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)

In the US and northern Europe, the prevalence of pregnant syphilitic women is estimated at .1-.6%, while in South Africa it was 7.6% in 1982. In 1978, there 108 cases in the US which increased to 268 reported cases in 1985. The increase of congenital syphilis (CS) by 25% from 1985 to 1988 was attributed to the spread of crack cocaine in the US. The rate was 10.5 cases/100,000 live births in the US during this period, a 21% increase. In contrast, in the Netherlands there were 2.5 cases/100,000 live births during 1982-85. Clinical symptoms appear 3 weeks after birth, but some are present at birth such as hepatosplenomegaly, bloated abdomen, cutaneous lesions, and nasal discharge turning into purulent rhinitis. Anemia occurs in 90% of children with CS. Generalized lymphadenopathy, splenomegaly with hepatomegaly, and syphilitic hepatitis may also occur. Syphilitic skeletal abnormalities include osteochondritis, periostitis, osteomyelitis, and osteitis. Meningovascular syphilis produces nervous system effects. CS complications include nephrotic syndrome and acute glomerulonephritis. Ocular abnormalities are caused by treponemes found in the cornea, sclera, uvea, retina and the optic nerve. Chorioretinitis and iridocyclitis are common ocular lesions. The pathogen Treponema pallidum can be diagnosed by dark field microscopy, by immunofluorescence, or by histopathological examination of silver-stained preparations. Pregnancy women with syphilis are treated with penicillin although failures have been reported after single or 2 or 3 in administrations of 2.4 MU benzathine penicillin and after giving tetracycline in 3rd trimester pregnancy. The CDC recommendation for treating infants with CS is iv 50,000 U/kg penicillin G every 8-12 hours for 10-14 days or im 50,000 U procaine penicillin once daily for 10-14 days. Single administration of 50,000 U/kg benzathine penicillin is recommended for newborn children whose mothers have been treated with erythromycin.
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PMID:Congenital syphilis. 161 61

Two pregnant women with secondary syphilis underwent amniocentesis and evaluation for fetal syphilis. In both cases, motile spirochetes, typical of Treponema pallidum, were observed during dark-field microscopic examination of the amniotic fluid. The presence of T pallidum was confirmed by antitreponemal monoclonal antibody immunofluorescence assays and by rabbit infectivity tests using the amniotic fluid. In the first case, an infant at 35 weeks' gestation delivered within 24 hours of amniocentesis had hepatosplenomegaly, osteochondritis, and neurosyphilis. In the second case, a fetus at 24 weeks' gestation was hydropic and a fetal blood sample showed anemia, thrombocytopenia, and elevated liver enzymes. Fetal syphilis was confirmed by rabbit infectivity testing using fetal blood obtained by funipuncture. This is the first report of the diagnosis of fetal syphilis by funipuncture and confirmation of the presence of virulent T pallidum in the blood of a human fetus. The mother was treated for secondary syphilis, but the infant had residual signs of congenital infection at birth 14 weeks later. Neonatal serum from the first case and fetal serum from the second case showed specific immunoglobulin M reactivity with the 47-kd antigen of T pallidum by Western blot assays. A new wild-type strain of T pallidum, designated DAL-1, was isolated from the amniotic fluid of the first case and is available for future studies. We conclude that the presence of T pallidum in amniotic fluid or fetal blood indicates fetal-placental infection. Further investigation is necessary to determine the pathogenesis of amniotic fluid infection and its role in the prenatal diagnosis of congenital syphilis.
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PMID:Identification of Treponema pallidum in amniotic fluid and fetal blood from pregnancies complicated by congenital syphilis. 192 18

Seven cases of early congenital syphilis have been recorded in the past 10 years in the Mersey Regional Health Authority. Antenatal serology was initially negative in five mothers, who were either incubating or acquired the infection later, and treatment had probably failed in two women given erythromycin for syphilis during pregnancy. Serology should be repeated later in pregnancy in those at high risk. Social factors that define this group include women who book for antenatal care late in pregnancy, have a past history of sexually transmitted disease, and have multiple consorts. Clinical signs in the infant such as failure to thrive, hepatosplenomegaly, symmetrical rash, rhinitis, and osteochondritis should alert the clinician to the possibility of congenital syphilis. Adequate management of mother and baby requires close liaison between the genitourinary physician, microbiologist, obstetrician, and paediatrician. Penicillin remains the treatment of choice.
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PMID:Early congenital syphilis still occurs. 384 73

Two infants, a two-month-old boy and a two-month-old girl adopted from Sri Lanka, were diagnosed as having congenital syphilis and treated accordingly. The girl presented with only skin symptoms and a developing pseudoparalysis of Parrot. The boy was in quite a bad condition, having rhinitis, an oedematous appearance, skin symptoms, severe anaemia and hepatosplenomegaly. In both patients serological blood tests and cerebrospinal fluid tests for lues were positive. Both children showed osteochondritis and periostitis of the long bones on X-rays. This disease is rare in the Netherlands, but it is important to diagnose it early, because early adequate treatment may result in complete cure.
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PMID:[Congenital syphilis as an imported disease]. 892 60

A preterm, very low birth weight infant was born to a mother with early latent syphilis who was treated 10 days and 3 days before delivery with 2.4 mU of benzathine penicillin. The infant had clinical, laboratory, and radiographic abnormalities consistent with congenital syphilis, ie, a Venereal Disease Research Laboratory test titer that was fourfold greater than was the maternal titer, hepatosplenomegaly, abnormal liver function tests, pneumonitis, osteochondritis of the long bones, and cerebrospinal fluid (CSF) examination showing a reactive Venereal Disease Research Laboratory test, pleocytosis, and elevated protein content. The infant died on the third day of life, and an autopsy revealed an evolving gumma of the anterior pituitary. Immunoglobulin M immunoblotting of serum and CSF was positive, and polymerase chain reaction detected Treponema pallidum DNA in endotracheal aspirate and CSF. This case highlights the pathologic abnormalities observed in congenital syphilis and focuses on the rare finding of an evolving anterior pituitary gumma. Furthermore, it documents the failure of maternal syphilis treatment during the last 4 weeks of pregnancy to cure fetal infection and supports the recommendation that all infants born to mothers with syphilis treated during the last 4 weeks of pregnancy should receive penicillin therapy.
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PMID:Pituitary gland gumma in congenital syphilis after failed maternal treatment: a case report. 1039 Feb 90