• Clinical and Experimental Pediatrics
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• v.48 no.3
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• pp.342-345
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• 2005

Neonatal lupus erythematosus(NLE) is a distinct subset of lupus characterized by cutaneous findings, cardiac conduction defects, hepatic or hematologic abnormalities. These manifestations are associated with the presence of maternal auto-antibodies such as anti-SSA/Ro, anti-SSB/La, and rarely anti-RNP($U_1RNP$) antibodies. Cases of $U_1RNP$ antibody-positive NLE have somewhat atypical cutaneous manifestation without cardiac or systemic abnormalities. We report a case of cutaneous NLE associated with $U_1RNP$ antibodies.

• Neonatal Medicine
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• v.18 no.2
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• pp.391-394
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• 2011

Neonatal lupus erythematosus (NLE) is a rare disease characterized by typical clinical features and the transplacental passage of maternal autoantibodies, particularly anti-SSA/Ro. The major clinical manifestations are cutaneous lupus lesions, congenital heart block, hematological disorders, and hepatobiliary diseases. We report a case of NLE presenting with multiple round and oval target-like erythematous skin lesions and abnormal liver function, born to a clinically asymptomatic mother whose diagnosis was made retrospectively only after her newborn's diagnosis. Both the infant and the mother were positive for the anti-SSA/Ro and anti-SSB/La antibodies.

• Tuberculosis and Respiratory Diseases
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• v.52 no.2
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• pp.179-185
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• 2002

A bronchus-associated lymphoid tissue(BALT) lymphoma of the lung is a rare disorder of patients with Sj$\ddot{o}$gren's syndrome. A 49-year-old woman was admitted for an evaluation of exertional dyspnea and general weakness which had persisted for two years. The patient had suffered from dry mouth and dry eyes for five years. The physical examinations showed a coarse breath sound with inspiratory crackles on the whole lung field, particularly on the both basal lungs. The laboratory data disclosed high titers of anti-nuclear antibodies, and anti-SSA (Ro), and anti-SSB (La) antibodies. Chest radiographs demonstrated the presence of bilateral, diffuse, reticulonodular densities in both lungs. Thin-section CT scans showed diffusely distributed mosaic pattern of an inhomogeneous attenuation extending over the entire lung zone. The histological findings from an open-lung biopsy specimen revealed an accumulation of lymphoid cells around the bronchioles and an extension of malignant lymphoma cells from the bronchiolar epithelium toward the alveolar space. Immunohistochemically, the neoplastic cells reacted positively to the CD 20 antigen and were focally positive for the UCHL 1 antigen. The histological diagnosis was consistent with a low grade marginal zone B-cell lymphoma originating in the BALT. Here, we present a case of a histologically proven BALT lymphoma of the lung in a patient with primary Sj$\ddot{o}$gren's Syndrome.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.381-387
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• 2006

Purpose : Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. Methods : The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. Results : There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). Conclusion : The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.