ATP-sensitive potassium channel congenital hyperinsulinism (KATP-HI) is the most common and most severe type of congenital hyperinsulinism,accounting for 40％-45％.It is due to the inactivating mutations of the ABCC8 and KCNJ11 gene which encode the ATP-sensitive potassium channel.Diazoxide is the main and preferred therapy for KATP-HI.For KATP-HI children who are unresponsive to medical therapy usually need different degrees of pancreatectomy to maintain normal blood sugar level.

The author proposed the doctor language of doctors basic characteristics, and analysized doctor-patient languages in the course of diagnosing and treating, summarized doctors language's basic principle.

As a transmembrane protein, CD47 is widely distributed in a variety of cells. It can bind to signal regulatory protein alpha (SIRPα) on macrophages and release inhibitory signals, thus avoiding phagocytosis of macrophages. In lymphoma cells, the expression of up-regulation of CD47 expression in lymphoma cells is one of the important mechanisms for inducing immune escape, and it is also a potential therapeutic target. This article reviews the research progress of CD47-induced immune escape, monoclonal antibodies targeting CD47 and cellular immunotherapy in the treatment of lymphoma.

Objective To investigate the relevance of the clinical score and pathology score and assess two scoring system including the British Islet Lupus Assessment Group2004 (BILAG2004) and systemic lupus erythematosus disease activity index 2000 (SLEI)AI2000) by analyzing the biopsy results and related laboratory tests on lupus nephritis (LN). Methods Retrospective analyzed 59 cases of patients with renal biopsy. All biopsied tissues were scored based on the to ISN/RPS2003 lupus nephritis pathological typing standards. Meanwhile, respective index (AI), chronic Index (CI) and tubulointerstitial lesions (TIL) score were used to evaluate the activities of lupus nephritis and SLEDAI2000 and BILAG2004 were used to evaluate the clinical disease activity. The adssociation between pathological changes and disease activities was assessed. Results ①According to ISN/RPS2003 pathological type standard, among these 59 patients, type Ⅳ was the most common type,which accounted for 44.07 percent of all biopsied tissues. ② For those patients. With LN typeⅡ,Ⅲ Ⅳ, their SLEDAI2000 score and BILAG2004 renal biopsy AI was positively correlated (00.05). Conclusion BILAG2004 score and SLEDAI2000 scoring system can be used to assess the disease pathology activity of patients with lupus nephritis. The study has found that BILAG2004 scoring system is superior to SLEDAI2000 score system for the assessment of LN patients on pathological activities, especially for with severe LN patients. As a non-invasive method of assessment, BILAG2004 score system is better than SLEDAI2000 score system in guiding clinical treatment.

Objective To investigate the value of 320-slice CT in diagnosis and intervention treatment of the hepatocellular carcinoma (HCC).Methods Retrospective analysis of 41 patients with HCC was performed,who underwent 320-slice CT three-phase scans and the CTA reconstruct before the transcatheter arterial chemoembolization (TACE).The origins,the distributions and the branches of the abdominal arteries were observed,respectively.Compared to the images of the DSA,the tumor feeding artery and portal vein tumor thrombus (PVTT)were analyzed.Results Compared to the images of the DSA,there was an obvious advantage in diagnosis of small HCC with the diameter <3 cm using the 320-slice CTA.However,there were no significant differences in display of hepatic artery variations,tumor feeding arteries and PVTT between the 320-slice CTA and the DSA.Conclusion The 320-slice CT and the CTA reconstruct can be helpful for more accurate diagnosis and evaluation for HCC,it can be used to direct the intervention treatment for HCC on basis of the images of the tumor feeding artery and PVTT.

Objective To investigate the value of the application of fibrobronchoscopy in extubation for patients suffering from acute exacerbation of chronic obstructive pulmonary disease(AECOPD)with low cough peak expiratory flow(CPEF). Methods A single-center prospective controlled study was conducted. The ventilated AECOPD patients who were cooperative at the time of extubation in Department of Critical Care Medicine of Guangzhou Institute of Respiratory Disease of Guangzhou Medical University from June 2009 to May 2014 were enrolled. All patients successfully passed the spontaneous breathing trial(SBT). Extubation was performed after determination of CPEF following energetic coughing. According to the CPEF,the patients were divided into CPEF≥60 L/min group (high CPEF group)and CPEF<60 L/min group(low CPEF group). After extubation,fibrobronchoscopic drainage was given to the patients in high CPEF group when necessary. Fibrobronchoscopic drainage was given to the patients in low CPEF group at least once a day,and the frequency of such treatment could be increased according to the patient's condition. If the patients did not require re-intubation within 48 hours,extubation was recorded as successful. The gender,age,acute physiology and chronic health evaluationⅡ(APACHEⅡ)score before extubation,ventilation time,the time of intensive care unit(ICU)stay,the mortality in ICU,the rate of re-intubation,the ability to cough and the frequency of application of fibrobronchoscopy after extubation were recorded. Results A total of 102 patients with AECOPD were enrolled,58 patients in high CPEF group and 44 in low CPEF group. Compared with high CPEF group,the mean age in low CPEF group was older(years:74.3±15.2 vs. 69.5±11.4,t=2.164,P=0.041),the time of ICU stay was significantly longer(days:20.1±11.2 vs. 17.4±7.3,t=2.274,P=0.030), but there was no significant difference in gender〔male/female(cases):35/9 vs. 45/13,χ2=0.057,P=0.812〕, APACHEⅡscore(11.9±1.9 vs. 10.3±4.2,t=1.290,P=0.200),mechanical ventilation time(days:14.8±10.8 vs. 13.3±9.6,t=0.677,P=0.501)and the rate of re-intubation〔18.18%(8/44)vs. 12.07%(7/58),χ2=1.412, P=0.235〕between low CPEF group and high CPEF group. The cough strength of patients in high CPEF group was almost alwaysstrong(52 cases),and in the low CPEF group,most of them wasmoderate(14 cases)orweak(26 cases). The frequency of application of fibrobronchoscopy in low CPEF group was higher than that in high CPEF group(times:4.1±1.8 vs. 1.3±0.9,t=2.626,P=0.011). All patients underwent weaning successfully,and no death occurred. Conclusion The application of fibrobronchoscopy in the extubated AECOPD patients with low CPEF can reduce the rate of re-intubation,avoid the prolonged ventilation,but cannot reduce the time of ICU stay.

Hepatic hemangioma is a common benign tumor of liver,while hepatic hemangioma with a diameter ＞ 3 cm is rarely seen in clinical practice.A female patient with a giant hepatic hemangioma (diameter =48 cm) received tumor resection via chest and abdomen joint incision at the Peking Union Medical College Hospital on April 10,2012.The patient received exploratory laparotomy for hepatic tumor via chest and abdomen joint incision 22 years ago,while the operation was failed due to intraoperative bleeding.The success of the operation benefited from comprehensive application of computed tomography angiography reconstruction technique,three-dimensional liver reserve function assessment,intraoperative controlled low central venous pressure,total hepatic vascular exclusion and precise hepatectomy technique.

Objective To investigate the protective effects of recombinant human erythropoietin (rHuEPO) on acute lung injury (ALI) induced by lipopolysaccharide (LPS).Methods Fourty-five rats were randomly (random number) assigned to three groups,namely control group,model group,and rHuEPO group.ALI was induced by intravenous injection of LPS (6 mg/kg).The rHuEPO (5000 U/kg) was injected intravenously into rats 60 min before LPS challenge.The general status of rats was observed.Twelve hours after modeling,the rats were sacrificed and the tissue samples including lung tissue and blood were collected.PaO2,PaCO2,pH,the lung wet/dry weight ratio,plasma cytokines [interleukin (IL) IL-6 and tumor necrosis factor-alpha (TNF-α)],and inducible nitric oxide synthase (iNOS) were detected.Cytokines were assayed with ELISA method.Pathological changes of lung tissues were observed under light microscope and transmission electron microscopy.Results (1) Compared with the control group,PaO2,pH in the model group and in the rHuEPO group were significantly lower (P ＜ 0.05),and PaCO2 were significantly higher (P ＜ 0.05).Compared with the model group,PaO2,pH in the rHuEPO group were significantly higher (P ＜ 0.05),and PaCO2 were significantly lower (P ＜ 0.05).(2) Compared with the control group,the W/D weight ratio of lung tissues in the model group and the rHuEPO group was significantly higher (P ＜ 0.05).Compared with the model group,the W/D weight ratio of lung tissues in the rHuEPO group significantly lower (P ＜ 0.05).(3) The levels of TNF-o,IL-6 and iNOS in serum of rats in the control group were lower than those in the model group and the rHuEPO group (P ＜0.01).The serum levels of TNF-α,IL-6 and iNOS of rats in the rHuEPO group were significantly lower compared with the model group (P ＜ 0.01).(4) The light microscopy and the transmission electron microscopy showed the model group had histopathologic changes with acute diffuse lung injury manifested by intra-alveolar hemorrhage,exudate,inflammatory cells infiltration,Ⅰ type and Ⅱ type epithelial cell necrosis and detachment,and the pathological changes of lung tissue in the rHuEPO group were not as serious as those in the LPS group,showing only a little inflammatory cells infiltration of focal alveoli.Conclusions Recombinant human erythropoietin can inhibit the genesis of TNF-α,IL-6 and iNOS in serum,modifying the inflammatory response and providing protective effects against acute lung injury induced by sepsis.

Plague, infectious disease in modern medicine, refers to a type of disease with strong pathogenicity and infectiousness, it refers to the infectious diseases of western medicine. Due to its wide variety, the knowledge and understanding of plagues of Traditional Chinese Medicine (TCM) doctors in different stages have evolved and developed with the times. This article, via collating ancient documents, differentiatesthe classification in TCM and analyzes itsrelated theories to perfect the type of plagues in TCM, providing the theoretical basis for the research of plague in modern times.

The microenvironment of lymphoma is an important factor affecting the development of lymphoma, which is involved in regulating the recognition and immune response of lymphoma cells by the immune system. In the era of immunotherapy of lymphoma, the state of microenvironment also affects the effect of monoclonal antibodies, small molecular compounds and other immune targeting drugs on lymphoma cells. Among them, microenvironment-related immune escape is one of the key factors leading to the failure of lymphoma treatment. This article reviews some microenvironment factors such as stromal immune cell subsets, vascular proliferation, hypoxia, immune checkpoint and the recent research progress of immune escape.