Clinical Genetics - 1998

254 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
HotSpots — A new feature in Clinical Genetics
VSD, hypospadias and normal psychomotor development in a patient with inv dup 8(q13‐q21.2)
Aortic root dilatation in Ehlers‐Danlos syndrome types I, II and III A report of five cases
Rsal and Mael intragenic RFLPs in the human HERG gene
Congenital bilateral absence of vas deferens with a new missense mutation (P499A) in the CFTR gene
Photoanthropometric study of craniofacial traits in individuals with Prader‐Willi syndrome on short‐term growth hormone therapy
Extending a hand to GLI3: functionally different GLI3 mutations in postaxial polydactyly, Greig's cephalopolysyndactyly syndrome and Pallister—Hall syndrome
Mutation screening of the LDLR gene and ApoB gene in patients with a phenotype of familial hypercholesterolemia and normal values in a functional LDL receptor/apolipoprotein B assay
<i>Sac</i> I identifies a biallelic polymorphism in the coding sequence of the gamma subunit of the epithelial sodium channel (ENaC): a candidate gene for hypertension
Phenotypic spectrum of tetrasomy 12p and prenatal counseling: potential underestimation of severity
Genetic landmarks through philately – Gregor Johann Mendel (1822–1884)
Prevalence of homozygosity for the deleted alleles of glutathione S‐transferase mu (GSTMl) and theta (GSTTl) among distinct ethnic groutx from Brazil: relevance to enviromental carcinogenesis? Brazilian Black subjects; Amazonian Indians; African descendants; distinct Brazilian racial groups;
Frequency analysis of autosomal dominant cerebellar ataxias in Japanese patients and clinical characterization of spinocerebellar ataxia type 6
ACTH receptor mutation in a girl with familial glucocorticoid deficiency
The 4q‐syndrome: delineation of the minimal critical region to within band 4q31
Recessive or dominant? Reclassification in the molecular age
Exploring gene—gene interactions in the etiology of neural tube defects
A deletion involving exons 2–4 in the iduronate‐2‐sulfatase gene of a patient with intermediate Hunter syndrome
Multifactorial age‐related macular degeneration allelic with autosomal recessive Stargardt macular dystrophy
Molecular genetic analysis of the gene encoding the trifunctional enzyme MTHFD (methylenetetrahydrofolate‐dehydrogenase, methenyltetrahydrofolate‐cyclohydrolase, formyltetrahydrofolate synthetase) in
DGGE screening of mutations in mismatch repair genes (hMSH2 and hMLH1) in 34 Swedish families with colorectal cancer Swedish
High frequency of IgE receptor FcεRIß variant (Leu181/Leu183) in Kuwaiti Arabs and its association with asthma
Duchenne mouse knocked out by two blows
Implications of genotyping of spouses to limit investigation of children in genetic hemochromatosis
Glucose‐6‐phosphatase gene (727G→T) splicing mutation is prevalent in Hong Kong Chinese patients with glycogen storage disease type la
Serum lipids and apolipoprotein E phenotypes in identical twins reared apart<sup>1</sup>
Detection and incidence of cryptic Y chromosome sequences in Turner syndrome patients
Sex ratio and absence of uniparental disomy in spontaneous abortions with a normal karyotype
G<sub>2</sub> repair in Nijmegen breakage syndrome: G<sub>2</sub> duration and effect of caffeine and cycloheximide in control and X‐ray irradiated lymphocytes
Discordant repeat size and phenotype in Kennedy syndrome
Molecular and cytogenate analysis of an X/autosomal translocation: 45, X, dic(X;17)(p22.2;p13)
Two cases of prenatally diagnosed diaphragmatic hernia accompanied by the same undescribed chromosomal deletion (15q24 <i>de novo</i>)
Apolipoprotein E genotypes in offspring with a positive and negative family history of premature myocardial infarction Slovak
Partners with reciprocal translocations: genetic counseling for the ‘double translocation’
Jumping translocation with partial duplications and triplications of chromosomes 7 and 15
Association of long variants of the dopamine D4 receptor exon 3 repeat polymorphism with Parkinson's disease
Female external genitalia, absent uterus, and probable agonadism in a 46,XY infant with bilateral upper amelia
A case of Prader – Willi syndrome arising as a result of familial unbalanced translocation t(11;15)(q25;q13)
The use of a specific clinical history in counselling a family with the balanced translocation 46,XY,t(4;12)(p15.2;q21.3): viable offspring with partial monosomy 4p and trisomy 12q
Prion protein: when more is less and less is more
Prevalence of high affinity IgE receptor [FcεRIβ] gene polymorphisms in Kuwaiti Arabs with asthma Kuwaiti Arabs
Announcements
Developmental eye disease — a genome era paradigm
Failure to identify the ryanodine receptor G1021A mutation in a large North American population with malignant hyperthermia European populations; North American
Fragile X syndrome, mental retardation and macroorchidism
Genetic testing and insurance in the United Kingdom
Risk factors for mortality in young children living under various socio‐economic conditions in Lahore, Pakistan: with particular reference to inbreeding
No association between the androgen receptor gene CAG repeat and impaired sperm production in Swedish men
Inherited breast cancer: an emerging picture
Paternal uniparental disomy of chromosome 6 and transient neonatal diabetes mellitus
Coronary artery disease is not associated with the E298 → D variant of the constitutive, endothelial nitric oxide synthase gene
New insights into the molecular and genetic mechanisms underlying idiopathic epilepsies
Three novel <i>PROC</i> gene lesions causing protein C deficiency
Two common mutations in the CLN2 gene underlie late infantile neuronal ceroid lipoluscinosis
Methods to detect the RYR1 G742A and A1565C mutations associated with malignant hyperthermia using a PCR‐modified restriction sites technique
Lack of association between ACE gene polymorphisms and 4 and asthma in British and Japanese populations
Generalised muscle hypertonia with mask‐like face (Freeman‐Sheldon syndrome) in a Tanzanian girl
The Met54Leu polymorphism of paraoxonase (PON) enzyme gene is not a genetic risk factor for non‐insulin‐dependent diabetes mellitus in Finns Finns
A bone master gene, CBFAI, culpable in Cleidocranial dysplasia
Killed by the clock
A presynaptic Parkinson gene: α‐synuclein
Genotype ‐phenotype correlation in myotonic dystrophy Austrian
A clinical, cytogenetic and molecular study of ten probands with supernumerary inv dup (15) marker chromosomes
Association between asthma and an intragenic variant of CC16 on chromosome 11q13
Getting too excited: potassium channelopathies and newborn epilepsy
Physician knowledge and attitudes towards molecular genetic (DNA) testing of their patients
A new case of Ambras syndrome associated with a paracentric inversion (8) (q12; q22)
Presenilin 1 Met146Leu variant due to an A&amp;T transversion in an early‐onset familial Alzheimer's disease pedigree from Argentina South American; Argentina; Italian
Sclerosteosis: report of a case in a black African man a white population of Dutch ancestry; black; black African man
A new period in time for geneticists Positional cloning of the mouse circadian clock gene
Who are our ancestors? Insights from mitochondrial mutations: molecular time machines over the millennia
Diabetes and pancreatic agenesis
Rapid determination of trisomy 18 parental origin using fluorescent PCR and small tandem repeat markers: case reports
Vascular anastomoses leading to amelia and cutis aplasia in a dizygotic twin pregnancy
A case of insertional translocation involving chromosomes 2 and 4
No relation between apolipoprotein E alleles and obstructive sleep apnea
Developmental Biology: Frontiers for Clinical Genetics: Overgrowth syndromes and genomie imprinting: from mouse to man
DNA diagnosis of FRAXA and FRAXE in Chinese children with neurodevelopmental disorders and fragile X syndrome
The intermediate alleles of the fragile X CGG repeat in patients with mental retardation
A novel missense mutation (402C → T) in exon 1 in the EDA gene in a family with X‐linked hypohidrotic ectodermal dysplasia Danish families
Detection of mtDNA deletion in Pearson syndrome by two independent PCR assays from Guthrie card
Polymorphisms at the angiotensinogen (AGT) and angiotensin II type 1 receptor (AT1R) loci and normal blood pressure Norwegians
A novel point mutation associated with alkaptonuria
A novel Ashkenazi predisposing mutation Ashkenazi
Pendred syndrome and the importance of being sulphated: Pendred syndrome is caused by a mutation in a putative sulphate transporter gene (PDS) Everett et al. (1997) Nat Genet 17: 411–421
Wilms' tumor and gonadal dysgenesis in a child with the 2q37.1 deletion syndrome
A novel mutation of the β‐glucocererebrosidase gene associated with neurologic manifestations in three sibs
Complex chromosomal rearrangements associated with congenital erythrophagocytotic histiocytosis
Apparent normalisation of fetal renal size in autosomal dominant polycystic kidney disease (PKD1)
A novel Mbo II polymorphism in exon 15 of the human adenomatous polyposis coli gene
A new kind of mutation: dinucleotide deletions in Alzheimer's disease transcripts
Developmental Biology: Frontiers for Clinical Genetics: Limb development: molecular dysmorphology is at hand!
Clinical, biochemical and molecular findings in a two‐generation Morquio A family
Mild cystic fibrosis mutations in Southern Sweden with special reference to S549I and T338I
Genotyping of the polymorphic <i>N</i>‐acetyltransferase (NAT2) and loss of heterozygosity in bladder cancer patients
Noonan syndrome associated with central giant cell granuloma
Hemochromatosis: genetics helps to define a multifactorial disease
Recent progress in the molecular genetics of congenital heart defects
New insights into the genetics of lissencephaly
Spinal muscular atrophy: a disease of altered RNA metabolism?
Functional mosaic trisomy of 1q12 1q21 resulting from X‐autosome insertion translocation with random inactivation
The yeast genome and clinical genetics
Genetics of speech sounds great
Life expectancy in British Marfan syndrome populations
Spinal and bulbar muscular atrophy (SBMA): Somatic stability of an expanded CAG repeat in fetal tissues
Involvement of 9q22.1–31.3 region in pyloric stenosis
Monozygotic monoamniotic twins discordant for urethral and anal atresia with vesicorectal fistula: a favourable combination of defects
Genetic landmarks through philately — The Habsburg jaw
HotSpots
Genetic variants of the human obesity (<i>OB</i>) gene in subjects with and without Prader—Willi syndrome: comparison with body mass index and weight
Genetic variation in paraoxonase‐2 is associated with variation in plasma lipoproteins in Canadian Oji‐Cree
Severe malformations in males from families with osteopathia striata with cranial sclerosis
Molecularly proven hypochondroplasia with cloverleaf skull deformity: a novel association
Detection of more than 91% cystic fibrosis mutations in a sample of the population from Reunion Island and identification of two novel mutations (A309G, S1255L) and one novel polymorphism (L49L)
Two brothers with hypospadias, hypertelorism, upper lid coloboma and mixed type hearing loss: a new syndrome
Developmental delay, expressive aphasia, hypotonia and dysmorphism in two brothers: an X‐linked mental retardation syndrome?
Announcements
Genetic landmarks through philately — symbols in medicine
Predictive testing for Huntington's disease: I. Predictors of uptake in South Wales
Corneal arcus, case finding and definition of individual clinical risk in heterozygous familial hypercholesterolaemia
Cell proliferation rate and nuclear morphometry in Roberts syndrome
Mosaic supernumerary marker chromosome identified as a der(3) by FISH
Announcements
A genetic diagnostic survey in a population of 202 mentally retarded institutionalized patients in the south of Brazil
Severe mental retardation ‐ distal arthrogryposis in the upper limbs and complex chromosomal rearrangements resulting from a lOq25→qter deletion
Jagged gene responsible in Alagille syndrome
The Asn9 variant of lipoprotein lipase is associated with the — 93G promoter mutation and an increased risk of coronary artery disease Dutch males
Complete identification of cystic fibrosis transmembrane conductance regulator mutations in the CF population of Saguenay Lac‐Saint‐Jean (Quebec, Canada)
Severe cystic fibrosis associated with a AF508/R347H + D979A compound heterozygous genotype German father
Caught in the middle: two genes troubled by trinucleotide expansion in myotonic dystrophy
Finding the significance of stats: insights into FGFR3 activating mutations in Thanatophoric dysplasia
Evaluation of a clinically applicable mutation screening technique for genetic diagnosis of familial hypercholesterolemia and familial defective apolipoprotein B Danish families
Ehlers‐Danlos syndrome and type III collagen abnormalities: a variable clinical
Detection of factor V Leiden mutation in severe pre‐eclamptic Hungarian women Hungarian
Identification of microsatellite markers tightly linked to the <i>Gli2</i> putative zinc finger transcription factor gene
Prenatal identification of<i>de novo</i>marker chromosomes using micro‐FISH approach
A novel Dral polymorphism in the 3‘ untranslated region of human glucose‐6‐phosphatase gene: useful for carrier detection and prenatal diagnosis of glycogen storage disease type 1a
Announcements
Brother/sister siblings affected with Hunter disease: evidence for skewed X chromosome inactivation
Skewed X inactivation in manifesting carriers of Duchenne muscular dystrophy
Clinical, morphological and biochemical features in the familial articular hypermobility syndrome (FAHS): a family study
Maternal cell contamination of buccal smear samples in nursing neonates
Inherited microdeletion in Xp21.3–22.1 involved in non‐specific mental retardation
Chromosomal instability in Marinesco‐Sjögren Syndrome associated with acute myeloblastic leukemia
Developmental Biology: Frontiers for Clinical Genetics
The new face of an old villain ‐ cholesterol
Defects in the human homolog of Drosophila diaphanous in non‐syndromic deafness
Deletion of chromosome 3q proximal region gives rise to a variable phenotype
Normal growth in Angelman syndrome due to paternal UPD
Chimerism detected by an unbalanced chromosome translocation: an alternative hypothesis
Centromeric alphoid DNA heteromorphisms of chromosome 22 revealed by FISH‐technique
The fatal attraction of polyglutamine‐containing proteins
Now there are eight: CAG expansion underlies spinocerebellar ataxia type 7
Prenatal diagnosis of low level trisomy 15 mosaicism: review of the literature
Tetranucleotide repeat polymorphism at the CCSP gene
Cerebral aqueductal stenosis as a presentation of deletion 6q25‐qter
Apolipoprotein E alleles in mothers of trisomy 18 conceptuses
Internet databases for clinical geneticists ‐ an overview<sup>1</sup>
Identification of iduronate sulfatase gene alterations in 70 unrelated Hunter patients
Glycogen storage disease type II: identification of a dinucleotide deletion and a common missense mutation in the lysosomal α‐glucosidase gene Dutch, The Netherlands, France, French
Developmental Biology: Frontiers for Clinical Genetics
An unexpected cause for combined deficiency of coagulation factors V and VIII
Linkage analysis in a large Spanish family with X‐linked retinitis pigmentosa: phenotype—genotype correlation Spanish
Low frequency of <i>RET</i> mutations in Hirschsprung disease in Sweden Swedish population‐based samples; Sweden; Swedish
Analysis of an interstitial deletion in a patient with Kallmann syndrome, X‐linked ichthyosis and mental retardation
Familial hypercholesterolemia: potential diagnostic value of mutation screening in a pediatric population of South Africa South African Afrikaners
SKALP/elafin gene polymorphisms are not associated with pustular forms of psoriasis
A hermaphrodite chimera resulting from in vitro fertilization
Molecular analysis of the BRCA2 gene in 16 breast/ovarian cancer Spanish families Spanish families
Polymorphic variants within the homeobox gene <i>MSX1: a candidate gene for developmental disorders</i>
Map refinement of the Usher syndrome type 1B gene, MYO7A, relative to 11q13.5 microsatellite markers
The common features of patients with partial trisomy of the long arm of chromosome 1
Pallister—Killian syndrome [i(12p)]: first pre‐natal diagnosis using cordocentesis in the second trimester confirmed by <i>in situ</i> hybridization
Isolation of a 370 kb YAC fragment spanning a translocation breakpoint at 3p14.1 associated with holoprosencephaly
Charcot‐Marie‐Tooth phenotype produced by a duplicated PMP22 gene as part of a 17P trisomy‐translocation to the X chromosome
Prenatal diagnosis of a trisomy 17p derived from a <i>de novo</i> non‐mosaic satellited marker
HotSpots
The long and the short of it: developmental genetics of the skeletal dysplasias
Carrier detection in Duchenne and Becker muscular dystrophy Argentine families
The evaluation of 15q proximal duplications by FISH
Ethical considerations for enzyme replacement therapy in neuronopathic Gaucher disease
Severe classical congenital muscular dystrophy and merosin expression
Molecular basis of cystic fibrosis in the Republic of Macedonia
A family with three germline mutations in <i>BRCAl</i> and <i>BRCA2</i> Ashkenazi Jewish
Dinucleotide repeat polymorphism within the tumor suppressor gene <i>PTCH</i> at 9q22
Distribution of CAG repeats in normal. and Huntington's disease patients in Israel
Acheiropodia: new cases from Brazil
Erratum
Lumping and splitting: molecular biology in the genetics clinic
22q11.2 deletions in a series of patients with non‐selective congenital heart defects: incidence, type of defects and parental origin
Characterization of a novel mutation in exon 10 of the adrenoleukodystrophy gene
Toward understanding the pathogenesis of craniosynostosis through clinical and molecular correlates
Holoprosencephaly: from Homer to Hedgehog
A highly polymorphic CA/GT repeat (LIMK1GT) within the Williams syndrome critical region
Kabuki (Niikawa‐Kuroki) syndrome associated with immunodeficiency
Short polyalanine expansion: a new class of triplet repeat disorder
Murine MPS I: insights into the pathogenesis of Hurler syndrome
Applicability of LDLR flanking microsatellite polymorphisms for prenatal diagnosis of homozygous state for familial hypercholesterolemia
A <i>‘de novo’</i> point mutation of the low‐density lipoprotein receptor gene in an Italian subject with primary hypercholesterolemia Italian
Deletion in chromosome region 22q11 in a child with CHARGE association
Neuronal nitric oxide synthase, nNOS, is not linked to infantile hypertrophic pyloric stenosis in three families
Phenotypic and genotypic variability in monozygotic triplets with Turner syndrome
<i>De novo</i> direct duplication 2 (p12 p21) with paternally inherited pericentric inversion 2p11.2 2q12.2
Autosomal dominant inheritance of adducted thumbs and other digital anomalies
Marden—Walker syndrome versus isolated distal arthrogryposis: Evidence that both conditions may be variable manifestations of the same mutated gene
Infectious complications of propionic acidemia in Saudia Arabia
Polymorphic exonic CAG microsatellites in the gene amplified in breast cancer (<i>AIB1</i> gene)
Apolipoprotein E and A‐IV polymorphisms in the Estonian population
A gene‐dosage PCR method for the detection of elastin gene deletions in patients with Williams syndrome
Neuropathological findings in Moebius syndrome
Variants of B cell lymphoma 6 (<i>BCL6</i>) and marked atopy
Predictive testing for Huntington's disease: II. Qualitative findings from a study of uptake in South Wales
Natural born killer: tau protein and its role in dementia
Analysis of the transactivation domain of the androgen receptor in patients with male infertilitv Indian or Middle‐Eastern subjects; Chinese
Identification of two novel mutations in the <i>OCRLI</i> gene in Japanese families with Lowe syndrome
A <i>de novo</i> mutation (C755T; Ser252Phe) in exon 6 of the proteolipid protein gene responsible for Pelizaeus‐Merzbacher disease
Genetics of Parkinson's disease
The incidence of cystic fibrosis in Scotland calculated from heterozygote frequencies
Failure to identify the ryanodine receptor G1021A mutation in a large North American population with malignant hyperthermia
A new feature in Clinical Genetics
Genotype-phenotype correlation in myotonic dystrophy
22q11.2 deletions in a series of patients with non-selective congenital heart defects: incidence, type of defects and parental origin
An unusual fragile X sibship: female compound heterozygote and male with a partially methylated full mutation
Severe cystic fibrosis associated with a DeltaF508/R347H+D979A compound heterozygous genotype
ACTH receptor mutation in a girl with familial glucocorticoid deficiency
X‐inactivation pattern in the liver of a manifesting female with ornithine transcarbamylase (OTC) deficiency
Complete identification of cystic fibrosis transmembrane conductance regulator mutations in the CF population of Saguenay Lac-Saint-Jean (Quebec, Canada)
Associations of angiotensinogen gene mutations with hypertension and myocardial infarction in a gulf population
Autosomal recessive familial exudative vitreoretinopathy: evidence for genetic heterogeneity
Genetic landmarks through philately - The Habsburg jaw
t(1;18)(q32.1;q22.1) associated with genitourinary malformations
Association between asthma and an intragenic variant of CC16 on chromosome 11q13
Presence of the AZF region in a female with an idic(Y)(q11)
Autosomal dominant carpal tunnel syndrome
The new Clinical Genetics: towards the millennium
Developmental eye disease - a genome era paradigm
Lumping and splitting: molecular biology in the genetics clinic
HotSpots
The Asn9 variant of lipoprotein lipase is associated with the -93G promoter mutation and an increased risk of coronary artery disease
The 4q-syndrome: delineation of the minimal critical region to within band 4q31
VSD, hypospadias and normal psychomotor development in a patient with inv dup 8(q13-q21.2)
Pallister-Killian syndrome [i(12p)]: first pre-natal diagnosis using cordocentesis in the second trimester confirmed by in situ hybridization
Unusual clinical features in an Angelman syndrome patient with uniparental disomy due to a translocation 15q15q
Frequency analysis of autosomal dominant cerebellar ataxias in Japanese patients and clinical characterization of spinocerebellar ataxia type 6
Mexican geneticists' opinions on disclosure issues
Molecular analysis of two pre‐mutations in myotonic dystrophy
A clinical, cytogenetic and molecular study of ten probands with supernumerary inv dup (15) marker chromosomes
I1307K <i>APC</i> and <i>hMLH1</i> mutations in a non‐Jewish family with hereditary non‐polyposis colorectal cancer French Canadian; Ashkenazi Jewish origins
Identification and characterization of a<i>de novo</i>partial trisomy 10p by comparative genomic hybridization (CGH)
Fragile X syndrome, mental retardation and macroorchidism
Two sisters with different chromosomal microdeletions: Rubinstein — Taybi syndrome and 22q deletion syndrome
Fragile X and Rett syndromes in Puerto Rico
Erratum
Variants of B cell lymphoma 6 (BCL6) and marked atopy