2015年 · 第54卷第03期

Complement system is an important component of human natural immune system, which can recognize and eliminate pathogenic microorganisms, apoptotic tissues and immune complexes. At present, there are three known complement activation pathways: classical pathway, bypass pathway and lectin pathway, involving more than 30 known plasma proteins; In order to prevent excessive activation of the complement system, numerous complement-activated regulatory proteins such as H factor are also included, and their activation can be finely controlled. However, if the complement is over-activated, it will lead to damage to the body itself, which in turn will lead to related diseases. Therefore, complement plays a "double-edged sword" role in the pathogenesis of immune-related diseases.

Glomerulonephritis (GN) is the most common urinary disease in China, and it is the main cause of chronic kidney disease and end-stage kidney disease in China. The pathogenesis of GN is complex, and epidemiological studies have shown that some patients with GN have a familial cluster onset. In addition, there are ethnic differences in the incidence of GN, such as IgA nephropathy is the most common in Asian GN; However, focal segmental glomerulosclerosis (FSGS) is relatively high in blacks, while IgA nephropathy is rare. The above epidemiological characteristics suggest that the genetic background difference plays an important role in the occurrence of GN. In recent years, with the improvement of genetic research methods and the continuous improvement of human genome database, the genetic research of GN has made a lot of progress, which makes our understanding of this kind of disease deep.

"Blood pressure should be boosted when it is low", a familiar phrase for medical staff. The front talks about diagnosis and the back talks about treatment; If the measurement of blood pressure is performed continuously, the front is monitoring, and the back is titration treatment; If the blood pressure is set to a target value in advance, the treatment becomes a goal-oriented treatment; If it is found that "blood pressure is low", look for relevant indicators at the same time, such as determining the treatment method by flow and resistance indicators, and determining the target value of raising blood pressure by tissue perfusion indicators, the treatment will become: the method is determined by the condition, and the degree is determined by tissue perfusion. Different from the initial treatment, the treatment at this time has been directly regulated, quantitative and individualized by the indicators reflecting the disease. This change in therapeutic behavior, from quantitative change to qualitative change, all comes from advances in the understanding of clinical hemodynamics.

The patient was a 50-year-old male. The main complaint was "lumbar swelling and pain for 2 months and edema of both lower limbs for 1 month". In September 2013, a painful mass of about 10 cm in diameter appeared in the left waist with a fluctuating sensation; Intermittent fever in the afternoon, with a maximum body temperature of 38.3℃, no night sweats, no cough or expectoration; Accompanied by persistent dull pain in the middle and upper abdomen, diarrhea after eating greasy food, and a small amount of oil in the feces; With symmetrical concave edema of both lower limbs, weight loss of 2 kg in 1 month. CT of the chest and abdomen in the local hospital revealed a cystic mass in the left psoas major muscle. The psoas major cyst was punctured and pale yellow clear fluid was drained. The nature of the drainage fluid was unknown. The pain improved and the temperature returned to normal. Prior denial of history of acute pancreatitis. He was admitted to our department with nephrotic syndrome in October 2013. Physical examination on admission: lean body, BMI 19.7 kg/m², blood pressure 110/70 mmHg (1 mmHg =0.133 kPa), no yellowing staining of skin and mucosa throughout the body; The abdominal wall is tough, tenderness under the xiphoid process, no rebound pain, and the liver and spleen are not touched under the costs; Mild concave edema of both lower limbs. Laboratory tests: Blood routine showed Hb 93 g/L; Blood biochemistry showed albumin 18 g/L, creatinine 66 μ mol/L, calcium 1.85 mmol/L, blood glucose 5.2 mmol/L, TC 4.20 mmol/L, TG 1.08 mmol/L, LDL-C 2.60 mmol/L; ESR 109 mm/1h; 24 h urine protein quantification 9.65 g; Urine routine showed 80 red blood cells/ml and 1.0 g/L protein; Fecal routine + occult blood: moderate fat droplets, occult blood (–). Tumor markers: CA19-9 was 64.8 U/ml (reference value: 0-37 U/ml), CA125 was 90.5 U/ml (reference value:<35 U/ml). Abdominopelvic enhanced CT (Figure 1): A space-occupying lesion was seen in the uncinate process of the pancreas, which showed irregular enhancement after enhancement, accompanied by dilatation of the pancreatic duct and pushing and compression of the superior mesenteric artery, and malignant lesions were not excluded; A huge cystic mass was found in the left psoas major muscle, extending downward from the left diaphragm roof to the end of the left iliopsoas muscle. PET-CT examination (Figure 2): The abnormal metabolic increase in the head and neck of the pancreas, the metabolism is significantly increased after delay, and the possibility of pancreatic cancer is high. Then, the psoas major cyst puncture was performed under the guidance of CT, and a total of about 200 ml of transparent and clear liquid was drawn out. Cystic fluid assay: WBC 155/high power field, total protein 4 g/L, creatinine 53 μ mol/L, TBil 0.1 μ mol/L, DBil 0.0 μ mol/L; Amylase 54 742 U/L, Lipase 486 838 U/L. Kidney biopsy pathology: immunofluorescence showed IgG (+ +), particle-like deposition along the glomerular basement membrane; Under the light microscope, the glomerular basement membrane was segmentally thickened, and a small number of nail processes were formed on the epithelial side; The pathological diagnosis was membranous nephropathy stage I-II. Diagnosis: pancreatic cancer is likely, pancreatic pseudocyst formation in the psoas major muscle, membranous nephropathy.

Familial hyperchyleemia (type I hyperlipidemia) is a rare autosomal recessive genetic disease, mainly due to the decrease of LPL activity caused by mutation of lipoprotein lipase (LPL) gene, thus reducing TG decomposition and increasing serum TG concentration. We clinically found a patient with recurrent acute pancreatitis during pregnancy due to hyperTGemia. After excluding secondary dyslipidemia factors such as hypothyroidism, Cushing's syndrome and diabetes, he was diagnosed with primary hyperTGemia and familial hyperchyleemia. The LPL gene of this family was detected by PCR technology to explore its molecular mechanism, which is reported below.

List of expert working groups(Sort by surname Chinese pinyin): Ding Yansheng (Department of Cardiology, Peking University First Hospital); Dong Jianzeng (Department of Cardiology, Beijing Anzhen Hospital affiliated to Capital Medical University); Fang Quan (Department of Cardiology, Peking Union Medical College, Peking Union Medical College, Chinese Academy of Medical Sciences); Guo Jihong (Heart Center, Peking University People's Hospital); Hu Dayi (Heart Center, Peking University People's Hospital); Huawei (Department of Cardiology, Fuwai Cardiovascular Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences); Huang Congxin (Department of Cardiology, People's Hospital of Wuhan University); Huang Dejia (Department of Cardiology, West China Hospital, Sichuan University); Jiang Wenping (Department of Cardiology, The First Affiliated Hospital of Suzhou University); Ke Yuannan (Department of Cardiology, China-Japan Friendship Hospital); Li Chunsheng (Emergency Department, Beijing Chaoyang Hospital affiliated to Capital Medical University); Liu Shaowen (Department of Cardiology, Shanghai First People's Hospital); Lu Yiming (Emergency Department, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine); Ma Changsheng (Department of Cardiology, Beijing Anzhen Hospital affiliated to Capital Medical University); Ma Jian (Department of Cardiology, Fuwai Cardiovascular Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences); Qi Xiangqian (Department of Cardiology, Tianjin TEDA International Cardiovascular Hospital); Sun Genyi (Department of Cardiology, Tianjin Chest Hospital); Wan Zheng (Department of Cardiology, General Hospital of Tianjin Medical University); Wu Shulin (Department of Cardiology, Guangdong People's Hospital, Guangdong Academy of Medical Sciences); Yang Xinchun (Department of Cardiology, Beijing Chaoyang Hospital affiliated to Capital Medical University); Yang Yanzong (Department of Cardiology, First Affiliated Hospital of Dalian Medical University); Yang Yanmin (Acute and Critical Care Center, Fuwai Cardiovascular Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences); Zhan Siyan (Center for Evidence-Based Medicine, Peking University School of Public Health); Zhang Ping (Department of Cardiology, Tsinghua Chang Gung Memorial Hospital, Beijing); Zheng Qiangsun (Department of Cardiology, Tangdu Hospital, Fourth Military Medical University); Zhu Jun (Acute and Critical Care Center, Fuwai Cardiovascular Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences)

invasive fungal disease (IFD) is a common infectious complication in patients with hematological malignancies. Epidemiological studies in China, Europe and America have shown that with the development of standard and high-dose chemotherapy and hematopoietic stem cell transplantation, the incidence of IFD in patients with hematological diseases is on the rise^{[1,2,3,4,5,6]}。 Domestic prospective, multicenter epidemiological studies showed that the overall incidence of IFD in patients with hematological malignancies receiving chemotherapy was 2.1%; In patients undergoing hematopoietic stem cell transplantation, the cumulative incidence of IFD was 3.8% in sibling-compatible transplantation and 7.1% in haploid-compatible transplantation^[6]。 The clinical symptoms and signs of IFD lack specificity, and the diagnosis of IFD depends on biopsy and/or mycological criteria of sterile site culture, so it is difficult to make a clear diagnosis. However, IFD is often accompanied by a high mortality rate, so the diagnosis and treatment of IFD has always been one of the difficulties that plague clinical hematology workers^[1,2,3,4,5]。

hematopoietic stem cell transplantation (HSCT) is one of the radical methods for the treatment of malignant diseases of the blood system. However, during transplantation preconditioning and hematopoietic reconstruction, patients will inevitably experience thrombocytopenia, resulting in severe bleeding; An important and effective means of supportive therapy is platelet transfusion^[1]。 platelet transfusion refractoriness (PTR) is one of the common clinical problems after HSCT, which seriously increases the risk of bleeding in patients. How to diagnose and treat PTR is one of the important clinical problems in the field of HSCT.

The incidence of acute kidney injury (AKI) in hospitalized patients was 21.6% in adults and 33.7% in children. The mortality rate of adult AKI patients was 23.9%^[1]。 Continuous renal replacement therapy (CRRT) is the primary renal replacement therapy for severe AKI due to its advantages in hemodynamic stability and solute volume management^[2]。 CRRT has received more and more attention and application in the treatment of severe AKI, multiple organ dysfunction, systemic inflammatory response syndrome and other clinical work. Relevant studies have discussed the initiation timing, mode selection, prescription dose, etc. of CRRT^[3,4,5]。 However, the downtime timing of CRRT is still lacking enough research. For patients with severe AKI, CRRT is the most important renal replacement therapy. Although the condition can be converted to intermittent renal replacement therapy for transition after stabilization, considering the instability and transport risk of severe patients, most patients currently adopt a single CRRT treatment mode before shutdown^[2,6]。 CRRT shutdown should be cautious, premature shutdown often leads to inadequate treatment, which can easily lead to poor prognostic outcomes, Uchino et al.^[7]The multicenter study showed that patients who received CRRT repeatedly had a higher in-hospital mortality rate than those who were successfully discontinued. However, excessive renal replacement therapy (RRT) not only increases medical expenses, but also increases the risk of complications such as bleeding and infection^[8]。 The guidelines recommended by the Global Organization to Improve the Outcome of Kidney Disease (KDIGO) in 2012 point to a lack of attention to the timing of downtime for CRRT in patients with AKI, and their definition of downtime is very vague.

The kidney plays a very important role in maintaining the metabolic balance of the body and the stability of the internal and external environment of cells, including the excretion of metabolites, the maintenance of water and electrolyte balance, the regulation of blood pressure and calcium and phosphorus metabolism, etc. Therefore, it is extremely important to correctly evaluate renal function. GFR is one of the important indicators for assessing renal function. However, GFR cannot be directly measured, but can only be obtained indirectly by measuring the filtration rate of endogenous or exogenous markers. Iohexol is a nonionic water-soluble contrast agent, which hardly binds to plasma proteins. It is completely excreted by the kidneys in the body, can be filtered freely through the glomeruli, and is not absorbed and secreted by the renal tubules. It is a good exogenous marker. Iohexol has high accuracy in clinical determination of GFR, and its plasma clearance rate is a new gold standard for determination of GFR. Moreover, its determination operation is convenient, the price is lower than that of isotope, and there is no pollution. Therefore, iohexol for determination of GFR (iGFR) is one of the renal function assessment methods worthy of popularization and application in China.

Crohn's disease (CD) is a recurrent nonspecific intestinal inflammatory disease. The exact pathogenesis is unknown. Current studies suggest that its pathogenesis is the result of the combination of environment, immunity, intestinal microecology and genes^[1]。 CD is a common disease in North America and Europe, but the incidence of CD in China has been increasing in recent years, ranking second in Asia^[2]。 In addition to typical intestinal manifestations, CD is also accompanied by extraintestinal damage such as joints, skin, eyes and oral mucosa, as well as systemic manifestations such as fever and weight loss. More and more studies have found that patients with CD have vitamin D deficiency^{[3,4,5,6,7,8,9]}。

Primary gout is a group of heterogeneous diseases that cause tissue damage due to long-term purine metabolism disorder, resulting in increased blood uric acid. When the concentration of uric acid in blood exceeds the physiological dissolution threshold of human body, uric acid crystals will be formed and deposited in the joints and surrounding tissues, manifesting as clinical features such as acute and chronic arthritis, joint deformities and tophi, which often involve the kidney and cause chronic interstitial nephritis and uric acid stones, resulting in acute and chronic renal failure^[1]。 In recent years, the prevalence of gout has been increasing year by year, and there are differences in region, age and sex. It has been confirmed that related diseases including hyperuricemia, metabolic syndrome, and risk factors including genetic factors, eating habits, alcohol, etc. can promote the occurrence and development of gout. In addition, as an independent risk factor for cardiovascular diseases, gout can induce and aggravate major diseases such as coronary heart disease and stroke, which seriously affects people's quality of life^[2]Therefore, it is widely valued by various countries. The following will summarize the research progress of gout epidemiology and related risk factors in recent years.

hemodynamics is concerned with the movement of blood. The movement of blood spreads all over the body, connecting tissues, cells and organs of the body like a network. The movement mode of blood in the body is different from that of general fluid mechanics, which is influenced by physiological, pathological and other factors. These factors make the movement of blood in various parts of the body maintain organic connection, interdependence, mutual regulation and mutual cause and effect. Blood consists of a variety of components. The movement of these components is not only manifested in the rapid movement in the cardiovascular system, but also in the slow movement between tissues and around cells, which participate in the metabolism of cells and the composition of the internal environment.

The effects of non-dihydropyridine (NDHP) calcium antagonists (CCBs) (NDHP-CCBs) and dihydropyridine CCBs have many commonalities, but also many differences. At present, the clinical research coverage of NDHP-CCBS (mainly diltiazem and verapamil) is much broader than the practical clinical application, and clinicians have insufficient understanding of NDHP-CCBS, and the selection rate is low. In order to promote and improve the rational clinical application of NDHP-CCBS by clinicians, the Cardiovascular Branch of Chinese Medical Association and the Hypertension Professional Committee of Chinese Medical Doctors Association jointly formulated this recommendation.

The patient was a 62-year-old female. He was admitted to the Department of Nephrology of our hospital on February 19, 2014 due to elevated serum creatinine for 20 d. In January 2014, the patient developed a cough and yellow phlegm after exertion, and his body temperature was normal. After taking cefaclor (0.25 g, 3 times/d) for 3 days, he developed eyelid and facial edema, and his urine volume decreased from 1 500 ml/d to 300 ml/d. During this period, he drank water as usual. On January 28th, he visited another hospital, and his blood pressure was measured at 165/93 mmHg (1 mmHg =0.133 kPa), and his blood creatinine was 456 μ mol/L. Anuria occurred on February 3, and blood creatinine was 1 067 μ mol/L and blood potassium was 6.9 mmol/L. Renal ultrasound: Diffuse lesions in both kidneys, thinning of the cortex, a small amount of hydrops in both renal pelvis, the long diameter of the left kidney is 13.9 cm, and the long diameter of the right kidney is 9.6 cm. Considering "chronic renal failure", the patient was treated with regular hemodialysis (3 times a week for 4 h each time), nifedipine controlled-release tablets (30 mg, twice/d) and metoprolol (25 mg, twice/d). The edema of the patient resolved and the blood pressure was controlled at 129-140/70-85 mmHg. Referred to our hospital on February 19th. Past history: In 2010, there was no obvious trigger for skin and sclera yellowing, and stool color became lighter. Total bilirubin (TBil) was 110.4 μ mol/L and direct bilirubin (DBil) was 81.1 μ mol/L. Abdominal CT: swelling of the pancreatic head, dilatation of intrahepatic and extrahepatic bile ducts, dilatation of the gallbladder, cystic duct and common bile duct. Endoscopic ultrasound showed that the hypoechoic size of the head of the pancreas was about 2.5 cm ×2.0 cm, and no malignant cells were found by puncture biopsy. The jaundice was gradually relieved after 2 months of treatment with polyene phosphatidylcholine (556 mg, 3 times/d) to protect the liver and ursodeoxycholic acid (250 mg, 1 time/d). In April 2012, bilateral parotid glands were inadvertently enlarged and gradually enlarged to the size of eggs. Needle aspiration smears of left submandibular and left parotid gland nodules were performed. Pathology showed proliferative lymphocytes and eosinophils, which was considered "eosinophilic lymphogranuloma". The mass shrank after taking traditional Chinese medicine (specifically unknown) for more than 2 months. Deny history of hypertension, diabetes, etc. Physical examination: body temperature 36.0℃, heart rate 64 beats/min, breathing 18 beats/min, blood pressure 131/79 mmHg; The swollen parotid gland can be palpable in front of both ears, 4 cm ×5 cm on the left, 2 cm ×3 cm on the right, hard in texture, nodular on the surface, irregular in shape, and tender; No obvious abnormalities were found in heart, lung and abdomen; There was no obvious edema in both lower limbs. Laboratory Test: WBC 5.34×10⁹/L, eosinophil count (Eos) 0.54×10⁹/L, Hb 94 g/L, PLT 309×10⁹/L; Albumin 31 g/L, ALT 12U/L, TBil 5.7 μ mol/L, DBil 1.9 μ mol/L, serum creatinine 997 μ mol/L, urea nitrogen 24.89 mmol/L, serum potassium 6.0 mmol/L, serum calcium 2.21 mmol/L, serum phosphorus 2.65 mmol/L; ESR 68 mm/1h, high sensitivity C-reactive protein 6.07 mg/L. Ultrasound of salivary glands: Multiple hypoechoic occurrences in left and right submandibular glands and bilateral parotid glands.

April 2014Kidney InternationalA clinical study "Clinical and immunological characteristics of anti-glomerular basement membrane (GBM) disease patients with membranous nephropathy" from the Department of Nephrology, Peking University First Hospital was published [Jia XY, Hu SY, Chen JL, et al. The clinical and immunological features of patients with combined anti-glomerular basal membrane disease and membranous nephropathy. Kidney Int, 2014, 85 (4): 945 – 952.]. The study found that compared with classical anti-GBM patients, anti-GBM patients with membranous nephropathy have significant particularities in clinical manifestations, renal prognosis and immunological properties of autoantibodies, thus clarifying the special clinical phenotype of this group of patients, and conducting in-depth research on the pathogenesis of this clinical phenotype.

November 2014Am J Kidney DisThe research team of Professor Zhang Hong and Professor Lu Jicheng of Peking University Institute of Nephrology, Peking University First Hospital completed the assessment of pregnancy outcome and risk factors in women with IgA nephropathy, the most common glomerulonephritis in China (Liu Y, Ma X, Lv J, et al. Risk factors for pregnancy outcomes in patients with IgA nephropathy: a matched cohort study. Am J Kidney Dis, 2014, 64 (5): 730 – 736). This study suggests through a matched cohort study that pregnancy in patients with IgA nephropathy with relatively normal renal function does not increase the risk of renal failure in patients.

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A prospective follow-up study in a non-diabetic population investigating whether urinary albumin excretion is associated with high levels of atherosclerotic Apo – B components (non-HDL – C, LDL – C, TG, and Apo – B). The results showed an association between increased urinary albumin excretion and increased Apo – B levels, factors that have side effects on cardiovascular events. Increased urinary albumin excretion and elevated Apo – B levels may share a pathogenic pathway in the pathogenesis of cardiovascular disease (J Am Soc Nephrol, 2014, 25:2906 – 2915).

A randomized controlled study investigated whether the beneficial effects of high-dose rosuvastatin on contrast-agent acute kidney injury (CI – AKI) in patients with acute coronary syndrome (ACS) were altered by the relationship between different baseline high-sensitivity C-reactive protein (hs – CRP) levels. The results suggest that short-term and medium-term clinical outcome improvements are more significant in patients with high baseline hs – CRP levels (JACC Cardiovasc Interv, 2014, 7: 1421 – 1429).