AGGRESSIVE PROGRESSION OF TAKAYASU'S ARTERITIS IN INFANCY: A CASE REPORT/AGRESIVNI OBLIK UZNAPREDOVALOG TAKAYASUOVA ARTERITISA U DOJENACKOJ DOBI: PRIKAZ SLUCAJA

• Published in: Acta clinica Croatica (Tisak) Vol. 58; no. 3; p. 535
• Main Authors: Pavic, Roman, Blazekovic, Robert, Divkovic, Dalibor, Marjanovic, Ksenija, Sipl, Mirna
• Format: Journal Article
• Language: English
• Published: Klinicki bolnicki centar Sestre milosrdnice 01.09.2019
• Subjects: Amputation; Angiography; Arteries; CAT scans; Croatia; Cyanosis; Development and progression; Diagnosis; Diagnostic imaging; Glomerulonephritis; Health aspects; Hypercoagulation; Immunosuppressive agents; Mortality; Surgery; Tachycardia; Thrombosis; Tomography; Croatia
• ISSN: 0353-9466; 1333-9451
• DOI: 10.20471/acc.2019.58.03.19

Takayasu's arteritis (TA) affects the aorta and its branches. Immunosuppressants are the usual course of therapy, while surgery has been used in acute cases. There is only scant information on TA in infancy, and the nonspecific symptoms in the initial stage of the disease make the diagnosis difficult and delayed, thus increasing the mortality rate. We describe a case of aggressive progression of TA in an infant. This child was the youngest to be affected with the disease as reported in the literature. A 3.5-month-old boy presented with cyanosis of both legs, tachycardia and antithrombin III deficiency. Computed tomography angiography (CTA) revealed thrombosis of distal aorta and both iliac arteries. Thrombectomy was performed at the level of both common femoral arteries. In addition, thigh amputation of the left leg had to be performed. TA was diagnosed postmortem with thrombosis of the distal aorta, its branches and upper mesenteric artery which was not occluded on previous CTA, glomerulonephritis and pulmonary parenchymal granulomatous infiltrations. Key words: Takayasu arteritis; Infant; Thrombosis; Immunosuppressive agents; Case reports Takayasuov arteritis (TA) zahvaca aortu i njezine ogranke. Imunosupresivi su obicno terapija ove bolesti, dok je kirursko lijecenje opisano samo u akutnim slucajevima. U literaturi ne postoji puno podataka o TA u dojenackoj dobi. Nespecificni simptomi na pocetku bolesti otezavaju postavljanje dijagnoze i povecavaju stopu smrtnosti. Bolesnik opisan u ovom prikazu slucaja je najmladi ikad objavljen u literaturi. Tri i pol mjeseca star djecak je imao cijanoticne obje noge, tahikardiju i manjak antitrombina III. Kompjutorizirana tomografija angiografija (CTA) je pokazala trombozu distalne aorte i obiju ilijacnih arterija. Trombektomija je odmah zatim ucinjena na razini obiju zajednickih femoralnih arterija. Daljnje komplikacije su zahtijevale natkoljenicnu amputaciju lijeve noge. TA je dijagnosticiran nakon smrti bolesnika pri obdukciji s vidljivom trombozom distalne aorte i njezinih ogranaka, gornje mezentericne arterije koja nije bila zahvacena na ranije ucinjenoj CTA te glomerulonefritisom i plucnom parenhimatoznom granulomatoznom infiltracijom. Kljucne rijeci: Takayasuov arteritis; Dojence; Tromboza; Imunosupresivna sredstva; Prikazi slucaja