Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
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The causes of all perinatal deaths at Mpilo Maternity Hospital were investigated over a 12-month period, during which there were a total of 466 stillbirths and 379 neonatal deaths, with a perinatal mortality rate of 36.0/1000 births in Bulawayo, Zimbabwe. The causes of death were in order of importance; congenital syphilis (20.5 pc), birth asphyxia (18.8 pc), unexplained stillbirths (11.8 pc), hyaline membrane disease (11.5 pc) neonatal septicaemia (10.8 pc), congenital malformations (7.7 pc), pregnancy induced hypertension (5.4 pc), placental abruption (4.9 pc), congenital infection (2.2 pc) and other causes (6.4 pc). Eleven pc of mothers booking in antenatal clinics had positive syphilis serology. Most were successfully treated. But over 400 mothers with early syphilis escaped treatment usually because they booked late or failed to book at all at antenatal clinics (74 pc) and occasionally because they had false negative results or were infected after early booking (27 pc). They delivered 101 stillbirths, most of whom died prematurely before labour and often had abdominal distension. There were 72 neonatal deaths, most of whom were preterm babies with respiratory distress and often hepatosplenomegaly. One half of the deaths from asphyxia were caused by prolonged obstructed labour and one quarter by prolapsed cord, stuck head in breech delivery and retained second twin. The incidence of both early and late onset neonatal septicaemia was very high with Group B Streptococci, Kliebsiella and Staphylococcus aureus the predominant pathogens. Improved antenatal, intrapartum and neonatal care could substantially reduce the perinatal mortality rate by preventing congenital syphilis and birth asphyxia and by treating hyaline membrane disease and neonatal septicaemia.
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PMID:The causes of perinatal mortality in Bulawayo, Zimbabwe. 147 75

Sexually transmitted diseases (STDs) in children may be transmitted by sexual abuse, by accidental contact, or perinatally. Although only 2% to 10% of abused children become infected, childhood syphilis, gonorrhea, condylomata acuminata, and Chlamydia trachomatis must always be considered. We reviewed data from our hospital regarding the frequency, prevalence, routes of transmission, and clinical features of these infections. Ninety-five percent of acquired syphilis in children is transmitted by sexual abuse. The perpetrator is usually someone the child knows or trusts. Of our 21 patients under 14 years of age with acquired syphilis, most were 4 to 8 years old. Girls were infected twice as often as boys. Sexual contact was confirmed in 71.4%. A chancre sore was infrequent in children; condylomata lata was the most frequent cutaneous lesion (80.9%). In the last 10 years, 102 cases of congenital syphilis were diagnosed in our hospital. The main clinical findings were bone involvement (78.7%), hepatosplenomegaly (68.8%), cutaneous lesions (50.8%), and jaundice (15.1%). Gonorrhea was detected in only nine children. Vulvovaginitis was the most common clinical manifestation. Sexual transmission was documented in three patients. Accidental contact with their infected mother occurred in two sisters. Three newborns acquired the disease during delivery. The STDs in children are a worrisome problem. Evaluation for sexual abuse should be done in all cases. Prevention and treatment of adults are the main steps to prevent these infections in children.
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PMID:Sexually transmitted diseases in children. 149 47

As part of a survey of the causes of perinatal mortality at Mpilo Maternity Hospital, 220 neonatal deaths and the mothers of 221 stillbirths were tested for HIV-1 antibodies. The HIV positive rate in neonatal deaths was 23.6% (95% confidence interval (CI) 18.0 to 29.2%), significantly higher than 15.4% (95% CI 10.6 to 20.1%) in stillbirths. Perinatal deaths from congenital malformations, birth asphyxia, pregnancy induced hypertension, placental abruption, and oFther non-infectious causes had similar low HIV positive rates averaging 8.1% (95% CI 3.9 to 12.3%). Deaths from septicaemia had a significantly greater rate of 39.3% (95% CI 27.0 to 51.6%) and the highest rate of 72.2% (95% CI 51.5 to 92.9%) was found in deaths from congenital infection other than syphilis, indicating that maternal HIV infection predisposes to neonatal septicaemia and congenital infection. Unexplained stillbirths also had a significantly greater rate of 22.4% (95% CI 10.7 to 34.1%), presumably because some died from unrecognised infection. The rate in deaths from congenital syphilis was 17.4% (95% CI 9.6 to 25.2%), indicating a significant but weak association between these two sexually transmitted diseases in Bulawayo. The rate in deaths from hyaline membrane disease was not significantly greater at 15.0% (95% CI 6.0 to 24.0%). By predisposing to infection, maternal HIV infection was estimated to increase the stillbirth rate by 1.6 times and the neonatal mortality rate by 2.7 times. It predisposed equally to early and late onset neonatal septicaemia, but more to infection from streptococci and staphylococci than from Gram negative enterobacteria. HIV positive deaths from congenital infection had respiratory distress and usually intrauterine growth retardation, hepatosplenomegaly, and congenital pneumonia on lung histology.
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PMID:HIV-1 infection and perinatal mortality in Zimbabwe. 159 95

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

Sonography of a fetus, later proved to have congenital syphilis, revealed hepatosplenomegaly, noncontinuous gastrointestinal tract obstruction, and placentomegaly. This unusual constellation of sonographic findings should raise suspicion of syphilis infection of the fetus.
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PMID:An unusual constellation of sonographic findings associated with congenital syphilis. 192 19

A baby with neonatal congenital syphilis who was born prematurely at 33-weeks' gestation with hepatosplenomegaly, ascites and respiratory distress that was evident at birth is presented.
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PMID:Congenital syphilis is with us still. 259 44

The first case of nonsyndromatic paucity of the intrahepatic bile ducts is reported in congenital syphilis. The patient, a 2-week-old female, was born at the 31st week of gestation, weighing 1,910 g. She had a high titer of IgM antibody to Treponema pallidum and sera from both parents also showed a positive reaction in the hemagglutination test for Treponema pallidum. The patient had hepatosplenomegaly and increasing jaundice, and died of respiratory failure on the 70th hospital day. Autopsy examination revealed paucity of the intrahepatic bile ducts, prominent giant cell transformation of hepatocytes, cholestasis and extramedullary hematopoiesis of the liver. The ratio of the number of intrahepatic bile ducts to that of the portal tracts was approximately 0.2:1. There was marked proliferation of atypical bile ductules at the margin of the portal tracts. The exact relation of this paucity to Treponema pallidum remains unknown.
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PMID:Nonsyndromatic paucity of intrahepatic bile ducts in congenital syphilis. A case report. 318 14

We studied 53 newborn babies with congenital syphilis. The common clinical features seen were low birth weight, hepatosplenomegaly, anaemia, jaundice, and symmetrical superficial desquamation of the skin affecting palms and soles. The presence of these clinical signs is highly suggestive of early congenital syphilis. Hydrops fetalis without rhesus or ABO isoimmunisation should always arouse the suspicion of congenital syphilis.
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PMID:Congenital syphilis in the newborn. 320 51

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


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