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Prevention of recurrence of congenital heart block with intravenous immunoglobulin and corticosteroid therapy: comment on the editorial by Buyon et al - Reply [Letter]

Buyon, JP; Kim, MY; Copel, JA; Friedman, DA
ISI:000180418500049
ISSN: 0004-3591
CID: 55590

Fetal cardiac function assessed by Doppler myocardial performance index (Tei Index)

Friedman, D; Buyon, J; Kim, M; Glickstein, J S
OBJECTIVES: The Tei Index (TI) is a useful, non-invasive, Doppler-derived myocardial performance tool which can be used to assess aspects of systolic and diastolic function. The aim of this study was to determine normal values of fetal left ventricular (LV) TI in second- and third- trimester fetuses and to compare these to other values reported in the literature. METHODS: Doppler waveforms of the LV outflow tracts were obtained in 74 second- and early third-trimester fetuses. The LV isovolumic contraction time (ICT), isovolumic relaxation time (IRT) and ejection time (ET) were measured and the TI calculated using the formula (ICT + IRT)/ET. The literature on LV myocardial function in the fetus was also reviewed. RESULTS: The normal TI in second- and early third-trimester fetuses (18-31 weeks' gestation) was 0.53 +/- 0.13. The ICT was 43 +/- 14 ms, the ET was 173 +/- 16 ms and the IRT was 48 +/- 13 ms. CONCLUSION: The TI can be easily obtained in the fetus without the need for precise anatomic imaging. The TI may be a useful tool to explore fetal myocardial function in different clinical situations
PMID: 12528158
ISSN: 0960-7692
CID: 132526

From antibody insult to fibrosis in neonatal lupus - the heart of the matter [Editorial]

Buyon, Jill P; Clancy, Robert M
Few diseases exemplify the integration of research from bench to bedside as well as neonatal lupus, often referred to as a model of passively acquired autoimmunity. In essence, this disease encompasses two patients, both the mother and her child. The signature histologic lesion of autoimmune-associated congenital heart block is fibrosis of the conducting tissue, and in some cases the surrounding myocardium. It is astounding how rapid and, in most cases, irreversible is the fibrotic response to injury. The mechanism by which maternal anti-SSA/Ro-SSB/La antibodies initiate and perpetuate inflammation, and eventuate in scarring of the atrioventricular node, is not yet defined. In vitro and in vivo studies suggest that one pathologic cascade leading to scarring may be initiated via apoptosis, resulting in translocation of SSA/Ro-SSB/La antigens and subsequent surface binding by maternal autoantibodies. These opsonized cardiocytes are phagocytosed by macrophages, which secrete factors that transdifferentiate fibroblasts into myofibroblasts, a scarring phenotype. Dissecting the individual components in this fibrotic pathway should provide insights into the rarity of irreversible injury and should form the basis of rational approaches to prevention and treatment
PMCID:333420
PMID: 14680501
ISSN: 1478-6362
CID: 46212

The autoimmune connection : essential information for women on diagnosis, treatment, and getting on with life

Baron-Faust, Rita; Buyon, Jill P
Chicago IL : Contemporary Books, 2003
Extent: xiv, 411 p. ; 23cm
ISBN: 0658021311
CID: 903

The autoimmune connection : essential information for women on diagnosis, treatment, and getting on with life

Baron-Faust, Rita; Buyon, Jill P
New York : McGraw-Hill, 2003
Extent: xiv, 411 p. ; 23cm
ISBN: 0071433155
CID: 904

Congenital heart block: do fetal factors fuel the fire from inflammation to fibrosis? [Editorial]

Buyon, Jill P; Rupel, Ann; Clancy, Robert M
PMID: 14596420
ISSN: 0961-2033
CID: 73534

Birth order, gender and recurrence rate in autoantibody-associated congenital heart block: implications for pathogenesis and family counseling [Letter]

Solomon, Daniel G; Rupel, Ann; Buyon, Jill P
PMID: 12945727
ISSN: 0961-2033
CID: 73535

Neonatal Lupus Syndromes: Clinical Features

Brucato, A; Buyon, JP
SCOPUS:67649639156
ISSN: 1571-5078
CID: 650962

Proposal for a new definition of congenital complete atrioventricular block

Brucato, A; Jonzon, A; Friedman, D; Allan, L D; Vignati, G; Gasparini, M; Stein, J I; Montella, S; Michaelsson, M; Buyon, J
The classic old definition of congenital heart block by Yater (1929) is still generally accepted: 'Heart block established in a young patient. There must be some evidence of the existence of the slow pulse at a fairly early age and absence of a history of any infection which might cause the condition after birth: notably diphtheria, rheumatic fever, chorea and congenital syphilis'. However, other definitions are used. We systematically reviewed 1825 cases from 38 separate studies. We conclude that complete AV blocks detected in utero in the absence of structural abnormalities differ from blocks detected later in life with respect to pathogenesis (they are generally associated with maternal anti-Ro/SSA antibodies), poorer childhood prognosis, increased risk of developing late-onset dilated cardiomyopathy, different maternal clinical features and increased risk of recurrence in future pregnancies. For these reasons we propose a new modern definition of congenital complete AV block which might be acceptable to cardiologists, rheumatologists, pediatricians and obstetricians: 'an AV block is defined as congenital if it is diagnosed in utero, at birth or within the neonatal period (0-27 days after birth)'.
PMID: 12873043
ISSN: 0961-2033
CID: 650472

C3, C4, CH50, anti-dsDNA antibodies and C3a complement split products are specific but not sensitive for predicting flares of systemic lupus (SLE) [Meeting Abstract]

Tseng, CE; Abramson, SB; Kim, M; Blemont, HM; Haines, K; Petri, M; Buyon, JP
ISI:000178421800524
ISSN: 0004-3591
CID: 37119