Congenital complete heart block in the newborn associated with maternal systemic lupus erythematosus and other connective tissue disorders.

Hardy, Judy; Solomon, S D; Banwell, G. S.; Beach, Roberta K.; Wright, Victor J.; Howard, F M

doi:10.1136/adc.54.1.7

Cited by 80 publications

(10 citation statements)

References 30 publications

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“…Other less common manifestations can occur, including a haemolytic anaemia and thrombocytopenia [5.7], The major morbidity of NLE is associated with CHB. which has a !0 30% mortality and a significant morbidity, e.'^pecially if diagnosed early in life [22][23][24]. It has been suggested that the antibody response to the La protein and the 52-kD Ro protein are important in the development of CHB [3,8-11.25-27].…”

Section: Discussionmentioning

confidence: 99%

Autoantibody response to the Ro/La particle may predict outcome in neonatal lupus erythematosus

Silverman

Buyon

Laxer

et al. 1995

Clinical and Experimental Immunology

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SUMMARYThis study was undertaken to determine the role of antibodies against both recombinant Ro (r-Ro) and La (r-La) proteins and polypeptides derived from the recombinant La protein in predicting fetal and neonatal outcome in children at risk to develop neonatal lupus erythematosus (NLE). All sera were obtained in the perinatal period and quantitative ELISA assays were used. We collected 41 maternal sera within 2 months of delivery ofa child with NLE (21 with congenital heart disease block (CHB) and 20 with dermalologic NLE) and 19 sera from anti-Ro and/or anti-La antibodypositive mothers with systemic lupus erythematosus (SLE) who delivered a child without NLE. All sera were tested for anti-r-La aod anti-r-Ro antibodies by ELISA, and most sera were tested for antibodies directed against La polypeptides by immunoblot. We found significantly higher anti-rLa antibody levels in the sera from mothers of children with NLE compared with sera from mothers of unaffected children (067 i 0-43 versus 0-14 ± 0-30: P < 0 0001). There was a statistically significant difference in the mean anti-r-La levels between the sera of mothers of children with CHB compared with dermatologic NLE (0-51 ±0 45 versus 083 ±0 37 respectively; /'^O 0091). When we examined antibodies directed against the recombinant 52-kD Ro protein, there was a statistically significant elevation of titres in the sera of mothers of NLE children (0 77±0 35) compared with non-NLE mothers (0'29±0 39; /*

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Section: Discussionmentioning

confidence: 99%

Autoantibody response to the Ro/La particle may predict outcome in neonatal lupus erythematosus

Silverman

Buyon

Laxer

et al. 1995

Clinical and Experimental Immunology

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“…Both mothers and babies have high mortality in systemic lupus erythematosus (SLE) pregnancies (1). In our patient with positive lupus anticoagulant (LAC), the fetus had high risks of abortion or stillbirth.…”

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confidence: 81%

Successful Delivery in a Pregnant Woman with Lupus Anticoagulant Positive Systemic Lupus Erythematosus Treated with Double Filtration Plasmapheresis

Takeshita

Turumi

Touma

et al. 2001

Therapeutic Apheresis

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The case was a 29 year old female who has suffered from systemic lupus erythematosus (SLE) since 15 years of age. The activity of SLE was low, and she took prednisolone orally. Her first pregnancy failed after 14 weeks. In the second pregnancy, she had thrombocytopenia, prolonged activated partial thromboplastin time (APTT), positive lupus anticoagulant (LAC) and thus was diagnosed with antiphospholipid antibody syndrome (APS). Combination therapy with steroids and aspirin was started, and she underwent treatment of double filtration plasmapheresis (DFPP) in the early stage of pregnancy. Her platelet count increased, and the value of APTT has normalized with DFPP treatment. She delivered successfully on the 32nd week of pregnancy. We think that DFPP is an effective and safe treatment in patients with an LAC positive pregnancy.

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“…Congenital complete heart block (CHB) results most commonly from an autoimmune process resulting in destruction of atrioventricular nodal conduction as well as myocardial inflammation 41 42. In the presence of an adequate junctional or ventricular escape rate, patients may remain asymptomatic though bradycardiac at rest and exhibiting a blunted maximum exercise heart rate.…”

Section: Disease-specific Clinical Presentationmentioning

confidence: 99%

Sudden cardiac arrest in children and young athletes: the importance of a detailed personal and family history in the pre-participation evaluation

2008

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Healthcare providers have become more aware of and concerned about paediatric sudden cardiac arrest. The diseases predisposing a patient to sudden cardiac arrest are all infrequently encountered. However, a detailed and comprehensive patient and family history may reveal warning signs and symptoms that identify a patient at higher risk for sudden cardiac arrest. Since many of these diseases are genetic, extensive family evaluation may uncover a previously undetected cardiac disease process and as well direct the development of a complete family evaluation and treatment plan. Published data document that in many cases preceding warning symptoms and signs are present, but may be misinterpreted or disregarded by medical staff. Attention to the details of patient history, family history and physical exam is critical to the success of any detection strategy, which can and should be widely applied.

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Congenital complete heart block in the newborn associated with maternal systemic lupus erythematosus and other connective tissue disorders.

Cited by 80 publications

References 30 publications

Autoantibody response to the Ro/La particle may predict outcome in neonatal lupus erythematosus

Autoantibody response to the Ro/La particle may predict outcome in neonatal lupus erythematosus

Successful Delivery in a Pregnant Woman with Lupus Anticoagulant Positive Systemic Lupus Erythematosus Treated with Double Filtration Plasmapheresis

Sudden cardiac arrest in children and young athletes: the importance of a detailed personal and family history in the pre-participation evaluation

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