Adult ; Female ; Herbicides ; poisoning ; Humans ; Paraquat ; poisoning ; Poisoning ; therapy

Adolescent ; Case-Control Studies ; Child ; Child, Preschool ; Heart Defects, Congenital ; blood ; immunology ; Humans ; Infant ; Lymphocyte Subsets ; Th1 Cells ; immunology ; Th2 Cells ; immunology

Objective To investigate the clinical characteristics and mutations of guanosine triphosphate eyclohydrolase (GCH) Ⅰ gene in patients with dopa-responsive dystonia (DRD). Methods Five families with 18 affected family members and 17 patients with sporadic DRD were examined. Patients were allocated into 3 groups according to onset time, either in childhood, or in adolescence or adult. Interview, physical examination, psychologic testings and CT/MR scan were performed. Mutation screening was performed on 26 patients and 1 normal family nember. Thirty-five healthy control subjects were matched for age and sex. Statistical analysis were conducted with the use of SPSS 13.0 computer software. Results(1)Most of patients started with dystonia. The main clinical manifestation was dystonia too. There was no difference among 3 groups.(2) There were significant differences in diurnal fluctuation among 3 groups(15/15,6/6,7/14, χ2=13.125,P=0.001). Diurnal fluctuation negatively correlated with age (r=-0.720, P<0.01).(3)The differences in postural tremor were also found among 3 groups (7/15,5/6,1/14, χ2=8.073, P=0.018). Postural tremor positively correlated with age (r=0.399, P=0.018).(4)There were differences in exaggeration of tendon among three groups(11/15,1/6,4/14, χ2=8.309, P=0.016). Exaggeration of tendon reflexes negatively correlated with age (r=-0.429, P=0.010).(5)The scores of Hamilton Depression Scale and Hamilton Anxiety Scale in patients were higher than those in controls.(6)DNA sequencing revealed a heterozygous A224G missense mutation(Tyr75Cys)located within exon 1 in one autosomal dominant inheritance family. Conclusions The manifestations of DRD varies. The clinical course is closely correlated with age. A missense mutation(A224G)in coding region of the GCH 1 gene probablyleads to the occurrence of DRD.

Objective To improve the understanding of blastic plasmacytoid dendric cell neoplasm (BPDCN). Methods The clinicopathological features of 2 cases with BPDCN diagnosed in the Department of Pathology in Cancer Hospital of Chinese Academy of Medical Sciences and Peking Union Medical College were analyzed retrospectively. The relevant literatures were reviewed as well. Results The two BPDCN patients were both elderly men with skin nodules. The tumor cells infiltrated diffusely into the whole dermis with sparing the epidermis. BPDCN cells were small to medium size with round or oval irregular nuclei and small nucleoli. Numerous mitoses figures were seen. Immunohistochemistry stain showed that CD4 and CD56 were positive and CD20 and CD3 were negative in the 2 BPDCN cases. Only 1 case was positive for CD123. Conclusions BPDCN is a rare malignant hematopoietic tumor, which is likely to involve skin. The features of morphology and immunophenotype are crucial to make correct diagnosis.

Objective To study the expression changes of placental insulin-like growth factor Ⅰ and Ⅱ (IGF-Ⅰ and IGF-Ⅰ) between pregnancies following the in vitro fertilization and embryo transfer (IVF-ET) and those conceived spontaneously.Methods The placenta were collected from 49 cases of pregnancies after IVF-ET(case group) and 49 cases of pregnancies who were normal (control group).The expressions of IGF-Ⅰ and IGF-Ⅱ mRNA in placenta were detected by reverse transcription-polymeras chain reaction (RT-PCR).The levels of IGF-Ⅰ and IGF-Ⅱ protein were determined by immunohistochemistry.Results The mRNA levels of the placental IGF-Ⅰ and IGF Ⅱ were 0.30 ±0.13 and 0.28 ±0.04 in the IVF-ET group,and 0.65 ±0.10 and 0.91 ±0.26 in the control group.The protein levels of the placental IGF-Ⅰ and IGF-Ⅱ were 0.26 ±0.04 and 0.29 ± 0.05 in the IVF-ET group,and 0.32 ± 0.07 and 0.34 ± 0.04 in the control group.The mRNA and protein expressions of IGF-Ⅰ and IGF-Ⅱ in human placenta were significantly decreased in IVF-ET group compared to control group (P ＜ 0.05).The incidence rate of low birth weight infant in IVF-ET group was significantly higher than control(P ＞ 0.05).Conclusions Expressions of placental IGF-Ⅰ and IGF-Ⅱ in the IVF-ET group did not affect fetal growth and development.

Objective To investigate the reliability of extravascular lung water index (EVLWI) and pulmonary vascular permeability index (PVPI) in assessing the severity of acute respiratory distress syndrome (ARDS) in critically ill patients.Methods Forty-six patients with ARDS,who were admitted in our emergency intensive care unit,aged 18-72 yr,weighing 46-72 kg,of Acute Physiology and Chronic Health Evaluation Ⅱ score 11-25,were divided into 3 groups:PaO2/FiO2 ≤ 100 mmHg severe group (n =16);100 mmHg ＜PaO2/FiO2 ≤ 200 mmHg moderate group (n =14);200 mmHg ＜PaO2/FiO2 ≤ 300 mmHg mild group (n=16).Before treatment,and at 24 and 72 h after diagnosis of ARDS,PVPI,EVLWI,cardiac index (CI),and intrathoracic blood volume index (ITBVI) were measured,and blood gas analysis was performed.PaO2/FiO2 was calculated.The 28 day fatality after admission to hospital was recorded.Person correlation of PVPI and EVLWI with PaO2/FiO2,ITBVI and CI was analyzed.Results The PVPI,EVLWI and fatality rate were significantly higher at each time point in moderate group and severe group than in mild group,and in severe group than in moderate group (P＜0.05).The correlation coefficient between PVPI and PaO2/FiO2 was 0.778,and between EVLWI and PaO2/FiO2 was-0.437 (P＜0.05).There was no correlation between CI and ITBVI (P＞0.05).The correlation coefficient between EVLWI and PaO2/FiO2 was-0.448,and between EVLWI and ITBVI was 0.347 (P＜0.05).There was no significant difference between the correlation coefficient between PVPI and PaO2/FiO2 and the correlation coefficient between EVLWI and PaO2/FiO2 (P＜0.05).Conclusion PVPI and EVLWI both can assess the severity of ARDS in critically ill patients,showing a consistent reliability.

BACKGROUND:Total knee arthroplasty mainly used fixed platform, but more and more movable platform was selected year by year in the clinic. Rotary platform theoretical y can apparently improve the curative effects, but it has not been verified in the clinic.
OBJECTIVE:To summarize the design characteristics and clinical results of Gemini MKII (Link, Germany) rotary platform.
METHODS:From January 2004 to January 2009, 108 patients (119 knees) including 31 males and 77 females were treated with total knee arthroplasty by using Gemini MKII system. There were 97 unilateral cases and 11 bilateral cases at the first stage of replacement. Preoperative diagnosis:1 case of hemophilic arthritis, 1 case of venereal Charcot’s disease, 8 cases of post-traumatic arthritis, 11 cases of rheumatoid arthritis, and 87 cases of degenerative osteoarthritis. The prosthesis was fixed with bone cement. None received patel ar replacement. RESULTS AND CONCLUSION:None experienced infection, femoral condyle fracture or nerve and vascular damage. Except bilateral replacement, the average operation time of unilateral replacement was (56±13) minutes. Average postoperative hemoglobin decreased (25±5) g/L. After replacement, hemoglobin maintained over 100 g/L in 83 patients (86%), without blood transfusion. Knee joint score and function score recovered to (92.0±4.7) points and (90.3±6.8) points at half years after replacement, and became (94.4±3.2) points and (91.6±5.9) points during final fol ow-up, which was significantly improved as compared with that pre-replacement (P<0.01). No revision surgery should be done for lining prolapse or spin out, osteolysis and prosthesis loosening. These results indicated that the early and midterm results of Gemini MKII rotating-platform prosthesis were satisfactory, but the long-term results remain to be confirmed.

Objectives In order to discuss the effect ofShujin-Huoxue decoction combined with gradually functional exercise for the ankle fracture postoperative function recovery.MethodsA total of 60 patients with ankle joint fracture were treated by random number table method, and were divided into the observation and control groups, 30 patients in each group. The observation group was treated with the Shujin-Huoxue decoction orally and topical application and gradually functional exercise therapy; while the control group was treated with compound sodium aescinate gel for topital application combined with gradual functional exercise therapy. Two months were a course, so both groups were treated a course. The stretching and flexion angles were measured by protractor. The ankle joint function was measued by the Baird-Jackson rating scale. The clinical curative effect was evaluated.Results The total effect rate in the observation group was 90.0% (27/30) and control group was 73.3% (22/30). There was significant difference between two groups (χ2=2.004,P=0.039). After treatment, the feet stretching Angle (15.69° ± 3.02°vs. 13.50° ± 2.36°,t=6.314) and plantar flexion Angle (32.07° ± 0.34°vs. 27.95° ± 5.40°,t=10.205) in the observation group were significantly higher than those in the control group (P<0.05).Conclusions TheShujin-Huoxue decoction combined with gradually functional exercise could improve the ankle activity and the clinical curative effect.

Objective To investigate the diagnosis value of immunophenotype and karyotypes in newly diagnosed chronic lymphocytic leukemia (CLL).Methods To retrospect the flow cytometry (FCM) immunophenotype and karyotypes characteristics in newly diagnosed 70 CLL cases.Results In all cases,the positive rates of CD19,CD20,CD5,CD23,CD22 were 100 ％,88.5 ％ (54/61),77.1％ (54/70),67.6 ％ (23/34)and 51.9 ％ (27/52),respectively.And 6 were misdiagnosed,2 was CD+5CD+19(+),but CD20,CD22 were strongly positive,final diagnosed as mantle cell lymphoma (MCL) by FISH t(11;14) examination and CyclinDl; CD+5CD+19(-) CLL were 16 cases (22.9 ％),but 4 were misdiagnosed,the misdiagnosis rate was 25 ％,significantly higher than that of CD+5CD+19(-) CLL (P =0.030).59 cases were examined by conventional cytogcnetic (CC),and 13 cases were with abnormal karyotypes,positive rate was 22.0 ％,with complex karyotypes in 5 cases (8.5 ％); 10 cases combined with FISH abnormalities karyotype examination rate was 60 ％.Conclusion Typical CLL immunophenotypic characteristics were CD5,CD19,CD23 co-expression,and CD－5 CLL with higher misdiagnosis rate,combined with CD20 (,) CD22 expression and karyotype analysis helps to CLL and other B lymphoid proliferative diseases (B-LPD) identification.Conventional cytogenetic detection combined with FISH scan can improve the recognition ability of abnormal chromosome.

Objective To evaluate the CT findings of pancreatic carcinoid tumors.Methods The CT imaging data of five patients with pancreatic carcinoid tumors confirmed by pathology were retrospectively analyzed.Results The tumors ranged in maximum diameter from 2.0 to 11.0 cm with a mean of 6.4 cm. On unenhanced CT,the tumors were slightly hypodense relative to the pancreatic parenchyma,homogenous in 2 cases,and heterogenous in 3 cases.One tumor showed calcification.After contrast material injection, the solid component of the tumor showed marked heterogenous enhancement on the arterial phase scanning in 3 cases,and mild heterogenous enhancement in 2 cases.The degree of tumor enhancement was less intense than the surrounding pancreatic parenchyma due to necrosis of various degree,which led to the cystic appearance of the tumor in 1 ease.On the portal phase scanning,all tumors showed marked enhancement similar to that of the pancreatic parenchyma.On the delayed phase scanning,the degree of enhancement was more intense than the surrounding pancreatic parenchyma in 1 case.Liver metastases with retroperitoneal lymphadenopathy and peripancreatic vessels invasion were seen in 1 case.No dilatation of the biliary tract or pancreatic duct was present.Conclusion The CT features of pancreatic carcinoid tumors included infrequent dilatation of the biliary tract or pancreatic duct and unusual vascular involvement,calcification within the mass,marked enhancement similar to that of the surrounding pancreatic parenchyma during the portal phase scanning and more intense during the delayed phase scanning.