Objective To study the clinical and genetic characteristics of congenital hyperinsulinism (CHI).Method From November 2016 to November 2018,a cohort of 21 patients with CHI were retrospectively reviewed in our hospital.The second generation sequencing was performed on Ion torrent platform to identify the genetic etiology of this disease.Result The average birth weight was 3 748 g.Overall,11 of the 21 patients (52.4％) were large for gestational age (＞ Pg0),and 90.5％ (19/21) onset within 3 days after birth.Seizures occurred in 7 cases (33.3％).Diazine was effective in alleviation in 6 patients,octreotide was effective in 8 patients,and diazine and octreotide were ineffective in 7 patients.11 patients underwent 18-fluoro-l-dopa PET-CT scan,indicating focal islet cell proliferation in 8 patients and diffuse islet cell proliferation in 3 patients.8 focal cases accepted the partial pancreatectomy (7 cases who failed to respond to drug therapy,1 case who received octreotide had significant liver damage),and all of these 8 cases were confirmed with focal islet cell proliferation by postoperative pathology.7 cases had normal glucose level after surgery,while 1 case developed insulin-dependent diabetes.Patients of 3 cases with diffuse islet cell proliferation had no consent for surgery.Gene mutations were identified in 61.9％ (13/21) of patients (12 cases of ABCC8 gene and 1 case of KCNJ11 gene).15 sequence changes were identified (14 in ABCC8 and 1 in KCNJ11).3 new ABCC8 gene mutations (exon13:c.1822C ＞ T,p.Q608X;exon22:c.2691delC,p.W898Gfs*5;exon33:c.4039C ＞T,p.Q1347X),and 1 new KCNJJ11 gene mutation (exon1:c.629T ＞ A,p.H315Y) were firstly reported.Conclusion Partial pancreatectomy is an effective treatment for those CHI not responsive to drug,however,with an attendant risk of developing diabetes.Mutations of ABCC8 gene and KCNJJ11 gene are the main pathogenic genes of CHI.Patients with different gene mutations may have different responses to drug treatment.Therefore,it is necessary to improve gene testing in clinical practice to guide treatment and prognosis.3 new ABCC8 gene mutations and 1 new KCNJJ11 gene mutation were first reported,which expand the spectra of gene mutation.

Objective:To describe the real experience of the elderly who has felt happy during the time they stayed at nursing homes, and discuss the meaning which is behind their experience during the COVID-19 pandemic, to provide scientific basis for improving their subjective well-being.Methods:Qualitative explanatory phenomenological analysis methods, and semi-structured in-depth interviews were used to interview 21 elderly people living in Guangzhou Songhe Nursing Home. Data analysis and subject extraction were carried out with reference to the Colaizzi 7-step analysis.Results:The real experience of subjective well-being of the elderly in nursing homes has six themes: be satisfied with the nursing home management service, be satisfied with harmonious interpersonal relationship, be satisfied with good health, be satisfied with family support, grateful and expectations for the social support system, be satisfied with and grateful for the epidemic prevention work during the COVID-19 epidemic.Conclusions:The elderly who live in a nursing home with perfect management and service, care for each other, are healthy, receive family and social support, are grateful, and understand family and social support will be more likely to feel happy. Improving the subjective well-being of the elderly in nursing homes requires the coordination and support of nursing homes, the elderly, families, society and other aspects.

Objective: To investigate the clinical efficacy of dorsal mosaic surgery with penis free flap for the treatment of anterior urethral stricture after TURP. Methods: We analyzed the clinical data of the patients with anterior urethral stricture after TURP from January 2010 to December 2017 in Yan′an hospital affiliated to Kunming medical university retrospectively. The patients' age ranged from 58 to 75 years, with an average of 64.3 years. The time from TURP to the diagnosis of anterior urethral stricture was 1-12 month, with an average of 3.5 months. 5 cases were urethral stricture at penis segment, 11 cases were urethral stricture at the junction of penis and scrotum, and the length of the narrow urethra was 2-5 cm, with an average of 3.4cm. The average maximum uroflowmetry in preoperative was (5.3±2.7) ml/s.11 cases were treated with regular urethral dilatation and the treatment durable time was more than 6 months, 5 cases were treated with intraurethral incision combined with urethral dilatation(1 or 2 times). 16 cases were not effective after receiving the above treatment, so that all cases were treated with dorsal mosaic surgery with penis free flap. Subarachnoid anesthesia combined with epidural anesthesia, the patient took the supine position.The distal end of urethral stricture was defined by urethral dilator. Incision from the ventral side of the urethra. The length of the incision was extended 0.5 cm based on the length of urethral stricture in urethral angiography.Anatomize the left and right sides of the urethral stricture and longitudinally incision the ventral side of the urethral cavernous body. The length of the incision was extended 0.5 cm to the normal urethral mucosa. The traction line retracts the ventral urethral edge along both sides.The corresponding medial line of the dorsal urethra was incised to the tunica albuginea, and the urethral edge of the dorsal side was separated from the tunica albuginea to form an elliptical region on the tunica albuginea.According to the size of the ellipse, the full thickness of the penis flap was taken, and the size of the flap was beyond the edge of the elliptical area about 0.3 cm.The free flap was covered with the 6-0 absorbable suture on the elliptical area (the skin surface was on the inner side of the urethra), the edge of the free flap was intermittently sutured with the urethral edge of the dorsal side, Multi-needle intermittent sutured flap surface on the corpus cavernosum bed.Using a silicone catheter as the stent tube of the new urethra, the 6-0 absorbable suture closes the ventral side of the incisional urethral sponge.The multi-layered meat film was sutured to prevent leakage of urine, and the fascia and skin were sutured layer by layer. The 5-0 absorbable thread sutures the wound after the foreskin was taken. Results: The operations were successfully completed. The operation time was 90-120 min, with an average of 102.3 min. The intraoperative blood loss was 10-30 ml. The symptoms of dysuria were relieved in all patients after removal of the catheter at 3 weeks postoperative. 4 weeks after surgery, no signs of urethral stricture were observed in urethrography. And the maximum flow rate was >15 ml/s in 13 cases, while 3 cases was 10-15 ml/s. The mean maximum flow rate in postoperative was [(20.4±7.3) ml/s], which was significantly higher than that in preoperative (t=7.7602, P<0.05). B-ultrasound showed 13 cases without residual urine and 3 cases of residual urine volume <30 ml. All patients had no serious complications such as urinary fistula, urethral diverticulum and extravasation of urine. After 1 year of follow-up, 1 patient was lost to follow up, and none of the remaining15 cases had urethral obstruction due to re-stricture. Conclusion Dorsal mosaic surgery with penis free flap could be an effective method and had no obvious complications for the treatment of anterior urethral stricture after TURP.

Objective To explore the feasibility and safety of flexible ureteroscope with tubeless in the treatment of middle or upper calyx renal calculi.Methods The clinical data of 107 patients with renal calculi treated from January 2015 to October 2018 were analyzed retrospectively.Age ranged from 18 to 55 years,with mean of (32.1 ± 5.2) years.Calculi was single,locating in the middle or upper calyx,with the diameter less than 2.0 cm,the CT value ≤ 800 HU,and mild renal hydronephrosis.All patients were routinely indwelling double-J tube using cystoscopy 2 weeks preoperatively,and ureteroscopic lithotripsy was performed.Fifty patients in group A were received tubeless treatment,and 57 patients in group B were given routinely indwelling double-J tube.The 50 patients in group A were (30.4 ± 5.9) years of age,including 33 males and 17 females,28 cases on the left and 22 cases on the right,24 cases locating in the upper calyx and 26 cases locating in the middle calyx,and calculi diameter of (1.3 ± 0.5) cm.The 57 patients in group B were (31.3 ± 5.4) years of age,including 35 males and 22 females,26 cases on the left and 31 cases on the right,27 cases locating in the upper calyx and 30 cases locating in the middle calyx,and diameter of (1.4 ± 0.4) cm.There were no significant difference in the demographics between the two groups (P ＞ 0.05).Results There were no obvious ureteral malformations,stenosis,polyps or tumors in the 107 cases intraoperatively,and the flexible ureteroscope sheath was placed smoothly.The operation time in group A [(48.2 ± 9.7) min] was significantly lower than that in group B [(51.7 ± 7.8) min,P ＜ 0.05].There was no significant difference in the calculi clearance rate between the two groups on the first day [92.0％ (46/50) vs.91.2％ (52/57)] and two weeks[96.0％ (48/50) vs.98.2％ (56/57)] after operation(P ＞ 0.05),and the calculi clearance rate reached 100％ at 1 month after operation.The incidence of hematuria in group A [24.0％ (12/50)] was significantly lower than that in group B [54.4％ (31/57),P =0.001].The incidence of bladder irritative symptoms in group A [14.0％ (7/50)] was significantly lower than that in group B [36.8％ (21/57),P =0.007].The incidence of lumbar and abdominal pain at 1 week,2 weeks and 1 month after operation was significantly lower in group A [32.0％ (16/50),8.0％ (4/50),2.0％ (1/50)] than that in group B [57.9％ (33/57),49.1％ (28/57),33.3％ (19/57),P ＜ 0.05].There was no significant difference between the two groups about the incidence of lumbar and abdominal pain at first day after operation [86.0％ (43/50) vs.84.2％ (48/57),P ＞ 0.05].Conclusions It was feasibility and safety to perform flexible ureteroscope with tubeless for the patients with renal primary and single calculi,ideal ureteral conditions (no malformations,stenosis,polyps or tumors),mild renal hydronephrosis,calculi,diameter ＜ 2.0 cm,CT value ≤ 800 HU,locating in the middle or upper calyx,and no history of urinary calculi.This procedure had not only similar calculi clearance rate compared with routinely indwelling double-J tube,but also has a lower incidence of complications (hematuria,bladder irritative symptoms,lumbar or abdominal pain).

Aggressive angiomyxoma (AAM) is a rare clinical entity. A case of AAM was reported in this paper. The patient presented with severe hydronephrosis of the left kidney and was diagnosed with a pelvic mass compressing the ureter. The patient underwent laparoscopic resection of the pelvic mass. The postoperative pathology and immunohistochemistry confirmed the diagnosis of AAM. The patient had no recurrence and metastasis after 9 months of follow-up.