The Proteus syndrome associated with life threatening hematuria.

Urology Division, Hôpital Sainte-Justine, Université de Montréal, Québec, Canada.
The Journal of Urology (Impact Factor: 4.47). 01/2004; 170(6 Pt 1):2418-9. DOI: 10.1097/01.ju.0000088341.38989.3a
Source: PubMed
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    • "In spite of aggressive treatment, patients can suffer severe functional and cosmetic consequences from this disorder [1]. Franc-Guimond et al. reported the first case of bladder hemangioma and PS [2]. This case, unlike ours, involved extensive intravesical venous lesions and an open approach (partial cystectomy) was entertained. "
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    ABSTRACT: A case of bladder hemangiomas in association with Proteus syndrome (PS) is described. PS is a rare hamartomatous disorder and comprises an association of verrucous epidermal nevi, infantile hemangiomas, lipoma-like subcutaneous hamartomas and asymmetrical overgrowth of almost any part of the body. To our knowledge, this is the second case reported of hematuria associated with bladder hemangiomas in PS.
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    ABSTRACT: Proteus syndrome is a rare hamartomatous disorder comprising a broad spectrum of congenital malformations and overgrowth of multiple tissues. Some rare urogenital malformations have been reported before. This case is unique for the presence of multiple genitourinary anomalies including retroperitoneal cystic mass, intra-abdominal testicle with hematoma and ureterovesical stricture apart from common clinic findings of proteus syndrome.
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    ABSTRACT: Gross hematuria in children is a common complaint that often leads patients to seek urgent care. The diagnostic evaluation can be chosen based on specific patient history and physical examination. When a patient is asymptomatic, hypercalciuria and mild forms of glomerulonephritis are common causes of gross hematuria. Although they are less common in children, special care should be taken to investigate for renal and bladder tumors.
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