October 1993

Neonatal Hemochromatosis Associated With Maternal Autoantibodies Against Ro/SS-A and La/SS-B Ribonucleoproteins

Author Affiliations

From the Institut fuer Pathologie "Georg Schmorl" (Dr Schoenlebe), Staedtisches Klinikum, Dresden, Germany; the Department of Medicine (Dr Buyon), Division of Rheumatology, and the Department of Pathology (Dr Greco), New York University Medical Center, the Department of Rheumatic Diseases and Molecular Medicine (Dr Buyon), The Hospital for Joint Diseases, the Department of Pediatrics (Dr Friedman), Division of Pediatric Cardiology, New York University School of Medicine, and The Rita and Stanley Kaplan Cancer Center (Dr Greco), New York, NY; and the Departments of Pediatrics (Dr Zitelli) and Pathology (Dr Knisely), Children's Hospital of Pittsburgh (Pa) and University of Pittsburgh.

Am J Dis Child. 1993;147(10):1072-1075. doi:10.1001/archpedi.1993.02160340058014

• Liver disease in the fetus and neonate may be associated with maternal Sjögren's syndrome (neonatal lupus erythematosus). The infant of a mother with Sjögren's syndrome and high anti-Ro/SS-A and anti-La/SS-B antibody titers presented at 2½ weeks of age with overwhelming hepatic insufficiency and died at 6 weeks of age. Autopsy confirmed the antemortem diagnosis of neonatal hemochromatosis. We discuss the possibility of a relationship between these two conditions.

(AJDC. 1993;147:1072-1075)