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Congenital Syphilis – in Theory and in Reality in Estonia, 1991-2005

Congenital Syphilis – in Theory and in Reality in Estonia, 1991-2005. Liis Toome Tallinn Children’s Hospital 15.09.2006. Incidence of S yphilis in 2001 per 100 000 P opulation. Estonia 2005 – 8,2/100 000. EpiNorth Journal 2002, No 4.

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Congenital Syphilis – in Theory and in Reality in Estonia, 1991-2005

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  1. Congenital Syphilis –in Theory and in Reality in Estonia, 1991-2005 Liis Toome Tallinn Children’s Hospital 15.09.2006

  2. Incidence of Syphilis in 2001 per 100 000 Population Estonia 2005 – 8,2/100 000 EpiNorth Journal 2002, No 4

  3. Congenital Syphilis in Estonia since 1991 • Aim of the study: • to describe the epidemiology of congenital syphilis in Estonia, 1991-2001(-2005) • Methods: • retrospective analysis of the cases of CS in children aged < 2 years • T. Rjabova, L. Toome • Tallinn Children’s Hospital • K. Kink • West Tallinn Central Hospital • E. Tamm • Children’s Clinic, Tartu University Hospitals • A. Kangur • North Estonia Regional Hospital

  4. Syphilis in Estonia, 1971 - 2001 Number of cases Health Protection Inspectorate, 2006

  5. The Incidence of Congenital Syphilisin Estonia, 1982 - 2005 0 - 14 years < 1 year

  6. Microbiology • T. Pallidum • A tightly coiled motile spirochete • Can be detected on dark-field microscopy • Has not been cultured in vitro Treponema Pallidum Treponema Pallidum on darkfield

  7. Transmission Sheffield JS et al, Am J Obstet Gynecol, 1999

  8. Early Congenital Syphilis -Clinical Manifestations • IUGR • Nonimmune hydrops fetalis • Enlarged placenta • Mucocutaneous manifestations • Persistent rhinitis (snuffles) • Maculopapular eruption • Superficial desquamation • Pemphigus syphiliticus • Condylomata lata

  9. Early Congenital Syphilis -Clinical Manifestations • Jaundice, hepatosplenomegaly • syphilitic hepatitis • Generalized lymphadenopathy • Hematologic manifestations • hemolytic anemia, thrombocytopenia • Bone lesions • osteochondritis, -myelitis, periostitis • pseudoparalysis of Parrot • Pneumonitis, nephrotic syndrome • Syphilitic leptomeningitis • Ocular manifestations • chorioretinitis, glaucoma, cataract Pneumonia alba Metaphyseal dystrophy Wimberger’s sign

  10. Serologic Diagnosis in the Infant

  11. Congenital Syphilis in Estonia, 1991-2005 n = 28 Tallinn - 12 cases, Harjumaa - 7 cases, Lääne-Virumaa - 5 cases

  12. Children’s Age at the Time of Diagnosisin Estonia, 1991-2005 (n = 28)

  13. Clinical Manifestations of Congenital Syphilis in Estonia, 1991-2005 (n = 28)

  14. Case 1 Newborn, syphilitic hepatitis • GA 36, BW 2529 g • jaundice from the birth • syphilitic hepatitis • hepatosplenomegaly • indirect bilirubin 245 mol/l • direct bilirubin 187 mol/l • elevated serum aminotransferases • Anemia, thrombocytopenia • Cardiolipin ag 4+ Treponemal ag 4+

  15. Case 21 month 2 weeks, pseudoparalysis of Parrot Bone lesions with superimposed fractures • BW 2900 g • 1 month • unexplained rhinitis • anemia Hgb 72 g/l, ER 2,5x1012 • CRP 187 mg/l • pneumonia? • Jarisch-Herxheimer reaction • Pseudoparalysis of Parrot • Serology • RPR 1 : 40 • TPHA 1 : 2560 Wimberger sign

  16. Case 31 month 3 weeks, syphilitic glomerulonephritis • maculopapular rash, rhinitis, abdominal distension • “snuffles”, syphilitic ileitis • rectal bleeding • syphilitic glomerulonephritis with nephrotic syndrome • generalized edema + ascitis • 5166 g  4154 g • macrohematuria • severe proteinuria • hepatosplenomegaly • panmetaphysitis • RPR 1 : 240, TPHA 1 : 640

  17. Case 42 months, “asymptomatic” • BW 3250 g • Incarcerated inguinal hernia • Anemia  Hgb 77 g/l, ER 2,4 x 1012 • Maculopapular eruption of the palms and soles, becoming coppery-brown • Hepato (+ 3,5 cm) spleno (+ 1,0 cm) megaly • Fever 38º • Jarisch-Herxheimer reaction, tº 40 º C • Serology • Cardiolipin ag 4+ • Treponemal ag 4+

  18. Case 56 months, Jarisch-Herxheimer Reaction • Term delivery, BW 2660 g • Persistent rhinitis • Mucocutaneous manifestations • Maculopapular eruption treated as allergic dermatitis and as scabies • Deep fissures radial to the angles of the mouth  rhagades • Hepatosplenomegaly, osteochondritis • SR 70 mm/h, Hgb 83 g/l • Jarisch-Herxheimer reaction • t° 39° C in 2 hours of treatment • RPR 1:640

  19. Case 61 year 1 month, manifestations of CNS • BW 2731 g, parenteral abuse of alcohol • During the first year of life • Failure to thrive • Maculopapular rash - atopic dermatitis, scabies? • Anemia, hepatomegaly • 1 year 1 month – 7,1 kg / 71 cm / OFC 44 cm • Mental retardation • Optic nerve atrophy • Brain CT – cortical atrophy • Serologic diagnosis • RPR 1 : 128 • WB IgG positive • CSF FTA-Abs 2+

  20. Treatment of the Newborn Remington & Klein, 2006

  21. Post-treatment Follow-up Rathbun KC, Sex Transm Dis 10:102, 1983

  22. Late Congenital Syphilis - after the first two years of the life • Dentition • Hutchinson’s teeth • Eye • interstitial keratitis • Ear • eighth nerve deafness • Skin, face • rhagades, “saddle nose” • CNS • mental retardation, HC • Bones and joints • “saber shins”, “Clutton’s joints” Hutchinson’s teeth “Saber shins” “Clutton’s joints” Remington, 2006

  23. Prevention • Congenital syphilis is a preventable disease! • At least one serologic test for syphilis during the first trimester • For communities with high prevalence of syphilis • repeated testing at the beginning of the third trimester • at delivery (not in infants) • Adequate treatment of infants in utero or at birth with subsequent follow-up

  24. Conclusions from Estonian Experience • Political and social changes in the beginning of independent Estonian Republic brought about • the increase of incidence of syphilis • the cases of congenital syphilis • After 15 years congenital syphilis is a disappearing disease thanks to • the decreased incidence of syphilis in the population • The increased awareness of the importance of adequate prevention of transmission of the disease to the fetus and the newborn

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