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1/3. Improved dysgammaglobulinaemia in congenital rubella syndrome after immunoglobulin therapy: correlation with CD154 expression.

    A boy with congenital rubella syndrome developed dysgammaglobulinaemia with elevated serum levels of IgM. CD154 was not induced on his peripheral blood mononuclear cells when rubella virus rna was detected in his throat swabs and peripheral blood by reverse transcriptase polymerase chain reaction. Following intravenous immunoglobulin therapy, improvement of immunoglobulin abnormalities, disappearance of rubella virus and normalisation of CD154 expression were demonstrated. CONCLUSION: These findings implicate the efficacy of intravenous immunoglobulin therapy for dysgammaglobulinaemia in congenital rubella syndrome and a role of CD154 for a prolonged virus infection.
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2/3. Duodenal stenosis, a new finding on congenital rubella syndrome: case description and literature review.

    Congenital rubella syndrome (CRS) continues to represent a public healthcare problem although an effective vaccination program. Gastrointestinal involvement is rather infrequent and the association of CRS with duodenal stenosis has been never reported. In this study a case of CRS with duodenal diaphragm is reported and the gastrointestinal diseases described in association with CRS are reviewed. A 10-month-old child affected by CRS with congenital hearth disease, perceptive deafness and microcephaly, was admitted because of vomiting and failure to thrive. An upper endoscopy demonstrated dilated proximal duodenum and a perforated diaphragm in the second segment of the duodenum. Endoscopic membranectomy was therefore performed. Two months later the patient was submitted to a further endoscopic evaluation that showed a partial diaphragm persistence and a second excision was performed. Follow-up one year after the first treatment showed good clinical conditions, reasonable physical growth and disappearance of vomiting. In conclusion we report the first case of CRS in association with duodenal stenosis. Duodenal stenosis in the absence of other intestinal localizations may be due to rubella capacity of infecting only small numbers of fetal cells but we cannot exclude that the duodenal stenosis in our patient be only a casual association.
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3/3. Congenital rubella syndrome with rubella virus-associated generalized brownish macules, indurated erythemas, papules, and pigmentation.

    BACKGROUND: We examined an infant with congenital rubella syndrome (CRS). The purpose of this report is to describe the skin manifestations in this patient and to prove that they were associated with rubella virus. OBSERVATIONS: A 7-month-old boy presented with generalized brownish macules, indurated erythemas, papules, and pigmentation. They first appeared at around 3 months of age. His mother had contracted rubella during the 14th gestational week. At the time of examination, rubella-specific IgM antibody was positive in both serum and cerebrospinal fluid of the baby. A physical exam had revealed deafness, mental and physical retardation, interstitial pneumonitis, and hepatosplenomegaly. A skin biopsy specimen showed a dense infiltration mainly of lymphocytes, with B cells predominant in the deep dermis. Electron microscopically abundant tubuloreticular structures were observed in capillary endothelial cells, lymphocytes, and dermal fibroblasts. Polymerase chain reaction (PCR) analysis suggested that rubella virus rna was present in the patient's skin specimen, cerebrospinal fluid, and total blood. CONCLUSIONS: The cutaneous manifestations of our patient were extraordinary and informative. These prominent skin lesions should be recognized as cutaneous markers of CRS.
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