1.Thrombotic thrombocytopenic purpura in pediatric patients.
Melanie STEELE ; Howard H W CHEN ; Jeremy STEELE ; Anthony K C CHAN ; Keith K LAU
Chinese Journal of Contemporary Pediatrics 2012;14(11):803-810
Although thrombotic thrombocytopenic purpura (TTP) is rarely seen in pediatric patients, failure to recognize this condition often leads to severe consequences and poor outcomes. Classic features of TTP include thrombocytopenia, microangiopathic hemolytic anemia, acute kidney injury, fever, and central nervous system involvement. However, patients suffering from this condition may not present with all of the symptoms simultaneously. Therefore, it is of utmost importance for healthcare providers to have a high index of suspicion. Laboratory investigations may reveal the presence of schistocytes on peripheral blood smear, negative Coombs test, high lactate dehydrogenase levels and severely low platelet counts. The etiology of TTP is mainly due to insufficient cleavage of the large multimers of von Willebrand factor (vWF) secondary to decreased activity of ADAMTS13 (a disintegrin and metalloprotease with Thrombospondin type 1 repeats, member 13). TTP can be broadly classified into familial TTP (Upshaw Schulman syndrome) and non-familial TTP. Familial TTP is due to a congenital deficiency of ADAMTS13. Its mainstay of therapy is initiation of plasmapheresis during the acute phase, followed by regular fresh frozen plasma (FFP) infusions. Alternatively, non-familial TTP is due to a decrease in ADAMTS13 activity secondary to the presence of anti-ADAMTS13 antibodies. Once again, the primary treatment is plasmapheresis; however, recent anecdotal data also supports the use of rituximab in select cases.
ADAM Proteins
;
genetics
;
ADAMTS13 Protein
;
Antibodies, Monoclonal, Murine-Derived
;
therapeutic use
;
Child
;
Humans
;
Plasmapheresis
;
Purpura, Thrombotic Thrombocytopenic
;
etiology
;
therapy
;
Rituximab
2.Spine Fractures of Patients with Ankylosing Spondylitis and Diffuse Idiopathic Skeletal Hyperostosis: Fracture Severity and Injury-Related Mortality at a Level I Trauma Center
Stephen Ryan CHEN ; Maria Amelia MUNSCH ; Joseph CHEN ; Brandon Keith COUCH ; Richard Alan WAWROSE ; Anthony Abimbade OYEKAN ; Joshua ADJEI ; William F. DONALDSON ; Joon Yung LEE ; Jeremy DeWitt SHAW
Asian Spine Journal 2023;17(3):549-558
Methods:
Patients presenting with a spine fracture were diagnosed with AS or DISH at a single tertiary care center between 2010 and 2019. We excluded those who lacked cross-sectional imaging or fractures occurring at spinal segments affected by ankylosis, as well as polytraumatized patients. Patient demographics, injury mechanism, fracture level, neurologic status, treatment, and 1-year mortality were recorded. Computed tomography imaging was reviewed by two independent readers and graded according to the indicated AO Spine Injury Classification System. Differences in fracture severity, treatment method, and mortality were examined using Student t -tests, chi-square tests, and two-proportion Z-tests with significance set to p <0.05.
Results:
We identified 167 patients with spine fracture diagnosed with AS or DISH. Patients with AS had more severe fractures and more commonly had surgery than patients with DISH (p <0.001). Despite these differences, 1-year mortality did not significantly differ between AS and DISH patients (p =0.14).
Conclusions
Although patients with AS suffered more severe fractures compared to DISH and more frequently underwent surgery for these injuries, outcomes and 1-year mortality did not differ significantly between the two groups. For patients with ASDs and fractures, outcomes appear similar regardless of treatment modality. Consequently, there may be an opportunity for critical reappraisal of operative indications in ASD and a larger role for nonoperative management in these challenging patients.
3.Minimally Invasive Approach to Supra-pubic and Non-Midline Lower Abdominal Ventral Hernia: An Extended Indication of TAPE Technique.
Joe King Man FAN ; Jeremy YIP ; Matrix FUNG ; Oswens Siu Hung LO ; Jianwen LIU ; Xuefei YANG ; Kejin CHEN ; Wai Lun LAW
Journal of Minimally Invasive Surgery 2017;20(3):84-92
Repair of lower abdominal incisional hernia is always a surgical challenge. TAPE technique has been described for the repair of supra-pubic midline incisional hernia with satisfactory outcome. Its indication can be extended for treatment of non-midline lower abdominal hernia. Peritoneal incision is created just below the hernia defect with pre-peritoneal dissection to expose supra-pubic preperitoneal space with Cooper's ligament exposed. Non-adhesive mesh then placed over preperitoneal space and partially intra-peritoneally, and cover the whole extra-peritoneal space prepared to ensure enough overlapping. Mesh is fixed by tackers for intra-peritoneal part, most inferior fixation points were at peritoneal incision line. Extra-peritoneal part of meshes is fixed at the safety zone and covered up by the peritoneal flap to avoid mesh migration. Fixation of the meshes at the lateral aspects were facilitated by the peritoneal flap and subsequent fibrosis and adhesion to the extra-peritoneal structures in cases of lateral lower abdominal hernia. Repair of midline and lateral lower abdominal incisional hernia with this novel modified technique with prosthetic mesh is safe and effective. A larger case series and longer follow-up is required for validation of this technique.
Fibrosis
;
Follow-Up Studies
;
Hernia
;
Hernia, Abdominal
;
Hernia, Ventral*
;
Incisional Hernia
;
Ligaments
4.The Association of Acquired T790M Mutation with Clinical Characteristics after Resistance to First-Line Epidermal Growth Factor Receptor Tyrosine Kinase Inhibitor in Lung Adenocarcinoma.
Yen Hsiang HUANG ; Kuo Hsuan HSU ; Jeng Sen TSENG ; Kun Chieh CHEN ; Chia Hung HSU ; Kang Yi SU ; Jeremy J W CHEN ; Huei Wen CHEN ; Sung Liang YU ; Tsung Ying YANG ; Gee Chen CHANG
Cancer Research and Treatment 2018;50(4):1294-1303
PURPOSE: The main objective of this study was to investigate the relationship among the clinical characteristics and the frequency of T790M mutation in advanced epidermal growth factor receptor (EGFR)–mutant lung adenocarcinoma patients with acquired resistance after firstline EGFR–tyrosine kinase inhibitor (TKI) treatment. MATERIALS AND METHODS: We enrolled EGFR-mutant stage IIIB-IV lung adenocarcinoma patients, who had progressed to prior EGFR-TKI therapy, and evaluated their rebiopsy EGFR mutation status. RESULTS: A total of 205 patients were enrolled for analysis. The overall T790M mutation rate of rebiopsy was 46.3%. The T790M mutation rates among patients with exon 19 deletion mutation, exon 21 L858R point mutation, and other mutations were 55.0%, 37.3%, and 27.3%, respectively. Baseline exon 19 deletion was associated with a significantly higher frequency of T790M mutation (adjusted odds ratio, 2.14; 95% confidence interval [CI], 1.20 to 3.83; p=0.010). In the exon 19 deletion subgroup, there was a greater prevalence of T790M mutation than other exon 19 deletion subtypes in patients with the Del E746-A750 mutation (61.6% vs. 40.6%; odds ratio, 2.35; 95% CI, 1.01 to 5.49; p=0.049). The progression-free survival (PFS) of first-line TKI treatment > 11 months was also associated with a higher T790M mutation rate (54.1% vs. 39.3%; adjusted odds ratio, 1.82; 95% CI, 1.02 to 3.25; p=0.044). Patients who underwent rebiopsy at metastatic sites had more chance to harbor T790M mutation (52.6% vs. 33.8%; adjusted odds ratio, 1.97; 95% CI, 1.06 to 3.67; p=0.032). CONCLUSION: PFS of first-line EGFR-TKI, rebiopsy site, EGFR exon 19 deletion and its subtype Del E746-A750 mutation are associated with the frequency of T790M mutation.
Adenocarcinoma*
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Disease-Free Survival
;
Epidermal Growth Factor*
;
Exons
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Humans
;
Lung Neoplasms
;
Lung*
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Mutation Rate
;
Odds Ratio
;
Phosphotransferases
;
Point Mutation
;
Prevalence
;
Receptor, Epidermal Growth Factor*
;
Sequence Deletion
5.The Clinical Outcomes of Different First-Line EGFR-TKIs Plus Bevacizumab in Advanced EGFR-Mutant Lung Adenocarcinoma
Yen-Hsiang HUANG ; Kuo-Hsuan HSU ; Chun-Shih CHIN ; Jeng-Sen TSENG ; Tsung-Ying YANG ; Kun-Chieh CHEN ; Kang-Yi SU ; Sung-Liang YU ; Jeremy J.W. CHEN ; Gee-Chen CHANG
Cancer Research and Treatment 2022;54(2):434-444
Purpose:
The aim of this study was to investigate the efficacy of various epidermal growth factor receptor (EGFR)–tyrosine kinase inhibitors (TKIs) plus bevacizumab in advanced EGFR-mutant lung adenocarcinoma patients.
Materials and Methods:
From August 2016 to October 2020, we enrolled advanced lung adenocarcinoma patients harboring exon 19 deletion or L858R receiving gefitinib, erlotinib and afatinib plus bevacizumab as the first-line treatment for the purposes of analysis.
Results:
A total of 36 patients were included in the final analysis. Three patients received gefitinib, 17 received erlotinib, and 16 received afatinib combined with bevacizumab as the first-line treatment. The objective response rate was 77.8%, and disease control rate was 94.4%. The overall median progression-free survival (PFS) was 16.4 months, while the median PFS was 17.1 months in patients with exon 19 deletion, and 16.2 months in patients with L858R mutation (p=0.311). Regarding the use of different EGFR-TKIs, the median PFS was 17.1 months in the erlotinib group and 21.6 months in the afatinib group (p=0.617). In patients with brain metastasis at baseline, the median PFS was 18.9 months in the erlotinib group and 16.4 months in the afatinib group (p=0.747). Amongst patients harboring exon 19 deletion, the median PFS was 16.2 months in the erlotinib group and not-reached in the afatinib group (p=0.141). In patients with L858R mutation, the median PFS was 18.9 months in the erlotinib group and 16.2 months in the afatinib group (p=0.481).
Conclusion
Our research demonstrates that not only erlotinib combined with bevacizumab, but also afatinib plus bevacizumab as first-line treatment, provides solid clinical efficacy in advanced EGFR-mutant lung adenocarcinoma patients.
6.Transitions of the Understanding and Definition of Primary Glaucoma.
Si-An LIU ; Zhen-Ni ZHAO ; Nan-Nan SUN ; Ying HAN ; Jeremy CHEN ; Zhi-Gang FAN
Chinese Medical Journal 2018;131(23):2852-2859
Objective:
In previous decades, glaucoma has been primarily attributed to elevated intraocular pressure (IOP), but this has gradually been replaced by the development of optic neuropathy as the central concept of glaucoma in developed countries. However, there still remain strong controversies in the definition of glaucoma in China. In this current review, we are going to discuss these controversies and elaborate on the historical transitions of the definition of glaucoma both in China and developed countries. Furthermore, we will briefly describe the "ocular-cranial pressure gradient" theory and discuss the relationship between glaucoma and degenerative diseases of the central nervous system (CNS) in order to show the complex pathogenesis of glaucoma and the importance for the modification to the definition of glaucoma.
Data Sources:
We performed a comprehensive search in both PubMed and SinoMed using the following keywords: (a) "primary glaucoma" and "guideline," (b) "ocular-cranial pressure gradient," and (c) "glaucoma," "Alzheimer's disease," and "Parkinson's disease." The literature search included the related articles with no restrictions on publication date.
Study Selection:
The primary references were Chinese and English articles including (a) original guidelines and expert consensuses of primary glaucoma, (b) reviews focusing on the differences between various versions of these guidelines and consensuses, and (c) papers about ocular-cranial pressure gradient theory and the relationship between glaucoma and CNS degenerative diseases.
Results:
The definitions and classifications of both primary open-angle glaucoma and primary angle-closure glaucoma differ between Chinese glaucoma consensuses and international primary glaucoma guidelines. Chinese definitions and classifications put more emphasis on the IOP, while international guidelines put more emphasis on the presence of optic neuropathy. The ocular-cranial pressure gradient theory and the research on the relationship between glaucoma and CNS degenerative diseases have provided new directions for exploring the pathogenesis of glaucoma.
Conclusions
As regards the definition and classification of primary glaucoma, we find that there are still some discrepancies between Chinese expert consensuses and international guidelines. Glaucoma is a disease with complex etiologies, while its common characteristic is a specific optic neuropathy. The current definition and understanding of glaucoma is an ongoing and evolving process, reflecting our latest available evidence on its pathogenesis. Chinese ophthalmology community may need to update our guidelines, accommodating these latest developments.
China
;
Glaucoma
;
diagnosis
;
physiopathology
;
Glaucoma, Angle-Closure
;
diagnosis
;
physiopathology
;
Glaucoma, Open-Angle
;
diagnosis
;
physiopathology
;
Humans
;
Intraocular Pressure
;
physiology
;
Ophthalmology
;
methods
7.Interaction Between Variations in Dopamine D2 and Serotonin 2A Receptor is Associated with Short-Term Response to Antipsychotics in Schizophrenia.
Liansheng ZHAO ; Huijuan WANG ; Yamin ZHANG ; Jinxue WEI ; Peiyan NI ; Hongyan REN ; Gang LI ; Qiang WANG ; Gavin P REYNOLDS ; Weihua YUE ; Wei DENG ; Hao YAN ; Liwen TAN ; Qi CHEN ; Guigang YANG ; Tianlan LU ; Lifang WANG ; Fuquan ZHANG ; Jianli YANG ; Keqing LI ; Luxian LV ; Qingrong TAN ; Yinfei LI ; Hua YU ; Hongyan ZHANG ; Xin MA ; Fude YANG ; Lingjiang LI ; Chuanyue WANG ; Huiyao WANG ; Xiaojing LI ; Wanjun GUO ; Xun HU ; Yang TIAN ; Xiaohong MA ; Jeremy COID ; Dai ZHANG ; Chao CHEN ; Tao LI ; Chinese Antipsychotics Pharmacogenomics Consortium
Neuroscience Bulletin 2019;35(6):1102-1105