title: [A rare cause of polyhydramnios: Steinert's syndrome. A clinical case report].
abstract: Steinert's syndrome is a systemic disease with autosome mother-to-child transmission, characterized by myotonia and muscular dystrophia. The syndrome's clinical characteristics include: respiratory and alimentation diseases, facial diplegia, generalized hypotonia, areflexia, atrophy, arthrogryposis, hydramnios, retard in psychomotor development, cataract and genital disorders. A case of pregnancy occurs in Steinert syndrome's patient with hydramnios as a predominant symptom, is presented. At birth, in the congenital neonatal form, there is grave, generalized hypotonia which causes a very quick death of the newborn. There are not many characters which can be found out by ultrasound: hydramnios, reduction of fetal tone and active movements, micrognathia. Consequently it is of the utmost importance in those cases an adequate prenatal genetic counseling and a correct obstetrical management.
authors.affiliation: Divisione di Ginecologia e Ostetricia, Ospedale Bolognini, Seriate, Bergamo.
authors.forename: G
authors.initials: G
authors.lastname: Palmerio
authors.forename: P
authors.initials: P
authors.lastname: Rosaschino
authors.forename: G
authors.initials: G
authors.lastname: Castelli
authors.forename: E
authors.initials: E
authors.lastname: Zambetti
authors.forename: P
authors.initials: P
authors.lastname: Bianchi
authors.forename: D
authors.initials: D
authors.lastname: Martinelli
mesh_terms: D000015:Abnormalities, Multiple / Q000209:etiology / Q000473:pathology; D000328:Adult; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009223:Myotonic Dystrophy / Q000150:complications* / Q000473:pathology; D006831:Polyhydramnios / Q000209:etiology* / Q000473:pathology; D011247:Pregnancy; D011263:Pregnancy Trimester, Third
vernacular_title: Una causa rara di polidramnios: la sindrome di Steinert. Descrizione di un caso clinico.