Clinical Genetics - 1996

205 articles | Last updated: 2025-12-03 14:12:56
Caucasian
8
White
1
European
3
Other
22
Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
High frequency of new mutations in North Indian Duchenne/Becker muscular dystrophy patients
Carrier identification of X‐linked adrenoleukodystrophy by measurement of very long chain fatty acids and lignoceric acid oxidation
Deciduous teeth in tuberous sclerosis
Parental origin and mechanisms of formation of three eases of 12p tetrasomy
A three‐allelic polymorphic system in exon 12 of the LDL receptor gene is highly informative for segregation analysis of familial hypercholesterolemia in the Spanish population Spanish population
Multifactorial inheritance of non‐syndromic macrocephaly
Medical Ethics:The right not to know—worthy of preservation any longer? An ethical perspective
Poland sequence in two siblings suggesting an autosomal inheritance transmission
<i>De novo</i> direct duplication 3 (p25→pter): a previously undescribed chromosomal aberration
Neuroradiology and clinical aspects of Usher syndrome
Glycerol kinase deficiency in two brothers with and without clinical manifestations
Discordant monozygotic twins with the Schimmelpenning‐Feuerstein‐Mims syndrome
Report of a new case of axial mesodermal dysplasia complex
Short Report on DNA Marker at Candidate Locus
DNA testing in familial hypertrophic cardiomyopathy: clinical and laboratory implications
Nonsyndromic X‐linked mental retardation: review and mapping of MRX29 to Xp21
Association of genetic variation at the β‐fibrinogen gene locus and plasma fibrinogen evels; interaction between allele frequency of the G/A<sup>‐455</sup>polymorphism, age and smoking
The Floating‐Harbor syndrome: two affected siblings in a family
Down syndrome associated with a retroperitoneal teratoma and Morgagni hernia
Desbuquois syndrome in an Arab Bedouin family Arab Bedouin
FISH characterization of the Xq21 breakpoint in a translocation carrier with premature ovarian failure
Congenital heart defect and conductive hypoacusia in a patient with the KBG syndrome
Craniofacial morphology of the tricho‐dento‐osseous syndrome
Fanconi anemia complementation group E: clinical and cytogenetic data of the first patient
APC intragenic haplotypes in familial adenomatous polyposis Portuguese; other populations
Respiratory complications of Ehlers‐Danlos syndrome type IV
High incidence of pre‐excitation syndrome in Japanese families with Leber's hereditary optic neuropathy Finns, Finnish, Japanese, ethnic groups (Finland, Japan)
Insertion/deletion polymorphism in the angiotensin‐converting‐enzyme gene and blood pressure during ergometry in normal males
Size of 22q deletions in four previously reported patients with conotruncal anomaly face syndrome
Mutational screening of APP gene in patients with early‐onset Alzheimer disease utilizing mismatched PCR‐RFLP
Rothmund‐Thomson syndrome in siblings: evidence for acquired <i>in vivo</i> mosaicism
Urticaria, arthralgia, and nephropathy without amyloidosis: another variant of the Muckle‐Wells syndrome?
Wolcott‐Rallison syndrome associated with congenital malformations and a mosaic deletion 15q 11–12
Jumping translocation in a phenotypically normal female
Benign external hydrocephalus in a boy with autosomal dominant microcephaly
An Avall polymorphism in the human apolipoprotein C‐II gene
Psychological and social impact of carrier screening for cystic fibrosis among pregnant women — a pilot study
Biliary malformation with renal tubular insufficiency in two male infants: third family report
Ehlers‐Danlos syndrome type IV caused by Gly400Glu, Gly595Cys and Glyl003Asp substitutions in collagen III: clinical features, biochemical screening, and molecular confirmation
Bloom's syndrome in a Turkish individual Turkish
Partial trisomy 6p due to maternal t(1;6) translocation
Homozygous deletion of exon 18 leads to degradation of the lysosomal α‐glucosidase precursor and to the infantile form of glycogen storage disease type II
Ataxia telangiectasia syndrome with B cell lymphoma
Synpolydactyly (Syndactyly Type II)
Erratum
Down syndrome with partial duplication and del (21) syndrome: study protocol and call for collaboration. Study I: clinical assessment
An improved method for the detection of Down's syndrome aneuploidy in uncultured amniocytes
The phenotype of a 45, X male with a Y/18 translocation
Two supernumerary marker chromosomes, derived from chromosome 6 and 9, in a boy with mild developmental delay
Preimplantation genetic testing for Huntington disease and certain other dominantly inherited disorders
Neurofibromatosis/Noonan phenotype: a variable feature of type 1 neurofibromatosis
Phocomelia, ectrodactyly, skull defect and urinary system anomaly: Schinzel‐phocomelia syndrome?
Association analyses of <i>Nsi</i>I RFLP of human insulin receptor gene in hypertensives
Genetic contributions to LDL‐C, Apo‐B and LDL‐C/Apo‐B ratio in a sample of Israeli offspring with a parental history of myocardial infarction
Presymptomatic molecular diagnosis of autosomal dominant polycystic kidney disease using PKD1‐and PKD2‐linked markers in Cypriot families Cypriot
Short Report on DNA Marker at Candidate Locus
UICC Symposium on Familial Cancer and Prevention Molecular Epidemiology: a New Strategy toward Cancer Control
Midline congenital anomalies: the estimated occurrence among American Indian and Alaska Native infants American Indian and Alaska Native
Exceptions to the rule: individuals with FAP specific CHRPE and mutations in exon 6 of the <i>APC</i> gene
Osteopenia, abnormal dentition, hydrops fetalis and communicating hydrocephalus: unusual early clinical signs in Coffin‐Lowry syndrome
Lack of association of angiotensin‐converting enzyme (<i>ACE</i>). Gene insertion/deletion polymorphism with CAD in two Asian populations
Deletion of the long arm of chromosome 6: two new patients and literature review
Molecular analysis of a Japanese family with Lesch‐Nyhan syndrome: identification of mutation and prenatal diagnosis
Variable clinical expression in a family with OI type IV due to deletion of three base pairs in COL1A1
Frequencies of five genetic polymorphisms in coronarographed patients and effects on lipid levels in a supposedly healthy population
Genomic distribution of 5‘ TTCCA 3’ repeat motif and its diagnostic potential in human Y‐chromosome‐related anomalies
Autosomal recessive retinitis pigmentosa in Spain: evaluation of four genes and two loci involved in the disease Spanish
Unbalanced karyotype, dup 14(q13‐q22), in a mother and her two children
Short stature, moderate mental retardation, hyperactivity, facial dysmorphism, skeletal abnormalities, and exaggerated ketosis: a new syndrome
<i>De novo</i> direct tandem duplication of a small segment of the short arm of chromosome 7 (p21.22→22.1)
DNA polymorphisms in linkage disequilibrium at the 3‘ end of the human APO AII gene: relationships with lipids, apolipoproteins and coronary heart disease European population
Reduction of CAG expansions in cerebellar cortex and spinal cord of DRPLA
Genetic analysis of the glucose‐6‐phosphatase mutation of type la glycogen storage disease in a Chinese family
Keipert syndrome in two brothers from Turkey
Life‐threatening cardiac involvement throughout life in a case of Costello syndrome Japanese
Clinical features in the Wiedemann‐Beckwith syndrome
Predictive and preimplantation genetic testing for Huntington's disease and other late onset dominant disorders: not in conflict but complementary
Antley‐Bixler syndrome
Primed <i>IN Situ</i> labelling (PRINS) as a rational procedure for identification of marker chromosomes using a panel of primers differentially tagging the human chromosomes
A history of miscarriages and mild prognathism as possible mode of presentation of mosaic trisomy 18 in women
Partial deletion of the long arm of chromosome 11: ten Japanese children
Further evidence of genetic heterogeneity in hereditary hydronephrosis
Congenital scalp defect, distal limb reduction anomalies, right spastic hemiplegia and hypoplasia of the left arteria cerebri media: Further evidence that interruption of early embryonic blood supply
Unusual segregation of t(11;22) resulting from crossing‐over followed by 3:1 disjunction at meiosis I
Identification of a double mutation in the low‐density lipoprotein receptor gene causing familial hypercholesterolemia
Short sequence repeats in intron 2 of the insulin receptor gene among black and white South Africans black
A G<sup>‐1</sup>‐to‐A acceptor splice site LDLR mutant allele leads to reduced relative transcript levels in patients with heterozygous familial hypercholesterolemia
<i>De novo</i> 46,XX, dir dup (11)(q13.3→q14.2) in a patient with mental retardation, congenital cardiopathy and thrombopenia
A case report: corpus callosum dysgenesis, microcephaly, infantile spasm, cleft lip‐palate, exophthalmos and psychomotor retardation
The CF carrier status is not associated with a diminished self‐concept or increased anxiety: results of psychometric testing after at least 1 year
Sex reversal in a child with the karyotype 46, XY, dup (1) (p22.3p32.3)
Deletion or triplication of the α3(VI) collagen gene in three patients with 2q37 chromosome aberrations and symptoms of collagen‐related disorders
A female with monosomy 18 mosaicism: a previously undescribed chromosome abnormality
A <i>Stu</i> I polymorphism in the human androgen receptor gene (AR)
A new form of skeletal dysplasia with amelogenesis imperfecta and platyspondyly
Peripheral blood cell counts in infants with Down's syndrome
The spectrum of ocular features in the Williams‐Beuren syndrome
An Iranian‐Armenian LDLR frameshift mutation causing familial hypercholesterolemia Iranian‐Armenian ("Iranian‐Armenian ancestry" and "family of Iranian‐Armenian origin")
Determination of cystic fibrosis carrier frequency for Zuni native Americans of New Mexico Zuni native Americans
Mild CF in a ΔF508/R347H compound heterozygote woman: does the manifestation of this genotype differ in the two sexes?
Announcement
Prenatal cystic fibrosis screening in a low‐risk population undergoing chorionic villus sampling for fetal karyotyping
Seven DNA polymorphisms in the LDL receptor gene: application to the study of familial hypercholesterolemia in Spain among European populations; Spanish, Swiss and Norwegians
High levels of Lp(a) lipoprotein in a family ith cases of severe pre‐eclampsia
Two brothers with multiple congenital anomalies and mental retardation due to disomy (X)(q12 → q13.3) inherited from the mother
Exclusion of Treacher Collins Franceschetti syndrome in a subject with tetralogy of Fallot and cryptorchidism
The evaluation of gonosomal mosaics: lymphocyte interphase nuclei analyzed by FISH
Molecular characterization of 21p‐variant chromosome
Chromosome 22q11 deletion and other chromosome aberrations in cases with cleft palate, congenital heart defects and/or mental disability. A survey based on the Danish Facial Cleft Register
Agenesis of the corpus callosum in Schinzel‐Giedion syndrome associated with 47, XXY karyotype
A concealed penis mimicking penile agenesis in an infant with trisomy 13
Short rib‐polydactyly syndrome in twins: Beemer‐Langer type with poly dactyly
Asymptomatic and late‐onset ornithine transcarbamylase (OTC) deficiency in males of a five‐generation family, caused by an A208T mutation
Dermatological manifestations of 71 Down syndrome children admitted to a clinical genetics unit
Routine clinical application of the FRAXA <i>Pfu</i> PCR assay: limits and utility
Is intensive follow‐up for early detection of tumors effective in children with Beckwith‐Wiedemann syndrome?
Phenotypic characterization of a patient homozygous for the D558N LDL receptor gene mutation
A case of pycnodysostosis with growth hormone deficiency
Subtle translocation (18;21) confirmed by FISH in a patient with Down syndrome
Short Report on DNA Marker at Candidate Locus: Dinucleotide polymorphism in the first intron of the human neuronal nicotinic acetylcholine receptor α4 subunit gene (CHRNA4)
Rapid detection of a mutation hot‐spot in the human androgen receptor
Siblings with congenital renal tubular acidosis and nerve deafness
A case report of 46, XX, del (21)(q22) <i>de novo</i> deletion associated with Imerslund‐Grasbeck syndrome
Nosology of fetal hypokinesia sequence based on CNS abnormalities: is there an Aase‐Smith syndrome?
Abnormal extracellular matrix in Ehlers‐Danlos syndrome type IV due to the substitution of glycine 934 by glutamic acid in the triple helical domain of type III collagen
Recombination between the postulated CCD/MHE/MHS locus and RYR1 gene markers
The apolipoprotein E gene in Binswanger's disease and vascular dementia
Outcomes and process in genetic counselling
Rett syndrome in Northern Tuscany (Italy): family tree studies
Silver‐Russell syndrome and exclusion of uniparental disomy
Tri‐amelia and phocomelia with multiple malformations resembling Roberts syndrome in a fetus: is it a variant or a new syndrome?
Partial deletion of 18p and partial duplication of 18q caused by a paternal pericentric inversion
Frequent intragenic polymorphism in the 3‘ untranslated region of the lissencephaly gene 1 (LIS‐1)
Dinucleotide repeat polymorphisms at <i>D9S171</i> and <i>D9S741</i> are identical
A new silent C to T polymorphism in the exon 3 of the oxytocin receptor gene
Increased paternal age in CHARGE association
CFC syndrome: report of familial cases
Attitudes towards termination for fetal abnormality: comparisons in three European countries
Maternal non‐recognition of Down syndrome in black South African infants black South African; African; Caucasoids
Detection of a <i>Sacl</i> restriction fragment length polymorphism at the human phenolsulphotransferase locus
How often has Lp(a) evolved?
Two novel mutations in the LDL receptor gene: common causes of familial hypercholesterolemia in a Spanish population Spanish ("Spanish patients" / "Spanish population")
A comparative study of X‐inactivation in Rett syndrome probands and control subjects
Multiple congenital malformations in an infant prenatally diagnosed with mosaicism for dup(1q) and del(Xq)
Bloom's syndrome. XIX. Cytogenetic and population evidence for genetic heterogeneity
Cohen syndrome: the clinical symptoms and stigmata at a young age
Coexistence of gene mutations causing Fabry disease and Duchenne muscular dystrophy in a Japanese boy
A mild form of Roberts/SC phocomelia syndrome with asymmetrical reduction of the upper limbs
Interstitial 6q deletion and Prader‐Willi‐like phenotype
Fibular dimelia and mirror foot without associated anomalies
Duodenal web in the syndrome of osteopathia striata with cranial sclerosis
Alopecia‐mental retardation syndrome associated with convulsions and hypergonadotropic hypogonadism
Homozygosity and heterozygosity for the transthyretin Leu64 mutation: clinical, biochemical and molecular findings
Generalized osteoporosis in a patient with oculocutaneous hypopigmentation syndrome (OOCHS), without cerebral defects A new syndrome?
G542X mutation in Mexican cystic fibrosis patients ethnic origin of the Mexican population; Mexican; Spain
Neuropsychological aspects of Marfan syndrome
Identification of two apolipoprotein variants, A‐I Karatsu (Tyr 100 → His) and A‐I Kurume (His 162 → Gin)
Screening for coeliac disease as a possible maternal risk factor for neural tube defect
On two patients with and without the classical Wolf‐Hirschhorn syndrome (WHS) sharing the same chromosome 4p16.3 specific probe deletion: evidence of a contiguous gene deletion syndrome
The molecular characterization of Gaucher disease in South Africa Gentile Caucasoids; Caucasoid
Short Report on DNA Marker at Candidate Locus
Short Report on DNA Marker at Candidate Locus
X‐linked exudative vitreoretinopathy caused by an arginine to leucine substitution (R121L) in the Norrie disease protein
Mitotic and meiotic stability of the CAG repeat in the X‐linked spinal and bulbar muscular atrophy gene
A cystic fibrosis patient homozygous for 621 + 1G→T mutation has a severe pulmonary disease, mild pancreatic insufficiency and a gastro‐esophageal reflux Polish CF families
Triphalangeal thumb and brachyectrodactyly syndrome: an uncommon entity with evidence of geographic distribution
Familial Alzheimer's disease co‐segregates with a Met 146 Ile substitution in presenilin‐1
Late juvenile metachromatic leukodystrophy (MLD) in three patients with a similar clinical course and identical mutation on one allele
Apolipoprotein E<sup>4</sup>allele in the normal elderly: neuropsychologic and brain MRI correlates
Trisomy 13/trisomy 18 mosaicism in an infant
Prenatal diagnosis of terminal deletion 7q and partial trisomy 3p in fetuses with holoprosencephaly
Cord blood immunoglobulin E in like‐sexed monozygotic and dizygotic twins
Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLO‐SL): a genealogical study of Swedish families of probable Finnish background Finnish origin; Finnish ancestors; Finnish influence; probable Finnish origin; Swedish families; Finnish origin; Finnish ancestors; Finnish influence; probable Finnish origin
Cerebral defects confirm midline developmental field disturbances in supernumerary der(22), t(11;22) syndrome
Partial trisomy 10 mosaicism with cutaneous manifestations: report of a case and review of the literature
Distinct facial appearance with nasal hypoplasia, constipation, severe mental retardation and hypotonia in two unrelated young males
Partial monosomy 10q and partial trisomy 9q with anal atresia due to maternal translocation: t(9;10)(q32;q26)
Three familial cases presenting with an immobile spine. Rigid spine or Emery‐Dreifuss syndrome?
COFS syndrome with familial 1;16 translocation
Further evidence for a syndrome of “apple peel” intestinal atresia, ocular anomalies and microcephaly
Mild form of beta‐ketothiolase deficiency (mitochondrial acetoacetyl‐CoA thiolase deficiency) in two Japanese siblings: identification of detectable residual activity and cross‐reactive material in EB Japanese
Familial translocation (X;3)(p22.3;p23): chromosomal <i>in situ</i> suppression (CISS) hybridization and inactivation pattern study
A new autosomal recessive syndrome of pachygyria
Terminal deletion of the long arm of chromosome 4 in a mother and two sons
Lack of association of angiotensin I‐converting enzyme gene polymorphism and premature myocardial infarction in Mauritian Indians Mauritian Indians
Population variation at the polymorphic ApaLI restriction enzyme site in intron 5 of the WT1 gene
Gaucher disease: studies of phenotype, molecular diagnosis and treatment
Risk estimates for balanced reciprocal translocation carriers — prenatal diagnosis experience
Announcement
RYR mutation G1021A (Gly341Arg) is not frequent in Danish and Swedish families with malignant hyperthermia susceptibility Scandinavian; Scandinavia; immigrant
Maternal origin of transferrin receptor positive cells in venous blood of pregnant women
Dandy‐Walker malformation and polydactyly: a possible expression of hydrolethalus syndrome
Marker chromosome identification by micro‐FISH
Severe dental aberrations in familial steroid dehydrogenase deficiency: a new association
Distribution of marker‐Y chromosome containing cells in different tissues of a Turner mosaic patient with mixed gonadal dysgenesis
Detection of the Pvull RFLP in intron 15 of the LDL receptor gene by long‐distance PCR
Four siblings with achalasia, alacrimia and neurological abnormalities in a consanguineous family
Branchio‐oto (BO) syndrome and oculoauriculo‐vertebral phenotype: overlapping clinical findings in a child from a BO family
A <i>de novo</i> interstitial deletion of chromosome 15 band q25 as revealed by FISH‐technique
Four frequently observed polymorphisms in the 3‘‐UTR of human peripheral myelin protein 22 (<i>PMP22</i>): identification of different haplotypes
A new year, a new start
Supernumerary ring chromosome 20 characterized by fluorescence <i>in situ</i> hybridization
Two novel missense mutations in the LDL receptor gene causing familial hypercholesterolemia
Trisomy 18 mosaicism associated with secondary amenorrhea: ratios of mosaicism in different samples and complications
Microcephaly, seizures, genital hypoplasia, and abnormalities of the hands and feet in a 4‐year‐old boy with possible Wiedemann syndrome