Congenital complete heart block (CCHB) is a rare disorder (maybe 1/20.000 live-born infants) that is caused by the transplacental transport of maternal antibodies (anti-Ro/SSA, particularly anti 52 kD, and/or anti- La/SSB). These antibodies may induce a myocarditis and/or may directly interact with calcium channel proteins, resulting in perturbation of transmembrane signaling at the level of the conduction tissue. The mothers have a specific HLA pattern: A1/B8/DR3/DQ2 and B44/DR5. Depending on the severity of the process the fetus may die in utero or a few days after birth, or survive to the perinatal period and have a near normal life; in most survivors a pacemaker must be implanted. Skin lesions, anemia, thrombocytopenia, hepatic cholestasis are other transient clinical features of the syndrome. The risk of recurrence of CCHB seems range from 8 to 20%. Most of the mothers are asymptomatic at delivery and are identified only by the birth of an affected child. Also their longterm outcome is more reassuring than generally assumed; in fact arthralgias and dry eyes are the commonest symptoms, and in many cases the symptoms are mild and occur many years after the index delivery; therefore the term 'neonatal lupus' is in fact misleading in most cases. A standard therapy for CCHB detected in utero does not exist; we are proposing a multicentre controlled randomized study of dexamethasone versus salbutamol for this condition.

Blocco cardiaco completo congenito e manifestazioni correlate (lupus neonatale) / A. Brucato, F. Franceschini, M.P. Pisoni, G. Vignati, L. Balossi, E. Coluccio, S. Pittau, A. Lojacono, L. Colombo, B. Canesi. - In: REUMATISMO. - ISSN 0048-7449. - 48:3(1996), pp. 245-258.

Blocco cardiaco completo congenito e manifestazioni correlate (lupus neonatale)

A. Brucato;
1996

Abstract

Congenital complete heart block (CCHB) is a rare disorder (maybe 1/20.000 live-born infants) that is caused by the transplacental transport of maternal antibodies (anti-Ro/SSA, particularly anti 52 kD, and/or anti- La/SSB). These antibodies may induce a myocarditis and/or may directly interact with calcium channel proteins, resulting in perturbation of transmembrane signaling at the level of the conduction tissue. The mothers have a specific HLA pattern: A1/B8/DR3/DQ2 and B44/DR5. Depending on the severity of the process the fetus may die in utero or a few days after birth, or survive to the perinatal period and have a near normal life; in most survivors a pacemaker must be implanted. Skin lesions, anemia, thrombocytopenia, hepatic cholestasis are other transient clinical features of the syndrome. The risk of recurrence of CCHB seems range from 8 to 20%. Most of the mothers are asymptomatic at delivery and are identified only by the birth of an affected child. Also their longterm outcome is more reassuring than generally assumed; in fact arthralgias and dry eyes are the commonest symptoms, and in many cases the symptoms are mild and occur many years after the index delivery; therefore the term 'neonatal lupus' is in fact misleading in most cases. A standard therapy for CCHB detected in utero does not exist; we are proposing a multicentre controlled randomized study of dexamethasone versus salbutamol for this condition.
Settore MED/09 - Medicina Interna
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/638678
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