Objective:To analyze the distribution characteristics of special types of diabetes in China, in order to provide a theoretical basis for the diagnosis and treatment of special types of diabetes.Methods:Pubmed, CNKI, and WanFang Data were searched for the case reports and clinical studies of special types of diabetes in China from 2011 to 2021. After independent literature screening by 2 researchers according to the inclusion and exclusion criteria, diseases and the number of corresponding cases included were extracted for statistics. The etiological composition and disease characteristics of three subtypes of special type diabetes were analyzed.Results:A total of 613 articles(7 377 patients)were included and roughly divided into eight subtypes of special type diabetes according to etiological classification for disease composition analysis. The results by ratio in descending order were as follows: mono-genetic gene defects in islet β-cell function, pancreatogenic diabetes, diabetes induced by drugs or chemicals, endocrine disease, mono-genetic gene defects in insulin action, other genetic syndromes associated with diabetes, infection, and uncommon immune-mediated diabetes. The disease composition of the three subtypes of special types of diabetes that we focused on were mono-genetic gene defects in islet β-cell function(50.21%), pancreatogenic diabetes(35.65%), and mono-genetic gene defects in insulin action(1.56%). The composition analysis of the special types of diabetes in each subtype showed that neonatal diabetes mellitus(NDM, n=1 749, 23.71%)and maturity onset diabetes in young(MODY, n=1 554, 21.07%)accounted for the largest proportions. According to the composition analysis of each subtype of MODY patients, the top three subtypes were MODY2(50.89%), MODY3(16.03%), and MODYX(8.91%). In addition, taking MODY as an example, patients with de novo mutations(DNMs)and(or)new mutation sites were summarized and analyzed. The results revealed 31 MODY patients with DNMs(1.99%) and 339 MODY patients with new mutation sites(21.81%). Conclusions:According to the literature analysis, NDM and MODY represent the largest proportion of patients with special type diabetes in China. MODY2 patients make up the largest proportion of MODY patients. In addition, diabetic patients carrying DNMs and(or)new mutation sites should be taken seriously.

Objective:To summarize the clinical features, radiological characteristics, therapy, and outcome of patients with spontaneous intracranial hypotension (SIH).Methods:The general information, clinical manifestations, auxiliary examinations, treatment, and outcomes in consecutive patients of SIH hospitalized in the Xuanwu Hospital, Capital Medical University from November 2018 to October 2022 were analyzed.Results:A total of 118 patients with a female-to-male ratio of 5∶4 were included and the ages were 17.00-71.00[39.00(34.00,46.75)]years with a preponderance in the age of 30-49 years. Almost all patients had orthostatic headaches (117/118, 99.2%), accompanied by nausea (90/118, 76.3%), vomiting (70/118, 59.3%), neck stiffness (88/118, 74.6%), tinnitus (57/118, 48.3%), and ear fullness (57/118, 48.3%). Brain magnetic resonance imaging (MRI) showed dural enhancement (97/113, 85.8%), enlarged venous sinus (88/113, 77.9%), subdural fluid collection (46/113, 40.7%), decreased suprasellar cistern (86/113, 76.1%), effacement of the prepontine cistern (86/113, 76.1%), diminished mamillopontine distance (80/113, 70.8%). The cerebrospinal fluid (CSF) leaks were detected in 90.7% (107/118) of the patients by magnetic resonance myelography but 54.3% (25/46) and 52.6% (20/38) by CT myelography and magnetic resonance myelography with gadolinium. Lumber puncture found CSF pressure<60 mmH 2O (1 mmH 2O=0.009 8 kPa) in 18.4% (19/103) of patients, increased CSF red blood cell counts in 50.6% (44/87) of patients, CSF pleocytosis in 44.8% (39/87) of patients, increased CSF protein concentrations in 57.5% (50/87) of patients. The headache completely disappeared after conservative treatment in 24.6% (31/118) of patients and after a single targeted epidural blood patch in 89.7% (78/87) of patients. A rebound headache after epidural blood patch treatment occurred in 66.0% (58/87) of patients. Conclusions:The patients with SIH almost manifested with orthostatic headache, and brain MRI and magnetic resonance myelography were suggested in those patients instead of CSF pressure by lumber puncture. Targeted epidural blood patch was effective and safe in SIH patients.

Congenital generalized lipodystrophy type 1 (CGL1) is an autosomal recessive genetic disease caused by mutations in AGPAT2 gene. The main clinical mainifestations include body subcutaneous fat loss, muscle hypertrophy, obvious subcutaneous veins, pseudoacromegaly, hirsutism, and acanthosis nigricans. What′s more, CGL1 is always accompanied by metabolic diseases. Therefore, it is easily misdiagnosed as metabolic syndrome, type 2 diabetes, polycystic ovary syndrome, acromegaly, or Cushing′s syndrome. Meanwhile, it is difficult to distinguish it from partial lipoatrophy syndrome. In this article, we present clinical and molecular characteristics of a patient with CGL1 and review mutations reported in literature to replenish current knowledge about this orphan disease.

Objective:To observe the clinical effect of Bairui grain combined with ambroxol atomization inhalation treating acute attack of chronic bronchitis (wind heat attact the lung symptom). Methods:A total of 150 patients with acute attack of chronic bronchitis who were admitted in our hospital from July 2015 to July 2018 were randomly divided into control and intervention groups (75 each group) by the random number table method. The control group received ambroxol atomization inhalatio based on the regular western medicine treatment; the intervention group took Bairui grain orally based on control group. Both of the two groups were treated for 2 weeks. Before and after the treatment, to score the clinical symptoms and wind-heat attact the lung symptom. Use pulmonary function meter to detect the ratio of FEV1 to estimated value (FEV1%) and FEV1/FVC; use ELISA to detect serum and sputum levels of TNF-α and IL-6. Record the extinction time of the symptoms (cough, wheezing, sputum, lung rumble) and evaluate the clinical efficacy. Results:The total efficacy rate of intervention group was 98.6% (72/73), which was significantly higher than control group 88.9% (64/73) ( χ2=4.354, P=0.037). The symptoms (cough, wheezing, sputum, lung rumble) extinction time of intervention group were significantly less than those of the control group ( t values 5.331, 5.590, 5.841, 6.305, respectively, all Ps<0.01). After the treatment, scores of clinical symptoms (cough, wheeze, cough, cough) and symptoms of wind-heat attacking lung (cough, wheezing, fever, dry mouth, stuffy nose, runny nose) of intervention group were significantly lower than those of the control group ( t values 4.990, 4.431, 5.221, 5.004, 5.652, 5.190, 5.311, 5.793, 5.643, respectively, all Ps<0.01). After treatment, the FEV1% (52.51% ± 5.63% vs. 47.30% ± 5.21%, t=8.931) and FEV1/FVC (61.57 ± 6.44 vs. 56.87 ±5.82, t=8.251) were significantly higher than the control group ( P<0.01). The serum level of TNF-α, IL-6 ( t values 5.331, 4.908) and the level of TNF-α, IL-6 ( t values 6.001, 4.803) in sputum were significantly lower than those of the control group ( P<0.01). Conclusions:The Bairui grain combined with ambroxol atomization inhalation can decrease the inflammotory cytokine levels of the acute attack patients with chronic bronchitis (syndrome of wind-heat attacking lung symptome), improve clinical symptoms and enhance efficacy.

Objective To set up a warning diagnostic model by using the commonly used clinical indicators in an attempt to provide a basis for the early,fast and accurate diagnosis of posttraumatic sepsis.Methods Based on the presence of sepsis,165 patients were grouped into sepsis group (n =45) and non-sepsis group (n =120).Body temperature,respiration,heart rate,C-reactive protein(CRP),white blood cell,blood platelet count(PLT),activated partial thromboplastin time (APTT),acute physiology and chronic health evaluation Ⅱ (APACHE Ⅱ) score and sepsis-related organ failure assessment(SOFA) score were tested to identify the independent predictors of sepsis.Warning diagnostic models of unweighted score (unwScore) and weighted score (wScore) for posttraumatic sepsis were constructed by combining the independent variables.Receiver operation characteristic curve (ROC) was used to evaluate the independent predictor and warning diagnostic models for posttraumatic sepsis.Results Body temperature,respiration,heart rate,CRP,APACHE Ⅱ score and SOFA score were significantly different between the two groups(P ＜ 0.05).Multiple analysis showed body temperature,CRP and APACHE Ⅱ score were independently associated with sepsis.With the ROC analysis,areal under the curve (AUC),sensitivity,specificity,positive predictive value and negative predictive value of unwScore (0.915,0.87,0.85,69.64％ and 94.50％) and wScore (0.931,0.96,0.78,63.24％ and 97.85％) were better than these of body temperature (0.855,0.84,0.78,59.38％ and 93.07％),CRP (0.761,0.64,0.80,55.77％ and 85.84％) and APACHE Ⅱ (0.884,0.84,0.82,64.41％ and 93.40％).Conclusions Body temperature,CRP and APACHE Ⅱ score are independent predictors of sepsis.Models combining body temperature,CRP and APACHE Ⅱ score demonstrate high performance in diagnosing sepsis in trauma patients.

Objective To construct 131 I labeled anti?EGFR immunoliposome nanoparticle ( 131 I?Cetuaximab ( C225)?BSA?PCL) , and investigate its inhibitory effect on EGFR?overexpressing cancer cells in vitro. Methods Anti?EGFR liposome nanoparticle C225?BSA?PCL and non?targeted liposomes BSA?PCL were constructed. The products were observed with transmission electron microscopy and dynamic light scat?tering. The EGFR?targeted binding and cellular uptake in EGFR?overexpressing cancer cells were observed with flow cytometry and confocal microscopy. Anti?EGFR and non?targeted liposomes were labeled with 131 I using the chloramine?T method. The targeted cell killing effects of 131 I labeled liposomes were analyzed using MTT assay. The time?dependent cellular uptake analysis was used to evaluate the slow?release effects of the 131 I labeled liposomes. The independent?samples t test was used for data analysis. Results The EG?FR?targeted liposome C225?BSA?PCL and non?targeted liposome BSA?PCL were successfully constructed, and the effective diameters were approximately 130-180 nm. Flow cytometry and confocal microscopy re?vealed significant uptake of C225?BSA?PCL in EGFR?overexpressing tumor cells. BSA?PCL could also bind to cells with minimal and weak tumor retention. The EGFR?targeted radioactive liposome 131I?C225?BSA?PCL showed greater targeted cell killing effect than non?targeted liposome 131I?BSA?PCL,the IC50 values of 131I?C225?BSA?PCL and 131 I?BSA?PCL were 0. 03-1. 32 and 0. 25-12. 19, respectively. The uptakes of 131 I?C225?BSA?PCL was higher than that of 131 I?BSA?PCL ( t=3.03-16.86, all P<0.05) and reached the maxi?mal level at 4 h after incubation. Conclusions The EGFR?targeted liposome C225?BSA?PCL demonstrated superior cellular binding and uptake on EGFR?overexpressing cancer cells compared with BSA?PCL. The EGFR?targeted radioactive liposome 131 I?C225?BSA?PCL had favorable intracellular retention and excellent targeted cell killing effect, and could effectively suppress the growth of EGFR?overexpressing cancer cells.

Objective To evaluate the efficacy of catheterization of left atrium for postoperative management in pediatric patients undergoing congenital heart disease complicated with moderate to severe pulmonary hypertension.Methods Sixty ASA Ⅲ or Ⅳ pediatric patients of both sexes,aged 9 months-14 yr,scheduled for elective congenital heart disease complicated with moderate-to-severe pulmonary hypertension under cardiopulmonary bypass,were randomly divided into 2 groups (n =30 each):central venous catheterzation group (C group)and catheterization of left atrium group (L group).After induction of anesthesia,triple-lumen central venous catheters were inserted in both groups.In group L,the right internal jugular vein was cannulated with a single lumen venous catheter passed through the left atrium for monitoring postoperative left atrial pressure (LAP) and administration of vasoactive drug and LAP was maintained at 8-12 mm Hg after operation.Duration of mechanical ventilation and duration of stay in intensive care unit were recorded.The amount of dopamine,dobutamine,nitroprusside,milrinone,and alprostadil consumed within 48 h after operation was also recorded.Results Compared with C group,the amount of dopamine,dobutamine,nitroprusside,milrinone,and alprostadil consumed was significantly reduced and duration of mechanical ventilation and duration of stay in intensive care unit were significantly shortened in group L.Conclusion Catheterization of left atrium is used for monitoring postoperative LAP,which can provide a basis for reasonable clinical prescription and optimize the efficacy of postoperative management in pediatric patients undergoing congenital heart disease complicated with moderate to severe pulmonary hypertension.

OBJECTIVETo study the cause of the seeds dormancy of Glehnia littoralis in vitro and to establish plant regeneration methods via somatic embryos.

METHODThe effects of endosperm and exogenous hormone on the seed dormancy breaking of G. littoralis and the effect of hormone concentration on embryonic callus induction and plant regeneration via somatic embryos were observed,

RESULTSThe germination rate of the seeds with 1/3 endosperm was the highest which achieved 31%. TDZ, 6-BA and GA3 treatment could not break seed dormancy but easily lead to abnormal seedlings. Embryogenic callus induction rates was up to 57% on MS supplemented with 1.0 mg x L(-1) 2,4-D. After 20 days culture, embryogenic calli were transferred to MS medium and cotyledonary embryos were formed in 40 days. The regenerated plants were obtained in 20 days.

CONCLUSIONAn effective system of plant regeneration of G. littoralis was established in this study.

Apiaceae ; physiology ; Endangered Species ; Plant Somatic Embryogenesis Techniques ; Plants, Medicinal ; physiology ; Regeneration ; Seeds ; physiology

Objective Transesophageal echocardiography (TEE) guided, minimally invasive perventricular device occlusion of ventricular septal defects ( VSDs) without cardiopulmonary bypass ( CPB) has been applied in multiple centers. We reported experiences and the mid-term results. Methods Four hundred and thirty-two cases from 4 cardiac centers were involved in the study. There were 235 males and 197 females, aged from 3 months to 15 years, with a body weight varying from 4.0 to 26.0 kg. Three hundred and fifty-one patients had perimembranous VSDs, 57 had intracristal or supracristal VSDs and 24 had muscular VSDs (17 had multiple muscular VSDs). The diameter of the VSD ranged from 3 to 12 (5.3 ±1.6 ) mm.For those with perimembranous or muscular VSDs, a 3 to 5 cm inferior sternotomy was made, but for those with intracristal or supracristal VSDs, a 2 to 3 cm incision was made parastemally through the left third intercostal space. Being monitored and guided with TEE, the device was deployed to occlude the VSD through the puncture at the free wall of the right ventricle. TEE was used for assessing the residual shunting, the left and right ventricular outlet tracts, valvular function and for detecting any arrhythmia, The devices would be released if the heart rhythm was normal, as well as the residual shunting and valvular regurgilalion were not detected. Results The procedure was completed successfully in 417 cases(96.5% ) and converted to traditional surgical closure with CPB in the other 15 cases(3.5% ). Concentric devices were used in 238 cases(57.1% )and eccentric devices were used in 179 patients(42.9% ). Successful procedures finished in less than 90 minutes, and the deployment and evaluation of the devices were completed in 5 to 60 (18. 2 ± 8.6) minutes. No residual shunt and detectable aortic or tricuspid insufficiency and arrhythmia was observed. Patients were extubated within 2 hours and discharged 3 to 5 days after the operation. During fellow-up period from 3 months to 2 years, no clinically significant complications occurred. Conclusion The minimally invasive device closure of VSD under TEE guidance without CPB is proved to be a simple, safe and effective treatment for a considerable number of children with VSD. Its use in the clinical practice should be encouraged.

OBJECTIVETo establish HPLC characteristic fingerprints of the saponins in Pulsatilla medicinal plants, and provide the basis for authentication and classification of Pulsatilla species.

METHODThe HPLC profiles were determined at 35 degrees C on a Symmetry C18 column (4.6 mm x 250 mm,5 microm) eluted with water (A) and acetonitrile (B) as mobile phases in a linear gradient elution with the flowrate of 0.5 mL x min(-1). The elution program was as follows: 0-8 min, 90% A to 77% A, 8-25 min, changed to 71% A, 25-40 min, to 60% A, 40-50 min, to 50% A, 50-75 min, to 10% A, 75-80 min, to 0% A. The detection wavelength was set at 210 nm.

RESULTThe different species of Pulsatilla showed different HPLC fingerprints, but with 10 common peaks. A cluster analysis of 14 accessions indicated that they were divided into four groups: all accessions from P. koreana were classified into group I, P. ambigua in group II, P. dahurica and P. turczaninovii in group III, and P. chinensis in group IV, respectively. The significant differences between P. koreana and P. dahurica, and between P. turczaninovii and P. ambigua were observed.

CONCLUSIONThe results obtained were in agreement with the traditional taxonomic study. The method was rapid and precise, not only can be used to classify and authenticate Pulsatilla species, but also provides important references for HPLC fingerprints and quality control of Pulsatilla medicinal plants.

Chromatography, High Pressure Liquid ; methods ; Cluster Analysis ; Plants, Medicinal ; chemistry ; classification ; Pulsatilla ; chemistry ; classification ; Quality Control