Renal medullary cystic diseases are a large class of heterogeneous diseases,and they are common in clinic,most of them often progress to end-stage renal disease.With recent advances in genetics,increasing number of genes and genetic mutations has been identified with the etiology of renal medullary cystic diseases.Although genetic testing can provide mass data for diagnosis,clinical manifestation are even more important for clinical diagnosis,differentiation and genetic counseling for the patients.In this review,the classification,renal presentations,extra-renal presentations,and genetic analysis of renal medullary cystic diseases will be discussed.

Objective To explore the cause, clinical feature, rescue measures and prognosis of tracheo-innominate artery fistula (TIF). Methods From January1995 to January 2015, there were 621 patients who were performed tubotomy, and 8 patients had TIF. The diagnosis of TIF were established by surgery exploration or autopsy. Results The interval between tracheostomy and TIF was 8- 78 d. Before TIF, hemoptysis occurred in 4 patients. When TIF occurred, bedside aid was performed and 4 patients quickly died of asphyxia because of massive blood in the trachea. The others lived long enough to reach the operating room. The family of 1 patient refused surgical therapy and he eventually died. Three patients accepted median sternotomy. One patient underwent vascular repair and died after surgery because of infection in repaired area. The other patients accepted ligation of the innominate artery without suction drains in the mediastinum and died after surgery because of re-bleeding due to mediastinal infection. The last one patient underwent ligation of the innominate artery with suction drains in the mediastinum, and was still alive without long-time neurological complications and re-bleeding after a follow-up of 14 months. Conclusion A prompt diagnosis and surgical intervention can save the life of TIF patient. Prevention is very vital because of the high mortality of this disease.

Objective To explore the correlation between serum uric acid levels and uric acid excretion indexes in children with primary nephrot?ic syndrome(PNS),and to reveal the prevalence of hyperuricemia and its possible causes in PNS. Methods The clinical data in 74 cases of pe?diatric PNS were retrospectively analyzed. These patients were grouped as hyperuric acid(HUA)or non?hyperuric acid(NUA),and their clinical features and laboratory findings were compared between two groups,including age,serum uric acid,serum creatinine,urea,cystatin C,triglycer?ide,cholesterol,albumin,creatinine clearance rate,24 hours urine uric acid,serum uric acid/serum creatinine,uric acid clearance,and the frac?tional excretion of uric acid and other indicators of differences. In addition ,a correlation analysis was carried out for the excretion of uric acid index. Results Several factors were lower in HUA group than that of NUA group ,such as the serum albumin and fractional excretion of uric acid(P1=0.034,P2=0.025);while the serum cystatin C and serum uric acid/serum creatinine ratio(P1=0.038,P2=0.001)was higher in HUA group. The uric acid excretion was negatively correlated with serum uric acid levels in all 74 children with PNS(r=-0.43,P=0.016),and the serum uric acid/creatinine ratio was positively correlated with serum uric acid(r=0.486,P=0.001). Conclusion Both the increase of blood uric acid production and excretion reduction were observed in children with PNS ,and the serum uric acid levels and renal tubular function is closely related.

This paper reports a case of melanotic neuroectodermal tumor of infancy (MNTI)arising in the maxilla of a 3-month-old male infant.The treatment included surgical excision of the lesion with safe margin,curettage of the maxilla and removal of associated developing tooth bud.Microscopically,it proved to be a dual tumor with small,neuroblastic-like cells and larger epithelial cells.Immunohistochemical staining demonstrated epitheloid cells HMB45(+),EMA(+),CK(+);neuroblast-like cells NSE(+),GFAP(+),S-100(+),but both cells Vim(+),CD45(-),Myogenin(-).The 18-mouth follow-up showed no recurrence or metastasis.The related literature was re-viewed.

A high-performance liquid chromatography/electrospray ionization tandem mass spectrometry (LC-ESI-MS/MS) method was developed and validated for the determination of mizoribine in human serum using thiamphenicol as internal standard (IS). The serum samples of mizoribine were precipitated with acetonitrile and separated by HPLC on a reversed phase C18 column with a mobile phase of 0.1% ammonium acetate water solution-methanol (47:53, v/v). Mizoribine and IS were detected in the multiple reaction monitoring mode with precursor/product ion transitions of m/z 258.2/126.0 and 354.1/185.2, respectively. The calibration curves were linear over the range of 0.02-2 microg mL(-1) for mizoribine. The limit of quantification (LOQ) was 0.02 microg mL(-1) with acceptable precision and accuracy. The validated method was successfully applied for the evaluation of a bioequivalence study on Chinese healthy volunteers. The main pharmacokinetics parameters after oral administration of 100 mg mizoribine test or reference formulation were as follows: Cmax (1.00 +/- 0.21), (1.00 +/- 0.22) microg mL(-1); AUC(0-infinity) (6.72 +/- 1.39), (6.48 +/- 1.44) microg h mL(-1); t1/2 (2.77 +/- 0.26), (2.66 +/- 0.29) h; tmax (2.95 +/- 0.78), (2.84 +/- 0.50) h.

Objective To explore the safety and efficacy of resistance training (RT) and aerobic exercise (AT) for low back pain during pregnancy. Methods From June to October, 2015, 34 women gestated 20-23 weeks with low back pain accepted resistance training and aero-bic gymnastics exercises twice a week for 12 weeks. They were measured with blood pressure and body mass index (BMI), and assessed with Visual Analogue Scale (VAS) of pain, Self-rating Anxiety Scale (SAS) and Oswestry Disability Index (ODI) before and after treatment. They were investigated the injury and symptoms with questionnaire. Results No musculoskeletal injuries and groin bleeding occurred. Symptoms, such as dizziness and pelvic pain were infrequent (1.5%). The BMI increased (t=-5.791, P<0.001) and the blood pressure did not obviously change (t=1.441, P=0.159). The muscle strength (t=-5.081, P<0.001) and endurance (t=-5.019, P<0.001) increased, and the scores of VAS (t=-5.179, P<0.001), ODI (t=-5.206, P<0.001) and SAS (t=-5.024, P<0.001) significantly improved after treatment. Con-clusion The resistance training combined with aerobic training program during pregnancy is safe and efficacious for pregnant women with low back pain.

Objective To investigate the effects of intrathecal (IT) lidocaine on propofol-induced sedation and content of lidocaine in brain tissues in rats.Methods Twenty male Sprague-Dawley rats,aged 2-3 months,weighing 250-350 g,were equally and randomly assigned to one of four groups:control group (group C),iv lidocaine group (group IV-L),IT normal saline group (group IT-NS) and IT lidocaine group (group IT-L).Groups IT-NS and IT-L received IT normal saline 15μl and 2％ lidocaine 15 μl,respectively.Group IV-L received iv 2％ lidocaine 15 μl.Propofol was infused starting from 10 min after IT or iv administration.When the eyelash reflex disappeared,the infusion of propofol was stopped and the dose of propofol consumed was recorded.The rats were sacrificed in IT-L and IV-L groups and brains were removed for determination of lidocaine level in brain tissues (by RP-HPLC).Results Compared with groups C,IV-L and IT-NS,the dose of propofol consumed when the eyelash reflex disappeared was significantly decreased in group IT-L (P ＜ 0.05).No significant difference was found in the propofol requirement when the eyelash reflex disappeared between groups C,IV-L and IT-NS and in the content of lidocaine in brain tissues between groups IV-L and IT-L (P ＞ 0.05).Conclusion Sedation induced by lidocaine administered intrathecally is not due to a direct action of lidocaine on the brain in rats.

Objective To observe the effect of low tidal volume lung protective ventilation management strategy on postoperative outcome of elderly patients with poor pulmonary function after abdominal surgery.Methods Eighty patients of poor pulmonary function undergoing open gastrointestinal surgery,male 64 cases,female 16 cases,aged over 65 years old,ASA physical status Ⅱ or Ⅲ,NYHA cardiac function Ⅱ or Ⅲ grade,expected operation time 2-4 h were screened.The patients were randomly divided into 2 groups: protective ventilation management group (group P) and conventional mechanical ventilation group (group C),40 cases in each group.Multi-mode anesthetic management was performed in both groups.The respiratory parameters were adjusted according to the group after tracheal intubation,and the respiratory rate was adjusted to maintain PETCO2 35-45 mm Hg.The blood gas evaluated postoperative oxygen and postoperative spontaneous breathing recovery time,recovery time,extubation time,PACU time,gastrointestinal function recovery time,ambulation time,hospital stay and cost of hospitalization were recorded.The occurrence of major complications were observed at 30 days after surgery.Results PaO2 of group C was significantly decreased at 1 and 3 days after surgery than that before operation (P<0.05),PaCO2 of group C was significantly higher at 1 and 3 days after surgery than that of group P (P<0.05);PACU residence time of group P was (76.63±29.72) min,significantly shorter than that of group C [(93.80±42.90) min] (P<0.05);The difference spontaneous breathing recovery time,awake time,extubation time,exhaust time,ambulation time,postoperative hospitalization time and hospitalization expenses of two group was not statistically significant.Within 30 d after operation,2 cases (5%) of respiratory failure patients,3 cases (7.5%) of pneumonia in group P;5 cases (12.5%)of respiratory failure patients,3 cases (7.5%) of pneumonia,postoperative hemorrhage in 1 cases (2.5%) and 1 cases (2.5%) delirium in group C,there was no significant difference of the main complications in 30 d after operation between two groups.Conclusion Under the condition of this research,low tidal volume lung protective ventilation management strategy can improve elderly patients with poor pulmonary function after abdominal surgery postoperative oxygen and help to reduce the occurrence of postoperative adverse reactions.

Objective To investigate the clinical characteristics of childhood anaphylactoid purpura.Methods Seven hundred and sixty children with anaphylactoid purpura were included in this retrospective study.The clinicopathological features of childhood anaphylactoid purpura were analyzed,including age at onset,gender,season at onset,clinical and pathological manifestations,and complications such as purpura nephritis.Results Childhood anaphylactoid purpura commonly affected preschool and school-age children,and usually occurred in winter or spring.Of these patients,265 (34.87％) had gastrointestinal symptoms,298 (39.21％) had joint involvement,and 223 (29.34％) had renal impairment.Purpura nephritis mainly manifested as haematuria,proteinuria and nephritic syndrome,and was diagnosed in 91.91％ (91) of the patients receiving renal biopsy.The pathological grade of purpura nephritis varied from Ⅱ to Ⅲ in these patients.The distribution pattern of purpura was associated with complications.Conclusions Anaphylactoid purpura has age and season predilection.Purpura in both lower and upper extremities is likely to be complicated by gastrointestinal haemorrhage and joint involvement,and lower extremity purpura is more frequently to be complicated by nephritis than purpura in both lower and upper extremities.In general,childhood anaphylactoid purpura is a mild condition with a good prognosis.

The severity of disease can be used to evaluate the current situation of patients as well as to predict the diseases outcome.In the meantime, the severity among different diseases has been more and more applied in the evaluation of the patients at hospital level.This study collected and summarized different types of international disea-ses grouping tools'characteristics and applications based on the severity of disease, and their suitability and practical values in hospital performance evaluation were compared and analyzed.