Journal of Medical Genetics - 1989

244 articles | Last updated: 2025-12-03 14:12:57
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T A T A T A T A
Catalog of Chromosome Aberrations in Cancer
A summary of 7q interstitial deletions and exclusion mapping of the gene for beta-glucuronidase.
Familial mental retardation associated with balanced chromosome rearrangement rcp t(8;11)(q24.3;p15.1).
Pituitary function studies in a case of mild Hunter's syndrome (MPS IIB).
Dystrophy: a revised definition.
Unknown syndrome: microcephaly, hypoplastic nose, exophthalmos, gum hyperplasia, cleft palate, low set ears, and osteosclerosis.
Informative Morphogenetic Variants in the Newborn Infant
Twinning and Twins
Genetic and Perinatal Effects of Abused Substances
Prenatal exclusion testing for Huntington's disease: a problem of too much information.
Does lumbosacral spina bifida arise by failure of neural folding or by defective canalisation?
Unilateral absence of the hand in second cousins.
An unusual case of mosaic Down's syndrome involving two different Robertsonian translocations.
Hirschsprung's disease, distinctive facies, and microcephaly.
Synchrony of oculocutaneous albinism, the Prader-Willi syndrome, and a normal karyotype.
Deletion (12)(q15q21.2).
Textbook of Human Genetics
Different options for prenatal testing for Huntington's disease using DNA probes.
The Cytogenetics of Mammalian Autosomal Rearrangements
Differential expression of the ICF (immunodeficiency, centromeric heterochromatin, facial anomalies) mutation in lymphocytes and fibroblasts.
Identification of the haplotype pattern associated with the mutant PKU allele in the Gypsy population of Wales. Gypsy population of Wales
De novo terminal deletion 7p22.1--pter in a child without craniosynostosis.
Correspondence
21-hydroxylase deficiency families with HLA identical affected and unaffected sibs.
The use of low density lipoprotein receptor activity of lymphocytes to determine the prevalence of familial hypercholesterolaemia in a rural South African community.
Hydrocephalus, tall stature, joint laxity, and kyphoscoliosis: a new inherited disorder of connective tissue?
Iris coloboma, ptosis, hypertelorism, and mental retardation.
The calcitonin-CGRP gene in the infantile hypercalcaemia/Williams-Beuren syndrome.
Transient nephrotic syndrome after anaesthesia resulting from a familial cryofibrinogen precipitating at 35 degrees C.
A single maxillary incisor as a manifestation of an ectodermal dysplasia.
Maternal translocation (9;18) with two abnormal offspring each with different chromosome derivatives.
The Foundations of Human Genetics
Choices, Not Chances--An Essential Guide to your Heredity and Health
Linkage disequilibrium in Huntington's disease: an improved localisation for the gene.
Fountain's syndrome: mental retardation, sensorineural deafness, skeletal abnormalities, and coarse face with full lips.
Abnormal chromosome complement resulting from a familial inversion of chromosome 2.
The Association of Physicians of Great Britain and Northern Ireland, Oxford, 14 to 15 April 1989
Childhood deaths in Down's syndrome. Survival curves and causes of death from a total population study in Queensland, Australia, 1976 to 1985.
Winchester's syndrome.
Unknown syndrome: abnormal facies, hypothyroidism, postaxial polydactyly, and severe retardation: a third patient.
Unknown syndrome: pachygyria, joint contractures, and facial abnormalities.
Practical Genetic Counselling
Interstitial deletion of the long arm of chromosome 2 with normal levels of isocitrate dehydrogenase.
Elements of Medical Genetics
Spondyloenchondrodysplasia.
Deleted Yq in the sterile son of a man with a satellited Y chromosome (Yqs).
Abstracts of the meeting of the Clinical Genetics Society held on 22 and 23 September 1988 at the University of Aberdeen
Absent or hypoplastic extraocular muscles?
Demand for DNA probe testing in three genetic centres in Britain (August 1986 to July 1987).
The recurrence risks for mild idiopathic mental retardation.
Holoprosencephaly: variation of expression in face and brain in three sibs.
Thanatophoric dysplasia in identical twins.
Gastrointestinal abnormalities in the syndrome of mosaic trisomy 9.
Ultrasound in the perinatal necropsy.
Microcephaly, mental retardation, cataracts, and hypogonadism in sibs: Martsolf's syndrome.
Dominant inheritance of Scheuermann's juvenile kyphosis.
Unknown syndrome: congenital heart disease, choanal stenosis, short stature, developmental delay, and dysmorphic facial features in a brother and sister.
Genetic heterogeneity in Waardenburg's syndrome.
Endocrine Genes: Analytical Methods, Experimental Approaches, and Selected Systems
Duchenne Muscular Dystrophy
Severe Silver-Russell syndrome.
Unknown syndrome: Noonan-like craniofacial features, digital anomalies, and premature birth.
Murderous science and the abuse of genetics
Fetal Diagnosis of Genetic Defects
A study of familial hypercholesterolaemia in Iceland using RFLPs.
Cone-rod congenital amaurosis associated with congenital hypertrichosis: an autosomal recessive condition.
Human Mating Patterns
Correspondence
Possibilities and limitation of prenatal diagnosis and carrier determination for Duchenne and Becker muscular dystrophy using cDNA probes.
New mutation to Huntington's disease.
Congenital generalised bone dysplasias: a clinical, radiological, and epidemiological survey.
Deletion of chromosome 5q and familial adenomatous polyposis.
Duplication 6p and deletion 9p.
Teratogenicity of ergotamine.
18p- syndrome with partial sacral agenesis.
Cat eye syndrome associated with aganglionosis of the small and large intestine.
Unknown syndrome: congenital heart disease, ptosis, hypodontia, and craniosynostosis.
Unknown syndrome in sibs: microcephaly, seizures, mental retardation, congenital heart disease, and skeletal abnormalities.
Annual Review of Genetics
Non-random association between alleles detected at D4S95 and D4S98 and the Huntington's disease gene.
A genetic study of von Recklinghausen neurofibromatosis in south east Wales. I. Prevalence, fitness, mutation rate, and effect of parental transmission on severity.
Correspondence
Interstitial deletion of distal 13q associated with Hirschsprung's disease.
Joseph Adams (1756-1818).
Carrier detection and early diagnosis of Wilson's disease by restriction fragment length polymorphism analysis.
Early onset Cockayne's syndrome: case reports with neuropathological and fibroblast studies.
Identification and characterisation of a small marker chromosome using non-isotopic in situ hybridisation with X and Y specific probes.
Limb anomalies in the CHARGE association.
Becker muscular dystrophy (BMD) and Klinefelter's syndrome: a possible cause of variable expression of BMD within a pedigree.
A third case of de novo partial trisomy 4p.
Short rib syndrome without polydactyly.
Van der Woude syndrome and limb defects: the chance of recurrence.
Fetal valproate phenotype is recognisable by mid pregnancy.
Inherited Disorders of the Skeleton
Frontonasal dysplasia, congenital heart defect, and short stature: a further observation.
Lethal osteogenesis imperfecta.
A possible human homologue for the mouse mutant disorganisation.
Disorganisation: a model for 'early amnion rupture'?
Blepharophimosis plus ovarian failure: a likely candidate for a contiguous gene syndrome.
Research in Retinitis Pigmentosa
The Prevention and Avoidance of Genetic Disease
Advances in Human Genetics
Evidence for genetic heterogeneity in tuberous sclerosis.
Pitfalls in counselling of the blepharophimosis, ptosis, epicanthus inversus syndrome (BPES)
A non-centromeric C band variant on chromosome 11q23.2.
Autosomal dominant sneezing disorder provoked by fullness of stomach.
Molecular Basis of Inherited Disease
Ceroid-Lipofuscinoses. Batten Disease and Allied Disorders
Germinal mosaicism increases the recurrence risk for 'new' Duchenne muscular dystrophy mutations.
Choanal atresia as a feature of ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome.
Outcome of de novo balanced translocations ascertained prenatally.
Genetic aspects of tuberous sclerosis in the west of Scotland.
Congenital hypothyroidism, spiky hair, and cleft palate.
Alpha thalassaemia: a potential source of error in DNA linkage studies for adult polycystic kidney disease.
Obesity and abnormal behaviour associated with interstitial deletion of chromosome 18 (q12.2q21.1).
Partial trisomy 16q secondary to a maternal 9;16 translocation.
The Neural Crest
A genetic study of von Recklinghausen neurofibromatosis in south east Wales. II. Guidelines for genetic counselling.
Thanatophoric dysplasia in identical twins.
Del(4)(q33----qter): another case report of a child with mild dysmorphism.
Hereditary distal muscular atrophy with vocal cord paralysis and sensorineural hearing loss: a dominant form of spinal muscular atrophy?
Knowledge and perceptions of haemoglobinopathy carrier screening among general practitioners in Cardiff.
A terminal deletion (14)(q31.1) in a child with microcephaly, narrow palate, gingival hypertrophy, protuberant ears, and mild mental retardation.
Familial distal trisomy 8(q24.13----qter).
The association of Angelman's syndrome with deletions within 15q11-13.
Distribution of haptoglobin phenotypes in oesophageal and gastric cancer.
Spontaneous and induced chromosome breakage in chorionic villus samples: a cytogenetic approach to first trimester prenatal diagnosis of ataxia telangiectasia syndrome.
Medical genetics in Israel.
Interstitial deletion of 11q.
Molecular genetics as a diagnostic service.
Molecular genetics in the National Health Service in Britain.
Genetic services in the context of DNA probes: what do they cost?
Guidelines for DNA banking. Report of the Clinical Genetics Society working party on DNA banking.
Consanguinity related prenatal and postnatal mortality of the populations of seven Pakistani Punjab cities.
Further evidence localising the gene for Hunter's syndrome to the distal region of the X chromosome long arm.
Y chromosome specific probes identify breakpoint in a 45,X/46,X,del(Y)(pter----q11.1:) karyotype of an infertile male.
Toluene embryopathy: two new cases.
Heterochromatin: Molecular and Structural Aspects
Clinical variability of osteogenesis imperfecta linked to COL1A2 and associated with a structural defect in the type I collagen molecule.
Prenatal diagnosis of beta thalassaemia based on restriction endonuclease analysis of amplified fetal DNA.
Population frequencies of three DNA alleles linked to the Duchenne muscular dystrophy gene.
The SHORT syndrome: further delineation and natural history.
Triphalangeal thumb
Genome Analysis: a Practical Approach
Familial translocation t(9;16).
3M dwarfism: a study of two further sibs.
Further delineation of the Yunis-Varon syndrome.
The megacystis-microcolon-intestinal hypoperistalsis syndrome: a fatal autosomal recessive condition.
The molecular basis of beta thalassaemia in Bulgaria.
A new interstitial deletion of 4q (q21.1::q22.1).
Genetic heterogeneity and mitochondrial DNA heteroplasmy in Leber's hereditary optic neuropathy.
Porencephalic cyst in pycnodysostosis.
Association of 13q deletion and Hirschsprung's disease.
Dominantly inherited cleft lip and palate.
Advances in Human Genetics
Rhodanese isozymes in three subjects with Leber's optic neuropathy.
Partial deletion 8q without Langer-Giedion syndrome: a recognisable syndrome.
Sibs with tetrasomy 18p born to a mother with trisomy 18p.
Interstitial deletion of 2(q33q36) in a child with congenital abnormalities.
X linked or autosomal recessive? A new approach to an old problem.
Exclusion of the COL2A1 gene as the mutation site in diastrophic dysplasia.
Moore-Federman syndrome and acromicric dysplasia: are they the same entity?
X linked mental retardation: a family with a separate syndrome?
Chromosome studies of males in an institution for the mentally handicapped.
Current trends in the prevalence at birth of neural tube defects in Singapore.
T cell receptor beta chain polymorphisms are associated with cystic fibrosis.
Fragile X testing in a diagnostic cytogenetics laboratory.
Intelligence and cognitive profile in the fra(X) syndrome: a longitudinal study in 18 fra(X) boys.
A case of two inversion (10) recombinants in a family.
An exclusion map for facioscapulohumeral (Landouzy-Dejerine) disease.
Linkage analysis in the spinal muscular atrophy type of facioscapulohumeral disease.
A genetic linkage study of facioscapulohumeral (Landouzy-Dejerine) disease with 24 polymorphic DNA probes.
Hypohidrotic ectodermal dysplasia, primary hypothyroidism, and agenesis of the corpus callosum.
Auralcephalosyndactyly: a new craniosynostosis syndrome or a variant of the Saethre-Chotzen syndrome?
Angelman's syndrome and 15q11-13 deletions.
Asymmetry in chromosome pairing: a major factor in de novo mutation and the production of genetic disease in man.
Osteoglophonic dysplasia.
Duplication of distal 17q from a maternal translocation: an additional case with some unique features.
Abstracts of the meeting of the Clinical Genetics Society and the Clinical Molecular Genetics Society held on 30 and 31 March 1989 at the University of Southampton
Reproductive behaviour and consistent patterns of abnormality in offspring of Vietnam veterans
The Developing Human. Clinically Oriented Embryology
Medical Genetics Handbook
Johanson-Blizzard syndrome.
Apple peel syndrome in sibs.
Alternate centromere inactivation in a pseudodicentric (15;20)(pter;pter) associated with a progressive neurological disorder.
Central nervous system malformations in Mohr's syndrome.
Updated results of the thalassaemia prevention programme carried out in Latium.
Issues and Reviews in Teratology
Medical Genetics: Principles and Practice
Nucleic Acid Probes in Diagnosis of Human Genetic Diseases
Diagnostic and counselling difficulties using a fully comprehensive screening protocol for families at risk for tuberous sclerosis.
Partial monosomy 3q in a boy with short stature, developmental delay, and mild dysmorphic features.
Genetic studies on a new deficiency gene (PI*Ztun) at the PI locus.
Estimation of age dependent penetrance in facioscapulohumeral muscular dystrophy by minimising ascertainment bias.
Features of Turner's and DiGeorge's syndromes in a child with an X;22 translocation.
Discordant, non-syndromic, congenital diaphragmatic defects in sibs.
Are abortions more or less frequent once prenatal diagnosis is available?
Correspondence
Vascular Birthmarks: Pathogenesis and Management
Genetics and Alzheimer's Disease
European Society for Human Genetics. Satellite meeting on meiotic microspreading
Familial supravalvular aortic stenosis: a genetic study.
Correspondence
Haplotypes identified by 10 DNA restriction fragment length polymorphisms at the human low density lipoprotein receptor gene locus.
Congenital central hypoventilation syndrome and Hirschsprung's disease in half sibs.
Abstracts of the meeting of the Association of Clinical Cytogeneticists held on 5 to 7 July 1988 in Edinburgh
Benign muscular dystrophy: risk calculation in families with consanguinity.
Mapping our Genes. Genome Projects: How Big, How Fast?
Hypertrichosis cubiti (hairy elbows) and short stature: a recognisable association.
Prenatal diagnosis of the megacystis-microcolon-intestinal hypoperistalsis syndrome.
Single base pair alterations as the predominant category of mutation in type I osteogenesis imperfecta.
Mendelian Inheritance in Man. Catalogs of Autosomal Dominant, Autosomal Recessive, and X-linked Phenotypes
Kyphomelic dysplasia.
Linkage analysis of French families with facioscapulohumeral muscular dystrophy.
A workshop on facioscapulohumeral (Landouzy-Dejerine) disease, Manchester, 16 to 17 November 1988.
Anencephaly: a vanishing problem in Bedouins?
Recent Advances in Ectodermal Dysplasias
The clinical significance of de novo structural rearrangements and markers detected prenatally by amniocentesis
A case of interstitial deletion of 10q25.2----q26.1.
Cancer Cytogenetics
Emery-Dreifuss syndrome.
Interstitial deletion, del(4)(q33q35.1), in a mother and two children.
Correlation of clinical and deletion data in Duchenne and Becker muscular dystrophy.
Cystic fibrosis: after the gene.
Presymptomatic detection and prenatal diagnosis for myotonic dystrophy by means of linked DNA markers.
A hungarian study on Werdnig-Hoffmann disease.
Misdiagnosed normal fetus owing to undetected germinal mosaicism for DMD deletion.
Three different, non-mosaic sex chromosome abnormalities (direct cytotrophoblasts, mesenchymal core cultures, and abortus skin fibroblasts): implications for elucidating chorionic villi mosaicism.
Prader-Willi Syndrome: Selected Research and Management Issues
Stickler's syndrome.
Is there an autosomal recessive form of the split hand and split foot malformation?
A new syndrome of autosomal recessive nephropathy, deafness, and hyperparathyroidism.
Fertility in a male with trisomy 21.
Neural tube defect recurrence after 'partial' vitamin supplementation.
A new recessive syndrome of unusual facies, digital abnormalities, and ichthyosis.
The Status of the Human Embryo: Perspectives from Moral Tradition
Dominantly inherited cleft lip and palate in two families.
Cranioectodermal dysplasia (Sensenbrenner's syndrome).
Testing for cystic fibrosis using allelic association.
Sibs lacking characteristic features of duplication of distal 17q.
Ring chromosome 15 in a patient with features of Fryns' syndrome.
Duchenne muscular dystrophy in Wales: a 15 year study, 1971 to 1986.
Duchenne muscular dystrophy in Wales: impact of DNA linkage analysis and cDNA deletion screening.
Moebius' syndrome with unilateral cerebellar hypoplasia.