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Mpoxou Varíola M é uma zoonose causada por vírus do gênero Orthopoxvirus, causadores também da varíola comum. É uma doença considerada rara e autolimitada, sendo endêmica em países africanos. Entretanto, no ano de 2022 ganhou destaque devido ao surto global que se iniciou, quando o mundo ainda se recuperava da pandemia da COVID-19. Dessa forma, por se tratar de uma doença emergente, a presente revisão visa pontuar aspectos gerais do que se sabe até o momento sobre a Mpox, desde sua imunopatogenia até as formas atuais de prevenção e cuidados pós-infecção
Mpox or Variola M is a zoonosis caused by viruses of the genus Orthopoxvirus, which also cause smallpox. It is a disease considered rare and self-limiting, being endemic in African countries. However, in 2022, it gained prominence due to the global outbreak that began when the world was still recovering from the COVID-19 pandemic. Thus, as it is an emerging disease, this review aims to point out general aspects of what is known so far about Mpox, from its immunopathogenesis to current forms of prevention and post-infection care
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Humans , Severe Acute Respiratory Syndrome , Mpox (monkeypox) , Viruses , Wounds and Injuries/virology , Smallpox , Delivery of Health CareABSTRACT
Objective:To summarize the clinical features and survival differences between human immunodeficiency virus (HIV)-positive and HIV-negative cervical cancer patients, and to explore the factors influencing the prognosis.Methods:The clinical data of patients with cervical cancer diagnosed and treated in Zhongnan Hospital of Wuhan University from January 2015 to January 2022 were retrospectively analyzed. There were 46 HIV-positive cases and 587 HIV-negative cases; all 46 HIV-positive patients had squamous cell carcinoma, while 504 HIV-negative patients had squamous cell carcinoma. According to age and clinical staging, 230 HIV-negative squamous cell carcinoma patients were screened to match with 46 HIV-positive squamous cell carcinoma patients according to 1∶5. The clinical features of HIV-positive and HIV-negative patients were compared in all matched patients with pathological type of squamous cell carcinoma; the Kaplan-Meire method was used to analyze the overall survival (OS) and the comparison of OS was made by using log-rank test. Multivariate Cox proportional risk model was used to analyze the independent factors affecting the OS of patients with cervical squamous cell carcinoma.Results:The differences in the age, pathological types, clinical staging between 46 HIV-positive patients and 587 HIV-negative patients were statistically significant (all P < 0.05). There were statistically significant differences in age and clinical staging between 46 HIV-positive squamous cell carcinoma patients and 504 HIV-negative squamous cell carcinoma patients (all P < 0.05). After 1∶5 matching, there were no statistically significant differences in the age, clinical staging between 46 patients with HIV-positive squamous cell carcinoma and 230 patients with HIV-negative squamous cell carcinoma. The OS of HIV-positive patients in the entire group,pathological type of squamous cell carcinoma or after pairing was worse than that of HIV-negative patients (all P < 0.001). The median OS time of HIV-positive patients was 63 months (95% CI 61-109 months), while the median OS time of HIV-negative patients was not reached (95% CI 165-178 months, 164-178 months, 143-173 months, respectively). Multivariate Cox regression analysis showed that clinical staging Ⅲ-Ⅳ was an independent risk factor for OS in patients with cervical squamous cell carcinoma (Ⅲ-Ⅳ vs. Ⅰ-Ⅱ: HR = 1.573, 95% CI 1.032-2.397, P = 0.035); HIV infection was an independent protective factor for OS (HIV-positive vs. HIV-negative: HR = 0.087, 95% CI 0.042-0.182, P < 0.001), indicating that HIV-positive patients had an advantage in OS compared to HIV-negative patients at the same age and clinical staging. Age was not an independent influencing factor for OS ( P > 0.05). Conclusions:The onset age of HIV-positive cervical cancer tends to be younger and the clinical staging is late when patients are diagnosed. HIV-positive patients have poor prognosis.
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Myosteatosis is the main manifestation of malnutrition and is a common complication of chronic liver diseases, with a negative impact on the progression and prognosis of liver diseases. There is no gold standard for the diagnosis of myosteatosis, and CT is a commonly used diagnostic method. Myosteatosis in chronic liver diseases has diverse mechanisms, and hyperammonemia, increased cathepsin D level in circulation, insulin resistance, mitochondrial dysfunction, and chronic systemic microinflammation play an important role in myosteatosis. Early intervention of these factors can improve prognosis. With reference to related studies in China and globally, this article reviews the features, diagnostic methods, pathogenesis, treatment, and intervention of myosteatosis.
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Objective:To explore the expression of serum connective tissue growth factor (CTGF), glyoxalase Ⅰ (GLO-I) and pyruvate kinase M2 (PKM2) in endometrial cancer and their relationship with clinicopathological characteristics.Methods:A total of 96 endometrial cancer patients in Yuechi County People's Hospital from February 2015 to February 2017 were selected as the research group, 48 patients with endometrial hyperplasia during the same period were selected as the benign control group, and 48 patients with healthy physical examination during the same period were selected as the healthy control group. The serum levels of CTGF, GLO-Ⅰ, and PKM2 in the three groups were analyzed. The correlation between serum levels of CTGF, GLO-Ⅰ and PKM2 in the research group was analyzed, and the relationship between each serum index and clinicopathological characteristics was analyzed.Results:The levels of serum CTGF, GLO-Ⅰ and PKM2 in the research group were higher than those in the benign control group and healthy control group: (184.31 ± 37.14) μg/L vs. (110.45 ± 20.59), (17.28 ± 0.42) μg/L; (95.17 ± 16.56) pmol/L vs. (56.29 ± 10.14), (9.08 ± 0.66) pmol/L; (20.25 ± 6.13) μg/L vs. (13.11 ± 4.58), (9.05 ± 2.74) μg/L; and the levels of serum CTGF, GLO-Ⅰ and PKM2 in the benign control group were higher than those in the healthy control group, there were statistical differences ( P<0.05). The results of Pearson correlation analysis showed that the level of CTGF had positive correlation with GLO-Ⅰ and PKM2 ( r = 0.713, 0.741, P<0.05), and the level of GLO-Ⅰ had positive correlation with PKM2 ( r = 0.823, P<0.05). The results of Spearman correlation analysis showed that the levels of CTGF, GLO-Ⅰ, PKM2 had positive correlation with FIGO stage ( r = 0.609, 0.704, 0.721; P<0.05), myometrial invasion depth ( r = 0.753, 0.695, 0.719; P<0.05), lymph node metastasis ( r = 0.776, 0.744, 0.640; P<0.05); had negative correlation with the degree of differentiation ( r = - 0.711, - 0.720, - 0.668; P<0.05). Conclusions:Serum CTGF, GLO-I, PKM2 expression levels are abnormally elevated in patients with endometrial cancer, which are significantly related to multiple clinicopathological characteristics.
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Objective:To analyze the expression of microRNA(miR)-205 and miR-24-3p in cervical cancer tissues and their correlation with survival prognosis.Methods:A total of 82 patients with cervical cancer(cervical cancer group), 60 patients with uterine fibroids(uterine fibroids group) and 40 healthy women (healthy control group) treated in Hubei Hospital of Integrated Traditional Chinese and Western Medicine from March 2014 to March 2015 were enrolled. The expression levels of miR-205 and miR-24-3p in the tissues of the three groups were compared. The receiver operative characteristic (ROC) curve was used to analyze the diagnostic value and zero threshold of the two indicators for cervical cancer. Analyzed the 5-year survival status of cervical cancer patients at the upper and lower thresholds, and then further discuss the risk factors that affected the prognosis of cervical cancer.Results:The relative expression levels of miR-205 and miR-24-3p in the cervical cancer group were significantly higher than those in the uterine fibroids group and the healthy control group ( P<0.01). ROC curve analysis showed that the cut-off value was 1.13 and 1.35, the area under the curve was the biggest, and the sensitivity in diagnosis of cervical cancer were 83.87%, 84.38%, the specificity were 75.00%, 82.42%.The survival rate analysis based on these results showed that the 5-year survival rate of patients with miR-205≥1.13 was 69.05%, which was significantly lower than patients with miR-205<1.13 (87.50%) ( P<0.05); the 5-year survival rate of patients with miR-24-3p≥1.35 was 68.89%, which was significantly lower than that of patients with miR-24-3p<1.35 (89.19%)( P<0.05). The results of the Cox regression model showed that moderately and highly differentiated, FIGO stage Ⅱ, depth of invasion ≥1/2, combined lymph node metastasis, and high levels of miR-205 and miR-24-3p were risk factors affecting the prognosis of cervical cancer patients ( P<0.05). Conclusions:Serum miR-205 and miR-24-3p are at high levels in cervical cancer tissues, and are closely related to the prognosis of patients. They are expected to become auxiliary diagnosis and prognostic indicators for cervical cancer.
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Cytomegalovirus hepatitis is a liver disease caused by human cytomegalovirus infection and is one of the most common liver diseases in children and immunocompromised individuals. This disease has no specific clinical manifestations and is easily confused with other types of viral hepatitis, which may lead to delayed treatment or mistreatment. Therefore, the early diagnosis of cytomegalovirus hepatitis is of vital importance, and patients should be given timely and effective treatment with appropriately selected antiviral drugs and course of treatment. This article reviews the recent research advances in the etiology, epidemiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prevention of cytomegalovirus hepatitis.
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Objective To investigate the clinical, biochemical, pathological, disease course, and prognostic features of drug-induced liver injury (DILI) patients with different types of bile duct injury. Methods Four patients who were diagnosed with bile duct injury-type DILI by liver biopsy in Shijiazhuang Fifth Hospital, from March 2015 to October 2010 were selected, and related data were collected, including clinical data, laboratory examinations, radiological examination, and prognosis.The semi-quantitative score was determined for liver pathological morphology, and each indicator was compared between the four patients. Results Bile duct injury-type DILI was more common in female patients, and most patients tended to have a good prognosis.Clinical symptoms, liver biochemical parameters, and prognosis varied with the site, grade, scope, regeneration, and repair of bile duct injury. Conclusion Liver biopsy is still the gold standard for making a definite diagnosis of bile duct injury-type DILI, understanding the condition of lesions, and judging the prognosis of this disease.
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Objective:To investigate the efficacy of intensity-modulated radiotherapy with sequential chemotherapy in the treatment of high-grade glioma and analyze the influential factors.Methods:A total of 56 patients with high-grade glioma who received treatment in Yantai Municipal Laiyang Central Hospital from January 2014 to January 2016 were retrospectively analyzed. All patients underwent three-dimensional conformal radiotherapy or enhanced radiotherapy. The use of bevacizumab, pathological grade, and preoperative and postoperative Karnofsky Performance Status scores in all patients were recorded. Cox and other proportional risk regression models were used to analyze the predictors of patient mortality and receiver operating characteristic (ROC) curve analysis was performed.Results:All patients were followed up to April 2022. Follow-up results showed that the median survival time of patients receiving concurrent chemotherapy with temozolomide and adjuvant chemotherapy with temozolomide was 11.6 months. Univariate analysis showed that pathological grade, Karnofsky Performance Status scores, and the degree of tumor resection were correlated with the prognosis of patients ( P = 0.022, 0.049, 0.022). Multivariate analysis showed that the degree of tumor resection and pathological grade were the independent influential factors of prognosis ( P = 0.010, 0.010). Survival curve analysis revealed that the median survival time of patients subjected to total tumor resection was 12.6 months and that of patients subjected to partial tumor resection was 4.8 months. The median survival time of patients subjected to total tumor resection was longer than that of patients subjected to partial tumor resection. The median survival time of patients with WHO grade Ⅲ tumors was 25.2 months, and it was 6.3 months for patients with WHO grade Ⅳ tumors. The median survival time of patients with WHO grade Ⅲ tumors was longer than that of patients with WHO grade Ⅳ tumors. The receiver operating characteristic curve analysis results showed that the area under the receiver operating characteristic curve plotted for using WHO classification of tumors in the neurological system and surgical methods to predict the death of patients with high-grade glioma was 0.783 and 0.814, respectively. WHO tumor grade and surgical methods for prediction of prognosis of high-grade glioma had high accuracy. Conclusion:Low pathological grade and total resection are independent protective factors for the prognosis of patients with high-grade glioma.
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Objective:To investigate the changes of Toll-like receptor 4(TLR4) level in peripheral blood of endometriosis (EMT) patients with infertility and its relationship with clinicopathological types and pregnancy outcomes.Methods:A total of 150 patients with EMT complicated with infertility (study group) admitted to Huangzhou District People′s Hospital of Huanggang City from May 2019 to March 2021 and 50 healthy married women (control group) who participated in physical examination during the same period were selected as the research objects. Reverse transcription polymerase chain reaction (RT-PCR) was used to determine the level of TLR4 in peripheral blood of the two groups, and different pathological types of EMT were compared. The receiver operating characteristic (ROC) curve was used to evaluate the predictive efficacy of TLR4 expression for postoperative pregnancy in EMT patients with infertility. At the same time, the relationship between changes in TLR4 level in peripheral blood and pregnancy outcomes was analyzed according to the TLR4 cut-off value.Results:The expression of TLR4 mRNA in peripheral blood in the study group was higher than that in the control group: 98.65 ± 10.63 vs. 1.27 ± 0.20, there was statistical difference ( t = 64.66, P<0.01). After treatment, the expression of TLR4 mRNA in peripheral blood of patients with simple peritoneal type, internal cystic type and adenomyosis type EMT complicated were decreased :81.13 ± 8.89 vs. 64.31 ± 6.44, 103.58 ± 10.01 vs. 85.40 ± 8.78, 118.69 ± 12.76 vs. 96.38 ± 9.96, There were statistical differences ( P<0.05); No matter before or after treatment, the expression of TLR4 mRNA in peripheral blood of patients with adenomyosis was higher than that of patients with internal cystic type and simple peritoneal type, while the expression of TLR4 mRNA in peripheral blood of patients with internal cystic type was higher than that of simple peritoneal type, and there were statistical differences ( P<0.05). The results of ROC curve analysis showed that when the area under the curve (AUC) was 0.787(95% CI 0.701 - 0.863, P<0.05) and the best cut-off value was 41.75, the sensitivity, specificity and accuracy of TLR4 mRNA expression in peripheral blood to predict postoperative pregnancy in EMT patients with infertility were 80.12%, 81.57% and 80.30%, respectively. Using the peripheral blood TLR4 mRNA expression of 41.75 as the cut-off value, 143 patients with EMT and infertility with complete follow-up data were divided into high expression group (58 cases, TLR4 mRNA≥41.75) and low expression group (85 cases, TLR4 mRNA < 41.75). The postoperative intrauterine pregnancy rate and total pregnancy rate in the high expression group were lower than those in the low expression group: 44.83% (26/58) vs. 70.59% (60/85), 58.62% (34/58) vs. 81.18% (69/85), and there were statistical differences ( χ2 = 9.54, 8.71, P<0.05). Conclusions:There is abnormal expression of TLR4 in peripheral blood of EMT patients with infertility, and its high expression is closely related to adverse clinicopathological features and pregnancy outcomes. Effective intervention of treatment and postoperative recovery according to the expression level of TLR4 mRNA can further improve the natural pregnancy rate of such patients.
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ObjectiveTo investigate the change in the proportion of non-B, non-C hepatocellular carcinoma (NBNC-HCC) in hepatocellular carcinoma, and to compare and analyze the clinicopathological features of NBNC-HCC. MethodsA total of 3 090 patients with hepatocellular carcinoma (HCC) who were diagnosed in Sichuan Provincial People’s Hospital from January 2011 to December 2021 were enrolled, and according to the hepatitis markers, they were divided into hepatitis virus infection-associated HCC group with 2 472 patients and NBNC-HCC group with 618 patients. According to the liver disease and metabolic risk factors, the NBNC-HCC group was further divided into metabolic disorder HCC group with 289 patients, alcoholic liver disease (ALD)-associated HCC group with 174 patients, and other HCC group with 155 patients. General information, laboratory markers, and pathological findings were collected from all HCC patients. The Mann-Whitney U test was used for comparison of continuous data between two groups, and the Kruskal-Wallis H test was used for comparison between three groups; the chi-square test was used for comparison of categorical data between groups, and the chi-square trend test was used to investigate the trend of the change in the proportion of NBNC-HCC in HCC. ResultsThe proportion of patients with NBNC-HCC in HCC increased from 13.7% in 2011 to 20.1% in 2021 (χ2=5.529, P=0.019), and compared with the hepatitis virus infection-associated HCC group, the NBNC-HCC group had a significantly higher proportion of patients with diabetes (28.0% vs 10.3%, χ2=129.482, P<0.001) or hypertension (33.2% vs 15.2%, χ2=105.079, P<0.001), a significantly lower proportion of patients with liver cirrhosis (44.5% vs 68.4%, χ2=122.563, P<0.001) or vascular invasion (20.4% vs 29.6%, χ2=7.749, P=0.005), and a significantly higher body mass index (BMI) (Z=-4.015, P<0.001). Compared with the ALD-HCC group, the metabolic disorder HCC group had a significantly higher BMI, a significantly lower FIB-4 index, and a significantly lower proportion of patients with liver cirrhosis (all P<0.05). ConclusionThere is a tendency of increase in the proportion of patients with NBNC-HCC in HCC, and NBNC-HCC often coexists with metabolic risk factors such as obesity, diabetes, and hypertension. Patients in the metabolic disorder HCC group may develop liver cancer in the absence of liver cirrhosis or in the early stage of liver fibrosis.
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Traditionally, the progression from compensated liver cirrhosis to decompensated liver cirrhosis has been considered an irreversible point in the natural history of the disease; however, with the suppression of underlying etiology, cure, and disease regression, this view is challenged by an increasing number of new evidence, and the idea of “recompensation of liver cirrhosis” is gradually being accepted. In recent years, scholars in China and globally have been exploring the specific definition of recompensation of liver cirrhosis and the clinical features of patients. By summarizing the recent studies on recompensation of liver cirrhosis in China and globally, integrating existing views, and analyzing related research evidence, this article points out the main challenges in the field of recompensation at this stage, including the lack of in-depth clinical and basic research, the need to define recompensation in the context of NAFLD, and related ethical issues, in order to provide new directions for future research in this field.
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Introducción: La paracoccidioidomicosis, es una micosis endémica en diferentes países de latinoamérica, incluyendo zonas de Colombia, con manifestación clínica muy variada ya que es considerada una enfermedad que puede ser crónica y sistémica. Objetivos: Exponer la importancia del examen clínico-estomatológico en la detección de diferentes entidades en sistema estomatognático, y el manejo multidisciplinario de paracoccidioidomicosis oral y sistémica. Caso clínico: paciente masculino en sexta década de vida, el cual presenta glosalgia y dolor de región ocular. Clínicamente presenta lesión de tipo granulomatoso en bordes laterales de lengua con evolución de hace 1 año aproximadamente, el resultado de la biopsia es paracoccidioidomicosis, se inicia manejo multidisciplinario con medicamentos intravenosos y orales, luego de 10 meses presenta resolución de este. Conclusiones: un buen interrogatorio, análisis de cuadro clínico y exámenes complementarios, son claves para un diagnóstico temprano y tratamiento oportuno, preservando la vida del paciente, especialmente en infecciones oportunistas como la paracoccidioidomicosis.
Introdução: A paracoccidioidomicose é uma micose endêmica em diferentes países da América Latina, incluindo áreas da Colômbia, com manifestação clínica muito variada por ser considerada uma doença que pode ser crônica e sistêmica. Objetivos: Expor a importância do exame clínico-estomatológico na detecção de diferentes entidades do sistema estomatognático e no manejo multidisciplinar da paracoccidioidomicose oral e sistêmica. Caso clínico: paciente do sexo masculino na sexta década de vida, que apresentava glossalgia e dor na região ocular. Clinicamente apresenta lesão tipo granulomatosa nas bordas laterais da língua com evolução de aproximadamente 1 ano, resultado da biópsia é paracoccidioidomicose, inicia-se manejo multidisciplinar com medicações endovenosas e orais, após 10 meses resolve. Conclusões: um bom questionamento, análise do quadro clínico e exames complementares são fundamentais para o diagnóstico precoce e tratamento oportuno, preservando a vida do paciente, principalmente nas infecções oportunistas como a paracoccidioidomicose.
Introduction: Paracoccidioidomycosis is an endemic mycosis in different Latin American countries, including areas of Colombia, with a very varied clinical manifestation since it is considered a disease that can be chronic and systemic. Objectives: To expose the importance of the clinical-stomatological examination in the detection of different entities in the stomatognathic system, and the multidisciplinary management of oral and systemic paracoccidioidomycosis. Clinical case: male patient in the sixth decade of life, who presented glossalgia and pain in the ocular region. Clinically, it presents a granulomatous-type lesion on the lateral edges of the tongue with evolution of approximately 1 year ago, the result of the biopsy is paracoccidioidomycosis, multidisciplinary management is started with intravenous and oral medications, after 10 months it resolves. Conclusions: a good questioning, analysis of the clinical picture and complementary tests are key to early diagnosis and timely treatment, preserving the patient's life, especially in opportunistic infections such as paracoccidioidomycosis.
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Objective:To investigate the clinical features of acute megakaryocytic leukemia (AMKL) in children.Methods:The clinical data of 14 children with AMKL in Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology from January 2012 to July 2021 were retrospectively analyzed, and the related literature was reviewed.Results:Among 14 children with AMKL, there were 5 males and 9 females, and the median age of onset was 19 months (0.1-109 months); 1 case was Down syndrome-related AMKL, and 13 cases were non-Down syndrome-related AMKL. Most of the children presented with fever, anemia or bleeding symptoms, and a few patients presented with joint pain as the primary symptom. Some children were accompanied by extramedullary infiltration such as hepatomegaly, splenomegaly or lymphadenovarix. Initial investigations of 14 children showed that the median white blood count, hemoglobin concentration and platelet count were 10.67×10 9/L [(6.56-83.62)×10 9/L], 84 g/L (55-121 g/L), 37×10 9/L [(8-1443) ×10 9/L], respectively, and the median proportion of naive cells in peripheral blood was 0.09 (0.00-0.79). Bone marrow smear showed that the primitive megakaryocytes were characterized by various size and irregular form, a few of which had cytoplasmic vacuoles, and the median proportion of bone marrow primitive megakaryocytes was 0.636 (0.332-0.976); the nuclei were round or irregular, with multiple nucleoli or hidden nucleoli. RAS staining was partially positive, and immunohistochemical assay showed that POX, AS-DNCE and α-NBE were negative. Detection of megakaryocyte-associated antigens by flow cytometry showed 12 children expressed CD41a or CD61, and 10 children expressed CD42b. Among 3 children who completed chemotherapy, 1 case of Down syndrome-related AMKL and 1 case of non-Down syndrome-related AMKL were event-free survival, and 1 case of non-Down syndrome-related AMKL died after bone marrow relapse. Conclusions:The clinical manifestations and biological characteristics of children with AMKL are complicated and the prognosis is poor. Some children can achieve disease-free survival through chemotherapy alone.
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Objective:To investigate the clinicopathological features and prognosis of mycosis fungoides.Methods:The clinical data of 34 patients with mycosis fungoides hospitalized in Liaoning Cancer Hospital from January 1996 to December 2016 were retrospectively analyzed. The clinicopathological characteristics and prognosis of the patients were summarized. The follow-up was up to June 2021. Kaplan-Meier method was used for survival analysis, and log-rank test was used to compare the overall survival (OS) of patients among different subgroups.Results:Among the 34 patients, 22 (64.7%) were male and 12 (35.3%) were female; the median onset age was 56.5 years (25-93 years). A total of 23 cases (67.6%) presented with the rash on the whole body, and the main manifestations of the rash in the tumor stage were tumor rupture (10 cases, 29.4%) and redness (7 cases, 20.6%); 20 cases (58.8%) were misdiagnosed at initial onset. In the terms of TNMB stage, 3 cases were at stage Ⅰ (8.8%), 11 cases were at stage Ⅱ(32.4%), 3 cases were at stage Ⅲ (8.8%), and 17 cases were at stage Ⅳ (50.0%); 17 cases (50.0%) had the lesions confined to the skin and 17 cases had distant metastasis. Among the patients with distant metastasis, 7 cases had visceral organs involvement, 5 cases had lymph nodes involvement, and 5 cases had bone marrow or peripheral blood involvement. There were 32 cases treated with first-line chemotherapy alone, 9 cases with radiotherapy alone, and 7 cases in combination with radiotherapy and chemotherapy. Among the 32 patients treated with first-line chemotherapy, 14 cases had complete remission (CR), 12 cases had partial remission (PR), and response rate (RR) was 81.3% (26 cases); among the 9 patients who received radiotherapy, 3 cases had CR, 5 cases had PR, and RR was 88.9% (8 /19). The median follow-up time was 142.5 months; until the last follow-up, 20 (58.8%) cases survived, 6 (17.6%) cases died and 8 (23.5%) cases lost the follow-up. Survival analysis showed that the median OS of the whole group was not reached. Compared with patients whose lesions were confined to the skin, patients with distant metastasis had poorer OS ( P = 0.039). Among patients with distant metastasis, those with lymph node involvement had better OS, followed by those with bone marrow or peripheral blood involvement, those with visceral organ involvement had the poorest OS ( P = 0.045). Conclusions:The clinical misdiagnosis rate of early-stage mycosis fungoides is high, and the diagnosis mainly depends on clinical, histological and pathological characteristics. Radiotherapy and chemotherapy have high efficiency for early-stage disease and the prognosis of patients with distant metastasis is poor. OS in patients with lymph node involvement is better than that in patients with bone marrow or peripheral blood involvement, and OS in patients with visceral organ involvement is the worst.
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Objective:To investigate clinicopathological and magnetic resonance imaging (MRI) characteristics of pediatric cardiac tumors.Methods:The clinical, pathological and MRI data of 7 patients with pediatric cardiac tumors confirmed by pathological examination in Children's Hospital of Chongqing Medical University from February 2012 to December 2016 were retrospectively analyzed.Results:There were 3 males and 4 females with first diagnosis age ranging from 1 month to 3 years. As for clinical presentation, most cases were featured with cardiac murmur and enlarged cardiac boundary; only 1 case had acute cerebral infarction, and 1 case did not show any abnormal performance. Pathological findings showed that 6 cases of benign tumors (including 2 cases of fibroma, 1 case of rhabdomyoma, 1 case of myxoma, 1 case of lipoma and 1 case of hemangioma), 1 case of malignant tumor (primitive neuroectodermal tumor of pericardium). MRI results showed that the signal intensity of malignant tumor was higher than that of normal myocardium in each sequence; significant differences were found in benign tumors; first-pass perfusion, cardiac cine image and late gadolinium enhancement were the most obvious.Conclusions:The clinical presentations of pediatric cardiac neoplasms are atypical. Each tumor type has pathognomonic pathological features. MRI has great advantages in the diagnosis and differential diagnosis of cardiac tumors especially for benign tumors.
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Objective To investigate the clinical and pathological features of children with glycogen storage disease (GSD). Methods A retrospective analysis was performed for ten children with GSD who were admitted to the Third Hospital of Hebei Medical University and The Fifth Medical Center of Chinese PLA General Hospital from January 2002 to January 2022, based on medical history, liver biochemistry, and liver biopsy, and population characteristics, clinical manifestations, biochemical parameters, and liver histopathological characteristics were compared and analyzed. Results All ten children had developmental retardation and a short stature, with the manifestations of abnormal liver function, mild weakness, poor appetite, yellow urine, and yellow eyes, and four children had hepatosplenomegaly. Among the ten children, six had the clinical manifestations of hypoglycemia, and one had bilateral gastrocnemius hypertrophy and positive Gower sign. Two children had positive CMV IgG. Liver histopathological manifestations included diffuse enlargement of hepatocytes, light cytoplasm, and small nucleus in the middle like plant cells, with or without fibrous tissue proliferation. Conclusion Most patients with GSD have developmental retardation and abnormal aminotransferases, and liver pathological examination shows specific pathological features.
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Objective To investigate the association of biliary, pancreatic, and ampullary tumors with the onset of acute pancreatitis (AP) and the clinical features of tumor-induced AP by retrospectively analyzing the clinical data of patients with tumor-induced AP. Methods Related clinical data were collected from the patients with AP who were admitted to The First Affiliated Hospital of Zhengzhou University from January 2013 to August 2021. The etiological composition of AP was analyzed, as well as the change in the incidence rate of tumor-induced AP; the clinical features of AP were compared between tumor-induced and non-tumor-induced AP and between the tumors at different locations to explore the pathogenesis of tumor-induced AP. For normally distributed continuous data, the t -test was used for comparison between two groups; a one-way analysis of variance was used for comparison between multiple groups, and the SNK- q test was used for further comparison between two groups. For non-normally distributed continuous data, the Mann-Whitney U test was used for comparison between two groups; the Kruskal-Wallis H test was used for comparison between multiple groups, and the Wilcoxon rank-sum test was used for further comparison between two groups. The chi-square test or the Fisher's exact test was used for comparison of dichotomous categorical data between groups, and the goodness-of-fit test was used for comparison of polytomous categorical data between groups. The receiver operating characteristic (ROC) curve was used to evaluate the differential factors for pancreatic tumor-induced AP, and a multivariate logistic regression analysis was used to investigate the independent predictive factors for tumor-induced AP. Results A total of 8106 patients with AP were enrolled, among whom 84 patients (1.04%) had tumor-induced AP (tumor group). The tumor group had a significantly higher mean age than the non-tumor group ( t =6.050, P < 0.001). The mean time from initial onset of AP to tumor diagnosis was 7.38 months. Among the 84 patients with tumor-induced AP, 75 (89.2%) had mild AP (MAP), 8 (9.5%) had moderate severe AP, and 1(1.2%) had severe AP; as for the origin of tumor, 11(13.1%) had tumor originating from the lower biliary tract, 13(15.5%) had tumor originating from the ampulla, and 60(71.4%) had tumor originating from the pancreas. Recurrence of AP (risk ratio [ RR ]=8.362, 95% confidence interval [ CI ]: 3.162-22.115, P < 0.001), pancreatic duct dilatation ( RR =10.996, 95% CI : 3.871-31.236, P < 0.001), bile duct dilatation ( RR =7.738, 95% CI : 2.521-23.752, P < 0.001), and leukocyte count ( RR =0.766, 95% CI : 0.666-0.881, P < 0.001) were independent predictive factors for tumor-induced AP. Conclusion Tumor-induced AP is common in middle-aged and elderly men, with the clinical manifestations of MAP, easy recurrence, pancreatic duct dilatation/bile duct dilatation, and a persistent increase in the tumor marker CA19-9. Imaging examination of the biliary, pancreatic, and ampullary regions should be enhanced for AP with the above characteristics and no apparent trigger, and follow-up should be strengthened to avoid the missed diagnosis of tumor and the influence on prognosis.
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Objective:To investigate the correlation between KRAS, NRAS and BRAF V600E gene mutations and the clinicopathological characteristics of patients with colorectal cancer.Methods:Specimens from 217 patients with colorectal cancer who underwent surgical resection and were pathologically confirmed in Shanxi Province Cancer Hospital from January 2020 to December 2021 were selected, and the clinical data of the patients were retrospectively analyzed. The mutation status of KRAS, NRAS and BRAF V600E genes were detected in the paraffin specimens of surgically-resected tissues by direct sequencing. The mutation rates of KRAS, NRAS and BRAF V600E were compared among patients with different clinicopathological characteristics.Results:The mutation rates of KRAS, NRAS and BRAF V600E in 217 patients with colorectal cancer were 48.4% (105/217), 4.1% (9/217) and 3.7% (8/217), of which 1 patient (0.5%) had both KRAS and NRAS mutations. NRAS gene mutation was not correlated with gender, age, tumor size, tumor location, pathological type, degree of differentiation, depth of invasion, lymph node metastasis, distant metastasis, TNM stage, hemangioma thrombus/nerve invasion (all P>0.05); KRAS mutation rate in patients ≥ 60 year old was higher than that in patients < 60 year old [55.3% (63/114) vs. 40.8% (42/103), χ2 = 4.55, P = 0.033),and there was no correlation between KRAS gene mutation and other clinicopathological features (all P > 0.05); the mutation rate of BRAF V600E gene in colorectal cancerpatients with distant metastasis was higher than that in patients without distant metastasis [16.7% (4/24) vs. 2.1% (4/193), P = 0.006], and there was no correlation between BRAF V600E gene mutation and other clinicopathological features (all P > 0.05). Conclusions:Older colorectal cancer patients may be prone to KRAS gene mutation, and the BRAF V600E gene mutation rate is higher in patients with distant metastasis, and there is no correlation between NRAS gene mutation and clinicopathological characteristics.
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Objective To investigate the clinical and pathological features of progressive familial intrahepatic cholestasis type 3 (PFIC3). Methods A retrospective analysis was performed for 1326 patients with unexplained liver disease who attended Nanjing Second Hospital from January 2017 to December 2019, among whom 8 patients were diagnosed with PFIC3 based on clinical/pathological manifestation and gene sequencing results (1 patient did not undergo liver biopsy due to contraindication). Clinical, laboratory, imaging, and pathological findings were analyzed and a literature review was performed for the pathology of ABCB4-related diseases to summarize the clinical and pathological features of PFIC-3. Results Among the 8 patients with PFIC3, there were 5 male patients and 3 female patients, with a median age of 29.5 years. Of all 8 patients, 4 (50%) manifested as chronic cholestasis and 4 (50%) manifested as biliary cirrhosis, among whom 3 (75%) had the manifestation of portal hypertension. As for biochemical examination, 75% (6/8) had an increase in alkaline phosphatase, and 100% (8/8) had an increase in gamma-glutamyl transpeptidase. As for imaging examination, 50% (4/8) had cholecystitis, 25% (2/8) had gallstones, 25% (2/8) had bile duct dilatation, 75% (6/8) had splenomegaly, and 25% (2/8) had liver cirrhosis. As for liver biopsy, all 7 patients manifested as bile duct injury and/or reduction, and 57.1% (5/7) had absence of the bile duct. Multidrug resistance P-glycoprotein 3 (MDR3) immunohistochemical staining showed normal expression in 42.9% (3/7) of the patients and reduced expression in 57.1% (4/7) of the patients. Literature review obtained 17 articles with a description of the bile duct or MDR3 immunohistochemistry. Among the 7 patients with low phospholipid-associated cholelithiasis, 71.4% (5/7) had normal bile duct, 14.3% (1/7) had bile duct reduction, and 14.3% (1/7) had absence of the bile duct; among the 6 patients with intrahepatic cholestasis of pregnancy, 16.7% (1/6) had normal bile duct, 50% (3/6) had bile duct reduction, and 33.3% (2/6) had absence of the bile duct; among the 8 patients with PFIC3, 25% (2/8) had bile duct reduction and 75% (6/8) had absence of bile duct; among the 21 patients with PFIC3, 9.5% (2/21) had normal expression of MDR3, 23.8% (5/21) had a reduction in the expression of MDR3, and 66.7% (14/21) had absence of the expression of MDR3. Conclusion PFIC3 mainly manifests as cholestasis, cholelithiasis, and hepatic fibrosis. Pathological manifestation includes bile duct injury and bile duct reduction or absence of the bile duct in severe cases, and the degree of injury is associated with disease severity. MDR3 immunohistochemistry may show normal expression, reduced expression, or absence of expression, and diagnosis cannot be excluded in patients with normal expression. Genetic testing can be performed for diagnosis when necessary.
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Objective:To correlate creatine kinase (CK) and creatine kinase- isoenzyme MB (CK-MB) with different states of bipolar disorder in patients.Methods:A total of 206 patients with bipolar disorder who received treatment in The 7 th People's Hospital of Wenzhou, China between January 2018 and June 2019 were included in the patient group. A total of 369 healthy controls who concurrently received physical examination were included in the control group. CK and CK-MB levels were detected in all participants. The Brief Psychiatric Rating Scale (BPRS), Hamilton Depression Rating Scale (HAMD)-7 scale, the Bech-Rafaelsen Melancholy Scale (BRMS), and modified version of the Overt Aggression Scale (MOAS) were used to evaluate the mental symptoms, depression, mania and aggression of patients. The CK and CK-MB levels were compared between patients with different states of bipolar disorder. Results:In the control group, CK and CK-MB levels in males were 112.5 (94.5, 156.5) U/L and 17.0 (15.0, 20.0) U/L, respectively, which were significantly higher than those in females [73.0 (61.0, 86.3) U/L, 15.0 (13.0, 18.0) U/L, Z = -9.732, -3.535, both P < 0.001). In the patient group, CK and CK-MB levels in males were 129.0 (80.0, 233.5) U/L, 12.0 (10.0, 17.0) U/L, respectively, which were significantly higher than those in females [73.0 (55.0, 94.0) U/L, 13.5 (11.0, 17.0) U/L, Z = -9.510, -4.746, both P < 0.001]. There was no significant difference in CK level in males between the control and patient groups ( Z = -1.003, P = 0.316), but significant difference in CK-MB level in males was observed between the two groups ( Z = -6.570, P < 0.001). There were significant differences in CK and CK-MB levels in females between the control and patient groups ( Z = -2.535, -9.707, P = 0.011, P < 0.001). In the patient group, CK level in the manic, depressive, and symptom-alleviated states was 132.0 (78.0, 297.0) U/L, 85.0 (56.0, 145.0) U/L, 128.0 (110.0, 165.0) U/L respectively in males, and it was 73.0 (49.0, 122.3) U/L, 51.0 (45.0, 67.0) U/L and 84.5 (61.0, 193.0) U/L, respectively in females. There was significant difference in CK level in males and females between different states of bipolar disorder ( χ2 = 9.019, 16.720, P = 0.011, P < 0.001). In males, CK level was correlated with the BPRS total score, BRMS total score, and MOAS total score in the manic state, as well as the BPRS total score in the symptom-alleviated state ( r = 0.282, 0.286, 0.236, 0.574). In females, CK level was correlated with the MOAS total score in the manic state ( r = 0.260). In males, CK-MB level was correlated with the BRMS total score in the manic and depressive states ( r = 0.186 and 0.496). In females, CK-MB level was correlated with the MOAS total score and the BRMS total score in the manic state ( r = 0.155, 0.572). Conclusion:CK and CK-MB levels are correlated with bipolar disorder in different states and they are of certain clinical significance and provide innovative insights into the diagnosis of bipolar disorder.