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Background: The role of surgical treatment for juvenile myasthenia gravis (MG) remains unclear. Here, we performed a retrospective study to evaluate the predictors of clinical outcome of juvenile MG treated with extended transsternal thymectomy., Methods: A total of 141 consecutive juvenile MG patients underwent extended transsternal thymectomy at an academic hospital over a 20-year period were reviewed. Thymectomy was performed in patients resistant to pyridostigmine therapy, with generalized symptoms or ocular MG with partial response to pyridostigmine for more than 2 years. Variables potentially affecting responses to extended transsternal thymectomy were evaluated using Kaplan-Meier analysis and Cox regression modeling. Complete stable remission (CSR) is defined as asymptomatic without medication for more than 12 months., Results: There were 96 patients with ocular MG and 45 generalized MG, the median age at disease onset was 6 years and that at operation was 12 years. Among 135 patients with complete postoperative follow-up, 34 (25.2%) achieved CSR, 28 (20.7%) experienced pharmacologic remission, 61 (45.2%) improved, 5 (3.7%) remained stable, and 7 (5.2%) deteriorated. The results indicated the disease-onset age greater than 6 years and age at operation greater than 12 years were both positively associated with CSR responses. Postoperative steroid treatments in ocular MG and preoperative disease duration in generalized MG (>12 months) were negatively associated with CSR responses., Conclusions: Extended transsternal thymectomy for Chinese juvenile MG patients has an efficacy comparable with reports from other ethnicities. Juvenile patients with disease-onset age greater than 6 years, age at operation greater than 12 years, and shorter disease duration of generalized MG are associated with favorable clinical outcomes., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The optimal treatment for ocular myasthenia gravis (OMG) remains controversial. We conducted a review of the long-term clinical outcomes of Chinese patients with OMG after extended transsternal thymectomy (ETT) to determine the efficacy of this procedure as a treatment for OMG., Methods: We reviewed the cases of 115 consecutive patients with OMG who underwent ETT at our Myasthenia Gravis Research Center between January 2006 and December 2008. Extended transsternal thymectomy was done in patients who had thymoma, resistance to pyridostigmine therapy, or relapse after immunosuppressive therapy. The patients' postoperative responses were defined as strict complete remission (SCR), consisting of an asymptomatic status without medication for more than 12 months; general complete remission (GCR), consisting of an asymptomatic status with low-dose single-drug therapy or without medication for more than 12 months; or improvement, consisting of fewer symptoms or less of a need for medication than before surgery., Results: The overall complication rate was 7.8%. None of the patients experienced a myasthenic crisis, progression to generalized myasthenia gravis, or mortality. Hyperplasia of the thymus was present in 106 of the 115 patients (92.2%). Among 110 patients on whom follow-up was done postoperatively, 29 (26.4%) were in SCR, 64 (58.2%) showed improvement, 7 (6.4%) remained unchanged, and 10 (9.1%) had a worsening of their conditions. Kaplan-Meier analysis revealed rates of GCR of 41.8% at 24 months and 47.3% at 48 months after surgery, and rates of SCR of 24.5% at 24 months and 26.4% at 48 months. Both univariate analysis and multivariate Cox regression analysis revealed that only preoperative duration of illness was positively associated with GCR (p < 0.001)., Conclusions: The results of the review indicate that ETT is a safe and effective treatment for OMG, especially in patients with illness of shorter duration., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The diagnosis of myasthenia gravis (MG) remains challenging. We performed a proteome-wide search for potential serum protein diagnostic markers for MG using surface-enhanced laser desorption/ionization (SELDI) time-of-flight mass spectrometry (TOFMS)., Methods: Proteomic spectra from 80 MG patients and 80 healthy individuals were generated by SELDI. Samples from 56 MG patients and 56 healthy individuals in the training set were analyzed to set up the decision tree. Samples from 24 MG patients and 24 healthy individuals were used for cross-validation testing., Results: The SELDI TOFMS analysis generated 101 peaks, representing differentially expressed proteins between 1000 and 20000 Da. Among them, 9 peaks were down-regulated and 30 others were up-regulated in the MG sera compared with the controls. The decision tree used the peak at M4168.94 Da and M1122.57 Da as splitters in the classification process. In the training set, 112 samples were classified as MG or control group, with a sensitivity of 100% and specificity of 89.3%; the 10-fold cross-validated analysis identified the optimal decision tree with the lowest relative cross-validated cost of 0.080. In the test set, the decision tree generated was able to identify 20 of 24 MG patients and 21 of 24 healthy individuals with a sensitivity of 83.3% and a specificity of 87.5%., Conclusions: SELDI TOFMS is a useful tool for the detection and identification of potential serum biomarkers that can diagnose MG with high sensitivity and specificity.

Aneurysmal bone cyst is a benign tumor of the skeletal system that rarely occurs in ribs. We report two cases of aneurysmal bone cyst in the first rib. The first patient was a 21-year-old woman with an aneurysmal bone cyst in the left first rib that was resected with an L incision. The second patient was a 42-year-old man with an aneurysmal bone cyst in the right first rib that was resected with a posterolateral incision but recurred 1 year later. An en bloc resection was performed, without recurrence to date. We also review this disease with emphasis on the etiology, clinicopathology, and treatment approaches.