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1.
Zhonghua Yan Ke Za Zhi ; 60(1): 64-71, 2024 Jan 11.
Artículo en Zh | MEDLINE | ID: mdl-38199770

RESUMEN

Objective: To analyze the clinical pathological and genetic mutation characteristics of conjunctival lymphoepithelial carcinoma. Methods: A retrospective case series study was conducted. Data from three patients diagnosed with conjunctival lymphoepithelial carcinoma and treated with tumor resection surgery at Tianjin Eye Hospital from January 2006 to December 2022 were collected. Four paraffin specimens (including one patient undergoing two surgeries) were subjected to immunohistochemical staining for epithelial antigen and lymphocytic antigen. Epstein-Barr virus (EBV)-encoded RNA (EBER) was detected using in situ hybridization, and whole-exome sequencing was performed on three specimens from two patients using next-generation sequencing methods. Results: All three patients were males aged over 65, with a disease duration ranging from 3 to 44 months. The tumors were unilateral, located on the bulbar or limbal conjunctiva, appearing red, with a maximum diameter of 4-20 mm. Imaging examinations revealed anterior location of the tumors with no involvement of the orbital bone, extraocular muscles, optic nerve, or paranasal sinuses. No local lymph node metastasis was observed in any patient. Pathological findings included undifferentiated carcinoma nests with significant reactive lymphocytic and plasma cell infiltration. Tumor cells were positive for pan-cytokeratin (CK-pan), epithelial membrane antigen (EMA), tumor protein 40 (p40), and tumor protein 63 (p63), with a cell proliferation index (Ki67) exceeding 80%. Cluster of differentiation 20 (CD20), CD3, and CD8 were positive for lymphocytes. In situ hybridization showed partial tumor cell expression of EBER in two specimens of one patient. Whole-exome sequencing revealed 58, 50, and 36 mutated genes in the three specimens, with enriched signaling pathways including melanoma signaling pathway, Notch1 signaling pathway, and RHOQ GTP cycle; enriched biochemical processes included amino acid starvation response, programmed cell death, regulation of lipid synthesis, sodium ion transport, and chromosome segregation. The common mutated gene in all three specimens was SZT2, and SZT2 was involved in the amino acid starvation response. One patient underwent a second complete resection surgery 40 months after partial excision, while the other two underwent complete resection surgery without recurrence. Two patients did not undergo radiation or chemotherapy, and one was lost to follow-up. Conclusions: Conjunctival lymphoepithelial carcinoma is associated with prominent lymphocytic and plasma cell infiltration, some cases are associated with EBV infection, and SZT2 mutations are present in conjunctival lymphoepithelial carcinoma.


Asunto(s)
Carcinoma , Neoplasias de la Conjuntiva , Infecciones por Virus de Epstein-Barr , Neoplasias Cutáneas , Masculino , Humanos , Femenino , Infecciones por Virus de Epstein-Barr/genética , Estudios Retrospectivos , Herpesvirus Humano 4 , Neoplasias de la Conjuntiva/genética , Aminoácidos , Proteínas del Tejido Nervioso
2.
Zhonghua Yi Xue Za Zhi ; 103(42): 3394-3401, 2023 Nov 14.
Artículo en Zh | MEDLINE | ID: mdl-37963737

RESUMEN

Objective: To analyze the clinical characteristics of patients with severe immune checkpoint inhibitors (ICIs) related myocarditis. Methods: A retrospective study was conducted on the 50 patients with ICIs-related myocarditis in the multidisciplinary cardio-oncology clinic of Zhongshan Hospital affiliated to Fudan University from April 2020 to April 2022. The age of patients was (63.7±10.8) years old, including 37 males and 13 females. The patients were divided into the mild group (n=37) and the severe group (n=13) according to severity. The differences of basic characteristics, clinical manifestations, laboratory tests, auxiliary examination, combined irAEs, treatment and outcomes between the two groups of patients were analyzed. Results: The immunotherapy time [M(Q1,Q3)] of patients in the mild group and severe group were 81 (49, 134) and 24 (20, 116) days, respectively (P<0.05). In the severe group, the levels of cTnT [0.605 (0.317, 1.072) µg/L], NT-proBNP [1 126 (386, 1 744) ng/L], CK-MB [78 (48, 238) U/L], and CK-MM [240 (45, 6 543) U/L] were higher than those in the mild group [0.104 (0.045, 0.189) µg/L, 237 (39, 785) ng/L, 24 (20, 33) U/L, 108 (72, 168) U/L, respectively] (all P<0.05). The left ventricular ejection fraction of the severe group [64% (57%, 65%)] was lower than that of the mild group [66% (63%, 69%)] (P<0.05), and the incidence of conduction block (n=4, 4/13) and abnormal ventricular wall motion (n=4, 4/13), the incidence of ICIs-related myositis (n=10, 10/13), ICIs-related hepatitis (n=4, 4/13) and ICIs-related neurotoxicity (n=4, 4/13) were higher than those in the mild group (n=1, 2.7%; n=2, 5.4%; n=16, 43.2%; n=2, 5.4%; n=1, 2.7%, respectively) (all P<0.05). The proportion of patients receiving intensified immunosuppressive therapy and mortality rate in the severe group were 12/13 (n=12) and 4/13 (n=4), which were both higher than those in the mild group [10.8% (n=4) and 0] (both P<0.05). Conclusions: The incidence of ICIs-related myocarditis is not high, but the severe rate and mortality are high. The differential diagnosis of severe ICIs related myocarditis should be combined with myocardial markers, electrocardiogram and echocardiogram, and early diagnosis and treatment can improve the prognosis of patients.


Asunto(s)
Inhibidores de Puntos de Control Inmunológico , Miocarditis , Masculino , Femenino , Humanos , Persona de Mediana Edad , Anciano , Inhibidores de Puntos de Control Inmunológico/efectos adversos , Miocarditis/inducido químicamente , Estudios Retrospectivos , Volumen Sistólico , Función Ventricular Izquierda
3.
Zhonghua Yan Ke Za Zhi ; 59(11): 930-936, 2023 Nov 11.
Artículo en Zh | MEDLINE | ID: mdl-37936361

RESUMEN

Objective: To investigate the clinical pathological characteristics of ocular adnexal follicular lymphoma (OAFL). Methods: A retrospective case series study was conducted. Clinical data of 10 OAFL patients diagnosed at Tianjin Eye Hospital from January 1990 to May 2022 were collected. The study analyzed general patient information, medical history, site of involvement, imaging, histopathology, and molecular detection. Among them, 7 cases underwent Epstein-Barr virus-encoded small RNA (EBER) and B-cell lymphoma protein 2 (BCL-2)/immunoglobulin heavy chain gene (IgH) translocation gene detection. Treatment and prognosis of patients were followed up. Results: All 10 patients (10 eyes) had unilateral involvement, including 5 males and 5 females, with an age range of 58 (43, 68) years. Clinical manifestations included eyelid swelling, pink conjunctival thickening, painless slow-growing masses in the lacrimal gland area, extraconal muscle cone, conjunctiva, lacrimal sac, or a combination of lacrimal sac and conjunctiva. Among them, 8 cases were primary, and 2 cases were secondary. According to the Ann Arbor staging, 8 cases were stage Ⅰ-Ⅱ E, and 2 cases were stage Ⅲ E. Histopathological grading revealed 6 cases of grade 1-2 and 3 cases of grade 3A. One case showed grade 3B in the lacrimal sac area and grade 1-2 in the conjunctiva. The predominant subtype was follicular in 4 cases, diffuse in 3 cases, and mixed in 2 cases. One case had a mixed subtype involving the lacrimal sac and conjunctiva. All patients expressed positivity for leukocyte differentiation antigens (CD) 20, CD21, and CD23. Nine cases were positive for CD10, with 1 case showing partial CD10 positivity. All patients were positive for B-cell lymphoma protein 6 (BCL-6), and 9 cases were positive for BCL-2. Specific markers CyclinD1 and Multiple Myeloma Oncogene Protein 1 (MUM-1) were negatively expressed in all cases. The Ki-67 proliferation index ranged from 10% to 90%. Molecular detection was performed in 7 patients, with none showing positive EBER in situ hybridization. However, 5 cases exhibited BCL2/IgH gene fusion. Among 7 patients with follow-up data, the median follow-up time was 17 (6, 34) months. Four patients achieved complete remission, 2 had partial remission, and 1 patient died due to lung infection. Conclusions: OAFL is a tumor originating from follicular center B-cells, characterized by positive expression of BCL-2, CD10, and BCL-6. It can involve the lacrimal gland, extraconal muscles, lacrimal sac, and conjunctiva. Patients generally have a favorable prognosis.


Asunto(s)
Infecciones por Virus de Epstein-Barr , Neoplasias del Ojo , Aparato Lagrimal , Linfoma de Células B , Linfoma Folicular , Masculino , Femenino , Humanos , Persona de Mediana Edad , Linfoma Folicular/diagnóstico , Linfoma Folicular/patología , Linfoma Folicular/terapia , Estudios Retrospectivos , Infecciones por Virus de Epstein-Barr/patología , Herpesvirus Humano 4/metabolismo , Neoplasias del Ojo/patología , Proteínas Proto-Oncogénicas c-bcl-2/genética , Proteínas Proto-Oncogénicas c-bcl-2/metabolismo , Aparato Lagrimal/patología
4.
Zhonghua Yan Ke Za Zhi ; 59(3): 207-212, 2023 Mar 11.
Artículo en Zh | MEDLINE | ID: mdl-36860108

RESUMEN

Objective: To analyze the clinical and pathological characteristics and prognosis of 35 cases of solitary fibrous tumor of ocular adnexal (SFT). Methods: This was a retrospective case series study. The clinical data of 35 cases of ocular adnexal SFT was collected in Tianjin Eye Hospital from January 2000 to December 2020. The clinical manifestations, imaging examination results, pathological characteristics, treatment of patients were analyzed, and patients were followed up. All cases were classified according to the 2013 classification of World Health Organization of tumors of soft tissue and bone. Results: There were 21 males (60.0%) and 14 females (40.0%). The age range was 17 to 83 years, and the median age was 44 (35, 54)years. All patients were unilateral, with 23 (65.7%) in the right eye and 12 (34.3%) in the left eye. The course of disease ranged from 2 months to 11 years, with an a median duration of 12(6,36)months. Clinical manifestations included exophthalmos, limited eye movements, diplopia, and tearing. All patients underwent surgical treatment of complete resection of the tumor. Ocular adnexal SFT mostly occurred in the upper orbit (19 cases, 73.1%). On imaging examination, the tumor showed well-circumscribed space occupying lesion that heterogeneously enhanced with contrast, and abundant blood flow signals in the tumors. MRI showed isointensity or low signal on T1WI, and significantly enhanced on T2WI, presenting intermediate-to-high heterogeneous signals. The tumor diameter was 2.1 (1.5, 2.6) cm. There were 23 cases (65.7%) of classic subtype, 2 cases (5.7%) of giant cell subtype, 8 cases (22.9%) of myxoid subtype, and 2 cases (5.7%) of malignancy. Immunohistochemical staining showed that Vimentin, CD34 and STAT6 were positively expressed in all patients. Twenty-one cases (60.0%) showed positive expression of BCL-2, and Ki-67 positive index ranged from 1.0% to 10.0%. Tumors in this group were all low-risk according to the Demicco risk stratification. Follow-up was available for 25 patients with a duration of 2 years to 14 years and 7 months, and the median follow-up time was 88 (61, 124) months. Two patients relapsed, and no distant metastasis or death was observed. Conclusions: Ocular adnexal SFT mainly presents as a painless, slow-growing mass. And most of them are typical SFT. The imaging manifestations are varied Ocular adnexal SFT generally follows a benign course, with a good prognosis after complete excision. Recurrence could occur many years after surgery which requiring careful and long-term follow-up.


Asunto(s)
Exoftalmia , Tumores Fibrosos Solitarios , Femenino , Masculino , Humanos , Adulto , Adolescente , Adulto Joven , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Estudios Retrospectivos , Ojo , Diplopía
5.
Zhonghua Nei Ke Za Zhi ; 61(8): 883-892, 2022 Aug 01.
Artículo en Zh | MEDLINE | ID: mdl-35922212

RESUMEN

Psoriatic arthritis is a chronic systemic autoimmune disease, characterized by psoriasis skin lesions and inflammation of the spine and joint. It has complicated clinical manifestations and individual variations. Nearly half of the patients will have joints erosion in two years, which is crippling. The severity of the skin and joint disease frequently do not correlate with each other. Currently, the understanding of the disease is insufficient in China with the lack of standardized diagnosis and treatment. Therefore, researchers from the Chinese Rheumatology Association formulated this specification based on the diagnosis and management experience together with guidelines at home and abroad. The specification summarizes the present situation of domestic diagnosis and treatment, aiming to standardize the diagnosis process and treatment protocols of psoriatic arthritis. Furthermore, it can reduce misdiagnosis and missed diagnosis, as well as improve the prognosis.


Asunto(s)
Artritis Psoriásica , Psoriasis , Reumatología , Artritis Psoriásica/diagnóstico , Artritis Psoriásica/terapia , China , Humanos , Pronóstico , Psoriasis/diagnóstico , Piel
6.
Zhonghua Yan Ke Za Zhi ; 58(9): 682-687, 2022 Sep 11.
Artículo en Zh | MEDLINE | ID: mdl-36069088

RESUMEN

Objective: To investigate the clinicopathological features of adult orbital xanthogranulomatous disease (AOXGD) with lacrimal gland reactive lymphoid hyperplasia. Methods: Retrospective case series study. The clinical and pathological data of AOXGD cases diagnosed and treated in Tianjin Eye Hospital from January 2002 to December 2021 was reviewed, and the clinical characteristics, radiologic findings and pathological characteristics of periocular and lacrimal gland lesions of 5 cases were retrospectively analyzed. The expression of IgG4 and IgG protein in periocular and lacrimal gland lesions was detected by immunohistochemical staining, and the role of IgG4 in AOXGD was preliminarily studied. Results: There were four females and one male with an average age of 53.8 years (39 to 77 years). Among the five AOXGD cases, there were three cases of adult-onset xanthogranuloma, one case of adult-onset asthma and periocular xanthogranuloma, and one case of necrobiotic xanthogranuloma. All cases involved both eyes. The swelling of eyelids was observed in five cases, and the yellow or pale yellow eyelid skin was found in two cases. Imaging examinations showed the tumor mainly involved the eyelids, subcutaneous tissues, anterior orbit and lacrimal gland. A large number of foam cells and typical Touton giant cells were found in the periorbital lesions, accompanied by different degrees of fibrosis. The fibrinoid necrosis was detected in one case of necrobiotic xanthogranuloma. The lacrimal gland lesions showed different types of reactive lymphoid hyperplasia, including IgG4-related disease in two cases, follicular lymphoid hyperplasia in two cases and focal lymphoid hyperplasia in one case. IgG4 levels of periorbital and lacrimal gland lesions were elevated in four cases. Asthma and elevated serum IgG4 were found in one case of adult-onset periocular xanthogranuloma. Three patients underwent surgical resection and adjuvant hormone or immunosuppressive therapy, and two patients underwent simple surgical resection. The patients were followed up for 1.5 to 10.0 years, one patient was lost, and four patients had no recurrence. Conclusions: AOXGD with lacrimal gland reactive lymphoid hyperplasia is a group of rare diseases. The periorbital lesions of that are characterized by proliferation of foamy histiocytes and Touton giant cells, and the lacrimal gland lesions of that manifest as IgG4-related disease in some cases.


Asunto(s)
Asma , Enfermedad Relacionada con Inmunoglobulina G4 , Aparato Lagrimal , Xantogranuloma Necrobiótico , Enfermedades Orbitales , Seudolinfoma , Xantomatosis , Adulto , Asma/complicaciones , Asma/patología , Femenino , Humanos , Hiperplasia/complicaciones , Hiperplasia/patología , Inmunoglobulina G , Aparato Lagrimal/patología , Masculino , Persona de Mediana Edad , Xantogranuloma Necrobiótico/diagnóstico , Xantogranuloma Necrobiótico/patología , Xantogranuloma Necrobiótico/terapia , Enfermedades Orbitales/terapia , Seudolinfoma/complicaciones , Seudolinfoma/patología , Estudios Retrospectivos , Xantomatosis/diagnóstico
7.
Proc Natl Acad Sci U S A ; 115(9): 1992-1997, 2018 02 27.
Artículo en Inglés | MEDLINE | ID: mdl-29440490

RESUMEN

Despite the widespread use of silicon in modern technology, its peculiar thermal expansion is not well understood. Adapting harmonic phonons to the specific volume at temperature, the quasiharmonic approximation, has become accepted for simulating the thermal expansion, but has given ambiguous interpretations for microscopic mechanisms. To test atomistic mechanisms, we performed inelastic neutron scattering experiments from 100 K to 1,500 K on a single crystal of silicon to measure the changes in phonon frequencies. Our state-of-the-art ab initio calculations, which fully account for phonon anharmonicity and nuclear quantum effects, reproduced the measured shifts of individual phonons with temperature, whereas quasiharmonic shifts were mostly of the wrong sign. Surprisingly, the accepted quasiharmonic model was found to predict the thermal expansion owing to a large cancellation of contributions from individual phonons.

8.
Zhonghua Yan Ke Za Zhi ; 57(5): 366-371, 2021 May 11.
Artículo en Zh | MEDLINE | ID: mdl-33915640

RESUMEN

Objective: To summarize the clinical and pathological characteristics of diffuse large B-cell lymphoma of ocular adnexal (OA-DLBCL). Methods: A retrospective case series study. Twenty-three cases of OA-DLBCL were collected in Tianjin Eye Hospital from January 2005 to December 2018. The clinical manifestations and imaging examination results were analyzed. The pathological characteristics and immune subtypes were analyzed with hematoxylin and eosin staining and immunohistochemistry. Eighteen patients completed the follow-up. Overall survival (OS) time was from the date of diagnosis to the date of death or the final follow-up (April 30, 2020). Clinical stages were analyzed by the Ann Arbor classification; Kaplan-Meier plots were used to visualize survival outcomes. The different risk groups were compared using Log-rank test. Results: There were 13 males and 10 females. The median age was 65 years (range, 43 to 82 years). Twenty-two patients had unilateral lymphoma (14 left eyes, 8 right eyes), and one patient had bilateral lymphoma. The OA-DLBCL was in the orbit in 14 patients, in the lacrimal gland in two patients, in both the lacrimal gland and the orbit in three patients, in both the lacrimal sac and the orbit in one patient, in the conjunctiva in one patient, in both the conjunctiva and the orbit in one patient, and in the skin of eyelids in one patient. Imaging examinations showed the tumors were of irregular soft tissue density. MRI showed the tumors were close to the extraocular muscles or ectocinerea. Centroblastic morphology was present in 21 specimens, and 2 specimens had immunoblastic morphology. Six patients were of germinal center B cell-like (GCB) type, and 17 were of non-GCB type. There were 2 patients with double expression of cellular-myelocytomatosis viral oncogene and B-cell lymphoma 2. The follow-up time ranged from 25 to 156 months, and the median follow-up time was 48 months. Five of them had primary OA-DLBCL, and 13 patients had secondary OA-DLBCL. Five patients were at the Ann Arbor clinical stage ⅠE, one was at stage ⅢE, and 12 were at stage ⅣE. During the follow-up, 8 patients survived, and 10 died. The 1-, 3-, and 5-year OS rates were 88.9%, 71.4% and 41.7%, respectively. Log-rank analysis indicated that the Ann Arbor clinical stage and age were related to the OS of OA-DLBCL (χ²=7.448, 8.804; both P<0.01). The gender, tumor size, molecular typing, Ki-67 index, and bone invasion were not related to the OS of OA-DLBCL (all P>0.05). Conclusions: OA-DLBCL mainly occurrs in the elderly population, unilaterally, and in the orbit. Most molecular types are non-GCB subtypes. The Ann Arbor clinical stage and age are associated with prognosis. (Chin J Ophthalmol, 2021, 57: 366-371).


Asunto(s)
Aparato Lagrimal , Linfoma de Células B Grandes Difuso , Anciano , Femenino , Humanos , Linfoma de Células B Grandes Difuso/diagnóstico por imagen , Masculino , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia
9.
Phys Rev Lett ; 124(12): 126803, 2020 Mar 27.
Artículo en Inglés | MEDLINE | ID: mdl-32281854

RESUMEN

We study the transport of surface electrons on superfluid helium through a microchannel structure in which the charge flow splits into two branches, one flowing straight and one turned at 90°. According to Ohm's law, an equal number of charges should flow into each branch. However, when the electrons are dressed by surface excitations (ripplons) to form polaronlike particles with sufficiently large effective mass, all the charge follows the straight path due to momentum conservation. This surface-wave induced transport is analogous to the motion of electrons coupled to surface acoustic waves in semiconductor 2DEGs.

10.
Zhonghua Yan Ke Za Zhi ; 56(5): 364-369, 2020 May 11.
Artículo en Zh | MEDLINE | ID: mdl-32450669

RESUMEN

Objective: To analyze the pathological classification and age distribution of primary neoplasms of the lacrimal drainage system. Methods: Retrospective case series study. A total of 64 patients (65 eyes) were diagnosed with primary neoplasms of the lacrimal drainage system and received surgery at Tianjin Eye Hospital from January 2006 to December 2016. All the clinical data of the patients were analyzed, including gender, diseased eye, age, clinical manifestations, composition of benign and malignant masses, and prognosis. The histopathological composition and age distribution of patients with primary lacrimal mass, lacrimal duct mass and lacrimal sac mass were analyzed according to the different diseased sites. Results: Twenty-three patients (24 eyes) were male, and 41 patients (41 eyes) were female. The right eye was involved in 36 patients, the left eye in 27 patients, and both eyes in one patient. The age at diagnosis ranged from 12 to 78 years old [mean, (46±4) years]. The course of disease was (13.1±4.2) months, ranging from 1 month to 7 years. The chief complaint was tear discharge in 43 patients, tumor in 19 patients, and abscess discharge in 2 patients. There were 29 patients with angular displacement and 21 patients with swelling pain. There were 51 patients with benign lesions, 4 with borderline lesions, and 9 with malignantlesions. These neoplasms consisted of primary peripunctal neoplasms in 17 patients, primary canalicular neoplasms in 2 patients, and primary lacrimal sac neoplasms in 45 patients. All primary peripunctal neoplasms (17 cases) were benign, among which nevi (10 cases) occupied the first place. All primary canalicular neoplasms (2 cases) were benign, there were 1 case of epidermoid cysts and 1 case of degenerative disease. Among the primary benign lacrimal sac masses (32 cases), mucous epithelial cysts (9 cases), dermoid cysts (6 cases), and epidermoid cysts (6 cases) occupied the first three places. Among the primary borderlin lacrimal sac masses (4 cases), there were 2 cases of giant cell tumor of soft tissue, 1 case of solitary fibrous tumor, and 1 case of inflammatory myofibroblastic tumor. The primary malignant mass of lacrimal sac (9 cases) was dominated by squamous cell carcinoma (3 cases). In terms of age distribution, the patients with primary peripunctal mass were mainly in the group of 40-59 years old (14 cases). The primary benign mass of lacrimal sac mainly occurred in the group of less than 40 years old (15 cases) and the group of 40-59 years old (11 cases). The patients with primary lacrimal sac borderline and malignant masses were all in the groups of over 40 years old. A total of 49 patients were followed up for 27 months to 16 years. The average follow-up time was (57.2±3.8) months. Lacrimal sac transitional cell carcinoma relapsed 7 months after surgery in one patient, and lacrimal sac melanoma relapsed 1 year after surgery in one patient. The patients did not relapse in 24 months and 38 months after surgery respectively. There were no recurrence of other cases. Conclusions: Primary peripunctal neoplasms are mostly characterized with benign lesions, among which nevi are most common. Mucous epithelial cysts, epidermoid cysts, and dermoid cysts are the major benign lacrimal sac neoplasms. Squamous cell carcinomas are the most common malignant lacrimal sac neoplasms. The malignant tumor of lacrimal sac often occurs in the middle-aged and elderly patients. (Chin J Ophthalmol, 2020, 56: 364-369).


Asunto(s)
Quiste Dermoide , Neoplasias del Ojo , Enfermedades del Aparato Lagrimal , Aparato Lagrimal , Adolescente , Adulto , Anciano , Niño , Neoplasias del Ojo/complicaciones , Neoplasias del Ojo/diagnóstico , Neoplasias del Ojo/terapia , Femenino , Humanos , Enfermedades del Aparato Lagrimal/complicaciones , Enfermedades del Aparato Lagrimal/diagnóstico , Enfermedades del Aparato Lagrimal/terapia , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Estudios Retrospectivos , Adulto Joven
11.
Exp Eye Res ; 178: 15-26, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-30218651

RESUMEN

Inherited retinal disease (IRD) affects about 1 in 3000 to 1 in 5000 individuals and is now believed to be the most common cause of blindness registration in developed countries. Until recently, the management of such conditions had been exclusively supportive. However, advances in molecular biology and medical engineering have now seen the rise of a variety of approaches to restore vision in patients with IRDs. Optogenetic approaches are primarily aimed at rendering secondary and tertiary neurons of the retina light-sensitive in order to replace degenerate or dysfunctional photoreceptors. Such approaches are attractive because they provide a "causative gene-independent" strategy, which may prove suitable for a variety of patients with IRD. We discuss theoretical and practical considerations in the selection of optogenetic molecules, vectors, surgical approaches and review previous trials of optogenetics for vision restoration. Optogenetic approaches to vision restoration have yielded promising results in pre-clinical trials and a phase I/II clinical trial is currently underway (ClinicalTrials.gov NCT02556736). Despite the significant inroads made in recent years, the ideal optogenetic molecule, vector and surgical approach have yet to be established.


Asunto(s)
Terapia Genética , Retinitis Pigmentosa/terapia , Trastornos de la Visión/rehabilitación , Enfermedades Hereditarias del Ojo/terapia , Vectores Genéticos , Humanos
12.
Zhonghua Gan Zang Bing Za Zhi ; 27(2): 149-152, 2019 Feb 20.
Artículo en Zh | MEDLINE | ID: mdl-30818923

RESUMEN

Hepatic fibrosis is a wound healing and scar repair reaction after liver injury, and is a common pathway for various chronic liver diseases. Activation and proliferation of hepatic stellate cells are the key links in the occurrence and development of hepatic fibrosis. Recent studies have shown that long non-coding RNAs are involved in regulating the activation, proliferation and apoptosis of hepatic stellate cells. Thus, probing its mechanism of action will provide a new strategy for the diagnosis, treatment and prognosis in liver fibrosis.


Asunto(s)
Cirrosis Hepática , ARN Largo no Codificante , Apoptosis , Investigación Biomédica/tendencias , Células Estrelladas Hepáticas , Humanos , Hígado
13.
Zhonghua Yan Ke Za Zhi ; 55(11): 834-841, 2019 Nov 11.
Artículo en Zh | MEDLINE | ID: mdl-31715680

RESUMEN

Objective: To summarize the clinical and pathological characteristics of IgG4-related ophthalmic disease (IgG4-ROD) involving the lacrimal gland. Methods: A retrospective case series study. Forty cases (56 eyes) of lacrimal gland lesions were collected in Tianjin Eye Hospital from January 2003 to January 2018 and confirmed by histopathology as lymphocyte and plasma cell infiltration with fibrosis of lacrimal gland tissue, excluding lymphoma, epithelial tumor, mesenchymal tumor and metastasis tumor. The clinical manifestations, serological and imaging examination of the patients were analyzed. Meanwhile, HE staining and immunohistochemical staining of IgG and IgG4 were performed on the pathological specimens. According to the diagnostic criteria, the cases were divided into the IgG4-ROD group and the non-IgG4-ROD group. The clinical and pathological characteristics of the two groups were statistically analyzed by Pearson chi-square and signed-rank test. Results: In the 40 cases (56 eyes), there were 15 cases (25 eyes) of IgG4-ROD and 25 cases (31 eyes) of non-IgG4-ROD. Statistically significant differences were observed between the two groups in the clinical and pathological characteristics (all P<0.05). About the distribution of eyes position, there were 10 binocular cases and 5 monocular cases in the IgG4-ROD group, and 6 binocular cases and 19 monocular cases in non-IgG4-ROD group (χ2=7.111).There were 21 eyes in the IgG4-ROD group and 5 eyes in the non-IgG4-ROD group about ptosis (χ2=25.631), 4 eyes in the IgG4-ROD group and 21 eyes in the non-IgG4-ROD group about ocular protrusion (χ2=14.992), 23 eyes in the IgG4-ROD group and 15 eyes in the non-IgG4-ROD group about the clear boundary of the tumor (χ2=12.069), 4 eyes in the IgG4-ROD group and 18 eyes in the non-IgG4-ROD group about the involvement of other orbital tissues (χ2=10.266) and 7 cases in the IgG4-ROD group and 3 cases in the non-IgG4-ROD group about the association with other systemic diseases (χ2=6.009). Compared with the non-IgG4-ROD group, the IgG4-ROD group had a heavier lymphocyte and plasma cell infiltration (+++,++,+; 10, 4, 1 vs. 6, 5, 12 eyes, Z=-3.153), and more lymphoid follicles (+++,++,+; 3, 6, 4 vs. 1, 2, 7 eyes, Z=-3.339), interstitial fibrosis was mostly striate (10 vs. 5 eyes, χ2=8.711), and there were a large number of IgG4+ plasma cells [96 (67, 135) vs. 4 (0, 12) cells per high power field, Z=-5.271] and ratio of IgG4+ plasma cells/IgG+ plasma cells [0.570 (0.500, 0.754) vs. 0.046 (0.000, 0.143), Z=-5.268, all P<0.05). Among the 10 cases of IgG4-ROD with serological examination, 9 cases showed elevated serum in IgG and IgG4. The ultrasonography and CT findings showed the lacrimal gland lesions in the IgG4-ROD group were mostly spindle or kidney shaped with clear boundaries, while the lesions in non-IgG4-ROD were mostly round or irregular with unclear boundaries. Conclusions: The lacrimal gland lesions of IgG4-ROD are characterized by bilaterally spindle or kidney shaped enlargement with clear boundaries. They are more associates with other systemic diseases. The pathological characteristics are a large number of IgG4+ plasma cells infiltration among the lacrimal gland tissue, interstitial striate fibrosis and a large number of lymphoid follicles. (Chin J Ophthalmol, 2019, 55: 834-841).


Asunto(s)
Enfermedad Relacionada con Inmunoglobulina G4/fisiopatología , Enfermedades del Aparato Lagrimal/fisiopatología , Humanos , Aparato Lagrimal/fisiopatología , Células Plasmáticas/patología , Estudios Retrospectivos
14.
Zhonghua Yan Ke Za Zhi ; 55(7): 531-535, 2019 Jul 11.
Artículo en Zh | MEDLINE | ID: mdl-31288357

RESUMEN

Objective: To describe the clinicopathological features of conjunctival actinic keratosis (AK) and relation to the infection of human papillomavirus (HPV). Method: Retrospective case series study. Eighteen cases (18 eyes) of conjunctival AK were obtained in Tianjin Eye Hospital and Institute (2005-2018). The clinical and histopathological features were studied. HPV was detected by a modified general primer HPV polymerase chain reaction (PCR) system in all formalin-fixed, paraffin-embedded specimens. Results: The male to female ratio was 5∶1. The mean age at diagnosis was 60 years (range: 43-79 years). Sixteen cases were located in the nasal interpalpebral region, and two cases were located in the temporal interpalpebral region. All cases were located in corneal limbus, and the mean distance of corneal invasion was 2 mm (range, 1-4 mm). The mean diameter was 4.6 mm (range, 2.0-8.0 mm). Clinically, most lesions (16 cases) appeared as a white or milky, flat plaque with clear borderline and conjunctival hyperemia; a few lesions (2 cases) showed a brown-black mass, partially white. Pathologically, conjunctival AK was a proliferation of epithelium with prominent parakeratosis or hyperkeratosis, stratum spinosm thickening and basal cell proliferation. Many AKs show solar elastosis and a mild inflammatory infiltrate of lymphocytes and plasma cells in the stroma. Most lesions (15 cases) were hypertrophic type, two cases were pigmented type, and one case was acantholytic type. HPV was negative in 18 cases. All case were removed by complete surgical excision. The rage of follow-up period was 1.0-10.4 years, ten cases were recorded, and no case recurred after surgical excision. Conclusions: Conjunctival AK is epithelial precancerous lesion that occurs in the keratoconjunctival margin. HPV infection might not be a causative factor in conjunctival AK. (Chin J Ophthalmol, 2019, 55: 531-535).


Asunto(s)
Queratosis Actínica , Papillomaviridae , Infecciones por Papillomavirus , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Estudios Retrospectivos
15.
Lupus ; 27(11): 1828-1837, 2018 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-30134759

RESUMEN

Background Cutaneous lupus erythematosus (CLE) includes a broad range of dermatologic manifestations. Periorbital involvement, however, is a relatively rare clinical presentation of CLE. Objectives This clinical study aimed to investigate the characteristics of this unique presentation of CLE in tertiary medical centers. Methods We enrolled patients with periorbital erythema and swelling as the presenting sign of lupus erythematosus, from January 2003 to November 2017, using the data of 553 pathologically proven CLE cases from the registration database of the Chang Gung Memorial Hospitals in Taiwan. Results We enrolled a total of 25 patients. The mean age was 46.7 years and 68% of the patients were female. Most of the patients (84.0%) presented with unilateral involvement, with the left orbit involved in 15 patients (60%); the upper eyelid was the most frequently involved (72%). Mean duration between the onset of clinical manifestations and the diagnosis of CLE was approximately 59 weeks. Nineteen patients had been previously misdiagnosed. All patients had features compatible with CLE on histopathological examination. In contrast, laboratory analysis of the autoimmune profile often revealed negative results, including those for antinuclear antibodies (25%). Notably, anti-SSA/SSB (45.5%) showed the highest positive rate. During follow-up, six patients developed systemic lupus erythematosus (SLE) and two patients developed Sjögren syndrome. Conclusions The diagnosis of CLE presenting as periorbital erythema and swelling is often delayed because of clinical mimicry and the high proportion of negative results on autoantibody tests. Increased clinical suspicion and prompt histopathological examination are crucial for early diagnosis. Moreover, one-fourth of the patients ultimately developed SLE, which highlights the importance of clinical awareness.


Asunto(s)
Edema/patología , Eritema/patología , Lupus Eritematoso Cutáneo/diagnóstico , Lupus Eritematoso Cutáneo/patología , Piel/patología , Adulto , Anciano , Anticuerpos Antinucleares/análisis , Femenino , Humanos , Masculino , Persona de Mediana Edad , Taiwán , Centros de Atención Terciaria , Adulto Joven
16.
Zhonghua Yan Ke Za Zhi ; 54(9): 661-664, 2018 Sep 11.
Artículo en Zh | MEDLINE | ID: mdl-30220180

RESUMEN

Objective: To summarize the clinical pathologic characteristics of conjunctival osseous choristoma. Methods: Retrospective case series study. Six cases of conjunctival osseous choristoma between January 2009 and June 2016 from Tianjin Eye Hosptial were reviewed retrospectively. The clinical features, CT characteristics and histological characteristics were analyzed. Results: The 6 cases includes 3 male patients and 3 females. The age of the patients ranged from 4 to 20 years. Three cases involved with the right eye and the other 3 involved with the left eye. Major clinical feature of the patient is the flat uplift tumor in the superior temporal quadrant of the conjunctiva. CT features of conjunctival osseous choristoma revealed a oval calcium spot of eyeball surface. All the patients received surgery. Pathological examination showed that the tumor is composed of bone tissue which was surrounded by fibrous tissue, and Haversian canals are present in compact bone tissue. Five patients were followed for 23 months to 8 years. None of the patients relapsed after operation. Conclusions: The major feature of conjuncital osseous choristoma is a tumor in the superior temporal quadrant of conjunctiva. Imaging examination reveals a oval calcium spot of eyeball surface. Pathological examination reveals that the osseous tumor is composed of compact bone tissue which was surrounded by fibrous tissue.Haversian canals are present in compact bone tissue.Diagnosis is dependent on histopathological examination. It is not prone to relapse after complete resection surgery. (Chin J Ophthalmol, 2018, 54: 661-664).


Asunto(s)
Coristoma , Neoplasias de la Conjuntiva , Adolescente , Huesos/patología , Niño , Preescolar , Coristoma/diagnóstico , Coristoma/patología , Neoplasias de la Conjuntiva/diagnóstico , Neoplasias de la Conjuntiva/patología , Femenino , Humanos , Masculino , Recurrencia , Estudios Retrospectivos , Adulto Joven
17.
Zhonghua Yan Ke Za Zhi ; 54(9): 665-670, 2018 Sep 11.
Artículo en Zh | MEDLINE | ID: mdl-30220181

RESUMEN

Objective: To investigate the clinical features, imaging features, diagnoses and therapeutic regimens of orbital ectopic meningiomas. Methods: A retrospective study. All the clinical data of 10 patients (10 eyes) with orbital ectopic meningiomas were analyzed retrospectively from August 1999 to October 2016. They included imaging data such as color Doppler ultrasound, CT and MRI, and information of diagnosis, pathology, therapeutic regimen and prognosis. Results: The age of orbital ectopic meningiomas was 7 to 68 years old. There were 4 males and 6 females. The clinical manifestations were mainly with swollen eyelid (8 cases), exophthalmos (7 cases), visual impairment (2 cases) and accidentally found a mass in the orbit with physical examination(2 cases). The tumor was located in (5 cases) or outside (5 cases) the muscular funnel of the orbit. The imaging features of CT included the irregular shape, unclear border, and extraocular muscular adhesions (10 cases). T1 weighted image of MRI showed low and medium signals and T2 weighted image showed medium and high signals(8 cases). There was no definite diagnosis before surgery. Ten patients were treated with surgery, and it was confirmed intraoperatively that the tumors were not adjacent to the optic nerve and orbital periosteum. The pathological diagnoses were mostly epithelial meningiomas (9 cases). There were no significant changes in visual acuity, but temporary eye movement disorders occurred postoperatively (10 cases). The patients were followed for 6 months to 6 years. Two cases underwent recurrent at 3 months and 2 years after operation, and received treatment of γ-knife radiation therapy. The tumor reduced after γ-knife radiotherapy, and had no change in 2 years and 6 years, respectively. Conclusions: As a kind of rare orbital tumors, it is difficult to diagnose orbital ectopic meningiomas accurately. The clinical features and imaging findings can help to detect the disease, but lacking the characteristics of optic nerve sheath meningiomas and periosteal meningiomas. Surgical resection could achieve a good prognosis without visual impairment. If the tumor recurs after pathological diagnosis, γ-knife conformal radiation therapy may still be effective for tumor recurrence. (Chin J Ophthalmol, 2018, 54: 665-670).


Asunto(s)
Neoplasias Meníngeas , Meningioma , Neoplasias Orbitales , Adolescente , Adulto , Anciano , Niño , Coristoma , Femenino , Humanos , Masculino , Neoplasias Meníngeas/diagnóstico , Neoplasias Meníngeas/terapia , Meningioma/diagnóstico , Meningioma/terapia , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Neoplasias Orbitales/diagnóstico , Neoplasias Orbitales/terapia , Estudios Retrospectivos , Adulto Joven
18.
Allergol Immunopathol (Madr) ; 44(2): 149-59, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-26318416

RESUMEN

BACKGROUND: Farnesol, a natural sesquiterpene alcohol in essential oils, was found to have potential for alleviating massive inflammation, oxidative stress and lung injury. However, effects of farnesol supplementation on allergic asthma remain unclear. OBJECTIVES: To clarify the puzzle, this work investigates the effects of farnesol on allergic asthma using an ovalbumin (OVA)-sensitised and challenged mouse model. METHODS: Farnesol was administered to OVA-sensitised and challenged mice for 5 weeks. Three farnesol doses, namely 5, 25 and 100mg farnesol/kg BW/day, non-sensitised control, dietary control, and positive control (dexamethasone 3mg/kg BW by gavage) were included. Sera and bronchoalveolar lavage fluids from the experimental mice were collected to measure farnesol concentrations, serum lipid profiles, antibody titres, differential cell counts or Th1/Th2 cytokines levels. RESULTS: The results showed that farnesol supplementation increased serum farnesol concentration dose-dependently, significantly increased (P<0.05) OVA-specific IgG2a/IgE antibody titre ratios, but decreased total IgE levels. Farnesol supplementation markedly reversed the aberrated LDL-c/HDL-c and HDL-c/TC ratios in the sera of asthmatic mice, suggesting that farnesol supplementation might ameliorate serum lipid profiles in the OVA-sensitised and challenged mice. CONCLUSION: Our results evidenced that farnesol supplementation might improve serum allergic antibody titres and lipid profiles in asthmatic mice.


Asunto(s)
Asma/dietoterapia , Farnesol/uso terapéutico , Aceites Volátiles/uso terapéutico , Animales , Asma/inmunología , Citocinas/metabolismo , Suplementos Dietéticos , Modelos Animales de Enfermedad , Farnesol/química , Femenino , Humanos , Inmunidad Humoral/efectos de los fármacos , Inmunoglobulina E/sangre , Metabolismo de los Lípidos/efectos de los fármacos , Lípidos/sangre , Ratones , Ratones Endogámicos BALB C , Ovalbúmina/inmunología , Sesquiterpenos/química , Balance Th1 - Th2/efectos de los fármacos
19.
Genet Mol Res ; 15(2)2016 Apr 27.
Artículo en Inglés | MEDLINE | ID: mdl-27173263

RESUMEN

The goal of the current study was to investigate the effects of pollution on aquatic organisms in the Yongcheng coal mine subsidence area. Crucian carp (Carassius auratus) were collected from Yongcheng natural fishpond (experimental group) and Tianmu Lake (control group), and the spleens were isolated for analysis. Subsequently, histological changes, DNA damage, and antioxidant enzyme activity were assessed. The result showed that there were more vacuoles, widened blood sinus cavities, increased partial dot necrosis, and a larger number of brown-yellow nodules in splenic sections stained with hematoxylin and eosin in the experimental group than in the control group. Additionally, it was not easy to distinguish red pulp from white pulp in the experimental group. The antioxidant enzyme activity in the experimental group was significantly lower than that in the control group (P < 0.01). Comet assay results showed varying degrees of tailing and DNA chain breaks in the experimental group, and further analysis demonstrated that the tail length and tail moment were significantly increased compared to those in the control group (P < 0.01). These results suggest that the spleen antioxidant defense system was severely damaged in crucian carp from the Yongcheng coal mine subsidence area.


Asunto(s)
Minas de Carbón , Proteínas de Peces/metabolismo , Peroxidasas/metabolismo , Bazo/efectos de los fármacos , Contaminación del Agua/efectos adversos , Animales , Carpas , China , Daño del ADN , Necrosis , Bazo/enzimología , Bazo/patología
20.
Zhonghua Bing Li Xue Za Zhi ; 45(12): 825-830, 2016 Dec 08.
Artículo en Zh | MEDLINE | ID: mdl-28056296

RESUMEN

Objective: To investigate the relationship between expression of PDGFRA/CMYC and clinicopathologic features of extranodal NK/T-cell lymphoma. Methods: Fifty-four cases of extranodal NK/T-cell lymphoma were included in the study.Immunohistochemistry was used to detect the expression of CD20, CD2, CD3, CD56, TIA1, GrB, Ki-67, PDGFRA and CMYC.In situ hybridization was performed to detect the presence of EBV encoded small RNA (EBER). Fifty cases of nasopharyngeal mucosal lymphoid tissue hyperplasia were used as normal control. Results: Among 54 cases of ENKTL, CD2, CD3, GrB, and TIA1 were expressed in all the tumors. CD56 was expressed in 47 cases (81.0%) and CD20 was not detectable in any cases. Ki-67 proliferative index expression of > 60% was found in 45 cases (83.3%). In situ hybridization for EBER was positive in all cases (100%). The positive expression rates of PDGFRA and CMYC in extranodal NK/T-cell lymphomas were 51.9%(28/54) and 53.7%(29/54), respectively, much higher than those in nasopharyngeal mucosal lymphoid tissue hyperplasia (0, P<0.05). There was a positive correlation between PDGFRA and CMYC (r=0.295, P<0.05). The expression of CMYC was correlated with clinical efficacy (P<0.05), but not with gender, age, Ann Arbor stage, B symptoms and therapeutic regimen (all P>0.05). The expression of PDGFRA was correlated with B symptoms (P<0.05), while not with gender, age, Ann Arbor stage, therapeutic regimen and clinical efficacy (all P>0.05). The co-expression of PDGFRA and CMYC was not correlated with gender, age, Ann Arbor stage, B symptoms, therapeutic regimen and clinical efficacy (P>0.05). Univariate analysis showed that the stage, clinical efficacy, CMYC protein and the co-expression of PDGFRA and CMYC were significantly correlated with the prognosis. The overall survival of the patients with CMYC positive expression was shorter than of that of the patients with negative expression (P<0.05). Multivariable Cox regression analysis further confirmed that clinical stage, CMYC protein expression, and the co-expression of PDGFRA and CMYC were independent prognostic factors in patients with extranodal NK/T-cell lymphoma. Conclusion: CMYC protein, and the co-expression of PDGFRA and CMYC can be as an independent prognostic factor in patients with extranodal NK/T-cell lymphoma and influence the prognosis of patients.


Asunto(s)
Linfoma Extranodal de Células NK-T/metabolismo , Neoplasias Nasofaríngeas/metabolismo , Proteínas de Neoplasias/metabolismo , Factor de Crecimiento Derivado de Plaquetas/metabolismo , Proteínas Proto-Oncogénicas c-myc/metabolismo , Factores de Edad , Antígenos CD/metabolismo , Antígeno CD56 , Femenino , Herpesvirus Humano 4/aislamiento & purificación , Humanos , Inmunohistoquímica , Hibridación in Situ , Linfoma Extranodal de Células NK-T/patología , Masculino , Pronóstico , Factores Sexuales
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