Objective To investigate the clinical significance of central venous oxygen saturation (ScvO2) monitoring in the patients after cardiac operation.Methods Fifty patients after cardiac operation were randomly selected into this study.After the patients entered ICU 2 h,ScvO2,mixed venous oxygen saturation (S(v)vO2),lactate (Lac) were detected at the same time and oxygen extraction rate (O2ER) was calculated.Fifty patients were divided into three groups according to the level of ScvO2,group A (ScvO2 ＜0.65,23 patients),group B (ScvO2 0.65-0.75,18 patients),group C (ScvO2 ＞ 0.75,9 patients).The correlation among ScvO2,S(v)vO2,O2ER were analyzed.The correlation between ScvO2 and Lac in each group were calculated.The level of Lac in three groups were compared.The incidence rate of complications,ventilator time and ICU stay time were compared.Results The level of ScvO2,S（v）O2,O2ER,Lac in 50patients were 0.656 ±0.086,0.639 ±0.081,0.356 ±0.084,(2.6 ± 1.3) mmol/L.The level of ScvO2 had significantly positive correlation with S(v)O2 (r =0.688,P ＜ 0.01),and had significantly negative correlation with O2ER (r =-0.640,P ＜0.01).In group A,the level of Lac had significantly negative correlation with ScvO2 (r =-0.772,P ＜ 0.01).In group C,the level of Lac had significantly positive correlation with ScvO2 (r =0.717,P ＜ 0.05).In group B,the level of Lac had no significant correlation with ScvO2 (r =-0.358,P ＞0.05).The level of Lac in group A and group C was significantly higher than that in group B [(2.0 ± 0.9),(4.8 ±2.1) mmol/L vs.(1.6 ±0.5) mmol/L] (P ＜0.05 or ＜0.01).The incidence rate of hyoxemia,low cardiac output syndrome and renal functional lesion in group A were significantly higher than those in group B (P ＜ 0.05).The incidence rate of liver functional lesion and renal functional lesion in group C were significantly higher than those in group B (P ＜ 0.05).The ventilator time and ICU stay time in group A andgroup C were significantly longer than those in group B (P ＜ 0.05).Conclusions ScvO2 is an ideal index to judge oxygen equilibrium in early period after cardiac operation.The lower and supranormal ScvO2 both suggest tissue hypoxia,resulting in increased postoperative complications and prolonged treatment.

Objective To research the variety and the imaging features of bone tumor and tumor-like lesions in the talus.Methods The imaging features of 33 cases of tumor and tumor-like lesions in the talus were reviewed retrospectively.All cases were confirmed by operation and pathology,All of 33 cases were performed X-ray examination,23 cases were examined by CT,and 11 cases were taken by MR.Results In 33 cases,24 cases were males,9 cases were females; including 7 cases of chondroblastoma(21.2％),7 cases of giant cell tumor(21.2％)(1 case of recurrence),6 cases of osteochondroma(18.2％),2 cases of osteoid osteoma(6.1％),7 cases of adjacent joint bone cyst(21.2％,2 cases of fibrous dysplasia of bone(6.1％),1 case of bone cyst(3％),1 case of malignant fibrous histiocytoma(MFH) of bone(3％).6 cases showed pathological fractures.The X-ray and CT imaging features of chondroblastoma,giant cell tumor,adjacent joint bone cyst,fibrous dysplasia of bone,bone cyst demonstrated cystic bony destruction.The common location of chondroblastoma were the posterior of talus(57％),expanding growth slightly,margin were mild osteosclerosis.The margin were osteosclerosis irregularly and osteal ridges showed in giant cell tumor.MRI features were different on pathologic basis,isointense and hypointense signal on T1WI and hyperintense signal on T2WI usually.The osteochondroma showed osseous protuberance connecting the talus,some cases showed calcification in the cap.The imaging of MFH in the talus X-ray and CT showed ill-defined osteolytic bony destruction,soft tissue-mass,no periosteal reactions and bone formation.MRI showed isointense and hypointense signal on T1WI and isointense and hyperintense signal on T2WI.The extent of tumour invasion clearly displayed.Conclusion Tumor and tumor-like lesion in the talus were rare.But there are great varieties.The benign tumor was more common than malignant tumor.Chondroblastoma,giant cell tumor,osteochondroma,osteoid osteoma and adjacent joint bone cyst were relatively common and had some imaging features.Malignant tumor rarely happened in the talus,but it is possible.

Objective:To investigate the treatment and prognosis of children with propionic acidemia (PA).Methods:This study involved 82 children with PA treated in the Department of Pediatric Endocrinol-ogy and Genetic Metabolism, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine from December 2002 to June 2020. Clinical data, including manifestations, laboratory test results, treatment strategy, and follow-up data, were summarized and analyzed using t-test or Mann-Whitney U test. Results:(1) Among the 82 cases consisting of 50 (61.0%) boys and 32 (39.0%) girls, 59 (72.0%) were diagnosed after clinical onset; 22 (26.8%) were diagnosed by newborn screening, including eight asymptomatic ones; the other one (1.2%) was asymptomatic but confirmed after the diagnosis of PA in the patient's sibling. The average age at first onset was 4.5 months (2 d-5 years) in 73 subjects, of which 28 (38.4%) were early-onset PA (within three months after birth). (2) Cranial MRI was performed on 26 cases, and abnormality was identified in 19 (73.1%) cases. (3) Hyperlactatemia was found in 16 cases among 30(53.3%) who underwent relevant examination with the average lactic acid level of 3.5 (2.1-4.3) μmol/L, while 35 out of 40 patients (87.5%) had hyperammonemia with an average blood ammonia level of 105.4 (34-907) μmol/L. (4) Among the 28 early-onset PA cases, 16 (57.1%) died, and 12 (42.9%) survived. There was no significant difference in the serum propionylcarnitine level, propionylcarnitine to acetylcarnitine ratio, urine 3-hydroxypropionic acid, or methylcitrate level between the survival and death cases. (5) Genetic mutations were detected in 75 patients (91.5%), among which 26 (34.7%) carried PCCA gene mutations and 48 (64%) with PCCB gene mutations. One patient (1.3%) harbored one known pathogenic mutation in each of the PCCA and PCCB genes. All mutations were inherited from the parents. (6) Followed up to June 2020, 57 (69.5%) patients survived, and 25 (30.5%) died from multiple organ failure secondary to severe acidosis, including 16 early-onset and nine late-onset cases. Conclusions:The primary treatment of PA is dietary control. Most PA patients are diagnosed after clinical onset, but symptoms may recur and even have developmental retardation despite treatment. Some of those diagnosed through newborn screening are asymptomatic after treatment. Newborn screening using tandem mass spectrometry is recommended for early diagnosis and treatment of PA.

Objective To measure the diameter of optic nerve sheath by ultrasonography to evaluate the change of intracranial pressure(ICP)in prone position ventilation,and to provide basis for prone position ventilation in patients with increased intracranial pressure (ICH). Methods A total of 58 patients with mechanical ventilation were treated with prospective clinical study from 2016.05.01 to 2017.05.01. The patients were treated with different PEEP and different positions(supine position and prone position),and detected optic nerve sheath diameter(ONSD)behind 3 mm of eye 3 mm by bedside ultrasound. The cause inducing increase of ICP was studied through the changes of ONSD and the data was analyzed by the paired t test. Results Prone position had a significant effect on patients with increased intracranial pressure. PEEP had a significant effect on MAP,Ppeak, Pplat,but had no effect on increased intracranial pressure. Conclusions Prone position ventilation significantly affect the ONSD. Therefore ,it weighs the pros and cons when patients with intracranial hypertension were received prone position ventilation.

Objective To observe the effects of moderate intensity exercise training combined with Xianlinggubao capsule on bone mineral density (BMD), bone metabolism and femoral biomechanics of ovariectomized rats, so as to provide lab references for osteoporosis prevention. Methods Fifty female SD rats were randomly divided into 5 groups, 10 rats in each group. Group A was the normal control; Group B was given 1 mL normal saline by gavage after ovariectomy for one week; Group C was given moderate intensity exercise training (exercise speed was 20 m/min, lasting for 60 min per day, continuous 5 days per week); Group D was given 1 mL Xianlinggubao capsule ［0.4 g/(kg · d)］ after 1-week ovariectomy; Group E was was given both 1 mL Xianlinggubao capsule and moderate intensity exercise training after 1-week ovariectomy. After 8 weeks of continuous treatment, blood biochemical indexes, BMD, micro CT and biomechanics of the femur and L5 vertebral body were detected. Results Compared with group B, the blood biochemical indexes of Group C-E were improved in varying degrees, the BMD of L5 vertebral body and femur were increased, the bone volume fraction, trabecular number and trabecular thickness of femur (or L5 vertebral body) were increased, the trabecular space and structural model index were decreased, the maximum load, maximum deflection and maximum stress of L5 vertebral body were increased, and the maximum stress of femur was increased. The maximum load, elastic load, elastic deflection, elastic modulus, elastic stress, maximum stress and elastic deflection increased, and the effect of Group E was the most obvious. Conclusions Moderate intensity exercise training combined with Xianlinggubao capsule can improve BMD, bone metabolism and bone microstructure, and improve bone mechanical properties of ovariectomized rats.

Objective:To investigate the effect of pulmonary vascular dysfunction in the prognosis of patients with acute lung injury (ALI).Methods:Patients with ALI who underwent pulmonary artery catheterization in the department of critical care medicine of Wuhan NO.1 Hospital from June 2017 to June 2019 were enrolled. The general information, clinical and hemodynamic indexes [central venous pressure (CVP), pulmonary artery wedge pressure (PAWP), pulmonary artery systolic pressure (sPAP), pulmonary artery diastolic pressure (dPAP), mean pulmonary artery pressure (mPAP), cardiac index (CI)], acute physiology and chronic health evaluation Ⅱ (APACHEⅡ) score, arterial blood gas parameters [pH, partial pressure of oxygen (PO 2), partial pressure of carbon dioxide (PCO 2), oxygenation index (PaO 2/FiO 2)], whether there was shock or not; ventilator parameters [platform pressure (Plat), positive end-expiratory pressure (PEEP)], etc. were recorded. Pulmonary artery oxygen saturation, pulmonary vascular function indexes [transpulmonary potential gradient (TPG) and pulmonary vascular resistance index (PVRi)] were calculated. The relationship between TPG, PVRi and mechanical ventilation time, the length of intensive care unit (ICU) stay, cardiovascular days and 60-day mortality were analyzed in patients with different prognosis of 60-day and whether the TPG increased (≥12 mmHg was defined as elevated TPG, 1 mmHg = 0.133 kPa). Results:A total of 65 patients were included in the study, including 30 males and 35 females; aged (48.9±15.2) years old. Forty-eight cases survived in 60-days, 17 died, and the 60-day mortality was 26.2%. At the baseline, there were no significant differences in cardiopulmonary function measurements, such as CVP, sPAP, dPAP, PAWP, CI, etc. between the two groups of patients with different prognosis. The APACHEⅡ score, shock ratio, TPG and PVRi of the death group were significant higher than those of the survival group [APACHEⅡ: 34±9 vs. 28±11, shock: 52.9% vs. 25.0%, TPG (mmHg): 16.2±1.9 vs. 14.6±2.1, PVRi (kPa·s·L -1): 31.8±4.2 vs. 29.7±3.5, all P < 0.05]. The 60-day mortality of 47 patients with TPG ≥ 12 mmHg was significantly higher than that of 18 patients with TPG < 12 mmHg (34.0% vs. 5.6%), and the mechanical ventilation time and the length of ICU stay were also significantly longer (days: 17±9 vs. 11±8, 16±5 vs. 12±5), and the cardiovascular days also increased significantly (days: 23±7 vs. 18±6), and the differences were statistically significant (all P < 0.05). Pearson correlation analysis showed that PVRi was significantly correlated with mechanical ventilation time, the length of ICU stay and cardiovascular days ( r1 = 0.317, P1 = 0.030; r2 = 0.277, P2 = 0.005; r3 = 0.285, P3 = 0.002). In the individual multivariate Logistic regression model, the highest PVRi was an independent risk factor for the 60-day mortality [odds ratio ( OR) = 30.5, 95% confidence interval was 20.4-43.1, P = 0.023]. Conclusion:Pulmonary vascular dysfunction is common in ALI patients and is independently associated with adverse outcomes.

Objective To investigate the impact of quercetin(Que)on postherpetic neuralgia(PHN)and chemokine ligand 3(CCL3,namely MIP-1α)/C-C chemokine receptor 1(CCR1)/C-C chemokine receptor 5(CCR5)signaling pathway in rats.Methods Sixty rats were divided into the control group(Con),the PHN group(model group),the L-Que(30 mg/kg)group,the M-Que(60 mg/kg)group,the H-Que(120 mg/kg)group and the H-Que+pathway activator MIP-1α(120 mg/kg Que+0.4 mg/kg recombinant MIP-1α)group.The mechanical paw withdrawal threshold(PWT)and thermal pain threshold(TWL)of rats were detected in each group.The kit was used to detect adenosine,Adenine ribonucleotide(AMP),adenosine diphosphate(ADP)and tumor necrosis factor in spinal dorsal horn samples-α(TNF-α),and interleukin-1 β(IL-1 β)levels in spinal dorsal horn samples.HE staining was applied to observe the pathological sections of spinal dorsal horn.Immunofluorescence staining was used to detect the activation of microglia in spinal dorsal horn.Western blot assay was applied to detect MIP-1α/CCR1/CCR5 signaling pathway protein expression.Results In the PHN group,the dorsal horn of the spinal cord was ruptured,the arrangement of nerve bundles was disordered,and inflammatory cell infiltration,edema,and slight atrophy of neurons appeared.Compared with the Con group,the PWT value,adenosine,AMP and ADP levels were obviously decreased in the PHN group(P＜0.05),and TWL value,TNF-α,IL-1β levels,the number of Iba1-positive microglia,MIP-1α,CCR1 and CCR5 protein levels were obviously increased(P＜0.05).After treatment with Que,the disordered arrangement of nerve bundles was improved,the infiltration of inflammatory cells was reduced,and the phenomenon of neuronal atrophy disappeared.Compared with the PHN group,the PWT value,adenosine,AMP and ADP levels were obviously increased in the L-Que group,the M-Que group and the H-Que group(P＜0.05).TWL value,TNF-αand IL-1β levels,the number of Iba1-positive microglia,and MIP-1α,CCR1 and CCR5 protein levels were obviously decreased(P＜0.05).The effect of Que was dose dependent.Compared with the H-Que group,PWT value,adenosine,AMP and ADP levels were obviously decreased in the H-Que+MIP-1α group(P＜0.05),and TWL value,TNF-α,IL-1β levels,the number of Iba1 positive microglia,MIP-1α,CCR1 and CCR5 protein levels were obviously increased(P＜0.05).Conclusion Que may reduce the inflammatory response in rats by inhibiting the MIP-1α/CCR1/CCR5 signaling pathway,thereby reducing PHN.

OBJECTIVETo analyze PCCA and PCCB gene mutations in 10 Chinese patients with propionic acidemia(PA).

METHODSGenomic DNA was extracted from peripheral blood leukocytes. The 39 exons and flanking sequences of the PCCA and PCCB genes were amplified with polymerase chain reaction and subjected to direct DNA sequencing.

RESULTSDNA sequencing has revealed that 7 patients have carried a PCCA gene mutation, 2 patients carried PCCB gene mutation and 1 patient carried mutations in both PCCA and PCCB genes. Ten PA mutations were confirmed, including 8 affecting the PCCA gene and 2 affecting the PCCB gene. Three PCCA mutations c.245G>A, IVS15+5del5, c.1288C>T and 2 PCCB mutations c.838insC, c.1087T>C were found for the first time.

CONCLUSIONAmong Chinese patients with propionic acidemia patients, their genetic mutations are mainly found on the PCCA gene.

Child, Preschool ; Female ; Humans ; Infant ; Male ; Methylmalonyl-CoA Decarboxylase ; genetics ; Mutation ; Propionic Acidemia ; genetics

OBJECTIVETo analyze the clinical and molecular features of a child with carnitine palmitoyltransferase 1A (CPT1A) deficiency.

METHODSClinical data of the child was collected. Blood acylcarnitine was determined with tandem mass spectrometry. DNA was extracted from the child and his parents. All exons and flanking regions of the CPT1A gene were analyzed by PCR and Sanger sequencing.

RESULTSAnalysis showed that the patient carried compound heterozygous mutations c.1787T>C and c.2201T>C of the CPT1A gene, which derived his father and mother, respectively. Both mutations were verified as novel through the retrieval of dbSNP, HGMD and 1000 genome databases. Bioinformatic analysis suggested that the mutations can affect protein function.

CONCLUSIONAcyl carnitine analysis has been the main method for the diagnosis of CPT1A deficiency. The c.1787T>C and c.2201T>C mutations of the CPT1A gene probably underlie the disease in this patient. Gene testing can provide important clues for genetic counseling and prenatal diagnosis.

Base Sequence ; Carnitine O-Palmitoyltransferase ; deficiency ; genetics ; Exons ; Female ; Humans ; Hypoglycemia ; enzymology ; genetics ; Infant ; Lipid Metabolism, Inborn Errors ; enzymology ; genetics ; Male ; Molecular Sequence Data ; Point Mutation ; Pregnancy

Objective To analyze the clinical features of patients with coronavirus disease 2019 (COVID-19) in Shanghai and to investigate the risk factors for disease progression to severe cases. Methods The clinical data of 292 adult patients with COVID-19 hospitalized in Shanghai Public Health Clinical Center from January 20, 2020 to February 10, 2020 were retrospectively analyzed, including 21 severe patients and 271 mild patients. The demographic characteristics, epidemiological history, history of underlying diseases and laboratory examinations were compared between the two groups. Measurement data were compared using t test or Mann-Whitney U test. The count data were compared using hi-square test. The binary logistic regression equation was used to analyze the risk factors for the progression of patients to severe cases. Results Among the 292 patients, 21 were severe cases with the rate of 7.2% (21/292). One patient died, and the mortality rate was 4.8% in severe patients. The severe patients aged (65.0±15.7) years old, 19 (90.5%) were male, 11 (52.4%) had underlying diseases, 7 (33.3%) had close relatives diagnosed with COVID-19. The mild patients aged (48.7±15.7) years old, 135 (49.8%) were male, 74 (27.3%) had underlying diseases, 36 (13.3%) had close relatives diagnosed with COVID-19. The differences between two groups were all significant statistically ( t =-4.730, χ 2 =12.930, 5.938 and 4.744, respectively, all P <0.05). Compared with the mild patients, the levels of absolute numbers of neutrophils, alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase, creatinine, serum cystatin C, C reactive protein (CRP), procalcitonin , D -dimer, pro-B-type natriuretic peptide (proBNP), serum myoglobin, creatine kinase (CK), creatine kinase isoenzyme (CK-MB), serum troponin I (cTnI) in severe patients were all significantly higher ( U =2 091.5, 1 928.0, 1 215.5, 729.0, 1 580.5, 1 375.5, 917.5, 789.5, 1 209.0, 1 434.0, 638.0, 964.5, 1 258.0 and 1 747.5, respectively, all P <0.05), while the levels of lymphocyte count, albumin, transferrin, CD3 + T lymphocyte count, CD8 + T lymphocyte count and CD4 + T lymphocyte count in severe patients were all significantly lower ( U =1 263.5, t =4.716, U =1 214.0, 962.0, 1 167.5 and 988.0, respectively, all P <0.05). Further logistic regression analysis showed that the albumin (odds ratio ( OR )=0.806, 95% CI 0.675-0.961), CRP ( OR =1.016, 95% CI 1.000-1.032), serum myoglobin ( OR =1.010, 95% CI 1.004-1.016), CD3 + T lymphocyte count ( OR =0.996, 95% CI 0.991-1.000) and CD8 + T lymphocyte count ( OR =1.006, 95% CI 1.001-1.010) at admission were independent risk factors for the progression of COVID-19 patients to severe illness (all P <0.05). Conclusions Severe cases of patients with COVID-19 in Shanghai are predominantly elderly men with underlying diseases. Albumin, CRP, serum myoglobin, CD3 + T lymphocyte count and CD8 + T lymphocyte count could be used as early warning indicators for severe cases, which deserve more clinical attention.