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 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 .

Objective:To measure the anatomical parameters of the simulated low tibial tunnel of posterior cruciate ligament (PCL) based on knee CT images so as to provide clinical reference for accurate location of the tunnel.Methods:The CT images of 201 healthy knee joints collected at Department of Orthopedics, The Second Hospital of Lanzhou University from June 2016 to September 2021 were used for simulation of the PCL low tibial tunnel. The anatomical parameters of the tibial tunnel were measured using the RadiAnt DICOM Viewer. The primary measures included the angle between tibial plateau and tibial tunnel (ATPT) and the perpendicular distances from the tibial tunnel entrance and exit point to the tibial plateau (L1 and L2). The secondary measures included the angle between tibial plateau and posterior slope (PSA), the angle between tibial anatomical axis and central line of tibial tunnel (ATAA), the angle between posterior tibial slope line and the central line of tibial tunnel (APST), the anterior and posterior diameter of tibial plateau (APD), the length of posterior tibial slope (LPTS), and the length of tibial tunnel (LTT). The measurement results were analyzed according to the body height (divided into 3 groups: a 1.00 to 1.60 m group, a 1.61 to 1.70 m group, and a ≥1.71 m group) and gender using the software IBM SPSS 26.Results:The primary measures: ATPT was 37.0°±4.5°, and L1 and L2 were respectively (57.8±7.4) mm and (34.5±3.3) mm. The secondary measures: PSA 128.1°±5.4°, ATAA 52.7°±4.1°, APST 89.1°±5.9°, APD was (32.9±2.6) mm, LPTS (20.5±2.4) mm, and LTT (40.9±5.7) mm. After grouping by gender, there was no significant difference in PSA between men and women ( P>0.05) while there were significant differences in the other indexes between men and women ( P<0.05). After grouping by body height, there was no significant difference in ATPT, PSA, APST or ATAA between the 3 groups (1.00 to 1.60 m group, 1.61 to 1.70 m group and ≥1.71 m group) ( P>0.05) while there were significant differences in L1, L2, APD, LPTS and LTT between the 3 groups ( P<0.05). Conclusions:Based on the knee CT images, the primary measures of PCL low tibial tunnel are as follows: the angle between tibial plateau and tibial tunnel is 37.0°±4.5°, and the perpendicular distances from the tibial tunnel entrance and exit point to the tibial plateau are (57.8±7.4) mm and (34.5±3.3) mm, respectively. Gender and body height are the important factors influencing the above measurement outcomes.