A 34-week-old male fetus with short rib polydactyly syndrome (SRPS) type I (Saldino-Noonan) with pancreatic cysts

A 34-week-old male fetus (first diagnosed at 28 weeks of gestation) with short rib polydactyly type I Saldino-Noonan syndrome is presented in this study. In the postmortem examination of the fetus, pancreatic dysplasia, multiple cysts and multicystic dysplastic kidneys, omphalomesenteric cyst...

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Main Authors: Sevim Balci, Gülçin Altinok, Fulya Tekşen, Muzaffer Eryilmaz
Format: Article
Language:English
Published: Hacettepe University Institute of Child Health 2003-04-01
Series:The Turkish Journal of Pediatrics
Online Access:https://turkjpediatr.org/article/view/2885
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author Sevim Balci
Gülçin Altinok
Fulya Tekşen
Muzaffer Eryilmaz
author_facet Sevim Balci
Gülçin Altinok
Fulya Tekşen
Muzaffer Eryilmaz
author_sort Sevim Balci
collection DOAJ
description A 34-week-old male fetus (first diagnosed at 28 weeks of gestation) with short rib polydactyly type I Saldino-Noonan syndrome is presented in this study. In the postmortem examination of the fetus, pancreatic dysplasia, multiple cysts and multicystic dysplastic kidneys, omphalomesenteric cyst, ascites, malrotation, micropenis, undescended testes, bilateral inguinal hernia and hydrops were observed. The parents were first-degree cousins. One male and one female sibling had similar findings and both had died after birth. Only a four-year-old healthy daughter was alive. We believe these findings will be helpful in the differential diagnosis of further lethal skeletal dysplasia cases.
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issn 0041-4301
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language English
publishDate 2003-04-01
publisher Hacettepe University Institute of Child Health
record_format Article
series The Turkish Journal of Pediatrics
spelling doaj-art-a5da581704514bbe869fe4bd2f3668d52025-08-20T03:01:13ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212003-04-01452A 34-week-old male fetus with short rib polydactyly syndrome (SRPS) type I (Saldino-Noonan) with pancreatic cystsSevim Balci0Gülçin AltinokFulya TekşenMuzaffer EryilmazDepartment of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey. A 34-week-old male fetus (first diagnosed at 28 weeks of gestation) with short rib polydactyly type I Saldino-Noonan syndrome is presented in this study. In the postmortem examination of the fetus, pancreatic dysplasia, multiple cysts and multicystic dysplastic kidneys, omphalomesenteric cyst, ascites, malrotation, micropenis, undescended testes, bilateral inguinal hernia and hydrops were observed. The parents were first-degree cousins. One male and one female sibling had similar findings and both had died after birth. Only a four-year-old healthy daughter was alive. We believe these findings will be helpful in the differential diagnosis of further lethal skeletal dysplasia cases. https://turkjpediatr.org/article/view/2885
spellingShingle Sevim Balci
Gülçin Altinok
Fulya Tekşen
Muzaffer Eryilmaz
A 34-week-old male fetus with short rib polydactyly syndrome (SRPS) type I (Saldino-Noonan) with pancreatic cysts
The Turkish Journal of Pediatrics
title A 34-week-old male fetus with short rib polydactyly syndrome (SRPS) type I (Saldino-Noonan) with pancreatic cysts
title_full A 34-week-old male fetus with short rib polydactyly syndrome (SRPS) type I (Saldino-Noonan) with pancreatic cysts
title_fullStr A 34-week-old male fetus with short rib polydactyly syndrome (SRPS) type I (Saldino-Noonan) with pancreatic cysts
title_full_unstemmed A 34-week-old male fetus with short rib polydactyly syndrome (SRPS) type I (Saldino-Noonan) with pancreatic cysts
title_short A 34-week-old male fetus with short rib polydactyly syndrome (SRPS) type I (Saldino-Noonan) with pancreatic cysts
title_sort 34 week old male fetus with short rib polydactyly syndrome srps type i saldino noonan with pancreatic cysts
url https://turkjpediatr.org/article/view/2885
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