Clinical Genetics - 1992

154 articles | Last updated: 2025-12-03 14:12:56
Caucasian
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7
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21
Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
Linkage analysis in properdin deficiency families: refined location in proximal Xp
Consanguineous marriages among parents of Down patients
Congenital hypertrophy of retinal pigment epithelium in patients with colonic polyps associated with cancer family syndrome
Congenital heart defects, hamartomas of the tongue and polysyndactyly in a sister and brother
Variable dystrophin expression in different muscles of a Duchenne muscular dystrophy carrier
Autism and ring chromosome 18 mosaicism
Ring Y chromosome: cytogenetic and molecular characterization
Typical and partial cat eye syndrome: identification of the marker chromosome by FISH
Structural abnormalities of the Y‐chromosome and craniosynostosis
MASA syndrome
Congenital hypoparathyroidism, seizure, extreme growth failure with developmental delay and dysmorphic features—another case of this new syndrome
Pseudohypoparathyroidism type I and Albright's hereditary osteodystrophy with a proximal 15q chromosomal deletion in mother and daughter
Reproductive behaviour following spontaneous loss of a pregnancy after prenatal diagnosis
DNA polymorphisms of the apolipoprotein B gene in Chinese coronary artery disease patients
Ring chromosome 22 and neurofibromatosis
Deletion of 15q12 in Angelman syndrome: report of 3 new cases
Fertility in myotonic dystrophy in Saguenay‐Lac‐St‐Jean: a historical perspective French Canadians
The Dyggve‐Melchior‐Clausen syndrome in Indian siblings
Analysis of three glucose transporter genes in a Caucasian population: no associations with non‐insulin‐dependent diabetes and obesity
Deletion ΔF508 and clinical expression of cystic fibrosis‐related liver disease
Detection of the Pro<sub>664</sub>‐Leu mutation in the low‐density lipoprotein receptor and its relation to lipoprotein(a) levels in patients with familial hypercholesterolemia of Dutch ancestry from Dutch ancestry; Dutch descent
Autosomal recessive gingival fibromatosis with distinctive facies
Analysis of CA/GT microsatellite polymorphism in IVS8 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene: a study of Italian CF families Southern European populations; Italian, Spanish
Choanal atresia in two unrelated patients with the Coffin‐Siris syndrome
A Bedouin kindred with 19 piebalds in 5 generations
Familial spastic paraplegia with neuropathy and poikiloderma. A new syndrome?
20 p duplication as a result of parental translocation: familial case report and a contribution to the clinical delineation of the syndrome
A syndrome of progressive sensorineural deafness and cataract inherited as an autosomal dominant trait
Hair‐nail dysplasia ‐ a new pure autosomal dominant ectodermal dysplasia
One hundred requests for predictive testing for Huntington's disease
Fragile site Xq27.3 in a family without mental retardation
Trisomy 18 mosaicism with complete peripheral lymphocyte trisomy and normal intelligence
Autosomal recessive blepharophimosis, ptosis, V‐esotropia, syndactyly and short stature
Heterogeneity of mutations in Bulgarian phenylketonuria haplotype 1 and 4 alleles
Cytogenetic studies of skin fibroblast cultures from a karyotypically normal female with dyskeratosis congenita
Cystic fibrosis: community knowledge and attitudes towards carrier screening and prenatal diagnosis
Familial multiple lateral telangiectatic nevi (port‐wine stains or nevi flammei)
Familial Turner syndrome
Branchio‐oculo‐facial and branchio‐oto‐renal syndromes are distinct entities
G<sub>M1</sub>‐gangliosidosis: tandem duplication within exon 3 of β‐galactosidase gene in an infantile patient
Dosage analysis at the CSF1 and CSF1R loci in a new case of partial trisomy 5q
Wolman disease: morphological, clinical and genetic studies on the first Scandinavian cases "Scandinavian"; "ancestors from the same restricted area"; "common ancestors during the 17th century
Amyloidogenic and non‐amyloidogenic transthyretin Asn 90 variants Portuguese and German populations; American family of Italian origin
Elevated serum levels of creatine kinase BB in autosomal dominant osteopetrosis Type II — a family study
DNA screening of hyperlipidemic Afrikaners for familial hypercholesterolemia Afrikaner
Confirmation of true mosaic trisomy 20 in a phenotypically normal liveborn male
Pitted enamel hypoplasia in tuberous sclerosis
A novel truncated apolipoprotein B (apo B55) in a patient with familial hypobetalipo‐proteinemia and atypical retinitis pigmentosa
A study of ten small supernumerary (marker) chromosomes identified by fluorescence <i>in situ</i> hybridization (FISH)
Prevalence of retinitis pigmentosa in Slovenia
Reassessment of a chromosome 12q + marker by fluorescent <i>in situ</i> hybridization (FISH)
The gene for Best's macular dystrophy is located at 11q13 in a Swedish family Swedish
Digito‐reno‐cerebral syndrome: confirmation of Eronen syndrome
Autosomal inheritance of “senile” retinitis pigmentosa. A report of a family with consanguinity
Clinical variability of cardio‐facio‐cutaneous syndrome: report of two additional cases
Aminoacidopathies among institutionalised mentally retarded in Kuwait
A new polymorphism in exon 11 of the LDL receptor gene in healthy people and in familial hypercholesterolemia subjects
<i>De novo</i> interstitial deletion 16(q12. 1q13) of paternal origin in a 10‐year‐old boy
Detection of the Pro<sub>664</sub>‐Leu mutation in the low‐density lipoprotein receptor and its relation to lipoprotein(a) levels in patients with familial hypercholesterolemia of Dutch ancestry from Dutch ancestry; of Dutch descent
The Wolf‐Hirschhorn syndrome in fetuses
A 9.6 kilobase deletion in the low density lipoprotein receptor gene in Norwegian familial hypercholesterolemia subjects Norwegian; Finnish
X‐inactivation in girls with Rett syndrome
Screening for the apolipoprotein B‐100 arginine<sub>3500</sub>→ glutamine mutation in patients with type III hyperlipoproteinemia
Genotyping of spinal muscular atrophy families with linked DNA probes
Reevaluation of the origin of a marker chromosome in a patient with 47,XX,r(13)(p11q34), +mar by molecular cytogenetics
Cytogenetic and dermatoglyphic findings in a familial case of hypomelanosis of Ito (incontinentia pigmenti achromians)
Population variation and genetics of plasma homocyst(e)ine level Norwegians
Severe pre‐ and postnatal growth retardation, developmental delay with hypotonia and marked hypotrophy of the distal extremities, dental anomalies, and eczematous skin. A new autosomal recessive entit
Further evidence consistent with Yqh as an indicator of risk of gonadal blastoma in Y‐bearing mosaic Turner syndrome
Severe Silver‐Russell syndrome and translocation (17;20) (q25;q13)
Oculocerebral syndrome with hypopigmentation (Cross syndrome): report of a new case Amish
No effect of a Taql polymorphism in DNA at the endothelin I (EDN1) locus on normal blood pressure level or variability
Experiences with risk estimates for carriers of chromosomal reciprocal translocations
The trichorhinophalangeal syndrome with repeated dislocation of the patella
A new chromosome 9 variant: an extra band within the 9qh region
Monosomy 6q: report on four new cases
Phenotypic discriminants in the Waardenburg syndrome
Biochemical investigation of a Brazilian patient with a defect in mitochondrial acetoacetyl‐coenzyme‐A thiolase
Transmission of breast cancer ‐ a controversy resolved Danish
Reply to Dr. Lin
Polymorphisms at the GLUT2 (β‐cell/liver) glucose transporter gene and non‐insulin‐dependent diabetes mellitus (NIDDM): analysis in affected pedigree members Italian population
Lethal congenital erythroderma: a newly recognised genetic disorder
Interstitial deletion of 17pl 1.2 with brain abnormalities
DNA deletion in patients with von Recklinghausen neurofibromatosis
Y‐derived sequence detected in minute chromosomes by polymerase chain reaction and <i>in situ</i> hybridization
Occipital scalp defect associated with valvular pulmonary stenosis. A new entity?
Cleft palate and complex chromosome rearrangements
Leber hereditary optic neuropathy: estimation of number of embryonic precursor cells and disease threshold in heterozygous affected females at the X‐linked locus
Autosomal recessive microcephaly with early onset seizures and spasticity
The Charcot‐Marie‐Tooth syndrome: perceptions of disability and projected use of DNA diagnostic tests
Partial trisomy 8q. Two case reports with maternal translocation and inverted insertion: phenotype analyses and reflections on the risk
Clinical, cytogenetic and molecular investigations in three patients with Wolf‐Hirschhorn syndrome
Spondylar dysplasia (SD)/brachyolmia (BO), type I: search for qualitative anomalies in glycosaminoglycans (GAG)
A case of human chimerism detected by unbalanced chromosomal translocation
Posterior scalp defects in Opitz syndrome. Another symptom related to a defect in midline development
Identical twins with an autosomal recessive form of spondylocostal dysostosis
Announcements
Gaudier patients with oculomotor abnormalities do not have a unique genotype non‐Ashkenazic American
An HLA study in 74 Danish haemochromatosis patients and in 21 of their families Danish; Central Europe; Central Sweden; England; Ireland; Australia; USA; Canada
Birth prevalence studies of the Crouzon syndrome: comparison of direct and indirect methods
A unique <i>de novo</i> interstitial deletion del(17) (q21.3q23) in a phenotypically abnormal infant
The thymic findings in stillborns with neural tube defects
Autosomal dominant congenital cataract; linkage relations; clinical and genetic heterogeneity Danish ("Danish pedigree")
A new form of X‐linked, high‐frequency, sensorineural deafness
A boy with Poland anomaly and facio‐auriculo‐vertebral dysplasia
Hereditary motor and sensory neuropathy type I, associated with aplasia cutis congenital possible X‐linked inheritance
Announcements
Influence of apolipoprotein B signal peptide insertion/deletion polymorphism on serum lipids and apolipoproteins in a Chinese population Caucasians (France)
Nonrandom chromosome breakpoints in 6q deletions
Detection of subtle reciprocal translocations by fluorescence <i>in situ</i> hybridization
Familial occurrence of ring chromosome 15
Concomitant occurrence of mucopolysaccharidosis IIIB and Glanzmann's thrombasthenia. Further evidence of a hyperactive α‐<i>N</i>‐acetylglucosaminidase‐producing allele
Dominantly inherited microcephaly, short stature and normal intelligence
Deletions, duplications and novel restriction fragment length polymorphism in Duchenne and Becker muscular dystrophies Southern Chinese
A fragile X family with high penetrance in females: risk heterogeneity?
Time of pubertal onset, testosterone levels and intelligence in 47,XXY males
Association of haptoglobin types with serum lipids and apolipoproteins in a Chinese population
X‐linked lymphoproliferative disease: prenatal detection of an unaffected histocompatible male
EEC syndrome (ectrodactyly, ectodermal dysplasia and cleft lip/palate) is on 7p11.2‐q21.3
Putative monosomy 21 in two patients: clinical findings and investigation using fluorescence <i>in situ</i> hybridization
X chromosome inactivation patterns in haematopoietic cells of female carriers of X‐linked severe combined immunodeficiency determined by methylation analysis at the hypervariable DXS255 locus
Enzyme deficiencies as the cause of hereditary nonspherocytic hemolytic anemia
DNA probe technology: implications for service planning in Britain
Nucleotide sequence and PCR‐amplification of a polymorphic Mbol site in human DNA marker D4S95 linked to the Huntington disease locus
Prenatal prediction of cystic fibrosis in a mother homozygous for the ΔF508 mutation
StyI polymorphism in an enhancer region of the second intron of the apolipoprotein B gene in hyper‐ and hypocholesterolemic subjects Norwegian; Czech; Czech descent
β‐Thalassaemia mutations and their linkage to β‐haplotypes in Tamil Nadu in Southern India
Deciphering the fluorescent variability of human genomic heterochromatin by DA/DAPI technique
Intrachromosomal insertion of chromosome 7
Differentiated recurrence risk estimations in the Prader‐Willi syndrome
Retinitis pigmentosa, AD type I: exclusion of linkage to D3S47 (C17) in a large South African family of British origin British origin
Human β‐mannosidosis: a 3‐year‐old boy with speech impairment and emotional instability European descent
Chromosomal aberrations in Sotos syndrome
Mental retardation with blepharo‐naso‐facial abnormalities and hand malformations: a new syndrome?
Interstitial deletion of chromosome 1 del (1) (q32 q42): case report and review of the literature
Homozygosity for the transthyretin‐Met30‐gene in seven individuals with familial amyloidosis with polyneuropathy detected by restriction enzyme analysis of amplified genomic DNA sequences Swedish
On the variable expression of the Brachmann‐de Lange syndrome
Treatment for B‐cell‐type lymphoma in a girl associated with Bloom's syndrome
Familial amyloidotic polyneuropathy: a new transthyretin position 30 mutation (alanine for valine) in a family of German descent family of German descent
A lethal syndrome resembling branchio‐oculo facial syndrome
The interstitial deletion of bands q33–35 of long arm of chromosome 7: a review with a new case report
A girl with 71,XXXXY karyotype
Quality control in routine chromosome analysis: prediction of total number of bands for the individual case analyzed
Carrier detection in X‐linked ocular albinism of the Nettleship‐Falls type by DNA analysis
Partial trisomy 19p: case report and natural history
Karyotype/phenotype correlation in females with short stature
Full 69,XXY triploidy and sex‐reversal: a further example of true hermaphrodism associated with multiple malformations
Prevalence, geographical distribution and genealogical investigations of mutation 188 of lipoprotein lipase gene in the French Canadian population of Québec emigrants from France to Québec in the 17th century; French Canadian; French Canadians; French Canadian population of Québec; emigrants from France
Announcements
Isodicentric chromosome 18 in an abnormal infant using chromosome specific DNA probe
Effect of chorionic villus sampling on utilization of prenatal diagnosis in women of advanced maternal age ethnic minorities
An additional patient with the 3C syndrome
Cri du chat syndrome due to meiotic recombination in a pericentric inversion 5 carrier
Genetic recombination between malignant hyperthermia and calcium release channel in skeletal muscle Swedish Families