Spontaneous rupture of large bilateral renal angiomyolipomas in a patient with Tuberous Sclerosis Complex: A case report and literature review

We present a case of hypovolemic shock due to spontaneous rupture of bilateral giant angiomyolipomas in a 35-year-old female patient with tuberous sclerosis complex (TSC).The hemodynamic instability of the patient leads to an immediate surgery and unilateral nephrectomy was done for the biggest angiomyolipomas. The review of the literature revealed only few cases of spontaneous rupture of renal angiomyolipomas of comparable size.
Source: Urology Case Reports - Category: Urology & Nephrology Source Type: research

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CONCLUSIONS: different predictors were identified to ECMO complications. The knowledge of these predictors enables the individualized targeting of preventive interventions by multidisciplinary team for modifiable factors, as well as intensification of monitoring for early recognition of non-modifiable factors. PMID: 32236371 [PubMed - in process]
Source: Revista Brasileira de Enfermagem - Category: Nursing Authors: Tags: Rev Bras Enferm Source Type: research
CONCLUSION: Older age, serous effusion, type 2 VT, presence of post-VTI perforation, and frequent VTI may be risk factors for myringosclerosis after VTI; the severity of myringosclerosis may vary based on the duration of effusion and frequency of VTI. PMID: 32242343 [PubMed - in process]
Source: J Korean Med Sci - Category: General Medicine Authors: Tags: J Korean Med Sci Source Type: research
CONCLUSION: The absence of vasospasm in the first DSA performed in patients with SAH and negative tomographic angiography could be a useful criterion for establishing an optimal follow-up protocol in these patients, without exposing them to unnecessary risks and radiation. PMID: 32242335 [PubMed - in process]
Source: Revista de Neurologia - Category: Neurology Authors: Tags: Rev Neurol Source Type: research
CONCLUSIONS: Laparoscopic access to RN in patients with renal cancer in ≤pT3a stage increased surgical time only in the first years, reduced hospital stayand postoperative complications and did not influence RFS, OS or CSS. PMID: 32240107 [PubMed - in process]
Source: Archivos Espanoles de Urologia - Category: Urology & Nephrology Tags: Arch Esp Urol Source Type: research
Abstract In patients with tuberous sclerosis complex (TSC), renal complications are not limited to bleeding angiomyolipoma (AML); although rare, end-stage renal disease (ESRD) may occur. New treatments (e.g., mammalian target of rapamycin (m-Tor) inhibitors) for AML might influence the epidemiology of ESRD in patients with TSC. In France, 99 patients with TSC from the Renal Epidemiology and Information Network (REIN) registry and having undergone renal replacement therapy (RRT) between 2002 and 2016 were included in the present study. Additional data were collected from the patients' medical charts. The mean&thins...
Source: Journal of Nephrology - Category: Urology & Nephrology Authors: Tags: J Nephrol Source Type: research
This report suggests that the transglutaminase FXIII, a blood coagulation factor, may have an important clinical benefit in treating recurrent or thoracentesis-refractory CPE in TSC-LAM. To our knowledge, this is the first known case in the literature describing the successful treatment of CPE with FXIII in TSC-LAM. Because CPE is rare and there is currently no gold standard for its management, regardless of etiology, further research is warranted to investigate the potential clinical use of FXIII as an effective and safe treatment strategy in selected patients. PMID: 30248722 [PubMed]
Source: Lymphology - Category: Internal Medicine Tags: Lymphology Source Type: research
ConclusionIn giant TS-associated RAML total nephrectomy, rather than conservative treatment, is the treatment of choice in order to reduce the risk of potentially life-threatening bleeding.
Source: African Journal of Urology - Category: Urology & Nephrology Source Type: research
We report a case of giant bilateral AML and discuss the diagnosis and treatment of it. The 22-year-old man was admitted due to bilateral flank pain, gross hematuria, and abdominal fullness. He had history of epilepsy, mental retardation, and delayed development during childhood. He had angiofibroma on his face since 10 years ago. Abdominal CT and MRI revealed large lobulated heterogeneous mass with fatty content. Based on those findings, we diagnosed the patient with bilateral giant renal AML. We gave conservative management for the patient and planned to total nephrectomy on the left kidney if the continued bleeding occur...
Source: Acta medica Indonesiana - Category: Internal Medicine Tags: Acta Med Indones Source Type: research
AbstractPurposeTo describe the patient characteristics, treatments, disease monitoring, and kidney function of patients with sporadic angiomyolipoma (sAML), stratified by the number and size of renal angiomyolipomas (AMLs).MethodsSingle-center retrospective analysis of patients with sAML treated from 1990 to 2015 in a dedicated clinic for inheritable tumor syndromes in a tertiary referral center from the Netherlands. Patients ’ first AML assessment at the clinic was defined as the index date. Patient characteristics were measured at the index date. Treatments, disease monitoring, and kidney function were measured pos...
Source: International Urology and Nephrology - Category: Urology & Nephrology Source Type: research
AbstractIn adult tuberous sclerosis complex (TSC) patients, renal complications are the leading cause of death. Beginning in childhood, up to 80  % of patients develop renal angiomyolipoma characterized by a size-dependent risk of life-threatening bleeding. After discovery of the two causative genes,TSC1 andTSC2, and the role of mammalian target of rapamycin (mTOR) regulation in the pathogenesis of TSC, an increasing number of clinical studies evaluating mTOR inhibition in TSC patients have shown impressive results in many organ manifestations, such as brain, lung, and kidney. For renal angiomyolipoma, mTOR inhibitor ...
Source: Pediatric Nephrology - Category: Urology & Nephrology Source Type: research
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