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.

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.

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.

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.

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 HLA-B27 in disease activity and the clinical features of axial spondyloarthritis(SpA). Methods Clinical data of 112 patients with axial SpA was collected and studied prospectively. Clinical manifestations and laboratory examination results of 82 HLA-B27 positive and 30 HLA-B27 negative patients with axial SpA were analyzed. Data source was from Chinese Rheumatism Data Center. Results (1)The average age of onset of HLA-B27 negative patients was significantly later than that of the positive patients , and there was no significant difference in the course of disease and the proportion of male and female patients. (2)The ratio of severe lesion of hip ,peripheral arthritis ,attachment inflammation and systemic symptoms of HLA-B27 negative group were significantly lower than those of HLA-B27 positive group. Familial aggregation phenomenon,uveitis and spine radiology changes in two groups had no significant difference.(3)The changes of disease activity index including erythrocyte sedimentation rate and C-reactive protein increased in HLA-B 27 negative group was significantly lower than those in HLA-B27 positive group. Conclusion There is strong correlation between axial SpA and HLA-B27. The average age of onset of HLA-B27 negative patients was significantly later than that of the positive patients. HLA-B27 negative patients manifested severe symptoms and worse prognosis.

Glycemic excursion was induced in SD rats by intraperitoneal injection of 50% glucose solution, and cells isolated from bone marrow of these rats showed cell clusters which expressed insulin, c-peptide, glucagon, somatostatin and islet amyloid polypeptide, and other genes related to islet-cells development and functions.

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.

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.