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Introduction: Wunderlich syndrome (WS) is a rare condition characterized by spontaneous, atraumatic renal hemorrhage. It often presents with non-specific symptoms and is typically diagnosed through computed tomography (CT). The most common presentation of WS includes the Lenk triad, which consists of flank pain, a palpable flank mass, and hypovolemic shock. If diagnosis and treatment are delayed, WS can rapidly progress and lead to unfavorable patient outcomes. Case Report: A 65-year-old male presented to the emergency department with severe sudden-onset left flank pain with subsequent CT angiogram demonstrating an actively bleeding left renal hematoma. The patient was managed conservatively with supportive care. His vitals remained stable, and he did not require any surgical or vascular interventions. Conclusion: Wunderlich syndrome is a spontaneous renal or perinephric hemorrhage occurring in the absence of trauma; it is rarely included in the differential for patients with flank pain but can become life-threatening when not recognized.

PurposeTo investigate factors affecting hemostasis in iatrogenic renal hemorrhage.MethodsSeventy-three patients with iatrogenic renal hemorrhage experiencing selective renal artery angiography between Jan 2015 and Dec 2020 were enrolled in this study. The clinical features, treatment modalities and outcomes were reviewed. Factors affecting hemostasis were analyzed by univariate and multivariate models using linear regression techniques. The optimum values of the independent factors to predict postangiographic hemostasis were conducted by receiver operating characteristic (ROC) curve analysis.ResultsOf the 73 iatrogenic renal hemorrhage patients, 47 (64.4%) patients had positive angiographic findings and received therapeutic embolization. Of the patients with negative angiographic findings, 20 (76.9%) and 6 (23.1%) received conservative therapy and prophylactic embolization, respectively. The red blood cell (RBC) count (OR = 0.61, P = 0.04), the hematuria time before angiography (OR = − 0.19, P < 0.01) and treatment modality were independent factors affecting hemostasis time. The ROC curve analysis showed that the RBC count of 3.5 × 109/L and the hematuria time before angiography of 7 days were the optimum indicators. Therapeutic embolization and prophylactic embolization were protective factors affecting hemostasis time compared with conservative treatment (OR = − 1.59, P = 0.02; OR = − 3.31, P < 0.01).ConclusionsThe hematuria time before selective renal artery angiography, the RBC count, and embolization treatment are associated with rapid hemostasis. Embolization is an effective strategy for iatrogenic renal hemorrhage, and also enables rapid hemostasis in patients with negative angiographic findings.

Purpose To present the radiological and clinical outcomes of super-selective transcatheter renal artery embolization in patients with renal injury hemorrhage, and share our experience. Methods 43 patients with renal injury hemorrhage who underwent 46 SRAEs were enrolled in this retrospective review study. Records, images, and outcomes were reviewed. The individual embolic method and its observed effects were investigated. Results Angiography showed free extravasation in 25 angiograms, pseudoaneurysm in 15 angiograms, and arteriovenous fistulas in 1 angiogram. Most patients achieved initial clinical success (38/43, 88.4%), and 41 patients achieved final clinical success (41/43, 95.3%). 9/11 patients who adopted empirical embolization achieved initial clinical success (81.8%). In our study, the combination of PVA particles and micro-coils has emerged as the most commonly utilized material combination (24/46, 52.2%). Significant differences in hemoglobin levels were observed before and after the embolization procedure ( p  = 0.026, 95%CI: 1.03–15.54). Post-embolization clinical follow-up showed no evidence of recurrent hematuria, progression of hematoma, hypertension, and no reflux of the embolic agent. Conclusion Though SRAE showed satisfactory results across a broad range of renal injury hemorrhage, there are still some aspects that need attention: (1) Surgical procedure should be understood, including the surgical site, access routes, and placement of implants, such as double-J stents. (2) In cases where identifying the bleeding point proves challenging, consider the possibility of an accessory renal artery. Trial registration Chinese Clinical Trial Registry ChiCTR2400085050, Registration Date: 30 May 2024, retrospectively, non-randomized.

Hantaan virus (HTNV) infects humans and causes hemorrhagic fever with renal syndrome (HFRS). The development of well-characterized animal models of HFRS could accelerate the testing of vaccine candidates and therapeutic agents and provide a useful tool for studying the pathogenesis of HFRS. Because NLRC3 has multiple immunoregulatory roles, we investigated the susceptibility of Nlrc3 −/− mice to HTNV infection in order to establish a new model of HFRS. Nlrc3 −/− mice developed weight loss, renal hemorrhage, and tubule dilation after HTNV infection, recapitulating many clinical symptoms of human HFRS. Moreover, infected Nlrc3 −/− mice showed higher viral loads in serum, spleen, and kidney than wild type C57BL/6 (WT) mice, and some of them manifested more hematological disorders and significant pathological changes within multiple organs than WT mice. Our results identify that HTNV infected Nlrc3 −/− mice can develop clinical symptoms and pathological changes resembling patients with HFRS, suggesting a new model for studying the pathogenesis and testing of candidate vaccines and therapeutics.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic necrotizing vasculitis that predominantly affects small vessels. In this report, we present a typical case of granulomatosis with polyangiitis (GPA) complicated by spontaneous renal hemorrhage (SRH), a rare but potentially severe condition. The patient developed SRH during immunosuppressive therapy but recovered following conservative treatment. We then conducted a systematic literature review on SRH in the context of AAV, and analyzed clinical features, management strategies, and patient prognosis. A total of 15 patients were enrolled for statistical analysis, comprising the one case reported in the current study and 14 from the literature. Of these patients, nine presented with GPA and six showed microscopic polyangiitis (MPA), with a sex distribution of 3:2 males to females. The average patient age was 54.5 years, and ranged from 25 to 82 years. Acute flank pain was the most common clinical manifestation, and was occasionally accompanied by anemia and shock. Treatment varied for the different patients. Eight patients received glucocorticoid and immunosuppressive agents that included rituximab, cyclophosphamide, and azathioprine; five patients underwent transcatheter arterial embolization (TAE); and one patient underwent nephrectomy. Our findings indicate that SRH typically occurs early in the course of AAV and correlates with disease activity, with renal aneurysm rupture as the primary cause. More than half of the patients respond well to corticosteroids and immunosuppressants. Timely TAE is essential for patients showing persistent deterioration despite conservative management.

The objective of this study is to evaluate the feasibility and safety of renal artery embolization (RAE) via transradial access (TRA) in patients with renal angiomyolipoma (AML) or renal hemorrhage. Data were collected for this prospective single-center study from 50 patients (51 ± 12 years; male:female, 11:39) who underwent RAE for renal AML (n = 46) or renal hemorrhage (n = 4) between November 2020 and January 2024. Patients with a Barbeau D waveform or a radial artery diameter of <1.5 mm were excluded. Technical success in patients with renal AML and renal hemorrhage was defined as achieving selective catheterization of the culprit artery with embolization, leading to flow stasis and the absence of bleeding evidence, respectively. Clinical success was indicated by a reduction in AML size on follow-up CT scans and the absence of bleeding signs without necessitating additional RAE. The EuroQol 5-Dimension 5-level (EQ-5D-5L) questionnaire was utilized to assess health-related quality of life (HRQoL). In one patient with AML, embolization could not be performed following selective catheterization and angiography due to the lack of visible tumor vascularity, resulting in a technical success rate of 98% (49/50). The clinical success rate was 96% (48/50 patients). No instances of TRA failure, conversion to transfemoral access (TFA), or hemostasis failure were noted. During the follow-up period, no major adverse events associated with the RAE occurred. Two patients exhibited asymptomatic radial artery occlusion, and one patient displayed asymptomatic partial thrombosis of the renal artery at the first follow-up visit. The EQ-5D-5L scores were 0.90 (95% confidence interval [CI]: 0.86-0.95) within 24 hours post-procedure and 0.89 (95% CI: 0.85-0.92) at the first follow-up ( = 0.332). TRA is a feasible and safe approach for performing RAE in patients with renal AML or hemorrhage. RAE performed using TRA demonstrated high HRQoL outcomes and may serve as a viable alternative to TFA for performing RAE.

Abstract IntroductionSpontaneous renal hemorrhage (SRH) in ANCA-associated vasculitis (AAV) is rare but fatal. We aimed to characterize clinical manifestations and managements of AAV patients with SRH.MethodHospitalized AAV patients were screened from January 2000 to April 2021, at Peking Union Medical College Hospital (PUMCH). Also, a systematic review was based on retrieving all the relevant literature from PubMed, MedlinePlus, and Web of Science until April 2021. Clinical features, management, and prognosis of the patients were collected and concluded.ResultsIn PUMCH, four out of 1640 AAV patients with SRH were included in our study; three had granulomatosis with polyangiitis (GPA) and one had microscopic polyangiitis (MPA). The ratio of men to women was 3 to 1, and the average age of onset was 55 years. The Birmingham Vasculitis Activity Score (BVAS) ranged from 21 to 23. Combining with documented reports, 13 patients were diagnosed as AAV complicated with SRH (including four from PUMCH), 7 with GPA, and 6 with MPA. Mean BVAS was 25.2 ± 6.6. The symptoms of SRH presented as severe back or abdominal pain. Patients with SRH to age- and gender-matched patients without SRH were compared, and we found that in the SRH group, the duration of disease was shorter, and BVAS, renal function, and inflammatory markers (WBC and ESR) were significantly greater, whereas Hb, Alb, and renal function greatly reduced.ConclusionThis is the first summary of clinical features and treatments of SRH in AAV. Patients with AAV in early stage and with high disease activity appeared to be more likely to develop SRH. Key Points• This is the first summary of clinical features and treatments of SRH in AAV.• SRH more likely occurs in AAV patients in the early stage (≤ 3 months) and with high disease activity.• Clinicians should be aware of the possibility of SRH when AAV patients complain of back or abdominal pain.

Spontaneous renal hemorrhage (SRH) is a diagnostic challenge and a significant cause of morbidity, and sometimes mortality. Early identification is essential to institute lifesaving and reno-protective interventions. In this review, we classify spontaneous renal hemorrhage by location, presentation and etiology. We also discuss the diagnostic approach to renal hemorrhage and optimum imaging modalities to arrive at the diagnosis. Finally, we review strategies to avoid missing a diagnosis of SRH and discuss the pitfalls of imaging in the presence of renal hemorrhage.

Background Wunderlich syndrome (WS) is a rare condition characterized by spontaneous renal hemorrhage in the absence of obvious trauma or iatrogenic injury. Given that most WS cases are life-threatening and require prompt intervention, timely identification and resolution are essential. Patients with connective tissue diseases (CTDs) account for a small proportion of reported WS cases; however, owing to the specific pathogenic mechanisms and treatments associated with CTDs, these patients exhibit distinctive pathological traits and clinical features in WS. Case presentation We present the identification and treatment process of WS in a patient with systemic lupus erythematosus. This patient suffered from sudden abdominal pain and a drastic decline in hemoglobin level accompanied by confusion of consciousness. After the abdominal computerized tomography scan revealed the presence of a renal hematoma, transcatheter arterial embolization was performed on her. Unexpectedly, three days later, the patient had severe anemia and consciousness disorders again. Highly suspecting renal rebleeding, we performed a repeated angiography for the patient. After confirming the bleeding, embolization was carried out again. The renal bleeding stopped, and the patient’s hemoglobin level gradually stabilized. Regrettably, this patient ultimately died due to multiple systemic infections. Conclusions WS that occurs in CTDs can evolve into critical and severe conditions. Infection, immune complex deposition, thrombocytopenia, abnormal coagulation function, complement activation, autoantibodies production, and glucocorticoid treatment in patients with CTDs are potentially linked to the development of WS. The treatment strategies for WS should be guided by hemodynamic status.

ANCA-associated vasculitis (AAV) is a group of autoimmune diseases characterized by necrotizing inflammation and fibrinoid necrosis of small- and medium-sized blood vessels. Renal involvement is common in AAV; however, renal hemorrhage is extremely rare, particularly in cases of eosinophilic granulomatosis with polyangiitis (EGPA). Spontaneous renal hemorrhage often presents as acute abdomen. We report a 51-year-old male with a prior diagnosis of EGPA who presented to the emergency department with acute left-sided abdominal pain and signs of hypovolemic shock. Imaging revealed a massive left perirenal hematoma without active contrast extravasation, suggestive of renal rupture. Initial management focused on hemostasis and stabilization, but recurrent contralateral renal hemorrhage occurred during hospitalization. Further immunological, histopathological, and bone marrow evaluations confirmed EGPA complicated by renal involvement. The patient was treated with corticosteroids, cyclophosphamide, and rituximab, but recurrent hemorrhage indicated rapid disease progression or insufficient therapeutic response. Due to financial constraints, the patient was discharged prematurely, precluding long-term follow-up. This case highlights the complexity of diagnosing and managing AAV-associated renal hemorrhage, particularly in EGPA patients. While the renal vascular changes observed, such as inflammation and potential necrosis, may be linked to the underlying vasculitis in EGPA, causality should be interpreted with caution, as other factors like coagulopathy, concurrent infections, or iatrogenic effects could contribute. Current literature suggests that ANCA-associated mechanisms, including NETs formation, play a role in vascular damage, but direct causation in rare complications like renal hemorrhage remains uncertain and requires further investigation. While EGPA treatment primarily involves immunosuppressive therapy targeting vasculitis and eosinophilia, spontaneous renal hemorrhage requires an integrated approach, including conservative management, interventional embolization, or surgical exploration in life-threatening cases. Reports of AAV-associated renal hemorrhage are rare, and large-scale studies are lacking, necessitating further research to optimize treatment strategies.