THROMBOTIC MICROANGIOPATHY IN CHILDREN : CAUSES, DIAGNOSTICS, TREATMENT, TRACKING AND OUTCOME

Cilj istraživanja: Cilj ovog rada bio je utvrditi pojavnost trombotične mikroangiopatije u djece liječene na Klinici za dječje bolesti u Kliničkom bolničkom centru Split, u razdoblju od siječnja 2006. do prosinca 2015. godine. Ispitanici i metode: Retrospektivno je pregledana bolnička dokumentacija, tj. povijesti bolesti i ambulantni nalazi 29 bolesnika sa slikom trombotične mikroangiopatije na Klinici za dječje bolesti KBC-a Split. Bolesnicima su analizirani sljedeći parametri: spol, dob, prisutnost proljeva i krvavog proljeva u kliničkoj slici, mogući uzročnik bolesti, prisutnost vrućice, razni laboratorijski parametri (vrijednosti hemoglobina, trombocita, shizocita, haptoglobina, proteina u urinu, bilirubina, LDH, ureje, kreatinina, C3, C4, D-dimera, Coombsovog testa, aktivnosti ADAMTS13), boja kože, prisutnost hematoma, neuroloških simptoma i arterijske hipertenzije. Također je zabilježen broj bolesnika liječenih u JILD-u, vrste terapijskih postupaka (transfuzija eritrocita, transfuzija svježe smrznute plazme, transfuzija trombocita, plazmafereza, diuretici, antibiotici, antihipertenzivni lijekovi, inotropi, dijaliza) te konačan ishod liječenja (živ, umro, premješten/nepoznato). Analizirani su i podaci iz ambulantnih nalaza čime se dobio uvid u postojanje mogućih konačnih posljedica u 29 bolesnika liječenih od trombotične mikroangiopatije u Klinici za dječje bolesti KBC Split. Rezultati: U izabranom razdoblju istraživanje je obuhvatilo 29 ispitanika, 18 (62%) dječaka i 11 (38%) djevojčica. 21 bolesnik je bio u dobi do 5 godina života (72%). Od 29 slučajeva trombotične mikroangiopatije, u 62% djece (n=18) nije izoliran uzročnik. Među tih 18 ispitanika je bio 1 slučaj aHUS-a posredovanog komplementom te 1 slučaj TTP-a. Izolirani su uzročnici: E. coli O111 (n=3), E. coli O26 (n=2), EPEC (nije poznat serotip), E. coli O118, E. coli O127, G+ (gram pozitivni) koki, Influenza A, Streptococcus pneumoniae. Proljev je bio simptom u svih bolesnika sa STEC-om. Također, proljev je imalo i 81% ispitanika kojima nije izoliran uzročnik u kojih se može sumnjati na D+ HUS. Krvavi proljev je imalo oko polovice ispitanika s proljevom (54%). Krvavi proljev su imali i ispitanici s aHUS-om, TTP-om te G+ kokima. Neurološki simptomi su se javili kod 7 ispitanika, a samo je jedan imao TTP. Smrtnost kod ispitanika je 4% (n=1). Zaključci: Pokazano je preklapanje kliničke slike i nekih laboratorijskih parametara među bolestima koje spadaju u TMA što ukazuje na važnost unapređenja dijagnostike s ciljem što uspješnijeg liječenja bolesnika.Objective: The aim of this paper was to determine the occurrence of thrombotic microangiopathy in children treated at the Department of Pediatrics, Split University Hospital in the period from January 2006 to December 2015. Subjects and methods: Retrospectively reviewed medical records of 29 patients with a clinical picture of thrombotic microangiopathy at the Department of Pediatrics, Split University Hospital. The following parameters were analyzed: gender, age, diarrhea and bloody diarrhea in the clinical picture, possible cause of the disease, presence of fever, various laboratory parameters (hemoglobin, platelet count, shizocytes, haptoglobin, urine protein, bilirubin, LDH, urea, creatinine , C3, C4, D-dimer, Coombs test, ADAMTS13 activity), skin color, presence of hematoma, neurological symptoms and arterial hypertension. The number of patients treated at PICU, types of therapeutic procedures (erythrocyte transfusions, fresh frozen plasma transfusions, platelet transfusions, plasma perfusion, diuretics, antibiotics, antihypertensive drugs, inotropes, dialysis) and ultimate outcome of treatment (live, dead, unknown). Data from outpatient findings were also analyzed to give an insight into the possible final outcome in 29 patients treated with thrombotic microangiopathy at the Department of Pediatrics, Split University Hospital. Results: In the selected period, the survey included 29 respondents, 18 (62%) boys and 11 (38%) girls. 21 patients were up to 5 years of age (72%). Out of 29 cases of thrombotic microangiopathy, in 62% of our respondents (n=18) cause was unknown. Among these 18 respondents was 1 case of aHUS mediated by complement and 1 TTP case. Isolated Causes: E. coli O111 (n=3), E. coli O26 (n=2), EPEC (unknown serotype), E. coli O118, E. coli O127, G + (Gram positive) cocci, Influenza A, Streptococcus pneumoniae. Diarrhea was a symptom in all patients with STEC. Also, diarrhea was found in 81% of subjects in which hasn't been isolated the suspected cause of D + HUS. About half of the patients with diarrhea had bloody diarrhea (54%). Bloody diarrhea was also a symptom in children with aHUS, TTP and G+ cocci. Neurological symptoms occurred in 7 subjects, and only one had TTP. Mortality among respondents was 4% (n=1). Conclusions: The clinical picture and some laboratory parameters that overlaped in various diseases of TMA have shown the importance of improving diagnostics in order to treat patients more succesfully and according to the cause of the disease