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A rare association of Down syndrome with Dandy eWalker variant, pulmonary hypertension and childhood interstitial lung disease: A case report of a prognostic dilemma

Mohd Kori, Anis Munirah and Alias, Amelia and Daud, Mariana and Yahaya, Nor Azni and Anuar, Muhamad Azamin (2022) A rare association of Down syndrome with Dandy eWalker variant, pulmonary hypertension and childhood interstitial lung disease: A case report of a prognostic dilemma. Journal of Taibah University Medical Sciences. ISSN 1658-3612 (In Press)

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Abstract

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.

Item Type: Article (Journal)
Uncontrolled Keywords: Childhood interstitial lung disease; Dandy eWalker variant; Down syndrome; Prognosis; Pulmonary hypertension
Subjects: R Medicine > RJ Pediatrics
Kulliyyahs/Centres/Divisions/Institutes (Can select more than one option. Press CONTROL button): Kulliyyah of Medicine
Kulliyyah of Medicine > Department of Paediatrics
Depositing User: Dr Muhamad Azamin Anuar
Date Deposited: 27 Jun 2022 16:42
Last Modified: 27 Jun 2022 16:44
URI: http://irep.iium.edu.my/id/eprint/98530

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