Giant angiomyolipoma in a tuberous sclerosis patient and review of the literature

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

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This study investigates the distribution of femoral metastases in cancer patients, specifically addressing the incidence of distal femoral metastases. PET/CT examinations routinely extend only to mid-thigh level, precluding detection of distal metastases. We found a total of 208 femoral metastases in 112 patients. 30% had distal femoral metastases in addition to other areas of involvement. 7% of patients with femoral metastases had only distal femur disease. 6 patients had distal pathologic fractures. Exclusion of the distal femur during PET/CT may result in a missed or delayed diagnosis that could contribute to the develo...
Source: Cancer Investigation - Category: Cancer & Oncology Tags: Cancer Invest Source Type: research
The objectives of this video are to describe common anatomic and pathologic challenges for achieving hemostasis, to outline the strategies for laparoscopic dissection to prevent bleeding and to deconstruct the process of regaining hemostasis when brisk bleeding occurs.
Source: American Journal of Obstetrics and Gynecology - Category: OBGYN Authors: Tags: Video Presentation Source Type: research
Death in America is changing from hospital to home, which demands complex skills by family caregivers. However, information from family members about the challenges of providing home hospice care until death is scant. To understand the challenges a family caregiver confronts when he/she decides to deliver hospice care and during the actual delivery of the hospice care, we used descriptive phenomenology methods to document the experience of 18 family caregivers as they delivered home hospice care. We learned the decision to begin home hospice was made by a physician, followed frequently by family resistance and refusal to a...
Source: Journal of Hospice and Palliative Nursing - Category: Nursing Tags: Feature Articles Source Type: research
Authors: Bailey HE Abstract A severe and unusual complication found in children with influenza is an acute necrotizing encephalopathy. A 20-month-old female with no significant past medical history was admitted to our facility, presenting with a 4-day history of worsening fever, upper respiratory symptoms, new-onset altered mental status and episodes of extensor posturing. The initial concern was a dystonic reaction secondary to promethazine following a recent diagnosis of influenza A virus. A head computed tomography scan indicated concern for widespread edema, and the video EEG revealed focal slowing in the front...
Source: The Neurodiagnostic Journal - Category: Neurology Tags: Neurodiagn J Source Type: research
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Source: European Journal of Emergency Medicine - Category: Emergency Medicine Tags: Perspective Articles Source Type: research
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
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
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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