Hemolitična bolezen ploda in novorojenčka (HBPN) nastane zaradi vezave aloimunskih protiteles na otrokove eritrocitne antigene, kar povzroči razpad eritrocitov ter nastanek hiperbilirubinemije in ...anemije novorojenčka. Aloimunska protitelesa, ki prehajajo preko posteljice iz materinega v plodov krvni obtok, so lahko naravno prisotna protitelesa anti-A in anti-B ali pa redkeje prisotna protitelesa, ki nastanejo po izosenzibilizaciji matere na tuje eritrocitne antigene, kot so protitelesa anti-D. Do izosenzibilizacije matere lahko pride ob transfuziji eritrocitov ali med nosečnostjo. HBPN zaradi protiteles anti-A in anti-B (HBPN AB0) lahko poteka z blago hiperbilirubinemijo in anemijo ali pa huje, z visoko hiperbilirubinemijo in izrazito anemijo, tako da je potrebna izmenjalna transfuzija krvi novorojenčka. HBPN zaradi protiteles anti-D je zaradi učinkovitega programa zaščite RhD negativnih nosečnic z imunoglobulini anti-D danes redka bolezen, njen potek pa v primerjavi s HBPN AB0 hujši.V prvem delu prispevka opišemo patofiziologijo, klinično sliko, diagnosticiranje, zdravljenje in preventivo HBPN. V drugem delu prispevka predstavljamo rezultate kohortne retrospektivne analize 109 bolnikov s HBPN, hospitaliziranih na Kliničnem oddelku za neonatologijo med januarjem 2007 in decembrom 2016. V 84,4 % so HBPN povzročila protitelesa anti-A ali anti-B, v 9,2 % protitelesa anti-D in v 6,4 % ostala, redkejša protitelesa. Bolniki s HBPN, povzročeno z različnimi protitelesi, se v rezultatih hematoloških preiskav niso statistično značilno razlikovali. Pozitiven direktni Coombsov test so imeli vsi novorojenčki s HBPN RhD in le 64 % novorojenčkov s HBPN AB0. Vsi bolniki s HBPN RhD so potrebovali izmenjalno transfuzijo ali znotrajmaternično transfuzijo.
Haemolytic disease of the foetus and newborn (HDFN) occurs when maternal alloimmune antibodies cross the placenta to the foetal circulation and cause destruction of erythrocytes, resulting in anaemia ...and hyperbilirubinemia. It can result from naturally present antibodies, such as anti-A and anti-B antibodies, or isoantibodies produced by women previously sensitised during pregnancy or transfusion of incompatible erythrocytes such as anti-D antibodies. RhD antigen is the most immunogenic and causes severe HDFN, while the spectrum of HDFN due to AB0 incompatibility ranges from subclinical to severe, requiring exchange transfusion. Thanks to effective and widely implemented prophylaxis with administration of anti-D immunoglobulin to RhD negative women, the HDFN due to anti-D is nowadays a rare disease. Present paper provides an overview on the pathophysiology, clinical characteristics, diagnostics, treatment and prevention of HDFN. In the second part of the article we present the results of a cohort retrospective analysis of 109 patients with HDFN hospitalised at the tertiary neonatal unit during 2007–2016. In 84.4 % of cases the underlying cause of HDFN were antibodies anti-A or anti-B, in 9.2 % of cases antibodies anti-D and in 6.4 % other more rare antibodies. HDFN caused by different antibodies did not result in statistically significant differences in the evaluated hematological tests. Direct Coombs test was positive in all patients with HDFN RhD and in 64 % of newborns with HDFN AB0. All patients with HDFN RhD required exchange transfusion or intrauterine transfusion.
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