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.

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.

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 investigate the effect of BPI-1095 on caspase-3 protein expression in middle artery occlusion(MCAO) rats.Methods Cerebral ischemia was induced with MCAO in adult male SD rats.Rats were randomly subjected into 6 groups with 15 rats in each group.Each rat has been given tested medication of different dosage and was sacrificed 24 h after treatment.The area of infarction was measured on each slice by image analysis system.Meanwhile,immunohistochemistry staining was used to identify caspase-3 expression in ischemic brain tissue.Results The infarcted area were significantly decreased in big and moderate dose treated rats(P<0.05,vs the placebo group).The expression of caspase-3 protein decreased in contralateral and ipsilateral hemisphere areas.The caspase-3 positive cell was significantly decreased in rats treated with big doses compared with placebo-or ASA-treated rats.Conclusion BPI-1095 shows neuroprotection in MCAO rats,which may related with the inhibition of caspase-3 expression resulting in apoptosis in penumbra.

Objective:To investigate the efficacy and safety of programmed death-1 (PD1) inhibitor combined with transcatheter arterial chemoembolization (TACE) in the treatment of huge primary liver cancer.Methods:From June 2016 to December 2019, the clinical data of 31 patients with huge primary liver cancer enrolled in the Central Hospital of Lishui were retrospectively collected and analyzed. The tumor size ranged from 10.1 to 18.8 cm, with an average of (14.2±2.3) cm. The patients were divided into TACE group (TACE treatment, 18 cases) and combined group (one week after TACE, patients receiving a dose of 200 mg PD1 inhibitor administration every 21 days, 13 cases), according to whether patients receiving PD1 inhibitors. The patients were followed up. The disease control rate (DCR) were compared between the two groups using Mann-Whitney U test. The median overall survival (OS) and progression free survival (PFS) were calculated by Kaplan-Meier method. Results:The DCR in combined group (53.8%, 7/13) was higher than that in TACE group (22.2%, 4/18), and the difference was statistically significant ( Z=-2.13, P=0.04). The median PFS (5.0 months) in combined group was longer than that in TACE group (3.0 months), the difference was statistically significant (χ2=4.39, P=0.04). The median OS (15 months) in combined group was longer than that in control group (9 months), and the difference was statistically significant (χ2=5.51, P=0.02). Conclusion:The combine PD1 inhibitors with TACE is an effective and safe therapy for huge primary liver cancer.

By summarizing and analyzing the clinical diagnosis and treatment experience of 17 cases of adrenal lymphangioma, the imaging characteristics and pathological types of the disease were discussed. The results showed that the imaging of adrenal lymphangioma was non-specific, and the appearance was similar to that of general cysts.Howerer, the density of the cyst was slightly higher than that of simple cysts. Some cases showed calcification on the cyst wall, and a few showed adenoma-like appearance. The diagnosis mainly depends on pathological examination. For those patients with tumors ≥4.0 cm, endocrine function, suspected malignancy, or obvious clinical symptoms, surgery is recommended.

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.