Prader Willi Syndrome, a rare genetic disorder
Dr Shahnaz Parvin Siddiqua, Dr Runa Laila, Dr Khorshed Alam, Dr Nargis Ara Begum
A 4½ years old male child was referred to United Hospital from BIRDEM with complaints of severe respiratory distress, generalized anasarca and severe respiratory acidosis; patient being in need of ventilator support. On examination the boy was dyspneic with severe chest indrawing, tachypneic, tachycardic, hypoxic in room air, hypotensive & had significantly severe crepitations in lungs, significant hepatomegaly. He was short (height <3rd centile) but obese (weight >97th centile). He had small hands and feet, narrow forehead, almond shaped eyes, central obesity & chryptorchidism (right side).
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