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BACKGROUND: To examine the safety and effectiveness of proactive tracheoplasty for pediatric ring-sling complexes. METHODS: We retrospectively collected the data from 304 children who were diagnosed with a ring-sling complex and underwent surgery at three cardiac centers in China between January 2010 and June 2023. Children were categorized into three surgical groups: concurrent sling and tracheal surgery (Group A, n=258), staged sling and tracheal surgery (Group B, n=25), and sling-only surgery (Group C, n=21). We compared perioperative clinical characteristics, tracheal morphology changes, and outcomes across the groups. RESULTS: The median age of the children was 1.2 (IQR: 0.7-1.9) years. The anomalous tracheobronchial arborization rates were higher in Groups A (52.5%) and B (60.0%) than in Group C (15.0%). The preoperative narrow-wide ratio (NWR) was lower in Groups A and B than in Group C, with values of 0.44 (IQR: 0.35-0.52), 0.44 (0.33-0.59), and 0.68 (0.54-0.72), respectively (P<0.001). Preoperative subcarina angles were similar among the groups (P=0.54). After specific surgeries, the NWR and subcarina angle significantly improved in Groups A and B but not in Group C. There were seven in-hospital deaths and two post-discharge deaths. Respiratory symptoms improved in Groups A and B but seven children in Group C remained respiratory dysfunction. Six children presented with residual stenosis of the left pulmonary artery. CONCLUSION: Concurrent sling and tracheal surgeries for children with the ring-sling complex are safe and effective and are especially preferable for those with NWR ≤0.6, long-segment or diffuse tracheal stenosis, anomalous tracheobronchial arborization, and pronounced respiratory symptoms.
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BACKGROUND: Sarcopenia is commonly seen in the older adults and increases in incidence with age, also in Parkinson's disease (PD). Although research has indicated that the development of sarcopenia in patients with PD may be related to both motor symptoms and non-motor symptoms (NMS), the precise relationship between the two conditions remains unclear. Therefore, we aimed to investigate the incidence of sarcopenia in patients with PD and its association with NMS. METHODS: The study included 123 patients with PD and 38 age- and sex-matched healthy controls (HC). All participants were evaluated for sarcopenia using the 2019 Asian Sarcopenia Diagnostic Criteria, and patients with PD underwent standard assessments of motor symptoms and NMS. Multiple logistic regression and receiver operating characteristic (ROC) curve analyses were used to examine the association between sarcopenia and NMS in patients with PD. RESULTS: The incidence of sarcopenia was significantly higher in patients with PD than in HC (26.8% vs. 10.4%, p = 0.046). Multiple logistic regression analysis revealed that poorer sleep quality (odds ratio [OR]: 1.245; 95% confidence interval [CI]: 1.011-1.533; p = 0.040) and fatigue (OR: 1.085, 95% CI: 1.006-1.170, p = 0.034) were independently associated with sarcopenia. ROC analysis indicated that the optimal cut-off value for Pittsburgh Sleep Quality Index (PSQI) scores was 10, with 72.7% sensitivity and 74.4% specificity (area under the curve [AUC] = 0.776, 95% CI: 0.683-0.868, p < 0.001). The optimal cut-off value for Fatigue Severity Scale (FSS) scores was 39, with 87% sensitivity and 50% specificity (AUC = 0.725, 95% CI: 0.629 -0.820, p < 0.001). Joint use of FSS and PSQI scores increased the predictive value for sarcopenia(AUC = 0.804, 95% CI: 0.724-0.885, p < 0.001). CONCLUSION: Patients with PD are more susceptible to sarcopenia than healthy older adults, and fatigue and poorer sleep are positively associated with sarcopenia. Further longitudinal studies are needed to clarify the causal relationships.
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Enfermedad de Parkinson , Sarcopenia , Humanos , Anciano , Estudios Transversales , Enfermedad de Parkinson/complicaciones , Enfermedad de Parkinson/diagnóstico , Enfermedad de Parkinson/epidemiología , Pueblos del Este de Asia , Sarcopenia/diagnóstico , Sarcopenia/epidemiología , FatigaRESUMEN
BACKGROUND: The interaction between tumor microenvironment (TME) and tumors offers various targets in mounting anti-tumor immunotherapies. However, the prognostic biomarkers in endometrial carcinoma (EC) are still limited. Here, we aimed to analyze the TME features and identify novel prognostic biomarkers for EC. METHODS: ESTIMATE, CIBERSORT, protein-protein interaction (PPI) network, univariate and multivariate Cox regression, and functional enrichment analysis were performed to identify immune- and survival-related hub genes as well as possible molecular mechanisms. The limma package and deconvolution algorithm were adopted to estimate the abundance of tumor-infiltrating immune cells (TICs) and their relationship with the target gene. In the validation section, tissue microarrays (TMAs) of EC and multiplex immunohistochemistry (m-IHC) were evaluated to validate the expression of TNFRSF4, and its correlation with immune markers, including CD4, CD8, and FOXP3. Besides, the receiver operating characteristic (ROC) curve was plotted to determine the diagnostic performance of TNFRSF4, CD4, CD8, and FOXP3 in EC. RESULTS: Two genes, TNFRSF4 and S1PR4, were screened out from 386 intersection differential expression genes (DEGs) shared by ImmuneScore and StromalScore in EC. Highlighted by TNFRSF4, we found that it was not only positively correlated with the TICs (mainly CD4+ T cells, CD8+ T cells, and Tregs) but significantly related to the prognosis in patients of EC, both verified by data from The Cancer Genome Altas (TCGA)-EC database and clinical samples. At the same time, the expression trend of TNFRSF4 was further confirmed by an integrated meta-analysis based on six microarrays from the Gene Expression Omnibus database (GEO). CONCLUSIONS: Collectively, TNFRSF4, a previously unrecognized key player in EC, could serve as a potential biomarker for prognosis prediction and immunomodulation of EC.
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Neoplasias Endometriales , Regulación Neoplásica de la Expresión Génica , Biomarcadores de Tumor/genética , Biomarcadores de Tumor/metabolismo , Linfocitos T CD8-positivos/metabolismo , Neoplasias Endometriales/patología , Femenino , Factores de Transcripción Forkhead/metabolismo , Humanos , Inmunomodulación/genética , Pronóstico , Receptores OX40/genética , Receptores OX40/metabolismo , Microambiente Tumoral/genéticaRESUMEN
PURPOSE: This study aimed to examine whether the different tumor-transplanted sites could construct a similar immunoinflammatory microenvironment and to investigate the interactions between tumor microenvironment cells. METHODS: The red fluorescent protein-SU3 (SU3-RFP) or SU3 glioma stem cells (GSC) were inoculated into the brain, liver, abdominal cavity, and subcutis of green fluorescent protein (GFP)-nude mice. The tumor tissues were taken to observe the tissue cell distribution. The single cell suspension of tumor tissues was prepared and cultured, while the SU3-RFP cells were co-cultured with the cells from GFP-transgenic mice. The RFP+, GFP+, and RFP+/GFP+ cells were traced by fluorescence microscope, and their protein expressions were determined by Western blot analysis. The markers of immunoinflammatory cells, including F4/80, CD11b, CD11c, CD80, CD47, and SIRP-α, were determined by RT-PCR and immunocytochemistry assays, respectively. RESULTS: The xenograft models of all transplant sites were inducible, and the red tumor cells of tumor tissues were encircled by a great quantity of host-derived green cells, including immunoinflammatory cells with CD80, F4/80, CD11b, and CD11c expressions, which might generate the cell colonies and possess the pseudopodia. Additionally, the interactions between red tumor cells and green immunoinflammatory cells, including cell fusion process and yellow fusion cell formation, were observed in cultured cells. The fusion cells-derived B4 cells with expressions of CD47 and SIRP-α proteins had the strong proliferation ability and tumorigenic effect. CONCLUSIONS: The similar tumor immunoinflammatory microenvironment was constructed by GSC in different transplant sites, and the cell fusion indicated a malignant transformation of the tumor microenvironment cells.
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Transformación Celular Neoplásica/inmunología , Glioma/inmunología , Inflamación/inmunología , Células Madre Neoplásicas/inmunología , Microambiente Tumoral/inmunología , Animales , Apoptosis , Comunicación Celular , Fusión Celular , Proliferación Celular , Transformación Celular Neoplásica/metabolismo , Transformación Celular Neoplásica/patología , Técnicas de Cocultivo , Glioma/metabolismo , Glioma/patología , Proteínas Fluorescentes Verdes/metabolismo , Humanos , Inflamación/metabolismo , Inflamación/patología , Proteínas Luminiscentes/metabolismo , Ratones , Ratones Endogámicos BALB C , Ratones Desnudos , Ratones Transgénicos , Células Madre Neoplásicas/metabolismo , Células Madre Neoplásicas/patología , Células Tumorales Cultivadas , Ensayos Antitumor por Modelo de Xenoinjerto , Proteína Fluorescente RojaRESUMEN
OBJECTIVE: To investigate the association between the hypertriglyceridemic waist (HTGW) phenotype and prediabetes in Chinese adults aged 40 years and older. METHODS: 12757 adults (4101 men and 8656 women) without diabetes or cardiovascular and cerebrovascular diseases, free of using lipid-modified agents, were enrolled in this cross-sectional study. The HTGW phenotype was defined as elevated serum triglyceride concentrations and enlarged waist circumference. A two-hour post 75 g oral glucose tolerance test was performed in all participants. Multiple logistic regression analysis was used to evaluate the relationship of the HTGW phenotype with prediabetes. RESULTS: Individuals with the HTGW phenotype had a higher adjusted odds ratio (OR: 1.70; 95% CI: 1.48-1.95) of prediabetes than those without the phenotype. There existed a strong relationship of the HTGW phenotype with impaired glucose tolerance (IGT) (OR: 1.83; 95% CI: 1.57-2.13), but not with impaired fasting glucose (IFG) (OR: 0.87; 95% CI: 0.65-1.17). Only women with the HTGW phenotype are significantly associated with the combined IFG and IGT (OR: 1.83; 95% CI: 1.28-2.62). CONCLUSIONS: The HTGW phenotype was a useful risk indicator and a practical screening tool to benefit in the early diagnosis and intervention for prediabetes, particularly for IGT and the combined IFG and IGT.
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Cintura Hipertrigliceridémica/complicaciones , Estado Prediabético/complicaciones , Circunferencia de la Cintura , Adulto , Anciano , Pueblo Asiatico , China , Estudios Transversales , Femenino , Intolerancia a la Glucosa , Prueba de Tolerancia a la Glucosa , Humanos , Cintura Hipertrigliceridémica/etnología , Lípidos/química , Masculino , Persona de Mediana Edad , Oportunidad Relativa , Fenotipo , Estado Prediabético/etnología , Prevalencia , Análisis de Regresión , Factores de Riesgo , Triglicéridos/sangreRESUMEN
Sellar malignant tumors are uncommon and usually reported as metastatic diseases from breast or lung cancers. Spindle cell carcinoma (SCC) is a rare malignancy and has been found in breast,oral cavity,lungs,kidneys,and hepatobiliary pancreatic system but not in sellar region. We report here the first case of isolated sellar SCC with aggressive features in Peking Union Medical College Hospital. This patient was referred to our hospital on September 9,2015 and discharged on October 16,2015. We described the clinical manifestations,imaging findings,and pathological features of this rare disease.
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Carcinoma/diagnóstico , Carcinoma/patología , Silla Turca/patología , HumanosRESUMEN
A 48-year-old woman was admitted with 15-mo history of abdominal pain, diarrhea and hematochezia, and 5-mo history of defecation difficulty. She had been successively admitted to nine hospitals, with an initial diagnosis of inflammatory bowel disease with stenotic sigmoid colon. Findings from computed tomography virtual colonoscopy, radiography with meglumine diatrizoate, endoscopic balloon dilatation, metallic stent implantation and later overall colonoscopy, coupled with the newfound knowledge of compound Qingdai pill-taking, led to a subsequent diagnosis of ischemic or toxic bowel disease with sigmoid colon stenosis. The patient was successfully treated by laparoscopic sigmoid colectomy, and postoperative pathological examination revealed ischemic or toxic injury of the sigmoid colon, providing a final diagnosis of drug-induced sigmoid colon stenosis. This case highlights that adequate awareness of drug-induced colon stenosis has a decisive role in avoiding misdiagnosis and mistreatment. The diagnostic and therapeutic experiences learnt from this case suggest that endoscopic balloon expansion and colonic metallic stent implantation as bridge treatments were demonstrated as crucial for the differential diagnosis of benign colonic stenosis. Skillful surgical technique and appropriate perioperative management helped to ensure the safety of our patient in subsequent surgery after long-term use of glucocorticoids.
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Colon Sigmoide/efectos de los fármacos , Constricción Patológica/diagnóstico , Diarrea/diagnóstico , Medicamentos Herbarios Chinos/efectos adversos , Enfermedades Inflamatorias del Intestino/diagnóstico , Obstrucción Intestinal/diagnóstico , Pitiriasis Rosada/tratamiento farmacológico , Dolor Abdominal/etiología , Dolor Abdominal/terapia , Antibacterianos/uso terapéutico , Biopsia , Colectomía/métodos , Colon Sigmoide/diagnóstico por imagen , Colon Sigmoide/patología , Colon Sigmoide/cirugía , Colonografía Tomográfica Computarizada , Colonoscopía/instrumentación , Colonoscopía/métodos , Estreñimiento/etiología , Constricción Patológica/inducido químicamente , Constricción Patológica/complicaciones , Constricción Patológica/terapia , Medios de Contraste/administración & dosificación , Diagnóstico Diferencial , Diarrea/etiología , Diarrea/microbiología , Diatrizoato de Meglumina/administración & dosificación , Dilatación/métodos , Femenino , Fluidoterapia , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/terapia , Humanos , Obstrucción Intestinal/inducido químicamente , Obstrucción Intestinal/complicaciones , Obstrucción Intestinal/terapia , Laparoscopía/métodos , Levofloxacino/uso terapéutico , Persona de Mediana Edad , Stents Metálicos AutoexpandiblesRESUMEN
BACKGROUND: Several previous studies have shown that snoring is associated with glucose metabolism and the development of diabetes, but rare study has shown the association between snoring frequency and prediabetes, particularly in China. We hypothesized that individuals who snore might have a higher risk of prediabetes. This study aimed to investigate the association between self-reported snoring and prediabetes in a Chinese population. METHODS: A cross-sectional study was performed in three large communities of Beijing from December 2011 to August 2012 by recruiting individuals aged ≥40 years old. All participants were requested to complete a detailed questionnaire and undergo anthropometric measurements. A 75 g oral glucose tolerance test was performed in individuals without diabetes. Blood samples of all participants were collected; blood glucose and blood fat levels were measured. Multivariate logistic regression models were built to assess the association between snoring frequency and prediabetes. RESULTS: A total of 13,592 participants (female: 66.56%; mean age: 56.8 ± 7.9 years; mean body mass index: 25.5 ± 3.4 kg/m2) were included in the final analysis. Of these, 30.9% were diagnosed with prediabetes, while 41.3% and 25.4% had occasional and habitual snoring, respectively. Habitual snoring was associated with an increased risk of prediabetes (odds ratio [OR]: 1.3, 95% confidence interval [CI]: 1.1-1.4, P< 0.001), after adjusting for diabetes and sleep-related confounders in the multivariable models. Habitual snoring was also associated with isolated impaired fasting glucose (IFG; OR: 1.3, 95% CI: 1.0-1.6; P< 0.001) and isolated impaired glucose tolerance (IGT; OR: 1.3, 95% CI: 1.2-1.5; P< 0.001), but not IFG + IGT (OR: 1.1, 95% CI: 0.9-1.4; P = 0.281). When stratified by total cholesterol (TC) levels, this association between habitual snoring and prediabetes was observed only in individuals with TC <5.6 mmol/L (OR: 1.4, 95% CI: 1.2-1.6; P< 0.001). CONCLUSIONS: Habitual snoring is associated with prediabetes, but only in individuals with TC <5.6 mmol/L. Further prospective studies are needed to confirm this finding.
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Diabetes Mellitus/epidemiología , Estado Prediabético/epidemiología , Ronquido/epidemiología , Adulto , Glucemia/metabolismo , Colesterol/sangre , Estudios Transversales , Diabetes Mellitus/sangre , Ayuno/sangre , Femenino , Intolerancia a la Glucosa/sangre , Intolerancia a la Glucosa/epidemiología , Intolerancia a la Glucosa/etiología , Humanos , Masculino , Persona de Mediana Edad , Oportunidad Relativa , Estado Prediabético/sangre , Estado Prediabético/etiología , Autoinforme , Ronquido/sangre , Ronquido/complicacionesRESUMEN
Objective To investigate the effects of miR-125a-5p on cell proliferation,apoptosis and cell cycle of pancreatic cancer cells.Methods The expression level of miR-125a-5p in pancreatic cancer was determined using quantitative real-time polymerase chain reaction analysis in 4 pairs of pancreatic cancer tissues and matched adjacent normal tissues samples. The expression of miR-125a-5p was downregulated in pancreatic cancer cell lines by transfection with miR-125a-5p inhibitor. Cell counting kit-8 assays was conducted to detect the growth ability of pancreatic cancer cell lines. Flow cytometry was applied to detect the cell cycle and apopotosis. Soft agar colony formation test was employed to assess the role of miR-125a-5p in process of malignant transformation.Results MiR-125a-5p was significantly highly expressed in pancreatic ductal adenocarcinoma tissues than adjacent normal tissues(P<0.05). After the expression level of miR-125a-5p in Panc-1 and MIA PaCa-2 was downregulated,the growth ability was suppressed(P<0.05),early apopotosis rate was promoted by 13.6% and 11.0% respectively(P<0.05),the amount of colony formation was reduced by 27.3% and 27.8%,respectively(P<0.05),and the percentage of S stage of Panc-1 was reduced by 11.8% (P<0.05).Conclusions The expression of miR-125a-5p is high in pancreatic ductal adenocarcinoma tissues. After the expression level of miR-125a-5p is downregulated,the growth ability,colony formation,and cell cycle of Panc-1 and MIA PaCa-2 are suppressed,and the early apopotosis rate will be promoted. Therefore,miR-125a-5p may play an oncogenic role in pancreatic ductal adenocarcinoma.
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Apoptosis , Carcinoma Ductal Pancreático/patología , Ciclo Celular , MicroARNs/metabolismo , Neoplasias Pancreáticas/patología , Línea Celular Tumoral , Proliferación Celular , Transformación Celular Neoplásica , Regulación hacia Abajo , Regulación Neoplásica de la Expresión Génica , Humanos , MicroARNs/genéticaRESUMEN
OBJECTIVE: Patients with malignant insulinoma always present with symptoms of severe hypoglycemia and have poor life expectancy. In addition, inoperable metastatic malignant insulinomas are very difficult to manage. The aim of this report is to present our successful experiences in diagnosis and treatment of this disease in 6 patients. PATIENTS/METHODS: Six patients (male 2, female 4) with malignant insulinomas were admitted into our hospital. Their clinical histories, including clinical presentations, endocrine evaluations, radiological images, pathological examination and treatments, were reviewed. RESULTS: The diagnosis of malignant insulinoma combined with liver metastases was confirmed in all patients by endocrine evaluation and radiological images. Patients 1-3 underwent surgical management. The primary and metastasized tumors were completely resected. After successful surgery, no hypoglycemia recurred. Patients 4-6 did not undergo surgery because of systemic disease and poor health. Instead, they were administrated with diazoxide 50 mg Three Times a Day (TID), with final doses up to 200-300 mg TID. These 3 patients had good responses to diazoxide administration. After treatment, the frequency and severity of hypoglycemia were improved significantly. All 6 patients had better life quality than previously expected. CONCLUSION: Combination of surgical and medical approaches can improve life quality and prolong survival of patients with malignant insulinomas.
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Insulinoma/patología , Neoplasias Pancreáticas/patología , Adulto , Diazóxido/uso terapéutico , Diuréticos/uso terapéutico , Femenino , Humanos , Hipoglucemia/tratamiento farmacológico , Hipoglucemia/etiología , Insulinoma/cirugía , Insulinoma/terapia , Neoplasias Hepáticas/patología , Neoplasias Hepáticas/secundario , Masculino , Persona de Mediana Edad , Neoplasias Pancreáticas/cirugía , Neoplasias Pancreáticas/terapia , Estudios Retrospectivos , Adulto JovenRESUMEN
BACKGROUND: Parathyroid hormone (PTH) stimulates aldosterone secretion in human adrenocortex and is regulated by the renin-angiotensin-aldosterone system. We speculated that measurement of PTH may be a valuable aid in the diagnosis of aldosterone-producing adenoma (APA). METHODS: To test this hypothesis, we recruited 142 patients with adrenal adenoma, of whom 84 had an APA and 58 had a nonfunctioning adrenal adenoma (NFA). Plasma levels of intact PTH, serum potassium, sodium, calcium, phosphate, 25(OH) vitamin D, plasma aldosterone concentration (PAC), plasma renin activity (PRA), and aldosterone to renin ratio (ARR) were measured in every patient. Computed tomography (CT) scanning of the adrenal gland and adrenal hormone levels was used to evaluate the function of the adrenal adenoma. We also evaluated the impact of renin-angiotensin-aldosterone system (RAAS) components on PTH from the recumbent-upright test in 15 patients with APA and 30 patients with NFA. RESULTS: Compared with NFA, PTH levels were significantly increased in patients with APA, and serum calcium and phosphate were significantly decreased. When position was changed from supine to upright, the variation in PTH levels was significantly higher in APA patients compared with NFA patients. Receiver operator characteristic (ROC) curves identified the Youden index, which corresponded to the best tradeoff of combined marker (ARR and PTH) with a sensitivity and specificity of 89.3% and 93.1%, respectively. CONCLUSIONS: The baseline and positional variation of serum PTH levels were significant in APA, thus PTH may be a promising auxiliary index for the clinical diagnosis of APA.
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Adenoma/diagnóstico , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Aldosterona/metabolismo , Biomarcadores de Tumor/sangre , Biomarcadores de Tumor/metabolismo , Hormona Paratiroidea/sangre , Adenoma/sangre , Adenoma/metabolismo , Neoplasias de las Glándulas Suprarrenales/sangre , Neoplasias de las Glándulas Suprarrenales/metabolismo , Neoplasias de las Glándulas Suprarrenales/orina , Adulto , Aldosterona/sangre , Aldosterona/orina , Área Bajo la Curva , Biomarcadores de Tumor/orina , Técnicas Electroquímicas , Femenino , Humanos , Masculino , Persona de Mediana Edad , Posicionamiento del Paciente , Valor Predictivo de las Pruebas , Curva ROC , Reproducibilidad de los Resultados , Posición Supina , Tomografía Computarizada por Rayos X , Regulación hacia ArribaRESUMEN
BACKGROUND: Reninoma is an extremely rare renal tumor characterized by excessive renin secretion causing secondary hypertension and hypokalemia. Reninoma is a benign and highly manageable lesion if it is discovered early and removed surgically. METHODS: We report six cases of reninoma and provide a literature review on this rare disease, highlighting the diagnostic evaluation and follow-up of each patient. RESULTS AND CONCLUSIONS: Reninoma should be considered in young adults with elevated renin activity and refractory hypertension. Imaging studies and selective venous catheterization are often helpful in identifying the lesion. In most cases of reninoma presenting with renin-mediated hypertension, conservative surgical treatment should be considered to remove the small, superficial lesion.
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Hipertensión/fisiopatología , Renina/fisiología , Adolescente , Adulto , Femenino , Humanos , Masculino , Adulto JovenRESUMEN
Large-cell neuroendocrine carcinoma (LCNEC) of the gallbladder is extremely rare. We present a 63-year-old Chinese female who was admitted with right upper quadrant pain and a quasi-circular tumor measuring 2.0 cm on the body of the gallbladder, as indicated by computed tomography. LCNEC combined with adenocarcinoma was immunohistochemically confirmed after open radical cholecystectomy. Postoperative recovery of this patient was uneventful, and no evidence of recurrence or metastasis was observed after 12 months of follow-up. LCNEC of the gallbladder is thought to be extremely rare and is usually found in combination with other histological carcinoma types, such as adenocarcinoma, as determined histologically. The prognosis is poor overall, but early detection with complete resection may result in a relatively good prognosis.
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Adenocarcinoma/patología , Carcinoma de Células Grandes/patología , Carcinoma Neuroendocrino/patología , Neoplasias de la Vesícula Biliar/patología , Neoplasias Primarias Múltiples/patología , Adenocarcinoma/cirugía , Carcinoma de Células Grandes/cirugía , Carcinoma Neuroendocrino/cirugía , Femenino , Neoplasias de la Vesícula Biliar/cirugía , Humanos , Persona de Mediana Edad , Neoplasias Primarias Múltiples/cirugía , Pronóstico , Tomografía Computarizada por Rayos XRESUMEN
Lipodystrophies is a really rare group of diseases characterized by altered body fat amount and/or repartition and serious insulin resistance.We reported a lean Chinese girl with acquired generalized lipodystrophy, who had a long history of poorly controlled diabetes mellitus (DM) despite with extremely high dose insulin (6 u/kg/d) therapy, combined with severe hypertriglyceridemia and acanthosis nigricans. The differential diagnosis of Lipodystrophies should be considered in lean patients presenting with early onset DM, combined with serious insulin resistance.
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BACKGROUND: The intraatrial conduit (IAC) Fontan procedure is one of the Fontan modifications and is usually not considered the first choice. In this large series, we report our experience of the IAC Fontan procedure for the treatment of a functional single ventricle and review its indications, techniques, and clinical outcomes. METHODS: Between 2009 and 2013, 101 patients with a functional single ventricle underwent an IAC Fontan procedure. The median surgical age was 44 months and weight was 15 kg. The cardiac malformations included tricuspid atresia in 10, double-inlet left ventricle in 8, double-inlet right ventricle in 15, double-outlet right ventricle in 18, congenitally corrected transposition of the great arteries in 11, complete atrioventricular septal defect in 8; criss-cross in 1, pulmonary atresia with intact ventricular septum in 3, Ebstein anomaly in 3, and others in 3. Heterotaxy syndrome was found in 21 patients. An intraatrial polytetrafluoroethylene conduit was implanted to construct the Fontan pathway. In 75 patients, a 2.7-mm to 4.5-mm fenestration was made to reduce the intraconduit pressure. Eighty-three patients had previously undergone a Glenn operation. Median follow-up was 29 months (range, 2 to 60 months). Doppler echocardiography, electrocardiography, and Holter monitoring were used to evaluate hemodynamic performance and arrhythmias. RESULTS: There were 2 hospital deaths, 1 Fontan takedown, and 3 midterm deaths. Conduit thrombosis developed in 1 patient. Atrial flutter developed in 2 patients, who underwent electrical cardioversion. Junctional bradycardia developed in 2 patients, but they did not require permanent pacemakers. Overall survival was 97.0% at 1 year and 94.1% at 5 years. CONCLUSIONS: The IAC modification provides excellent operative and midterm outcomes in most patients with a functional single ventricle. However, a longer follow-up time is required to demonstrate its real advantages.
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Procedimiento de Fontan/métodos , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/cirugía , Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Arteria Pulmonar/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Vena Cava Inferior/cirugíaRESUMEN
OBJECTIVE: In children with coronary disease, clinical decision should be based on detailed measurements of the coronary arteries by two-dimensional echocardiography. We aimed to establish coronary artery reference indexed diameter and z scores regression equations in a large cohort of Chinese Han children. METHODS: We measured the diameter of the proximal right (RCA), left main (LMCA), left anterior descending, and left circumflex coronary artery, and of the aortic annulus, and calculated the coronary-aorta index (coronary artery-to-aortic annulus ratio) in 506 Chinese Han children with normal hearts whose ages ranged from 1 day to 18 years. Regression analyses were performed, relating the coronary artery dimensions to body surface area (BSA). Several models were used, and the best model (yielding the maximum adjusted R(2) ) was chosen to establish a z score calculator. RESULTS: Based on cubic regression, (M) = ß0 + ß1 × BSA + ß2 × BSA(2) + ß3 × BSA(3) , the adjusted R(2) values were 0.515, 0.553, 0.505, and 0.518 for the RCA, LMCA, left anterior descending, and left circumflex coronary artery models, respectively. RCA/aortic annulus was 0.14 ± 0.02 (range, 0.07-0.24) and LMCA/AOA was 0.15 ± 0.03 (range, 0.10-0.28). CONCLUSIONS: Our results provide reference values of coronary artery z scores, regression equation, and coronary-aorta index as a quick guide to determine coronary dilation in Chinese Han children.
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Vasos Coronarios/anatomía & histología , Adolescente , Aorta/anatomía & histología , Aorta/diagnóstico por imagen , Pesos y Medidas Corporales , Niño , Preescolar , China , Estudios de Cohortes , Vasos Coronarios/diagnóstico por imagen , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Valores de Referencia , UltrasonografíaRESUMEN
Early serous carcinoma in fallopian tube or serous tubal intraepithelial carcinoma (STIC), an early lesion limited to the epithelium of the fallopian tube and firstly identified from specimen obtained by prophylactic salpingo-oophorectomy, has provided insight into pelvic high grade serous carcinoma (HGSC). Increasing evidence indicates that STIC is a likely precursor for HGSC and several studies have focused on this lesion and its clinical significance. This review addresses recent advances in recognizing STIC and its correlation with HGSC and ovarian carcinogenesis. It also describes evidence regarding the fallopian tube as a source of some HGSCs, the protocol for optimizing histological evaluation of the tubes, the spectrum of tubal lesions from benign to noninvasive carcinoma, changes in diagnostic criteria from purely morphologic characteristics to a combination of morphologic features and molecular biomarkers, and new studies about potential biomarkers. However, the direct evidence regarding STIC as the precursor of HGSC is still tantalizing due to other possibilities that may also explain the origin of pelvic HGSC. Further molecular genetic studies are required to address this important question.
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Carcinoma in Situ/patología , Cistadenocarcinoma Seroso/patología , Neoplasias de las Trompas Uterinas/patología , Femenino , HumanosRESUMEN
Primary gastric B-cell non-Hodgkin's lymphoma (B-NHL) has a similar morphocytological presentation to severe chronic gastritis, which complicates the pathological diagnosis of this disease. To investigate the practicality and utility of the BIOMED-2 standardized primer system for the diagnosis of primary gastric B-NHL from endoscopic biopsy specimens, we selected 65 cases of archived paraffin-embedded primary gastric B-NHL specimens as well as 27 cases of severe chronic gastritis samples to serve as a negative control group. The positivity rates of immunoglobulin heavy chain (IgH) gene rearrangements detected by the BIOMED-2 standardized primer system for the two groups were 86.4% and 12.0%, respectively, which are significantly different (p < 0.05). Importantly, the combined detection of the five groups of the IgH primer system increased the detection rate of B-NHL. These findings indicate that the BIOMED-2 standardized primer system is suitable for formalin-fixed and paraffin-embedded (FFPE) specimens and is valuable as a secondary diagnostic tool for primary gastric B-NHL as well as the differential diagnosis of severe chronic gastritis.