A rare association of Down syndrome with Dandy eWalker variant, pulmonary hypertension and childhood interstitial lung disease: A case report of a prognostic dilemma
Associations among Down syndrome (DS), Dandy eWalker variant (DWv), pulmonary hypertension (PH) and childhood interstitial lung disease (chILD) are extremely rare. Several cases of trisomy disorders with DandyeWalker malformation (DWM) and neuro�developmental outcomes have been reported. The ext...
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| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English English |
| Published: |
Elsevier
2022
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| Subjects: | |
| Online Access: | http://irep.iium.edu.my/98530/1/No%206%20-%20Down%20with%20DWV%2C%20PPH%20and%20CHILD%20JTUMED%20June%202022.pdf http://irep.iium.edu.my/98530/7/JTUMED%20letter.pdf http://irep.iium.edu.my/98530/ |
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| Summary: | Associations among Down syndrome (DS), Dandy
eWalker variant (DWv), pulmonary hypertension (PH)
and childhood interstitial lung disease (chILD) are
extremely rare. Several cases of trisomy disorders with
DandyeWalker malformation (DWM) and neuro�developmental outcomes have been reported. The extent to
which moderate to severe pulmonary hypertension with
complications of patent ductus arteriosus (PDA), PH and
chILD leads to substantial morbidity and mortality in in�fants with DS and DWM should be studied. We report the
case of an ex-premature 15-month-old girl with confirmed
DS with DWv, who developed PH and chILD. This is the
first case study reporting the complexity of multiple associ�ations involving DS and DWv. This case led to a prognostic
dilemma and required compassionate parental counselling
because of the child’s uncertain future. |
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