Kidney damage due to tuberous sclerosis complex: Management recommendations - 05/03/13
Abstract |
Objective |
To deduce recommendations from the literature on the management of kidney damage caused by tuberous sclerosis complex (TSC).
Material and methods |
Five practitioners have written up recommendations after reviewing the literature. They were evaluated by 14 experts using a 9 level scale (1: complete disagreement; 9: complete agreement), then reworded until each item received a median score of greater than or equal to 8.
Results |
Forty-eight to 80% of patients with TSC have kidney disease with the presence of angiomyolipomas (AML), cysts, cancers and/or progression towards renal insufficiency. An abdominal ultrasound (and serum creatinine level if there is an abnormality) is recommended as soon as the TSC is diagnosed. The evaluation should be repeated every 3 to 5years if it is normal. Numerous and voluminous cysts are suggestive of associated polycystosis. After 20 years of age, the monitoring should be based on CT scan or MRI, which are more precise in the monitoring of AML. The biopsy of a renal mass should be discussed if there are calcifications, central necrosis or rapid growth. Lymphangioleiomyomatosis should be screened for in women via pulmonary CT scan at 18 and 30 to 40 years of age. Haemorrhagic rupture of an AML should be treated in first-line by embolisation. Asymptomatic AMLs that cumulate risk factors for bleeding (size>80mm, predominant vascular contingent, micro-aneurisms) should be preventively treated, if possible by embolisation. The role of mTOR inhibitors remains to be defined.
Conclusion |
Monitoring and a standardised treatment are necessary to improve the treatment of renal damage caused by TSC.
Le texte complet de cet article est disponible en PDF.Keywords : Tuberous sclerosis complex, Kidney, Angiomyolipoma, Embolisation, mTOR
Plan
☆ | These guidelines have originally been published under the reference Rouvière O, Nivet H. Grenier N, Zini L, Lechevallier E. Prog Urol 2012;22(7):367–79 and have been translated and are reproduced with permission from the publisher. The original reference must be used when citing the article. |
Vol 94 - N° 3
P. 225-237 - mars 2013 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.