2016年 · 第96卷第16期

The etiology of pulmonary hypertension involves many disciplines, which requires clinicians to have multi-disciplinary knowledge and flexible differential diagnosis thinking. For a patient with pulmonary hypertension, a standardized etiology screening process is very important. Respiratory doctors should consider cardiovascular disease factors. Cardiologists should not forget the causes of hypoxia and lung diseases. Pulmonary hypertension related to connective tissue and other related diseases should not be ignored. Clinicians should constantly accumulate experience, improve the diagnosis level, and reduce misdiagnosis and missed diagnosis. The diagnosis of pulmonary hypertension requires the clinician to perform a detailed physical examination based on the patient's symptoms, and make a comprehensive analysis of each examination to confirm whether the patient meets the hemodynamic diagnostic criteria of pulmonary hypertension, and to clarify the etiology, cardiac function and the degree of hemodynamic damage. The results of each examination need at least the evaluation and discussion of experts in cardiology, respiratory medicine, rheumatology and immunology, imaging and other departments. For patients with complex causes, multidisciplinary screening and co-diagnosis and treatment are particularly important.

chronic thromboembolic pulmonary hypertension (CTEPH) is a relatively common chronic disease. Thromboembolus blocks the proximal pulmonary artery and remodeling the distal pulmonary blood vessels, resulting in progressive increase of pulmonary vascular resistance and pulmonary artery pressure, which eventually leads to right heart failure and even death. It is often manifested as dyspnea, fatigue, and decreased exercise endurance. Its disability rate is high and the prognosis is poor.

Based on the "3+2" training mode of assistant general practitioners put forward in the "Guiding Opinions of the State Council on Establishing General Practitioner System" (Guofa [2011] No.23), Zhejiang Province issued the "Standardized Training Program for Assistant General Practitioners in Zhejiang Province (Trial)" and the "Standardized Training Standard for Assistant General Practitioners in Zhejiang Province (Trial)", which opened a precedent for the training of assistant general practitioners in China. Training qualified assistant general practitioners through the "3+2" model can gradually narrow the huge gap between the lack of rural health personnel and the construction goals put forward in the guiding opinions of the State Council. It is the key to improve the level of primary medical and health services, and it is also an effective measure to break through the bottleneck and promote the continuous deepening of the reform of medical and health system^[1]。 In 2012, the National Health and Family Planning Commission formulated and issued the Training Standards for Assistant General Practitioners (Trial) with reference to the Standardized Training Standards for Assistant General Practitioners in Zhejiang Province (Trial), which marked the national promotion of Zhejiang model. The principles, contents and implementation process of the standardized training practice of assistant general practitioners in Zhejiang Province are discussed as follows.

Interventional treatment of craniocerebral diseases includes intracranial aneurysm, cranial arteriovenous malformation, acute ischemic stroke and carotid stenosis, and deep brain stimulation in Parkinson's disease. This expert consensus aims to improve the level of anesthesia management for interventional treatment of craniocerebral diseases in China, reduce perioperative-related complications, and improve postoperative outcomes of patients. Due to the limited evidence of evidence-based medicine in this field, individualized anesthesia management needs to be implemented in clinical practice according to the specific conditions of patients and with reference to the consensus of this expert.

The newborn male, born 2 h, was born by caesarean section at term. The main cause was multiple scalp defects in the parieto-occipital region after birth and was admitted to hospital 2 h. Personal history and family history are not special. Admission physical examination: The body temperature was 37.0 ℃, and three irregular scalp defects were seen near the midline of the parieto-occipital region, with the extent of about 3.0 cm ×1.0 cm, 4.0 cm ×1.5 cm and 1.5 cm ×1.5 cm, respectively, with local scab, redness of the base, blood and fluid oozing on the surface, and no hair coverage. CT of the head: partial defect of the top skull. Diagnosis: Congenital skin agenesis: (1) neonatal scalp defect with infection; (2) Skull defect (Figure 1)。 After admission, the scalp defect was washed with normal saline and Kangfuxin liquid, and the recombinant bovine basic fibroblast growth factor and mupirocin ointment were applied externally. Finally, the scalp defect was covered with petrolatum gauze, put into an incubator, and vitamin K was introduced to prevent bleeding, cefmenoxime was used to prevent infection, pediatric compound amino acid nutritional support, immunoglobulin was used to enhance immunity, and oxiracetam was used to nourish cranial nerves. After 13 days of treatment, the scalp defect of the child was basically healed, and he was discharged. Follow-up after 1 week of discharge, the healing area of the scalp defect of the child was replaced by scar tissue. Follow-up after 3 months of discharge showed that the extent of the skull defect was reduced.

Patient, male, 83 years old (bladder cancer diagnosed at 63 years old). In 1995, he was diagnosed with bladder cancer in Peking Union Medical College Hospital and underwent partial cystectomy. The pathological report showed that bladder papillary transitional cell carcinoma grade II. Four local recurrences occurred in June 1997, January 2002, January 2003 and May 2004, all of which underwent local electrical resection. In October 2008, the patient developed low back and leg pain. Bone scan performed in the local hospital showed that multiple nuclear radiation concentrations throughout the body, and multiple bone metastases were considered. The first dose of zolephosphate 4 mg intravenous drip was started on 30 October 2008, and then infused once every two months on average. Bone pain was relieved after administration. The last dose was on 17 May 2015, with a total of approximately 38 doses and a total dose of 152 mg.

In recent years, population studies have found that iron deficiency is more common in patients with pulmonary hypertension (PH), and may aggravate the progression of the disease. The research on targeted treatment with iron supplement is also gradually being carried out, and some progress has been made. However, the mechanism of iron deficiency and the occurrence and development of PH needs to be further explored. The effect of iron deficiency on PH and the related research status and possible mechanism of iron therapy are described below.

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with an incidence rate of about 1~2/1 million. Children under 10 years old and adults aged 40~50 years old are the two peak age groups of adrenocortical carcinoma. In recent years, the diagnosis and treatment of ACC has undergone major changes, gradually shifting to individualized and precision medicine^[1]。 In order to further improve the diagnosis and treatment of adrenocortical carcinoma, the research progress related to adrenocortical carcinoma in recent years is reviewed as follows.

neutral lipid storage disorders (NLSDs) are autosomal recessive diseases caused by the intracellular deposition of triglycerides (TG) in different tissues. Its onset is caused by mutation of adipose triglyceride lipase (ATGL), also known as patatin-like phospholipase domain protein 2 (PNPLA2) gene, which leads to NLSDs with myopathy and myocardial vascular disease, which is mainly muscle damage^[1]or Dorfman-Chanarin syndrome with ichthyosis and fatty liver as the main manifestations caused by mutations in the comparative gene identification-58 (CGI-58), also known as α/β hydrolase domain-containing protein 5 (ABHD5) gene^[2]。