Clinical Genetics - 1990

156 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
Diagnostic molecular genetics of the fragile X
Consanguinity and the genetic control of Down syndrome
Skin mastocytosis with short stature, conductive hearing loss and microtia: a new syndrome Sephardic Jewish
Use of the RB1 cDNA as a diagnostic probe in retinoblastoma families
Inherited quantitative DNA variation in the LPA (“apolipoprotein (a)”) gene
Prenatal diagnosis in Pelizaeus‐Merzbacher disease using RFLP analysis Finnish
On the familial occurrence of congenital bilateral absence of vas deferens
Somatic and germ‐line mosaicism in autosomal dominant antecubital pterygium
CSF and urine biogenic amine metabolites in Rett syndrome
Apolipoprotein A‐l/C‐lll gene cluster polymorphism in Saudi Arabians, Filipinos and Caucasians Saudi Arabian, Filipino, Arab
Sotos syndrome and de <i>novo</i> balanced autosomal translocation (t(3;6)(p21;p21))
Monosomy X found at first trimester CVS: a diagnostic and counselling dilemma
The female and the fragile X syndrome: data on clinical and psychological findings in 7 fra(X) carriers
21st Symposium of the European Society of Human Genetics GENETICS IN CANCER &amp; DEVELOPMENT Groningen May 11–13, 1989 Abstracts
A familial interstitial deletion of the long arm of chromosome 21
The cis‐configuration effects of rare chromosomal fragile sites
Announcements
Retinitis pigmentosa: genetic mapping in X‐linked and autosomal forms of the disease Irish origin; Irish pedigree; United States
Analysis of mosaic states in amniotic fluid using the <i>in‐situ</i> colony technique
Time trends (1980–1987) of ten selected informative morphogenetic variants in a newborn population
Amelogenesis imperfecta with taurodontism and the tricho‐dento‐osseous syndrome: separate conditions or a spectrum of disease?
Families with X‐linked liver glycogenosis due to phosphorylase kinase deficiency
Spinal muscular atrophy type I combined with atrial septal defect in three sibs
Exclusion mapping of 12 X‐linked disease loci and 10 DNA probes from the long arm of the X‐chromosome
Morphologic variants in parents of children with malformation syndromes: are they indicators of somatic mosaicism?
Disomic balanced reciprocal translocation
Incontinentia pigmenti: XXY male with a family history
The Proteus syndrome: association with nephrogenic diabetes insipidus
Friedreich's ataxia: a descriptive epidemiological study in an Italian population
Very early onset Huntington's disease: genetic mechanism and risk to siblings
Bone marrow transplantation in canine GM1 gangliosidosis
Brachy/ectrodactyly and absence or hypoplasia of the fibula: an autosomal dominant condition with low penetrance and variable expressivity Italian
Fetal hydrops in Sardinia: implications for genetic counselling Sardinian; Mediterranean populations
Genetic marker family studies in familial Mediterranean fever (FMF) in Armenians Armenians; Mediterranean and near Eastern populations
Primordial osteodysplastic dwarfism type I in association with corneal clouding: evidence for autosomal recessive inheritance
The Niikawa‐Kuroki (Kabuki make‐up) syndrome in a Moslem Arab child Moslem Arab; non-Japanese; Arab descent; Japanese
Association of DNA‐haplotypes in the human LDL‐receptor gene with normal serum cholesterol levels from Germany
A possible relationship between Beckwith‐Wiedemann syndrome, urinary tract anomaly and prune belly syndrome
Excess of HLA parental sharing in families with Turner patients
Emery‐Dreifuss syndrome in three generations of females, including identical twins
Clinical expression of Menkes syndrome in females
Re‐evaluation of GM2346 from a del(16)(q22) to t(4;16)(q35;q22.1)
Diagnosis of familial amyloidotic polyneuropathy in France French; origin in Germany; family from Picardy; family from Illinois (with origin in Germany)
Announcements
A cost‐benefit analysis of prenatal diagnosis by amniocentesis in Denmark
Batten's disease: failure of allogeneic bone marrow transplantation to arrest disease progression in a canine model
RFLP analysis for diagnosis of haemophilia A in the German Democratic Republic
Maternal serum markers in screening for Down syndrome
Lack of specificity of DA/DAPI fluorescence
Defective oxidation of pristanic acid by fibroblasts from patients with disorders in propionic acid metabolism
Mental retardation, craniofacial dysmorphism, hypogonadism, diabetes mellitus and epilepsy in four siblings. A “new” mental retardation syndrome
Deletion of chromosome 1p: a short review
Trisomy 17p due to a t(8; 17) (p23; p11.2)pat translocation. Case report and review of the literature
Proximal 15q variant as possible pitfall in the cytogenetic diagnosis of Prader‐Willi syndrome
A case of 46,XX,r(X) (p1q1) diagnosed by<i>in situ</i>hybridization
A linkage study of malignant hyperthermia (MH) German families
High resolution banding of an unusual reciprocal translocation in recurrent abortions
Three‐generation transmission of Hirschsprung's disease
Terminal 7q deletion as a cause of holoprosencephaly
Phenotype of two males with abnormal Y chromosomes
Marfan syndrome: a diagnostic dilemma
Haplotype analysis for CF‐linked DNA polymorphisms in Switzerland
Rate of recombination of chromosomes 21 in parents of children with Down syndrome
Restriction site polymorphism at the LPA (Lp(a) apoliprotein; apoliprotein(a)) locus
Duplication 3p and cyclopia
Real effects of consanguinity
Ratio of fetal to maternal DNA is less than 1 in 5000 at different gestational ages in maternal blood
Rhizomelic chondrodysplasia punctata and survival beyond one year: a review of the literature and five case reports
Megalocornea, macrocephaly, mental and motor retardation (MMMM)
Sporadic late onset ornithine transcarbamylase deficiency in a boy with somatic mosaicism for an intragenic deletion
Familial caudal dysgenesis: evidence for a major dominant gene
Hemophilia B: diagnostic value of RFLP analysis in 19 of the 20 known Finnish families
Congenital cardiovascular malformations (CCVM) and structural chromosome abnormalities: a report of 9 cases and literature review
Lacrimo‐auriculo‐dento‐digital (LADD) syndrome with renal and foot anomalies
Reproductive planning after genetic counselling: a perspective from the last decade
Neurofibromatosis 2: a clinically and genetically heterogeneous disease? Report on 10 sporadic cases
Autosomal dominant craniosynostosis of the sutura metopica
Letters to the Editors
Exclusion mapping of the Cohen syndrome gene from the Prader‐Willi syndrome locus
Common intragenic and extragenic polymorphisms of blood coagulation factors VIII and IX are different in Chinese and Caucasian populations Chinese population
Craniofrontonasal dysostosis: variable expression in a three‐generation family
Normal size of hands and feet in Prader‐Willi syndrome
Abstract
Molecular Genetics of Diseases: Clinical Applications
Hereditary amyloidosis: detection of variant prealbumin genes by restriction enzyme analysis of amplified genomic DNA sequences
Serum protein markers in Chinese schizophrenics ‐ haptoglobin types and transferrin and group‐specific component subtypes
Premature centromeric divisions and prominent telomeres in a patient with persistent Mullerian duct syndrome
Radial ray defects and associated anomalies; unique nature of the radial deficiencies and facial dysmorphism in the TAR syndrome
Charcot‐Marie‐Tooth disease: call for patients
A dicentric variant of chromosome 6: characterization by use of <i>in situ</i> hybridisation with the biotinylated probe p308
Fraser syndrome (cryptophthalmos with syndactyly) in the fetus and newborn
5p;12q translocation with manifestations of cri du chat syndrome and Marfanoid arachnodactyly
Genetic counselling in Duchenne and Becker muscular dystrophy is problematic when carrier studies give controversial results Finnish families
A child with cystic fibrosis: I. Parental knowledge about the genetic transmission of CF and about DNA‐diagnostic procedures
A child with cystic fibrosis: II. Subsequent family planning decisions, reproduction and use of prenatal diagnosis
Announcements
Xbal polymorphism of the apolipoprotein B gene influences plasma lipid response to diet intervention
Hunter disease (mucopolysaccharidosis type II) in a karyotypically normal girl
Robinow syndrome: report of two patients with cystic kidney disease
Neurodevelopmentai models of mental illness
Fraser syndrome and mouse ‘bleb’ mutants
Autosomal dominant familial spastic paraplegia: report of a large New England family
Abnormalities of the cerebellum in oro‐facio‐digital syndrome II (Mohr syndrome)
Trisomy of chromosome 20/isochrome 12p
Heart‐hand syndrome II. A report of Tabatznik syndrome with new findings
Right‐sided microtia and conductive hearing loss with variable expressivity in three generations
Partial trisomy 4p resulting from a balanced intrachromosomal insertion, 4(q313p14p16)
Congenital tracheal stenosis in Pfeiffer syndrome
Phenotypic variability in Meckel–Gruber syndrome Bedouin
Activity of glucocerebrosidase in extracts of different cell types from type 1 Gaucher disease patients Portuguese
Frontonasal dysplasia or craniofrontonasal dysplasia and the Poland anomaly?
Possible association of rare autosomal folate sensitive fragile sites and idiopathic mental retardation: a blind controlled population study
Interstitial deletion of the short arm of chromosome 4 in a boy with mild psychomotor retardation and dysmorphism
Letter to the Editors
Announcement
A new syndrome of aphalangy, hemivertebrae, and urogenital‐intestinal dysgenesis
Cardio‐facio cutaneous syndrome: neurological manifestations
Diabetes mellitus in a young man with Bloom's syndrome
Partial lipodystrophy syndromes – a further male case
Homozygous C4A deficiency in systemic lupus erythematosus: analysis of patients from a defined population
Attitudes of parents of cystic fibrosis children towards neonatal screening and antenatal diagnosis
An isolated case of Duchenne muscular dystrophy (DMD) in a female with a deletion of DMD cDNA
Bes, Aesop and Morgante: reflections of achondroplasia
A new syndrome of familial short stature, small hands, valvular heart disease and a characteristic facies
Metachromatic leukodystrophy in Greece: observations on 4 cases
Clinical considerations in Buschke‐Ollendorff syndrome
The phakomatoses as paracrine growth disorders (paracrinopathies)
Complex familial translocation
The common fragile site in band q27 of the human X chromosome is not coincident with the fragile X
Comparison of anatomical location of squamous cell carcinoma within the oral cavity and oropharynx with the incidence of <i>in vitro</i> hyperdiploidy
X‐linked myotubular myopathy: a linkage study
Dominant branchial cleft syndrome with characteristics of both branchio‐oto‐renal and branchio‐oculo‐facial syndrome
How necessary is a chromosomal analysis in growth‐retarded girls?
Trisomy 20q. A new case and further phenotypic delineation
Consanguinity and confounding
Announcements
Monozygotic twins concordant for Rubinstein‐Taybi syndrome
Frequency and effects of the apolipoprotein A‐IV polymorphism
<i>In situ</i> hybridization analysis of isodicentric X‐chromosomes with short arm fusion
Intragenic deletions in 164 boys with Duchenne muscular dystrophy (DMD) studied with dystrophin cDNA
High urinary excretion of N‐(pyrrole‐2‐carboxyl) glycine in type II hyperprolinemia
Pseudoinflammatory fundus dystrophy: a follow‐up study
Chromosome breakage in lymphocytes from members of cancer families showing autosomal dominant inheritance
A male patient with 48,XXYY syndrome: importance of distinction from Klinefelter's syndrome
An evaluation of 75 terminations of pregnancy based on abnormal laboratory findings at first trimester CVS
Letter to the Editors
Autosomal dominant osteopetrosis type II with “malignant” presentation: further support for heterogeneity?
Association of hypercholesterolemia and apolipoprotein E4 in school children
Phenylalanine hydroxylase gene: silent mutation uncovers evolutionary origin of different alleles
X‐linked myopia: Bornholm Eye Disease
Gene‐gene interaction between the low density lipoprotein receptor and apolipoprotein E loci affects lipid levels
The wrinkly skin syndrome: a report of a case and review of the literature Jewish Moroccan
Amniotic fluid microvillar enzyme activity in fetal malformations
A familial form of convulsive disorder with or without mental retardation limited to females: extension of a pedigree limits possible genetic mechanisms
Three years' diagnostic experience with direct karyotyping of neonatal blood
Deficiency of distal 8p —: report of two cases and review of the literature