The role of liver transplantation in COACH syndrome (Joubert syndrome with congenital hepatic fibrosis): A review of the literature.

BACKGROUND: COACH syndrome is a rare autosomal recessive genetic disease characterized by liver fibrosis, which leads to severe complications related to portal hypertension. However, only a few patients with COACH syndrome undergoing liver transplantation (LT) have been reported. MATERIALS AND METHODS: We herein report the outcomes of four children who underwent LT for COACH syndrome at our institute and review three previously reported cases to elucidate the role of LT in COACH syndrome. RESULTS: All four patients in our institute were female, and three received living donors LT. All patients were diagnosed with COACH syndrome by genetic testing. LT was performed in these patients at 3, 7, 9, and 14 years old. The indication for LT was varices related to portal hypertension in all patients. One showed an intrapulmonary shunt. Blood tests revealed renal impairment due to nephronophthisis in three patients, and one developed renal insufficiency after LT. The liver function was maintained in all patients. A literature review revealed detailed information for three more patients. The indication for LT in these three cases was portal hypertension, such as bleeding from esophageal varices. One patient had chronic renal failure on hemodialysis at LT and underwent combined liver and kidney transplantation. Of these three previous patients, one died from hepatic failure due to de novo HCV infection 3 years after LT. CONCLUSIONS: LT should be considered an effective treatment for COACH syndrome in patients with severe portal hypertension. However, a detailed follow-up of the renal function is necessary.

Visual function in children with Joubert syndrome.

AIM: To describe visual function in children with Joubert syndrome and to investigate its possible association with diagnostic and developmental aspects. METHOD: This retrospective cross-sectional work included 59 patients (33 male; mean age 9 years 2 months, standard deviation 6 years 3 months, range 4 months to 23 years) diagnosed with Joubert syndrome from January 2002 to December 2020. Data about clinical (neurological, neuro-ophthalmological, developmental/cognitive) and diagnostic (e.g. genetic testing, neuroimaging, systemic involvement) evaluations were collected in a data set during a review of medical records. Clinical and diagnostic variables were described in terms of raw counts and percentages. A χ2 test was conducted to investigate their association with neuropsychological skills. RESULTS: Ocular motor apraxia was highly represented in our cohort (75%), with a high prevalence of refractive defects and retinal abnormalities. Developmental delay/intellectual disability was frequent (in 69.5% of the sample), associated with retinal dystrophy (p = 0.047) and reduced visual acuity both for near (p = 0.014) and for far distances (p = 0.017). INTERPRETATION: On the basis of the relevance of oculomotor and perceptual alterations and their impact on overall and cognitive impairment, we encourage early and multidisciplinary assessment and follow-up of visual function in children with Joubert syndrome. This would help in planning a personalized rehabilitation to sustain functional vision. Further studies will be important to explore the link between biological aspects and global functioning in children with Joubert syndrome. WHAT THIS PAPER ADDS: Perceptual deficits and oculomotor impairments frequently coexist in Joubert syndrome. Retinal dysfunction may be present despite the absence of funduscopic abnormalities. Both perceptual and oculomotor impairments negatively affect cognitive development in Joubert syndrome.

A clinical review of congenital hepatic fibrosis diagnosed in adulthood: presentation, complications, and outcomes.

INTRODUCTION AND OBJECTIVES: Congenital hepatic fibrosis (CHF) is a rare condition characterized by biliary tract changes and a geographic pattern of liver fibrosis. Liver biopsy is essential to confirm its diagnosis. The absence of specific clinical indicators in adults often leads to delays in diagnosis and management, while the natural history has not been well described. We sought to define the presentation and outcomes of adults with biopsy-proven CHF. MATERIALS AND METHODS: A retrospective chart review was conducted of patients diagnosed with CHF by liver biopsy. Continuous variables were summarized with the sample median and range. Categorical variables were summarized with number and percentage of patients. RESULTS: We identified 24 patients evaluated over a 20-year period, with a median age of 51 years (range 22-72 years) at initial presentation; 14 were male. The most common imaging findings were renal cysts (91.3%), splenomegaly (69.6%), and a cirrhotic-appearing liver (60.9%). The most commonly treated liver-related complications were cholangitis (45.8%), varices (45.8%), and hepatic encephalopathy (25%). Two patients died with a median length of follow-up of 2.9 years (range: 0.0-20.0 years). Two patients underwent transjugular intrahepatic portosystemic shunt (TIPS) placement to manage bleeding esophageal varices. Eight patients underwent liver transplantation (LT), the most common indication being decompensated disease (50%). CONCLUSIONS: CHF should be considered when patients present with cholangitis and/or complications of portal hypertension and have a cirrhotic appearing liver and renal cysts on imaging. Depending upon the disease severity, interventions such as TIPS or LT may be required.

Characteristics and Outcomes of Patients With Pregnancy-Related End-Stage Kidney Disease.

Importance: The incidence of pregnancy-related acute kidney injury is increasing and is associated with significant maternal morbidity including progression to end-stage kidney disease (ESKD). Little is known about characteristics and long-term outcomes of patients who develop pregnancy-related ESKD. Objectives: To examine the characteristics and clinical outcomes of patients with pregnancy-related ESKD and to investigate associations between pre-ESKD nephrology care and outcomes. Design, Setting, and Participants: This was a cohort study of 183 640 reproductive-aged women with incident ESKD between January 1, 2000, and November 20, 2020, from the US Renal Data System and maternal data from births captured in the US Centers for Disease Control and Prevention publicly available natality data. Data were analyzed from December 2022 to June 2023. Exposure: Pregnancy-related primary cause of ESKD, per International Classification of Diseases, Ninth Revision (ICD-9) and ICD-10 codes reported at ESKD onset by the primary nephrologist on Centers for Medicare and Medicaid Services form 2728. Main Outcomes Measures: Multivariable Cox proportional hazards and competing risk models were constructed to examine time to (1) mortality, (2) access to kidney transplant (joining the waiting list or receiving a live donor transplant), and (3) receipt of transplant after joining the waitlist. Results: A total of 341 patients with a pregnancy-related primary cause of ESKD were identified (mean [SD] age 30.2 [7.3]). Compared with the general US birthing population, Black patients were overrepresented among those with pregnancy-related ESKD (109 patients [31.9%] vs 585 268 patients [16.2%]). In adjusted analyses, patients with pregnancy-related ESKD had similar or lower hazards of mortality compared with those with glomerulonephritis or cystic kidney disease (adjusted hazard ratio [aHR], 0.96; 95% CI, 0.76-1.19), diabetes or hypertension (aHR, 0.49; 95% CI, 0.39-0.61), or other or unknown primary causes of ESKD (aHR, 0.60; 95% CI, 0.48-0.75). Despite this, patients with pregnancy-related ESKD had significantly lower access to kidney transplant compared with those with other causes of ESKD, including (1) glomerulonephritis or cystic kidney disease (adjusted subhazard ratio [aSHR], 0.51; 95% CI, 0.43-0.66), (2) diabetes or hypertension (aSHR, 0.81; 95% CI, 0.67-0.98), and (3) other or unkown cause (aSHR, 0.82; 95% CI, 0.67-0.99). Those with pregnancy-related ESKD were less likely to have nephrology care or have a graft or arteriovenous fistula placed before ESKD onset (nephrology care: adjusted relative risk [aRR], 0.47; 95% CI, 0.40-0.56; graft or arteriovenous fistula placed: aRR, 0.31; 95% CI, 0.17-0.57). Conclusion and Relevance: In this study, those with pregnancy-related ESKD had reduced access to transplant and nephrology care, which could exacerbate existing disparities in a disproportionately Black population. Increased access to care could improve quality of life and health outcomes among these young adults with high potential for long-term survival.

Relationship Between Pancreatic Cysts and Cysts in Other Organs in Patients Undergoing Medical Checkup.

OBJECTIVE: We investigated the association between pancreatic cysts and cystic diseases of other organs using abdominal ultrasonography in patients undergoing medical checkup. METHODS: Between April 2021 and March 2022, 4496 patients had a comprehensive medical checkup at our hospital, which included abdominal ultrasonography. RESULTS: Among 4496 patients, 172 (3.8%), 1592 (35.4%), and 1425 (31.7%) had pancreatic, liver, and renal cysts, respectively. Multivariate analysis revealed that the significant factors were female sex and the presence of renal cysts. CONCLUSION: Pancreatic cysts were more common in females. Renal cysts are relatively commonly detected on abdominal ultrasonography. If renal cysts are detected, comorbidities with pancreatic cysts should be considered.

A novel mutation of the RPGRIP1L gene in a Chinese boy with Joubert syndrome with oculorenal involvement.

BACKGROUND: Joubert syndrome (JS) is a rare genetically heterogeneous primary ciliopathy characterized by a pathognomonic cerebellar and brainstem malformation, the "molar tooth sign", and variable organ involvement (such as eye, kidney, liver, and skeleton). Here, we present a case of JS in a Chinese boy. CASE PRESENTATION: An 11-year-old Chinese boy presented with neonatal asphyxiation and hypoxia, strabismus, subsequent developmental delay, ataxia and end-stage kidney disease (ESKD). Routine blood tests showed severe anemia, increasing blood urea nitrogen and creatinine, elevated parathyroid hormone, hypocalcemia, hypokalemia and metabolic acidosis. Urine tests showed mild proteinuria. Ultrasound showed two small kidneys. Brain magnetic resonance imaging (MRI) showed dysplasia of the cerebellar vermis and extension of the upper cerebellar feet with the "molar tooth sign". Genetic analysis showed novel compound heterozygous mutations in the RPGRIP1L gene [p.L447fs*7(p.Leu447fsTer7) and p.G908V (p.Gly908Val)]. CONCLUSION: In the present study, we identified novel compound heterozygous mutations in the RPGRIP1L gene in a Chinese boy. The clinical and genetic findings of this study will expand the understanding of JS.

[Effect of a simple kidney cyst on renal function].

INTRODUCTION: Renal cysts are a common disease that occurs at a rate of 7-10%. Currently there are no clinical recommendations for the treatment of patients with simple renal cysts. In the current literature there is some evidence that a simple renal cyst has negative effects on renal function. Decreased renal function occurs due to partial atrophy and loss of the renal parenchyma (in the "crater" area at the base of the cyst) caused by compression. Therefore, the efforts to analyze the effect of simple kidney cysts on kidney function and identify the characteristics of the cyst that affect renal function to determine the indications for surgical treatment remains a substantial task. The aim of the study was to analyze the effect of simple renal cysts on renal function, to investigate the relationship between cyst size, atrophied parenchyma volume, and renal function, and to determine indications for surgical treatment of simple renal cysts. MATERIALS AND METHODS: We conducted a prospective cohort study. The study included 109 patients with simple renal cysts. Patients with a solitary cyst of the right or left renal kidney, grade I-II according to Bosniak classification, were included in the study. The estimated glomerular filtration rate (eGFR) of the patients was calculated using various formulas. A contrast CT scan of the urinary tract was also performed to determine the maximum size of the cyst, calculate the volume of the renal parenchyma, and the volume of the lost (atrophied) parenchyma. Patients underwent renal scintigraphy with calculation of total GFR and split renal function. We analyzed the symmetry of the function of both kidneys by comparing the GFR of the affected and healthy kidneys, analyzed the relationship between the presence of a kidney cyst and a decrease in GFR, between the maximum size of a renal cyst and a decrease in its function compared with that of a healthy kidney. We also analyzed the correspondence of total GFR values obtained in renal scintigraphy and GFR values calculated according to the formulas. RESULTS: Data from 109 patients were available for analysis; the mean blood creatinine was 87.4 mol/L. The median maximum cyst size was 80 mm. The median baseline volume of the affected kidney parenchyma was 174 ml, the median volume of the lost parenchyma was 49 ml, and the median proportion of the lost parenchyma was 28%. The median total GFR was 77.07 ml/min. The median GFR of the healthy kidney was 45.49 mL/min, and the median GFR of the kidney affected by the cyst was 34.46 mL/min. The median difference in GFR of the healthy and affected kidney units was 11 mL/min and was statistically significant. Comparison of the eGFR values obtained by the formulas with the reference values of GFR obtained by scintigraphy showed that the Cockcroft-Gault formula with standardization on the body surface area calculated closest eGFR values to the reference ones. Correlation analysis revealed a statistically significant association between the proportion of lost parenchyma volume and the maximum cyst size: =0.37 with 95% CI [0.20; 0.52] (p-value = 0). A multivariate logistic regression model revealed that a statistically significant factor influencing the probability of a significant decrease in GFR was the percent of lost renal parenchyma volume (OR=1,13; =0). CONCLUSIONS: Our study showed that growth of renal cysts associated with renal parenchyma atrophy and decrease of GFR of the affected kidney. An increase in the volume of atrophied parenchyma leads to the decrease in GFR of the affected kidney. The obtained data suggest that performing dynamic renal scintigraphy to assess the decrease in affected renal function and determine the indications for surgical treatment of renal cysts is a reasonable recommendation. According to the results of the study, the loss of 20% of the renal parenchyma can be considered an indication for renal scintigraphy. The Cockcroft-Gault formula with standardization on the body surface area allows to calculate closest GFR values to those obtained by scintigraphy and, therefore, can be recommended as the optimal formula for calculating eGFR in daily clinical practice.

Non-suppression of renin by renal cysts in a subset of patients with primary aldosteronism-a prospective observational single center study.

BACKGROUND: Screening for primary aldosteronism is based on measuring aldosterone-to-renin ratio. Non-suppressed renin may cause false negative screening results, and such patients may miss focused, potentially curable treatment. We investigated the association between renal cysts and non-suppressed plasma renin. METHODS: Altogether, 114 consecutive patients with confirmed primary aldosteronism undergoing adrenal vein sampling were prospectively recruited between October 7, 2020 and December 30, 2021. During the procedure, plasma samples for renin analyses were collected from the right and left renal veins and the inferior vena cava. Renal cysts were identified using contrast-enhanced computed tomography. RESULTS: Renal cysts were found in 58.2% of the 114 patients. Neither screening nor renal vein renin concentrations were significantly different in patients with and without cysts, or when the kidneys with and without cysts were evaluated. However, cysts were significantly more prevalent in the "high-normal renin" group (cut point 23.0 mU/L) than in the "low to low-normal renin" group (90.9%, n = 11 vs. 56.0%, n = 102, P = .027, respectively). All patients ≤50 years of age in the "high-normal renin" group had renal cysts. Strong correlations were found between renin concentrations in the right and left renal veins (r = .984), and between renin concentration and renin activity in the inferior vena cava (r = .817). CONCLUSION: Renal cysts are found in the majority of patients with primary aldosteronism, and they may interfere with diagnostics, especially in patients aged 50 years or less. In patients with non-suppressed renin due to renal cysts, aldosterone-to-renin ratio below the diagnostic threshold does not always exclude the diagnosis of primary aldosteronism.

Unusual association of Wilms' tumor with cystic diseases of kidney: A pathologic surprise.

Wilms' tumor (or nephroblastoma) is the most common renal malignancy in the pediatric population which consists of blastemal, epithelial, and stromal elements in variable proportions. The occurrence of renal cysts in children and infants is a rare phenomenon and is possibly an outcome of developmental aberrations in mesonephric blastema. The coincidental association of nephroblastoma with renal cysts is a very rare finding. Here, we describe two cases of Wilms' tumor with an unusual association between glomerulocystic kidney disease and multicystic dysplastic kidney.

Bilateral Intraorbital Opticmeningoceles in Joubert Syndrome.

Congenital opticmeningoceles was the term coined to describe large pseudocystic lesions of the intraorbital segment of the optic nerve. This extremely rare congenital anomaly was reported unilaterally only in nonsyndromic patients with fully developed eyes. The authors describe here a 10-month-old girl with a previous diagnosis of Joubert syndrome who presented with the same type of optic nerve malformation in OU. Molecular genetic analysis disclosed a pathogenic variant of the TMEM67 gene which is associated with various types of ciliopathies.

The Landscape of HNF1B Deficiency: A Syndrome Not Yet Fully Explored.

The hepatocyte nuclear factor 1ß (HNF1B) gene is involved in the development of specialized epithelia of several organs during the early and late phases of embryogenesis, performing its function mainly by regulating the cell cycle and apoptosis pathways. The first pathogenic variant of HNF1B (namely, R177X) was reported in 1997 and is associated with the maturity-onset diabetes of the young. Since then, more than 230 different HNF1B variants have been reported, revealing a multifaceted syndrome with complex and heterogenous genetic, pathologic, and clinical profiles, mainly affecting the pediatric population. The pancreas and kidneys are the most frequently affected organs, resulting in diabetes, renal cysts, and a decrease in renal function, leading, in 2001, to the definition of HNF1B deficiency syndrome, including renal cysts and diabetes. However, several other organs and systems have since emerged as being affected by HNF1B defect, while diabetes and renal cysts are not always present. Especially, liver involvement has generally been overlooked but recently emerged as particularly relevant (mostly showing chronically elevated liver enzymes) and with a putative relation with tumor development, thus requiring a more granular analysis. Nowadays, HNF1B-associated disease has been recognized as a clinical entity with a broader and more variable multisystem phenotype, but the reasons for the phenotypic heterogeneity are still poorly understood. In this review, we aimed to describe the multifaceted nature of HNF1B deficiency in the pediatric and adult populations: we analyzed the genetic, phenotypic, and clinical features of this complex and misdiagnosed syndrome, covering the most frequent, unusual, and recently identified traits.

Prevalence and associated risk factors of simple renal cysts in primary hyperparathyroidism.

PURPOSE: Previous studies have pointed to the role of parathormone (PTH) in the development of simple renal cysts. However, there is insufficient evidence related to simple renal cysts in primary hyperparathyroidism (PHPT). Therefore, this study aimed to evaluate the prevalence and risk factors associated with renal cysts in PHPT patients. METHODS: In this retrospective study, we included 235 patients treated surgically for PHPT and 147 control subjects. The clinical and physical examination findings, laboratory test results, and neck and urinary system ultrasonography examination findings were evaluated. Postoperative parathyroid adenoma volume and weight were also recorded. RESULTS: Simple renal cysts were found in 53 PHPT patients (22.6%) and in 15 control subjects (10.2%) (p = 0.002). Kidney stones were found in 33 PHPT patients (14%) and in only seven control subjects (4.8%) (p = 0.004). The presence of PHPT and high PTH levels independently supported the development of a simple renal cyst. Advanced age was determined as a risk factor for the development of simple renal cysts, and the presence of kidney stones was an independent predictor of simple renal cysts in PHPT patients. There was no relationship between the presence of simple renal cysts and the volume and weight of parathyroid adenoma. CONCLUSION: A diagnosis of PHPT, high PTH levels, advanced age, and kidney stones are independent risk factors for simple renal cysts in PHPT patients. These patients should be evaluated regularly for complications of renal cysts and nephrolithiasis.

Genotype and phenotype analysis and transplantation strategy in children with kidney failure caused by NPHP.

BACKGROUND: Nephronophthisis-related ciliopathies (NPHP-RC) have strong genotype and phenotype heterogeneity, and the transplantation strategy of Boichis syndrome is still controversial. Our purpose was to examine associations of genotype and phenotype in children with NPHP-RC and analyze the transplantation strategies of different phenotypes. METHODS: The records of children with NPHP treated at our center from 01/2018 to 03/2021 were retrospectively reviewed. Inclusion criteria were a diagnosis of NPHP, received kidney transplantation, and received whole exome sequencing (WES) or nephropathy gene panel testing. RESULTS: Twenty-nine children with NPHP were included. Nine children (31%) had NPHP1 mutations, and all presented with isolated nephropathy. Eighteen of 20 patients with non-NPHP1 mutations had compound heterozygous mutations, and 70% had extrarenal phenotype. Age at disease presentation (11.2 ± 1.94 years) and the development of kidney failure (12.4 ± 2.70 years) were later in children with NPHP1 mutations than those with non-NPHP1 mutations (5.2 ± 2.83 years and 5.7 ± 2.92 years, respectively). Four of six children with NPHP3 mutations were diagnosed with Boichis syndrome due to liver fibrosis. Isolated kidney transplantation resulted in good outcomes for patients with mild or moderate liver fibrosis without portal hypertension, while cholestasis was common postoperatively and could be resolved with ursodeoxycholic acid. CONCLUSIONS: NPHP1 mutations are the most common in children with NPHP, and the phenotype of NPHP1 mutation is significantly different from that of non-NPHP1 mutation. For NPHP patients with mild to moderate liver fibrosis without portal hypertension, timely treatment of cholestasis could prevent the rapid progression of liver function damage after isolated kidney transplantation. A higher resolution version of the Graphical abstract is available as Supplementary information.

Renal Cyst after Heminephrectomy in a Pediatric Patient: Complication or Normality?

INTRODUCTION: In this work, we present two cases of heminephrectomies with postoperative findings of cystic lesions in the bed of the excised renal segment. MATERIAL AND METHODS: Description of the clinical cases, therapeutic management and description of the ultrasound findings. A review of the published cases was carried out. RESULTS: We present a case of open heminephrectomy due to atrophy of the upper hemirrenal in a case of ureterocele, and another case with bilateral lower heminephrectomy in a case of vesicoureteral reflux disease. In the first case, the cystic lesion disappeared during follow-up, while in the second, the cysts remained stable. In none of the cases published in the literature was it necessary to perform any intervention to resolve the cysts. CONCLUSIONS: The appearance of cystic lesions in the heminephrectomy bed in pediatric patients is a radiological finding that occurs frequently. Due to its favorable evolution, without the need for additional procedures for its resolution, we do not consider the presence of these lesions as a complication after heminephrectomy.

Association of simple renal cysts with metabolic syndrome in adults.

Background and aims: Metabolic syndrome is common nowadays and may increase risk of hypertension, type 2 diabetes mellitus, cardiovascular complications and even mortality. Renal cysts are also frequently found during routine examination. However, the relationship between simple renal cysts (SRCs) and metabolic syndrome remains unclear. This study aimed to investigate the association of SRCs with metabolic syndrome. Methods: A total of 16,216 subjects aged ≥18 years were enrolled in this study. SRCs were diagnosed with ultrasonography by finding: sharp, thin posterior walls, a round/oval shape, absence of internal echoes, and posterior enhancement. SRCs were categorized by number (0, 1, and ≥2) and size (<2 and ≥2 cm). Metabolic syndrome was diagnosed according to the consensus statement from the International Diabetes Federation. Results: In multivariate analysis, SRCs were positively related to metabolic syndrome (OR: 1.18, 95% CI: 1.06-1.34). The risk of metabolic syndrome was higher for SRCs with a number ≥2 (OR: 1.35, 95% CI: 1.08-1.68) and size ≥2 cm (OR: 1.33, 95% CI: 1.10-1.61). When considering the SRC number and size concomitantly, SRCs with a number ≥2/size ≥2 cm (OR: 1.42, 95% CI: 1.02-1.98) or <2/size ≥2 cm (OR: 1.30, 95% CI: 1.04-1.62) were positively related to metabolic syndrome. Conclusions: Simple renal cysts were found to be related to a higher risk of metabolic syndrome, and the association is more significant in those with larger (sizes ≥2cm) or plural (numbers ≥2) SRCs.

Ocular Manifestations Leading to a Diagnosis of Joubert Syndrome Related Disorder.

INTRODUCTION: Joubert syndrome is an inherited disorder of rare occurrence usually presenting as developmental delay, hypotonia, hyperpnea and ataxia. The diagnosis is confirmed by characteristic findings in neuroimaging. Involvement of ocular, renal and hepatic systems can be present. Joubert syndrome presenting first to an ophthalmologist is very uncommon. CASE: A twenty-one-year female, with history of delayed milestones, infantile hemiplegia with hearing and visual impairment was referred for visual assessment. On systemic examination, ataxic gait was present. CT head showed hypoplasia of postero-inferior portion of vermis with communication between 4th ventricle and cisterna magna with variable degree of cerebellar dysgenesis. The neurological, ophthalmological and radiological findings of this patient were consistent with Joubert syndrome related disorder. CONCLUSION: We hereby report a case of Joubert syndrome related disorder with ocular involvement which after correlation with neurological findings and neuroimaging led us to the diagnosis of this rare disorder. The renal and hepatic functions in these patients need to be monitored.

[Relationship between simple renal cyst and adverse events in patients receiving thoracic endovascular aortic repair for Stanford B aortic dissection].

Objective: To explore the prognostic value of simple renal cyst (SRC) for adverse events in patients receiving thoracic endovascular aortic repair (TEVAR) for Stanford B aortic dissection (TBAD). Methods: This study is a retrospective cohort study. Consecutive patients receiving TEVAR for TBAD between January 2010 and December 2015 were enrolled in this study. The patients were divided into SRC group and non-SRC group. With sex and age ±2 years old as matching factors, SRC group and non-SRC group were matched by 1∶1. Collect and compare the differences of clinical data between the two groups. Adverse events were recorded through outpatient, telephone follow-up and in-hospital review. After adjusting for confounding factors, multivariate Cox regression was used to analyze the risk factors of aortic adverse events. Kaplan-Meier method was used to analyze the survival curve of SRC group and non-SRC group. Results: A total of 692 consecutive patients were recruited. Patients were divided into SRC group (n=235) and non-SRC group (n=457). After 1∶1 matching, there were 229 cases in SRC group and no SRC group respectively. The age of SRC group was (62.3±10.4) years old, 209 cases were male (91.3%), and the age of no SRC group was (62.0±10.2) years old, 209 cases were male (91.3%). Cox regression analysis showed that, after adjusting for confounding factors, comorbid SRC (HR=1.991, 95%CI: 1.090-3.673, P=0.025), TEVAR in the acute phase (HR=13.635, 95%CI: 5.969-31.147, P=0.001), general anesthesia (HR=2.012, 95%CI: 1.066-3.799, P=0.031) are independent factors of aortic-adverse events after TEVAR for TBAD. Kaplan-Meier analysis showed that the cumulative survival rate of SRC group was significantly lower than non-SRC group (log-rank P=0.031, 0.005). Conclusion: SRC is an independent predictor of aortic-related adverse events in patients following TEVAR for TBAD.