Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Congenital cutaneous candidiasis (CCC) is a rare disease acquired by an ascending route, liable to affect the offspring of pregnant women suffering from vulvovaginitis. The cutaneous lesions are present at birth or within the first hours of life. Some infants may present with respiratory distress or clinical signs of sepsis during the first 2 days of life. We report four new cases of CCC, three of which presented transient respiratory distress and clinical signs of sepsis with hepatosplenomegaly. The evolution was favourable in all three cases with topical and oral therapy. We emphasize the self-limited character of this disease, although preterm infants may be at risk of systemic spread. Only one infant presented paronychia as a late complication.
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PMID:Congenital cutaneous candidiasis: report of four cases and review of the literature. 204 6

Lymphoproliferative disorders (LPD) occur often in EBV-infected patients, especially in solid-organ and haematopoietic stem cell transplant recipients. The risk of developing LPD ranges from 1 to 25% and depends on the type of transplantation. We are presenting the case of a 9-year-old boy with acute myelogenous leukaemia in second remission, who developed LPD after matched unrelated donor bone marrow transplantation (MUD BMT) not identical in two loci. On day 50 after BMT the patient presented with fever and symptoms of paronychia. Two weeks later, bilateral cervical tenderness and adenopathy and hepatosplenomegaly developed. Bone marrow biopsy confirmed continuing remission. An extensive infection workup, including bacterial, mycotic and CMV infection yielded negative results. Basing on clinical picture and suspecting LPD, EBV-PCR was performed. The patient was found to have extremely high EBV DNA levels (4.905.152 genomes/mcg) in the peripheral blood. On days 64 and 73 after BMT, the patient received two doses of rituximab (MabThera) (375 mg/m(2)) After the first dose of rituximab EBV DNA copy numbers decreased to 707.723/mcg. However the patient's general condition was worsening; 71 days after BMT increasing aplasia and symptoms of venoocclusive disease (VOD) developed. The patient received two doses of defibrotide (Novarid). Despite of intensive therapy, progressive hepatic failure and increasing pulmonary oedema led to the patient's death, on day 96 after BMT.
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PMID:[Lymphoproliferative disorder as a complication after haematopoietic stem cell transplantation]. 1953 35