American Journal of Medical Genetics Part A - 2004

477 articles | Last updated: 2025-12-03 14:12:55
Caucasian
8
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2
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4
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32
Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
Interstitial deletion of the long arm of chromosome 5 in a boy with multiple congenital anomalies and mental retardation: Molecular characterization of the deleted region to 5q22.3q23.3
Further delineation of Kabuki syndrome in 48 well‐defined new individuals
Two novel mutations in <i>SLC6A8</i> cause creatine transporter defect and distinctive X‐linked mental retardation in two unrelated Dutch families Dutch
Megalourethra: A report of three cases associated with maternal diabetes and a review of the literature—is sonic hedgehog the common pathway?
Dentin phosphoprotein compound mutation in dentin sialophosphoprotein causes dentinogenesis imperfecta type III
Neonatal phenotype in Kabuki syndrome
Autosomal recessive non‐immune hydrops fetalis caused by systemic lymphangiectasia
Trisomy 13 due to rea(13q;13q) is caused by i(13) and not rob(13;13)(q10;q10) in the majority of cases
Study of folate receptor genes in nonsyndromic familial and sporadic cleft lip with or without clef palate cases Italian population
Prenatal diagnosis of a double trisomy 48, XXY, +13: Klinefelter and Patau syndromes
Ocular phenotypes of three genetic variants of Bardet–Biedl syndrome
Ullrich congenital muscular dystrophy: Connective tissue abnormalities in the skin support overlap with Ehlers–Danlos syndromes
Diagnostic FISH probes for del(17)(p11.2p11.2) associated with Smith–Magenis syndrome should contain the <i>RAI1</i> gene
Pulmonary atresia/ventricular septal defect associated with facial port‐wine stain and retinal vascular abnormality: A new constellation or deletion in chromosome 22q11.2?
<i>MTHFR</i> 677C‐T polymorphism is not excluded as maternal risk for Down syndrome among Turkish women
Delineation of an interstitial 9q22 deletion in basal cell nevus syndrome
Natural history of cardiac involvement in geleophysic dysplasia
Kabuki syndrome: Clinical data in 20 patients, literature review, and further guidelines for preventive management
The <i>PTPN11</i> gene is not implicated in nonsyndromic hypertrophic cardiomyopathy
Subtelomeric 6p deletion: Clinical, FISH, and array CGH characterization of two cases
A clinical report of a patient with two abnormal cell lines: 46,XX,del(21)(q22.1) and 47,XX,+3
A locus for Bowen–Conradi syndrome maps to chromosome region 12p13.3 Hutterite population; other populations
Iris heterochromia: A variable feature in Verloes–Koulischer‐oral‐acral syndrome
Reply to Unger: The mildest form of campomelic dysplasia
Maternal isodisomy of the telomeric end of chromosome 2 is responsible for a case of primary hyperoxaluria type 1
Fryns syndrome with Hirschsprung disease: Support for possible neural crest involvement
Characterization of liver involvement in defects of cholesterol biosynthesis: Long‐term follow‐up and review
Velocardiofacial syndrome with single central incisor
Down syndrome with pure partial trisomy 21q22 due to a paternal insertion (4;21) uncovered by uncultured amniotic fluid interphase FISH
Expressive language in children with Kabuki syndrome
Supernumerary ring chromosome 7 mosaicism: Case report, investigation of the gene content, and delineation of the phenotype
Kabuki syndrome is not caused by an 8p duplication: A cytogenetic study in 20 patients
Towards improved clinical characterization of Leber congenital amaurosis: Neurological and systemic findings
Severe, neonatal‐onset OTC deficiency in twin sisters with a de novo balanced reciprocal translocation t(X;5)(p21.1;q11)
Carbimazole embryopathy: An emerging phenotype
The mildest form of campomelic dysplasia
Association of anophthalmia and esophageal atresia: Four new cases identified by the anophthalmia/microphthalmia clinical registry
Molecular and cytogenetic characterization of a non‐mosaic isodicentric Y chromosome in a patient with Klinefelter syndrome
Aplasia cutis congenita, skull defect, brain heterotopia, and intestinal lymphangiectasia
Behavioral aspects of Angelman syndrome: A case control study
Pfeiffer type cardiocranial syndrome and van Nesselrooij syndrome—variable manifestations of a single disorder?
Rett syndrome in females with CTS hot spot deletions: A disorder profile
<i>MECP2</i> mutation analysis in patients with mental retardation
Additional case of Tsukahara's syndrome or new syndrome: Further delineation of the association of microcephaly and radio‐ulnar synostosis
Unique survival in chrondrodysplasia‐hermaphrodism syndrome
Hand involvement in Schmid metaphyseal chondrodysplasia
The real earliest historical evidence of Down syndrome
Clericuzio type poikiloderma with neutropenia is distinct from Rothmund–Thomson syndrome
Changing profile of couples seeking genetic counseling for consanguinity in Australia Asian origin; Western countries; Western Europe; ethnic origin; ethnic profiles
Insights from genomic microarrays into structural chromosome rearrangements
A novel syndrome resembling Desbuquois dysplasia
COL11A2 mutation associated with autosomal recessive Weissenbacher–Zweymuller syndrome: Molecular and clinical overlap with otospondylomegaepiphyseal dysplasia (OSMED)
Mild phenotypes in a series of patients with Opitz GBBB syndrome with <i>MID1</i> mutations
A newly recognized syndrome involving limbs, pelvis, and genital organs or a variant of Al‐Awadi/Raas‐Rothschild syndrome?
Craniofacial dyssynostosis: Description of the first four Spanish cases and review Spanish; Spanish ancestors; Spanish population; Spanish ancestry
Familial hyper‐ and hypopigmentation with age‐related pattern change
Mapping nonverbal IQ in young boys with fragile X syndrome
Congenital stationary night blindness: Report of an autosomal recessive family and linkage analysis
A disorder resembling pseudoachondroplasia but without COMP mutation
AGG interspersion patterns in the CGG repeat of the <i>FMR1</i> gene and linked DXS548/FRAXAC1 haplotypes in Brazilian populations
Novel case of dup(3q) syndrome due to a de novo interstitial duplication 3q24‐q26.31 with minimal overlap to the dup(3q) critical region
A novel mutation (E767K) in the second extracellular loop of the calcium sensing receptor in a family with autosomal dominant hypocalcemia
Recurrent adjacent‐2 segregation of a familial t(14;21)(q11.2;q11.2): Phenotypic comparison of two brothers and a paternal aunt inheriting the der(14)
Fetal presentation of PHACES syndrome
Mosaic trisomy 22: Report of a patient with normal intelligence
Familial lipomyelomeningocele: A further report
Novel <i>COL9A3</i> mutation in a family with multiple epiphyseal dysplasia
A 2‐bp deletion in the <i>GJA1</i> gene is associated with oculo‐dento‐digital dysplasia with palmoplantar keratoderma Dutch kindred
Mechanistic and epidemiologic considerations in the evaluation of adverse birth outcomes following gestational exposure to statins
Further delineation of Frank–ter Haar syndrome Turkish descent
A dysmorphic boy with 4qter deletion and 4q32.3‐34.3 duplication: Clinical, cytogenetic, and molecular findings
Functional analysis of polymorphic variation within the promoter and 5′ untranslated region of the neurofibromatosis type 1 (<i>NF1</i>) gene
Novel mutation in the tyrosine kinase domain of FGFR2 in a patient with Pfeiffer syndrome
Somatic mosaicism of chromosome 7 in a highly proliferating melanocytic congenital naevus in a ring chromosome 7 patient
Subcutaneous neurofibromas are associated with mortality in neurofibromatosis 1: A cohort study of 703 patients
Autosomal dominant flat umbilicus Chinese
Three new families with arterial tortuosity syndrome Moroccan
Symposium introduction
Autoimmune disorders in Kabuki syndrome
Intellectual abilities and adaptive behavior of children and adolescents with Kabuki syndrome: A preliminary study
Developmental outcome in Kabuki syndrome
Genotype–phenotype correlations for a wide spectrum of mutations in the Wilson disease gene (<i>ATP7B</i>)
Craniofacial structure in Marfan syndrome: A cephalometric study
High prevalence of isolated pericardial effusion in Down syndrome
Phenotypic and molecular characterization of Bruck syndrome (osteogenesis imperfecta with contractures of the large joints) caused by a recessive mutation in <i>PLOD2</i>
Reevaluating confined placental mosaicism
Pronounced short stature in a girl with tricho‐rhino‐phalangeal syndrome II (TRPS II, Langer–Giedion syndrome) and growth hormone deficiency
Symptomatic Chiari I malformation in Kabuki syndrome
A novel frameshift mutation of <i>FOXC2</i> gene in a family with hereditary lymphedema‐distichiasis syndrome associated with renal disease and diabetes mellitus German–Irish family
Heterogeneous basis of the type VIB form of Ehlers–Danlos syndrome (EDS VIB) that is unrelated to decreased collagen lysyl hydroxylation
Association of partial trisomy 9p and the Dandy‐Walker malformation
Unexpected death due to refractory metabolic acidosis and massive hemolysis in a young infant with Prader–Willi syndrome
KBG syndrome in a cohort of Italian patients Italian
Novel and recurrent exon 13 mutations of <i>COMP</i> in pseudoachondroplasia
Two sisters with Silver–Russell phenotype
A founder effect in three large Newfoundland families with a novel clinically variable spastic ataxia and supranuclear gaze palsy
Response to correspondence by Dr. Finsterer and Dr. Stöllberger: Heterogenous myopathic background of left ventricular hypertrabeculation/noncompaction
Precision in phenotyping and genotyping
Fatal lymphoproliferative disorder in a child with Schimke immuno‐osseous dysplasia
Chromosome 22q11 deletion and pachygyria characterized by array‐based comparative genomic hybridization
A new three‐generational family with frontometaphyseal dysplasia, male‐to‐female transmission, and a previously reported<i>FLNA</i>mutation
Cumming syndrome with heterotaxia, campomelia and absent uterus/fallopian tubes
Opitz “C” trigonocephaly‐like syndrome in a patient with terminal deletion of 2p and partial duplication of 17q
Localization of MRX82: A new nonsyndromic X‐linked mental retardation locus to Xq24‐q25 in a Basque family Basque
De novo interstitial direct duplication of Xq21.1q25 associated with skewed X‐inactivation pattern
Multisystem study of 20 older adults with Williams syndrome
Mild phenotype in two unrelated patients with a partial deletion of 21q22.2‐q22.3 defined by FISH and molecular studies
Phenotypic manifestations of the OCTN2 V295X mutation: Sudden infant death and carnitine‐responsive cardiomyopathy in Roma families Hungarian Roma (Gypsy); Roma subpopulations
Mutational analysis of <i>BARHL1</i> and <i>BARX1</i> in three new patients with Joubert syndrome
Total is more than the sum of the parts: Phenotyping the heart in cardiovascular genetics clinics
Absence of deletion at the SOX2 locus in a case of microphthalmia and esophageal atresia
Living with achondroplasia: Quality of life evaluation following cervico‐medullary decompression
Cerebellar hypoplasia, zonular cataract, and peripheral neuropathy in trisomy 17 mosaicism
Is SHORT syndrome another phenotypic variation of PITX2?
Three patients with 9p deletions including DMRT1 and DMRT2: A girl with XY complement, bilateral ovotestes, and extreme growth retardation, and two XX females with normal pubertal development
Intelligence in individuals with a neurofibromatosis type 1 microdeletion
Clinical report of congenital lymphatic malformations and partial gigantism of the hands associated with a heterogeneous karyotype
Re: Down syndrome and folic acid deficiency
Response to letter: Down syndrome and folic acid deficiency
Partial trisomy of chromosome 10(q22–q24) due to maternal insertional translocation (15;10)
Congenital mydriasis, patent ductus arteriosus, and congenital cystic lung disease: New syndromic spectrum?
Ethical issues in identifying and recruiting participants for familial genetic research
Ganglioglioma in a Sotos syndrome patient with an <i>NSD1</i> deletion
Trisomy 21 is associated with variable defects in cytotrophoblast differentiation along the invasive pathway
Chromosome 2q terminal deletion: Report of 6 new patients and review of phenotype‐breakpoint correlations in 66 individuals
Increased risk of symptomatic gallbladder disease in adults with Down syndrome
DUP25 remains unconfirmed
Molecular cytogenetic characterization of ring chromosome 15 in three unrelated patients
Genomewide search and association studies in a Finnish celiac disease population: Identification of a novel locus and replication of the HLA and CTLA4 loci
Maternal and fetal genetic factors account for most of familial aggregation of preeclampsia: A population‐based Swedish cohort study
Monozygotic twins discordant for frontonasal malformation
Anophthalmia–esophageal–genital syndrome: A further case to define the phenotype
A case of tangier disease with a novel mutation in the c‐terminal region of ATP‐binding cassette transporter A1
Congenital heart defects associated with Smith–Magenis syndrome: Two cases of total anomalous pulmonary venous return
Clinical variability in KBG syndrome: Report of three unrelated families
Ocular coloboma and high myopia with Hirschsprung disease associated with a novel ZFHX1B missense mutation and trisomy 21
Proving pathogenicity: When evolution is not enough
No evidence of SALL4‐mutations in isolated sporadic duane retraction “syndrome” (DURS)
Multiple circumferential skin creases: Another sign of genetic mosaicism?
Proteomic studies identified a single nucleotide polymorphism in glyoxalase I as autism susceptibility factor
Girl with monosomy 1p36 and Angelman syndrome due to unbalanced der(1) transmission of a maternal translocation t(1;15)(p36.3;q13.1)
A locus for posterior polymorphous corneal dystrophy (<i>PPCD3</i>) maps to chromosome 10
Clinical variability in a Noonan syndrome family with a new <i>PTPN11</i> gene mutation
Deficiency of pyruvate dehydrogenase caused by novel and known mutations in the E<sub>1</sub>α subunit
Costello syndrome with pancreatic islet cell hyperplasia
Renal disease in Arima syndrome is nephronophthisis as in other Joubert‐related Cerebello–oculo–renal syndromes
Feeling at risk: How women interpret their familial breast cancer risk
A case of Adams–Oliver syndrome with associated brain and pulmonary involvement: Further evidence of vascular pathology?
UUGPGC program director job description
Corrections
Another patient with cryptic unbalanced translocation between chromosomes 4q and 18q: Evidence by microarray CGH
Unusual nasal duplication in two patients
Two novel and one recurrent <i>PTPN11</i> mutations in LEOPARD syndrome
A new neocentromere locus on chromosome 13 resulting in mosaic tetrasomy for distal 13q and an asymmetric phenotype
Description of a case of distal 2p trisomy by array‐based comparative genomic hybridization: A high resolution genome‐wide investigation for chromosomal aneuploidy in a single assay
Partial tetrasomy 15 due to a unique inverted triplication of chromosome15q24‐q26
Eyelid myxoma in Carney complex without <i>PRKAR1A</i> allelic loss
Bilateral anophthalmia and esophageal atresia: Report of a new patient and review of the literature
The natural history, including orofacial features of three patients with Ehlers–Danlos syndrome, dermatosparaxis type (EDS type VIIC)
Skewed X‐inactivation in carriers establishes linkage in an X‐linked deafness–mental retardation syndrome
Detection of an interstitial deletion of 2q21‐22 by high resolution comparative genomic hybridization in a child with multiple congenital anomalies and an apparent balanced translocation
Classical West “syndrome” phenotype with a subtelomeric 4p trisomy
Women's satisfaction with genetic counseling for hereditary breast‐ovarian cancer: Psychological aspects
Middle and inner ear malformations in velocardiofacial syndrome
Mutations in <i>PITX2</i> may contribute to cases of omphalocele and VATER‐like syndromes
Mosaicism of the CAG repeat sequence in the Huntington disease gene in a pair of monozygotic twins
Dilated ascending aorta in a child with ring chromosome 21 syndrome
Blepharo–cheilo–dontic (BCD) syndrome with agenesis of thyroid broadens the phenotype and extends the discussion about the genes
Distal arthrogryposis 5: A dominant syndrome of peripheral contractures and ophthalmoplegia
Mitochondrial DNA control region sequence variation in migraine headache and cyclic vomiting syndrome
A novel missense homozygous inactivating mutation in the fourth transmembrane helix of the luteinizing hormone receptor in leydig cell hypoplasia
Self‐healing congenital verruciform hyperkeratosis
Clearing up the confusion over the glutamate carboxypeptidase II gene
Two patients with monomelic ulnar duplication with mirror hand polydactyly: Segmental Laurin–Sandrow syndrome
Assisted reproductive technology and congenital overgrowth: Some speculations on a case of Pallister–Killian syndrome
A case of Kabuki (Niikawa‐Kuroki) syndrome associated with manifestations resembling C‐trigonocephaly syndrome
Supernumerary ring chromosome 8: Clinical and molecular cytogenetic characterization in a case report
Microcephalic osteodysplastic primordial dwarfism with severe microdontia and skin anomalies: Confirmation of a new syndrome
Mosaic ring 12p and total anomalous pulmonary venous return
In reply to: Proving pathogenicity—when evolution is not enough
A stop codon mutation in COL11A2 induces exon skipping and leads to non‐ocular Stickler syndrome
A newly recognized skeletal dysplasia with rhizomelic limbs and retinitis pigmentosa
The KBG syndrome: Confirmation of autosomal dominant inheritance and further delineation of the phenotype Turkish
The first Finnish patient with the Floating‐Harbor syndrome: The follow‐up of eight years
Septo‐optic dysplasia with digital anomalies—a recurrent pattern syndrome
Cerebro–reno–digital (Meckel‐like) syndrome with limb malformations and acetabular spurs in two sibs: A new MCA syndrome?
Comments on “congenital Diaphragmatic Hernia: Is 15q26.1‐26.2 a candidate locus?”
Lack of genetic dispositions to hyperhomocysteinemia in Alzheimer disease
46,XY sex‐reversal (Swyer syndrome) and congenital diaphragmatic hernia
Familial insertion (3;5)(q25.3;q22.1q31.3) with deletion or duplication of chromosome region 5q22.1‐5q31.3 in ten unbalanced carriers
A mixed epigenetic/genetic model for oligogenic inheritance of autism with a limited role for <i>UBE3A</i>
Mutation analysis of the tumor suppressor<i>PTEN</i>and the glypican 3 (<i>GPC3</i>) gene in patients diagnosed with Proteus syndrome
Diagnosis of Proteus syndrome was correct
Mutations in the EVC1 gene are not a common finding in the Ellis‐van Creveld and short rib‐polydactyly type III syndromes
The genetics of deafness
Oculo‐palato‐cerebral syndrome: A third case supporting autosomal recessive inheritance Moroccan Jewish descent
Soft tissue facial areas and volumes in subjects with Down syndrome
“The Lion, the Witch and the Wardrobe”: Impact on sibs of individuals with AAT deficiency
Genetic significance of skewed X‐chromosome inactivation in premature ovarian failure
Association of <i>CYP17</i> with HLA‐B27‐negative seronegative spondyloarthropathy in Japanese males
Unmasking 15q12 deletion using microarray‐based comparative genomic hybridization in a mentally retarded boy with r(Y)
<i>BRCA1/2</i>testing in hereditary breast and ovarian cancer families: Effectiveness of problem‐solving training as a counseling intervention
Homozygosity mapping of lethal congenital contractural syndrome type 2 (LCCS2) to a 6 cM interval on chromosome 12q13
Congenital arhinia with de novo reciprocal translocation, t(3;12)(q13.2;p11.2)
Exploratory study of the feasibility and utility of the colored eco‐genetic relationship map (CEGRM) in women at high genetic risk of developing breast cancer
Do parents and grandparents of patients with achondroplasia have a higher cancer risk?
Nasal dimensions in normal subjects: Conventional anthropometry versus computerized anthropometry central European origin; European data
Characterization of the phenotype and definition of the deletion in a new patient with ring chromosome 22
Congenital nephrosis, mesangial sclerosis, and distinct eye abnormalities with microcoria: An autosomal recessive syndrome
Homozygous <i>SMN1</i> deletions in unaffected family members and modification of the phenotype by <i>SMN2</i>
Genome scan for loci involved in nonsyndromic cleft lip with or without cleft palate in families from West Bengal, India
Lay people's understanding of and preference against the word “mutation”
TSPY detection in blood, buccal, and urine cells of patients with 45,X karyotype
Reassessment of the Proteus syndrome literature: Application of diagnostic criteria to published cases
Memorial lecture—hereditary sensory defects: From genes to pathogenesis
45,X/47,XXX/47,XX, del(Y)(p?)/46,XX mosaicism causing true hermaphroditism
Varying loads of the mitochondrial DNA A3243G mutation in different tissues: Implications for diagnosis
Prenatal diagnosis of P450 oxidoreductase deficiency (ORD): A disorder causing low pregnancy estriol, maternal and fetal virilization, and the Antley–Bixler syndrome phenotype
Reply to Kirk et al.
Thioredoxin interacting protein in Dutch families with familial combined hyperlipidemia Dutch
Majewski osteodysplastic primordial dwarfism type II (MOPD II): Natural history and clinical findings
Pregnancy outcome following rubella vaccination: A prospective controlled study
Macrocephaly‐cutis marmorata telangiectatica congenita: Report of six new patients and a review
Candidate locus for Gilles de la Tourette syndrome/obsessive compulsive disorder/chronic tic disorder at 18q22
Brachydactyly and short stature in a kindred with early‐onset parkinsonism Dutch kindred
Prevalence of Jews as subjects in genetic research: Figures, explanation, and potential implications Jews; Jewish; 'population isolates'; 'population groups'; 'communities'; 'endogamous'
A patient with Prader–Willi syndrome and a supernumerary marker chromosome r(15)(q11.1‐13p11.1)pat and maternal heterodisomy
Paternal uniparental disomy of chromosome 14: Confirmation of a clinically‐recognizable phenotype
Significant association between nonsyndromic oral clefts and arylhydrocarbon receptor nuclear translocator (ARNT) Japanese population
Book Review
Book Review
Interaction of <i>RET</i> proto‐oncogene codon 609 germline mutations with RET haplotypes characterized by c.135G&gt;A alleles modifying MEN 2A or HSCR phenotypes
ICF syndrome in a girl with DNA hypomethylation but without detectable DNMT3B mutation
Hyperinsulinemic hypoglycemia in a newborn infant with trisomy 13
Marked phenotypic variability in progressive diaphyseal dysplasia (Camurati–Engelmann disease): Report of a four‐generation pedigree, identification of a mutation in TGFB1, and review
Growth hormone deficiency in a case of cerebrofaciothoracic syndrome in one of two affected siblings
A patient with a de novo 15q24q26.1 interstitial deletion, developmental delay, mild dysmorphism, and very blue irises
<i>CGI‐58/ABHD5</i> gene is mutated in Dorfman–Chanarin syndrome
Five years of molecular diagnosis of Fragile X syndrome (1997–2001): A collaborative study reporting 95% of the activity in France
Early development of occipital horns in a classical Menkes patient
Craniofacial dyssynostosis: Case report and review
Fragile X syndrome phenotype with normal <i>FMR1</i> gene studies
Abnormal pericyte recruitment as a cause for pulmonary hypertension in Adams–Oliver syndrome
Spondylometaphyseal dysplasia with cone‐rod dystrophy
Congenital glaucoma and brain stem atrophy as features of Aicardi–Goutières syndrome
“You have shown me my end”: Attitudes toward presymptomatic testing for familial amyotrophic lateral sclerosis
Donepezil effects on language in children with Down syndrome: Results of the first 22‐week pilot clinical trial
Autism spectrum disorder in fragile X syndrome: Communication, social interaction, and specific behaviors
Transmission of ring chromosome 13 from a mother to daughter with both having a 46,XX, r(13)(p13q34) karyotype
Absent inner dynein arms in a fetus with familial hydrocephalus‐situs abnormality
Mutation analysis of MECP2 and determination of the X‐inactivation pattern in Hungarian Rett syndrome patients Hungarian
Elucidation of a cryptic interstitial 7q31.3 deletion in a patient with a language disorder and mild mental retardation by array‐CGH
Reply to letter by Volpi regarding: “RNA processing defects of the helicase gene <i>RECQL4</i> in a compound heterozygous Rothmund–Thomson patient”
Subtelomeric deletions of chromosome 9q: A novel microdeletion syndrome
Clinical presentation of 13 patients with subtelomeric rearrangements and a review of the literature
Giant omphalocele and “prune belly” sequence as components of the Beckwith–Wiedemann syndrome
A de novo complex karyotype with two independent balanced translocations and a double inversion of chromosome 6 presenting with multiple congenital anomalies
Renal defects and limb deficiencies in 197 infants: Is it possible to define the “acrorenal syndrome”?
Mild developmental delay in terminal chromosome 6p deletion
Newly recognized syndrome with heminasal aplasia and ocular anomalies or wider spectrum of heminasal aplasia/atypical clefting syndrome?
Longitudinal evolution of unidentified bright objects in children with neurofibromatosis‐1
Inheritance of most X‐linked traits is not dominant or recessive, just X‐linked
Mitotic and meiotic instability of a telomere association involving the Y chromosome
Diastrophic dysplasia and atelosteogenesis type II as expression of compound heterozygosis: First report of a Mexican patient and genotype–phenotype correlation
Identification of a <i>SEDL</i> gene mutation in an individual with Leber hereditary optic neuropathy and spondyloepiphyseal dysplasia
Further delineation of the odonto–onycho–dermal dysplasia syndrome
Glucose 6‐phosphate dehydrogenase (G6PD) deficiency in elderly Chinese women heterozygous for G6PD variants
Spondyloperipheral dysplasia is caused by truncating mutations in the C‐propeptide of COL2A1
Duplication 4p and deletion 4p (Wolf–Hirschhorn syndrome) due to complementary gametes from a 3:1 segregation of a maternal balanced t(4;13)(p16;q11) translocation
Glutamate carboxipeptidase II (<i>GCPII</i>) His475Tyr polymorphism and association studies
Costello syndrome with growth hormone deficiency and hypoglycemia: A new report and review of the endocrine associations
Hydrolethalus syndrome, in contrast to Smith–Lemli–Opitz syndrome, is not due to a defect in post‐squalene cholesterol biosynthesis: A case report
Paternal transmission of fragile X syndrome
Heterogenous myopathic background of left ventricular hypertrabeculation/noncompaction
Surrendered autonomy for genetic screening
Worry about ovarian cancer risk and use of screening by high‐risk women: How you recruit affects what you find
A novel <i>MSX1</i> mutation in hypodontia
Impact of excluding cases with known chromosomal abnormalities on the prevalence of structural birth defects, Hawaii, 1986–1999
Primary trabeculodysgenesis in association with neonatal Marfan syndrome
A novel NOG mutation Pro37Arg in a family with tarsal and carpal synostoses
Molecular characterization of a cryptic 2q37 deletion in a patient with Albright hereditary osteodystrophy‐like phenotype
Adaptive skills, cognitive, and behavioral characteristics of Costello syndrome
Growth hormone deficiency in Costello syndrome
Behavioral pattern profile: A tool for the description of behavior to be used in the genetics clinic
Endangered species
Dent disease‐like phenotype and the chloride channel ClC‐4 (<i>CLCN4</i>) gene
New autosomal recessive syndrome with short stature and facio–auriculo–thoracic malformations
Discordance between phenotypic appearance and genotypic findings in a familial case of classical Ehlers–Danlos syndrome
Familial acromelic frontonasal dysostosis: Autosomal dominant inheritance with reduced penetrance
Outcome in Down syndrome fetuses with cardiac anomalies
Phenotype characterization and natural history of spondylothoracic dysplasia syndrome: A series of 27 new cases Puerto Rican population
Response to “…spondylothoracic dysplasia syndrome: A series of 27 new cases” by Cornier et al.
Mutations in IRF6 do not cause Bartsocas–Papas syndrome in a family with two affected sibs
Letter in response to “RNA processing defects of the helicase gene <i>RECQL4</i> in a compound heterozygous Rothmund–Thomson patient” by Beghini et al.
Book Review
Czech Dysplasia Metatarsal Type
A newly recognized chondrodysplasia with multiple dislocations
A locus for nonspecific X‐linked mental retardation mapped to a 22.3 cM region of Xp11.3‐q22.3 Chinese
Subtelomere FISH in 50 children with mental retardation and minor anomalies, identified by a checklist, detects 10 rearrangements including a de novo balanced translocation of chromosomes 17p13.3 and
Mutation frequencies for glycogen storage disease Ia in the Ashkenazi Jewish population cosmopolitan European populations
Congenital idiopathic intestinal pseudo‐obstruction and hydrocephalus with stenosis of the aqueduct of sylvius
Search for loss of heterozygosity and mutation analysis of <i>KRIT1</i> gene in CCM patients
Atypical findings in Kabuki syndrome: Report of 8 patients in a series of 20 and review of the literature
De novo translocation t(5;18)(q33.1;q12.1) associated with autistic disorder
Hypomorphic alleles within the <i>EBP</i> gene cause a phenotype quite different from Conradi–Hünermann–Happle syndrome
Endocrine abnormalities in patients with Jacobsen (11q−) syndrome
The 11q terminal deletion disorder: A prospective study of 110 cases
Genotype/phenotype analysis in a patient with pure and complete trisomy 12p
Does bilirubin protect against hemochromatosis gene (<i>HFE</i>) related mortality?
Circulating COMP is decreased in pseudoachondroplasia and multiple epiphyseal dysplasia patients carrying COMP mutations
Re: Familial cryptic translocation (2;17) ascertained through recurrent spontaneous abortions: Bruyere H, Rajcan‐Separovic E, Doyle J, Pantzar T, Langlois S
Analysis of the developmental <i>SIX6</i> homeobox gene in patients with anophthalmia/microphthalmia
Increased prevalence of unprovoked seizures in patients with a 22q11.2 deletion
<i>parkin</i> mutation analysis in clinic patients with early‐onset Parkinson's disease
Is brachydactyly type Ballard a variant of brachydactyly type E?
Undetectable maternal serum uE3 and postnatal abnormal sterol and steroid metabolism in Antley–Bixler syndrome
A novel mutation of tumor necrosis factor receptor alpha type 1 associated with TRAPS and amyloidosis
Choanal atresia associated with prenatal methimazole exposure: Three new patients
Ring chromosome 18q and jumping translocation 18p in an adult male with hypergonadotrophic hypogonadism
Inverted duplication of 15q with terminal deletion in a multiple malformed newborn with intrauterine growth failure and lethal phenotype
Functional disomy of Xp including duplication of <i>DAX1</i> gene with sex reversal due to t(X;Y)(p21.2;p11.3)
Pregnancy in a healthy woman with untreated citrullinemia
Gene discovery in the auditory system using a tissue specific approach
Turner syndrome: Evaluation of prenatal diagnosis in 19 European registries
Germline mosaicism resulting in the transmission of severe hemophilia B from a grandfather with a mild deficiency
Infant with severe penicillamine embryopathy born to a woman with Wilson disease
Phenotypic and molecular variability of the holoprosencephalic spectrum
Severe lower limb defects in exstrophy of the cloaca
Newly recognized autosomal recessive acrofacial dysostosis syndrome resembling Nager syndrome
Kantaputra mesomelic dysplasia: A second reported family
MICRO syndrome: An entity distinct from COFS syndrome
Psychological impact of genetic testing for familial hypercholesterolemia within a previously aware population: A randomized controlled trial
Infantile systemic hyalinosis: A clinicopathological study
Novel mutations associated with metachromatic leukodystrophy: Phenotype and expression studies in nine Czech and Slovak patients
Malignant degeneration of presacral teratoma in the Currarino anomaly
Perrault syndrome: Evidence for progressive nervous system involvement
Medical privacy and the disclosure of personal medical information: The beliefs and experiences of those with genetic and other clinical conditions
Ring (Y) in two azoospermic men
Nonmosaic smallest duplication of 12q24.31‐qter: The first reported case
Congenital cataract, microphthalmia, hypoplasia of corpus callosum and hypogenitalism: Report and review of Micro syndrome
A new insight into fragile X syndrome among Basque population Caucasian populations
Oto‐onycho‐peroneal syndrome: Further delineation and first fetal report
Phenotype and X inactivation in 45,X/46,X,r(X) cases
RE: Segmental neurofibromatosis in childhood [Listernick et al., 2003: Am. J. Med. Genet. 121A:132–135.]
Is cascade testing a sensible method of screening a population for autosomal recessive disorders?
Early hearing detection and intervention programs: Opportunities for genetic services
Interstitial deletion of chromosome 2q32‐34 associated with multiple congenital anomalies and a urea cycle defect (CPS I deficiency)
Osteopathia striata with cranial sclerosis: Clinical, radiological, and bone histological findings in an adolescent girl French-Canadian
Polymorphisms in the methylenetetrahydrofolate reductase and methionine synthase reductase genes and homocysteine levels in Brazilian children
Mesomelic dysplasia, Kantaputra type: Clinical report, prenatal diagnosis, no evidence for <i>SHOX</i> deletion/mutation
Compound heterozygous mutations of cytochrome P450 oxidoreductase gene (<i>POR</i>) in two patients with Antley–Bixler syndrome
Severe congenital myopathy with Möbius, Robin, and Poland sequences: New aspects of the Carey–Fineman–Ziter syndrome
Professor Robin Michael Winter 1950–2004: An appreciation
Pontine hypoplasia in Carey–Fineman–Ziter (CFZ) syndrome
The Carey–Fineman–Ziter syndrome: Follow‐up of the original siblings and comments on pathogenesis
FISH characterization of a dicentric Yq (p11.32) isochromosome in an azoospermic male
Toriello–Carey syndrome and unbalanced translocation t(8;18)(p12;q22)
Attitudes of deaf individuals towards genetic testing
Mutation analysis of the <i>M6b</i> gene in patients with Pelizaeus–Merzbacher‐like syndrome
On the reported 8p22‐p23.1 duplication in Kabuki make‐up syndrome (KMS) and its absence in patients with typical KMS
Genetics and alcoholism among at‐risk relatives II: Interest and concerns about hypothetical genetic testing for alcoholism risk
Pseudoaminopterin syndrome and trisomy 9
Superficial siderosis: A potentially important cause of genetic as well as non‐genetic deafness
Genetics and alcoholism among at‐risk relatives I: Perceptions of cause, risk, and control
Intrauterine onset of acute neuropathic type 2 Gaucher disease: Identification of a novel insertion sequence
Outcomes of systematic screening for optic pathway tumors in children with Neurofibromatosis Type 1
Möbius sequence, Robin complex, and hypotonia: Severe expression of brainstem disruption spectrum versus Carey–Fineman–Ziter syndrome
Genetic influences on life span and survival among elderly African‐Americans, Caribbean Hispanics, and Caucasians African‐Americans, Caribbean Hispanics
Knowledge without truth: Screening for complications of neurofibromatosis type 1 in childhood
Reply to the letter from Marino et al. “Outcome in Down syndrome fetuses with cardiac anomalies”
De novo complex chromosomal rearrangements (CCR) involving chromosome 1, 5, and 6 resulting in microdeletion for 6q14 in a female carrier with psychotic disorder
Newly recognized syndrome of metaphyseal undermodeling, spondylar dysplasia, and overgrowth: Report of two adolescents and a child
Cognitive and adaptive behavior profiles of children with Angelman syndrome
Genetic heterogeneity in Usher syndrome
Disomy of distal Xq in males: Case report and overview
Craniosynostosis with extra copy of <i>MSX2</i> in a patient with partial 5q‐trisomy
Molecular characterization of inv dup del(8p): Analysis of five cases 26% in Europeans; 39% in a normal Japanese population
Is G6PD A‐ deficiency associated with recurrent stillbirths in The Gambia?
Microcephalic osteodysplastic primordial dwarfism type II: A child with café au lait lesions, cutis marmorata, and moyamoya disease
Clinical and genetic heterogeneity in Desbuquois dysplasia
Cardiomyopathy in Coffin–Lowry syndrome
Risk calculations: Still essential in the molecular age
A new patient with pure trisomy 4p resulting from isochromosome formation and whole arm translocation
Proposed association between the <i>COL1A1</i> and <i>COL1A2</i> genes and otosclerosis is not supported by a case‐control study in Spain
Identification of a new nonsense mutation (Tyr129Stop) of the SRY gene in a newborn infant with XY sex‐reversal
Infantile metachromatic leukodystrophy (MLD) in a compound heterozygote for the c.459 + 1G &gt; A mutation and a complete deletion of the <i>ARSA</i> gene
Clinical application of genetic testing for deafness
A kindred with Cockayne syndrome caused by multiple splicing variants of the <i>CSA</i> gene
<i>SHOX</i> mutations in a family and a fetus with Langer mesomelic dwarfism
Identification of a mutation in the <i>PKD2</i> gene in a family with age‐related macular degeneration
Prospective study comparing HR‐CGH and subtelomeric FISH for investigation of individuals with mental retardation and dysmorphic features and an update of a study using only HR‐CGH
Trisomy 14pter → q21: A case with associated ovarian germ cell tumor and review of the literature
Molecular cytogenetic characterization of two small chromosome 8 derived supernumerary mosaic markers
Spondyloenchondrodysplasia: Clinical variability in three cases
Induction of premature chromatid separation (PCS) in individuals with PCS trait and in normal controls
Hermansky–Pudlak syndrome type 4 in a patient from Sri Lanka with pulmonary fibrosis
Novel Connexin 43 (GJA1) mutation causes oculo–dento–digital dysplasia with curly hair
Mild Wolf–Hirschhorn phenotype and partial gh deficiency in a patient with a 4p terminal deletion
Clinical, cytogenetic, and molecular characterization of a patient with a de novo interstitial 22q12 duplication
Investigation of the W185X nonsense mutation of <i>PVRL1</i> gene in Italian nonsyndromic cleft lip and palate patients Italian
Analysis of chromosome segregation in sperm from a chromosome 2 inversion heterozygote and assessment of an interchromosomal effect
New case of thyroid dysgenesis and clinical signs of hypothyroidism in Williams syndrome
Hereditary fetal brain degeneration resembling fetal brain disruption sequence in two sibships Dutch descent; Hindu population of Surinam
Natural history of twin disruption sequence
Growth, behavior, and clinical findings in 27 patients with Kabuki (Niikawa–Kuroki) syndrome
Hypertelorism, ptosis, and myopia associated with drug‐resistant epilepsy, mental delay, growth deficiency, ectodermal defects, and osteopenia
Early diagnosis of Wolf–Hirschhorn syndrome triggered by a life‐threatening event: Congenital diaphragmatic hernia
Identification of 14 rare marker chromosomes and derivatives by spectral karyotyping in prenatal and postnatal diagnosis
Severe subacute G<sub>M2</sub> gangliosidosis caused by an apparently silent <i>HEXA</i> mutation (V324V) that results in aberrant splicing and reduced <i>HEXA</i> mRNA
Microcephalic osteodysplastic primordial short stature type II with café‐au‐lait spots and moyamoya disease: Another patient
46,XX sex reversal with partial duplication of chromosome arm 22q
A chromosome 21‐derived minute marker in a mosaic trisomy 21 background: Implications for risk assessments in marker chromosome cases
Metabolic and molecular basis of peroxisomal disorders: A review
Integrative classification of morphology and molecular genetics in central nervous system malformations
3D analysis of facial morphology
Cohen syndrome in the Ohio Amish Amish; Amish kindreds; the Amish; ethnic populations; ethnic group
Macrocephaly, mental retardation, dysmorphism, and spastic paraplegia
Growth hormone deficiency, anorectal agenesis, and brachycamptodactyly: A phenotype overlapping Stratton–Parker syndrome
Ablepharon‐macrostomia syndrome in a 46‐year‐old woman
A rapid diagnostic method for a retrotransposal insertional mutation into the <i>FCMD</i> gene in Japanese patients with Fukuyama congenital muscular dystrophy
A novel missense mutation in the galactosyltransferase‐I (<i>B4GALT7</i>) gene in a family exhibiting facioskeletal anomalies and Ehlers–Danlos syndrome resembling the progeroid type
A 3‐bp deletion mutation of <i>PTPN11</i> in an infant with severe Noonan syndrome including hydrops fetalis and juvenile myelomonocytic leukemia
New onset focal weakness in children with Down syndrome
Attenuated phenotype in a child with trisomy for 1q due to unbalanced X;1 translocation [46,X,der(X),t(X;1)(q28;q32.1)]
Non‐neurogenic sarcomas in four children and young adults with neurofibromatosis type 1
Gene responsible for mitochondrial myopathy and sideroblastic anemia (MSA) maps to chromosome 12q24.33 Jewish-Iranian; Israeli; Iranian
Polyvalvular heart disease associated with short stature, facial anomalies, and mental retardation: An additional familial report
A female with complete lack of Müllerian fusion, postaxial polydactyly, and tetralogy of fallot: Genetic heterogeneity of McKusick–Kaufman syndrome or a unique syndrome?
Methylenetetrahydrofolate reductase enzyme polymorphisms as maternal risk for down syndrome among Turkish women
Myotonic dystrophy—no evidence for preferential transmission of the mutated allele: A prenatal analysis
Minimal clinical expression of the holoprosencephaly spectrum and of Currarino syndrome due to different cytogenetic rearrangements deleting the <i>sonic hedgehog</i> gene and the <i>HLXB9</i> gene at
Treacher Collins syndrome with craniosynostosis, choanal atresia, and esophageal regurgitation caused by a novel nonsense mutation in <i>TCOF1</i>
A retrospective survey of perinatal risk factors of 104 living children with holoprosencephaly
Cerebellum—small brain but large confusion: A review of selected cerebellar malformations and disruptions
X‐linked cardioskeletal myopathy and neutropenia (Barth syndrome): An update
A severe form of amyloidotic polyneuropathy in a Costa Rican family with a rare transthyretin mutation (Glu54Lys)
Biochemical diagnosis of Antley–Bixler syndrome by steroid analysis
Can amputated digits point to clues about etiology?
A further case of coincidental Prader–Willi and Klinefelter syndromes
9q34.3 deletion syndrome in three unrelated children
Novel <i>SURF1</i> mutation in a child with subacute encephalopathy and without the radiological features of Leigh Syndrome
Premature ovarian failure and FRAXA premutation: Positive correlation in a Brazilian survey
Autosomal dominant inheritance of infantile myofibromatosis
Ala67Thr polymorphism in the Agouti‐related peptide gene is associated with inherited leanness in humans
Book review
The challenge in hidden treasures: A never ending story
Peroxisome biogenesis disorders with prolonged survival: Phenotypic expression in a cohort of 31 patients
Large deletion of the <i>GJB6</i> gene in deaf patients heterozygous for the <i>GJB2</i> gene mutation: Genotypic and phenotypic analysis
Genetic heterogeneity in the multiple lentigines/LEOPARD/Noonan syndromes
Two sibs with fibrochondrogenesis
<i>TGFB1</i> mutations in four new families with Camurati–Engelmann disease: Confirmation of independently arising LAP‐domain‐specific mutations
Discordant phenotypes in first cousins with <i>UBE3A</i> frameshift mutation
Risk of sudden death in the Williams–Beuren syndrome
Clinical, MRI, and pathological features of polymicrogyria in chromosome 22q11 deletion syndrome
Twenty‐four new cases of <i>WT1</i> germline mutations and review of the literature: Genotype/phenotype correlations for Wilms tumor development
Marfanoid habitus with abnormal situs
Klippel–Feil anomaly and neural tube defects
Fine mapping and clinical reevaluation of a Brazilian pedigree with a severe form of X‐linked mental retardation associated with other neurological dysfunction
Identification of mutations in <i>TCOF1</i>: Use of molecular analysis in the pre‐ and postnatal diagnosis of Treacher Collins syndrome
<i>CDKN1C</i> mutation in Wiedemann–Beckwith syndrome patients reduces RNA splicing efficiency and identifies a splicing enhancer
Isolated congenital anosmia with morphologically normal olfactory bulb in two Iranian families: A new clinical entity?
Elevated catecholamine metabolites in patients with Costello syndrome
Psychotic disorders in Prader–Willi syndrome
Leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia (LACH) in two brothers: A novel syndrome?
Unusual features in a patient with neurofibromatosis type 1: Multiple subcutaneous lipomas, a juvenile polyp in ascending colon, congenital intrahepatic portosystemic venous shunt, and horseshoe kidne
Segmentation anomalies of the vertebras and ribs: One expression of the primary developmental field
DNA studies of mono‐ and pseudodicentric isochromosomes 18q
Goethe's bone and the beginnings of morphology
Mutation of <i>SFTPC</i> in infantile pulmonary alveolar proteinosis with or without fibrosing lung disease endogamous white settler population of Réunion Island
A familial balanced inverted insertion ins(15)(q15q13q11.2) producing Prader–Willi syndrome, Angelman syndrome and duplication of 15q11.2‐q13 in a single family: Importance of differentiation from a p
Social behavior profile in young males with fragile X syndrome: Characteristics and specificity
Growth of heterokaryotic monozygotic twins discordant for Ullrich–Turner syndrome during the first years of life Turkish
Genetic analysis of candidate loci in non‐syndromic cleft lip families from Antioquia‐Colombia and Ohio
Clinical Cytogeneticist
National society of genetic counselors 23rd annual education conference <i>call for abstracts</i>
Erratum
Erratum