Objective To improve the recognition of nervous system symptoms of inborn errors.Methods Five patients with organic acidemias were screened by urine organic acid analysis(gas chromotography-mass spectrometry,GC/MS),3 cases of methylmalolic acidemias(MMA) and 2 cases of propionic acidemias(PA) were confirmed.They were treated with special diet and medicine after diagnosis.Result The improvement of mental development was observed after treatment.Conclusions Most of organic acidemias involve nervous systems,causing non-specific symptoms of nervous system as lethergy,seizures,mental retardation.Inborn errors of metabolism shall be kept in mind when causes of the symtoms of acidosis,seisures,mental retardation and lethergy are investigated.GC/MS is a very important method in diagnosis of organic acidemias.Early diagnosis and early treatment can improve the mental prognosis.

Animals ; Herbicides ; poisoning ; Humans ; Paraquat ; poisoning

OBJECTIVETo evaluate the method and effectiveness of rigid-bronchoscopic cryosurgery for advanced central lung cancer.

METHODSForty-eight patients were enrolled in this study from June 2002 to December 2008, including 33 male and 15 female. The average age was 70 years (ranged from 45 to 83 years old). For the 48 patients, 38 cases were patients with advanced central lung cancer who were not suitable for surgery, and the remaining 10 cases were patients with local recurrence in trachea or main bronchus postoperatively. Cryosurgery was performed 120 times for all patients, 2.5 times per patient on average. The trachea or bronchus station, symptom such as dyspnea, hemoptysis, respiratory function and quality of life were observed.

RESULTSThe unblocked ratio of trachea and bronchi was 97%. All patients got satisfied improvement ratio of symptoms, 87.5% for dyspnea, 72.9% for cough, 93.8% for hemoptysis and 62.5% for chest pain. Respiratory function tests showed that both the mean forced expiratory volume in first second and forced vital capacity got an improvement from (1.03+/-0.05) L to (1.85+/-0.13) L and from (1.69+/-0.18) L to (2.96+/-0.14) L respectively (P<0.01). Karnofsky score also got no less than 20 scores improvement. The Follow-up time was 6 to 62 months. The longest survival was 62 months. The median survival time was 20 months. There was no severe perioperative complications and mortality except for 3 cases of moderate exeduation.

CONCLUSIONSCryosurgery is easy to perform with minimal complications. Not only could it provide an effective and rapid control of symptoms caused by central lung cancer, it could also unobstructed bronchus promptly and improve patients' quality of life.

Aged ; Aged, 80 and over ; Bronchoscopy ; Cryosurgery ; methods ; Female ; Humans ; Lung Neoplasms ; surgery ; Male ; Middle Aged ; Treatment Outcome

OBJECTIVETo investigate the severity related influencing factor and treatment strategy of severe acute pancreatitis with early organ dysfunction.

METHODSFrom July 2007 to December 2008, 167 patients with severe acute pancreatitis were treated in the Surgical Department of Ruijin Hospital. The relationships between the happening of early organ dysfunction and outcome of the patients were observed, with operative or nonoperative treatment strategy.

RESULTSAmong 167 patients, 68 patients have early organ dysfunction, in which 39 with single organ dysfunction and 29 with multiple organ dysfunction. The early organ dysfunction were involved in 47.1% in cardiovascular system, 35.3% in lung and 29.4% in kidney. Aging (P < 0.05) and higher APACHE II score (P < 0.05) predicted a poor prognosis, which were benefit from early operation.

CONCLUSIONSThe mortality of the patients with SAP is related to age, and the degree of organ dysfunction as well. In the first phase of the disease, the selection of operation depends on the trends and the degree of early organ dysfunction before infected necrosis happens, with the aid of SOFA score as a scale.

Acute Disease ; Aging ; Humans ; Multiple Organ Failure ; Pancreatitis ; diagnosis ; Prognosis

OBJECTIVETo investigate the relationship between the clinical character and therapeutic strategy and prognosis in severe acute pancreatitis.

METHODSFrom January 2001 to December 2005, 783 patients with SAP were treated. Therapeutic strategy was selected based on the preliminary scheme for diagnosis and treatment of severe acute pancreatitis by pancreatic surgery society of CMA. All the patients were divided into biliary group and non-biliary group, while 375 patients in biliary group, with 182 patients treated operatively and 193 patients treated nonoperatively; and 408 patients in non-biliary group, with 147 patients treated operatively and 261 patients treated nonoperatively.

RESULTSThere were 698 survivals, the overall survival rate was 89.1%. 357 survivals in the biliary SAP group, the survival rate was 95.0%, in which 171 survivals from operation treated cases, with the survival rate of 94.0%, and 186 survivals from non-operation treated cases, with the survival rate of 96.4%; 341 survivals in the non-biliary SAP group, the survival rate was 84.0%, in which 110 survivals from operation treated cases, with the survival rate of 74.8%, and 231 survivals from non-operation treated cases, with the survival rate of 88.5%. 48.3% patients of the survival group had organ dysfunction, and 18.3% patients had multiple organ dysfunctions, while 100% patients of the death group had organ dysfunction, and 97.6% patients had multiple organ dysfunction. Respiratory dysfunction was found to be the most common cause totally followed by nerve system dysfunction and shock, with the rates of 26.3%, 11.7% and 10.3%, respectively. Respiratory dysfunction, renal dysfunction and cardiac dysfunction are most commonly in death group, with the rate of 94.1%, 60.0% and 60.0%, respectively. The rate of fungi infection in the survival group and death group were 8.9% and 37.6%. The rates of alimentary tract fistula in the survival and death group were 0.9% and 14.1%, respectively.

CONCLUSIONSThe therapy aiming at the cause for biliary SAP and the operation aiming at infected pancreatic necrosis is helpful to improve curative rate; MODS is the main cause of death in severe acute pancreatitis. Respiratory dysfunction, renal dysfunction and cardiac dysfunction are high risk factors.

Female ; Humans ; Male ; Middle Aged ; Pancreatitis, Acute Necrotizing ; diagnosis ; mortality ; therapy ; Prognosis ; Retrospective Studies ; Survival Rate

OBJECTIVETo evaluate the health-related quality of life and postdischarge long-term outcome after severe acute pancreatitis.

METHODSThe hospital records of patients with SAP discharged healthy from January 2003 to December 2003 were reviewed. The Rand 36-item Health Survey with accessory question was mailed to each patient. The means and deviations for each of eight scales scores of SF-36 were calculated, the study population scores were compared with general Chinese population; Univariate analysis was applied to determining the effects of variables such as age, sex, causes of disease, mode of treatment, frequency of surgery, financial burden, length of stay, chronic complications. Accessory questions were analyzed separately.

RESULTSThe means and deviations for each of eight scales (PF, RP, RE, BP, VT, MH, SF, GH) scores of SF-36 in SAP patients were 83 +/- 15, 62 +/- 42, 69 +/- 36, 80 +/- 15, 69 +/- 19, 72 +/- 15, 75 +/- 18, 65 +/- 18, compared with general people. Except RP and SF, the others were similar. In the ANOVA of Physical Component Summary, the three variables mode of treatment, financial burden and length of stay were included (P < 0.05), while in that of Mental Component Summary, the two variables of gender and financial burden were included (P < 0.05).

CONCLUSIONSThe health-related quality of life in SAP patients is similar to that of general people. Greater attention should be given to mode of treatment, length of stay and financial burden to improve quality of life.

APACHE ; Adolescent ; Adult ; Aged ; Analysis of Variance ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Pancreatitis, Acute Necrotizing ; psychology ; therapy ; Quality of Life ; Retrospective Studies ; Surveys and Questionnaires ; Survivors ; Treatment Outcome

Objective: To explore the safety and efficacy of a novel lead locking device (LLD) in the procedure of cardiac lead extraction for heart rhythm implants. Methods: A total of 6 patients using LLD for cardiac lead extraction in our hospital were retrospectively reviewed. Clinical parameters, the reason of cardiac lead extraction, lead locking stylet condition, outcome of lead extraction and operative complications were summarized. Results: There were 6 patients including 1 female with the median age at 62.5 years. LLD was used and 13 cardiac leads were extracted including 1 scrap electrode wire and 12 functional electrode wire. Among those, LLD was successfully inserted and locked on the top of 11/13 (85%) leads for whole procedure and 2 (15%) leads were not locked for whole procedure; 12 (92 %) leads were completely removed and 1 (8%) lead was partially removed. No severe complications occurred. Conclusion: The novel LLD may safely and effectively extract electrode lead which is beneficial for complete cardiac lead extraction.

AIM To study the chemical constituents from the large polar fraction of the roots of Lindera reflexa Hemsl.and their antitumor activities.METHODS The large polar fraction from the roots of L.reflexa was isolated and purified by silica gel column,Sephadex LH-20 gel column,semi-preparative HPLC and ODS medium pressure column,then the structures of obtained compounds were identified by physicochemical properties and spectral data.The antitumor activities were determined by MTT method.RESULTS Thirteen compounds were isolated and identified as 2,6-dimethoxy-4-hydroxyphenyl-1-O-β-D-glucopyranoside(1),3-hydroxy-4,5-dimethoxyphenol-β-D-glucopyranoside(2),syringin(3),1-O-3,4-dimethoxy-5-hydroxyphenyl-(6-O-3,5-dimethoxygalloyl)-β-D-glucopyranoside(4),p-cymen-7-yl β-D-glucopyranoside(5),pisumionoside(6),staphylionoside D(7),dendranthemoside B(8),lynoiside(9),nudiposide(10),icariside B1(11),(2S)-pinocembrin-7-O-(6-O-α-L-rhamnopyranosyl-β-D-glucopyranoside)(12),(+)-N-(methoxycarbonyl)-N-norboldine(13).Compounds 3 and 13 showed obvious cytotoxicity against human lung cancer cells(A549)and human gastric cancer cells(MGC80-3).CONCLUSION Compounds 1-13 are isolated from the roots of L.reflexa for the first time.Compounds 3 and 13 have good anti-tumor activities.

To investigate the effect of catalpol on high-fat diet(HFD)-induced nonalcoholic fatty liver disease(NAFLD)and its underlying molecular mechanisms. Sixty C57BL/6J male mice were randomly divided into six groups:control group;HFD group;HFD+catalpol(100 mg/kg)group;HFD+catalpol(200 mg/kg)group;HFD+catalpol(400 mg/kg)group;and HFD+atorvastatin calcium(ATC)(30 mg/kg)group.The control group was fed a normal diet containing 4.4 kJ/g fat,whereas the other five groups were fed a high-fat diet containing 19.8 kJ/g fat.Mice in the catalpol or ATC treatment groups were administered by gavage for different doses of catalpol or ATC,whereas other mice were treated with saline.Body weight was measured once a week.Experiments were terminated after 18 weeks,and blood and liver samples were collected after an overnight fast(12 hours)for analysis.The body weight and liver weight were measured and the levels of serum total cholesterol(TC),triglyceride(TG),high-density lipoprotein cholesterol(HDL-C),low-density lipoprotein cholesterol(LDL-C),alanine aminotransferase(ALT),and aspartate transaminase(AST)as well as inflammatory factors tumor necrosis factor-α(TNF-α),interleukin(IL)-1β,and IL-6 were determined by commercially available kits.Liver sections were stained with Oil Red O and HE to investigate the lipid accumulation and histopathological changes.The protein expressions of nuclear factor kappa-B(NF-κB)p65,inhibitor of nuclear factor kappa-B α(IκBα),B-cell lymphoma-2(Bcl-2),Bcl-2 associated x protein(Bax),and Caspase-3 were determined by Western blot. Compared to the model group,the body weight gains(all =0.001),liver index(=0.008,=0.001,=0.001),ALT(=0.004,=0.001,=0.001),and AST(=0.008,=0.001,=0.001)were significantly decreased in catalpol treatment groups,and the serum levels of TC(=0.005,=0.001),TG (all =0.001),and LDL-C(all =0.001)were also significantly decreased in middle and high dose groups,and the serum level of HDL-C was significantly increased in high group(=0.009).Moreover,compared to the model group,the degree of liver injury and lipid accumulation were obviously decreased in the catalpol treatment groups according to the pathology.Similarly,the release of inflammatory factors was significantly inhibited by the treatment with catalpol.The results of Western blot showed that the protein levels of NF-κB p65(=0.014,=0.001,=0.001)and Caspase-3(all =0.001)in the livers of HFD-fed mice were significantly reduced by catalpol treatment.In addition,the protein level of IκBα(=0.028,=0.001,=0.001)and the ratio of Bcl-2/Bax in high dose group(=0.003)was increased by treatment with catalpol. Catalpol can effectively improve the body weight gains,liver index,dyslipidemia,and lipid accumulation in HFD-fed mice and inhibit the release of inflammatory factors and hepatocyte apoptosis,thereby preventing the development of NAFLD induced by HFD.
Animals ; Diet, High-Fat ; Iridoid Glucosides ; Male ; Mice ; Mice, Inbred C57BL ; Non-alcoholic Fatty Liver Disease

Objective:To analyze the clinical features, genetic characteristics, treatment and follow-up results of patients with hydrocephalus caused by methylmalonic acidemia combined with homocysteinuria, and to discuss the optimal strategies for assessing and treating such patients.Methods:From January 1998 to December 2020, 76 patients with hydrocephalus due to methylmalonic acidemia combined with homocysteinuria in the Department of Pediatrics in 11 hospitals including Peking University First Hospital were diagnosed by biochemical, genetic analysis and brain imaging examination. The patients were divided into operation-group and non-operation-group according to whether they underwent ventriculoperitoneal shunt. The clinical features, laboratory examinations, genotype, and follow-up data were retrospectively analyzed. Data were compared between the two groups using rank sum test, and categorical data were compared using χ 2 test. Results:Among the 76 patients (51 male, 25 female), 5 were detected by newborn screening, while 71 were diagnosed after clinical onset, 68 cases (96%) had early-onset, 3 cases (4%) had late-onset. The most common clinical manifestations of 74 cases with complete data were psychomotor retardation in 74 cases (100%), visual impairment in 74 cases (100%), epilepsy in 44 cases (59%), anemia in 31 cases (42%), hypotonia or hypertonia in 21 cases (28%), feeding difficulties in 19 cases (26%) and disturbance of consciousness in 17 cases (23%). Genetic analysis was performed in 76 cases, all of whom had MMACHC gene variations, including 30 homozygous variations of MMACHC c.609G>A. The most common variations were c.609G>A (94, 62.7%), followed by c.658_660del (18, 12.0%), c.567dupT (9, 6.0%) and c.217C>T (8, 5.3%). Therapy including cobalamin intramuscular injection, L-carnitine and betaine were initiated immediately after diagnosis. A ventriculoperitoneal shunt operation was performed in 41 cases (operation group), and 31 patients improved after metabolic intervention (non-operation group). There was no significant difference in the age of onset, the age of diagnosis, the blood total homocysteine, methionine, and urinary methylmalonic acid concentration between the two groups (all P>0.05). The symptoms of psychomotor development, epilepsy, and visual impairments improved gradually after a long-term follow-up in the operation group. Conclusions:Hydrocephalus is a severe complication of methylmalonic acidemia combined with homocysteinuria. The most common clinical manifestations are psychomotor retardation, visual impairment, and epilepsy. It usually occurs in early-onset patients. Early diagnosis and etiological treatment are very important. Hydrocephalus may improve after metabolic intervention in some patients. For patients with severe ventricular dilatation, prompt surgical intervention can improve the prognosis.