Clinical Genetics - 1994

167 articles | Last updated: 2025-12-03 14:12:56
Caucasian
5
White
1
European
3
Other
20
Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
Congenital cutis laxa with ligamentous laxity and delayed development, Dandy‐Walker malformation and minor heart and osseous defects
An epidemiological/genetic study of mental subnormality in Assiut Governorate, Egypt
Defective G2 repair in Down syndrome: effect of caffeine, adenosine and niacinamide in control and X‐ray irradiated lymphocytes
Direct transmission of a tandem duplication in the short arm of chromosome 8
A new, highly informative Smal polymorphism in intron 7 of the low density lipoprotein receptor (LDLR) gene
A fluorescent<i>in situ</i>hybridization analysis of the chromosome constitution of ejaculated sperm in a 47, XYY male
Duchenne muscular dystrophy and spinal muscular atrophy type I segregating in the same family
Non‐expression of von Hippel‐Lindau phenotype in an obligate gene carrier
Oto‐palato‐digital syndrome type II in two unrelated boys
Clinical aspects of the MASA syndrome in a large family, including expressing females
Partial trisomy and monosomy 8p due to inversion duplication
Gaucher disease: N370S glucocerebrosidase gene frequency in the Portuguese population Portuguese population; Ashkenazi Jewish genetic background
The apolipoprotein B signal peptide insertion/deletion polymorphism is not associated with myocardial infarction in Norway Norwegians
Associations of genotypes at the apolipoprotein AI‐CIII‐AIV, apolipoprotein B and lipoprotein lipase gene loci with coronary atherosclerosis and high density lipoprotein subclasses
Molecular and clinical analyses of cystic fibrosis in the South of Spain
Charcot‐Marie‐Tooth disease type 1A: the parental origin of a <i>de novo</i> 17p11.2‐p12 duplication Danish
X;1 translocation in a female Menkes patient: characterization by fluorescence <i>in situ</i> hybridization
Familial translocation t(10;14) (q26.1;q32.3): report of three offspring with 10q deletion and 14q duplication
Oligodontia, short stature and small head circumference with normal intelligence
Short tarsus — absence of lower eyelashes: an autosomal dominant condition
A de novo 6q11—q15 duplication investigated by chromosome painting
Preface
Welcoming address by Nils Rettersøl Chairman of the Board of the Sig. K. Thoresen Foundation
Identification of genetic variation that determines levels of plasma triglycerides and hypercoagulability
Heredity in dementia of the Alzheimer type
Clinical features and natural history of Beckwith‐Wiedemann syndrome: presentation of 74 new cases
Osteoarthropathia psoriatica of one leg: a manifestation of somatic mosaicism?
The influence of genetic counselling in the era of DNA testing on knowledge, reproductive intentions and psychological wellbeing
DNA polymorphisms of human apolipoprotein A‐IV gene: frequency and effects on lipid, lipoprotein and apolipoprotein levels in a French population French population
A case of de novo interstitial deletion of chromosome 9(p12p13)
Association between genetic variation at the APO AI‐CIII‐AIV gene cluster and familial combined hyperlipidaemia
Changes in Lp(a) lipoprotein and other plasma proteins during acute myocardial infarction
Partial trisomy of the short arm of chromosome 18 due to inversion duplication and direct duplication
No effect of a BglI polymorphism at the renin (REN) locus on blood pressure level or variability Norwegians
A constitutional mutation within the retinoblastoma gene detected by PFGE
Prenatal detection of cri du chat syndrome on uncultured amniocytes using fluorescence <i>in situ</i> hybridization (FISH)
Thyroid cancer in a case with the Alagille syndrome
A rare silent C to T mutation in exon 7 of the low density lipoprotein receptor (LDLR) gene
Chromosome instability in lymphocytes from patients with celiac disease
Apolipoprotein B gene polymorphisms in ischemic heart disease and hypercholesterolemia: effects of age and sex
Roberts‐SC phocomelia syndrome: a case with additional anomalies
Autosomal dominant inheritance of thyroglossal duct cyst
Variability gene effects of DNA polymorphisms at the apo B, apo AI/C III and apo E loci on serum lipids: the Cardiovascular Risk in Young Finns Study
The Charcot‐Marie‐Tooth syndrome: clinical aspects from a population study in South Wales, UK
Cerebro‐facio‐articular syndrome of Van Maldergem: confirmation of a new MR/MCA syndrome
A variant of the cerebro‐oculo‐facio‐skeletal syndrome with congenital ectropion and a case of lamellar ichthyosis in the same family
Prader‐Willi‐like phenotype in fragile X syndrome
Achondroplasia with XXY karyotype
Discordance between direct and PHA‐stimulated chromosome preparations from neonates
The apolipoprotein C‐II variant apoC‐II<sub>Lys19→Thr</sub> is not associated with dyslipidemia in an affected kindred
Patient and parental attitudes toward genetic screening and its implications at an adult cystic fibrosis centre
Melkersson‐Rosenthal syndrome and de novo autosomal t(9;21)(p11;p11) translocation
Central nervous system abnormalities in chromosome deletion at 11q23 Japanese
Announcements
Characterization of β‐thalassemia mutations by denaturing gradient gel electrophoresis: patterns in the Mediterranean mutations Mediterranean
Familial occurrence of Hirschsprung's disease
Currarino triad (anorectal malformation, sacral bony abnormality and presacral mass) with partial trisomy of chromosomes 13q and 20p
Mixed gonadal dysgenesis and cell line differentiation. Case presentation and literature review
A <i>Rsal</i> RFLP at the human myelin protein zero (<i>MPZ</i>) locus
Familial mosaic Turner syndrome
Ambral syndrome and congenital generalized hypertrichosis
Opening remarks by Bjarne A. Waaler, President of the Norwegian Academy of Science and Letters
Traversing the biological complexity in the hierarchy between genome and CAD endpoints in the population at large
Gene environment interaction and plasma triglyceride levels: the crucial role of lipoprotein lipase
Apolipoprotein(a): structural and functional consequences of mutations in kringle type 10 (or kringle 4–37)
Genetic and environmental influences on LDL subclass phenotypes
The XbaI polymorphism at the apolipoprotein B locus and risk of atherosclerotic disease
DNA polymorphism at the locus for angiotensinogen I‐converting enzyme in Norwegian patients with myocardial infarction and controls
Complications to the complex inheritance of schizophrenia
The genetic structure of personality and learning: a phylogenetic model
Sister chromatid exchange in families with Angelman or Prader‐Willi syndrome
Interstitial deletion of 22q11 in DiGeorge syndrome detected by high resolution and molecular analysis
Diphallus and associated anomalies with balanced autosomal chromosomal translocation
Unilateral renal aplasia in X‐linked Kallmann's syndrome
Determination of haemophilia A carrier status from hair samples using polymerase chain reaction technique
Congenital cardiac defect in a patient with mosaic 45,X/46,XX,i(21q) karyotype
A cytogenetic register of trisomies in Scotland: results of the first 2 years (1989, 1990)
A new simple and rapid dual assay for AFP and free β hCG in screening for Down syndrome
Congenital myopathy with fiber type disproportion: a family with a chromosomal translocation t(10; 17) may indicate candidate gene regions
Exclusion of an elastin gene (ELN) mutation as the cause of pseudoxanthoma elasticum (PXE) in one family
An 87 bp deletion in exon 5 of the LDL receptor gene in a mother and her son with familial hypercholesterolemia
The gene for familial dystonia with myoclonic jerks responsive to alcohol is not located on the distal end of 9q Ashkenazi Jewish; Gentile; Swedish
A new human mtDNA polymorphism: tRNAGln/4336 (T→C)
Ethnic differences in the occurrence of the M1(ala<sup>213</sup>) haplotype of alpha‐1‐antitrypsin in asthmatic and non‐asthmatic black and white South Africans
Informativity of intragenic microsatellites for carrier detection and prenatal diagnosis of cystic fibrosis in the Italian population Italian population
Announcements
No effect of insertion/ deletion polymorphism at the ACE locus on normal blood pressure level or variability
Multiple coagulation defects and the Cohen syndrome
Two transthyretin mutations (glu42gly, his90asn) in an Italian family with amyloidosis Italian, Japanese, Portuguese, German
Plasminogen with type‐I mutation in the Chinese Han population Chinese Han population; Japanese population
Inbreeding and congenital heart diseases in a North Indian population
X‐linked recessive nephritis with mental retardation, sensorineural hearing loss, and macrocephaly
The Kabuki make‐up (Niikawa‐Kuroki) syndrome and isolated transient hyperphosphatasemia
Macrocephaly, distinct craniofacial appearance and spastic paraplegia: an autosomal recessive subtype of complicated spastic paraplegia
DNA carrier detection in X‐linked progressive cone dystrophy
Umbilical findings in Aarskog syndrome
Pitted enamel hypoplasia in tuberous sclerosis
Video display terminal — the risk of trisomy 18?
Rett syndrome: random X chromosome inactivation
Mild phenotype and normal gonadal function in females with 4p trisomy due to unbalanced t(X;4)(p22.1;p14)
Down syndrome associated with systemic lupus erythematosus: a mere coincidence or a significant association?
Down syndrome and male fertility: PCR‐derived fingerprinting, serological and andrological investigations
Etiological subgroups in non‐syndromic isolated cleft palate. A genetic‐epidemiological study of 52 Danish birth cohorts
Familial translocation (X;3) (p22.3;p23): chromosomal in situ suppression (CISS) hybridization and inactivation pattern study
Patient with <i>de novo</i> 12p+ syndrome identified as dir dup (12) (p13) using subchromosomal painting libraries from somatic cell hybrids
Antley‐Bixler syndrome: report of a patient and review of literature
New allele of probe D17S61 present in the Charcot‐Marie‐Tooth 1A duplication
Studies on the structure and function of the apolipoprotein(a) gene
Importance of Lp(a) lipoprotein and HLA genotypes in atherosclerosis and diabetes
High Lp(a) lipoprotein level in maternal serum may interfere with placental circulation and cause fetal growth retardation
Mice transgenic for the human LCAT gene
Colorectal cancer: lessons for genetic counselling and care for families European countries
Heredity in personality disorders — an overview
Adults with Williams‐Beuren syndrome: evaluation of the medical, psychological and behavioral aspects
Predictive testing for Huntington disease: social characteristics and knowledge of applicants, attitudes to the test procedure and decisions made after testing
Microcephaly with large anterior fontanelle, generalized convulsions, micropenis, and distinct anomalies of the hands and feet. Another example of Wiedemann syndrome?
An alanine<sup>29</sup>‐serine variant in exon 2 of the low density lipoprotein receptor gene: no association with hypercholesterolemia
Inv dup(15): contribution to the clinical definition of phenotype
Analysis of 40 known cystic fibrosis mutations in South African patients
Search for three known mutations in the RYR1 gene in 48 Danish families with malignant hyperthermia
Clinical, ultrastructural and biochemical studies in two sibs with Ehlers‐Danlos syndrome type VI‐B‐like features
Chediak‐Higashi syndrome with cerebellar cortical atrophy detected by MRI
Occipital horn syndrome: report of a patient and review of the literature
Male with type II autosomal recessive cutis laxa
Myoclonic epilepsy and a maternally derived deletion of 15pter→13
Announcements
Metaphase quality can be monitored by automatic counting of bands
Minimal genetic influences on plasma fibrinogen level in adult males in the NHLBI twin study
A woman with an apparent non‐mosaic 45,X delivered a 46,X,der(X) liveborn female
X‐linked mental retardation exhibiting linkage to DXS255 and PGKP1: a new MRX family (MRX14) with localization in the pericentromeric region French family
Fine mapping of X‐linked clasped thumb and mental retardation (MASA syndrome) in Xq28
Fragile X syndrome with extra microchromosome
Hereditary pubertal genu valgum in Iran
The primary hereditary form of distal renal tubular acidosis: clinical and genetic studies in 60‐member kindred
Onychotrichodysplasia and chronic neutropenia without mental retardation (ONS): a second case report European patients; Mexican ancestry
Effects of consanguinity on anthropometric measurements of newborn infants
Announcement
De novo isochromosome 18p in two patients: cytogenetic diagnosis and confirmation by chromosome painting
A new case of “complete” trisomy 5p with isochromosome 5p associated with a de novo translocation t(5;8)(q11;p23)
A PCR‐based test for a polymorphism within the human NF1 gene
Linkage analysis of 62 X‐chromosomal loci excludes the X chromosome in an Icelandic family showing apparent X‐linked recessive inheritance of neural tube defects Icelandic
Increased frequency of apolipoprotein B signal peptide sp24/24 in patients with coronary artery disease. General allele survey in the population of Taiwan and comparison with Caucasians Chinese Han ethnic origin; Taiwanese
Allele frequencies of Mp6D‐9 and GATT markers in 32 Turkish cystic fibrosis families
Announcements
The KBG syndrome: follow‐up data on three affected brothers
HLA‐A, B and DR antigens in patients with gonadal dysgenesis
Marfan and cri du chat syndromes in an 18‐month‐old child: evidence of phenotype interaction
A stigmatizing effect of the carrier status for cystic fibrosis?
Prenatal sex determination by in situ hybridization on fetal nucleated cells in maternal whole venous blood
Molecular characterisation of an Italian G6PD variant responsible for chronic non‐spherocytic haemolytic anaemia arisen independently in Europe and Asia; Italian; Japanese (Japanese subject); Europe; Asia
Identification of a non‐fluorescent isodicentric Y chromosome by molecular cytogenetic techniques
Confounding results of Lp(a) lipoprotein measurements with some test kits
From Ag phenotyping to molecular genetics: apolipoprotein B, serum lipid levels and coronary artery disease in Finland Finnish; Finns; Finnish population; Finnish individuals
Evidence for single gene contributions to hypertension and lipid disturbances: definition, genetics, and clinical significance
Genetic markers in hypercholesterolemic and normocholesterolemic Czech children Czech
The angiotensin‐converting enzyme (ACE) genetic polymorphism: its relationship with plasma ACE level and myocardial infarction
Towards acceptable practices for antenatal and neonatal screening for disease or disease risk
A <i>Bgl</i> I polymorphism in the human elastin gene (ELN)
Announcements
DNA polymorphisms at the locus for human cholesteryl ester transfer protein (CETP) are associated with macro‐ and microangiopathy in non‐insulin‐dependent diabetes mellitus
Linkage between severe atopy and chromosome 11q13 in Japanese families Japanese
A 12‐year preventive program for β‐thalassemia in Northern Sardinia
Complex chromosomal rearrangements: some breakpoints may have cellular adaptive significance
Possible localization of a major gene for cleft lip and palate to 4q
Exclusion of linkage to 14q23‐24 in a family with Holt‐Oram syndrome
Single mandibular incisor in a patient with del (18p) anomaly
No effect on blood pressure level or variability of polymorphisms in DNA at the locus for atrial natriuretic factor (ANF) Norwegians