Abstract

A REVIEW ON BECKWITH WIEDEMANN SYNDROME

Prajapati Anil*, Urvashi Sharma, Muley Preeti, Malviya Sapna and Kharia Anil

Abstract

Beckwith-Wiedemann syndrome (BWS) is a disorder involving overgrowth conditions. It is also characterized by a broad spectrum of symptoms and physical conditions that changes in range and severity from person to person. In this syndrome many individuals show conditions including above-average birth weight and increased growth after birth, uncontrolled growth of one side of the body. The syndrome occurs mainly in infants. Imprinted genes tend to be grouped together or clustered together. Several im In addition to H19 itself, this locus also produces a micro-RNA, called miR-675, an antisense protein coding transcript, called H19 opposite tumor suppressor (HOTS), and a long inter-genic antisense transcript called 91H printed genes are found in a cluster on chromosome 11p. The broad range of potential symptoms can affect many different organs of the body. Affected individuals may not have all of the symptoms listed in article. Cytogenetically detectable abnormalities involving chromosome 11p15 are found in 1% or fewer of affected individuals. Molecular genetic testing can identify epigenetic and genomic alterations of chromosome 11p15 in individuals with BWS. Surgery may be performed during early puberty to equalize significant differences in leg length secondary to hemi-hyperplasia; craniofacial surgery may benefit individuals with facial hemi-hyperplasia (a symptom of BWS). More symptoms are mainly Macrosomia, Macroglossia, Hemihyperplasia, Omphalocele, Placental mesenchymal dysplasia, Cleft palate and others.

Keywords: Beckwith-wiedemann syndrome, clinical aspects, H19 genetic details, symptoms and treatment management.