Clinical Genetics - 1993

147 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
Interstitial deletion del(17) (q21.3q23 or 24.2) syndrome
Announcements
Low frequency mosaicism of normal cells in a 16‐year‐old girl with trisomy 18
Acute adrenal crisis complicating hypertensive congenital adrenal hyperplasia due to 11β‐hydroxylase deficiency
<i>De novo</i> t(5p;21q) in a patient previously diagnosed as monosomy 21
Geneticists approach ethics: an international survey
Affected sibs with fragde X syndrome exhibit an age‐dependent decrease in the size of the fragile X full mutation
Eronen syndrome identical with DOOR syndrome?
Kallmann syndrome in two sisters with other developmental anomalies also affecting their father
Oxygen radicals potentiate the genetic toxicity of tobacco‐specific nitrosamines
A case of Johanson‐Blizzard syndrome complicated by diabetes mellitus
Familial thyroglossal duct cyst
High‐resolution chromosome analysis in autosomal recessive disorders: Laurence‐Moon‐Bardet‐Biedl syndrome
Attitudes towards prenatal diagnosis and selective abortion among patients with retinitis pigmentosa or choroideremia as well as among their relatives
Announcement
Fertility and the cri du chat syndrome
Chromosome aberrations in Rubinstein‐Taybi syndrome
Exclusion map of the gene for neuraminidase from 10(pter → p15.1)
Taurodontism of the mandibular first permanent molar distinguishes between the tricho‐dento‐osseous (TDO) syndrome and amelogenesis imperfecta
Correlates of genetic risk for non‐syndromic cleft lip with or without cleft palate
Ring chromosome 20 with loss of telomeric sequences detected by multicolour PRINS
A simplified method for detection of the mutations predominantly causing cystic fibrosis and phenylketonuria in Polish families
Second observation of Silver‐Russel syndrome in a carrier of a reciprocal translocation with one breakpoint at site 17q25
Retinitis pigmentosa: problems associated with genetic classification
Slipped capital femoral epiphysis associated with Rubinstein‐Taybi syndrome
Y chromosome mosaicism in the gonads, but not in the blood, of a girl with the Turner phenotype and virilized external genitalia
Further evidence for the location of the blepharophimosis syndrome (BPES) at 3q22.3‐q23
Molecular cytogenetic analysis of a familial pericentric inversion of chromosome 12
Apolipoprotein B gene DNA polymorphisms are associated with macro‐ and microangiopathy in non‐insulin‐dependent diabetes mellitus
Mosaic trisomy 16 in a thriving infant; maternal heterodisomy for chromosome 16
No effect of TaqI polymorphism at the human renal kallikrein (KLK1) locus on normal blood pressure level or variability
Apple peel intestinal atresia in siblings with ocular anomalies and microcephaly
Serum lipoprotein(a) levels in elderly black and white men in the Charleston Heart Study
Additional case of <i>de novo</i> interstitial deletion del(17)(q21.3q23) and expansion of the phenotype
Insertion/deletion (I/D) polymorphism at the locus for angiotensin I‐converting enzyme and myocardial infarction
Adenine for guanine substitution — pairs 5‘ to the apolipoprotein (APO) A4 gene: relation with hgh density lipoprotein cholesterol and APO A‐I concentrations
Frequency of the arylsulphatase A pseudodeficiency allele in the Spanish population Spanish population
Fluorescence <i>in‐situ</i> hybridisation and molecular studies used in the characterisation of a Robertsonian translocation (13<sub>q</sub>15<sub>q</sub>) in Prader‐Willi syndrome
Trisomy 13 (Patau syndrome) with an 11‐year survival
Intrafamilial variation in Leber hereditary optic neuropathy revealed by direct mutation analysis Swedish
A case of full triploidy (69,XXX) of paternal origin with unusually long survival time
Normal genetic variation at the low density lipoprotein receptor (LDLR) locus influences cholesterol levels in children Czechoslovakia; Czech children; population studied
Different clinical features in monozygotic twins: a case of 7q — syndrome
Normal immunological status in four patients with ectrodactyly‐ectodermal dysplasia‐clefting syndrome (EEC‐syndrome)
Huntington's disease: predictive testing and the molecular genetics laboratory
Deletion analysis maps ocular albinism proximal to the steroid sulphatase locus
Case reports of malformations associated with maternal diabetes: history and critique
Genotype analysis in cystic fibrosis in relation to the occurrence of diabetes mellitus
Neu‐Laxova syndrome: report of a case from Turkey Turkish parents; from Turkey
Miller postaxial acrofacial dysostosis syndrome. Follow‐up data of a family and confirmation of autosomal recessive inheritance
Prenatal diagnosis of congenital sialidosis
Congenital contractural arachnodactyly in two double second cousins: possible homozygosity Bedouin
Proposed: an international code of ethics for medical genetics
Frontonasal malformation and reciprocal translocation t(15;22)(q22;q13)
Parental origin of the supernumerary chromosome in trisomy 18
Infertility in carriers of two bisatellited marker chromosomes
Frontonasal dysplasia, lipoma of the corpus callosum and tetralogy of Fallot
Kaufman oculocerebrofacial syndrome: report of two new cases and further delineation Two unrelated Mexican girls; the mongoloid slanted eyes
46XY/47XYY mosaicism and fragile X
DNA polymorphisms of the apolipoprotein B gene are associated with obesity and serum lipids in healthy Indians in Singapore
Ambras syndrome: delineation of a unique hypertrichosis universalis congenita and association with a balanced pericentric inversion (8) (p11.2; q22) Greek
Anthropometric and craniofacial patterns in mentally retarded males with emphasis on the fragile X syndrome
Prenatal detection of an inverted X chromosome in a male
A family with autosomal dominant polycystic kidney disease not linked to chromosome 16p13.3 Sicilian origin
The Myhre syndrome: report of two cases
Dacryocystitis associated with osteopoikilosis
Reply to the letter from Woods and Smith
Neither uniparental disomy nor skewed X‐inactivation explains Rett syndrome
Xbal polymorphism in DNA at the apolipoprotein B locus is associated with myocardial infarction (MI)
Application of fluorescence <i>in situ</i> hybridization for early prenatal diagnosis of partial trisomy 6p/monosomy 6q due to a familial pericentric inversion
Familial syndactyly type III—report of a large pedigree
Familial exudative vitreoretinopathy: multiple modes of inheritance
Insertion /deletion (I/D) polymorphism at the locus for angiotensin I‐converting enzyme and parental history of myocardial infarction
Immunofluorescence imaging diagnosis of Fabry heterozygotes using confocal laser scanning microscopy
Twins and their mildly affected mother with Weaver syndrome
Hypoalphalipoproteinaemia and polymorphisms associated with reduced expression of the apolipoprotein A‐I gene and resolution of disputed paternity in a large English family
A balanced autosomal translocation (3;9) associated with primary hypogonadism and dorsal spine stenosis
Late‐onset familial amyloid polyneuropathy with the TTR Met 30 mutation in France French; Portuguese
Early onset cerebellar ataxia with retained tendon reflexes: prevalence and gene frequency in an Italian population Italian population; Northwestern Italy
On planting alfalfa and growing orchids: the cloning of the gene causing Huntington disease
The hand‐foot‐genital syndrome: on the variable expression in affected males
Chromosome painting using FISH (fluorescence <i>in situ</i> hybridization) with chromosome‐6‐specific library demonstrates the origin of a <i>de novo</i> 6q+ marker chromosome
Germinal mosaicism in a Duchenne muscular dystrophy family: implications for genetic counselling
Linkage analysis‐in a family with complete type congenital stationary night blindness with and without myopia
Satellite polymorphism or Y/autosome translocation?
Familial bilateral antecubital pterygia with severe renal involvement in nail‐patella syndrome
Frequency of the ΔF508 and exon 11 mutations in Norwegian cystic fibrosis patients
Phenotypic intrafamilial heterogeneity in cystic fibrosis
The breakpoints of the EEC syndrome (ectrodactyly, ectodermal dysplasia and cleft lip/palate) confirmed to 7q11.21 and 9p12 by fluorescence <i>in situ</i> hybridization
Association of Shokeir syndrome (congenital universal alopecia, epilepsy, mental subnormality and pyorrhea) and giant pigmented nevus
Effect of the fragile X anomaly on body proportions estimated by pedigree analysis
Application of molecular and cytogenetic techniques to the detection of a <i>de novo</i> unbalanced t(11q;21q) in a patient previously diagnosed as having monosomy 21
Prenatal screening for cystic fibrosis: attitudes and responses of participants
Mspl RFLP in exon 18 of the LDL receptor gene detectable by PCR
Bilateral vocal cord paralysis in Williams syndrome
Prenatal diagnosis of fragile X syndrome by direct detection of the dynamic mutation due to an unstable DNA sequence
Haplotype analysis at the low density lipoprotein receptor locus in normal and familial hypercholesterolemia Norwegian subjects Norwegian (Norwegian population/subjects)
Williams syndrome and subaortic stenosis
Suggestion of linkage between manic‐depressive illness and the enzyme phosphoglycolate phosphatase (PGP) on chromosome 16p Danish
Two simultaneous terminal deletions in the same patient: a one—vs two‐hit origin
Percussion myoedema in a Pakistani boy with Berardinelli Seip lipodystrophy syndrome
Four <i>RSAI</i> restriction fragment melting polymorphisms in the region of the insulin receptor gene encoding for the alpha subunit
Restrictive dermopathy: a disorder of skin differentiation with abnormal integrin expression
A mild form of mucolipidosis type III in four Baluch siblings
11q trisomy detected by fluorescence <i>in situ</i> hybridization
Disease profile of 400 institutionalized mentally retarded patients in Kuwait
Erythrocyte membrane cation carrier in Down syndrome
Guadalajara camptodactyly syndrome type I. A corroborative family
Characterization of a deleted Y chromosome in a male with Turner stigmata
Familial occurrence of hereditary renal adysplasia with Müllerian anomalies
A case of a female infant with simultaneous occurrence of <i>de novo</i> terminal deletions on chromosome 14q and 20p
Heritable unstable DNA sequences and hypermethylation associated with fragile X syndrome in Japanese families
Attitudes of Dutch general practitioners towards presymptomatic DNA‐testing for Huntington disease
Hyperkeratosis‐hyperpigmentation syndrome: a confirmative case
46,XY del(18) (q21.3q22.2) with mosaicism of r(18) and a milder form of the 18q— syndrome
Multipoint linkage analysis in X‐linked juvenile retinosclusis
Nager acrofacial dysostosis: minor familial manifestations supporting dominant inheritance
Del(2q) — cause of the wrinkly skin syndrome?
Identification of the chromosome 14 origin of a C‐negative marker associated with a 14q32 deletion by chromosome painting
Can cytomegalovirus cause brachydactyly? A case report and review of the literature
XbaI polymorphism of the apolipoprotein B gene and plasma lipid and lipoprotein response to dietary fat and cholesterol: a clinical trial
Screening of deletions and RFLP analysis in Turkish DMD/BMD families by PCR
Craniofacial anthropometric studies in Waardenburg syndrome type I
A family study on isolated congenital radial and tibial deficiencies in Hungary, 1975–1984
Malignant lymphoma in a Bloom's syndrome patient treated with insulin
Announcement
Ridge hypoplasia and ridge dissociation: minor physical anomalies in autistic children
Announcements
MCA/MR syndrome with features of Hallermann‐Streiff syndrome and 4q deficiency/ 14q duplication
Progressive pseudorheumatoid arthritis of childhood (PPAC) and normal adult height
Case of human chimerism detected by unbalanced chromosome translocation?
<i>De novo</i> DNA rearrangement in atypical facioscapulohumeral muscular dystrophy
Oral‐facial‐digital syndrome with fibular aplasia: a new variant
Alternate, adjacent 2 and 3:1 meiotic segregation products from a balanced t(13;18) (q12;q11) carrier
Consanguineous marriages among parents of patients with Down syndrome
Retinitis pigmentosa in Southern Africa
Cranial hemihypertrophy with ipsilateral naevoid streaks, intellectual handicap and epilepsy: a report of two cases
Fra(16)(q21) in a family with four spontaneous abortions
Cowden syndrome: report of a large family with macrocephaly and increased severity of signs in subsequent generations
<i>De novo</i> interstitial deletion of the long arm of chromosome 3:46, XX, del(3) (q25.1q26.1)
Hemophilia B in a 46,XX female probably caused by non‐random X inactivation
Infant mortality in myotonic dystrophy in Saguenay‐Lac‐St‐Jean: a historical perspective
A preliminary trial of couple screening for cystic fibrosis: designing an appropriate information leaflet
Familial complex chromosomal rearrangement resulting in duplication/deletion of 6q1c to 6q16
The DM mutation; diagnostic applications in the Finnish population Finnish population
Phenotype of 49,XXYYY
Identification of a supernumerary der(18) chromosome by a rational strategy for the cytogenetic typing of small marker chromosomes with chromosome‐specific DNA probes