Slimības gaita un terapija pacientei ar limfangioleiomiomatozi
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Date
2021
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Rīgas Stradiņa universitāte
Rīga Stradiņš University
Rīga Stradiņš University
Abstract
Ievads.
Limfangioleiomiomatoze (LAM) ir reta neoplastiska slimība, kas visbiežāk skar reproduktīva vecuma sievietes. LAM raksturo plaušaudu cistiskā destrukcija un progresējoša elpošanas mazspēja. LAM raksturīgi simptomi un pazīmes ir: elpas trūkums fiziskās slodzes laikā, rekurenti pneimotoraksi un vēdera dobuma audzēji, ieskaitot nieru angiomiolipomas un limfangiomiomas.
Klīniskais gadījums.
Paula Stradiņa Klīniskās universitātes slimnīcā ar labās nieres angiolipomas plīsumu un sekundāru retroperitoneālo hematomu tika stacionēta 46 gadus veca, citādi vesela, sieviete - veikta labās puses nefrektomija (2006. gads). Datortomogrāfija uzrādīja cistiskas izmaiņas plaušās. Diagnozes precizēšanai veikta video-asistēta torakoskopiska (VATS) marginālā biopsija - tika apstiprināta LAM diagnoze. Nākamajos gados paciente atzīmē progresējošu elpas trūkumu. 2017. gadā - spontāns sekundārs labās puses pneimotorakss - tika veikta pleiras dobuma drenāža. Dažas dienas vēlāk - atkārtots pneimotorakss - tika veikta labās puses VATS un daļēja pleirektomija. Pacientei dināmikā pasliktinās elpošanas funkcijas testi. 2018. gadā pacientei tiek nozīmēta Sirolimus terapija. Uz saņemtās terapijas fona paciente sasniedz plaušu funkcionālo testu stabilizēšanos un simptomu mazināšanos.
Secinājumi.
LAM ir ārkārtīgi reta slimība. Pēc LAM Foundation datiem prevalence ir no 3 līdz 5 sievietēm uz miljonu sieviešu. Visbiežāk slimība manifestējas ar plaušu slimības pazīmēm un simptomiem, bet iespējamas arī ekstrapulmonālās manifestācijas, īpaši nieru angiomiolipomas. Šis gadījums demonstrē LAM diagnostiku, ārstēšanu un slimības gaitu ilgstošā laika posmā. Sirolimus ir vienīgais medikaments, kas uzrādīja efektīvitāti LAM progresijas apturēšanai. Indikācija LAM ārstēšanai 2015. gadā ir apstiprinājusi ASV Pārtikas un zāļu pārvalde.
Backgrounds. Lymphangioleiomyomatosis (LAM) is a rare neoplastic disease which almost exclusively affects women. LAM is characterized by a cystic destruction of the lung and progressive respiratory failure. Symptoms and signs include dyspnea on exertion, recurrent pneumothorax, abdominal tumors including renal angiomyolipomas and lymphangiomyomas. Case report. A 46-years old previously healthy woman was reffered in Pauls Stradins Clinical University hospital with right kidney angiolipoma rupture and secondary retroperitoneal hematoma – the surgery was performed (2006 year). CT findings showed cystic changes in lungs; VATS marginal biopsy was performed – the diagnosis of LAM was confirmed. In the next years the patient notes progressive shortness of breath. In 2017 – secondary spontaneous right side pneumotorax – pleural space drainage was performed. A few days later – repeated pneumotorax – right side VATS and partial pleirectomy was performed. Gradual deterioration of respiratory function tests in the patient. In 2018 the patient was prescribed Sirolimus therapy. The patient achieved stabilization of lung functional tests and reduction of symptoms under treatment. Conclusions. LAM is extremely rare disease. The LAM Foundation estimated a prevalence of 3 to 5 women per million women. Most commonly, patients have the signs and symptoms of lung disease but can also present with manifestations arising from extrapulmonary sites, especially renal angiomyolipomas. This case demonstrates the diagnosis, management, and course of the disease over a long period of time. Sirolimus is the only medicine that has been shown to be effective to stop the disease from progressing. Indications for the treatment of LAM have been approved by US Food and Drug Administration in 2015.
Backgrounds. Lymphangioleiomyomatosis (LAM) is a rare neoplastic disease which almost exclusively affects women. LAM is characterized by a cystic destruction of the lung and progressive respiratory failure. Symptoms and signs include dyspnea on exertion, recurrent pneumothorax, abdominal tumors including renal angiomyolipomas and lymphangiomyomas. Case report. A 46-years old previously healthy woman was reffered in Pauls Stradins Clinical University hospital with right kidney angiolipoma rupture and secondary retroperitoneal hematoma – the surgery was performed (2006 year). CT findings showed cystic changes in lungs; VATS marginal biopsy was performed – the diagnosis of LAM was confirmed. In the next years the patient notes progressive shortness of breath. In 2017 – secondary spontaneous right side pneumotorax – pleural space drainage was performed. A few days later – repeated pneumotorax – right side VATS and partial pleirectomy was performed. Gradual deterioration of respiratory function tests in the patient. In 2018 the patient was prescribed Sirolimus therapy. The patient achieved stabilization of lung functional tests and reduction of symptoms under treatment. Conclusions. LAM is extremely rare disease. The LAM Foundation estimated a prevalence of 3 to 5 women per million women. Most commonly, patients have the signs and symptoms of lung disease but can also present with manifestations arising from extrapulmonary sites, especially renal angiomyolipomas. This case demonstrates the diagnosis, management, and course of the disease over a long period of time. Sirolimus is the only medicine that has been shown to be effective to stop the disease from progressing. Indications for the treatment of LAM have been approved by US Food and Drug Administration in 2015.
Description
Medicīna
Medicine
Veselības aprūpe
Health Care
Medicine
Veselības aprūpe
Health Care
Keywords
limfangioleiomiomatoze, intersticiālās plaušu slimības, lymphangioleiomyomatosis, interstitial lung disease