BACKGROUNDFibrosing mediastinitis (FM) is a rare disease. FM is thought to be related to prior granulomatous mediastinal infection, such as histoplasmosis or tuberculosis. The majority of cases have been reported in endemic regions for histoplasmosis. The characteristics of cases of FM in China, where the prevalence of tuberculosis is high, have not been reported. We analyzed the clinical, imaging, and bronchoscopic features of Chinese patients with FM to promote awareness of this disease.

METHODSBetween January 2005 and June 2015, twenty patients were diagnosed with FM in our hospital. Medical records and follow-up data were collected. Imaging and biopsy findings were reviewed by radiologists and pathologists.

RESULTSA total of 20 patients were analyzed (8 males and 12 females). The age ranged from 43 to 88 years with a mean age of 69.5 years. Previous or latent tuberculosis was found in 12 cases. Clinical symptoms included dyspnea (18/20), cough (17/20), expectoration (7/20), and recurrent pneumonia (3/20). Chest computed tomography scans showed a diffuse, homogeneous, soft tissue process throughout the mediastinum and hila with compression of bronchial and pulmonary vessels. Calcification was common (15/20). Pulmonary hypertension was present in 9 of 20 cases. Diffuse black pigmentation in the bronchial mucosa was frequently seen on bronchoscopy (12/13). The patients' response to antituberculosis treatment was inconsistent.

CONCLUSIONSFM in Chinese patients is most likely associated with tuberculosis. Some characteristics of FM are different from cases caused by histoplasmosis.

Adult ; Aged ; Aged, 80 and over ; Asian Continental Ancestry Group ; China ; Female ; Histoplasmosis ; complications ; diagnosis ; Humans ; Hypertension, Pulmonary ; diagnosis ; physiopathology ; Male ; Mediastinitis ; diagnosis ; etiology ; physiopathology ; Middle Aged ; Sclerosis ; diagnosis ; etiology ; physiopathology ; Tuberculosis ; diagnosis ; physiopathology

Portal hypertension as a consequence of liver cirrhosis is responsible for serious complications such as variceal bleeding, ascites and hepatic encephalopathy. Successful pharmacological treatment of portal hypertension can prevent the risk of the variceal bleeding, and contribute to reduce the morbidity and mortality in patients with liver cirrhosis. To identify the effect of drugs on portal hypertension, portal pressure was evaluated accurately before and after the drug administration. The hepatic venous pressure gradient has been accepted as the gold-standard method for assessing the severity of portal hypertension and the response to drug treatment. The mean hepatic venous pressure gradient was 15.1+/-5.4 mmHg in Korean cirrhotic patients who had experienced variceal bleeding. Non-selective beta blockers are the treatment of choice for primary and secondary prevention of variceal bleeding. The dose of propranolol should be subsequently adjusted until the resting heart rate had been reduced by 25% or less than 55 beats per minute. It has been reported that the optimal dose of propranolol is variable due to racial differences in cardiovascular receptor sensitivity. In Korean patients with portal hypertension and liver cirrhosis, the mean required dose of propranolol to reach target heart rate was 165 mg (range; 80-280 mg). This review covers mainly the results of the pharmacological therapy of portal hypertension in Korean cirrhotic patients.
Adrenergic beta-Antagonists/administration & dosage ; Hepatic Veins ; Humans ; Hypertension, Portal/diagnosis/*drug therapy/physiopathology ; Korea ; Liver Cirrhosis/complications/physiopathology ; Propranolol/administration & dosage ; Venous Pressure/drug effects

Tei-Index is a useful echocardiographic parameter for evaluating global cardiac function. Conventionally, a value and b value, which the calculation of Tei index is based on, are measured in different cardiac cycle. Therefore, accuracy of Tei index is damaged by cycle dependent variation, especially in patients with arrhythmia. The newly established Dual Doppler modality allows a synchronous measurement of both a value and b value. This study was aimed to explore the value of Tei index measured by Dual Doppler in cardiac function estimation in patients with hypertension. One hundred and forty patients with hypertension were recuited and divided into three groups according to cardiac function. The a value and b value were generated from the transmitral and transaortic flow spectrum of the same cardiac cyle by Dual Doppler modality. The results showed that Tei index was significantly higher in the cardiac dysfunction group than in the control group. There was a high correlation between Tei index and NYHA cardiac performance. Tei index measured by Dual Doppler modality is a highly reproducible parameter with high sensitivity and specificity for cardiac function estimation, even for patients with arrthymia.
Adult ; Aged ; Echocardiography, Doppler, Color ; Female ; Humans ; Hypertension ; complications ; diagnostic imaging ; physiopathology ; Male ; Middle Aged ; Ventricular Dysfunction, Left ; diagnosis ; etiology ; physiopathology ; Ventricular Function ; Young Adult

OBJECTIVE: To explore the change in ambulatory blood pressure monitoring (ABPM) value and the sympathetic nervous system (SNS) level in children with primary nephrotic syndrome(PNS) and their relationship. METHODS: ABPM and casual blood pressure(CBP) were tested in 114 children with PNS and 12 normal children as a control group. The 24-h urine noradrenaline(NA), adrenaline(A) and dopamine(DA) content were detected through high-performance liquid chromatography with electrochemical luminescence and the correlation with ABP was analyzed. RESULTS: Among 114 children with PNS, 101 had elevated blood pressure (88.6%), 45 showed high incidence of masked hypertension (39.5%), and 80 non-dipper blood pressure (70.2%). Systolic blood pressure level and blood pressure load were greater than diastolic blood pressure. NA, A, and DA levels of the PNS group were significantly higher than those of the control group, while those of the elevated blood pressure group were significantly higher than those of the normal blood pressure group in PNS children. SNS levels were positively correlated with blood pressure levels and blood pressure load, and negatively correlated with night BP decreasing rates. CONCLUSION Children with PNS have high incidence of hypertension with large proportion of masked hypertension and non-dipper blood pressure. Severe masked hypertension classification should be set up. In PNS children, SNS activity is elevated that might evaluate the blood pressure level and decrease blood pressure circadian rhythm.
Adolescent ; Blood Pressure ; physiology ; Blood Pressure Monitoring, Ambulatory ; Case-Control Studies ; Child ; Child, Preschool ; Female ; Humans ; Hypertension ; diagnosis ; etiology ; Male ; Nephrotic Syndrome ; complications ; physiopathology ; Sympathetic Nervous System ; physiopathology

Portal hypertension is a severe consequence of chronic liver diseases and is responsible for the main clinical complications of liver cirrhosis. Hepatic venous pressure gradient (HVPG) measurement is the best available method to evaluate the presence and severity of portal hypertension. Clinically significant portal hypertension is defined as an increase in HVPG to >10 mmHg. In this condition, the complications of portal hypertension might begin to appear. HVPG measurement is increasingly used in the clinical fields, and the HVPG is a robust surrogate marker in many clinical applications such as diagnosis, risk stratification, identification of patients with hepatocellular carcinoma who are candidates for liver resection, monitoring of the efficacy of medical treatment, and assessment of progression of portal hypertension. Patients who had a reduction in HVPG of > or =20% or to < or =12 mmHg in response to drug therapy are defined as responders. Responders have a markedly decreased risk of bleeding/rebleeding, ascites, and spontaneous bacterial peritonitis, which results in improved survival. This review provides clinical use of HVPG measurement in the field of liver disease.
Chronic Disease ; Hemodynamics ; Hemorrhage/etiology ; Hepatic Veins/physiology ; Humans ; Hypertension, Portal/complications ; Liver Cirrhosis/diagnosis ; Liver Diseases/complications/*physiopathology ; Portal Pressure

OBJECTIVETo evaluate the predictive value of portal vein flow rate preoperative for portal vein thrombosis (PVT) after periesophagastric devascularization in hepatitis B cirrhosis-related portal hypertension.

METHODSFrom January 2007 to July 2008, 45 patients with portal hypertension caused by hepatitis B cirrhosis were performed splenectomy with peri-esophagogastric devascularization in the same medical group in West China Hospital of Sichuan University. The portal vein flow rate and the diameter of portal vein were measured with doppler sonography respectively before and after the operation. At the same time, the level of PT and PLT were detected. The weight of spleens were measured after operation.

RESULTSThirteen cases suffered from PVT postoperatively. Portal vein flow rate was significantly lower in patients with PVT postoperation than that in patients without PVT (P < 0.01). In patients with PVT (n = 13) postoperation, the preoperative portal vein flow rate was (19.5 +/- 5.3) cm/s. Among the 13 cases, there were 12 cases whose flow rate were lower than 25 cm/s, and 1 case whose flow rate was 32. 3 cm/s; In patients without PVT (n = 32), the preoperative portal vein flow rate was (9.6 +/- 8.0) cm/s. In patients with lower rate (n = 17), the incidence rate of PVT was 70.6%; in patients with higher rate (n = 28), the incidence rate of PVT was 3.6%. The incidence rate of PVT in patients with lower rate was significantly lower than patients with higher rate (P < 0.01). The diameter of portal vein in patients with PVT was significantly wider than patients without PVT. The diameter of portal vein was negative correlative with the portal vein flow rate. The value 25 cm/s was of diagnostic efficiency, the sensitivity was 92.3%, and specificity was 70.6%.

CONCLUSIONSThe portal vein flow rate preoperative can be used as an early predictor of portal vein thrombosis after periesophagastric devascularization in hepatitis B cirrhosis-related portal hypertension to give a guide to clinical work.

Adult ; Aged ; Blood Flow Velocity ; Female ; Humans ; Hypertension, Portal ; etiology ; physiopathology ; surgery ; Liver Cirrhosis ; complications ; Male ; Middle Aged ; Portal Vein ; diagnostic imaging ; physiopathology ; Postoperative Complications ; diagnosis ; etiology ; Preoperative Care ; Risk Factors ; Splenectomy ; Ultrasonography ; Venous Thrombosis ; diagnosis ; etiology

No abstract available.
Chronic Disease ; Cryopyrin-Associated Periodic Syndromes/*complications/diagnosis ; Gonioscopy ; Humans ; *Intraocular Pressure ; Male ; Microscopy, Acoustic ; Middle Aged ; Ocular Hypertension/diagnosis/*etiology/physiopathology ; Rare Diseases

It is known that blood pressure variability (BPV) can independently affect target organ damage (TOD), even with normal blood pressure. There have been few studieson chronic kidney disease (CKD) patients. We evaluated the relationship between BPV and TOD in a cross-sectional, multicenter study on hypertensive CKD patients. We evaluated 1,173 patients using 24-hr ambulatory blood pressure monitoring. BPV was defined as the average real variability, with a mean value of the absolute differences between consecutive readings of systolic blood pressure. TOD was defined as left ventricular hypertrophy (LVH) (by the Romhilt-Estes score > or =4 in electrocardiography) and kidney injury (as determined from an estimated glomerular filtration rate [eGFR]<30 mL/min/1.73 m2 and proteinuria).The mean BPV of the subjects was 15.9+/-4.63 mmHg. BPV displayed a positive relationship with LVH in a univariate analysis and after adjustment for multi-variables (odds ratio per 1 mmHg increase in BPV: 1.053, P=0.006). In contrast, BPV had no relationship with kidney injury. These data suggest that BPV may be positively associated with LVH in hypertensive CKD patients.
Adult ; Aged ; Blood Pressure/*physiology ; Blood Pressure Monitoring, Ambulatory ; Cross-Sectional Studies ; Electrocardiography ; Female ; Glomerular Filtration Rate ; Humans ; Hypertension/*complications/*diagnosis ; Hypertrophy, Left Ventricular/*physiopathology ; Kidney/injuries ; Male ; Middle Aged ; Odds Ratio ; Proteinuria/complications ; Renal Insufficiency, Chronic/*complications/*diagnosis

No abstract available.
Adult ; Angiography, Digital Subtraction ; Angioplasty, Balloon ; *Blood Pressure ; Female ; Fibromuscular Dysplasia/*complications/diagnosis ; Humans ; Hypertension, Renovascular/diagnosis/*etiology/physiopathology/therapy ; Renal Artery Obstruction/diagnosis/*etiology/physiopathology/therapy ; Treatment Outcome

BACKGROUND/AIMS: The relationships between the hepatic venous pressure gradient (HVPG) and the status of cirrhosis, complications of portal hypertension and the severity of cirrhosis are not clear. The aim of this study was to determine the relationships between HVPG and the complications or status of cirrhosis. METHODS: The HVPG, gastroesophageal varices, Child-Pugh score, Model for End-Stage Liver Disease (MELD) score, presence of ascites, recent bleeding history and the status of cirrhosis were assessed in a cohort of 172 patients (156 males, 16 females) with liver cirrhosis. RESULTS: The HVPG was 15.6+/-5.1 (mean+/-SD) mmHg (4-33 mmHg) and was significantly higher in patients in the decompensated stage than in those in the compensated stage (16.6+/-4.3 vs. 10.8+/-6.1 mmHg, respectively; P<0.01). HVPG was higher in bleeders than in nonbleeders (16.9+/-4.5 vs. 12.8+/-5.3 mmHg, respectively; P<0.01), and in patients with ascites than in those without ascites (16.4+/-4.1 vs. 14.5+/-6.2 mmHg, respectively; P<0.05). HVPG was significantly lower in the presence of gastric varices than in their absence (14.0+/-3.4 vs. 16.0+/-5.3 mmHg, respectively; P<0.05); however, no significant correlation was detected between HVPG and the grade of esophageal varices (P>0.05). HVPG was significantly higher in Child's B cirrhosis (n=87, 15.6+/-4.7 mmHg) and Child's C cirrhosis (n=36, 18.4+/-4.7 mmHg) than in Child's A cirrhosis (n=49, 13.7+/-5.1 mmHg; P<0.01). HVPG also was strongly correlated with the MELD score (P<0.01). The time required to measure the HVPG was 11.2+/-6.4 min, and only three cases of minor complication occurred during the procedure. CONCLUSIONS: HVPG was correlated with the severity of liver cirrhosis, presence of ascites, and risk of variceal bleeding in patients with liver cirrhosis.
Adult ; Ascites/*complications ; Cohort Studies ; Data Interpretation, Statistical ; Esophageal and Gastric Varices/*complications/diagnosis ; Female ; Hepatic Veins/*physiopathology ; Humans ; Hypertension, Portal/complications/*physiopathology ; Korea ; Liver Cirrhosis/complications/*diagnosis/physiopathology ; Male ; Middle Aged ; Predictive Value of Tests ; ROC Curve ; Severity of Illness Index ; Venous Pressure