4 antibody seropositive patients.

Case: A 49-year-old right-hand-dominant man sustained an auger-related injury that resulted in open dislocation at the left wrist and palm, with complete amputation of the distal aspect of the forearm and the hand. The injury at the elbow included instability with an ulnar coronoid fracture, posterior dislocation of the ulna, and posterolateral dislocation of the radius. To restore stability of the forearm stump and elbow, we performed a complete resection of the radius, open reduction and internal fixation of the coronoid tip, a repair of the lateral collateral ligament, and transfer of the distal biceps tendon to the coronoid. Conclusion: The procedure stabilized the elbow, allowing for early mobilization. The patient was eventually fitted with a prosthesis that allowed him to return to full-time manual labor in a rural setting.

Study Design. This study retrospectively analyzes prospectively collected data. Objective. Here, we aim to determine the influence of preoperative and 12-month modified Japanese Orthopedic Association (mJOA) on satisfaction and understand the change in mJOA severity classification after surgical management of degenerative cervical myelopathy (DCM). Summary of Background Data. DCM is a progressive degenerative spine disease resulting from cervical cord compression. The natural progression of DCM is variable; some patients experience periods of stability, while others rapidly deteriorate following disease onset. The mJOA is commonly used to grade and categorize myelopathy symptoms, but its association with postoperative satisfaction has not been previously explored. Methods. The quality and outcomes database (QOD) was queried for patients undergoing elective surgery for DCM. Patients were divided into mild (≥14), moderate (9 to 13), or severe (<9) categories on the mJOA scores. A McNemar–Bowker test was used to assess whether a significant proportion of patients changed mJOA category between preoperative and 12 months postoperative. A multivariable proportional odds ordinal logistic regression model was fitted with 12-month satisfaction as the outcome of interest. Results. We identified 1963 patients who underwent elective surgery for DCM and completed 12-months follow-ups. Comparing mJOA severity level preoperatively and at 12 months revealed that 55% remained in the same category, 37% improved, and 7% moved to a worse category. After adjusting for baseline and surgery-specific variables, the 12-month mJOA category had the highest impact on patient satisfaction (P < 0.001). Conclusion. Patient satisfaction is an indispensable tool for measuring quality of care after spine surgery. In this sample, 12-month mJOA category, regardless of preop mJOA, was significantly correlated with satisfaction. Given these findings, it is important to advise patients of the probability that surgery will change their mJOA severity classification and the changes required to achieve postoperative satisfaction. Level of Evidence: 3