[Prenatal diagnosis of upper limb malformations]. 1980

J F Mattei, and J Cristofari, and M Gamerre, and S Ayme, and F Giraud

Three children with thrombopenia and radial aplasia were examined at the Centre de Génétique Médicale of Marsielles from 1974 to 1976. In each case the genetic basis of the anomaly was discussed with the family at genetic counseling underlining the high risk of recurrence related to the autosomal recessive transmission. Each proband was the only child of the couple. In the first case the father and mother, each having one child from a previous marriage, decided to have no more children. In the second case, the use of fetoscopy and its limitations were discussed as another pregnancy was desired. However, the parents finally refused the procedure during pregnancy which yielded a second child with phocomelia who did not survive. In the third and most recent case the proband died. Since the young couple strongly desired another child, ultrasonic monitoring of pregnancy was cautiously suggested and its limitations clearly exposed. Echography was regularly repeated from the third month of pregnancy. The forearms were never detected on echography, nor on fetal radiography. Pregnancy was thus interrupted with prostaglandins at 21 weeks gestational age. On examination the fetus presented bilateral aplasia of the forearm. Based on these observations, the authors discuss the problems raised by prenatal diagnosis of upper limb malformations.

UI MeSH Term Description Entries
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008297 Male Males
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D011296 Prenatal Diagnosis Determination of the nature of a pathological condition or disease in the postimplantation EMBRYO; FETUS; or pregnant female before birth. Diagnosis, Prenatal,Fetal Diagnosis,Fetal Imaging,Fetal Screening,Intrauterine Diagnosis,Antenatal Diagnosis,Antenatal Screening,Diagnosis, Antenatal,Diagnosis, Intrauterine,Prenatal Screening,Antenatal Diagnoses,Antenatal Screenings,Diagnosis, Fetal,Fetal Diagnoses,Fetal Imagings,Fetal Screenings,Imaging, Fetal,Intrauterine Diagnoses,Prenatal Diagnoses,Prenatal Screenings,Screening, Antenatal,Screening, Fetal,Screening, Prenatal
D004480 Ectromelia Gross hypo- or aplasia of one or more long bones of one or more limbs. The concept includes amelia, hemimelia, phocomelia, and sirenomelia. Amelia,Congenital Limb Reduction Deformities,Congenital Limb Reduction Deformity,Hemimelia,Phocomelia,Sirenomelia,Fused Legs and Feet,Mermaid Malformation,Mermaid Syndrome,Sirenomelia Sequence,Sirenomelus,Malformation, Mermaid,Sequence, Sirenomelia,Sirenomelia Sequences,Syndrome, Mermaid
D005260 Female Females
D005808 Genes, Recessive Genes that influence the PHENOTYPE only in the homozygous state. Conditions, Recessive Genetic,Genetic Conditions, Recessive,Recessive Genetic Conditions,Condition, Recessive Genetic,Gene, Recessive,Genetic Condition, Recessive,Recessive Gene,Recessive Genes,Recessive Genetic Condition
D005817 Genetic Counseling An educational process that provides information and advice to individuals or families about a genetic condition that may affect them. The purpose is to help individuals make informed decisions about marriage, reproduction, and other health management issues based on information about the genetic disease, the available diagnostic tests, and management programs. Psychosocial support is usually offered. Counseling, Genetic,Genetic Counseling, Prenatal,Prenatal Genetic Counseling
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes

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