2016年 · 第44卷第09期

In recent years, more and more people are enthusiastic about investing in hospitals. Many people are saying that medical care is very profitable. Although many hospitals are positioned as private non-profit hospitals, investors still look forward to three-year returns. Five years is too long, so they seize the day. Many hospitals rely on false advertisements to lobby for patients everywhere, compete with public hospitals for resources, and strive for the share of national medical insurance. Overall, these hospitals are unsuccessful. Medical treatment is benevolent, benevolent and benevolent. The core of the hospital is service, care, medical safety and quality. If you don't pay attention to these, it will be difficult to succeed.

Senile weakness is a more and more serious problem in the medical service system. Attaching importance to the diagnosis and treatment of senile weakness will help to improve the quality of life of the elderly.

A 19-year-old female was admitted to our department due to "repeated chest tightness and chest pain for more than 4 months, accompanied by several episodes of Aspen syndrome". On the morning of May 6, 2015, the patient suffered from sudden chest tightness and chest pain, and then lost consciousness, accompanied by foaming at the mouth and twitching of limbs. He was given cardiopulmonary resuscitation, and monitoring showed ventricular fibrillation. After defibrillation, he suffered multiple consecutive cardiac arrests, and then rescued and restored sinus rhythm. After waking up with aphasia, right limb hemiplegia, right upper and lower limb muscle strength grade 0. CT of head and neck showed a large area of brain swelling in the left frontotemporal and occipital lobes, with a high possibility of cerebral embolism, and was treated with dehydration and intracranial pressure. On May 18th, the patient was admitted to the brain surgery department of our hospital, and there was no obvious abnormality in whole brain angiography. On June 7, the patient suffered from sudden precordial pain and lost consciousness with convulsions. Electrocardiogram showed ventricular fibrillation after extensive ST segment depression, and third-degree atrioventricular block after cardiopulmonary resuscitation, and a temporary pacemaker was implanted. After that, the patients suffered from cardiac arrest and electromechanical separation on June 12, 20 and 24, respectively. All patients were rescued by chest compression and their sinus rhythm was restored. Physical examination: aphasia, right upper limb muscle strength grade 0, lower limb muscle strength grade 3, but no special findings. Combined with the ST-segment changes at the onset, it is considered that the onset of Aspen syndrome is caused by cardiac ischemia caused by coronary artery spasm. On June 25, dual-source CT of the coronary artery showed that the proximal lumen of the right coronary artery (RCA) was approximately occluded about 4.73 cm (Figure 1)； Coronary angiography showed no abnormalities in the left coronary artery and complete proximal occlusion of the right coronary artery (Figure 2)。 Three stents were implanted in the right coronary artery, and ventricular fibrillation and cardiac arrest did not reoccur.

A 65-year-old female was hospitalized on May 22, 2015 due to "intermittent chest tightness, shortness of breath for 1 year and aggravation for 1 month". The local hospital once diagnosed "valvular heart disease", but after treatment improved, no systemic treatment was performed. The patient had no history of hypertension, diabetes and infectious diseases. Physical examination after admission: body temperature 36.6 ℃, pulse 80 beats/min, breathing 14 beats/min, blood pressure 106/74 mmHg (1 mmHg =0.133 kPa); Reclining position; There was no bulge in the precordial area, no deformity in the thorax, normal apical beat, no tremor and pericardial friction sound, slightly enlarged heart boundary, 80 beats/min heart rate, uniform heart rhythm, and no pathological murmur in the auscultation area of the heart; Dry and wet rales were not heard in both lungs; No enlargement of liver and spleen was observed; No depressed edema was observed in both lower limbs. On the second day of admission, blood routine showed 4.08×10 white blood cells⁹/L, lymphocytes 1.39×10⁹/L, red blood cells 3.91×10¹²/L, hemoglobin 118 g/L, platelets 171×10⁹/L, and the erythrocyte sedimentation rate was 16 mm/1 h. There were no abnormalities in the ECG. Lateral chest X-ray showed a round high-density shadow of the left atrium (Figure 1)。 Echocardiography showed a heterogeneous strong echo of 3.9 cm ×2.6 cm in the left atrium, considering left atrial myxoma. Chest CT showed a ring-like dense shadow in the left atrial area (Figure 2)。

A 61-year-old male was admitted to the Department of Cardiology of our hospital in April 2015 due to "recurrent palpitations for 9 months". Past health, no bad habits such as tobacco and alcohol, and family history of cardiovascular diseases. Physical examination at admission: blood pressure 160/75 mmHg (1 mmHg =0.133 kPa), no enlargement of superficial lymph nodes and thyroid glands, coarse breathing sounds in both lungs, and no obvious dry and wet rales; The heart boundary was slightly enlarged to the left, the heart rhythm was uniform, the heart rate was 89 beats/min, and no murmur was heard in each valve area. Thyroid function test: free triiodothyronine: 5.45 pmol/L (reference value 3.1-6.8 pmol/L), free thyroxine: 19.17 pmol/L (reference value 12-22 pmol/L), thyrotropin: 0.76 mIU/L (reference value 0.72-4.2 mIU/L); There were no obvious abnormalities in blood routine and biochemistry. Holter electrocardiogram showed paroxysmal atrial fibrillation (AF), and catheter ablation of AF was proposed. Preoperative transthoracic echocardiography showed: the left atrial diameter was increased (anteroposterior diameter 44 mm), and no obvious spontaneous development was found in it. An irregular mass was detected near the opening of the left atrial appendage, about 22 mm ×16 mm ×9 mm in size, with uneven echo, large base, irregular edges, and poor mobility (Figure 1)； Left atrial mural thrombosis is considered. Therefore, catheter ablation was postponed, and low molecular weight heparin calcium and warfarin bridging anticoagulation were given successively. Patients were followed up in outpatient clinic, and the international standardized ratio (INR) was 2.3~2.7 after three reexaminations. One month later, the patient was re-admitted due to fever (body temperature 38.5 ℃). Chest CT showed infection of the lower lobes of both lungs, multiple enlarged lymph nodes in the mediastinum, the largest lymph node diameter was about 1.6 cm; The abnormal mass volume in the left atrium was about 23 mm ×19 mm ×12 mm, which was larger than that of the preoperative echocardiography, and no abnormality was found in the tumor marker examination. The patient's body temperature returned to normal after anti-inflammatory and anticoagulant treatment in the hospital. Due to the increased frequency of atrial fibrillation attacks, the patient was transferred to the surgical department to undergo "thrombectomy + bipolar radiofrequency ablation". During the operation, a hard mass about 25 mm ×20 mm ×18 mm in size was seen near the left atrial appendage, with a large base and uneven surface. The rapid pathology during the operation suggested that it was a "malignant tumor", so radiofrequency ablation was abandoned, and the tumor was extensively dissected in the atrium. Postoperative routine paraffin section was diagnosed as "ALK-negative anaplastic large cell lymphoma" (Figure 2)； Immunophenotype detection: CD3 ε (+), CD30 (+), PCK (-), ALK (-), CD79a (-), CD20 (-), MPO (-), bc16 (-), Ki67 positive rate 85%. PCR detection suggests clonal rearrangement of TCR γ gene, which supports the above pathological diagnosis. It was recommended that the patient continue to visit the hematology or oncology department, but the patient refused and was automatically discharged, followed up by telephone, and died 4 months later.

A 45-year-old female was admitted to the hospital on 16 September 2015 due to "intermittent palpitations, syncope for 6 years, and the defibrillator electrode was found exposed for 1 month". The patient began to have recurrent palpitations and syncope 6 years ago. The electrocardiogram showed non-persistent ventricular tachycardia and prolonged QT interval, and was diagnosed as long QT syndrome. A V-196 implantable cardioverter defibrillator (ICD) manufactured by St. Jude, USA was implanted using a Riata dual-coil passive ICD electrode (Model 1570-65). After implantation, the patient did not redevelop syncope, and the ICD was repeatedly discharged 8 times. One month ago, it was found that the ICD battery was depleted at follow-up, and the silicone tube of the ICD electrode in the right atrium was ruptured under X-ray fluoroscopy. He was admitted to our hospital for further diagnosis and treatment. Physical examination after admission: the vital signs were stable, an old surgical scar about 5 cm long was visible under the right clavicle, the breathing sounds of both lungs were clear, the heart rhythm was uniform, no murmur was heard in the auscultation area of each valve, the abdominal physical examination was negative, and there was no edema in both lower limbs. No abnormalities were found in routine preoperative laboratory tests.

A 33-year-old female was admitted to the hospital due to "fever for 2 weeks, aggravated with swelling and pain of hands and feet for 1 d". He had a history of congenital atrial septal defect for 28 years. He underwent atrial septal defect closure in an external hospital 2 years ago. He reported that he recovered well after operation. Physical examination at admission showed that the body temperature was as high as 38.5 ℃, 19 breaths/min, blood pressure 107/67 mmHg (1 mmHg =0.133 kPa), and pulse 85 beats/min. The breathing sounds of both lungs were thick, and scattered dry rales could be heard. No murmur and additional heart sounds could be heard in the auscultation areas of each valve. The back of the left hand and the right first toe and the surrounding soft tissues were moderately swollen, non-depressed, with obvious redness and swelling, local skin temperature increased, and positive tenderness. Generally in poor condition. Laboratory tests showed a white blood cell count of 5.99×10⁹/L, neutrophil ratio 82%, hemoglobin 117 g/L, hypersensitive C-reactive protein 14 mg/L, blood bacterial culture Streptococcus agalactiae positive once. Fasting blood glucose was 16.61 mmol/L after admission, 2h postprandial blood glucose was 21.26 mmol/L, and random fingertip blood glucose fluctuated from 25 to 35 mmol/L. Multi-slice CT of the lung showed that multiple patchy, striped and nodular high-density shadows could be seen in both lungs, with unclear boundaries. The volume and density of some lung tissues in the middle lobe of the right lung, the lingual lobe of the left lung and the lower lobe of both lungs decreased. Localized low-density shadows were observed in the upper lobe of the right lung and the lower lobe of the left lung. No obvious enlarged lymph node shadow was found in the mediastinum. The occluder shadow can be seen in the atrial septum. CT diagnosis was inflammation of both lungs, and partial atelectasis of middle lobe of right lung, lingual lobe of left lung and lower lobe of both lungs; Localized emphysema of the upper lobe of the right lung and the lower lobe of the left lung. Preoperative transthoracic echocardiography (TTE) showed strong echo of the occluder in the atrial septum, and the position of the occluder was fixed. Color Doppler ultrasound (CDFI) showed a small amount of transeptal blood flow signal at the upper edge of the occluder. A substantial irregular isoechoic attachment was observed on the right atrial side of the occluder, about 14 mm ×9 mm, with little activity with the cardiac cycle (Figure 1)。 Echocardiographic diagnosis was abnormal echo on the right atrial side of the occluder, without excluding the formation of neoplasms. Intraoperative esophageal echocardiography (TEE) showed strong echo of the occluder in the atrial septum with fixed position, a small amount of transseptal blood flow signal on the upper edge of the occluder, substantial mass-like isoecho attachment on the right atrial surface of the occluder, about 19 mm ×15 mm, with little activity with the cardiac cycle, and equivalent substantial echo attachment on the left atrial surface of the occluder, about 6 mm ×7 mm, with greater activity with the cardiac cycle.

pulmonary vasoconstriction, remodeling and thrombosis in situ play an important role in the occurrence and development of pulmonary arterial hypertension (PAH). Pulmonary vasoconstriction is mainly caused by the imbalance of vasoconstriction and relaxation factors, while pulmonary vascular remodeling is related to many factors. Studies have found that inflammation and immune abnormalities, endothelial damage, genetic factors (BMPR2, ALK1, CAV1, KCNK3, SMAD9, EIF2AK4 gene mutations)^[1], endothelial-derived growth factor, iron deficiency^[2]estrogen^[3]Pulmonary artery smooth muscle cells and endothelial cells apoptosis and proliferation imbalance^[4]And so on may cause pulmonary vascular remodeling. However, the pathogenesis of PAH is not completely clear at present. Exploring the new pathogenesis of PAH will provide a scientific basis for the development of new targeted drugs.

pulmonary arterial hypertension (PAH) is a type of pulmonary vascular disease characterized by progressive increase in pulmonary artery pressure and gradual increase in pulmonary vascular resistance, which eventually leads to right heart failure and death. Genetics is closely related to this disease, and seven PAH-causing genes have been discovered. However, the pathogenic genes of PAH are incomplete and different, which suggests that genetic modification factors are involved in the regulation of the occurrence and development of PAH. In-depth understanding of the genetic modification factors of PAH not only helps to elucidate the genetic and pathological mechanisms of disease, but may also suggest new targets for disease treatment. This paper mainly summarizes the latest research results of modification factors in the field of PAH genetics, and summarizes the main genetic modification factors according to their pathways.

In 2006, the American Heart Association (AHA) defined cardiomyopathy as a heterogeneous group of cardiomyopathies that include abnormal mechanical and electrical activity of the Heart caused by various etiologies. Primary cardiomyopathy includes hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, left ventricular incompaction and partially dilated cardiomyopathy^[1]。 The pathogenesis of primary cardiomyopathy has not been fully elucidated, but with the deepening of molecular biochemistry and molecular genetics research, more and more evidence shows that cytoskeletal proteins play a very important role in it. Recent studies have found that there are many highly correlated skeletal protein gene mutations in patients with primary cardiomyopathy, such as desmin gene, β-myosin heavy chain gene and sartonectin gene related to dilated cardiomyopathy, and α-cardiac actin gene, cardiac troponin I gene and β-myosin heavy chain gene related to hypertrophic cardiomyopathy. This article focuses on the research progress of the relationship between cytoskeletal proteins and the pathogenesis of primary cardiomyopathy.