Amyloidosis as a Systemic Disease in Context

Publication date: Available online 17 January 2020Source: Canadian Journal of CardiologyAuthor(s): Sarah AM. Cuddy, Rodney H. FalkAbstractThe systemic amyloidoses are a group of diseases, all characterized by the deposition of amyloid, a material formed from misfolding of proteins, in one or more organs. The 2 commonest forms of amyloidosis, transthyretin amyloidosis (derived from wild-type {ATTRwt} or mutant transthyretin {ATTRm}) and light chain (AL) amyloidosis, derived from abnormal circulating light chains produced by a plasma cell dyscrasia. Both frequently involve the heart, producing an infiltrative cardiomyopathy with restrictive pathophysiology. Although advances in echocardiographic, magnetic resonance and nuclear imaging have rendered diagnosis of cardiac amyloidosis easier, diagnosis is still often delayed. This review focuses on non-cardiac manifestations of AL and TTR amyloidosis that may aid the cardiologist in making an earlier diagnosis of cardiac amyloidosis in a patient with cardiac symptoms (such as periorbital purpura in AL amyloidosis and a history of carpal tunnel syndrome and ruptured biceps tendon in ATTR)). It also focuses on the unique challenges that treatment of cardiac amyloidosis poses because of concomitant non-cardiac disease, such as nephrotic syndrome-related edema and the hypotension of autonomic neuropathy, and stresses the importance of a precise typing of amyloidosis and of a multidisciplinary approach to therapy.
Source: Canadian Journal of Cardiology - Category: Cardiology Source Type: research

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We report a case of a young male with no prior genitourinary history who presents to an emergency department with sudden onset gross hematuria, clot retention, and right-sided flank pain. On evaluation, he was found to have a renal artery aneurysm bleeding into his collecting system and underwent renal artery embolization and rapid resolution of his hematuria. Renal vascular pathology should be considered in the differential diagnosis and timely diagnosis of this condition is imperative as surgical interventions have proven to be life-saving. PMID: 32065873 [PubMed - in process]
Source: Canadian Journal of Urology - Category: Urology & Nephrology Tags: Can J Urol Source Type: research
Authors: Tabakin AL, Farber NJ, Barone J Abstract Testicular torsion is a common pediatric emergency. Predisposing factors and other risk factors for testicular torsion have not been fully elucidated. This case report highlights teenage brothers who each experienced right-sided testicular torsion at nearly the same age, just 1 year apart. Because of their parents' urgency in seeking medical attention, the affected testicles in both patients were salvaged. We also review the current literature in regards to etiology, inheritance, and patient and parental education. PMID: 32065872 [PubMed - in process]
Source: Canadian Journal of Urology - Category: Urology & Nephrology Tags: Can J Urol Source Type: research
CONCLUSION: The use of RGP prior to ureteral stent placement for an obstructing ureteral stone with concomitant UTI was not associated with unfavorable clinical outcomes in our retrospective series. While these findings support the safety of RGP in this setting, prospective trials are warranted. PMID: 32065871 [PubMed - in process]
Source: Canadian Journal of Urology - Category: Urology & Nephrology Tags: Can J Urol Source Type: research
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Source: Canadian Journal of Urology - Category: Urology & Nephrology Tags: Can J Urol Source Type: research
Authors: Peyton CC, Hajiran A, Morgan K, Azizi M, Tang D, Chipollini J, Gilbert SM, Poch M, Sexton WJ, Spiess PE Abstract INTRODUCTION: To describe the incidence, contemporary management, risk factors and outcomes of urinary leak following open and robotic partial nephrectomy at a tertiary care, comprehensive cancer center. MATERIALS AND METHODS: We reviewed 975 patients who underwent partial nephrectomy at Moffitt Cancer Center from January 2009 to May 2017. Patient demographic, perioperative and follow up data was recorded and compared stratified for postoperative urine leak. Fisher's exact and Wilcoxon sum-r...
Source: Canadian Journal of Urology - Category: Urology & Nephrology Tags: Can J Urol Source Type: research
Authors: Petrossian RA, Dynda D, Delfino K, El-Zawahry A, McVary KT Abstract INTRODUCTION: The role of organized sleep in overall health and quality-of-life (QoL) is critical. Nocturia necessarily disrupts the normal sleep cycle and negatively impacts one's health, work productivity, and QoL. We investigated, for the first time in an exploratory pilot, the effectiveness of mirabegron for improving sleep disturbance and nocturia. MATERIALS AND METHODS: This was a prospective, open-label 12-week trial evaluating the efficacy of mirabegron in 34 men and women with disordered sleep and lower urinary tract symptoms ...
Source: Canadian Journal of Urology - Category: Urology & Nephrology Tags: Can J Urol Source Type: research
Authors: Kim EH, Andriole GL PMID: 32065867 [PubMed - in process]
Source: Canadian Journal of Urology - Category: Urology & Nephrology Tags: Can J Urol Source Type: research
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Source: Canadian Journal of Urology - Category: Urology & Nephrology Tags: Can J Urol Source Type: research
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Source: Canadian Journal of Urology - Category: Urology & Nephrology Tags: Can J Urol Source Type: research
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Source: Canadian Journal of Urology - Category: Urology & Nephrology Tags: Can J Urol Source Type: research
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