Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Burkitt's lymphoma presented atypically in a six-year-old Nigerian girl with back pain, oliguria and facial oedema following a fall at school. Two weeks later, she developed bilateral ptosis, hepatomegaly and ascites. Burkitt's lymphoma cells were found in both ascitic and cerebrospinal fluids. She was successfully treated with intravenous cyclophosphamide and intrathecal methotrexate but later developed fatal herpes zoster at the same time as the resident doctor developed chicken pox. Chart's review showed that she had been in brief contact with chicken pox during a short stay in a transit ward prior to full admission.
 ...
PMID:Fatal herpes zoster in Burkitt's lymphoma following contact with chicken pox. 59 65

The authors report a case of chickenpox exposure during the first three months of pregnancy. Ultrasonographic monitoring of the fetus revealed signs of fetal infection starting from 32 weeks of amenorrhea (hepatomegaly, ascites, pleural effusion) but clinical examination of the child at birth showed nothing abnormal. The diagnosis was confirmed by the development of herpes zoster in the child after birth. The difficulty of determining a fetal prognosis during pregnancy is emphasised.
 ...
PMID:[Chickenpox and pregnancy]. 147 Aug 22

Glycyrrhizin (GL) has an inhibitory effect on several viruses including human immunodeficiency virus type 1 (HIV-1) and varicella-zoster virus (VZV). In addition, some therapeutic and prophylactic effects on chronic active viral hepatitis have been claimed for GL. In this study, 0.2% GL dissolved in saline (2 mg/ml GL), supplemented with 2% glycine and 0.1% cysteine (Stronger Neo-Minophagen C, SNMC) was administered intravenously in a dose of 50 ml/day for a period of more than one week to three infants with cytomegalovirus (CMV) infection who exhibited abnormal liver function or hepatomegaly. Liver function had become normal at the end of the course of SNMC. These findings suggest that GL might have therapeutic effects on liver dysfunction associated with CMV infections.
 ...
PMID:Effect of glycyrrhizin in children with liver dysfunction associated with cytomegalovirus infection. 807 26

This report describes the clinical spectrum and outcome of the hemophagocytic syndrome (HS) in 5 HIV infected patients. All 5 patients presented with fever, hepatomegaly and/or splenomegaly, confusion or coma and respiratory symptoms. Severe anemia was associated with thrombocytopenia and with neutropenia in 4 cases. Diffuse intravascular coagulopathy was present in 2 cases. Liver function tests were abnormal in three patients. The diagnosis of HS was made 2 to 12 weeks after the onset of symptoms and required in most patients repeated examinations of the bone-marrow, showing infiltration by histiocytes with prominent phagocytosis of blood cells. In one case this infiltration was not seen in the bone-marrow but only in the liver and the spleen. Varicella, mycobacterium infection, oesophageal candidiasis, Kaposi sarcoma were observed in the evolution of 3 patients. Anaplastic large cell Ki-1 lymphoma was present in one case. Four patients died as a result of complications of HS. The one patient with lymphoma survived.
 ...
PMID:[Hemophagocytic syndrome in HIV infection]. 824 41

We describe the history of a girl with interferon-gamma-receptor (IFNgammaR1) deficiency and studies performed to identify the molecular and clinical characteristics of this recently discovered disorder. This is the first report of a child from Northern Europe with IFNgammaR1 deficiency. The patient, now 7 years old, first presented with swelling and reddening at the Bacille Calmette-Guerin (BCG) vaccination site, swelling of lymph nodes, hepatomegaly, and an unusually severe varicella rash at the age of 4 months. At that time, she was diagnosed with BCG histiocytosis without typical granuloma formation and was treated with antituberculous agents. During the clinical course of her illness, several different types of atypical mycobacteria and (for the first time in an IFNgammaR1-deficient patient) Listeria monocytogenes were detected. Flow cytometric analysis showed that the patient's monocytes could not bind a monoclonal antibody specific for the IFNgamma-receptor. Our analysis of mRNA derived from the alpha-chain (IFNgammaR1) gene of this receptor revealed deletions of 173 bp and 4 bp in cDNA sequences originating from individual alleles. The 173 bp deletion was located between nucleotide positions 200 and 372, exactly matching those of exon 3, and the 4 bp deletion was located between nucleotide positions 561 and 564 of the coding region of the cDNA. Analysis of genomic DNA revealed the presence of a G to T transition at the 5'end of the splice consensus sequence of intron 3, which explains the absence of exon 3. The other allele carried the 4-base-pair deletion (ACTC) at nucleotide positions 15-18 of exon 5. Twelve months after an allo\geneic bone marrow transplantation, the patient had clinically improved.
 ...
PMID:Listeria monocytogenes and recurrent mycobacterial infections in a child with complete interferon-gamma-receptor (IFNgammaR1) deficiency: mutational analysis and evaluation of therapeutic options. 1048 Apr 27

We experienced a case of virus-associated hemophagocytic syndrome (VAHS) after varicella-zoster virus (VZV) infection. The patient, a 101-year-old man, presented with anemia, thrombocytopenia and jaundice two weeks after successful antiviral treatment for the VZV. Histiocytes were detected in the bone marrow examination (2.2%); however, hepatomegaly and triglycemia remained unobserved throughout the course. Reactivation of VZV was detected serologically. The patient died after five weeks because of persistent cytopenia and liver failure refractory to steroid treatment. An autopsy revealed hemophagocytosis in the bone marrow, lung, spleen and liver.
 ...
PMID:[Elderly patient with varicella-zoster virus-associated hemophagocytic syndrome refractory to steroid therapy]. 1644 Aug 10

A 31-year-old woman contracted acute varicella at 13 weeks of gestation. Severe hydrops fetalis, hepatomegaly, and intrauterine fetal death were detected at 16 weeks of gestation by ultrasound examinations. An examination at autopsy, histopathology, and polymerase chain reaction (PCR) provided evidence of varicella-zoster virus (VZV) infection of the fetus. Second trimester intrauterine fetal death caused by mother to fetus infection of VZV is extremely rare.
 ...
PMID:Second trimester fetal death caused by varicella-zoster virus infection. 2336 80