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Tuberous sclerosis complex(TSC) is a rare autosomal dominant genetic disorder caused by mutations in the TSC1 or TSC2 genes. The disease can cause multiple organ lesions. The most common renal lesions are renal angiomyolipoma(RAML). In recent years, China has made progresss in the understanding of TSC-RAML, so that the standardization of the diagnosis and management of TSC-RAML have improved. Efforts have been taken in the studies of the pathogenesis and treatment of TSC-RAML. This article reviews the diagnosis, therapy, and research development of TSC-RAML in China.

ObjectiveTuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disease involving multiple organ system. Renal angiomyolipoma (RAML) is a leading cause of death for adult TSC patients. Our study aims at investigating the clinical manifestations of TSC-RAML to enable clinicians to have a better understanding of the disease in Chinese patients.MethodsWe reviewed retrospectively the data collected from patients with TSC-RAML in Peking Union Medical College Hospital from January 2014 to January 2023. We also collected information about the age, gender, imageological examination, RAML stage, and comorbidities.ResultsA total of 186 TSC-RAML patients were registered, 65 of whom were males and 121 were females(male-to-female ratio 1∶1.86). The median age of all patients was 31 years old. Totally, 117 cases (62.9%)of RAML were rated stage 6. Twenty-two cases (11.8%) had a history of tumor rupture and bleeding. Research shows statistical differences between high and low grade on RAML rupture bleeding(P=0.0475). Angiofibromas/fibrous cephalic plaque (155/186, 83.3%), subependymal nodules(103/146, 70.5%), lymphangioleiomyomatosis (102/157, 65.0%), hypomelanotic macules (114/186, 61.3%), and shagreen patch (83/186, 44.6%)were the most common clinical manifestations. All patients with lymphangioleiomyomatosis(LAM) were female(P < 0.0001).ConclusionsFemale patients predominated TSC-RAML patients. Most RAML were in stage 6. About 11.8% cases had a history of tumor rupture and hemorrhage. The higher grade meant the higher proportion of tumor rupture and hemorrhage. Dermatological and nervous lesions were the most common comorbidities. All patients with LAM in this study were female.

Tuberous sclerosis complex (TSC) is an autosomal dominant hereditary disease that affects multiple organs and systems throughout the body. TSC-associated kidney disease is the leading cause of death in adult TSC patients. This article retrospectively analyzed the characteristics of one TSC-related renal giant angiomyolipoma(RAML)treated with surgery. The patient, 25 years old, was diagnosed with tuberous sclerosis complex in 2000 due to multiple maculopapular rashes on both cheeks. At a regular follow-up in July 2019, imaging examinations revealed a tumor in the left lower quadrant with a maximum cross-sectional area of 16 cm×7 cm. Genetic testing showed a loss of heterozygosity in the EX18_ 41 of TSC2. After the diagnosis was confirmed, open left partial nephrectomy was performed, during which multiple tumors were found on the kidney surface and the largest one was located on the ventral side with a diameter of approximately 20 cm. After the renal artery was occluded, kidney tumors were completely enucleate. Postoperative pathological confirmed the diagnosis of angiomyolipoma. This case provides a reference for the treatment of TSC-related renal giant hamartoma.

Objective To exploratively analyze the efficacy and safety of combined laparoscopy and cystoscopy in the treatment of bladder diverticula. Methods Clinical data of patients who received bladder diverticulectomy in Peking Union Medical College Hospital from March 2007 to September 2022 were retrospectively collected.The patients were divided into open/laparoscopy alone group and combined laparoscopy and cystoscopy group based on surgical approach.Surgery-related outcomes and complication rates were compared. Results A total of 11 patients with bladder diverticula who met the inclusion and exclusion criteria were enrolled in thestudy, including 6 patients in the open/laparoscopy alone group and 5 patients in the combined laparoscopy and cystoscopy group.The median maximum diameter of diverticula in the open/laparoscopy alone group and combined laparoscopy and cystoscopy group were 7.5(4.5, 11.5) cm and 5.2(5.0, 7.9) cm, respectively, and the median diameter of diverticula neck were 1.1(1.0, 1.6) cm and 1.5(1.1, 1.8) cm, respectively.Operations were successfully performed in both two groups and the patients had normal urination after drawing the catheter.No recurrence of bladder diverticulum at 6 to 60 months of follow-up.The median operative time[150.0(142.5, 180.0) min vs. 160.0(108.8, 300.0) min], blood loss[50.0(35.0, 50.0) mL vs. 50.0(45.0, 62.5) mL]and indwelling time of drainage tube[5.0(3.5, 5.5) d vs. 4.5(4.0, 6.8) d]were similar between the two group.Compared to the open/laparoscopy alone group, the combined laparoscopy and cystoscopy group had a shorter postoperative hospital stay[6.0(6.0, 8.5) d vs. 9.5(7.0, 16.0) d]and longer indwelling time of urinary catheter[14.0(10.5, 14.0) d vs. 11.5(6.5, 13.3) d].No complications occurred in the combined laparoscopy and cystoscopy group.In the open/laparoscopy alone group, 2 patients underwent postoperative complications, including 1 patient with deep vein thrombosis of the lower extremities and 1 patient with hyperpyrexia. Conclusion Preliminary results show that cystoscopy combined with laparoscopic resection for bladder diverticula can achieve similar outcomes as open surgery alone or laparoscopic surgery, and with greater safety and faster postoperative patient recovery.

Abstract Embedded ZeroBlock Coding and context modeling (EZBC) algorithm has high compression performance. However, it consumes large amounts of memory space because an Amplitude Quadtree of wavelet coefficients and other two link lists would be built during the encoding process. This is one of the big challenges for EZBC to be used in real time or hardware applications. An improved EZBC algorithm based on bit length of coefficients was brought forward in this article. It uses Bit Length Quadtree to complete the coding process and output the context for Arithmetic Coder. It can achieve the same compression performance as EZBC and save more than 75% memory space required in the encoding process. As Bit Length Quadtree can quickly locate the wavelet coefficients and judge their significance, the improved algorithm can dramatically accelerate the encoding speed. These improvements are also beneficial for hardware. PACS: 42.30.Va, 42.30.Wb

Sludge flocs encapsulate a significant amount of bound water, resulting in poor dewatering efficiency. To address this issue, the addition of alum (KAl(SO4)2·12H2O) has emerged as an effective strategy for enhancing sludge dewatering performance. This research specifically investigates the effects of various alum dosages on the dewatering characteristics of sludge. The introduction of alum alters internal temperature, pH levels, and intracellular osmotic pressure, which facilitates the release of trapped water within the flocs. Additionally, alum accelerates sludge sedimentation and promotes the agglomeration of smaller particles into larger ones, further improving dewatering efficiency. However, determining the optimal alum dosage for sludge conditioning is essential, as excessive amounts can lead to a decrease in dewatering performance. [ABSTRACT FROM AUTHOR]

Background: Everolimus is an effective treatment for renal angiomyolipoma associated with TSC (TSC-RAML). However, its impact on hematologic parameters in TSC-RAML patients remains unclear. Methods: Hematologic data were collected from TSC-RAML patients undergoing everolimus treatment in two registered clinical trials. Dynamic changes in hematologic parameters during treatment were analyzed. Additionally, we also explored variations in hematologic impact based on gender and age within the patient population. Result: A total of 55 patients from the two clinical trials are included in this analysis. Hemoglobin, white blood cells (WBC), lymphocytes, neutrophils, and platelet showed significant decreases during everolimus treatment (P < 0.05). However, the decline in hemoglobin, WBC, and neutrophils attenuated by the 12th month (P ≥ 0.05). Aspartate transaminase (AST), Alanine transferase (ALT), total cholesterol (TC), and triglyceride (TG) increased significantly during everolimus treatment (P < 0.05), and these increases persisted throughout the year-long treatment. Hemoglobin decreased significantly more in male patients (− 15 vs − 6, P = 0.010), and AST showed a more significant increase in males (7.0 vs 3.0, P = 0.041). Platelet counts decreased significantly more in younger patients (≤ 30 years old) compared to older patients (− 50 vs − 14, P = 0.020). Conclusion: Everolimus administration in TSC-RAML patients may increase hematologic risks, with male and younger patients potentially exhibiting greater susceptibility to these effects. [ABSTRACT FROM AUTHOR]

Enzymatic modification, compared with traditional starch (St) chemical modification methods, avoids using toxic solvents and toxic catalysts. The horseradish peroxidase (HRP), being the most commonly employed natural green initiator, has found extensive application in starch grafting modification as a substitute for chemical initiators. HRP‐catalyzed grafting of starch with vinyl zwitterionic monomers has not been reported. Here, modified starch (St‐PSBMA) is first synthesized by grafting zwitterionic sulfobetaine [2‐(Methacryloyloxy)ethyl]dimethyl‐(3‐sulfopropyl) (SBMA) onto starch by HRP/Acetylacetone(ACAC) binary initiation system catalyzed. The modified starch‐based hydrogel is then prepared by the repeated freeze‐thaw method. Compared with native starch‐based hydrogels, which bind water molecules only through hydrogen bonding, the ability of modified starch‐based hydrogels to bind water molecules is enhanced by ionic solvation. The modified starch hydrogels also have excellent antiprotein adsorption and antibacterial properties due to the presence of a strong hydration layer. The modified starch‐based hydrogel also has excellent sensitivity and stability when used as a sensor for detecting joint motion. The binary HRP initiating system, employed in this study instead of the traditional three‐component HRP initiating system, offers a novel and promising approach for grafting modification of starch without the need for strong oxidant hydrogen peroxide. [ABSTRACT FROM AUTHOR]