Objective To explore the clinical features of congenital hepatic fibrosis (CHF) in children.MethodsA total of 111 patients diagnosed with CHF during January 2002 to June 2015 were included and divided into children group and adult group according to the age at diagnosis. The clinical data including gender, age, clinical symptoms, signs, laboratory examinations, and imaging characteristics were retrospectively analyzed and compared between two groups.ResultsThere were high incidences of splenomegaly and liver cirrhosis in both children and adult groups, and no difference was found between two groups (P>?0.05). The fatigue, jaundice, and hepatosplenomegaly were more common in children group while the renal cyst and liver cyst were more common in adult group, and the differences were statistically signiifcant (P all?

Objective To assess the clinical effect of Percutaneous transforaminal endoscopic spinal surgery for central lumbar spinal stenosis.Methods Total 92 patients (44 males and 48 females with average age of 68.6± 12.4 years) with central lumbar spinal stenosis were enrolled in our study during February 2012 and July 2016.All patients were treated with Percutaneous transforaminal endoscopic spinal surgery.Clinical and Radiographic evaluation were investigated on 1 week,3 months,6 months,12 months postoperatively and final follow-up.Visual analogue scale (VAS) for low back pain and leg pain,Oswestry disability index(ODI) for low back pain were used to evaluate the clinical efficacy of surgery.Pre and postoperative Dural sac cross sectional area(DSCA) was measured.And the correlation between changes of DSCA and clinical efficacy was analyzed.Results The operation time was 45-1 15 min.The mean operation time was 75±15 min.the mean blood loss was 15±5 ml (range 8-50 ml).All patients were followed for 12-46 months (24.5±5.3 months).VAS for low back pain,VAS for leg pain,and OD1 were significantly improved from 6.75± 1.28,7.79± 1.15 and 39.82％ ±5.06％ preoperatively to 2.21± 1.08,2.16± 1.14 and 9.82％ ±3.69％ at the latest follow-up.Dural sac cross sectional area significantly increased from 55.35±12.18 mm2 preoperatively to (102.36±15.38) mm2 at the latest follow-up.Correlation coefficient with DSCA change was-0.480 for ODI change,-0.612 for VAS(low back pain) change,-0.637 for VAS (leg pain)(P ＜ 0.05);obvious positive correlation existed between the change of DSCA and patient's clinical efficacy.It showed that the change of DSCA was positively correlated with the patient's clinical efficacy.The clinical results were excellent in 56 cases.good in 29,fair in 4,and poor in 3 based on the MacNab criteria.92.39％ demonstrated a good-to-excellent outcome.One case occurred cauterizing syndrome,3 cases of recurrence,and 2 cases of Dural tear.There was no severe vascular or nerve injury.Conclusion Percutaneous transforaminal endoscopic spinal surgery provides a new minimally invasive treatment for central lumbar spinal stenosis.It is safe and effective.It is found that the change of DSCA is positively correlated with the clinical efficacy of surgery.

INTRODUCTIONMinimally invasive surgery (MIS) is increasingly used in the treatment of lumbar stenosis. However, it is still not clear if the employment of minimally invasive surgical techniques can achieve superior clinical outcomes compared to standard open laminectomy.

MATERIALS AND METHODSAn extensive literature review regarding the clinical outcome, safety, and efficiency of MIS and standard open surgery (OS) in the treatment of lumbar stenosis was conducted on Medline, Cochrane, EMBASE, and Google Scholar databases up to 19 August 2016.

RESULTSSixteen studies that enrolled a total of 1580 patients with surgically-indicated lumbar stenosis were identified; 793 patients underwent MIS and 787 patients underwent conventional OS. No significant difference was found in the improvement of Oswestry Disability Index (ODI) (P = 0.718) and operation time (P = 0.322) between patients from different treatment groups. MIS was associated with better visual analogue scale (VAS) for back pain (P = 0.01), shorter length of hospital stay (P <0.001), and lower blood loss (P <0.001).

CONCLUSIONOur findings indicate that both MIS and standard OS can effectively manage patients with lumbar stenosis and lead to comparable clinical outcomes. Further studies are necessary to evaluate MIS with different types of conventional surgery for lumbar stenosis.

Objective:To investigate the clinical practice of chronic kidney disease-mineral and bone disorder (CKD-MBD) in maintenance hemodialysis patients in Shanghai, and to better understand the changes of clinical practice for CKD-MBD.Methods:Sixty-four hospitals with qualified dialysis center in Shanghai were selected for questionnaire survey as of March 2019. The survey questionnaire included the number of hemodialysis and peritoneal dialysis patients, the implementation of CKD-MBD guidelines, the learning of CKD-MBD guidelines, the detection and distribution of CKD-MBD biochemical indicators, the treatment of hyperphosphatemia, the treatment of secondary heperparathyroidism (SHPT) and renal bone disease, and the concentration of calcium ion in dialysate. The results were compared with previous survey data in 2011.Results:There were sixty-three hospitals included in this study, with 10 168 maintenance hemodialysis patients and 4 610 maintenance peritoneal dialysis patients in Shanghai. 84.1%(53/63) hospitals implemented the guidelines smoothly, which increased by 28.5% compared with the rate (55.6%) of 2011. The successful implementation rates for guidelines in secondary and tertiary hospitals were 83.3%(25/30) and 84.8%(28/33) , which increased by 44.0% and 11.7% respectively (39.3% of secondary hospitals and 73.1% of tertiary hospitals in 2011). All hospitals carried out the detection for serum calcium and phosphorus. The rate for parathyroid hormone (PTH), total alkaline phosphatase (AKP), bone specific alkaline phosphatase (BAP), 25-hydroxy vitamin D[25(OH)D], and other bone metabolism-related biomarkers were 98.4%(62/63), 90.5%(57/63), 19.0%(12/63), 90.5%(57/63) and 42.9%(27/63), respectively; coronary artery CT, lumbar lateral X-ray plain, echocardiography, bone mineral density, and vascular ultrasound were carried out in 68.3%(43/63), 74.6%(47/63), 100.0%(63/63), 68.3%(43/63)and 69.8%(44/63), respectively. Compared with 2011, the proportion of detection for PTH, AKP, BAP, 25(OH)D, coronary artery CT, lumbar lateral film and echocardiography increased by 2.1%, 1.6%, 0.5%, 47.9%, 14.6%, 20.9% and 1.9%, respectively. The proportion of patients with serum phosphorus ranging in 0.80-1.45 mmol/L(KDIGO guideline), serum phosphorus ranging in 0.80-1.78 mmol/L(KDOQI guideline), calcium ranging in 2.10-2.54 mmol/L, and PTH ranging in 150-600 ng/L were 37.0%(3 323/8 969), 50.7%(4 571/9 018), 60.2%(5 568/9 244) and 33.2%(3 018/9 087). Compared with 2011(39.6%, 53.5% and 34.1%), the proportion of patients with ideal serum phosphorus (0.80-1.78 mmol/L) and calcium (2.10-2.54 mmol/L) levels increased by 11.1% and 6.7% respectively, and the proportion with PTH 150-300 ng/L decreased by 0.9%. The proportion of hospitals for using non-calcium phosphate binders (lanthanum carbonate from 1.9% to 87.3% and sevelamer carbonate from 14.8% to 63.5%) and surgical treatment (from 38.9% to 68.3%) for SHPT dramatically increased.Conclusions:Through the availability of medicine increases, and nephrologists gain deeper understanding in management and treatment of CKD-MBD, the detection rate for CKD-MBD indicators and the eligible rate have significantly improved compared with those in 2011. However, the comprehensive management of CKD-MBD in Shanghai still faces great challenges. It is still necessary to further improve eligible rate for serum phosphorus and iPTH, so as to provide more evidences and management strategies for integrated management of end-stage renal disease and prevention of abnormal calcium and phosphorus metabolism in patients.