ObjectiveTo explore the clinical manifestations of infant cytomegalovirus infection in different age. MethodThe clinical data of 237 infants who suffered from cytomegalovirus infection was analyzed retrospectively and divided into three groups: 0-3 months old (77 cases,group A) ,4-6 months old ( 65 cases , group B ) , 7-12 months old ( 95 cases , group C ). ResultsThe incidence of respiratory infection was the highest among all infectious organs in three groups, the numbers of patients who had wheeze in group A was less than that in the other two groups[24.7％(19/77) vs. 61.5％ (40/65), 61.1％( 58/95 )](P ＜ 0.01 ).The incidence of jaundice decreased gradually as the babies grew up[23.4％( 18/77 )→7.7％(5/65 )→1.1％( 1/95 )](P ＜ 0.05). There was no significant difference in diarrhoea and bleeding among three groups (P＞0.05 ). The proportion of alanine aminotransferase], aspartate amino transferase increasing was similar among three groups, but gamma-glutamyl transferase (GGT) was different, the proportion of GGT increasing was 77.9％ (60/77) in group A which was higher than that in the other two groups[10.8％ ( 7/65 ), 2.1％ ( 2/95 )].Granulocytopenia in group B was obviously decreased compared with the other two groups (P ＜ 0.05 ), anemia was easily occurred in group C (P ＜ 0.05 ). ConclusionThe injury of cytomegalovirus infection may be related to month old.

Objective To investigate the number, structure and demand of rehabilitation professionals hospitals above grade 2A in Jiangsu. Methods 176 hospitals above grade 2A in Jiangsu were investigated with questionnaire in the topics of general condition of department of rehabilitation medicine, structure of rehabilitation professionals and demand of rehabilitation professional. Results 64% of the hospitals has established department of rehabilitation. Rehabilitation professionals, such as the rehabilitation physician, therapists, nurses and engineer were in a total of 1657, and 231 professionals were needed further. Conclusion There are problems of lack of the department of rehabilitation medicine and rehabilitation professional, with the imbalance structure of professional in hospitals above grade 2A in Jiangsu.

Objective To investigate the clinical characteristics,treatment effect,long-term follow up results,guanosine triphosphate (GTP) cyrclohydrolase Ⅰ (GCH Ⅰ)gene and tyrosine hydroxylase(TH) gene mutations in patients with dopa-responsive dystonia (DRD).Methods The clinical features of 3 families with 4 affected members were analyzed and all of 4 patients were screened for mutations of the GCH Ⅰ gene and TH gene with DNA sequences.Results Four patients were females,average age at onset was (15.3 ± 5.6) years (range:from 9 to 20 years).The initial symptoms were a gait disorder,stiffness or tremor of the lower limbs in all patients presented with diurnal fluctuation.As the increase of disease duration,bilateral hand tremor was found in three patients,systemic torsion was found in one patient and torticollis was found in one patient.All patients' symptoms were in complete remission after administration of low dose of levodopa.Four patients were followed up for 0.5 to 10.0 years,and all were still responsive to the levodopa treatment and effective dosage was decreased as the increase of the disease duration.No longterm side effects of levodopa had occurred after long-term treatment.One patient was found to have c.607G ＞A(p,Gly203Arg) heterogenetic mutation in GCH I gene.Molecular analysis revealed a compound heterozygous mutation in the TH gene (p.Y447Ter and p.V468M) in one patient.No point mutations in both genes were found in other patients.Conclusions DRD patients have dramatic and sustained response to levodopa and no long-term side effects of levodopa after long-term treatment.The detection of GCH Ⅰ and TH gene mutations is helpful in early diagnosis but the negative results could not exclude the diagnosis of DRD.

Objective To study the influence of Salviae Miltiorrhizae Radix et Rhizome (SMR) and Carthami Flos (CF) before and after compatibility on activitis of cytochrome P 1A2 (CYP1A2),cytochrome P2E1 (CYP2E1),and cytochrome P3A4 (CYP3A4) from rat liver microsomes.Methods Using caffeine,chlorzoxazone,and midazolam as the probe drugs ofCYP1A2,CYP2E1,and CYP3A4,the SD rats were randomized divided into four groups:control group,SMR (1.2 g crude drug/kg) group,CF (0.4 g crude drug/kg) group,and SMR (1.2 g crude drug/kg) + CF (0.4 g crude drug/kg) group.According to the above dose,rats were ig given drugs for 7 d.Rats were injected with caffeine,chlorzoxazone,and midazolam solution in tail vein 30 rain after the last administration,and the blood was collected at different time points.Metronidazole as internal standard,method has been established to determine the levels of caffeine,chlorzoxazone,and midazolam to evaluate the activities of CYP1A2,CYP2E1,and CYP3A4 by HPLC.Results Compared with control group,SMR increased the clearance rates (CL) of caffeine,chlorzoxazone,and midazolam,reduced the AUC,and t1/2 was also show a decreasing trend,but the difference was not significant.In CF group,CL of caffeine and chlorzoxazone was decreased,but the difference is not significant.CL of midazolam significantly decreased (P ＜ 0.01).AUC of chlorzoxazone increased,but the difference was not significant.AUC of caffeine and midazolam increased significantly (P ＜ 0.05 and 0.01).In SMR + CF group,the CL of caffeine and chlorzoxazone decreased significantly (P ＜ 0.05),the AUC of caffeine and chlorzoxazone increased significantly (P ＜ 0.05),and t1/2 also showed a decreasing trend,but the difference is not significant.Conclusion Compatibility of SMR and CF has an inhibitive effect on CYP1A2 and CYP2E1 in rats,and it could be one of the mechanisms ofinteractive synergy.

Objective To evaluate the clinical feasibility of grading of ureteral stricture in renal allograft.Methods The clinical data of 9 case of ureteral stricture after renal transplantion from February 2015 to September 2015 were retrospectively analyzed and relative articles were reviewed to evaluate the significance of ureteral stricture grading.The diagnosis of ureteral stenosis was made by serum creatinine elevation,ultrasound of allograft kidneys and presence of stricture on a pyelogram.Grade 1 ureteral stenosis was defined as graft function deterioration with presence of hydronephrosis on ultrasound,but no obvious stricture identified on a pyelogranm.Grade 2 was defined as serum creatinine elevation,presence of hydronephrosis on ultrasound,and a focal (＜1 cm) distal ureteral stricture at the ureteral anastomotic site on a pyelogram.Grade 3 was defined as serum creatinine elevation,hydronephrosis on ultrasound,and a long segment (＞1 cm) distal ureteral stricture extending to proximal ureter or pelvis on pyelogram.Results One case,6 cases and 2 cases were classified to grade 1,2 (stricture length ＜1 cm) and 3 (＞1 cm),respectively.One case of grade 1,6 cases of grade 2 and 1 case of grade 3 were successfully dilated by balloon dilation catheter and endoureterotomy.One case of grade 3 was converted to open reconstructive surgery.Surgery success rate of grade 1 and 2 was 100％ (7/7) and 100％ (6/6) 6 and 9 months after surgery,respectively.Ureteral stenosis did not recur in one case of grade 1 and 6 cases of grade 2 during a follow-up period of 12 months after surgery.However,one case of grade 3 reoccurred 6 months after surgery,and reoperation was performed and there was no recurrence up to now (10 months).Conclusion Wuzhi tablet acted as an effective agent that makes rifampicin containing anti-tuberculosis chemotherapy possible and safe by stabilizing blood concentration of tacrolimus in post-renal transplant tuberculosis patients,which also reduced the dosage of tacrolimus as well as the risk of acute rejection greatly.

Objective To explore the expression of anti-MICA antibodies and evaluate its influence on acute rejection and renal function in early period after renal transplantation. Methods A total of 29 sensitized subjects (PRA＞20 %) were enrolled in this study. All the patients underwent protein A immunoabsorption treatment and the expression of anti-MICA antibodies was detected before and after treatment. Triple immunosuppressive regimen consisting of tacrolimus, mycophenolate mofetil (MMF) and steroid was given to prevent graft rejection. The correlation between the expression of anti-MICA antibodies and acute rejection or serum creatinine (SCr) level was analyzed.Results The expression of anti-MICA antibodies was detected in 8 candidates (27. 6 % ,8/29) ,and 6 kinds of anti-MICA antibodies simultaneously expressed were found in one individual, 3 kinds in one case,and sole kind in 6 patients. There was no significant difference in acute rejection rate between positive anti-MICA antibodies group and negative group [37.5 % (3/8) vs 38. 1% (8/21), P＞0.05). The positive expression rate of anti-MICA antibodies in the recipients with PRA ≥40% was higher than that in those with PRA ＜40% [43. 8 % (7/16) vs 7. 7 % (1/13),P＜0.05]. The SCr level in patients positive for anti-MICA antibodies was markedly higher than that in those negative anti-MICA antibodies at the 1st week postoperatively ( 135.4 ± 21.4 vs 108. 6 -+ 31.6 μmol/L, P＜0.05). The SCr level in the patients with positive anti-MICA antibodies, however, was reduced to the normal range at the 2nd week after surgery (P＞0.05). The levels of anti-MICA antibodies were continuously decreased in the candidates undergoing protein A irnmunoadsorption treatment. Conclusion Higher expression of anti-MICA antibodies exists in sensitized recipients and possesses an influence on the recovery of renal function in early postoperative period. Protein A immunoadsorption can eliminate anti-MICA antibodies effectively in sensitized recipients.

Objective To assess the role of muhi-detector row CT (MDCT) in preoperative evaluation of living renal donors. Methods The data of 104 potential donors who underwent MDCT were retrospectively analyzed. All the candidates underwent 64-MDCT examination. First,unenhanced scans were performed on the kidneys. After administration of Ⅳ contrast medium,enhanced CT images of the arterial phase, venous phase, and excretory phase were obtained. The enhanced scan scope was from the top of diaphragmatic muscle to pubic symphysis. The scanning data obtained was post-processed for reconstructed images. The anatomy and variations displayed in MDCT images on kidneys, ureters, arteries and veins were recorded. The findings in surgery constituted the standard of reference for imaging findings, and the recorded results from images were compared with the findings in surgery to assess the role of MDCT in evaluation of potential donors. Results MDCT examination was successfully performed on 104 candidates. Anomalies of kidneys and ureters were found in 8 donors before surgery. The prevalence of accessory arteries and early branching in image was 27. 2 % (28/103) and 12. 6 % (13/103) respectively. There were 3 candidates with double veins and 3 with retroaortie left renal vein found in preoperative assessment. Ninety-three candidates underwent successful donor nephreetomy. The anomalies and variations of kidneys and ureters in images were all confirmed surgically. The detection rate of the accessory renal artery (ARA) was 80 %. The ARAs measuring > 1 mm in diameter and early branching were all detected by MDCT.The findings of veins found in performed sides coincided with those of MDCT images. Conclusion MDCT can accurately assess the anatomic information and variations of the donors' kidneys, and facilitate triaging donors and planning operation proposal

Objective:To explore the method of ultrasonography for detecting the fetal umbilical vein diameter, blood flow volume and normalized volume blood flow and establish normal reference ranges with umbilical vein diameter, blood flow volume and normalized blood flow and Z-scores for umbilical vein diameter and blood volume flow.Methods:This was a prospective study on 907 normal fetuses in the Second Xiangya Hospital, Central South University and Women and Children Healthcare Hospital of Zhuzhou from March 2019 to December 2020. The umbilical vein diameter (Duv), umbilical vein blood flow volume (Quv) and normalized volume blood flow (nQ = Quv/estimated fetal weight) of the free loop of umbilical vein (FUV) and fetal intra-abdominal umbilical vein (IUV) were collected. And the mean values and 90% confidence intervals of Duv, Quv and nQ in two segments of umbilical veins at different gestational ages were calculated. Regression analysis of Duv, Quv and nQ were performed with gestational age (GA), and the parameters of umbilical vein in different segments were compared. Finally, with gestational age (GA) as the independent variable, Z-scores of the Duv and Quv were built.Results:The mean values and 90% confidence intervals of Duv, Quv, and nQ in 858 (94.6%) normal fetal umbilical veins were successfully obtained. The Duv, Quv of the FUV and IUV increased as pregnancy progressed. The Quadratic curve of Duv and Linear curve of Quv were of the highest fitnesses, respectively( r=0.951, 0.941, 0.986, 0.982; all P<0.001). While nQ increased with GA followed by a decreased trend, and the Quadratic curve was the highest fitting curve of nQ( r=0.610, 0.611; all P<0.001). Duv-FUV was greater than Duv-IUV( P<0.001), nQ-FUV was bigger than Quv-IUV( P=0.001), and he difference was not statistically significant between Quv-FUV and Quv-IUV( P=0.133). Z-scores models of Duv and Quv were successfully established, and all Z-scores were Gaussian distribution. Conclusions:The normal ranges and Z-scores of umbilical vein parameters are useful to improve the evaluation of placental circulation and provide a strong basis for the monitoring of fetus-related diseases and the evaluation of pregnancy prognosis. The choice of FUV or IUV umbilical vein to evaluate placental circulation may depend on the actual situation in clinical application.

OBJECTIVE: To analyze the clinical, familial and hereditary features of myotonic dystrophy to improve the knowledge and provide molecule evidence for gene diagnosis and prenatal diagnosis of myotonic dystrophy or dystrophia myotonia (DM) families. METHODS: Clinical data of 2 DM families were collected based on the probands. The number of trinucleotide CTG repeat in the 3' untranslated region of myotonic dystrophy protein kinase (DMPK) gene on chromosome 19 was determined by DNA sequence and repeat fragment. RESULTS: Except for 1 subclinical patient, another 5 patients progressed slowly with the features of myotonic muscular weakness and atrophy. One patient had hatchet face, 1 had cataract and diabetes mellitus, and the other 3 were bald. Electromyologram showed 3 patients had myotonic discharge and myopathic abnormalities. The number of trinucleotide CTG repeat in the 3' untranslated region of DMPK gene of 5 patients exceeded 50. CONCLUSION DM can be anticipated. Gene analysis can verify the disease and identify subclinical patients. It helps to prevent the DM births by hereditary consultation performing prenatal diagnosis.
Adolescent ; Adult ; Female ; Humans ; Male ; Myotonic Dystrophy ; diagnosis ; genetics ; Myotonin-Protein Kinase ; Pedigree ; Polymerase Chain Reaction ; methods ; Protein-Serine-Threonine Kinases ; genetics ; Trinucleotide Repeats

BACKGROUNDOrgan preservation keeps the quality of the organs under prolonged ischemia. Continuous machine perfusions are gaining an important position in clinical research and practice. The aim of this study was to evaluate the protective effect of continuous hypothermic machine perfusion transport system (AirdriveTM) on cold ischemic injury of canine kidney.

METHODSTen kidneys of five healthy preserving canines were taken out after general anesthesia. Five kidneys were stored using common cold preservation (CCP group) by immersing it in the organ preservation solution, mixed with water and ice, and kept in a cold room at 4°C. The other five kidneys were stored using continuous machine perfusion preservation (CMP group) and were placed into the Airdrive(TM) continuous machine perfusion device at room temperature. The renal tissues were examined by histopathology, electron microscopy, and mitochondrial activity check at different time points.

RESULTSHistologic sections showed that the structures of the ten renal tissues were similar during the first 24 hours. After 48 hours, the CCP group showed more pronounced changes, as the renal tubular epithelial cells were more obvious than those in the glomeruli. Oxygen consumption rate of state III and IV respiration in the CCP group decreased after 12-48 hours and increased at 48 hours, respectively, when compared to the CMP group (P < 0.05). Cortex respiratory control ratio and phosphorus oxygen ratio were significantly higher in the CMP group at 48 hours.

CONCLUSIONWith prolonged storage time, the effect of continuous hypothermic machine perfusion transport system is better than that of common cold preservation on canine kidney.