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Tuesday 14 June 2016

FIRST HARLEQUIN BABY BORN IN INDIA

 India’s first Harlequin baby passed away on Monday afternoon after a 48-hour struggle by the medical experts at Lata Mangeshkar Hospital. The baby girl, suffering from an extremely rare genetic disorder known as Harlequin-type ichthyosis, was born without external bodily skin and undeveloped eyes.

The baby, born to a farmer couple from Wadi, was their first child. Born at the Nagpur Hospital, the baby weighed 1.8 kg and was born prematurely by a C-section delivery. The medical experts of the hospital, who immediately recognised the condition of the child, moved the baby to NICU.


Harlequin icthyosis

Harlequin ichthyosis is a severe genetic disorder that mainly affects the skin. Infants with this condition are born with very hard, thick skin covering most of their bodies. The skin forms large, diamond-shaped plates that are separated by deep cracks (fissures). These skin abnormalities affect the shape of the eyelids, nose, mouth, and ears, and limit movement of the arms and legs. Restricted movement of the chest can lead to breathing difficulties and respiratory failure.

The skin normally forms a protective barrier between the body and its surrounding environment. The skin abnormalities associated with harlequin ichthyosis disrupt this barrier, making it more difficult for affected infants to control water loss, regulate their body temperature, and fight infections. Infants with harlequin ichthyosis often experience an excessive loss of fluids (dehydration) and develop life-threatening infections in the first few weeks of life. It used to be very rare for affected infants to survive the newborn period. However, with intensive medical support and improved treatment, people with this disorder now have a better chance of living into childhood and adolescence.