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Hydrops fetalis Fósturbjúgur

Hydrops fetalis Fósturbjúgur. Helga Björk Pálsdóttir. Sagan. Hydrops fetalis verið lýst í gegnum aldirnar Um 400 f.Kr lýst af Hippokrates 1943 skilgreindi Potter 2 flokka af hydrops fetalis- immune og non-immune 1969 Rhesus varnir hefjast á Íslandi. Hydrops fetalis Skilgreining.

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Hydrops fetalis Fósturbjúgur

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  1. Hydrops fetalisFósturbjúgur Helga Björk Pálsdóttir

  2. Sagan • Hydrops fetalis verið lýst í gegnum aldirnar • Um 400 f.Kr lýst af Hippokrates • 1943 skilgreindi Potter 2 flokka af hydrops fetalis- immune og non-immune • 1969 Rhesus varnir hefjast á Íslandi

  3. Hydrops fetalisSkilgreining • Of mikil vökvahleðsla/bjúgur í líkamshólfum fósturs • Vökvasöfnun í a.m.k 2 líkamshólfum • Subcutant vef • Fleiðru • Pericardium • Kviðarholi

  4. Meingerð • Aukinn interstitial vökvi myndaður miðað við upptöku í lympatik kerfið • ↑ bláæðaþrýstingur • ↓ lympatic drainage • ↓ onkótiskur þrýstingur í háræðum

  5. Orsakir • Immune (<10%) • Non-Immune (<90%) 1/1500-1/3800 fæðingar • Hjartavandamál • Lungnavandamál • Sýkingar • Erfðagallar • Idiopathic

  6. Greining • Í móðurkviði: Ómskoðun • Tilviljanagreining • Aukin hæð legbotns • Minnkaðar hreyfingar fósturs • Eftir fæðingu

  7. Meðferð • Í móðurkviði • Intrauterine inngrip • Eftir fæðingu • Öndunaraðstoð • Blóðrás • Greina undirliggjandi orsök

  8. Immune hydrops fetalis • Rhesus hemolytic disease • Minor blood groups hemolytic disease • ABO hemolytic disease

  9. Rhesus hemolytic disease • Algengasta orsök immune hydrops fetalis • 15% Íslendinga rhesus neg • Tíðni minnkað með rhesus vörnum • Ekki fyrsta meðganga • Mildur sjúkdómur  hydrops fetalis

  10. Minor blood groups hemolytic disease • Kell, Duffy • Mildur sjúkdómur  hydrops fetalis ABO hemolytic disease • Vægari sjúkdómur • Hydrops fetalis mjög sjaldgæft

  11. Cardiovascular Malformation Left heart hypoplasia Atriventricular canal defect Right heart hypoplasia Closure of foramen ovale Single ventricle Transposition of great arteries Ventricular septal defect Atrial septal defect Tetralogy of Fallot Tachyarrhytmia Atrial flutter Paroxysmal atrial tachycardia Wolff-Parkinson-White Supraventricular tachycardia Bradyarrhytmia High output failure Cardiac tumors Cardiomyopathy Litningafrávik Turner Trisomy 21 Trisomy 18 Trisomy 13 Triploidy Non-immune hydrops fetalis • Infections • CMV • Toxoplasmosis • Parvovirusd B19 • Syphilis • Herpes • Rubella • Coxsackievirus • Leptospirosis • Hematologic • Alfha-thalassemia • Fetomaternal transfusion • Parvovirus B19 • In uteri hemorrhage • Red cell enzyme deficiencie • Thrombosis of major vessels • Leukemia • Red cell aplasias • Urinary • Urethral stenosis/atresia • Posterior urethral valves • Congenital nephrosis • Prune Belly syndrome • Gastrointestinal • Midgut volvulus • Malrotation of intestines • Meconium peritonitis • Hepatic fibrosis • Cholstasis • Biliary atresia • Hepatitis • Metabolic • Gaucher’s disease • GM1 gangliosidosis • Sialadosis • Hurler syndrome

  12. Non-immune hydrops fetalis • Erfðasjúkdómar • Litningafrávik (10%) • Turner syndrome • Þrístæða 21, 13, 18, 12 • Mortality ~100% • Metabolic storage disease (1-15%) • Autosomal víkjandi

  13. Non-immune hydrops fetalis • Hjarta og æðasjúkdómar (40%) • Structural • AVSD • Hypoplastiskt hjarta • Ventricular septal defect • Atrial septal defect • Arrythmia • Tachyarrhytmias –high output hjartabilun • Bradyarrhytmias –low output hjartabilun

  14. Non-immune hydrops fetalis • Anemia (10-30% allra tilfella) • Blæðingar • Hemolysa • Galli í myndun RBK • Alfa-talassemia major- algengt í SA-Asíu

  15. Non-immune hydrops fetalis • Sýkingar (8%) • Parvovirus B19 algengastur • TORCH • Syphilis, varicella, adenovirus, coxsachie

  16. Horfur • Mortality 50% • Mismunandi horfur eftir orsök • Immune • Non-immune: 50-98% mortality • Verri horfur • Hydrops fetalis snemma í meðgöngu • Fleiðru effusion og polyhydramnion fyrir 20v • Apgar skor <3 við 5 mín • Þörf á mikilli öndunaraðstoð • Litningafrávik • Þroskaröskun?

  17. Takk fyrir

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