Objective To study and to improve the understanding of the etiology, diagnosis and treatment of familial congenital adrenal hyperplasia(CAH) due to 17? hydroxylase deficiency. Methods 2 rare cases of 17? hydroxylase deficiency in a family were studied with review of the literature. Results 2 cases were definitely diagnosed,one being male pseudohermaphroditism and the other female hypogonadism.Treatment consisted of replacement doses of glucocorticoid hormones and supplemented by estrogen therapy.The serum potassium became normal and the blood pressure was kept below 150/90 mm Hg. Conclusions 17? hydroxylase dificiency is a kind of hypertensive forms of CAH and is inherited as an autosomal recessive trait,its prevalence being rare.It is associated with hypogonadism or pseudohermaphroditism, hypertension or hypokalemia and with absent/decreased 17 OHCS and 17 KS.Hypoaldosteronism and positive dexamathasone inhibition test are the outstanding features.

Objective To access the efficacy of the arterial based complexity (ABC) scoring system in predicting complexity of LPN.Methods A total of 70 patients underwent laparoscopic partial nephrectomy from January 2013 to November 2015 were enrolled in the retrospective analysis.Among those patients,53 (75.7％) were males and 17 (24.3％) were females.Their mean age was 52 years,ranged from 44 to 61 years.The average BMI was 28 kg/m2,ranged from 25 to 32 kg/m2.Before operation,the average creatinine was 70.5 μmol/L,ranged from 60.5 to 81.0 μmol/L.43 patients accepted the procedure via retro-peritoneal approach and 27 patients accepted the procedure via peritoneal approach.38 tumor located in the left kidney and 32 tumor located in the right kidney.By using ABC scoring system,four readers independently scored contrast-enhanced computed tomography images of 70 patients who underwent laparoscopic partial nephrectomy.Interobserver variability was assessed with kappa values and percentage of exact matches between each pairwise combination of readers.Logistics regression was used to evaluate the association between reference scores and ischemia time,estimated blood loss,operation time.Results The operative duration ranged from 100 to 180 min,mean 147 min.The mean ischemic time ranged from 15 to 37 min,mean 24 min.The average blood loss was 210 ml (ranging 50-380 ml).The mean hospitalization was 16 days (ranging 11-21 days).The urine leakage was noticed in 2 patients after the operation.The size of tumor ranged from 1.8 to 3.0 cm,mean 2.5 cm.The pathological classification included renal cell carcinoma in 65 cases,renal cell carcinoma with cystic changing in 2 cases,renal chromophobe cell carcinoma in 2 cases,right renal solitary fibroma in 1 case.Based on the ABC scorirng system,15 cases were in 1 degree,29 cases were in 2 degree,17 cases were in 3S degree and 9 cases were in 3H degree.Pairwise comparisons of readers' score assignments were significantly correlated;average kappa =0.492,across all reader pairs.The average proportion of exact matches was 65％,the average proportion that differ between a level or less than a level was 98.6％.Logistics regression between the complexity score system and surgical outcomes showed significant associations between reference category assignments and warm ischemia time and estimated blood loss (P ＜ 0.05),but showed no significant associations with operation time (P ＞ 0.05).Conclusions The ABC scoring system for LPN demonstrated good correlation with perioperative morbidity.This system is a novel anatomy-reproducible tool developed to help patients and doctors understand the complexity of renal masses and predict the outcomes of kidney surgery.

Recent studies find that the expression of hepatoma-derived growth factor (HDGF) is closely correlated with the tumorigenesis,progression,metastasis,therapy and prognosis of various malignancies.Though the mechanism of the interaction of HDGF and tumors is still unclear,the research of HDGF serving as a tumor marker in malignancies and a new target for tumor treatment has become a hotspot in the research of tumor.

Objective To improve the diagnosis and therapy of primary renal synovial sarcoma . Methods A single case of primary renal synovial sarcoma was reported .A 54-year-old female presented with left flank pain for six days .Enhanced CT scan revealed a nearly-circular mass with the diameter of 2.0 cm in the superior portion of the left kidney .It showed low and high density with punctate calcification .There were low and high density in left kidney around .A possible diagnosis of left renal tumor with rupturing was given prior to exploratory operation .During the operation , a large hematoma was found on the lateral peri-re-nal fascia, which adjoined with other organs .The hematoma was opened and the 1 000 ml dated clot and blood were removed .The tissue inside the hematoma showed fish meat like .The fast pathologic examination showed sarcomatoid tissue , then the case underwent radical left nephrectomy . Results Gross pathologic examination showed a ashen and fish-liked tumor of 3.0 cm×2.5 cm×1.5 cm with multiple sites of necrosis and hemorrhage .The histopathological examination revealed the tumor cells were spindle shaped .The tumor cells organized as bundle with indistinct border line and larger quantity of mitotic phase , which were positive for BCL-2, vimentin, EMA, CD79a and SMA, but negative for CD34, CK and CD10.A diagnosis of renal syn-ovial sarcoma was then determined .Targeted therapy with sunitinib ( 37.5 mg po qd ) was carried out postopreratively .There was no recurrence and metastasis during the six months following up . Conclusions Primary renal synovial sarcoma is an extremely rare entity .It is very difficult to diagnose through clinical symptoms and imaging .The accurate diagnosis is based on pathology or genetic and molecular tests .Radical nephrectomy and adjuvant therapy are the preferred strategy for the treatment .The prognosis is poor .Targeted therapy with sunitinib may be effective .

0.05). Conclusions TVT is a safe, effective and minimally invasive surgical procedure for treatment of female stress urinary incontinence.