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Monozygotic twinning and Wiedemann-Beckwith syndrome

✍ Scribed by Franceschini, Piergiorgio ;Guala, Andrea ;Vardeu, Maria Paola ;Franceschini, Daniele

Publisher
John Wiley and Sons
Year
1993
Tongue
English
Weight
223 KB
Volume
46
Category
Article
ISSN
0148-7299

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✦ Synopsis

Wiedemann-Beckwith syndrome (WBS) has already been noted to have asymmetric expression in monozygous (MZ) twins. Up to now 12 sets of MZ twins with WBS have been reported (Table I). To this series we would like to add 2 more sets of female twins, both discordant for WBS.

TWIN PAIR 1 This set of twins was born by cesarean section at 35 weeks to normal nonconsanguineous parents aged, respectively, 31 years (mother) and 33 years (father). Pregnancy was complicated by toxemia. The affected twin was the second born of 2 females with a birthweight of 2,810 g (75th centile for gestational age), a length of 47 cm (50th centile), an occipitofrontal circumference ( O F 0 of 32.5 cm (50th centile), and Apgar score of 4 at one minute and 8 at 5 minutes. A macroglossia was noted at birth. The firstborn nonaffected twin weighed 2,640 g (50th centile), had a length of 49 cm (75th centile), an OFC of 32 cm (50th centile), and an Apgar score of 9 at one and 5 minutes. No noticeable anomalies were detected. The placenta was monochorionic and diamniotic. No postnatal hypoglycemia, respiratory distress, or aniridia were noted in either twin.

Anthropometric values of both twins obtained at 20 months (Fig. 1) are reported in Table 11. The affected twin weighed 14 kg (97th centile) and was noted to have macroglossia and indentations on the posterior rim of the helix. No hemihypertrophy or skeletal asymmetry were noted. An abdominal ultrasound examination at 18 months showed kidneys and adrenals of normal size.

G-banded prometaphase chromosomes of both twins were normal (46,XX). HLA and extended erythrocyte antigen phenotype carried out on both twins and their parents confirmed monozygosity.

TWIN PAIR 2

The twins, both females, were the product of the second pregnancy of healthy, normal parents aged, respec-

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