2018年 · 第98卷第02期

Pulmonary hypertension is a common clinical disease, with complex etiology, rapid progression, and high mortality and disability rate. At present, domestic and foreign guidelines divide pulmonary hypertension into five categories, of which the first category is called arterial pulmonary hypertension (PAH). In recent 20 years, with the in-depth research on the pathogenesis of pulmonary hypertension, targeted therapeutic drugs targeting the pathogenesis of PAH have been widely used in clinical practice. However, although these targeted drugs effectively improve cardiac function and quality of life in patients with PAH, PAH remains a progressive disease with poor prognosis^[1]。

In the 20th century, many people died of infectious diseases at a relatively young age, but in the 21st century, the death issue faced by people has changed dramatically because successful public health strategies and advanced medical technologies have significantly increased people's life expectancy. At the same time, these changes have changed attitudes towards death^[1]Going to the end of life with dignity has become the expectation of more people. Hospice care refers to the comprehensive services provided by the hospice team for critically ill patients and their families when medical measures for the purpose of cure cannot control the deterioration of the condition and the patient's expected life may be less than 6 months. In this paper, the end-of-life care for the elderly mainly refers to the comprehensive services specially provided for the dying elderly and their families over the age of 60^[2]。 The purpose of this paper is to explore the challenges and policy predicaments faced by the hospice service for the elderly from the perspective of diversified disciplines, and to face the limitations of traditional concepts, advocate the construction of a new view of life and death, combine macro system with micro practice, and discuss the countermeasures of hospice service for the elderly in depth.

Breast cancer is one of the most common malignancies in women. The incidence of female breast cancer in China is increasing year by year, with about 270,000 new patients every year^[1]Among them, 3% to 8% of breast cancer patients are metastatic at first diagnosis^[2]。 Even in early patients who have undergone surgery and standardized treatment, 30% to 40% will still relapse and metastasize^[3]。 Although advanced breast cancer is difficult to cure at present, it can alleviate the clinical symptoms of patients, improve the quality of life of patients, and further prolong the survival time of patients by developing new therapeutic drugs and optimizing treatment models.

Hemophagocytic syndrome (HPS), also known as hemophagocytic lymphohistiocytosis (HLH), is an immune-mediated life-threatening disease. HLH can affect people of all ages, not only in patients with congenital genetic susceptibility to immunodeficiency, but also in an increasing number of patients with autoimmune diseases, persistent infections, malignancies or immunosuppression, thus involving a multidisciplinary cross-section. In order to improve clinicians' knowledge and understanding of HLH and provide standardized clinical practice guidance, relevant experts at home and abroad demonstrated and extensively solicited opinions, and reached the following consensus on the diagnostic procedures, laboratory tests, diagnostic criteria and treatment principles of HLH.

Interstitial cystitis/bladder pain syndrome (IC/BPS) is a clinical syndrome that has plagued patients for a long time. At present, the diagnosis mostly follows the definition of urodynamic and female urology society: that is, an unpleasant sensation (including pain, pressure and discomfort) perceived to be associated with the bladder, which can be combined with lower urinary tract symptoms, and the course of the disease is more than 6 weeks, except for infection or other clear causes^[1]。 Because of the unknown etiology, variable symptoms and lack of specific diagnostic methods, the clinical diagnosis and treatment of IC/BPS are difficult. Patients with newly diagnosed IC/BPS at work were confirmed to have urothelial carcinoma of the bladder after a long period of diagnosis and treatment^[2,3,4,5]。 This study analyzed the clinical data of 412 patients newly diagnosed with IC/BPS in Peking Union Medical College Hospital from January 2005 to December 2015. Among them, 3 patients were diagnosed with bladder urothelial carcinoma 16, 27 and 19 months after the diagnosis of IC/BPS, respectively. The diagnosis and treatment results are reported as follows.

Allergic bronchopulmonary aspergillosis (ABPA) is an immune-mediated lung disease mainly caused by the body's allergy to Aspergillus fumigatus^[1]； It mainly occurs in patients with bronchial asthma (asthma for short) and pulmonary cystic fibrosis, and can also occur in patients with chronic obstructive pulmonary disease^[2]。 The disease often manifests as migratory or recurrent pulmonary infiltrates, including bronchiectasis, mucous plug impaction, tree bud sign and small nodules^[3]Pulmonary fibrosis may occur in the later stage. Common forms of airway mucous impaction include double track sign, finger cuff sign and toothpaste sign^[4]。 Carcinoembryonic antigen (CEA) is usually a marker of malignant tumors. Clinically, some patients with ABPA show abnormal chest CT occupation and elevated serum CEA, which is difficult to distinguish from lung malignant tumors. The clinical data of 7 patients with ABPA and increased serum CEA are retrospectively analyzed as follows.

With the extensive development of anterior cervical fusion surgery and the development of techniques to preserve motor segment function, adjacent segment disease (ASD) after cervical fusion has received more and more attention. ASD refers to new radiculopathy or myelopathy in adjacent active segments after anterior cervical fusion; Simply imaging degenerative changes of adjacent segments such as disc degeneration, osteophyte formation, articular process hyperplasia and spinal canal stenosis without clinical symptoms are called cervical adjacent segment degeneration^[1]。 Studies have shown that the incidence of adjacent segment degeneration after cervical fusion is 50%-92%, while the incidence of ASD is 6.1%-16.9%^[2]。 The short-term effects of 12 patients with ASD treated with zero-notch intervertebral cage (Zero-P) are retrospectively analyzed as follows.

Epithelioid hemangioendothelioma (EHE) is a rare, low-grade malignant tumor of vascular origin. It mostly occurs in soft tissues and organs such as lungs, bones, brain and small intestine^[1]。 EHE originating from the liver is less, mainly affecting adults (30-40 years old), more common in women and rare in children. The clinical manifestations and imaging examinations were not specific, and histologically they were easily confused with hepatocellular or intrahepatic bile duct epithelial malignancies. This paper reports a case of liver EHE diagnosed by puncture biopsy. Combined with relevant literatures, the clinical manifestations, pathological changes and differential diagnosis of this disease are comprehensively analyzed and summarized, so as to improve the understanding of this disease.

Case 1, male, 71 years old, with primary myelofibrosis (PMF) for more than 5 years and blood in sputum for 1 day, was admitted to hospital with "acute myeloid leukemia" on April 24, 2017. The patient was examined in January 2012 due to splenomegaly (200 mm ×59 mm) with hemogram abnormalities: white blood cells (WBC): 15.86×10⁹/L, hemoglobin (Hb): 117 g/L, platelets (PLT): 560×10⁹/L, PMF was confirmed by bone marrow smear and bone marrow biopsy, reticular fiber staining + + + +, and JAK2V617F mutation was positive. Hydroxyurea, thalidomide, calcitriol and interferon were given, and the condition was stable. March 2015 Abdominal distension with asthenia, Hb: 83 g/L, WBC: 2.71×10⁹/L, PLT: 203×10⁹/L, blood smear: original granule 22%, bone marrow dry draw, progression to acute myeloid leukemia, recommended chemotherapy, family refused. After that, severe abdominal distension, lower extremity edema, severe anemia, Hb fluctuating between 50 and 58 g/L, requiring monthly blood transfusion, each transfusion of 6 to 8 U red blood cells. Signed an informed consent form with the patient and started oral administration of rapamycin target protein (mTOR) inhibitor sirolimus capsules 0.5 mg/d in August 2015. In October 2015, the blood routine was improved compared with the previous, Hb: 66 g/L, WBC: 5.56×10⁹/L, neutrophils 41.7%, PLT 344×10⁹/L, the transfusion interval was lengthened to 2 months. After January 2016, the blood transfusion was withdrawn, the spleen was reduced, the abdominal distension was reduced, and there was no edema in the lower limbs. Abdominal ultrasound in February 2016: spleen 171 mm ×60 mm, in May 2016, blood routine was reviewed, Hb: 79 g/L, WBC: 25.38×10⁹/L, PLT: 214×10⁹/L, because the anemia was reduced but the white blood cells rose, the dose of sirolimus was increased to 1 mg/d, and the condition continued to improve, Hb: 75~88 g/L, WBC: 20×10⁹/L for 16 months. In January 2017, the patient's condition progressed, the anemia worsened, and he received another blood transfusion. He came to the hospital with blood in the sputum 1 day before this admission. Blood routine : Hb: 59 g/L, WBC: 110×10⁹/L, PLT: 30×10⁹/L, 64% of blood smear particles. Physical examination: anemic appearance, giant spleen, peripheral blood flow test: 70% of the original granule, JAK2V617F mutation still positive, MPL mutation negative, CALR mutation negative, acute myeloid leukemia diagnosed, PMF conversion, chemotherapy recommended, family refused, treatment abandoned.

Clostridium difficile is a gram-positive, toxin-producing or non-toxin-producing anaerobic bacillus. The bacteria spread mainly through the fecal-oral route, and the symptoms of infection ranged from mild diarrhea to life-threatening pseudomembranous colitis. The overall mortality rate was 6%, rising to 13.5% in elderly patients^[1]。 Clostridium difficile spores are highly resistant to disinfectants such as ethanol, soap, heat, and dryness, and can survive on the surface of objects for months to years, facilitating their spread. It is generally believed that Clostridium difficile infection (CDI) is related to the use of antimicrobial drugs and the history of hospitalization, and the use of antimicrobial drugs can lead to eosinopenia, which is the main risk factor of CDI^[2]。 However, some patients with CDI were not associated with a recent history of exposure to antimicrobial drugs, while the use of proton pump inhibitors and dyskinesia could induce microbiological dysbiosis and lead to CDI^[3,4]。 Thanks to a boost from the Human Microbiology Project and the European Human Gut Metagenome Project (MetaHIT), many experts have studied the relationship between human health and disease and the microbiome, focusing in particular on the study of metabolic activity that maintains and resists the gut microbiome colonized by C. difficile, and how antimicrobial drugs can alter the gut microbiome leading to C. difficile susceptibility. In this paper, the relationship between CDI and intestinal microbiota is discussed, and the mechanism of CDI infection and the latest prevention and treatment scheme are discussed from the perspective of metabolism, so as to discover new therapeutic targets.