title: [Pulmonary thromboembolism as a late complication of mitochondrial myopathy (Kearns-Sayer syndrome ].
abstract: A case of pulmonary thromboembolism with transient pulmonary hypertension of a rare cause is presented. In 24-year-old woman myasthenia was recognised on the ground of ptosis and fixation of eyes muscles from the 14th year of age. The treatment with mestinon was ineffective. Before planned thymectomy serious disturbances of heart rhythm and conduction were confirmed. Stimulator was implanted and thymectomy was done. No improvement of neurological state was observed despite the treatment with mestinone and prednisone. When she was 30 years old disease of lung appeared with fever, cough and parenchymal and pleural lesions of right lung. Antibiotic therapy was ineffective. CT scan and US examination revealed large thrombus inside the right auricle connected with stimulator electrodes. Embolisation of right pulmonary arteria was confirmed also. Treatment with heparin was ineffective and thrombectomy was performed. Exact neurological examination stated that the patient had no typical symptoms of myasthenia and that symptoms related with eyes and heart could be result of mitochondrial myopathy. Diagnosis was confirmed by EMG examination and muscle biopsy.
authors.affiliation: III Kliniki Chorób Płuc IGiCHP w Warszawie.
authors.forename: Justyna
authors.initials: J
authors.lastname: Fijołek
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Wiatr
authors.forename: Anna
authors.initials: A
authors.lastname: Wiechecka
authors.forename: Adam
authors.initials: A
authors.lastname: Torbicki
authors.forename: Andrzej
authors.initials: A
authors.lastname: Biederman
authors.forename: Anatol
authors.initials: A
authors.lastname: Mickielewicz
authors.forename: Kazimierz
authors.initials: K
authors.lastname: Roszkowski-Sliz
mesh_terms: D000328:Adult; D001763:Blepharoptosis / Q000209:etiology; D003937:Diagnosis, Differential; D005260:Female; D006325:Heart Atria / Q000503:physiopathology; D006337:Heart Murmurs / Q000209:etiology; D006801:Humans; D007625:Kearns-Sayre Syndrome / Q000175:diagnosis* / Q000503:physiopathology*; D011655:Pulmonary Embolism / Q000209:etiology* / Q000628:therapy*; D017131:Thrombectomy; D013997:Time Factors