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1.
Artigo em Chinês | MEDLINE | ID: mdl-38561259

RESUMO

Objective: To investigate the clinical characteristics and voice outcomes after laryngeal microsurgery for vocal fold epidermoid cysts coexisting with sulcus vocalis. Methods: The clinical data of 115 vocal fold epidermoid cysts coexisting with sulcus vocalis patients in Shandong provincial ENT hospital, were retrospectively analyzed, including 49 males and 66 females, aged 17-70 years old, and the duration of hoarseness ranged from 6 months to 30 years. All patients underwent surgery through suspension laryngoscope and microscope under general anestgesia. Ninety-four patients were treated with microflap excision of sulcus vocalis, cyst wall, and contents.And 21 patients that occulted with mucosal bridges were applied mucosal bridges resection (2 cases) and mucosal bridges reconstruction (19 cases) respectively. Videolaryngoscopy, subjective voice evaluation (GRBAS), objective voice evaluation, and Voice Handicap Index(VHI) were performed before and after surgery. All patients underwent histopathologic examination and follow-up after the procedure. The preoperative acoustic parameters of patients with vocal fold epidermoid cysts coexisting with sulcus vocalis were compared with those of vocal fold mucus retention cysts and simple vocal fold epidermoid cysts by independent samples t-test. The patients were compared by paired t-test for preoperative and postoperative parameters. Results: Significant reduction or lack of mucosal waves were shown via videolaryngostroboscopy in all 115 cases.In addition, vascular changes including dilation, tortuousness, increased branches, and abrupt direction change were shown on the cystic area. Eighty-one patients were detected cysts and/or sulcus vocalis by preoperative laryngoscopy, and intraoperative microscopic findings in the remaining 34 patients. The intraoperative microscopic examination revealed a focal pouch-like deficit plunging into the vocal ligament or muscle. The deep surface of the mucosal bridges was sulcus vocalis, and that in 89 cysts was lined with caseous content. Histopathology demonstrated a cystic cavity structure lined with squamous epithelium and caseous keratin desquamation inside the cystic cavity. Four of 115 patients were lost at follow-up and excluded from the analysis of voice outcomes after surgery. There was no significant mucosal wave and the voice quality in all but 14 patients 1month after surgery. Except for the fundamental frequency and noise harmonic ratio, all other voice parameters[ G, R, B, A, VHI-10, jitter, shimmer, maximum phonatory time (MPT) ]showed a significant improvement 3 months after surgery(t=15.82, 20.82, 17.61, 7.30, 38.88, 7.84, 5.88, -6.26, respectively, P<0.05). Then mucosal waves and the voice quality were gradually improved and became steady in 6 months after surgery. The subjective and objective voice parameters[G, R, B, A, VHI-10, jitter, shimmer, noise to harmonic ratio(NHR), MPT], except for the fundamental frequency, were all significantly improved(t=23.47, 25.79, 18.37, 9.84, 54.45, 10.68, 8.07, 3.24, -9.08, respectively, P<0.05). In addition, there were 2 patients with no significant improvement after the operation. Steady function with no complications was observed during the 12 months (up to 3 years in 34 patients) follow-up period in 111 patients. Conclusion: Ruptured vocal fold epidermoid cysts can result in sulcus vocalis and mucosal bridges. Characteristics changes in preoperative videolaryngoscopy are effective diagnostic tools. The complete excision of the cyst wall and repair of the lamina propria can lead to satisfactory long-term effects.


Assuntos
Cisto Epidérmico , Doenças da Laringe , Masculino , Feminino , Humanos , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Prega Vocal/patologia , Cisto Epidérmico/complicações , Cisto Epidérmico/cirurgia , Cisto Epidérmico/patologia , Estudos Retrospectivos , Doenças da Laringe/cirurgia , Doenças da Laringe/patologia , Qualidade da Voz , Resultado do Tratamento
2.
Zhonghua Yan Ke Za Zhi ; 60(3): 250-256, 2024 Mar 11.
Artigo em Chinês | MEDLINE | ID: mdl-38462373

RESUMO

Objective: To analyze the changes in optical coherence tomography angiography(OCTA)-related parameters before and after idiopathic macular hole (IMH) internal limiting membrane (ILM) flap inversion surgery and assess their impact on retinal sensitivity. Methods: A retrospective case series study was conducted, collecting clinical data of 30 patients (30 eyes) diagnosed with IMH who underwent vitrectomy combined with ILM flap inversion surgery at Shanxi Eye Hospital, affiliated with Shanxi Medical University, between January 2020 and December 2021. Visual acuity and best-corrected visual acuity were examined preoperatively and at 1, 3, and 6 months postoperatively. Microperimetry measured retinal sensitivity (RS), and OCTA measured retinal thickness (RT) as well as vessel density (VD) in the superficial capillary plexus (SCP) and deep capillary plexus (DCP). Statistical analysis was performed using t-tests, repeated measures analysis of variance, and Pearson correlation analysis. Results: Thirty patients with IMH (30 eyes) were included, with 3 males (3 eyes) and 27 females (27 eyes). The mean age was (62.5±3.0) years, and the follow-up time was (96.3±1.3) days, with a 100% closure rate of macular holes postoperatively. RT in the macular was significantly lower at 1, 3, and 6 months postoperatively compared to preoperative values (F=46.21, P<0.001). The RT in the upper macular region showed statistically significant differences at different time points (P<0.001). VD in the SCP layer showed no significant differences between the upper and lower macular regions at various time points (F=3.21, P=0.601). VD in the upper region of the DCP layer increased at 1, 3, and 6 months postoperatively (P<0.001). RS in the macular was higher at 1, 3, and 6 months postoperatively compared to preoperative values (F=52.01, P<0.001). RS in the lower macular region increased at 3 and 6 months postoperatively (P<0.001), while in the upper region, it increased only at 6 months postoperatively (P<0.001). There was a positive correlation between RS and RT at 1 and 3 months postoperatively, but not at 6 months postoperatively in the upper macular region (r=0.40, P=0.071). In the lower macular region, there was a positive correlation between RS and RT at 1 and 3 months postoperatively (P<0.001). There was no correlation between RS in the upper macular region at 6 months postoperatively and preoperative RT (r=0.43, P=0.072), but there was a positive correlation with RT at 3 months postoperatively (r=0.58, P=0.041). Conclusions: After idiopathic macular hole internal limiting membrane flap inversion surgery, the OCTA-related parameters have changed. There are transient changes in deep vascular parameters and thinning of the retinal layers at the ILM inversion site, leading to decreased sensitivity.


Assuntos
Perfurações Retinianas , Masculino , Feminino , Humanos , Pessoa de Meia-Idade , Idoso , Perfurações Retinianas/cirurgia , Perfurações Retinianas/diagnóstico , Estudos Retrospectivos , Tomografia de Coerência Óptica , Retina , Vitrectomia/métodos
3.
Zhonghua Xue Ye Xue Za Zhi ; 44(7): 578-581, 2023 Jul 14.
Artigo em Chinês | MEDLINE | ID: mdl-37749039

RESUMO

Objective: To evaluate the clinical outcomes and safety of haploidentical hematopoietic stem cell transplantation (haplo-HSCT) using a conditioning regimen based on total body irradiation (TBI) and rabbit anti-human thymocyte globulin (rATG) in the management of chemotherapy-resistant advanced peripheral T-cell lymphoma (PTCL) . Methods: Clinical data of 11 patients with chemotherapy-resistant advanced PTCL who underwent haplo-HSCT with a TBI+rATG-based conditioning regimen at the Department of Hematology, Shanghai Liquan Hospital and Shanghai Zhaxin Integrated Traditional Chinese and Western Medicine Hospital, from September 2019 to December 2022 were retrospectively analyzed. Results: ①Among the 11 patients (six males and five females), with a median age of 40 years (range: 22-58 years), there were six cases of PTCL, not otherwise specified (PTCL-NOS), three cases of angioimmunoblastic T-cell lymphoma (AITL), one case of large-cell transformation of mycosis fungoides (MF-LCT), and one case of T-cell large granular lymphocytic leukemia (T-LGLL). According to the Lugano staging system, all patients were in stage Ⅲ or Ⅳ, and eight patients had B symptoms. Before transplantation, the median number of prior lines of chemotherapy was 4 (range: 2-10), and all patients had progressive disease (PD). The median time from diagnosis to transplantation was 17 months (range: 6-36 months). ②The conditioning regimen consisted of a TBI dose of 10 Gy, administered at 2 Gy on day -8 and 4 Gy from day -7 to day -6, rATG was administered at a daily dose of 2.5 mg/kg from day -5 to day -2. Etoposide (VP-16) was given at a dose of 15 mg/kg/d from day -5 to day -4, while cyclophosphamide (CTX) was administered at a dose of 50 mg/kg/d from day -3 to day -2. In patients with central nervous system involvement, etoposide and cyclophosphamide were replaced with thiotepa (TT) at a dose of 5 mg/kg/d from day -5 to day -4. Additionally, cytarabine (Ara-C) was added at a dose of 2.0 g/m(2) twice a day from day -3 to day -2 into the conditioning. ③Successful engraftment was achieved in all patients, with a median time to neutrophil engraftment of 14.5 d (range: 11-16 d) and a median time to platelet engraftment of 13 days (range: 8-18 days). Acute graft-versus-host disease (aGVHD) occurred in one patient (grade Ⅰ-Ⅱ), and another patient experienced grade Ⅲ-Ⅳ aGVHD. Among the eight survivors, four developed chronic GVHD (cGVHD). ④Post-transplantation, nine patients achieved complete response (CR). ⑤Hematopoietic suppression occurred in all patients after conditioning, with three experiencing diarrhea, four developing mucositis, three exhibiting elevated transaminase/bilirubin levels, and seven developing infectious complications. These non-hematologic adverse events were effectively managed. ⑥At one year post-transplantation, the non-relapse mortality (NRM) was (22.5±14.0) %, the cumulative incidence of relapse (CIR) was (20.2±12.7) %, and overall survival (OS) rate was (72.7±13.4) %, and disease-free survival (DFS) rate was (63.6±14.5) % . Conclusion: TBI+rATG-based conditioning regimen for haplo-HSCT is an effective and safe treatment approach for patients with chemotherapy-resistant advanced PTCL.


Assuntos
Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Linfoma de Células T Periférico , Masculino , Feminino , Humanos , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Etoposídeo , Linfoma de Células T Periférico/tratamento farmacológico , Irradiação Corporal Total/efeitos adversos , Estudos Retrospectivos , China , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Ciclofosfamida , Doença Enxerto-Hospedeiro/etiologia , Doença Enxerto-Hospedeiro/patologia , Condicionamento Pré-Transplante/efeitos adversos
4.
Zhonghua Wei Chang Wai Ke Za Zhi ; 26(9): 847-852, 2023 Sep 25.
Artigo em Chinês | MEDLINE | ID: mdl-37709692

RESUMO

Objective: To examine the clinical value of rapid detection of drug-resistant bacteria by immunochromatography and the effects of rapid detection on the prognosis of patients with severe intra-abdominal infection complicated by carbapenem-resistant Enterobacteriaceae (CRE) bloodstream infection. Methods: This was a retrospective cohort study. We analyzed clinical data of 73 patients with severe abdominal infections with sepsis or septic shock complicated by CRE bloodstream infection admitted to the general surgery department of Jinling Hospital between February 2022 and February 2023. Patients were divided into a colloidal gold immunochromatographic assay (GICA) group (17 patients) and conventional testing group (56 patients) based on whether a GICA for CRE had been performed on the patients' first blood culture sample during the diagnosis and treatment process. There were no statistically significant differences between the GICA and conventional testing groups in age ([55.9±17.3] vs. [47.6±16.4] years), sex ([16 men vs. one woman ] vs. [41 men vs. 15 women]), median Charlson comorbidity index (3.0[2.0,4.0] vs. 3.0[2.0, 4.8]), septic shock (10 vs. 39), or acute kidney injury (8 vs. 40) (all P>0.05). Both groups routinely underwent traditional bacterial identification and drug susceptibility testing. Additionally, patients in the GICA group were tested directly for positive blood cultures using a GICA carbapenemase test kit. The main outcomes were mortality rates on Days 28 and 90 after the first identification of CRE bloodstream infection in both groups. We also compared the microbial clearance rate, duration of hospitalization and intensive care unit stay, and time from onset of CRE bloodstream infection to initiation of targeted and appropriate antibiotics between the two groups. Results: The rate of microbial clearance of bloodstream infection was significantly greater in the GICA group than in the conventional testing group (15/17 vs. 34/56 [60.7%], χ2=4.476, P=0.034), whereas the 28-day mortality tended to be lower in the GICA than conventional testing group [5/17 vs. 44.6% [25/56], χ2=1.250, P=0.264). The 90-day mortality (8/17 vs. 53.6% [30/56], χ2=0.222, P=0.638), median duration of hospitalization (37.0 [18.0, 46.5] days vs. 45.5 [32.2, 64.8] days, Z=-1.867, P=0.062), and median duration of intensive care unit stay (18.0 [6.5, 35.0] days vs. 32.0 [5.0, 51.8] days, Z=-1.251, P=0.209). The median time between the onset of bloodstream infection and administration of antibiotics was 49.0 (38.0, 69.0) hours in the GICA group, which is significantly shorter than the 163.0 (111.8, 190.0) hours in the conventional testing group (Z=-5.731, P<0.001). The median time between the onset of bloodstream infection and administration of appropriate antibiotics was 40.0 (34.0, 80.0) hours in the GICA group, which is shorter than in the conventional testing group (68.0 [38.2, 118.8]) hours; however, this difference is not statistically significant (Z=-1.686, P=0.093). Conclusions: GICA can provide information on carbapenemase- producing pathogens faster than traditional drug sensitivity testing, enabling early administration of the optimal antibiotics. The strategy of 'carbapenemase detection first' for managing bacterial infection has the potential to improve prognosis of patients and reduce mortality rate.


Assuntos
Infecções Intra-Abdominais , Mycobacterium tuberculosis , Sepse , Choque Séptico , Masculino , Humanos , Feminino , Testes de Sensibilidade Microbiana , Estudos Retrospectivos , Prognóstico , Infecções Intra-Abdominais/tratamento farmacológico , Antibacterianos/uso terapêutico
5.
Zhonghua Yan Ke Za Zhi ; 59(7): 542-549, 2023 Jul 11.
Artigo em Chinês | MEDLINE | ID: mdl-37408425

RESUMO

Objective: To investigate the clinical effects of the bow-tie adjustable suture technique in managing overcorrection in patients with intermittent exotropia after surgery. Methods: This was a retrospective case series study. Clinical data were collected from children with intermittent exotropia who underwent strabismus correction surgery, including the bow-tie adjustable suture technique and conventional techniques, at the Department of Strabismus and Pediatric Ophthalmology, Shanxi Eye Hospital, from January 2020 to September 2021. Children with postoperative esodeviation≥15 prism diopters (PD) within the first 6 days were treated differently based on the surgical technique and their individual conditions, including suture adjustment and conservative treatment. The overcorrection rate and its changes among different surgical groups, the recovery of ocular alignment and binocular visual function after different treatment methods in children with overcorrection on the sixth postoperative day, and the postoperative complications in different surgical groups were observed. Statistical analysis was performed using independent samples t-test, Wilcoxon rank-sum test, repeated-measures analysis of variance, Bonferroni test, chi-square test, or Fisher's exact probability test, as appropriate. Results: A total of 643 children who underwent intermittent exotropia correction surgery were included in the study. Among them, 325 children underwent the bow-tie adjustable suture technique, with 185 males and 140 females, and the mean age was (9.50±2.69) years. The remaining 318 children underwent conventional techniques, with 176 males and 142 females, and the mean age was (9.90±2.67) years. There were no statistically significant differences in age and gender distribution between the two surgical groups (all P>0.05). On the first postoperative day, among children who underwent the bow-tie adjustable suture technique, 40 had an esodeviation of≥10 PD, resulting in an overcorrection rate of 12.3% (40/325), while among children who underwent conventional techniques, 32 had an esodeviation of≥10 PD, resulting in an overcorrection rate of 10.1% (32/318). On the sixth postoperative day, these rates decreased to 5.5% (18/325) and 3.1% (10/318) in the two groups, respectively. At 1, 6, and 12 months postoperatively, the overcorrection rate in children who underwent the bow-tie adjustable suture technique was 0, while in children who underwent conventional techniques, the overcorrection rate did not show a significant decrease compared to before surgery. The differences between the two surgical groups were statistically significant (all P<0.05). On the sixth postoperative day, among children with an esodeviation of≥15 PD, 13 underwent suture adjustment and 7 received conservative treatment. The results of repeated-measures analysis of variance showed statistically significant differences in near and distance esodeviation angles among children who received different treatment methods (F=145.20, 106.87, both P<0.001), as well as statistically significant differences in near and distance esodeviation angles at different time points within each group of children (F=81.67, 35.09, both P<0.001). There were also significant differences in the trends of change in near and distance esodeviation angles at different time points among children who received different treatment methods (F=79.90, 36.73, both P<0.001). Further pairwise comparisons showed significant differences in near and distance esodeviation angles between the sixth postoperative day and 1, 6, and 12 months postoperatively in children who underwent suture adjustment (all P<0.05), while no statistically significant differences were observed in children who received conservative treatment (all P>0.05). At 12 months postoperatively, among the 13 children who underwent suture adjustment, 12 achieved stereopsis, while among the 7 children who received conservative treatment, all became stereo-blind after removing the prismatic correction. No serious complications occurred in any of the children postoperatively. Conclusion: The proportion of children with intermittent exotropia who achieved orthotropic alignment one year after surgery was relatively low among those who had an overcorrection of≥15 PD on the sixth postoperative day. The bow-tie adjustable suture technique is a simple and effective approach for managing overcorrection in patients with intermittent exotropia. Adjusting the sutures on the sixth postoperative day can reduce the overcorrection rate and is considered a safe and effective method.


Assuntos
Esotropia , Exotropia , Estrabismo , Masculino , Criança , Feminino , Humanos , Exotropia/cirurgia , Resultado do Tratamento , Seguimentos , Esotropia/cirurgia , Estudos Retrospectivos , Acuidade Visual , Procedimentos Cirúrgicos Oftalmológicos/métodos , Estrabismo/cirurgia , Músculos Oculomotores/cirurgia , Visão Binocular , Técnicas de Sutura , Doença Crônica
6.
Zhonghua Yi Xue Za Zhi ; 103(26): 2000-2005, 2023 Jul 11.
Artigo em Chinês | MEDLINE | ID: mdl-37438082

RESUMO

Objective: To investigate the effect of cytokine-induced killer cells (CIK) combined with mFOLFOX6 regimen (fluorouracil+oxaliplatin+calcium folinate) on advanced colorectal cancer. Methods: 161 patients with advanced colon cancer admitted to the Fourth Clinical College of Xinxiang Medical College, Xinxiang Central Hospital of Henan Province from 2019 to 2021 were selected and divided into the study group (mFOLFOX6 regimen chemotherapy+CIK therapy) with 80 cases and the control group (mFOLFOX6 regimen chemotherapy) with 81 cases. The KPS score, median survival time, progression-free survival time, the proportion of Treg in peripheral blood mononuclear cells, the levels of CD4+, CD4+/CD8+, the expression levels of Foxp3 and CD127 mRNA, the quality-of-life score and the occurrence of toxic and side effects were compared between the two groups before and after chemotherapy. Results: The ages of patients in the study group and the control group were (64.8±7.5) and (66.1±7.0) years old, respectively, and the proportions of males were 62.5% (50 cases) and 50.6% (41 cases), respectively (both P values>0.05). After 2 cycles of chemotherapy and at the end of chemotherapy, the proportions of Treg, Foxp3 and CD127 mRNA in the study group were lower than those in the control group [2 cycles of chemotherapy: (4.33±0.86)% vs (4.89±1.20)%, (0.61±0.22) vs (0.73±0.20), (0.58±0.13) vs (0.70±0.15); after chemotherapy: (2.66±0.70)% vs (3.31±0.84)%, (0.43±0.18) vs (0.51±0.16), (0.41±0.10) vs (0.50±0.13)] (all P values<0.05). The KPS scores of the study group were higher than those of the control group [2 cycles of chemotherapy: (68.41±5.41) points vs (65.38±5.11) points; after chemotherapy: (72.08±5.94) points vs (67.95±5.51) points] (all P values<0.05). The median survival time of the study group was (16.8±2.2) months, which was higher than that of the control group [(11.2±1.8) months]. The progression-free survival time of the study group was also higher than that of the control group [(9.5±1.1) months vs (6.4±1.2) months], and the mortality rate was lower than that of the control group [11.3% (9 cases) vs 31.3% (25 cases)] (all P values<0.001). After chemotherapy, the scores of physical function, emotional function and role function in the study group were higher than those in the control group, and the scores of pains, fatigue and insomnia in the study group were lower than those in the control group (all P values<0.05). Conclusions: CIK combined with mFOLFOX6 regimen for advanced colorectal cancer can improve and regulate the immune function of patients, prolong the survival time of patients, and enhance the quality of life of patients.


Assuntos
Neoplasias do Colo , Neoplasias Colorretais , Células Matadoras Induzidas por Citocinas , Masculino , Humanos , Pessoa de Meia-Idade , Idoso , Qualidade de Vida , Neoplasias Colorretais/tratamento farmacológico , Fatores de Transcrição Forkhead
7.
Balkan J Med Genet ; 25(2): 29-36, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-37265968

RESUMO

Epidermal growth factor receptor (EGFR) mutation status differs according to ethnicity, gender, smoking history, and histology types. The present study aimed to evaluate EGFR mutation status in patients with non-small cell lung cancer (NSCLC) and further explore its association with clinical characteristics and prognosis in advanced NSCLC patients (Stage IIIB-IV). 238 NSCLC patients were enrolled in this study from October 2016 through December 2019. Patient characteristics and clinical data including age, gender, smoking history, histology types, tumor stage, survival status, and time were collected via electronic medical record system or telephone. 21 somatic mutations which spanned exons 18-21 of EGFR were detected using the amplification refractory mutation system-polymerase chain reaction (ARMS-PCR) method, followed by analysis of links to clinical characteristics, progression-free survival (PFS) and overall survival (OS). 103 patients were detected harboring EGFR mutations among the 238 cases tested (43.3%), and exons 19 and 21 were the highest mutation frequencies, with 20.6% and 19.3% respectively. The EGFR mutation rate was much higher in female versus male (57.4% vs 31.5%, p <0.001), in non-smokers compared to smokers (56.8% vs 25.9%, p <0.001), and in those with adenocarcinoma than other histology types (48.3% vs 3.7%, p <0.001). For patients in advanced stage, median PFS was 11 months in patients harboring EGFR mutations, versus 4 months in patients with wild type EGFR (p <0.001); median OS was 24 versus 12 months (p <0.001). Never smoking (p = 0.042) and adenocarcinoma (p = 0.007) were independent favorable factors for EGFR mutations. Our data strengthen the findings of high prevalence of EGFR mutations in Asian patients with NSCLC. Mutations are prevalent in those patients who are female, adenocarcinoma, and have never smoked. Moreover, advanced EGFR mutation-positive patients have better PFS and OS than those with wild type EGFR.

8.
Zhonghua Yi Xue Za Zhi ; 103(23): 1781-1786, 2023 Jun 20.
Artigo em Chinês | MEDLINE | ID: mdl-37305938

RESUMO

Objective: To investigate the clinical efficacy and safety of 125I seed implantation in the treatment of mediastinal lymph node metastasis of lung cancer. Methods: Clinical data of 36 patients who underwent CT-guided 125I seed implantation for mediastinal lymph node metastasis of lung cancer from August 2013 to April 2020 in three hospitals of the Northern radioactive particle implantation treatment collaboration group were retrospectively collected, including 24 males and 12 females, aged 46 to 84 years. Cox regression model was used to analyze the relationship between local control rate, survival rate and tumor stage, pathological type, postoperative D90, postoperative D100 and other variables, and to analyze the occurrence of complications. Results: The objective response rate of CT-guided 125I seed implantation in the treatment of mediastinal lymph node metastasis of lung cancer was 75% (27/36), the median control time was 12 months, the 1-year local control rate was 47.2% (17/36), and the median survival time was 17 months. The 1-year and 2-year survival rates were 61.1% (22/36) and 22.2% (8/36) respectively. Univariate analysis showed that in the treatment of mediastinal lymph node metastasis with CT-guided 125I implantation, factors related to local control included tumor stage (HR=5.246, 95%CI: 2.243-12.268, P<0.001), postoperative D90 (HR=0.191, 95%CI: 0.085-0.431, P<0.001), postoperative D100 (HR=0.240, 95%CI: 0.108-0.533, P<0.001); The factors affecting survival were tumor stage (HR=2.712, 95%CI: 1.356-5.425, P=0.005), postoperative D90 (HR=0.110, 95%CI: 0.041-0.294, P<0.001), postoperative D100 (HR=0.212, 95%CI: 0.092-0.489, P<0.001). Multivariate analysis showed that tumor stage (HR=5.305, 95%CI: 2.187-12.872, P<0.001) and postoperative D100 (HR=0.237, 95%CI: 0.099-0.568, P<0.001) were correlated with local control rate. Tumor stage (HR=2.347, 95%CI: 1.095-5.032, P=0.028) and postoperative D90 (HR=0.144, 95%CI: 0.051-0.410, P<0.001) were correlated with survival. In terms of complications, 9 of the 36 patients had pneumothorax, and 1 of them was cured by closed thoracic drainage for severe pneumothorax; 5 cases developed pulmonary hemorrhage and 5 cases developed hemoptysis, which recovered after hemostasis treatment. One case developed pulmonary infection and recovered after anti-inflammatory treatment. No radiation esophagitis and radiation pneumonia occurred; No grade 3 or higher complications occurred. Conclusion: 125I seed implantation in the treatment of lung cancer mediastinal lymph node metastasis has a high local control rate and controllable adverse effects.


Assuntos
Neoplasias Pulmonares , Pneumotórax , Feminino , Masculino , Humanos , Metástase Linfática , Estudos Retrospectivos , Resultado do Tratamento
9.
Zhonghua Yi Xue Za Zhi ; 103(3): 199-204, 2023 Jan 17.
Artigo em Chinês | MEDLINE | ID: mdl-36649991

RESUMO

Objective: According to the formula provided by the TG43 report [AAPM TG43 (2004)] proposed by the American Association of Physicists in Medicine (AAPM) in 2004, we calculated the dose distribution around the radioactive iodine-125 particles, and verified the calculation accuracy of the radioactive iodine-125 particles treatment planning system. Methods: AAPM TG43 (2004) report provides two calculation methods when calculating the dose around a single radioactive source. The calculation method that does not consider the geometric structure of the radioactive source is called point source calculation method, and the calculation method that considers the geometric structure of the radioactive source is called line source calculation method. Assuming a single Amersham 6711 radioactive iodine-125 particle with an activity of 100 U, the following point doses were calculated according to the two calculation methods provided by AAPM TG43 (2004) report, at 0°, 90° directions, distances 0.5, 1, 1.5, 2, 2.5, 3, 3.5, 4, 4.5, 5, 5.5 and 6 cm; In the direction of 45°, the doses at 0.71, 1.41, 2.12, 2.83, 3.54, 4.24, 4.95, 5.66, 6.36, 7.07, 7.78 and 8.49 cm. On the clinically used brachytherapy planning system variseeds 8.0, the above two calculation methods are used to calculate the corresponding activity and the dose around the corresponding type of radioactive iodine-125 particles, and the function of capturing points to templates built in the planning system is used to accurately find the above corresponding point position, using a single measurement of the above corresponding point dose; and comparation of the results were performed to see if there is a statistical difference. Results: The AAPM TG43 report uses point source calculation method to calculate the dose of single Amersham 6711 radioactive iodine-125 particles with activity of 100 U at 0° and 90° directions. The points with the same distance and the same dose are 8 082.18, 1 870.08, 756.58, 381.47, 217.11, 131.91, 86.55, 58.32, 39.97, 27.42, 19.74, 14.13 Gy, respectively, at 0.5, 1, 1.5, 2, 2.5, 3, 3.5, 4, 4.5, 5, 5.5 and 6 cm away from them. In the 45° direction, the doses at the distances of 0.71, 1.41, 2.12, 2.83, 3.54, 4.24, 4.95, 5.66, 6.36, 7.07, 7.78 and 8.49 cm are 3 957.37, 865.83, 329.99, 155.69, 84.10, 48.50, 28.49, 17.80, 11.37, 7.38, 4.98 and 3.39 Gy, respectively; For line source calculation method, radioactive particles are at the same distance as above. The doses at each point in the direction of 0° are 3 128.71, 755.44, 330.30, 180.53, 107.74, 68.56, 46.40, 32.22, 22.70, 16.00, 11.51, 8.24 Gy, respectively. The doses at each point in the direction of 90° are 8 306.46, 1 981.01, 802.74, 405.38, 230.60, 140.03, 91.83, 61.84, 42.36, 29.05, 20.91, 14.97 Gy; In the 45° direction, the dose at the corresponding distance as above is 4 020.78, 877.43, 333.49, 156.93, 84.69, 48.81, 28.65, 17.89, 11.42, 7.41, 4.99 and 3.40 Gy, respectively. The maximum dose difference (0.3%) between the two methods is 7.78 cm in the 45° direction, the maximum difference (-0.3%) between the two methods is 8.49 cm in the 45° direction, and the value of other sampling points is less than 0.3%. The closer the Amersham 6711 iodine-125 particles are to the source in the directions of 0°, 45°, and 90°, the faster the dose will drop, and the dose will drop gradually as the distance increases. Conclusion: The brachytherapy planning system variseeds 8.0 and the AAPM TG43 report calculate a maximum dose difference of 0.3%, which can accurately calculate the dose distribution around radioactive iodine-125 seeds, and provide a reliable tool for the clinical implementation of radioactive iodine-125 particles implantation for tumor treatment.


Assuntos
Braquiterapia , Neoplasias da Glândula Tireoide , Humanos , Radioisótopos do Iodo , Dosagem Radioterapêutica , Método de Monte Carlo , Planejamento da Radioterapia Assistida por Computador/métodos , Radiometria/métodos
11.
Eur Rev Med Pharmacol Sci ; 26(18): 6725-6741, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-36196721

RESUMO

OBJECTIVE: Stomach adenocarcinoma (STAD) is the major cancer worldwide with high morbidity and mortality rate. Late diagnosis and limited treatment options of STAD lead to disease progression, spread, and metastasis. Therefore, finding a new biomarker to diagnosis and treatment is very important for STAD in clinical practice. MATERIALS AND METHODS: The clinical data, transcriptome data and CCLE data were downloaded from TCGA database and CCLE database, respectively. TIMER website, TISIDB website and CIBERSORT methodology were used to analyse immune infiltration. R software and R package were used to analyse gene difference expression, determine co-expression genes, conduct gene enrichment analyses, construct a prognostic signature and establish nomogram. RESULTS: MASP1 was decreased in STAD compared with normal tissue at the mRNA level (p < 0.001). The enrichment analysis showed that mismatch repair (MMR) was related to the MASP1 gene. Up-regulation of MAPS1 expression was positively associated with dendritic cells (p < 0.01), neutrophils (p < 0.05), macrophages (p < 0.001), CD4+ T cells (p < 0.001) and B cells (p < 0.05). A four-gene prognostic signature was determined based on MASP1-related immunomodulators. The prognostic signature was an independent prognostic predictor in STAD. Finally, we established a nomogram to forecast survival and the nomogram has a good prediction accuracy. CONCLUSIONS: In STAD, MASP1 is closely related to immunity. MASP1 has the potential to positively regulate the abundance of immune cells. The MASP1-related prognosis signature and nomogram can accurately predict the survival of patients with STAD. Therefore, MASP1 is likely to be a diagnosis and promising immunotherapy target spot in STAD clinical practice.


Assuntos
Adenocarcinoma , Serina Proteases Associadas a Proteína de Ligação a Manose , Neoplasias Gástricas , Adenocarcinoma/diagnóstico , Adenocarcinoma/genética , Adenocarcinoma/metabolismo , Humanos , Serina Proteases Associadas a Proteína de Ligação a Manose/genética , Serina Proteases Associadas a Proteína de Ligação a Manose/metabolismo , Prognóstico , RNA Mensageiro/genética , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/genética , Neoplasias Gástricas/metabolismo
12.
Zhonghua Gan Zang Bing Za Zhi ; 30(9): 947-953, 2022 Sep 20.
Artigo em Chinês | MEDLINE | ID: mdl-36299188

RESUMO

Objective: To investigate the dynamic expression pattern of carcinoembryonic Wnt3a and its early monitoring value using a hepatocellular carcinoma model. Methods: Forty-eight Sprague Dawley (SD) rats were fed with pellet feed containing 2-acetylaminofluorene (2-AAF, 0.05%) to induce hepatocarcinogenesis, and control rats were fed a pellet diet. Liver tissue and blood samples were collected every two weeks. Liver tissues were pathologically examined using HE staining and grouped. The gene and Wnt3a mRNA expression were analyzed by genome-wide microarray. The expression and distribution of Wnt3a in liver tissue were analyzed by immunohistochemistry. Wnt3a concentration in liver tissue and serum was quantified by enzyme-linked immunosorbent assay. Statistical methods such as χ2 test, Mann-Whitney test and analysis of variance were used to analyze the differences between groups. Results: According to the pathological examination results, the rat livers were divided into four groups: control, hepatocyte degeneration, precancerous lesions and hepatocellular carcinoma. Genome-wide expression profiling analysis and comparison with the control group revealed that 268 and 312 genes were up-regulated and 57 and 201 genes were down-regulated in the precancerous and cancerous group when signal logarithm ratio (SLR) was >8 log2cy5/cy3, and these significantly altered genes mainly involved in cell proliferation, signal transduction, tumor metastasis, and apoptosis. The expression of Wnt3a at mRNA level was significantly increased in all stages of cancer induction, including degeneration group (1.15±0.24, q=8.227), precancerous group (1.85±0.18, q=12.361) and cancerous group (2.59±0.55, q=18.082). Compared with the control group (0.25±0.11, F=121.103, P<0.001), the degeneration group, the precancerous group and the liver cancer group were up-regulated by 4.6, 7.4 and 10.4-folds, respectively. Immunohistochemistry showed that compared with the control group, the positive rate of Wnt3a in the degeneration group was 66.7% (12/18, χ2=10.701, P=0.001), and both the precancerous and liver cancer groups were positive (9/9, χ2=17.115, P<0.001). Wnt3a expression was gradually increased in liver and blood samples during the process of carcinogenesis, and the difference between two groups was statistically significant (F=176.711, P<0.001). Wnt3a overexpression was secreted into blood stream via cancerous liver tissue, and there was a linear correlation between Wnt3a levels in blood and liver samples (r=0.732, P<0.001). Conclusions: Wnt3a overexpression is closely related with hepatocellular carcinogenesis, and thus may become a new monitoring marker.


Assuntos
Carcinoma Hepatocelular , Neoplasias Hepáticas , Lesões Pré-Cancerosas , Ratos , Animais , Carcinoma Hepatocelular/patologia , Neoplasias Hepáticas/patologia , Ratos Sprague-Dawley , Carcinogênese/metabolismo , 2-Acetilaminofluoreno , RNA Mensageiro/metabolismo
13.
Artigo em Chinês | MEDLINE | ID: mdl-35866276

RESUMO

Objective: To report the experience of the application of internal carotid artery stent in skull base surgery, and to clarify the important role of internal carotid artery stent in skull base surgery. Methods: A retrospective study of 22 cases with skull base neoplasms implanted with internal carotid artery stents in the Department of ENT Head and Neck Surgery at the Sixth People's Hospital affiliated with Shanghai Jiao Tong University between July 2019 and January 2021 was conducted. Among them, 17 were male and 5 were female, aged between 33 and 75 years. There were 5 cases on the left, 16 cases on the right, and 1 case on both sides. Of these, there were 4 cases of jugular paraganglioma, 1 case of chondrosarcoma in the jugular foramen, 1 case of carotid body paraganglioma, and 16 cases of nasopharyngeal carcinoma after radiotherapy. Results: The degree of internal carotid artery erosion was assessed by computed tomography angiography (CTA), magnetic resonance imaging and digital subtraction angiography (DSA) images in 22 patients before surgery. It was found that the internal carotid artery was involved to varying degrees in all patients, so internal carotid artery stents were implanted before surgery. Tumor tissue was found to surround the internal carotid artery to varying degrees. Total or subtotal tumor resection was performed in all patients, and no intraoperative and postoperative complications occurred. The postoperative follow-up was 5 months to 2 years, and all patients had no complications such as spontaneous bleeding and pseudo aneurysm. There were no signs of stenosis or occlusion of the internal carotid artery stent segment in all cases. Conclusions: For patients with skull base tumors, preoperative imaging indicates the limited involvement of the internal carotid artery, and internal carotid artery stent implantation before surgery is a safe and effective treatment.


Assuntos
Artéria Carótida Interna , Neoplasias da Base do Crânio , Adulto , Idoso , Artéria Carótida Interna/cirurgia , China , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Base do Crânio/patologia , Base do Crânio/cirurgia , Neoplasias da Base do Crânio/patologia , Neoplasias da Base do Crânio/cirurgia , Stents
15.
Zhonghua Yan Ke Za Zhi ; 57(11): 861-864, 2021 Nov 11.
Artigo em Chinês | MEDLINE | ID: mdl-34743473

RESUMO

Primary orbital melanoma (POM) is a sort of extremely rare orbital malignancy. It often associates with melanin lesion such as congenital ocular melanocytosis and blue nevus. The most common clinical manifestation of POM is progressive painless proptosis. There are some difficulties in diagnosing this disease. MRI shows characteristic expression of this disease as an important way to diagnose POM, while pathology is the gold standard. Surgery is the mainstay of treatment in patients with POM. In addition, adjuvant radiotherapy and chemotherapy should be considered. This article systematically reviews the research progress of pathogenesis, clinical manifestation, auxiliary examination, differential diagnosis and treatment of POM, in order to provide some thoughts for the future study of the rare malignancy. (Chin J Ophthalmol, 2021, 57: 861-864).


Assuntos
Melanoma , Neoplasias Orbitárias , Diagnóstico Diferencial , Humanos , Imageamento por Ressonância Magnética , Melanoma/diagnóstico , Melanoma/terapia , Neoplasias Orbitárias/diagnóstico por imagem , Neoplasias Orbitárias/terapia
16.
J Dairy Sci ; 104(12): 12496-12507, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34593232

RESUMO

This study evaluated the effects of incremental tributyrin supplementation in pasteurized waste milk on growth performance, health, and blood metabolism of dairy calves before and after weaning. Forty-eight newborn female Holstein dairy calves (39.6 ± 2.75 kg; mean ± standard deviation) were blocked by age and randomly assigned to 3 treatments: pasteurized waste milk (1) without supplementation, (2) with 1 g/L of tributyrin products (unprotected solid powder; containing 35% tributyrin), or (3) with 2 g/L of tributyrin products. The calves were weaned on d 56 and were raised until d 77. Data were analyzed for the preweaning, postweaning, and overall periods. The results showed that starter intake and hay intake were not different among treatments in any period of the trial, but the crude protein intake tended to increase linearly with tributyrin supplementation during the overall period. Although tributyrin supplementation had no effects on body weight during preweaning and overall periods, body weight increased linearly with tributyrin supplementation postweaning. The average daily gain tended to increase linearly during postweaning and overall periods. No effects were observed on feed efficiency in any period. A positive linear relationship between body length and tributyrin supplementation was observed during the postweaning period, but no differences were found for the other body structural measurements in any period. The results of diarrhea showed that tributyrin concentration had a negative linear relationship with diarrhea frequency during preweaning and overall periods. The rectal temperature did not differ among treatments in any period, but a treatment × week effect for rectal body temperature was observed. For blood metabolism, tributyrin supplementation had no effects on insulin, growth hormone, total protein, albumin, or globulin. No differences were found in serum amyloid A concentration in any of the periods, yet haptoglobin concentration decreased linearly with increasing tributyrin concentration during postweaning and overall periods. Endothelin concentration showed a tendency to decrease linearly during preweaning and postweaning periods and decreased linearly with tributyrin supplementation during the overall period. An increasing tributyrin concentration was associated with a negative linear relationship with IL-1ß concentration during the preweaning period, and no differences were found in the other periods. The concentration of IL-6 and tumor necrosis factor α were not different among treatments in any of the periods. These data suggest that increasing the concentration of tributyrin in pasteurized waste milk could increase growth performance and health of dairy calves, and incremental tributyrin supplementation could linearly reduce haptoglobin, endothelin, and IL-1ß concentrations, indicating a positive effect of tributyrin on alleviating oxidative stress and inflammatory status of dairy calves. Calves fed pasteurized waste milk supplemented with tributyrin products (containing 35% tributyrin) at 2 g/L compared with 1 g/L of milk had more improved growth and health.


Assuntos
Ração Animal , Leite , Ração Animal/análise , Animais , Peso Corporal , Bovinos , Dieta , Suplementos Nutricionais , Feminino , Triglicerídeos , Desmame
17.
Artigo em Chinês | MEDLINE | ID: mdl-34256485

RESUMO

Objective: We conducted a Meta-analysis to investigate the necessity of postural restrictions after manual reduction in the treatment of posterior canal benign paroxysmal positional vertigo (PC-BPPV). Methods: We searched PubMed, EBSCO, Proquest, Web of Science databases, Ovid, and screened eligible studies that investigated the effect of post-maneuver postural restriction in treating patients with PC-BPPV. Outcomes included the efficacy of treatment and recurrence. Meta-analysis was performed using Stata 15.0 software. Results: Studies of the single visit efficiency included 11 references, with a sample size of 1 733 cases. The Meta-analysis results showed that the difference in the efficacy between the postural restricted group and the non-postural restricted group in PC-BPPV patients was statistically significant(RR=1.12, 95%CI=1.07-1.18, P<0.001). There were 12 references included in the study on the total efficiency, with a cumulative sample size of 1763 cases. There was no statistically significant difference between the effect of postural restriction after manipulative reduction and that of simple manipulative reduction (RR=1.03, 95%CI=0.99-1.08, P=0.118). There were 5 references included in the study of recurrence rate, and the cumulative sample size was 659 cases. There was no statistically significant difference in the recurrence rate between the postural restricted group and the non-postural restricted group(RR=0.98, 95%CI=0.62-1.54, P=0.937). Conclusions: In comparison with non-postural restriction group, post-maneuver postural restriction after a single visit can improve the treatment effective rate of PC-BPPV and contribute to the improvement in the symptoms of patients in a short term. However, postural restrictions has no significant effect on the final prognosis of PC-BPPV, and it also has no significant effect on the recurrence.


Assuntos
Vertigem Posicional Paroxística Benigna , Posicionamento do Paciente , Vertigem Posicional Paroxística Benigna/terapia , Meio Ambiente , Humanos , Recidiva , Canais Semicirculares , Resultado do Tratamento
18.
Zhonghua Yi Xue Za Zhi ; 101(21): 1583-1591, 2021 Jun 08.
Artigo em Chinês | MEDLINE | ID: mdl-34098685

RESUMO

Objective: Investigate the current situation of lung cancer cough diagnosis and treatment and the awareness of related issues among Chinese medical providers. Methods: Doctors, nurses, pharmacists from the oncology department, respiratory department, or general department were investigated using an electronic questionnaire from January 29 to March 29, 2021. There were 25 questions about lung cancer in the questionnaire. The questionnaire was including the most common accompanying symptoms in patients with lung cancer, the incidence of lung cancer-related cough, the assessment of the proportion of central antitussive drugs, and the commonly used central antitussives. Results: Questionnaires from 2 424 medical providers were collected from 402 hospitals in 21 provincial administrative units. Cough was the most common symptom in lung cancer. Most physicians believed that the incidence of lung cancer-related cough was 51%~75%, while the proportion of patients satisfied with the treatment was only 11%~20%. The evaluation of lung cancer-related cough was seriously insufficient. The leading cause of lung cancer-related cough was tumors. And the main problem was the inadequate antitussive effect of drugs in lung cancer-related cough management. The proportion of central antitussive medication usage in the secondary and tertiary hospitals was 93.9% and 92.0%, significantly higher than 75.0% in Primary hospitals (χ²=8.390, P=0.015). The proportion of the physicians who underhanded that codeine is at risk of addiction was 76.6% and 72.0% in the secondary and tertiary hospitals, which were significantly higher than 53.9% in Primary hospitals (χ²=9.240, P=0.010). In different occupations, the proportions of doctors and pharmacists who knew the risk of addicting to codeine were 73.0% and 82.6%, which were significantly higher than the 66.4% of nurses (χ²=21.200, P<0.001). The Chinese medical providers were lack of training about the basic knowledge of using central antitussive medication. Conclusions: The proportion of patients who were satisfied with the effect of cough treatment is low. The medical staff did not have enough awareness of this. There was an urgent need to develop a consensus and standardize lung cancer cough diagnosis and treatment in China.


Assuntos
Antitussígenos , Neoplasias Pulmonares , Antitussígenos/uso terapêutico , China/epidemiologia , Tosse/tratamento farmacológico , Tosse/etiologia , Humanos , Neoplasias Pulmonares/complicações , Inquéritos e Questionários
19.
Zhonghua Gan Zang Bing Za Zhi ; 29(4): 326-331, 2021 Apr 20.
Artigo em Chinês | MEDLINE | ID: mdl-33979958

RESUMO

Objective: To analyze the clinical efficacy and safety of camrelizumab combined with apatinib as a second-line therapy for unresectable hepatocellular carcinoma (HCC). Methods: Ninety-four cases with mid-and advanced-stage HCC who received camrelizumab combined with apatinib as second-line treatment were enrolled. Routine blood test, blood biochemical indexes, tumor stage, tumor imaging characteristics, previous treatment strategies and other clinical data before treatment were documented. Imaging examination follow-up results and adverse reactions during treatment were followed up until the end of follow-up or loss of follow-up or death. Kaplan-Meier method was used to analyze the clinical efficacy. Results: As of the last follow-up, 94 cases with mid-and advanced-stage HCC had received camrelizumab combined with apatinib as second-line treatment. Among them, 15 cases were lost to follow-up, 31 cases died, and 48 cases survived. The overall remission rate was 31.9%. The overall disease control rate was 71.3%. The median time to disease-free progression was 6.6 months. The median time to disease progression was not yet available. The 1-year cumulative survival rate was 62.3%. Grade 3 and above adverse reactions mainly included were thrombocytopenia (7.4%), abdominal pain (4.3%), active hepatitis (4.3%), leukopenia (4.3%), diarrhea (3.2%), hand-foot syndrome (3.2%). All adverse reactions were effectively controlled. Conclusion: Camrelizumab combined with apatinib can effectively prolong the survival period of patients with mid-and advanced-stage HCC, and it is well tolerated.


Assuntos
Carcinoma Hepatocelular , Neoplasias Hepáticas , Anticorpos Monoclonais Humanizados , Carcinoma Hepatocelular/tratamento farmacológico , Humanos , Neoplasias Hepáticas/tratamento farmacológico , Piridinas , Estudos Retrospectivos , Resultado do Tratamento
20.
Zhonghua Er Ke Za Zhi ; 59(4): 322-326, 2021 Apr 02.
Artigo em Chinês | MEDLINE | ID: mdl-33775053

RESUMO

Objective: To analyze the clinical features and efficacy of enzyme replacement therapy in 4 children with Fabry disease. Methods: A retrospective analysis of the clinical manifestations, laboratory findings, genetic variations and treatment were conducted in 4 children with Fabry disease in Children's Hospital of Zhejiang University School of Medicine from January 2014 to July 2020. Results: All four children (2 males, 2 females) with onset age of 12.4 (6.0-16.8) years were diagnosed based on clinical features, α-Gal A enzyme activity, genetic analysis and family history. The clinical manifestations varied in 4 children. All patients had left ventricular hypertrophy and abnormal urinalysis results, 1 case of neuropathic pain, 2 cases of hypohidrosis, 1 case of insipidus, but no angiokeratomas or hearing abnormalities were found. Three missense mutations of GLA gene were identified: c.424T>C (p.C142R), C.335G>A (p.R112H) and c.644A>G (p.N215S). The first two gene mutations were classical phenotypes, and the last one had also been reported in a classic case. In Case 1, no severe adverse events were reported in the first two months of agalsidase beta treatment. The dosage was 1 mg/kg once every 2 weeks. Symptoms of pain intensity and hypohidrosis were improved. Transiently elevated proteinuria was observed but it returned to normal after a week without any treatment. Conclusions: Clinical manifestations of Fabry disease varied in childhood. Multidisciplinary collaboration is required for its early diagnosis and treatment. Severe adverse events are rare in children with short-term therapy of agalsidase beta.


Assuntos
Doença de Fabry , Adolescente , Idade de Início , Criança , Terapia de Reposição de Enzimas , Doença de Fabry/tratamento farmacológico , Doença de Fabry/genética , Feminino , Humanos , Masculino , Estudos Retrospectivos , Resultado do Tratamento
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