Document Detail

Massive increase of foetal abdominal circumference due to hereditary polycystic kidney disease
MedLine Citation:
PMID:  20574939     Owner:  NLM     Status:  MEDLINE    
Autosomal recessive polycystic kidney disease (ARPKD) is a rare condition with a poor prognosis. We report on a 30-year-old primagravid woman in the 34th) week of gestation who was admitted to our hospital. ARPKD of the foetus had been sonographically suspected since the 26th week of gestation. Ultrasound examination showed big polycystic kidneys on both sides. The non-consanguineous parents wanted a maximum therapy for the infant. Foetal digitalisation because of heart insufficiency and prophylactic lung maturation was started. In the further course, Doppler sonographic values worsened and a Caesarean section was performed in the 34th week of gestation at the demand of the parents and due to the expected problems in case of a vaginal delivery. The weight of the newborn was 3,780 g and the abdominal circumference was 50 cm. The newborn was intubated immediately after birth and artificial ventilation was performed. Extracorporeal membrane oxygenation was not possible due to the bad cardial condition. The boy died 16 h after delivery. The parents refused genetic examination and autopsy of the newborn. ARPKD is a severe disease that may have obstetric relevance, due to the massively increased abdominal circumference. Therefore, termination of pregnancy or preterm induction of labor should be considered in order to avoid Caesarean section. Additionally, early prenatal diagnosis with genetic analysis of PRKD1 in cases of suspected ARPKD can be helpful.
L Dukic; R Schaffelder; T Schaible; M Sütterlin; J Siemer
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Publication Detail:
Type:  Case Reports; English Abstract; Journal Article     Date:  2010-06-23
Journal Detail:
Title:  Zeitschrift für Geburtshilfe und Neonatologie     Volume:  214     ISSN:  1439-1651     ISO Abbreviation:  Z Geburtshilfe Neonatol     Publication Date:  2010 Jun 
Date Detail:
Created Date:  2010-06-24     Completed Date:  2010-08-17     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9508901     Medline TA:  Z Geburtshilfe Neonatol     Country:  Germany    
Other Details:
Languages:  ger     Pagination:  119-22     Citation Subset:  IM    
Copyright Information:
Georg Thieme Verlag KG Stuttgart, New York.
Frauenklinik, Universitätsklinikum Mannheim.
Vernacular Title:
Massive fetale Bauchumfangszunahme bei hereditärer Zystennierenerkrankung.
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MeSH Terms
Abdomen / ultrasonography*
Cesarean Section
Fatal Outcome
Fetal Macrosomia / ultrasonography*
Infant, Newborn
Polycystic Kidney, Autosomal Recessive / ultrasonography*
Pregnancy Trimester, Third
Ultrasonography, Doppler
Ultrasonography, Prenatal*

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