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BACKGROUND: Parathyroidectomy is the only curative treatment for primary hyperparathyroidism (PHPT). Ultrasound (US) and technetium-99 m sestamibi (99mTc-MIBI) scintigraphy are recommended as the first-line localization imaging modalities for PHPT in adults, but the value of preoperative imaging in pediatric patients has not been reported. OBJECTIVE: To evaluate the added value of 99mTc-MIBI scintigraphy in pediatric PHPT patients with positive ultrasound results. MATERIALS AND METHODS: Pediatric patients (≤18 years old) who were diagnosed with PHPT and underwent surgical treatment in Peking Union Medical College Hospital between January 2003 and January 2021 were included in this study. Demographic and clinical characteristics, preoperative localization US, 99mTc-MIBI scintigraphy and pathology results were collected. Preoperative localization results were evaluated by comparison with surgical and pathological findings. RESULTS: There were 32 pediatric PHPT patients with median age of 14.7 ± 2.5 years who all proved to have single-gland disease without ectopic lesions. The median lesion size was 2.85 cm (range 1.0-5.8 cm). All patients underwent US and 99mTc-MIBI scintigraphy. Neck US demonstrated 100% sensitivity. Of 32 patients with a positive US, 99mTc-MIBI scintigraphy was concordant in 30 (93.8%). In 2 patients (6.3%), US reported suspected multigland disease, which was correctly diagnosed by 99mTc-MIBI scintigraphy as single lesions. CONCLUSION: In pediatric PHPT patients, US achieved high sensitivity for preoperative localization. 99mTc-MIBI scintigraphy for pediatric patients with positive US results would not increase the sensitivity. Implementation of 99mTc-MIBI scintigraphy could increase the specificity in pediatric patients with multigland disease suspected by US.
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Hiperparatireoidismo Primário , Tecnécio Tc 99m Sestamibi , Adulto , Humanos , Criança , Adolescente , Hiperparatireoidismo Primário/diagnóstico por imagem , Hiperparatireoidismo Primário/cirurgia , Cintilografia , Ultrassonografia/métodos , Sensibilidade e Especificidade , Compostos RadiofarmacêuticosRESUMO
BACKGROUND: Sporadic lymphangioleiomyomatosis (S-LAM) is a rare neoplasm with heterogeneous clinical features that is conventionally considered to be related to TSC2. This study serves to elucidate the mutation landscape and potential correlation between S-LAM genomic profiles and clinical phenotypes. METHODS: Genomic profiles of 22 S-LAM patients were obtained by sequencing genomic DNA and cell-free DNA from various specimens using an NGS (next-generation sequencing)-based tumor-driver gene panel. Detected mutations were summarized. Symptoms, serum vascular endothelial growth factor D (VEGF-D) values, pulmonary function, and six-minute walk distance (6MWD) were compared among groups with different TSC2 status and genotypes to analyze genotype-phenotype correlations. RESULTS: 67 Variants in 43 genes were detected, with a TSC2 mutation detection rate of 68.2%. The TSC2 detection rate was similar in specimens obtained either through transbronchial lung biopsy (TBLB) or surgical lung biopsy (70.0% vs. 69.2%, p > 0.05). A novel mutation in VEZF1 (c.A659G) was detected in four participants and may represent a mild disease state. TSC2 mutation was significantly related to a shorter 6MWD (p < 0.05), and a higher percentage of VEGF-D over 800 pg/mL (p < 0.05); stop-gain mutation was significantly related to a higher prevalence of pneumothorax. CONCLUSIONS: Tumor-driver mutations in genes other than TSC2 may have a role in S-LAM, and TBLB specimens are practical alternatives for genomic analysis. TSC2 mutation detectability and types are related to the disease severity and phenotypes of S-LAM.
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Proteínas de Ligação a DNA , Neoplasias Pulmonares , Linfangioleiomiomatose , Fatores de Transcrição , Proteína 2 do Complexo Esclerose Tuberosa , Ácidos Nucleicos Livres , Proteínas de Ligação a DNA/genética , Estudos de Associação Genética , Humanos , Neoplasias Pulmonares/genética , Linfangioleiomiomatose/genética , Mutação , Fatores de Transcrição/genética , Proteína 2 do Complexo Esclerose Tuberosa/genética , Fator D de Crescimento do Endotélio Vascular/genéticaRESUMO
Previous studies have shown a significant correlation between depression, resilience, self-efficacy, self-esteem and hope among schizophrenia patients, but few studies have examined the mechanisms among these factors. This study aimed to analyse the relationships among resilience, self-efficacy, self-esteem and hope as influencing factors of depression in schizophrenia patients. A cross-sectional study was conducted, and 361 schizophrenia patients were recruited. They were interviewed about their self-efficacy, self-esteem, hope, resilience and depressive symptoms. A structural equation model was used to examine the direct and indirect associations between them. The global model showed good model fit and the structural equation model revealed that the direct pathway of resilience on depression was significant, and the indirect pathway of self-efficacy, self-esteem and hope on depression via resilience was also significant. Notably, self-efficacy, self-esteem and hope did not directly affect depressive symptoms. These findings support the mediating model of self-efficacy, self-esteem and hope relevant to the depressive symptoms of schizophrenia patients in China, in which resilience plays a pivotal role. These findings elucidate the factors which may be associated with depressive symptoms and imply that enhancing resilience in people with schizophrenia could be beneficial. Moreover, studies with detailed longitudinal assessments are necessary to confirm these findings.
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Depressão/psicologia , Esperança , Resiliência Psicológica , Esquizofrenia , Psicologia do Esquizofrênico , Autoimagem , Autoeficácia , Adulto , China , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Objective To compare the efficacy and safety of tacrolimus with those of cyclosporine in treating idiopathic membranous nephropathy (IMN) via network meta-analysis. Methods Databases including PubMed,Embase,CENTRAL (Cochrane),Wanfang Database,CNKI,and VIP citation database were searched for relevant studies according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Package Meta 4.5.0 and Gemtc 0.8.1 in R 3.3.1 were used to analyze the included studies. Results In this network meta-analysis,the complete remission rate (RR=0.98,95% CI:0.70-1.40)and the total remission rate (RR=1.00,95% CI:0.90-1.20)of idiopathic membranous nephropathy did not differ significantly between IMN patients treated with cyclosporine A or tacrolimusand,nor did the incidences of hepatic dysfunction(RR=1.40,95% CI:0.52-4.00),infection(RR=0.75,95% CI:0.18-3.10),or gastrointestinal syndrome(RR=2.1,95% CI:0.36-28.00). Conclusion Cyclosporine A seems to have similar effectiveness and safety to tacrolimus in treating IMN.
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Ciclosporina/uso terapêutico , Glomerulonefrite Membranosa/tratamento farmacológico , Imunossupressores/uso terapêutico , Metanálise em Rede , Tacrolimo/uso terapêutico , HumanosRESUMO
INTRODUCTION: Stricture is a common complication in Crohn's disease (CD). Accurate identification of strictures that poorly respond to biologic therapy is essential for making optimal therapeutic decisions. The aim of this study was to determine the association between ultrasound characteristics of strictures and their therapeutic outcomes. METHODS: Consecutive CD patients with symptomatic strictures scheduled for biologic therapy were retrospectively recruited at a tertiary hospital. Baseline intestinal ultrasound was conducted to assess stricture characteristics, including bowel wall thickness, length, stratification, vascularity, and creeping fat wrapping angle. Patients were followed up for a minimum of 1 year, during which long-term outcomes including surgery, steroid-free clinical remission, and mucosal healing were recorded. Statistical analyses were performed. RESULTS: A total of 43 patients were enrolled. Strictures were located in the ileocecal region (39.5%), colon (37.2%), anastomosis (20.9%), and small intestine (2.3%). The median follow-up time was 17 months (interquartile range 7-25), with 27 patients (62.8%) undergoing surgery. On multivariant analysis, creeping fat wrapping angle > 180° (odds ratio: 6.2, 95% confidence interval [CI]: 1.1-41.1) and a high Limberg score (odds ratio: 2.3, 95% CI: 1.4-6.0) were independent predictors of surgery, with an area under the curve of 0.771 (95% CI: 0.602-0.940), accuracy of 83.7%, sensitivity of 96.3%, and specificity of 62.5%. On Cox survival analysis, creeping fat >180° was significantly associated with surgery (hazard ratio, 5.2; 95% CI: 1.2-21.8; P = 0.03). In addition, creeping fat was significantly associated with steroid-free clinical remission ( P = 0.015) and mucosal healing ( P = 0.06). DISCUSSION: Intestinal ultrasound characteristics can predict outcomes in patients with stricturing CD who undertook biologic therapy.
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Produtos Biológicos , Doença de Crohn , Ultrassonografia , Humanos , Doença de Crohn/diagnóstico por imagem , Doença de Crohn/tratamento farmacológico , Masculino , Feminino , Adulto , Estudos Retrospectivos , Constrição Patológica/diagnóstico por imagem , Constrição Patológica/etiologia , Produtos Biológicos/uso terapêutico , Resultado do Tratamento , Pessoa de Meia-Idade , Adulto Jovem , Infliximab/uso terapêutico , Seguimentos , Mucosa Intestinal/diagnóstico por imagem , Mucosa Intestinal/patologiaRESUMO
Background: The identification of multigland disease (MGD) in primary hyperparathyroidism (PHPT) patients is essential for minimally invasive surgical decision-making. Objective: To develop a nomogram based on ultrasound (US) findings and clinical factors to predict MGD in PHPT patients. Materials and methods: Patients with PHPT who had surgery between March 2021 and January 2022 were consecutively enrolled to this study. Biochemical and clinicopathological data were recorded. US images were analyzed to extract US features for prediction. Logistic regression analyses were used to identify MGD risk factors. A nomogram was constructed based on these factors and its performance evaluated by area under the receiver operating characteristic curve (AUC), calibration curve, Hosmer-Lemeshow tests, and decision curve analysis (DCA). Results: A total of 102 PHPT patients were included; 82 (80.4%) had single-gland disease (SGD) and 20 (19.6%) had MGD. Using multivariate analyses, MGD was positively correlated with age (odds ratio (OR) = 1.033, 95% confidence interval (CI): 0.190-4.047), PTH levels (OR = 1.001, 95% CI: 1.000-1.002), multiple endocrine neoplasia type 1 (MEN1) (OR = 29.730, 95% CI: 3.089-836.785), US size (OR = 1.198, 95% CI: 0.647-2.088), and US texture (cystic-solid) (OR = 5.357, 95% CI: 0.499-62.912). MGD was negatively correlated with gender (OR = 0.985, 95% CI: 0.190-4.047), calcium levels (OR = 0.453, 95% CI: 0.070-2.448), and symptoms (yes) (OR = 0.935, 95% CI: 0.257-13.365). The nomogram showed good discrimination with an AUC = 0.77 (0.68-0.85) and good agreement in predicting MGD in PHPT patients. Also, 65 points was recommended as a cut-off value, with specificity = 0.94 and sensitivity = 0.50. Conclusion: US was useful in evaluating MGD. Combining US and clinical features in a nomogram showed good diagnostic performance for predicting MGD.
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Hiperparatireoidismo Primário , Humanos , Hiperparatireoidismo Primário/complicações , Hiperparatireoidismo Primário/diagnóstico por imagem , Hiperparatireoidismo Primário/cirurgia , Estudos Retrospectivos , Curva ROC , NomogramasRESUMO
BACKGROUND: Lymphangioleiomyomatosis is a progressive diffuse cystic lung disease with approximately 85% survival at 10 years. The determinants of disease progression and mortality after the introduction of sirolimus therapy and vascular endothelial growth factor D (VEGF-D) as a biomarker have not been well defined. RESEARCH QUESTION: Which factors, including VEGF-D and sirolimus therapy, influence disease progression and survival prognosis in patients with lymphangioleiomyomatosis? STUDY DESIGN AND METHODS: The progression dataset and the survival dataset included 282 and 574 patients, respectively, from Peking Union Medical College Hospital, Beijing, China. A mixed-effects model was used to compute the rate of decline in FEV1, and generalized linear models were used to identify variables affecting FEV1 decline. A Cox proportional hazards model was used to explore the association between clinical variables and the outcomes of death or lung transplantation in patients with lymphangioleiomyomatosis. RESULTS: VEGF-D levels and sirolimus treatment were associated with FEV1 changes and survival prognosis. Compared with patients with VEGF-D of < 800 pg/mL at baseline, patients with VEGF-D of ≥ 800 pg/mL lost FEV1 faster (SE, -38.86 mL/y; 95% CI, -73.90 to -3.82 mL/y; P = .031). The 8-year cumulative survival rates of patients with VEGF-D of ≥ 2,000 pg/mL and < 2,000 pg/mL were 82.9% and 95.1%, respectively (P = .014). The generalized linear regression model also demonstrated the benefit of delaying the decline of FEV1 by 65.56 mL/y (95% CI, 29.06-102.06 mL/y) in patients treated with sirolimus compared with those without sirolimus (P < .001). The 8-year risk of death was reduced by 85.1% (hazard ratio, 0.149; 95% CI, 0.075-0.299) after sirolimus treatment. After inverse treatment probability weighting, the risks of death in the sirolimus group were reduced by 85.6%. CT scan results of grade III severity were associated with worse progression than results of grades I or II severity. Patients with baseline FEV1 of 70% predicted or St. George's Respiratory Questionnaire Symptoms domain 50 or higher predicted a higher risk of worse survival. INTERPRETATION: Serum VEGF-D levels, a biomarker of lymphangioleiomyomatosis, are associated with disease progression and survival. Sirolimus therapy is associated with slower disease progression and better survival in patients with lymphangioleiomyomatosis. TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT03193892; URL: www. CLINICALTRIALS: gov.
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Neoplasias Pulmonares , Linfangioleiomiomatose , Humanos , Linfangioleiomiomatose/tratamento farmacológico , Fator D de Crescimento do Endotélio Vascular/metabolismo , Sirolimo/uso terapêutico , Biomarcadores , Progressão da Doença , Neoplasias Pulmonares/tratamento farmacológicoRESUMO
Background: Although fatigue has been shown to be strongly associated with falls risk, very few studies have focused on its mechanism involved in community-dwelling older subjects. The purpose of this study was to explore the relationship between fatigue and falls risk and its internal mechanism by constructing a chain mediation model. Methods: A cross-sectional study design was adopted. A convenience sample of 270 older adults was recruited from July to October 2021 in an urban community, in Beijing, China. The participants completed the 14-item Fatigue Scale (FS-14), Falls Efficacy Scale International (FES-I), the Short Physical Performance Battery (SPPB) and Fall-Risk Self-Assessment Questionnaire (FRQ) to measure fatigue, falls efficacy, lower limb function and falls risk. The theory of unpleasant symptoms was used as a conceptual framework. Structural equation modeling (SEM) was utilized to test the hypothetical model. Results: The overall fit of final model was found to be satisfactory: χ2/df = 1.61, CFI = 0.971, TLI = 0.962, RMSEA = 0.049 (95% CI 0.030/0.066) and SRMR = 0.023. Fatigue had a direct effect on falls risk (ß = 0.559, S.E. = 0.089, 95% CI 0.380/0.731), and it also had indirect effects on falls risk (ß = 0.303, S.E. = 0.072, 95% CI 0.173/0.460) through mediating factors. Falls efficacy and lower limb function were the main mediating variables, and there was a chain mediating effect (ß = 0.015, S.E. = 0.010, 95% CI 0.003/0.046). Conclusions: Our study suggests that fatigue can influence falls risk among the elderly in China. There are many mediating paths between fatigue and falls risk. These results may help healthcare professionals to better understand the inherent relationship between fatigue and fall risk that may benefit older adults.
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Acidentes por Quedas , Fadiga , Idoso , China/epidemiologia , Estudos Transversais , Fadiga/epidemiologia , Humanos , Extremidade Inferior , Equilíbrio PosturalRESUMO
PURPOSE: To explore the rate of post-stroke disability and its associated factors in the third year following discharge from inpatient rehabilitation in Northeast China. DESIGN: A prospective cohort study. METHODS: A total of 522 persons who were hospitalized with a diagnosis of stroke were recruited consecutively between April 2015 and December 2015 and followed for 3 years. The primary outcome was disability, which was assessed using the Modified Barthel Index (MBI), a cutoff score of ≤95 indicates disability. Plausible risk factors of disability were selected from available variables to perform multivariate logistic regression analysis. FINDINGS: The proportion of post-stroke patients with disability decreased from 63.8% to 46.7% at 3-year follow-up. The factors associated with post-stroke disability were age, neurological deficits, cognitive function, depression, and social support. CONCLUSIONS/CLINICAL RELEVANCE: Disability continues to be a significant issue for individuals after a stroke, and community health workers should perform targeted assessments and interventions to decrease disability, and pay special attention to individuals who are at greatest risk of post-stroke disability.
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BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare disease that almost exclusively affects women of reproductive age. Patients are warned of the increased risks if they become pregnant. However, information on pregnancy in patients after the diagnosis of LAM is limited. METHODS: Patients were collected from the LAM registry study at Peking Union Medical College Hospital, Beijing, China. Patients with a history of pregnancy after the diagnosis of LAM were included. Medical records were reviewed, and baseline information and data during and after pregnancy were collected in May 2018. RESULTS: Thirty patients with a total of 34 pregnancies after the diagnosis of LAM were included. Livebirth, spontaneous abortion and induced abortion occurred in 10, 6 and 18 pregnancies, respectively. Sirolimus treatment was common (17/34). A total of 6/10, 5/6, and 6/18 patients with livebirths, spontaneous abortions, and induced abortions respectively, had a history of sirolimus treatment. Ten pregnancies (29.4%) had LAM-associated complications during pregnancy, including the exacerbation of dyspnea in 7 patients, pneumothorax in 3 patients (2 resulting in induced abortion and 1 successful parturition), and spontaneous bleeding of renal angiomyolipomas in 2 patients (both having successful parturition). No chylothorax was found during pregnancy. There were six pregnancies in six patients (17.6%) who had a history of livebirth after sirolimus treatment for LAM (all having successful parturition and healthy infants); two of these patients reported exacerbated dyspnea after parturition compared with before pregnancy. CONCLUSIONS: Patients with LAM, especially those taking sirolimus before pregnancy, were at a higher risk of spontaneous abortion. Complications such as pneumothorax, bleeding of renal angiomyolipoma, and exacerbated dyspnea during pregnancy were common. In patients without spontaneous abortion, sirolimus discontinuation before or during pregnancy did not lead to increased adverse neonatal outcomes.
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Angiomiolipoma , Neoplasias Renais , Neoplasias Pulmonares , Linfangioleiomiomatose , Feminino , Humanos , Lactente , Recém-Nascido , Gravidez , China , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/tratamento farmacológico , Linfangioleiomiomatose/diagnóstico , Linfangioleiomiomatose/tratamento farmacológicoRESUMO
BACKGROUND: The differential diagnosis of diffuse cystic lung disease (DCLD) is a clinical challenge. We wish to analyze the distribution of the etiology of DCLD based on data from a single lymphangioleiomyomatosis (LAM) clinic. METHODS: All DCLD patients at the LAM Clinic of Peking Union Medical College Hospital between January 2006 and December 2019 were analyzed. Information on the demographic, clinical, radiological, and pathological features was collected. RESULTS: A total of 1010 patients with DCLD on CT scan were evaluated. A sum of 711(70.4%) patients were diagnosed with definite or probable LAM. Other diagnoses included Birt-Hogg-Dubé syndrome (46), Sjogren's syndrome (38), pulmonary Langerhans cell histiocytosis (14), lung tumors (3), Castleman disease (2), antineutrophil cytoplasmic antibody-associated vasculitis (2), systemic lupus erythematosus (1), Marfan syndrome (1), amyloidosis (1), congenital cystic adenomatoid malformation of the lung (1), and pleuroparenchymal fibroelastosis (1). In the 38 patients diagnosed with Sjogren's syndrome, 2 were diagnosed with light-chain deposition disease, 2 were diagnosed with amyloidosis and 1 was diagnosed with lymphocytic interstitial pneumonia. One hundred and eighty-nine patients (18.7%) were undiagnosed. Lung biopsy results were available in 27 patients in the undiagnosed DCLD group but did not provide a diagnosis. CONCLUSION: Approximately 70% of DCLD patients in our LAM clinic had LAM. The common differential diagnoses included Birt-Hogg-Dubé syndrome, Sjogren's syndrome, and pulmonary Langerhans cell histiocytosis. Detailed clinical information and laboratory, genetic, and pathological investigations provide correct diagnoses in most patients with DCLD.