Patau Syndrome
Το Σύνδρομο Patau (τρισωμία 13) έχει υψηλό ποσοστό θνησιμότητας με πολλαπλές συγγενείς ανωμαλίες που οδηγούν σε σοβαρή σωματική και νοητική μειονεξία.
• Αυτό συνήθως οφείλεται σε τρισωμία με ένα επιπλέον χρωμόσωμα 13, αντί του κανονικού ζεύγους που υπάρχει σε όλα τα κύτταρα.
• Μια ασύμμετρη μετατόπιση χρωμοσώματος μπορεί να συμβεί και ονομάζεται Robertsonian μετάθεση. Αυτό συμβαίνει όταν ένα επιπλέον αντίγραφο του χρωμοσώματος 13 συνδέεται σε ένα άλλο χρωμόσωμα.
• Υπάρχουν επίσης διάφοροι μωσαϊκισμοί, όπου ορισμένα κύτταρα είναι φυσιολογικά, με 46 χρωμοσώματα και άλλα έχουν το επιπλέον χρωμόσωμα. Τα βρέφη με παραλλαγές μωσαϊκισμού τείνουν να είναι λιγότερο σοβαρά άρρωστα.
Επιδημιολογία
Η τρισωμία 13 συναντάται μεταξύ 1 σε 5.000 και 1 σε 29.000 γεννήσεις και είναι η τρίτη πιο συχνή αυτοσωματική τρισωμία στα νεογνά μετά από τη τρισωμία 21 και τρισωμία 18.
Παράγοντες κινδύνου
• Ένα ατομικό ή οικογενειακό ιστορικό αυξάνει τον κίνδυνο να γεννηθεί ένα πάσχων παιδί.
• Ο κίνδυνος αυξάνει με την ηλικίας της μητέρας, αλλά όχι τόσο σημαντικά όσο με το σύνδρομο Down (τρισωμία 21) ή το σύνδρομο Edwards (τρισωμία 18).
Χαρακτηριστικά
Πολλά έμβρυα δεν επιβιώνουν μέχρι τη γέννα και άλλα γεννιούνται νεκρά. Τα χαρακτηριστικά γνωρίσματα περιλαμβάνουν:
- Ενδομήτρια καθυστέρηση ανάπτυξης και χαμηλό βάρος γέννησης.
• Συγγενείς ανωμαλίες της καρδιάς (80%):
Περιλαμβάνουν μεσοκολπική επικοινωνία, μεσοκοιλιακή επικοινωνία, ανοικτός αρτηριακός πόρος.
• Ολοπροσεγκεφαλία: ο εγκέφαλος δεν διαιρείται σε δύο ίσα μέρη. Αυτό μπορεί να δημιουργήσει προβλήματα της μέσης γραμμής του προσώπου όπως:
• λαγώχειλο και το λυκόστομα.
• Μικροφθαλμία ή ανοφθαλμία.
• ρινική δυσμορφία.
• Υποτελορισμό (μειωμένη απόσταση μεταξύ των οφθαλμών) ή κύκλωπα.
• Άλλες ανωμαλίες του εγκεφάλου ή του κεντρικού νευρικού συστήματος όπως οι εξής:
• ανωμαλίες του νευρικού συστήματος.
• Άλλες ανατομικές ανωμαλίες του εγκεφάλου
• σοβαρή μαθησιακή δυσκολία.
• Προβλήματα με τον έλεγχο της αναπνοής (κεντρική άπνοια).
• Άλλες κρανιοπροσωπικές ανωμαλίες περιλαμβάνουν:
• Μικροκεφαλία.
• γαστρεντερικές ανωμαλίες: ομφαλοκήλη, εξόμφαλος, κήλες.
• ουρογεννητικού δυσμορφίες: πολυκυστικούς νεφρούς, micropenis ή υπερτροφία της κλειτορίδας.
• Ανωμαλίες των χεριών και των ποδιών: polydactyly (επιπλέον δάχτυλα χεριών ή των ποδιών), rocker-bottom πόδια.
Η διαφορική διάγνωση
• Παιδιά με σύνδρομο Patau και το σύνδρομο Edwards μπορεί να έχουν παρόμοια χαρακτηριστικά και να είναι δύσκολο να διαφοροποιηθούν.
Πρόγνωση και Αντιμετώπιση
• Θεραπεία στα ζωντανά βρέφη είναι υποστηρικτική.
• Οι γονείς θα χρειαστούν μεγάλη υποστήριξη και παροχή συμβουλών.
• Αν το σύνδρομο Patau οφείλεται σε μη ισορροπημένη μετατόπιση χρωμοσώματος ή δομική χρωμοσωμική ανωμαλία και οι δύο γονείς θα πρέπει να υποβληθούν σε χρωμοσωμική ανάλυση. Αυτό έχει σημασία για τις μελλοντικές εγκυμοσύνες, λόγω του υψηλότερου κινδύνου υποτροπής.
• Εξέταση και προγεννητική διάγνωση θα πρέπει να προσφέρεται σε μελλοντικές εγκυμοσύνες. Οι γυναίκες που είχαν μία προηγούμενη εγκυμοσύνη με τρισωμία, και ειδικά κάτω των 35 ετών διατρέχουν αυξημένο κίνδυνο.
• Το προσδόκιμο ζωής είναι πολύ περιορισμένο. Η διάμεση επιβίωση είναι 2,5 ημέρες.
• Περίπου το 50% ζουν περισσότερο από 1 εβδομάδα.
• 5-10% των βρεφών ζουν περισσότερο από 1 έτος.
• Οι επιζώντες έχουν νοητική υστέρηση και αναπτυξιακή καθυστέρηση. Επιληπτικές κρίσεις και δυσκολία στη σίτιση είναι κοινές.
Προγεννητική διάγνωση
- Ειδικά υπερηχογραφικά ευρήματα και κυτταρογενετικές μελέτες μπορεί να προτείνουν τρισωμία 13. Τα ευρήματα περιλαμβάνουν αυξημένη αυχενική διαφάνεια, καρδιακές ανωμαλίες, ανωμαλίες του νευρικού σωλήνα, έλλειμμα του προσώπου, νεφρικές ανωμαλίες και ομφαλοκήλη.
• Μια μελέτη από το Kings College Hospital έδειξε ότι στον υπέρηχο 11 – 13 (+6) εβδομάδων, η μέτρηση της εμβρυϊκής αυχενικής διαφάνειας, του εμβρυϊκού καρδιακού ρυθμού και εξέταση του εμβρύου για ολοπροσεγκεφαλία, εξόμφαλο και μεγακύστη μπορεί να προσδιορίσει> 90% των εμβρύων με τρισωμία 13.
• Στο πρώτο τρίμηνο πολλαπλοί δείκτες (αυτοί που προσφέρονται σήμερα για τον έλεγχο σύνδρομο Down), μπορούν επίσης να βοηθήσουν να προσδιορίστει ένα έμβρυο με τρισωμία 13 ή 18. Δείκτες περιλαμβάνουν την ηλικία της μητέρας, μέτρηση αυχενικής διαφάνειας, η σχετιζόμενη με τη κύηση πρωτεϊνη –A (PAPP-A) και η ανθρώπινη χοριακή γοναδοτροπίνη (hCG). Στο υπερηχογράφημα του εμβρύου μπορεί επίσης να παρουσιάστουν ανωμαλίες.
• Η αμνιοπαρακέντηση ή λήψη τροφοβλάστης είναι απαραίτητη για να γίνει μια οριστική προγεννητική διάγνωση.
• Κυτταρογενετική μελέτη εμβρυϊκού αίματος μπορεί επίσης να πραγματοποιηθεί.
• Οι μητέρες ηλικίας άνω των 35 μπορούν να επιλέξουν να κάνουν κατ ‘ευθείαν διαγνωστικές εξετάσεις.
Πολύ πρόσφατα υπάρχει η δυνατότητα μιας μη επεμβατικής δοκιμασίας, με μια απλή εξέταση αίματος από τη μητέρα και την ανίχνευση εμβρυικού DNA στο αίμα της. Τα αποτελέσματα μπορούν να δείξουν, με ένα ποσοστό αξιοπιστίας της τάξεως του 99%, εάν το έμβρυο έχει το σύνδρομο ή όχι, αποφεύγοντας τον κίνδυνο αποβολής μιας επεμβατικής δοκιμασίας.
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