Hereditary hemochromatosis is a rare autosomal genetic disorder that can cause multi-organ dysfunction in the liver,pancreas,spleen,heart and pituitary gland,with diverse clinical manifestations,make the diagnosis difficult.In recent years,with the deepening of clinical understanding and the development of genetic diagnosis tools,the diagnostic rate of this disease has increased significantly.In this paper,we report a case of hereditary hemochromatosis type 3 involving multiple organs and complicated by severe heart failure,aiming to improve the clinicians'understanding of this disease and reduce the leakage and misdiagnosis.

BACKGROUND: Ventricular remodeling after acute anterior wall ST-segment elevation myocardial infarction (AAMI) is an important factor in occurrence of heart failure which additionally results in poor prognosis. Therefore, the treatment of ventricular remodeling needs to be further optimized. Compound Danshen Dripping Pills (CDDP), a traditional Chinese medicine, exerts a protective effect on microcirculatory disturbance caused by ischemia-reperfusion injury and attenuates ventricular remodeling after myocardial infarction. OBJECTIVE: This study is designed to evaluate the efficacy and safety of CDDP in improving ventricular remodeling and cardiac function after AAMI on a larger scale. METHODS: This study is a multi-center, randomized, double-blind, placebo-controlled, parallel-group clinical trial. The total of 268 patients with AAMI after primary percutaneous coronary intervention (pPCI) will be randomly assigned 1:1 to the CDDP group (n=134) and control group (n=134) with a follow-up of 48 weeks. Both groups will be treated with standard therapy of ST-segment elevation myocardial infarction (STEMI), with the CDDP group administrating 20 tablets of CDDP before pPCI and 10 tablets 3 times daily after pPCI, and the control group treated with a placebo simultaneously. The primary endpoint is 48-week echocardiographic outcomes including left ventricular ejection fraction (LVEF), left ventricular end-diastolic volume index (LVEDVI), and left ventricular end-systolic volume index (LVESVI). The secondary endpoint includes the change in N terminal pro-B-type natriuretic peptide (NT-proBNP) level, arrhythmias, and cardiovascular events (death, cardiac arrest, or cardiopulmonary resuscitation, rehospitalization due to heart failure or angina pectoris, deterioration of cardiac function, and stroke). Investigators and patients are both blinded to the allocated treatment. DISCUSSION This prospective study will investigate the efficacy and safety of CDDP in improving ventricular remodeling and cardiac function in patients undergoing pPCI for a first AAMI. Patients in the CDDP group will be compared with those in the control group. If certified to be effective, CDDP treatment in AAMI will probably be advised on a larger scale. (Trial registration No. NCT05000411).
Humans ; ST Elevation Myocardial Infarction/therapy* ; Stroke Volume ; Ventricular Remodeling ; Prospective Studies ; Microcirculation ; Ventricular Function, Left ; Myocardial Infarction/etiology* ; Treatment Outcome ; Percutaneous Coronary Intervention/adverse effects* ; Heart Failure/drug therapy* ; Drugs, Chinese Herbal/therapeutic use* ; Randomized Controlled Trials as Topic ; Multicenter Studies as Topic

OBJECTIVE: To evaluate the feasibility and tolerability of metoprolol standard dosing pathway (MSDP) in Chinese patients with acute coronary syndrome (ACS). METHODS: In this multicenter, prospective, open label, single-arm and interventional study that was conducted from February 2018 to April 2019 in fifteen Chinese hospitals. A total of 998 hospitalized patients aged ≥ 18 years and diagnosed with ACS were included. The MSDP was applied to all eligible ACS patients based on the standard treatment recommended by international guidelines. The primary endpoint was the percentage of patients achieving the target dose at discharge (V2). The secondary endpoints included the heart rate and blood pressure at V2 and four weeks after discharge (V4), and percentage of patients experiencing bradycardia (heart rate < 50 beats/min), hypotension (blood pressure < 90/60 mmHg) and transient cardiac dysfunction at V2 and V4. RESULTS: Of the 998 patients, 29.46% of patients achieved the target dose (≥ 95 mg/d) at V2. The total population was divided into two groups: target group (patients achieving the target dose at V2) and non-target group (patients not achieving the target dose at V2). There was significant difference in the reduction of heart rate from baseline to discharge in the two groups (-4.97 ± 11.90 beats/min vs. -2.70 ± 9.47 beats/min, P = 0.034). There was no significant difference in the proportion of bradycardia that occurred in the two groups at V2 (0 vs. 0, P = 1.000) and V4 (0.81% vs. 0.33%, P = 0.715). There was no significant difference in the proportion of hypotension between the two groups at V2 (0.004% vs. 0.004%, P = 1.000) and V4 (0 vs. 0.005%, P = 0.560). No transient cardiac dysfunction occurred in two groups during the study. A total of five adverse events (1.70%) and one serious adverse event (0.34%) were related to the pathway in target group. CONCLUSIONS In Chinese ACS patients, the feasibility and tolerability of the MSDP have been proved to be acceptable.

Humans ; Heart Atria ; Biopsy ; Surgical Instruments ; Catheters ; Lymphoma/pathology*

Objective: To analyze the long-term outcome of unoperated Ebstein's anomaly (EA) patients aged over 18 years, and to evaluate the related factor of outcomes. Methods: The data of 48 unoperated EA patients from March 2004 to December 2008 in the First Hospital of Tsinghua University, were analyzed. The clinical data of the patients were collected, and patients received regular echocardiography, ECG and chest X-ray examinations. Septal leaflet attachment ratio (SLAr) was calculated based on transthoracic echocardiography imagines. The patients were divided into 3 groups according to SLAr: SLAr<0.45 (n=18), 0.45≤SLAr≤0.60 (n=21) and SLAr>0.60 (n=9). Chest X-ray was used for measurement of cardiothoracic ratio (CTR). Kaplan Meier survival curve was used to calculate the long-term survival rate. Cox proportional hazards regression model was used to analyze the influencing factors of death. Results: There were 19 males, and the mean age at diagnosis was (21.3±11.1) years. Forty-two patients (87.5%) were complicated with arrhythmia, including W-P-W syndrome (n=4), supraventricular tachycardia (n=16), right bundle branch block (n=37), and atrial fibrillation (n=2). The mean duration of follow-up was (148.8±16.8) months, the follow-up rate was 100% with no loss-to-follow up. Nine cases (18.8%) died during follow-up: 6 cases (12.5%) died of cardiac origin, including 3 cases of heart failure, 1 case of arrhythmia, and 2 cases of sudden death; 1 case died of accident; 2 cases died from unknown causes. During the follow-up period, the survival rates were 17/18, 19/21 (90.5%) and 3/9 in the SLAr<0.45, 0.45≤SLAr≤0.60 and SLAr>0.60 group, respectively. According to Kaplan-Meier survival curve, the 5-year survival rates among the three groups were 100%, 100% and 78%, respectively. The 10-year survival rates among the three groups were 94%, 95% and 44%, respectively. Decreased activity tolerance and heart failure were found in 7 patients (6 patients in SLAr>0.60 group and 1 patient in 0.45≤SLAr≤0.60 group). Two patients had cerebrovascular embolism. There were 3 cases with tachyarrhythmia lasting more than 24 hours. Cox regression analysis showed that the risk of death was higher in patients with SLAr>0.60 than in patients with SLAr<0.45 (HR=12.375, 95%CI 1.692-22.146, P=0.015); the risk of death in patients with CTR≥0.65 was 1.306 times higher than that in patients with CTR<0.65 (HR=1.306, 95%CI 0.417-12.754, P=0.038). Conclusions: EA patients often combines with arrhythmia. For unoperated EA patients, SLAr>0.60 and CTR≥0.65 are risk factors of death. EA patients with arrhythmia should be actively treated with drugs or radiofrequency ablation.

Coinfection/drug therapy* ; HIV ; HIV Infections/drug therapy* ; Hepacivirus ; Hepatitis B/drug therapy* ; Hepatitis C/drug therapy* ; Humans ; Prevalence

OBJECTIVE: To study the correlation between fractional exhaled nitric oxide (FeNO) and airway reversibility in children with IgE-mediated asthma. METHODS: A total of 86 children, aged 6-14 years, who were initially diagnosed with acute attack of asthma from September 2016 to August 2018 were enrolled as subjects. According to the results of serum specific IgE, they were divided into IgE mediated group with 61 children and non-IgE mediated group with 25 children. According to the results of allergen detection, the IgE mediated group was further divided into four groups with one, two, three, and four or more positive allergens. FeNO and the parameters of pulmonary ventilation function before and after dilation test were measured. Pearson correlation analysis was used to evaluate the correlation of FeNO with each parameter of pulmonary function. RESULTS: The IgE mediated group had significantly higher FeNO than the non-IgE mediated group (P<0.05). FeNO increased with the increase in the number of positive serum specific allergens (P<0.05). In the IgE mediated group, FeNO level was positively correlated with the change in forced expiratory volume in the first second (FEV1) and the improvement in percentage of predicted FEV1 after medication in bronchial dilation test (r=0.655 and 0.473 respectively, P<0.05). The FeNO level was not correlated with FEV1, percentage of predicted FEV1, peak expiratory flow (PEF), change in PEF after medication, percentage of predicted PEF (PEF%pred), and improvement in PEF%pred after medication (P>0.05). In the non-IgE mediated group, FeNO level was not correlated with the above indicators (P>0.05). CONCLUSIONS FeNO level is associated with the degree of allergies. For children with IgE-mediated asthma, FeNO is positively correlated with airway reversibility, which has a certain value in the diagnosis of asthma, disease evaluation, and understanding of airway reversibility. For children with non-IgE-mediated asthma, FeNO cannot be used to evaluate airway reversibility. These two types of asthma should be treated differently.
Adolescent ; Asthma ; Breath Tests ; Child ; Forced Expiratory Volume ; Humans ; Immunoglobulin E ; Nitric Oxide ; Respiratory Function Tests

OBJECTIVE: To systematic evaluate the outcome of open-wedge high tibial osteotomy(OWHTO) and unicomartmental knee arthroplasty (UKA) in treating medial compartment osteoarthritis of the knee. METHODS: According to the retrieval strategy made by the Cochrane collaboration, a computer-base research of Medline, Pubmed, EMbase, Cochrane Library, CBM, CNKI, and Wanfang databases was performed and search deadline was March 2018. Related Chinese and English orthopedic journals and conference papers were manually searched. Controlled studies of OWHTO and UKA in the treatment of medial knee osteoarthritis were included. The quality of included researches was evaluated, and the data of postoperative knee function, complications, total knee arthroplasty(TKA) revision rates, and postoperative pain were extracted. Meta analysis was performed using the RevMan 5.0 software. RESULTS: A total of 8 articles that met the criteria were included containing a total of 675 patients. Meta-analysis showed that there was no significant difference in postoperative HSS score, knee score, functional score, and Lysholm score between the OWHTO and UKA groups(=0.32, =0.87, =0.22, =0.53). The range of joint motion in the OWHTO group was better than that in the UKA group, and the difference was statistically significant(=0.009). There was no significant difference in postoperative complications and the rates of revision to TKA between the two groups(=0.81, =0.23). There was no difference in postoperative knee pain between the two groups. CONCLUSIONS In the treatment of medial compartmental osteoarthritis of the knee that meets the surgical indications, OWHTO had better postoperative joint mobility. The results were similar in postoperative knee score, postoperative complications, and postoperative TKA revision rates between OWHTO and UKA groups.
Arthroplasty, Replacement, Knee ; Humans ; Knee Joint ; Osteoarthritis, Knee ; Osteotomy ; Tibia ; Treatment Outcome

Vacuum sealing drainage (VSD) is frequently used in abdominal surgeries. However, relevant guidelines are rare. Chinese Trauma Surgeon Association organized a committee composed of 28 experts across China in July 2017, aiming to provide an evidence-based recommendation for the application of VSD in abdominal surgeries. Eleven questions regarding the use of VSD in abdominal surgeries were addressed: (1) which type of materials should be respectively chosen for the intraperitoneal cavity, retroperitoneal cavity and superficial incisions? (2) Can VSD be preventively used for a high-risk abdominal incision with primary suture? (3) Can VSD be used in severely contaminated/infected abdominal surgical sites? (4) Can VSD be used for temporary abdominal cavity closure under some special conditions such as severe abdominal trauma, infection, liver transplantation and intra-abdominal volume increment in abdominal compartment syndrome? (5) Can VSD be used in abdominal organ inflammation, injury, or postoperative drainage? (6) Can VSD be used in the treatment of intestinal fistula and pancreatic fistula? (7) Can VSD be used in the treatment of intra-abdominal and extra-peritoneal abscess? (8) Can VSD be used in the treatment of abdominal wall wounds, wound cavity, and defects? (9) Does VSD increase the risk of bleeding? (10) Does VSD increase the risk of intestinal wall injury? (11) Does VSD increase the risk of peritoneal adhesion? Focusing on these questions, evidence-based recommendations were given accordingly. VSD was strongly recommended regarding the questions 2-4. Weak recommendations were made regarding questions 1 and 5-11. Proper use of VSD in abdominal surgeries can lower the risk of infection in abdominal incisions with primary suture, treat severely contaminated/infected surgical sites and facilitate temporary abdominal cavity closure.
Abdomen ; surgery ; China ; Drainage ; methods ; Evidence-Based Medicine ; Humans ; Practice Guidelines as Topic ; Societies, Medical ; organization & administration ; Surgical Wound Infection ; prevention & control ; Traumatology ; organization & administration ; Vacuum

Electrocardiography ; Emergency Medical Services ; Humans ; Myocardial Infarction ; Out-of-Hospital Cardiac Arrest ; therapy ; Percutaneous Coronary Intervention ; ST Elevation Myocardial Infarction ; therapy ; Treatment Outcome