Table 3

Incidence of clinical symptomes in the exomphalos-short limbs-macrogonadism syndrome as compared to Beckwith-Wiedemann syndrome

Exomphalos-short limbs-macrogonadism syndrome (this study)Beckwith-Wiedemann syndrome (Elliott et al3)
Exomphalos7/7 (100%)60%
Macrosomia4/7 (57%)87%
Macroglossia0/7 (0%)99%
Ear abnormalities1/7 (14%)75%
Kidney abnormalities4/7 (57%)62%
Adrenal cytomegaly6/7 (85%)Frequent
Langherans cell hyperplasia4/7 (57%)Classical
Macrogonadism6/6 (100%)Possible
Short limbs7/7 (100%)0%
Nuchal web7/7 (100%)0%
Sex ratio6/11.1/1