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)

Ganciclovir has been shown to be effective against cytomegalovirus (CMV) in vitro, but its role in the treatment of congenital cytomegalovirus infection is unknown. We describe its use in the management of two cases of congenital CMV infections. The drug was well tolerated and virus shedding in the urine was eliminated, though hepatosplenomegaly and neurodevelopmental delay persisted in both cases.
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PMID:Ganciclovir treatment of congenital cytomegalovirus infection: a report of two cases. 839 4

Approximately 0.5-1% of all newborns are born infected with cytomegalovirus (CMV), but of these only one out of ten show symptoms at birth, most often with hepatosplenomegaly, thrombocytopenia, and/or brain affection. Of the remaining nine, one may later develop sequelae with hearing loss and/or mental retardation. CMV infection may also be acquired perinatally or in the newborn period, and may cause pneumonia and/or sepsis, possibly also gastrointestinal symptoms like blood in the stool, and poor weight-gain. We have diagnosed CMV infection in ten neonates and infants, and describe these patients in terms of symptoms, diagnosis and treatment. Ganciclovir is being tested in clinical trials as a treatment for congenital CMV infection, and was given to two of our patients with apparently good results.
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PMID:[Cytomegalovirus infection in neonates. Diagnosis and therapeutic experiences]. 919 23

This case report is about an elderly man who presented with a long-standing history of high-grade fever and weight loss. He initially had only hepatosplenomegaly, but then developed jaundice. He also had pancytopenia and raised liver enzymes. His septic screen was negative, but he had a positive Monospot test and immunoglobulin G for Epstein-Barr virus. The liver biopsy showed sinusoidal phagocytosis and the subsequent bone marrow aspiration and biopsy showed significant hemophagocytosis, hence Hemophagocytic syndrome was diagnosed. The fever was refractory to antibiotic and anti-tuberculosis therapy, but it responded only partially to steroids. Full response was only noticed following anti-viral treatment in the form of intravenous Ganciclovir. The patient's general condition, liver enzymes, bilirubin, hematological parameters and even the weight returned back to their normal range 2 weeks after Ganciclovir therapy. Cessation of this drug resulted in relapse of his symptoms and oral antivirals did not help. Splenectomy, steroid pulse therapy and immunosuppressive treatment were only partially helpful. Reintroduction of Ganciclovir did help for a short period. We conclude that our patient had virus-associated hemophagocytic syndrome most likely related to Epstein-Barr virus infection, which was then confirmed by the splenic biopsy, and that Ganciclovir can be of great help in eradicating the virus and treating the disease, provided that it is given for a long enough period.
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PMID:Hemophagocytic syndrome. 1136 69

Neonatal cytomegalovirus (CMV) infection is common, has myriad presentations and severe sequelae. Six neonates clinically suspected of CMV infection were confirmed by qualitative PCR (Digene) and evaluated. Those with persistent viremia were treated with Ganciclovir intravenously for 4-6 weeks, and continued orally, if required, with close monitoring. All had prolonged jaundice, hepatosplenomegaly and hematological manifestations in the acute stage. Complications included developmental delay (66%), sensorineural hearing loss (SNHL) (33%), chorioretinitis and obstructive jaundice (18% each). Three cleared viremia spontaneously. The remaining were offered Ganciclovir. One declined, and two completed therapy with clinical resolution and no adverse events. Accurate diagnosis of neonatal CMV enables appropriate treatment with Ganciclovir, which can reverse end-organ damage and limit sequelae.
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PMID:Cytomegalovirus infection in six neonates. 1943 62

Hepatic angiosarcomas are uncommon, highly aggressive tumors, rarely seen in children. A 3-month-old female infant was admitted to hospital for evaluation of multiple petechiae on her body. She had hepatosplenomegaly and scattered petechiae over her entire body. Laboratory tests indicated thrombocytopenia and positive cytomegalovirus (CMV) polymerase chain reaction. Ganciclovir was started, and the platelet count increased. After 4 months the patient was readmitted to hospital for drowsy mental status and eventually died from severe bleeding. Needle biopsy of the liver was performed after receiving written consent from the parents. Pathological findings of the liver lesion included features consistent with hepatic angiosarcoma. There have been no previous reports of hepatic angiosarcoma in Korean infants. Here, we report an infant with hepatosplenomegaly and thrombocytopenia who was diagnosed with hepatic angiosarcoma mimicking congenital CMV infection.
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PMID:Hepatic angiosarcoma mimicking congenital cytomegalovirus infection in an infant with thrombocytopenia. 2571 70