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Browsing by Author "Dasgupta, MK"

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    Greek Warrior Helmet Facies (Wolf-hirschhorn Syndrome)
    (Nepal Paediatric Society (JNPS), 2014) Biswas, P; Choudhury, R; Gayen, S; Guha, D; Roy, S; Dasgupta, MK
    Abstract: Wolf-Hirschhorn syndrome (WHS) is caused by a chromosomal deletion of the band 4p16.3 with characteristic craniofacial features -’Greek warrior helmet’5 facies (prominent glabella, hypertelorism, broad beaked nose and frontal bossing), high-arched eyebrows, protruding eyes, epicanthal folds, short philtrum, distinct mouth with downturned corners, micrognathia, dysplastic ears, preauricular tags. Till date there are very few case reports of Wolf-Hirschhorn syndrome.Here we report a case that had characteristic dysmorphic facies (Figure 1) ‘Greek warrior helmet’ and was diagnosed as a case of WHS. But presence of Meningo-encephalocele and lissencephaly is rarely reported in literature in association with Wolf-hirschhorn syndrome till date.
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    Unilateral Hypoplastic Kidney in a Case of Holt-Oram Syndrome
    (Nepal Paediatric Society (JNPS), 2013) Dasgupta, MK; Dutta, A; Sarkar, S; Patra, C; Dey, C
    Abstract: Holt-Oram syndrome (HOS) is characterised by skeletal abnormalities of the upper limb & congenital cardiac defects. Here we report a case of one day old male baby with skeletal abnormalities of forearms and hands, atrial septal defect of ostium secundum type and unilateral hypoplastic kidney. Till date, other associated anomalies like extra-hepatic portal hypertension, renal artery malformations etc have been reported with Holt-Oram syndrome, but it is the first case to be reported in association with hypoplastic kidney.

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