Clinical Genetics - 1991

147 articles | Last updated: 2025-12-03 14:12:56
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Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
Brief clinical report: a 46,XY phenotypic female with Smith‐Lemli‐Opitz syndrome
Detection of mitochondrial DNA deletions in blood using the polymerase chain reaction: non‐invasive diagnosis of mitochondrial myopathy
Anthropometric study with emphasis on hand and foot measurements in the Prader‐Willi syndrome: sex, age and chromosome effects
Routine applications of DNA fingerprinting with the oligonucleotide probe (CAC)<sub>5</sub>/(GTG)<sub>5</sub>
Using the polymerase chain reaction to maintain DNA probe inventories in clinical and diagnostic laboratories
Short arm deletion of chromosome 1: del(1)(p13.3 p22.3) in a female infant with an extreme tetralogy of Fallot
Trisomy 18 and 18p‐ features in a case of isochromosome 18q [46,XY,i(18q)]: prenatal diagnosis and autopsy report
Annual Meeting of the European Society of Human Genetics
Atypical facio‐scapulo‐humeral muscular dystrophy — a counselling dilemma
The ancestry of spondyloepimetaphyseal dysplasia with joint laxity (SEMDJL) in South Africa
Characterization of the frequency of delta F508 mutation and CF haplotypes in Swedish families Swedish families
Familial Jarcho‐Levin syndrome rare in Europe; Puerto Ricans; Sicilian family
Progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (PEHO syndrome)
Transcobalamins in the etiology of neural tube defects
High resolution‐banded chromosomes from patients with Sotos syndrome
Importance of accurate diagnosis in counseling for neural tube defects diagnosed prenatally
DNA deletions in the low density lipoprotein (LDL) receptor gene in Danish families with familial hypercholesterolemia Danish ("Danish families" / "Danish patients")
Prenatal diagnosis of lethal osteogenesis imperfecta in twin pregnancy
The inheritance of conotruncal malformations: a review and report of two siblings with tetralogy of Fallot with pulmonary atresia
A simplified protocol for fluorescence <i>in situ</i> hybridization with repetitive DNA probes and its use in clinical cytogenetics
22nd Annual Symposium of the European Society of Human Genetics GENETICS IN THE 21st CENTURY
Trembling chin — a report of this inheritable dominant character in a four‐generation Canadian family
Enhancement of amniotic fluid cell growth for genetic amniocentesis
EEC syndrome (ectrodactyly, ectodermal dysplasia and cleft lip/palate) with a balanced reciprocal translocation between 7q11.21 and 9p12 (or 7p11.2 and 9q12) in three generations
Nine mutations in the cystic fibrosis (CF) gene account for 80% of the CF chromosomes in French patients
Balanced pericentric inversion 8(p23q13) in a child with rhizomelic chondrodysplasia punctata and his mother
A Taql RFLP at the human renal kallikrein (KLK1) locus
Two Japanese cases with aspartylglycosaminuria: clinical and morphological features Finnish populations; Japanese; Asian countries
Nasopalpebral lipoma‐coloboma syndrome
An autosomal recessive form of benign familial neonatal seizures
Announcement
Age of onset in vitiligo: relationship with HLA supratypes
Inv dup (8) (p21.1 → 22.1): further case report and a new hypothesis on the origin of the chromosome abnormality
Tetrasomy 9p: an emerging syndrome
Dizygotic twins concordant for truncus arteriosus
Announcement
Frequency of the phenylalanine deletion (ΔF<sub>508</sub>) in the CF gene of Belgian cystic fibrosis patients
Unstable translocations: a new case?
XY translocation in a boy with ichthyosis, hypogonadism, short stature and mental retardation
Hemophilia A: genetic prediction and linkage studies in all available families in Finland Finnish population
Announcement
Within‐individual variation in serum cholesterol levels: association with DNA polymorphisms at the apolipoprotein B and AI‐CIII‐AIV loci in patients with peripheral arterial disease
Application of fluorescence <i>in situ</i> hybridization techniques in clinical genetics: use of two alphoid repeat probes detecting the centromeres of chromosomes 13 and 21 or chromosomes 14 and 22,
Prader‐Willi syndrome and Robertsonian translocations involving chromosome 15
Multiplex PCR excludes Duchenne muscular dystrophy in a twin pregnancy
Automated multiple‐cell karyotyping: a clinical feasibility study
Metaphyseal acroscyphodysplasia
IBM‐PC compatible software for establishing metacarpophalangeal pattern profiles
A chromosome 17q <i>de novo</i> paracentric inversion in a patient with campomelic dysplasia; case report and etiologic hypothesis
Molecular identification of a small supernumerary marker chromosome by <i>in situ</i> hybridization: diagnosis of an isochromosome 18p with probe L1.84
Atelosteogenesis I and boomerang dysplasia: a question of nosology
Identification of the 664 proline to leucine mutation in the low density lipoprotein receptor in four unrelated patients with familial hypercholesterolaemia in the UK Asian Indian origin
Extra small marker chromosome associated with normal phenotype due to 3:1 disjunction of t(14;22) in a parent. Implications for the origin of marker chromosomes
Smith‐Lemli‐Opitz syndrome in female, monozygotic twins
Apolipoprotein B polymorphism and altered apolipoprotein B concentrations in Congolese blacks
Saethre‐Chotzen syndrome (ACS III) in four generations
Genetic epidemiology of cystic fibrosis in Saguenay‐Lac‐St‐Jean (Quebec, Canada)
Brachyolmia: a skeletal dysplasia with an altered mucopolysaccharide excretion
Familial Caffey's disease and late recurrence in a child Arabic‐Christian
Announcements
Vitreous involvement in familial amyloidotic neuropathy: a genealogical and genetic study
Coronary artery disease and apolipoprotein A‐I/C‐III gene polymorphism: a study of Saudi Arabians
A new transthyretin variant from a patient with familial amyloidotic polyneuropathy has asparagine substituted for histidine at position 90
Glyceryl ethers in peroxisomal disease
Pigmentary dysplasias in long survivors with mosaic trisomy 18: report of two cases
The cerebro‐reno‐digital syndromes: a new community
Rapp‐Hodgkin hypohidrotic ectodermal dysplasia syndrome
Beals syndrome: clinical and molecular investigations in a kindred of Indian descent Indian descent
Screening for fra(x) mutation and Klinefelter syndrome in mental institutions
The direct early diagnosis of cystic fibrosis by the detection of the deltaF508 CFTR gene mutation in a prematurely delivered boy
Monosomy of 1p13.3–22.3 in twins
Cutaneous lymphangioma and amegakaryocytic thrombocytopenia in Noonan syndrome
Holt‐Oram syndrome in four half‐siblings with unaffected parents: brief clinical report
The value of deletion analysis for carrier detection in Duchenne muscular dystrophy (DMD)
The ΔF508 mutation in mild adult forms of cystic fibrosis (CF)
Cutis laxa: autosomal dominant inheritance in five generations
Piebaldism: an autonomous autosomal dominant entity
A 15‐item checklist for screening mentally retarded males for the fragile X syndrome
Acrodysostosis in two generations: an autosomal dominant syndrome
Interstitial deletion of chromosome 2q associated with ovarian dysgenesis
Personality in 47,XXY males during adolescence
A clinical, cytogenetic and familial study of 307 mentally retarded, institutionalized, adult male patients with special interest for fra(X) negative X‐linked mental retardation
Malformations in a child with dup (7 pter‐p15.1) and del (7 q36‐qter) as a result of familial pericentric inversion
Second case report of del(4) (q25q27) and review of the literature
Long‐term follow‐up in females with Ullrich‐Turner syndrome
Germinal mosaicism in Crouzon syndrome. A family with three affected siblings of normal parents
A balanced complex chromosomal rearrangement (BCCR) with phenotypic effect
Association of a genetic polymorphism in human apolipoprotein B‐100 with intermediate density lipoprotein concentrations
Identification of a marker chromosome as inv dup(15) by molecular analysis
Cell line segregation in a 45,X/46,XY mosaic child with asymmetric leg growth
Biochemical effect of liver transplantation in two Swedish patients with familial amyloidotic polyneuropathy (FAP‐met<sup>30</sup>)
Hypoplasia of the corpus callosum and growth hormone deficiency in a boy with the XXXXY syndrome
Influence of serum paraoxonase polymorphism on serum lipids and apolipoproteins Chinese
Heterogeneity for mutations in medium chain acyl‐CoA dehydrogenase deficiency in the UK population
Linkage analysis for the diagnosis of autosomal dominant polycystic kidney disease, and for the determination of genetic heterogeneity in Italian families Italian
Usher syndrome: results of a screening program in Colombia
Apparent Fryns' syndrome and aneuploidy: evidence for a disturbance of the midline developmental field
<i>De novo</i> 13q partial duplication identified by cytogenetic, biochemical and molecular approaches
Genetic mapping of loci for X‐linked retinitis pigmentosa
Cohen syndrome: fertility in a female patient
Diagnostic reliability of the cytogenetic centromere heteromorphism in the human X chromosome
Erratum
Genetic studies of human apolipoproteins. XVI. APOE polymorphism and cholesterol levels in the Mayans of the Yucatan Peninsula, Mexico European admixture
Recurrent neuroleptic malignant syndrome associated with inv dup(15) and mental retardation
The prevalence of chromosome diseases in the general population of Sichuan, China
Robertsonian translocation and an extra microchromosome: independent origin identified by <i>in situ</i> hybridization
Counselling under genetic heterogeneity: a practical approach
Ring chromosome 4 in a child with mild dysmorphic signs
Restriction fragment length polymorphism analysis of the C1‐inhibitor gene in hereditary C1‐inhibitor deficiency
Dominantly inherited microcephaly, hypotelorism and normal intelligence
Germ‐line mosaicism in Waardenburg syndrome
Hereditary hemorrhagic telangiectasia: report of 15 affected cases in a Mexican family Mexican
Hypertrophic cardiomyopathy in late‐onset variant of Fabry disease with high residual activity of <i>α</i>‐galactosidase A
A new family with chorioretinal dystrophy, spinocerebellar ataxia and hypogonadotropic hypogonadism (Boucher‐Neuhäuser syndrome)
Familial short rib syndrome, type Beemer, with pyloric stenosis and short intestine, one case diagnosed prenatally
Tuberous sclerosis in two sibs of normal parents
Addendum: A new syndrome of aphalangy, hemivertebrae and urogenital‐intestinal dysgenesis
Chediak‐Higashi syndrome: report of a case with an ovarian tumor
Announcements
Monozygotic twin girls with diploid/triploid chromosome mosaicism and cutaneous pigmentary dysplasia
Association of annular pancreas and duodenal obstruction – evidence for Mendelian inheritance?
Hypothesis: association of the critical region of trisomy 18 and 18q2—syndrome with dermatoglyphic findings and a growth suppressor (deleted in colon cancer) locus
Gene deletions in Japanese patients with Duchenne and Becker muscular dystrophies: deletion study and carrier detection
Estimates of heritability of plasma homocyst(e)ine levels in aging adult male twins
An apparently new mental retardation syndrome in three elderly sisters
Clinical investigation of females with the Martin‐Bell syndrome and risk assessment for carrier status
Robertsonian translocation and an extra microchromosome: independent origin identified by <i>in situ</i> hybridisation
Announcements
DNA markers in candidate genes
Absence of predictable phenotypic expression in proximal 15q duplications
Patients with deletions of 9q22q34 do not define a syndrome: three case reports and a literature review
Poland‐Moebius syndrome in a boy and Poland syndrome in his mother
Insulin‐dependent diabetes developed in a young man with Bloom's syndrome
Acrodysgenital dwarfism or Smith‐Lemli‐Opitz type II syndrome
First treatment of family with hereditary adductor cord paralysis
Mosaic trisomy 8 associated with jejunal duplication
Ectrodactyly‐ectodermal dysplasiaclefting syndrome (EEC): the clinical variation and prenatal diagnosis
Tuberous sclerosis in a child with de novo translocation t(3; 12) (p26.3; q23.3)
X‐linked mental retardation with bilateral clasped thumbs: report of another affected family Sephardi Jewish family
Endocrine and exocrine pancreatic function and the ΔF508 mutation in cystic fibrosis
Mosaic tetraploidy in a liveborn infant with features of the DiGeorge anomaly
23rd Annual Symposium of the European Society of Human Genetics July 13–16, 1991 Leuven, Belgium European Society of Human Genetics
Simultaneous expression of the rare and common fragile sites on the X chromosome
Strengths and weaknesses in the cognitive profile of youngsters with Prader‐Willi syndrome
Tests of performance of four semiautomatic metaphase‐finding and karyotyping systems
Robertsonian translocations in Prader‐Willi syndrome
Chromosome aberrations in Sotos syndrome