Clinical Features of Lymphangioleiomyomatosis Complicated by Renal Angiomyolipomas
| Autor: | Naoko Mato, Takakiyo Nakaya, Hideaki Yamasawa, Yoshiko Mizushina, Masashi Bando, Yukihiko Sugiyama, Tatsuya Hosono, Yoshikazu Ishii |
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| Rok vydání: | 2011 |
| Předmět: |
Adult
medicine.medical_specialty Lung Neoplasms medicine.medical_treatment Angiomyolipoma Nephrectomy Neoplasms Multiple Primary Tuberous sclerosis Tuberous Sclerosis immune system diseases hemic and lymphatic diseases Internal Medicine medicine Humans In patient Lymphangioleiomyomatosis Major complication neoplasms Retrospective Studies business.industry Mean age General Medicine Middle Aged Prognosis bacterial infections and mycoses medicine.disease Kidney Neoplasms Surgery Female lipids (amino acids peptides and proteins) business Complication Bilateral Nephrectomy |
| Zdroj: | Internal Medicine. 50:285-289 |
| ISSN: | 1349-7235 0918-2918 |
| Popis: | Objective Renal angiomyolipomas (R-AMLs) are major complications of lymphangioleiomyomatosis (LAM). The objective of this study was to better understand the influence of R-AMLs in patients with LAM on the prognosis and other clinical factors related to respiration, and to investigate the management of R-AMLs in patients with LAM. Patients and Methods We retrospectively investigated the clinical features of 7 patients with LAM [4 were TSC (Tuberous sclerosis complex)-LAM and 3 were S (sporadic)-LAM] complicated by R-AMLs admitted to our hospital from 1997 to 2008. Results All patients were females and the mean age at diagnosis of LAM was 40.7 years (31.7 years for TSC-LAM and 52.7 years for S-LAM). Although 5 patients had symptoms related to R-AMLs, only 1 patient experienced symptoms related to R-AMLs at the time of diagnosis. Five patients had bilateral and 2 patients had unilateral R-AMLs. R-AMLs ruptured in 4 cases (3 patients were TSC-LAM) including 2 patients in whom they ruptured bilaterally, and who underwent bilateral nephrectomy. In 1 case, unilateral R-AMLs grew larger and appeared on the other side during the follow-up period. Conclusion Although only rare cases of LAM show symptoms related to R-AMLs initially, R-AMLs are a notable complication. To avoid nephrectomy, R-AMLs should be diagnosed when they are small and should be followed up carefully by periodic echograms or CT scans. |
| Databáze: | OpenAIRE |
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