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J. pediatr. (Rio J.) ; J. pediatr. (Rio J.);100(4): 384-391, July-Aug. 2024. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1564757

ABSTRACT

Abstract Objective: To investigate the incidence, clinical and genetic characteristics of pediatric lymphoma patients of China with inborn errors of immunity (IEI)-related gene mutations, which have not been fully studied. Method: From Jan. 2020 to Mar. 2023, IEI-related genetic mutations were retrospectively explored in 108 children with lymphomas admitted to Beijing Children's Hospital by NGS. Genetic rule and clinical characteristics as well as treatment outcomes were compared between patients with or without IEI-related gene mutations. Results: A total of 17 patients (15.7 %) harbored IEI-associated mutations, including 4 cases with X-linked lymphoproliferative syndrome (XLP), 3 cases had mutations in tumor necrosis factor receptor superfamily 13B (TNFRSF13B), 2 cases with Activated p110 syndrome (APDS). Patients with IEI all had alteration of immunocompetence with decreased levels of immunoglobulin and lymphocyte subsets. Recurrent infection existed in 41.2 % of patients. The 18-month event-free survival (EFS) and the overall response rate (ORR) of patients with IEI are significantly lower than those without IEI (33.86% vs. 73.26 %, p = 0.011; 52.94% vs. 87.91 %, p = 0.002, respectively). In addition, patients with IEI had a higher progression disease (PD) rate of 23.5 % than those without IEI of 4.4% (p = 0.006). Conclusion: The present study demonstrated that IEI-associated lymphomas were much more common than originally appreciated in pediatric lymphomas, and those were insensitive to treatment and more likely to progress or relapse. The genomic analysis and a thorough review of the medical history of IEI can be used to distinguish them from pediatric lymphomas without IEI, which are beneficial for the early diagnosis and direct intervention.

2.
Article | IMSEAR | ID: sea-242070

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Background: Malaria, caused by protozoa of the genus Plasmodium, remains a significant public health concern, especially in subtropical and tropical regions. Kolkata, in particular, has seen a high prevalence of malaria, with Plasmodium falciparum and Plasmodium vivax being the primary species. This study investigates the clinical features and vector distribution of malaria in the Kolkata region. The aim is to evaluate the clinical features of malaria and corresponding vectors in the Kolkata region. Methods: This retrospective study, conducted from November 2023 to March 2024, analyzed data from patients diagnosed with malaria over the past three years in Kolkata. Data were collected from hospital records, including blood reports, microscopy results, and patient histories. Patients were surveyed using a structured questionnaire to gather socio-economic and anthropometric data. Statistical analysis was performed using SPSS 27, with significance set at p<0.05. Results: The study included 103 falciparum and 97 vivax malaria patients. No significant differences were found in demographic and socio-economic characteristics between the groups. Severe disease was more common in falciparum malaria (82.5% vs. 33.0%, p=0.042). Hypoglycemia was significantly higher in falciparum patients (59.2% vs. 8.2%, p=0.044), while splenomegaly was more prevalent in vivax patients (43.3% vs. 15.5%, p=0.0455). No significant differences were observed for relapses, cerebral malaria, or renal failure. Conclusion: The study concludes that falciparum malaria typically presents with continuous fever and greater severity, including higher incidences of hypoglycemia, whereas vivax malaria is associated with recurrent fever and splenomegaly. Thus, alongside standard regimens, symptomatic treatment tailored to the specific type of malaria is necessary.

3.
Article | IMSEAR | ID: sea-240556

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Introduction: Impacted maxillary canines present diagnostic and therapeutic challenges in dental practice. Existing classification systems often lack comprehensive consideration of etiology, histopathology, and interdisciplinary collaboration. Objective: This study proposes a novel classification system integrating clinical, histopathological, and radiological insights to enhance understanding and management of impacted maxillary canines. Materials and Methods: A multidisciplinary panel collaborated to develop the classification system. Literature review, clinical case studies, and histopathological examinations informed the framework, resulting in five major types and corresponding subtypes. Results: The classification system includes developmental, traumatic, pathological, iatrogenic, and mixed etiologies, each with distinct subtypes. Integration of clinical, histopathological, and radiological criteria facilitates interdisciplinary collaboration and tailored interventions. Conclusion: The proposed classification system enhances diagnostic precision, treatment planning, and interdisciplinary collaboration for impacted maxillary canines, promoting personalized management strategies.

4.
Article | IMSEAR | ID: sea-232529

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Background: The frequency of Chlamydia trachomatis infection in infertile Indian women and potential risk factors associated with the infection is not well understood. To improve the primordial prevention C. trachomatis infection in developing countries, there is an urgent need to understand the prevalence of the infection among women with infertility and establish the common risk factors associated with this. This study was conducted to determine prevalence of Chlamydia trachomatis infection in infertile women attending an infertility clinic in north India and the associated socio-demographic and clinical features associated with it.Methods: Endocervical swabs, collected from 105 infertile women were tested for C. trachomatis by real time-PCR and direct gram’s stain. A detailed clinical history and examination was done on each subject during sample collection. The study group was then divided into two comparison groups and p factor was determined and factors with significant correlation were established.Results: Total 9 out of 105 infertile women visiting infertility clinic were RT-PCR positive for Chlamydia trachomatis. The socio-demographic factors that significantly correlated with Chlamydia trachomatis infection were lower age group, rural locality and illiteracy. The clinical history and examination findings that significantly correlated with the infection were past history of RTI/STI in the subject, history of RTI/STI in husband, cervical/ vaginal discharge, lower abdominal pain, burning micturition, erythema of genitalia, backache, dyspareunia and dysmenorrhea. The gram’s stain finding confirmed the active infection by presence of pus cells.Conclusions: The study concluded that the socio-demographic risk factor for Chlamydia trachomatis infection among infertile women is lower age group, rural locality and illiteracy while several clinical features that are red flags for the presence of such infection are past history of reproductive tract infection along with partner, cervical/vagina discharge, lower abdominal pain, burning micturition, erythema of genitalia, backache, dyspareunia and dysmenorrhea that should never be overseen.

5.
Article in Chinese | WPRIM | ID: wpr-1019052

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Objective To explore the clinical characteristics and risk factors of death during hospitalization in patients with community-acquired pneumonia(CAP)complicated with diabetes mellitus(DM).Methods A retrospective analysis was performed on 566 patients with CAP hospitalized in the Second Affiliated Hospital of Kunming Medical University from January 2018 to January 2022.The patients were divided into simple CAP group(n=478)and CAP combined with diabetes(CAP+DM)group(n=88)according to whether they had diabetes,and then CAP+DM group(n=88)was divided into survival group(n=69)and death group(n =19)according to whether the patients died during hospitalization.The clinical data and laboratory test indicators of patients in different groups were compared.Cox regression analysis was used to screen the risk factors of death during hospitalization in the CAP+DM group.Receiver operating characteristic(ROC)curve was plotted to evaluate the predictive value of independent risk factors on hospitalization death.Results Compared with the simple CAP group,the CAP+DM group had significant differences in age,concomitant hypertension,coronary heart disease,CURB-65 score,neutrophil to lymphocyte ratio(NLR),C-reactive protein(CRP),procalcitonin(PCT),albumin(ALB),prealbumin(PA),glucose(GLU),serum potassium(K),calcium(Ca),phosphorus(P),magnesium(Mg),lactic acid(Lac),non-invasive ventilation time,ICU occupancy rate and mortality rate(P<0.05);Compared with the survival group,there were statistically significant differences in CURB-65 score,NLR,CRP,PCT,GIU,ALB,PA,serum iron(Fe),Ca,non-invasive ventilation time,and ICU admission rate among the death group patients(P<0.05).Cox regression analysis showed that the increase of NLR level and the decrease in PA level were the risk factors for in-hospital death in patients with CAP complicated with diabetes(P<0.05).When the PA cutoff value was 91 mg/L,the AUC,sensitivity,and specificity for predicting in-hospital death of CAP patients with diabetes were 0.849,84.2%and 81.2%,respectively.Conclusion Patients with CAP combined with diabetes are more serious and have worse prognosis than those with CAP alone.PA has a good predictive value for the prognosis of these patients.Early detection and active intervention should be carried out to reduce the in-hospital mortality of patients.

6.
Article in Chinese | WPRIM | ID: wpr-1019086

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Objective To analyze the clinical characteristics of 310 patients with anti-tuberculosis drug-induced liver injury(ATB-DILI),to explore prognostic influencing factors,and to provide reference for its prevention and treatment.Methods Primary tuberculosis patients hospitalized in the Department of Tuberculosis of the Third People's Hospital of Kunming from November 2020 to November 2022 who met the diagnosis of ATB-DILI were enrolled.Statistics by gender,age,history,type of tuberculosis,co-morbidities,frequency of anti-tuberculosis regimens leading to liver injury,use of hepatoprotective drugs,and management and regression were performed to analyze the clinical characteristics of the patients and the factors influencing their prognosis.Results 310 patients were included,male,148(47.74%)and female,162(52.26%).The mean age was 44.33±17.47 years.Thirty-four patients had a history of allergy.The combination of isoniazid,rifampicin,pyrazinamide,and ethambutol(244 patients,78.71%)was the anti-tuberculosis regimen that resulted in the highest number of cases of hepatic injury.The median time between initiation of the tuberculosis regimen and the development of hepatic injury in patients with ATB-DILI was 30 d,and the mean duration of hospitalization was 16.39±7.01 d.The most used hepatoprotective drug was reduced glutathione(154 patients,49.68%),and most patients used a combination of 2 hepatoprotective drugs(128 patients,41.29%).Liver injury improved in 257 cases(82.90%)and failed in 53 cases(17.10%).The differences in alcohol consumption,severity,clinical staging,TT,ALP,TBIL,DBIL,IBIL,and GGT were statistically significant compared to those who did not recover(P<0.05),and severity and high ALP were independent risk factors for poor prognosis.Conclusions Patients should be carefully asked if they have a history of basic liver disease and alcoholism before using anti-tuberculosis drugs.In the course of anti-tuberculosis treatment,the combined use of anti-tuberculosis drugs is more serious than the use of single drugs to cause liver damage.Drugs that may cause liver damage should be used with caution and improved anti-tuberculosis programs should be explored.At the same time,liver function should be monitored regularly during anti-tuberculosis treatment,especially 30 days after medication,in order to reduce the occurrence of adverse reactions.

7.
The Journal of Practical Medicine ; (24): 43-47,52, 2024.
Article in Chinese | WPRIM | ID: wpr-1020703

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Objective To analyze the clinical characteristics of lung adenocarcinoma patients with positive EGFR mutations detected in pleural effusion.Methods We retrospectively analyzed the clinical characteristics including gender,age,smoking history,presence of other underlying diseases(such as COPD,cardiovascular disease,and diabetes),site of pleural fluid,feature of pleural fluid,and TNM stage in patients with lung adenocar-cinoma who had been admitted to the first Affiliated Hospital of Bengbu Medical College from 2020.01 to 2022.12 for the first time by the detection of EGFR mutation positive in pleural effusion.The data were statistically analyzed using the SPSS 26.0 software.Results A total of 126 patients were screened for enrollment,including 61 patients(48.41%)with EGFR exon 19 deletion mutation(19del),56 patients(44.44%)with exon 21 L858R mutation(21L858R),and 9 patients(7.14%)with non-classical mutations.Univariate analysis showed that the three muta-tion subtypes were statistically significant in terms of gender,age,smoking history,and presence of COPD(P<0.05 for all comparisons),but not in terms of pleural fluid site,feature of pleural fluid,tumor size,and presence of cardiovascular disease,diabetes mellitus,presence of distant metastases,and mediastinal lymph node metastases(P>0.05 for all comparisons);Multivariate analysis showed that 21 L858R mutation was more likely to be found in male,older age,non-smoking,and presence of COPD than 19del mutation;non-classical mutation was more likely to be found in male than 19del mutation.Conclusions There are significant differences among the three mutation subtypes in sex,age,smoking history,and presence of COPD,but not in pleural fluid location,feature of pleural fluid,tumor size,presence of cardiovascular disease or diabetes mellitus,presence of distant metastases,or medias-tinal lymph node metastases;Among lung adenocarcinoma patients with positive EGFR mutations in pleural fluid,21 L858R mutation mostly occurs in male,older age,non-smokers,and those complicated with COPD,while non-classical mutation mainly develops in male.However,more case studies are needed to confirm the above conclusions.

8.
Article in Chinese | WPRIM | ID: wpr-1020764

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Objective To construct a risk prediction model of pulmonary involvement based on chest CT and clinical feature in patients with primary Sjogren's syndrome(pSS),and to explore the risk prediction value of the model.Methods A total of 360 pSS patients who had been treated at Handan Hospital of Traditional Chinese Medicine from October 2020 to August 2023 were retrospectively selected as study objects,and were then divided into a modeling group(252 patients)and a verification group(108 patients)according to a ratio of 7∶3.The patients in the modeling group were divided into a control group(201 patients)and an involvement group(51 patients)based on presence or absence of lung involvement.The data on clinical characteristics and features of chest high-resolution CT(HRCT)in the modeling group was collected.Univariate analysis was performed among the groups to determine the relevant factors affecting lung involvement in pSS patients.Binary logistic regression analysis was performed on related factors to screen independent risk factors.A prediction model was established based on the independent risk factors.A verification and value analysis of the column-line prediction model were completed through data collection of the verification group.Results Age,disease course,cough,Raynaud's phenomenon,C-reactive protein(CRP),anti-SSA antibody,and HRCT were the relevant factors affecting lung involvement in pSS patients(all P<0.05).Further binary logistic regression analysis showed that old age,prolonged disease course,cough and abnormal HRCT imaging were independent risk factors for lung involvement in SS patients(all P<0.05).A nomogram risk prediction model was constructed based on independent factors.The model verification results indicated that the calibration chart showed better performance in the prediction model.The AUC of the area under the receiver operating characteristic(ROC)curve was 0.993 the modeling group and 0.995 in the validation group.Conclusions The clinical characteristics and the results of chest CT are closely related with lung involvement in patients with pSS.Old age,prolonged disease course,cough,and abnormal HRCT imaging are independent risk factors affecting lung involvement in patients with pSS.The prediction model established on this basis has a higher predictive value for the occurrence of lung involvement in patients receiving after-loading radiotherapy.

9.
Article in Chinese | WPRIM | ID: wpr-1022360

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Objective:To summarize the clinical characteristics and first aid measures of children with drowning,and to provide scientific basis for the treatment and prevention of drowning in children.Methods:The clinical data of drowning children admitted to the Children's Medical Center of the First Affiliated Hospital of Hunan Normal University(Hunan Provincial People's Hospital)from January 2011 to December 2021 were retrospectively summarized. Baseline characteristics,prehospital emergency care and prognosis of the included children were analyzed.According to the time span, drowning children were divided into two stages, the first stage (2011-2016) and the second stage (2017-2021). According to cerebral performance category assessment scale,drowning children with cardiac arrest were divided into two groups: a group with good neurological prognosis and a group with poor neurological prognosis. The baseline characteristics,pre-hospital emergency care, and prognosis of drowning children during different periods and with different prognosis were analyzed.Results:A total of 53 children were enrolled during the study period,with 24 cases in the first phase(2011-2016)and 29 cases in the second phase(2017-2021).Boys accounted for 73.6%(39/53)of the cases, the age ranged from 1 month and 24 days to 14 years old,with 1-4 years old being the most common,accounting for 58.5%(31/53), and the season in which drowning occurred was more common in the summer(39.6%, 21/53).Six cases (11.3%) had significant neurological sequelae, and the mortality rate was 3.8%(2/53).There was a decrease in the proportion of drowning with water control in the second stage compared to the first stage(37.5% vs. 13.79%, P=0.046).Thirty-two(60.4%)children experienced on-site cardiopulmonary resuscitation(CPR) after drowning,with 24 cases in the group with good neurological prognosis and 8 cases in the group with poor neurological prognosis.Children in the poor neurological prognosis group required CPR for a long duration [10.00(1.50, 25.00)min],had lower body temperature,Glasgow Coma Score,pH and higher blood glucose levels on admission(all P < 0.05). Conclusion:Drowning is most common in preschool children,common in boys,and drowning prevention measures for young children deserve focused attention. Children found to be in cardiac arrest at the time of drowning have a long duration of drowning and CPR,which predicts a poor neurological prognosis,and timely and effective on-site CPR is the key to reducing the drowning rate.

10.
Article in Chinese | WPRIM | ID: wpr-1023205

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Objective:To analyze the clinical and radiological features of patients with resected pancreatic serous cystic neoplasm (SCN).Methods:A retrospective analysis was conducted on the clinical and radiological data of 172 patients with pancreatic SCN who underwent surgical resection and were pathological confirmed at Peking Union Medical College Hospital from September 2014 to April 2022. These patients were categorized into two cohorts including 2014—2018 cohort ( n=84) and 2019—2022 ( n=88) for comparative analysis based on the operation time. Results:The radiological diagnostic accuracy for SCN was found to be notably low at 11.6%(20/172), which was mostly misdiagnosed as mucinous cystic neoplasms (86/172, 50.0%) and intraductal papillary mucinous neoplasms (35/172, 20.3%). No statistically significant variance was observed on the misdiagnosis rate between the two cohorts. There was a substantial increase of the number of lesions located in pancreatic body and tail (76.1% vs 53.6%, P=0.004) and a marked decrease in the number of those located in pancreatic head and neck (22.7% vs 39.3%, P=0.019) in 2019-2022 cohort, compared to the 2014-2018 cohort. Furthermore, there was a significant reduction in the occurrence of SCN calcification and peri-lesional venous alterations (22.8% vs 45.1%, P=0.006; 25.0% vs 46.4%, P=0.003), a decrease in the presence of fibrous scarring within SCN (31.8% vs 45.2%, P=0.070), and an increase in cases with dilatation of the upstream main pancreatic duct (28.2% vs 16.3%, P=0.065). The clinical characteristics and the rate of postoperative complications did not significantly differ between the two cohorts. Complication rates after pancreatoduodenectomy were significantly higher than those following distal pancreatectomy (46.4% vs 22.9%, P=0.010). The presence of clinical symptoms was significantly linked to the size of SCN and the expansion of the main pancreatic duct (45.4% vs 29.3%, P=0.032; 64.9% vs 31.2%, P<0.001). Conclusions:The accuracy of preoperative radiological diagnosis for SCN is still low, with a noted increase in the proportion of SCN exhibiting atypical radiological features in recent years. The frequency of postoperative complications is relatively high and does not have an obvious decreased trend.

11.
Chinese Journal of Endemiology ; (12): 133-136, 2024.
Article in Chinese | WPRIM | ID: wpr-1023998

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Objective:Clinical characteristics and diagnosis and treatment process was reported and analyzed of a patient with brucellosis complicated with thyroid abscess, providing reference for the clinical diagnosis of brucellosis complicated with thyroid abscess.Methods:Clinical medical records of a patient with brucellosis complicated with thyroid abscess who was treated at the General Surgery Department of Yanchi County People's Hospital in Wuzhong City, Ningxia Hui Autonomous Region in November 2021 were collected. The clinical manifestations, blood routine, brucella antibodies, thyroid function, bacterial culture, thyroid ultrasound and other examination results, as well as the diagnosis and treatment process, were comprehensively analyzed. Results:The patient was a male, 61 years old, who presented with a neck mass without typical clinical manifestations of brucellosis. Thyroid ultrasound revealed a space occupying lesion, and the preliminary diagnosis was thyroid cystadenoma. Thyroid right lobe and isthmus resection surgery was performed. During the operation, it was found that some of the thyroid glands were tightly adhered to the cervical blood vessels, so the resection surgery was changed to abscess drainage, and the drainage fluid was purulent and bloody. The bacterial culture result of thyroid purulent fluid (intraoperative puncture fluid and postoperative drainage fluid) was brucella lamblia, and the serum brucella test tube agglutination test titer was 1 ∶ 400 (+++). The patient improved and was discharged after local drainage and anti brucella treatment. Follow up for 4 months showed no abnormalities. Conclusions:Brucellosis which begins with a local infection of the thyroid gland is extremely rare, with no characteristic clinical manifestations, and is prone to misdiagnosis. Timely correction of the surgical plan during the treatment process avoids the removal of the patient's thyroid, which has a certain clinical reference value.

12.
China Journal of Endoscopy ; (12): 85-90, 2024.
Article in Chinese | WPRIM | ID: wpr-1024809

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Objective To report the clinical features,imaging findings and endoscopic dacryocystosinostomy(En-DCR)of acquired lacrimal sac mucocele(ALSM).Methods 63 patients(63 eyes)with ALSM treated with En-DCR combined with bicanalicular silicone tube intubation from January 2016 to March 2021 were reviewed.The clinical features,imaging findings,and surgical treatment of the included patients were analyzed by preoperative examination and 12 month postoperative follow-up.Fifty-seven patients(21 males and 36 females)were enrolled in this study at last,including 30 right eyes and 27 left eyes,25~71 years old,with an average age of(52.89±11.66)years old.All eyes with history of epiphora and purulent secretion.Results CT examination revealed enlargement of the lacrimal sac,but no destruction of the adjacent bone.MRI imaging showed enlargement of the lacrimal sac,fluid collection separated from adjacent tissues by a thin rim,corresponding to mucocele in the sac and increase in the sac diameters in all analyzed cases.The mass was found to shrink significantly when the lacrimal sac was opened during the surgery,and the swelling was completely relieved within 7 days post-operation.After 12 months of follow-up,the anatomical success rate of En-DCR was 92.98%(53/57),the functional success rate was 89.47%(51/57),no complications such as mucocele recurrence,diminution of vision and infection were found.Conclusion All the patients with ALSM had a history of previous lacrimal duct obstruction.Imaging examinations are valuable for the diagnosis of ALSM.En-DCR for ALSM is safe and effective,and worthy of clinical promotion.

13.
China Journal of Endoscopy ; (12): 87-90, 2024.
Article in Chinese | WPRIM | ID: wpr-1024834

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Objective To analyze the etiology and clinical characteristics of multiple clustered polyps in the ileocecal region.Methods Nine patients with multiple clustered polyps only in the ileocecal region found from 2017 to 2022 were retrospectively collected,and their etiology and clinical characteristics were analyzed.Results There were 9 patients with multiple clustered polyps in the ileocecal region in this group.There were 7 males and 2 females.The age of onset was 28~73 years old,52(40.0,62.5)years old.There were 8 cases of abdominal discomfort or abdominal pain,4 cases of chronic diarrhea,2 cases of mucous stool and 1 case of dry stool.The number of polyps ranged from 4~17,and the diameter of polyps ranged from 0.2~1.0 cm.Pathological examination showed chronic inflammation of ileocecal mucosa with polyp formation in 8 cases,and canalicular adenoma with mild dysplasia in 1 case.7 cases ate more red meat food or processed products.6 cases had repeat colonoscopy,and 3 cases had recurrence.Conclusion Multiple clustered polyps appearing only in the ileocecal region are a special type of polyps closely related to inflammation,and endoscopic surgery is recommended followed by attention to control inflammation.

14.
Article in Chinese | WPRIM | ID: wpr-1026948

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Objective:To explore the early differential diagnosis method by comparing the clinical characteristics of acquired immunodeficiency syndrome (AIDS) patients complicated with tuberculous meningitis (TBM) and cryptococcus neoformans meningitis (CNM).Methods:The AIDS patients admitted to Guangzhou Eighth People′s Hospital, Guangzhou Medical University from January 2011 to February 2022 and diagnosed with combined TBM and CNM after discharge respectively were included. A retrospective study was performed to analyze the clinical features of 21 AIDS patients complicated with TBM (TBM group) and 54 AIDS patients with CNM (CNM group) (all cases were confirmed by etiology). The data of meningitis-related symptoms and signs, blood routine test, CD4 + T lymphocyte counts, imaging characteristics and cerebrospinal fluid examination at admission were collected and analyzed. Statistical analysis was performed by using independent sample t test, rank sum test or chi-square test. Results:The age of patients in the TBM group was (44.6±12.9) years old, which was older than that of patients in the CNM ((37.6±12.6) years old), the difference was statistically significant ( t=-2.15, P=0.035). Forty-eight cases (88.89%) and seven cases (12.96%) in the CNM group experienced headaches and consciousness disorders respectively, with statistically significant differences compared to those in the TBM group (13 cases (61.90%) and nine cases (42.86%), respectively) ( χ2=7.25, P=0.007 and χ2=8.05, P=0.005, respectively). The proportion of leukopenia was 27.78%(15/54), and proportion of thrombocytopenia was 16.67%(9/54) in the CNM group, which were higher than those in the TBM group (4.76%(1/21) and 0(0/21), respectively), and the differences were statistically significant ( χ2=4.77, P=0.029 and χ2=3.98, P=0.042, respectively). The CD4 + T lymphocyte count in the TBM group was 74.0(92.0)/μL, which was higher than 19.5(56.5)/μL in the CNM group, and the difference was statistically significant ( Z=-2.87, P=0.009). The CNM group had 46 cases (85.19%) with cerebrospinal fluid pressure >180 mmH 2O(1 mmH 2O=0.009 8 kPa) and 24 cases (44.44%) with cerebrospinal fluid pressure >330 mmH 2O, which were significantly higher than those in the TBM group with seven cases (33.33%) and four cases (19.05%), respectively, and the differences were statistically significant ( χ2=19.61, P<0.001 and χ2=4.17, P=0.041, respectively). Fifty-two point three eight percent (11/21) of patients in the TBM group had a white blood cell counts>200×10 6/L in the cerebrospinal fluid, which was higher than that in the CNM group (1.85%(1/54)), with a statistically significant difference ( χ2=27.23, P<0.001). The white blood cell counts, protein and adenosine deaminase levels in the cerebrospinal fluid of TBM group were significantly higher than those in the CNM group (200.00(579.50)×10 6/L vs 17.50(66.25)×10 6/L, 1 863(2 858) mg/L vs 672 (513) mg/L and 6.60 (8.55) U/L vs 1.95(2.60) U/L, respectively), and the cerebrospinal fluid chloride level was lower than that in the CNM group ((107.71±8.22) mmol/L vs (115.99±6.55) mmol/L), and all the differences were statistically significant ( Z=4.11, P<0.001, Z=21.23, P=0.008, Z=2.09, P=0.040 and t=4.57, P<0.001, respectively). There was no significant difference in cerebrospinal fluid glucose between the TBM group and the CNM group ((1.86±1.22) mmol/L vs (2.34±1.05) mmol/L, t=-1.72, P=0.090). The proportion of patients with bilateral lung lesions in the TBM group was higher than that in the CNM group, and the difference was statistically significant (100.00%(21/21) vs 40.74% (22/54), χ2=-6.53, P=0.011). Conclusions:Patients with AIDS complicated with TBM are more likely to have consciousness disorders, inflammatory response in the cerebrospinal fluid, and more bilateral lung lesions. In contrast, patients with AIDS complicated with CNM are more frequently to experience severe headache and significant elevation of cerebrospinal fluid pressure, leukopenia and thrombocytopenia, and lower peripheral blood CD4 + T lymphocyte counts.

15.
JOURNAL OF RARE DISEASES ; (4): 57-62, 2024.
Article in Chinese | WPRIM | ID: wpr-1032067

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ObjectiveTuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disease involving multiple organ system. Renal angiomyolipoma (RAML) is a leading cause of death for adult TSC patients. Our study aims at investigating the clinical manifestations of TSC-RAML to enable clinicians to have a better understanding of the disease in Chinese patients.MethodsWe reviewed retrospectively the data collected from patients with TSC-RAML in Peking Union Medical College Hospital from January 2014 to January 2023. We also collected information about the age, gender, imageological examination, RAML stage, and comorbidities.ResultsA total of 186 TSC-RAML patients were registered, 65 of whom were males and 121 were females(male-to-female ratio 1∶1.86). The median age of all patients was 31 years old. Totally, 117 cases (62.9%)of RAML were rated stage 6. Twenty-two cases (11.8%) had a history of tumor rupture and bleeding. Research shows statistical differences between high and low grade on RAML rupture bleeding(P=0.0475). Angiofibromas/fibrous cephalic plaque (155/186, 83.3%), subependymal nodules(103/146, 70.5%), lymphangioleiomyomatosis (102/157, 65.0%), hypomelanotic macules (114/186, 61.3%), and shagreen patch (83/186, 44.6%)were the most common clinical manifestations. All patients with lymphangioleiomyomatosis(LAM) were female(P < 0.0001).ConclusionsFemale patients predominated TSC-RAML patients. Most RAML were in stage 6. About 11.8% cases had a history of tumor rupture and hemorrhage. The higher grade meant the higher proportion of tumor rupture and hemorrhage. Dermatological and nervous lesions were the most common comorbidities. All patients with LAM in this study were female.

16.
China Modern Doctor ; (36): 5-9, 2024.
Article in Chinese | WPRIM | ID: wpr-1038112

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Objective To investigate the clinical manifestations of familial adenomatous polyposis(FAP).Methods The clinical data of 100 patients with FAP diagnosed in the First Affiliated Hospital of Zhengzhou University from 2011 to 2021 were analyzed retrospectively.Results The main clinical manifestations were bloody stool(44.0%),abdominal pain(40.0%),changes of stool characteristics(25.0%),abdominal distension(18.0%)and diarrhea(17.0%);The most common types of adenomas were villous tubular adenoma(44.9%)and tubular adenoma(32.1%);Extraintestinal manifestations:3 cases of desmoid fibroma,2 cases of osteoma and 1 case of lipoma.47 cases of gastric polyps and the mostly pathological type was fundic gland polyp(34.0%).18 cases of duodenal polyps including 6 cases of adenomatous polyps and most were located at descending duodenum(61.1%).Adenoma canceration occurred in 21 patients.The average age was 38.7 and the canceration mainly occurs in the rectum.The gender,family history,age of onset,number of adenomas,diameter and pathological type of adenomas were statistically compared between patients with and without canceration.It was found that there were significant differences in gender,average age of onset,number of adenoma polyps,diameter of adenoma and pathological type between the two groups(P<0.05).Conclusions FAP is mainly characterized by bloody stool and abdominal pain,with high canceration rate.The risk factors include the age of onset,the number and size of adenomas and the pathological type.The main pathological types are villous tubular adenoma and tubular adenoma.At present,preventive colorectal resection is still the most effective way to treat FAP.No matter what type of methods patients choose,they should be followed up strictly under endoscopy in order to find the lesions in time and treat if necessary.

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China Modern Doctor ; (36): 34-37, 2024.
Article in Chinese | WPRIM | ID: wpr-1038155

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Objective To investigate the clinical features of hospitalized children with pertussis.Methods The clinical data of 87 hospitalized children with pertussis admitted to Women and Children's Hospital Affiliated to Xiamen University from January 2019 to December 2022 were retrospectively analyzed.According to the age of the children,they were divided into group A(≤3 months of age,35 cases),group B(4-12 months of age,36 cases)and group C(>12 months of age,16 cases).The clinical features of children at different ages were compared.Results The majority of children with pertussis were<1 year old(81.61%),and August was the month of high incidence.Spastic cough(72.41%)was the main clinical manifestation.Other manifestations included redness or cyanosis,vomiting after coughing,crow echo,etc.There were statistically significant differences in the incidence of spastic cough,redness or cyanosis and the contact history of patients with chronic cough among three groups(P<0.05).There were significant differences in the incidence of pneumonia and the average length of hospital stay among three groups(P<0.05).There was significant difference in C-reactive protein level among three groups(P<0.05).Among 87 children with pertussis,37 cases(42.53%)were infected with other pathogens,among which virus infection was the most common.The treatment is effective with macrolides and compound sulfamethoxazole.Conclusion Pertussis is more common in infants under 1 year of age,and the incidence is high in August.Chronic cough patients with close contact may be the source of infection.Spastic cough is the main clinical symptom of children;the younger,the more common symptoms such as spastic cough,redness and cyanosis,the higher incidence of pneumonia and the longer hospital stay.

18.
Article in Chinese | WPRIM | ID: wpr-1006571

ABSTRACT

As a commonly used traditional Chinese medicine in clinical practice, Tripterygium wilfordii has the functions of dispelling wind and removing dampness, detoxicating and destroying parasites, detumescence, pain relief, promoting blood circulation, and dredging collateral. Modern pharmacological studies show that it also has other functions such as anticancer, anti-inflammation, and immunosuppression. It has been widely used to treat autoimmune diseases, renal diseases, and tumors. T. wilfordii contains a variety of chemical components, among which triptolide (TP) can cause varying degrees of damage to human digestive, circulatory, reproductive, and other systems, with liver injury being the most common one, which greatly limits the development of TP in new drug research and industrial application. Therefore, the authors focused on the research hotspot of TP-induced liver injury and summarized relevant Chinese and international literature regarding the clinical manifestations, injury mechanisms, and detoxification strategies of TP-induced liver injury. This helps to provide a scientific basis for the clinical drug safety and scientific drug supervision of TP. The clinical manifestations of TP-induced liver injury are mostly abnormal transaminases, loss of appetite, nausea and vomiting, anorexia, yellow staining of skin and sclera, and yellow urine. The mechanisms of the above clinical manifestations involve apoptosis, oxidative stress, influence on cytochrome P450 superfamily, macrophage polarization, regulation of biological clock gene Clock, etc. Among them, cell apoptosis is related to neurogenic locus notch homolog protein 1 (Notch1), dynamin-related protein 1 (Drp1)-cytochrome C (Cyt C), phosphatidylinositide 3-kinases (PI3K)/protein kinase B (Akt), mitogen-activated protein kinase (MAPK), tumor suppressor protein 53 (p53), Fas cell surface death receptor (Fas)/Caspase-8, and other signaling pathways. Oxidative stress is related to inhibition of nuclear factor-erythroid 2-related factor 2 (NRF2) signaling pathway, promotion of cytochrome P450 2E1 (CYP2E1) expression, and excessive accumulation of reactive oxygen (ROS). The influence of the cytochrome P450 superfamily is manifested as reducing the substrate affinity, activity, and expression of cytochrome P450 3A (CYP3A), cytochrome P450 2C9 (CYP2C9), cytochrome P450 2C19 (CYP2C19), and cytochrome P450 1A2 (CYP1A2). Promoting the transformation of macrophages into the M1 type is related to the secretion of inflammatory factors and the accumulation of endotoxin, and the internal rhythmic regulation of the biological clock gene Clock, is related to the expression of cytochrome P450 3A11 (CYP3A11) metabolic enzyme. The detoxification strategies in the clinical application include herbs-processing detoxification strategy and drug-pairing detoxification. The traditional Chinese medicines and monomers that are helpful for detoxification include Glycyrrhiza uralensis, Paeonia lactiflora, Lysimachia christinae, Rehmannia glutinosa, saffron, and paeoniflorin. The reviews and discussion about these topics can help to provide more references for related research and clinical application of TP.

19.
JOURNAL OF RARE DISEASES ; (4): 57-62, 2024.
Article in English | WPRIM | ID: wpr-1006918

ABSTRACT

ObjectiveTuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disease involving multiple organ system. Renal angiomyolipoma (RAML) is a leading cause of death for adult TSC patients. Our study aims at investigating the clinical manifestations of TSC-RAML to enable clinicians to have a better understanding of the disease in Chinese patients.MethodsWe reviewed retrospectively the data collected from patients with TSC-RAML in Peking Union Medical College Hospital from January 2014 to January 2023. We also collected information about the age, gender, imageological examination, RAML stage, and comorbidities.ResultsA total of 186 TSC-RAML patients were registered, 65 of whom were males and 121 were females(male-to-female ratio 1∶1.86). The median age of all patients was 31 years old. Totally, 117 cases (62.9%)of RAML were rated stage 6. Twenty-two cases (11.8%) had a history of tumor rupture and bleeding. Research shows statistical differences between high and low grade on RAML rupture bleeding(P=0.0475). Angiofibromas/fibrous cephalic plaque (155/186, 83.3%), subependymal nodules(103/146, 70.5%), lymphangioleiomyomatosis (102/157, 65.0%), hypomelanotic macules (114/186, 61.3%), and shagreen patch (83/186, 44.6%)were the most common clinical manifestations. All patients with lymphangioleiomyomatosis(LAM) were female(P < 0.0001).ConclusionsFemale patients predominated TSC-RAML patients. Most RAML were in stage 6. About 11.8% cases had a history of tumor rupture and hemorrhage. The higher grade meant the higher proportion of tumor rupture and hemorrhage. Dermatological and nervous lesions were the most common comorbidities. All patients with LAM in this study were female.

20.
Article in Chinese | WPRIM | ID: wpr-1005924

ABSTRACT

Objective To analyze the clinical characteristics and influencing factors of non-small cell lung cancer (NSCLC) patients with chronic obstructive pulmonary disease (COPD) in Hubei province, and to provide a theoretical basis for the diagnosis and treatment of NSCLC patients with COPD. Methods A total of 246 NSCLC patients admitted to our hospital from 2018 to 2020 were selected and divided into control group (without COPD, n=125) and observation group (with COPD, n=121) according to COPD. The clinical characteristics of chest pain, hemoptysis, emasculation, atelectasis and pleural effusion were compared between the two groups. The values of FEV1/FVC, RV/TLC and DLCO in the two groups were measured by pulmonary function detector. The age, gender, smoking, smoking history, proportion of lung squamous cell carcinoma, TNM stage and other clinical data of all subjects were analyzed by self-made survey scale of our hospital. Univariate analysis and logistic regression were used to analyze the risk factors of COPD in NSCLC patients. Results Among 246 NSCLC patients, 121 patients (49.19%) were complicated with COPD, including 76 males and 45 females, and there was a statistical difference between the two groups (χ2=4.891, P>0.05). The average age of the observation group (61.02±4.82) was significantly higher than that of the control group (59.76±4.73) (t=2.069, P0.05). Male (OR=2.982), smoking history (OR=2.623) and lung squamous cell carcinoma (OR=3.147) were risk factors for COPD in NSCLC patients (P<0.05). Conclusions NSCLC patients with COPD are more common in male smokers in Hubei Province, often accompanied by pleural effusion , severe hemoptysis and other symptoms , and their lung function is decreased. Early detection and standardized treatment of COPD in the treatment of NSCLC can improve the prognosis of patients.

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