Lymphangioleiomyomatosis: solitary abdominal manifestation (2009: 9b)
| Autor: | Detlef Katenkamp, Sandra Pauls, Hans-Juergen Brambs, Anne-Kristin Possekel |
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| Rok vydání: | 2009 |
| Předmět: |
Radiography
Abdominal medicine.medical_specialty Abdominal pain Exploratory laparotomy medicine.medical_treatment Young Adult medicine Humans Radiology Nuclear Medicine and imaging Abdominal Neoplasms Lymphangioleiomyomatosis Neuroradiology Lung medicine.diagnostic_test business.industry Interventional radiology General Medicine medicine.disease Surgery medicine.anatomical_structure Female Radiology medicine.symptom Tomography X-Ray Computed business Rare disease |
| Zdroj: | European Radiology. 19:3015-3018 |
| ISSN: | 1432-1084 0938-7994 |
| DOI: | 10.1007/s00330-008-1275-4 |
| Popis: | Lymphangioleiomyomatosis (LAM) is a rare disease that usually affects premenopausal woman and is characterized by cystic lung lesions and lymphatic disorders. We report a case of a 23-year-old woman who presented with diffuse, but increasing abdominal pain. Transabdominal ultrasound showed multiple cystic formations. Due to the patient's uncharacteristic symptoms, an exploratory laparotomy with tissue sampling was performed, and the diagnosis of LAM was confirmed by two independent pathologists. With computed tomography a broad abdominal, but no pulmonary, manifestation could be established. During sirolimus therapy the patient showed clinical benefit, but only slight progress in computed tomography. |
| Databáze: | OpenAIRE |
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