Clinical Genetics - 1995

158 articles | Last updated: 2025-12-03 14:12:56
Caucasian
3
White
2
European
1
Other
24
Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
Effect of the StuI polymorphism in the LDL receptor gene (Ala 370 to Thr) on lipid levels in healthy individuals Icelandic
Split foot and developmental retardation associated with a deletion of three microsatellite markers in 7q21.2‐q22.1
Phenotypic heterogeneity in CF sibs compound heterozygous for the G85E and 621 + 1G→T mutations
Association of <i>Hin</i>cII RFLP of low density lipoprotein receptor gene with obesity in essential hypertensives
Skipping of exon 12 as a consequence of a point mutation (1898 + 5G → T) in the cystic fibrosis transmembrane conductance regulator gene found in a consanguineous Chinese family Chinese origin
Skin color and cancer mortality among black men in the Charleston Heart Study
Symptomatic heterozygosity in the Ellis‐van Creveld syndrome?
A case of Marden‐Walker syndrome with Dandy‐Walker malformation
Screening of neurofibromatosis type 1 gene: identification of a large deletion and of an intronic variant
Aplasia cutis congenita and associated disorders: an update
Detection of α‐thalassemia‐1 (Southeast Asian type) and its application for prenatal diagnosis
Atypical methylmalonic aciduria with progressive encephalopathy, microcephaly and cataract in two siblings — a new recessive syndrome?
Health care utilization and perceptions of health among adolescents and adults with Turner syndrome
Molecular characterization of trisomic segment 3p24.1→3pter: a case with review of the literature
Tracheoesophageal anomalies in oculoauriculovertebral (Goldenhar) spectrum
Noonan syndrome with café‐au‐lait spots and multiple lentigines syndrome are not linked to the neurofibromatosis type 1 locus
Identification of the valine 408 to methionine mutation in the LDL receptor in a Greek patient with homozygous familial hypercholesterolemia Greek, Greeks, African Afrikaners, Holland (Dutch)
Population frequency of apolipoprotein E5 (Glu3→Lys) and E7 (Glu244→Lys, Glu245→Lys) variants in western Japana Japanese
Clinical and molecular analysis of a Japanese boy with Morquio B disease Japanese boy
The concurrence of the blepharophimosis, ptosis, epicanthus inversus syndrome (BPES) and Langer type of mesomelic dwarfism in the same patient. Evidence of the location of Langer type of mesomelic dwa
A rare G6490 → substitution at the last nucleotide of exon 10 of the glucocerebrosidase gene in two unrelated Italian Gaucher patients Italian, Japanese
Clinical phenotype associated with terminal 2q37 deletion
Monosomy 21q: two cases of del(21q) and review of the literature
Cytogenetic and molecular cytogenetic studies of a case of interstitial deletion of proximal 15q
Unexpected familial recurrence of iris coloboma. A delayed mutation mechanism?
A <i>Taq</i>l and a <i>Bam</i>HI polymorphism in the COL4A4 gene on chromosome 2q35‐37
Characterization of a ring chromosome 21 by FISH‐technique
Late diagnosis of Down syndrome due to incorrect cytogenetic diagnosis and extreme prematurity
A new syndrome with cardiac malformation, cleft lip‐palate, microcephaly and digital anomalies?
Pericentric inversion with a minute deletion of the Y chromosome in a severely oligozoospermic man
Down syndrome and systemic lupus erythematosus
Charcot‐Marie‐Tooth type 1B neuropathy: third mutation of serine 63 codon in the major peripheral myelin glycoprotein P0 gene
Common genetic variants of lipoprotein lipase that relate to lipid transport in patients with premature coronary artery disease
Molecular genetic analysis of exons 1 to 6 of the APC gene in non‐polyposis familial colorectal cancer
Trisomy 3 mosaicism on CVS: case report with literature review and propositions for investigation and counseling
Trisomy 1 mosaicism only detected on a direct chromosome preparation in a neonate
Familial transmission of a duplication‐deficiency X chromosome associated with partial Turner syndrome
<i>De novo</i> balanced 5;21 translocation in a child with acrobrachycephaly, ventriculomegaly, pulmonary stenosis, ectopic anus and mental retardation
Cockayne syndrome type III with high intelligence
Announcements
Evidence for genetic influences on smoking in adult women twins
Familial intestinal polyatresia syndrome
A small supernumerary marker chromosome X identified by <i>in situ</i> hybridization
Risk estimates for developing motor neurone disease in first‐degree relatives
Maternal 3;13 chromosome insertion, with severe pre‐eclampsia
Increased frequency of the rare PstI allele (P2) in a population of CAD patients in Northern Greece Northern Greek population; Greek population
Dicentric chromosome Y associated with Leydig cell agenesis and sex reversal
Antley‐Bixler syndrome and esophageal atresia in a patient with trisomy 21
Impact of a common mutation of the LDL receptor gene, in French‐Canadian patients with familial hypercholesterolemia, on means, variances and correlations among traits of lipid metabolism French‐Canadian
Retinitis pigmentosa in India: a genetic and segregation analysis
Pelizaeus‐Merzbacher disease: a point mutation in exon 6 of the proteolipid protein (<i>PLP</i>) gene
A rare silent G to T mutation in exon 4 of the human low density lipoprotein receptor gene
Familial mental retardation and progressive spasticity
Correlation between magnitude of CAG repeat length alterations and length of the paternal repeat in paternally inherited Huntington's disease
Exclusion mapping of the benign hereditary chorea gene from the Huntington's disease locus: report of a family Greek
A family with unusual Waardenburg syndrome type I (WSI), cleft lip (palate), and Hirschsprung disease is not linked to <i>PAX</i> 3
Clinical and molecular genetic findings in five patients with Miller‐Dieker syndrome
Dinucleotide repeat polymorphism at the human CD59 locus
Acro‐pectoro‐renal field defect with contralateral ureteropelvic junction obstruction
A recombination event in the closely linked plasminogen and apolipoprotein(a) gene loci Italian family; Japanese families
Constitutional heteromorphism of 9q13→q21 in a patient with chronic myelogenous leukemia
Cystic fibrosis mutations and immotile cilia syndrome
Parental origin of the X chromosome, X chromosome mosaicism and screening for “hidden” Y chromosome in 45,X Turner syndrome ascertained cytogenetically
A search for three known RYR1 gene mutations in 41 Swedish families with predisposition to malignant hyperthermia
Lung hypoplasia and severe pulmonary hypertension in an infant with double heterozygosity for spondyloepiphyseal dysplasia congenita and achondroplasia
The Pvull restriction site in the second intron of the human steroid 21‐hydroxylase gene CYP21 is polymorphic
A novel insertional mutation of a single base in exon 12 of the dystrophin gene
Sister chromatid exchange in Prader‐Willi syndrome families
Rett syndrome: potential gene sources ‐ phenotypical variability Swedish
The Coffin‐Siris syndrome: data on mental development, language, behavior and social skills in 12 children
Mosaic “tetrasomy” 8p: case report and review of the literature
Chronological difference in walking impairment among Japanese group A xeroderma pigmentosum (XP‐A) patients with various combinations of mutation sites Japanese
Adenine phosphoribosyltransferase deficiency identified by urinary sediment analysis: cellular and molecular confirmation Japanese people
Sequence and STS of two new single copy DNA markers p21.6A (D6S116) and p21.14A (D6S122) on chromosome region 6q14‐15; D6S122 highly homologous to the Igk chain
Mohr syndrome (oro‐facial‐digital syndrome II) ‐ a familial case with different phenotypic findings
Vascular ring leading to tracheoesophageal compression in a patient with Rubinstein‐Taybi syndrome
Charcot‐Marie‐Tooth disease: molecular characterization of patients from Central and Southern Italy Italians included; patients from Central and Southern Italy
Factors influencing whether or not couples seek genetic counselling: an explorative study in a paediatric surgical unit
Announcements
Psychosocial and sexual functioning in women with Turner syndrome
Mild cystic fibrosis disease in three Mexican delta‐F508/G551S compound heterozygous siblings
A BamHI polymorphism in the human cytochrome P450 gene, CYP2D6
Premature thelarche in Möbius syndrome
Autosomal recessive retinitis pigmentosa locus maps on chromosome 1q in a large consanguineous family from Pakistan Pakistani family; genetic isolates
Event‐related potentials (ERPs) and intelligence in neonatally identified 47, XXY males
Confidentiality in counseling for X‐linked conditions
Variable expression of the popliteal pterygium syndrome in two 3‐generation families
Problems arising in correlating clinical and molecular data in myotonic dystrophy
Identification of a breast tumor with microsatellite instability in a potential carrier of the hereditary non‐polyposis colon cancer trait
Retinoblastoma in association with the chromosome breakage syndromes Fanconi's anaemia and Bloom's syndrome: clinical and cytogenetic findings
A further report of Brachmann‐de Lange syndrome in two sibs with normal parents
A Taql polymorphism in a gene belonging to the human apoprotein(a)‐plasminogen gene family on the telomeric region of chromosome 6 (6q26‐27)
Normal testicular histology in a mid‐trimester 49,XXXXY fetus
Clinical follow up of a girl with “mental retardation with pterygia, shortness and distinct facial appearance” (Haspeslagh syndrome)
Complexity of molecular genetics of dyslipidemia in a family highly susceptible to ischemic heart disease Danish
Clinical traits and molecular findings in 46,XX males Mexican males
Aarskog syndrome: severe neurological deficit with spastic hemiplegia resulting from perinatal cerebrovascular accidents in two non‐related males
Monozygotic twins concordant for Rubinstein‐Taybi syndrome: changing phenotype during infancy
Opitz BBBG syndrome: new family with late‐onset, serious complication
Southern analysis reveals a large deletion at the hypoxanthine phosphoribosyltransferase locus in a patient with Lesch‐Nyhan syndrome
Congenital heart disease in the 48,XXYY syndrome
Joint dislocation and cerebral anomalies are consistently associated with oral‐facial‐digital syndrome type IV
Aplasia of the optic nerve in two cases of partial trisomy 10q24‐ter
Genetics in democratic societies ‐ the Nordic perspective
Duplication Xp22.2 and pseudoisodicentric Yq detected by FISH and PCR in a sterile male
A frequent Hhal polymorphism in intron 9 of the low density lipoprotein receptor gene detected by the denaturing gradient gel electrophoresis technique
Muscle expression of glucose‐6‐phosphate dehydrogenase deficiency in different variants Mediterranean, Seattle‐like, A-variants
Image quality in digital chromosome analysis systems
Apolipoprotein E allele frequencies in a South African Indian female population South African Indian female population; South Africans of Indian (Asian) ancestry; Indian nurses
Pericentric inversions of chromosome 4: report of a new family and review of the literature
Central nervous system abnormalities in chromosome deletion at 11q23: is it true?
Increased frequency of apolipoprotein ε2 allele in non‐insulin dependent diabetic (NIDDM) patients with nephropathy general Japanese population
Increased and decreased relative risk for noninsulin‐dependent diabetes mellitus conferred by HLA class II and by CD4 alleles
Clinical overlap of Beckwith‐Wiedemann, Perlman and Simpson‐Golabi‐Behmel syndromes: a diagnostic pitfall
Haspeslagh syndrome without severe mental retardation and pterygia?
Gorlin‐Chaudhry‐Moss or Saethre‐Chotzen syndrome?
A simple method to detect the RYR1 mutation G1021A, a cause of malignant hyperthermia susceptibility
A highly informative BamHI RFLP in the type IV collagen α5 chain gene (COL4A5)
Plasma lipids and lipoproteins response to a dietary challenge: analysis of four candidate genes
Familial microcephaly with severe neurological deficits: a description of five affected siblings
Sperm chromosome complements in a man heterozygous for a reciprocal translocation 46,XY,t(9;13)(q21.1;q21.2) and a review of the literature
Hartwig Cleve
Congenital cardiac malformations in Adams‐Oliver syndrome
An informative <i>Hindlll</i> polymorphism associated with the βB1 crystallin gene (CRYBB1) on human chromosome 22
Reply
Announcements
Quantitation of fibrillin immunofluorescence in fibroblast cultures in the Marfan syndrome
Cytogenetic analysis of 23 Japanese patients with amyotrophic lateral sclerosis Japanese
Familial benign setting‐sun phenomenon in healthy newborns
Triosephosphate isomerase deficiency: biochemical and molecular genetic analysis for prenatal diagnosis
Familial hypertryptophanemia in two siblings
Restriction fragment length polymorphisms at the apoprotein genes AI, CIII and B‐100 and in the 5‘ flanking region of the insulin gene as possible markers of coronary heart disease German origin
Inter‐ and intrafamilial variability in mucolipidosis II (I‐cell disease)
Periumbilical skin length measurements in the newborn
New familial association between ocular coloboma and loose anagen syndrome
Genetic analysis of 20 families with autosomal dominant adult polycystic kidney disease from South West Thames Region Italian origin
Video display terminals: risk of trisomy 18?
Interstitial deletion of 8p: report of two patients and review of the literature
Identification of a CD40L gene mutation and genetic counselling in a family with immunodeficiency with hyperimmunoglobulinemia M
Vitamin D dependent rickets type II and normal vitamin D receptor cDNA sequence. A cluster in a rural area of Cauca, Colombia, with more than 200 affected children
Lack of association of apolipoprotein E polymorphism with plasma Lp(a) levels in the Chinese
Retinitis pigmentosa in Spain Spanish origin; Spain
On the nosology of the “primary true microcephaly, chorioretinal dysplasia, lymphoedema” association
Molecular cytogenetic studies of duplication 9q32→q34.3 inserted into 9q13
A case of Alström syndrome associated with diabetes insipidus
Spondylo‐camptodactyly syndrome: a distinct autosomal dominant entity?
Polysyndactyly and asymptomatic hypothalamic hamartoma in mother and son: a variant of Pallister‐Hall syndrome
Osteopenia, abnormal dentition, hydrops fetalis and communicating hydrocephalus
Mitochondrial gene mutations in familial non‐insulin‐dependent diabetes mellitus in Taiwan
A novel missense mutation in exon 3 of the COL4A5 gene associated with late‐onset Alport syndrome
Christian's spondylo‐digital syndrome: second familial case
Announcements
Association between a dimorphic site on chromosome 12 and clinical diagnosis of hypertension in three independent populations
Two brothers with an unbalanced 8;17 translocation and infantile pyloric stenosis
Partial deletion 11q: report of a case with a large terminal deletion 11q21‐qter without loss of telomeric sequences, and review of the literature
Course of autosomal recessive polycystic kidney disease (ARPKD) in siblings: a clinical comparison of 20 sibships
18p monosomy with GH‐deficiency and empty sella: good response to GH‐treatment
A Taql RFLP in the type IV collagen α5 chain gene (COL4A5) with the rare allele more frequently found in seven Chinese Alport syndrome patients Chinese