Clinical Genetics - 1987

166 articles | Last updated: 2025-12-03 14:12:56
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Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
Heterozygous manifestations of Langer mesomelic dysplasia
A male infant with holoprosencephaly, associated with ring chromosome 21
A case report of a de novo tandem duplication (5p) (p14 → pter)
Osteopetrosis
A distinct congenital motor and sensory neuropathy (neuronal type) with dysmorphic features in a father and two sons. A variant of Charcot‐Marie‐Tooth disease
Public records and recognition of genetic disease in Scotland
Ichthyosis ‐ Cheek ‐ Eyebrow (ICE) syndrome: a new autosomal dominant disorder
Major genes of eye color and hair color linked to LU and SE Danish
Contribution to the 18q‐ syndrome. A patient with del (18) (q22.3qter)
Albinism, or the NOACH syndrome
A phenotypic male with true hermaphroditism and a 46,XX/46,XY/ 47,XXY karyotype
Molecular heterogeneity of translocations associated with muscular dystrophy
A final word on the tricho‐rhino‐phalangeal syndromes
Is disordered folate metabolism the basis for the genetic predisposition to neural tube defects?
Trichodysplasia‐xeroderma: an autosomal dominant condition
Three cases of partial trisomy 9q in one generation due to maternal reciprocal t(6;8;9) translocation
Trisomy 2q and monosomy 11q in the same individual: the importance of considering the deleted segment
Letter to the Editors
A peculiar subphenotype in the fra(X) syndrome: extreme obesity‐short stature‐stubby hands and feet‐diffuse hyperpigmentation. Further evidence of disturbed hypothalamic function in the fra(X) syndrom
Postmortem Menkes diagnosis from carrier testing of female relatives
Deletion of the DXS165 locus in patients with classical Choroideremia
Announcements
Erratum
Prader‐Willi syndrome in two siblings: one with normal karyotype, one with a terminal deletion of distal Xq
Homozygous variegate porphyria A severe skin disease of Infancy
Progressive mental regression in siblings with Morquio disease Type B (mucopolysaccharidosis IV B)
X‐linked dysmorphic syndrome with mental retardation
X‐linked muscular dystrophy with early contractures and cardiomyopathy (Emery‐Dreifuss type)
Family resemblance for fasting blood glucose: the Jerusalem Lipid Research Clinic ethnicity; origin groups; Israeli population sample; population
Isochromosome (18q) in siblings*
Effect of screening for cystic fibrosis on the influence of genetic counseling
Genetic history: II. The Cohens of London
PEDIGREE‐PLOT: a computer program for plotting pedigrees
Carrier detection of haemophilia A using DNA markers in families with an isolated affected male
Predicting intellectual functioning in 47,XXY boys from characteristics of sibs
Familial transmission of a ring chromosome 21
Tandem duplication (1) (q11 → q22) in a male infant with multiple congenital malformations
Robertsonian translocation and an extra microchromosome: aetiology and effect on meiotic segregation
Proximal duplication of the long arm of chromosome 10 (10q11.2 → 10q22): a distinct clinical entity
Parental age, and how extra isochromosomes (secondary trisomy) arise
Dyslexia and chromosome 15 heteromorphism: negative lod score in a Danish material
A case of first trimester chromosomal mosaicism confined to the cultivation of the gestational products
Partial trisomy 3q syndrome inherited from familal t(3;9) (q26.1;p23)*
Severity and recurrence risk of congenital heart defects exemplified by atrial septal defect secundum
The ulnar‐mammary syndrome: an autosomal dominant pleiotropic gene
Ichthyosis‐cheek‐eyebrow syndrome (ICE(<sub>skin</sub>))
Carrier Detection in DMD
Trends in European Multiple Sclerosis Research Lyon, France September 3–5, 1987
Genetic heterogeneity in X‐linked agammaglobulinemia complicates carrier detection and prenatal diagnosis
Mental retardation in a patient with Maroteaux‐Lamy
Cardiovascular complications in the Ehlers‐Danlos syndrome with minimal external findings
Cerebellar ataxia and total albinism
Estimation of prognosis and prevalence of retinitis pigmentosa and Usher syndrome in Norway Laps
How do carriers of hemophilia experience prenatal diagnosis by fetal blood s ampling?
Syndrome of microcephaly, deafness/ malformed ears, mental retardation and peculiar facies in a mother and son
Fragile sites are unrelated to reciprocal translocation breakpoints
The Angelman (Happy Puppet) syndrome: is it autosomal recessive?
Congenital cutis laxa
Single maxillary central incisor and coloboma in hypomelanosis of Ko black
A patient with onychotrichodysplasia, neutropenia and normal intelligence
Centric fission, centromere‐telomere fusion and isochromosome formation: a possible origin of a de novo 12p trisomy
Deletion of the short arm of chromosome 20
Announcement
Distal symphalangism with involvement of the thumbs and great toes
Isolated autosomal recessive renal magnesium loss in two sisters
Gamma‐glutamyl transferase activity in the amniotic fluid of fetuses with chromosomal aberrations and inborn errors of metabolism
Unusual pedigree patterns in families with spinal muscular atrophy
Convoluted cells as a marker for maternal cell contamination in CVS culture
What is the ichthyosis in the so‐called ichthyosis‐cheek‐eyebrow (ICE) syndrome?
18ph+ is a so‐called normal chromosomal variant
A rapid and efficient screening method for DNA restriction fragment length polymorphisms
Introduction of genomic diagnosis of classical phenylketonuria to the health care system of the German Democratic Republic
Difficulties encountered in a randomization trial of CVS versus amniocentesis for prenatal diagnosis
An aminopterin‐like syndrome without aminopterin (ASSAS)
Trisomy of the short arm of chromosome 5: autopsy data in a malformed newborn with inv dup (5)(p13.1 → p15.3)
18ph+ is a normal chromosomal variant
Microcephalic osteodysplastic dwarfism (Type ll‐like) in siblings
X‐linked myotubular myopathy: intrafamilial variability and normal muscle biopsy in a heterozygous female
Heterogeneity of pseudoxanthoma elasticum: delineation of a new form? Dutch and French‐Huguenot stock; Afrikaner descent
Effect of combinations of antioxidants on oxygen radical‐induced sister chromatid exchanges
Suggestion of linkage of a major locus for nonsyndromic orofacial cleft with F13A and tentative assignment to chromosome 6 Danish
Familial absence of the trapezius muscle with associated shoulder girdle abnormalities
Clinical anophthalmos in a family
Monosomy and trisomy of 15q24→qter in a family with a translocation t(6;15)(P25;q24)
Evidence for a sperm mutation resulting in Duchenne muscular dystrophy
X‐linked retinoschisis is closely linked to DXS41 and DXS16 but not DXS85
Ehlers‐Danlos syndrome: yet another type?
Postaxial polydactyly in association with neurofibromatosis
Announcement
“Unclassifiable” weak A blood group and deficient H phenotype (Hm) in one pedigree
Triphalangeal thumb and brachy‐ectrodactyly syndrome
Bloom's syndrome with porokeratosis of Mibeili and multiple cancers of the skin, lung and colon
Chromosomal aberrations in 85 mentally retarded patients examined by high resolution banding
Robinow syndrome: report of two patients and review of literature
Centromeric instability of chromosomes 1, 9 and 16 with variable immune deficiency. Support of a new syndrome
Roberts syndrome and SC phocomelia. A single genetic entity
A new interstitial deletion of chromosome No. 4 del(4) (q22::q25)
Kallmann syndrome associated with choanal atresia
Proximal 15q variant with normal phenotype in three unrelated individuals
Oro‐facial‐digital syndrome II. Transitional type between the Mohr and the Majewski syndromes: report of two new cases
Supernumary marker chromosomes in a mentally retarded population identified as in vdup(15)
Linkage of G8 (D4S10) in two Swedish families with Huntington's disease
Familial dysbetalipoproteinemic subjects with the E3/E2 phenotype exhibit an E2 isoform with only one cysteine residue
Free proximal trisomy 21 without the Down syndrome
Pitted enamel hypoplasia in tuberous sclerosis patients and first‐degree relatives
Relative effect of parental birth weight on infant birth weight at term Scandinavian
Prenatal diagnosis of glycerol‐kinase deficiency associated with a DNA deletion on the short arm of the X‐chromosome
Tetrasomy for the short arm of chromosome 12 with accessory isochromosome (+i(12p)) and a marked LDH‐B gene dosage effect
Incidence of familial dysautonomia in Israel 1977–1981 Ashkenazi Jews; North American Ashkenazi Jews
Inverted tandem duplication generates a duplication deficiency of chromosome 8p
Hereditary hypotrichosis simplex of the scalp Spanish kindred; Jewish‑Yemenite kindred
Major locus for red hair color linked to MNS blood groups on chromosome 4 Danish
Fragile (X) syndrome: a study of the psychological profile in 23 prepubertal patients
Holoprosencephaly associated with ring chromosome 21
Fragile 19p13 in a family with mental illness
In vitro hyperdiploidy in dermal fibroblasts: evidence for genetic predisposition in aerodigestive tract cancer
Prenatal diagnosis in Becker muscular dystrophy
Population cytogenetics of autosomal fragile sites
Pseudodeficiency of arylsulfatase A: a counseling dilemma
Inv(4)(p16q21). A five‐generation pedigree with 24 carriers and no recombin ants
Congenital ichthyosis with alopecia, eclabion, ectropion and mental retardation ‐ a new genetic syndrome inbred North‐Swedish family
European Society for Human Genetics Cardiff, Wales June 30‐July 3, 1988
Unusual in vivo rearrangements of the Y‐chromosome in two males
A new patella syndrome
Technical and biological aspects of pseudomosaicism and mosaicism
Tetraploidy with hydrops fetalis, cystic nuchal hygroma and 90, XX karyotype
Autosomal dominant endosteal hyperostosis Spanish
An abnormal pattern of amniotic fluid microvillar enzymes signalling fetal cystic fibrosis
Assignment of the locus order DXS28‐ DXS67‐DMD as a spin‐off from diagnostic DNA marker analysis in a family with Duchenne muscular dystrophy
Announcement
Diagnosis of arylsulfatase A deficiency in intact cultured cells using a fluorescent derivative of cerebroside sulfate
Adult Turner syndrome associated with chylous ascites and vascular anomalies
Autosomal recessive inheritance of idiopathic dilated cardiomyopathy in a Madeira Portuguese kindred Madeira Portuguese
Becker's allelic model to explain unusual pedigrees with spinal muscular atrophy
28th Annual Short Course in Medical and Experimental Mammalian Genetics
Female carriers of Duchenne muscular dystrophy: a dilemma
Choroideremia: close linkage to DXYS1 and DXYS12 demonstrated by segregation analysis and historical‐genealogical evidence
Partial duplication 16q: report of two affected siblings resulting from a maternal translocation and literature review
Effect of vitamin E and ticlopidine on platelet aggregation in Fabry's disease
Letters to the Editors: Reply to a response by Sherman &amp; Iselius to comments on the segregation of balanced pericentric inversions.1
Announcement
Familial transmission of Wolf syndrome resulting from specific deletion 4p16 from t(4;8)(p16;p21) mat.
Autosomal dominant inheritance of cervical ribs
A Japanese patient with the Dubowitz syndrome Japanese; ethnic groups
Pallister‐Killian syndrome: cytogenetic and molecular studies
Convoluted cells as a marker for maternal cell contamination in CVS cultures
The Brachmann‐de Lange syndrome in two siblings of normal parents
Autosomal dominant epidermolysis bullosa dystrophics: are the Cockayne‐ Touraine, the Pasini and the Bart‐types different expressions of the same mutant gene?
Elevated 1,25‐dihydroxyvitamin D and normocalcaemia in presumed familial Williams syndrome
Trisomy 18 clustering in Kuwait
A boy with Down's syndrome having recombinant chromosome 21 but no SOD‐1 excess
On the origin of recurrent trisomy 21: determination using chromosomal and DNA polymorphisms
A boy with thanatophoric dysplasia surviving 212 days
Similar facia! anomalies in patients with dicentric Y and long arm Y deletion
New type of spinocerebellar degeneration syndrome in a northern Swedish population northern Swedish population; geographically isolated northern Swedish population
A new autosomal dominant craniofacial deafness syndrome Jewish
Transcultural study of Turner's syndrome
Multiple pterygium syndrome: a case complicated by malignant hyperthermia
Non‐mosaic isodicentric X‐chromosome in a patient with secondary amenorrhea
Langer‐Giedion syndrome with del 8 (q24.13‐q24.22)
Gene for incontinentia pigmenti maps to band Xp11 with an (X;10) (p11;q22) translocation
Genetic history: I. The Schneersons of Lubavich
Chromosome studies in IgA‐deficient patients
Announcement
Type III congenital cystic adenomatoid malformation of the lung: another cause of elevated alpha fetoprotein?
Three‐generation transmission of Hirschsprung's disease