American Journal of Medical Genetics Part A - 2006

500 articles | Last updated: 2025-12-03 14:12:55
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T A T A T A T A
Nuchal cystic hygroma in five fetuses from 1819 to 1826 in the Meckel‐anatomical collections at the University of Halle, Germany
Autosomal‐recessive syndrome with alopecia, hypogonadism, progressive extra‐pyramidal disorder, white matter disease, sensory neural deafness, diabetes mellitus, and low IGF1
Confidentiality versus duty to inform—An empirical study on attitudes towards the handling of genetic information Norwegian, Swedish
Hemangiomas: Their uses and abuses
Book review
Studies with <i>MMP9</i> gene promoter polymorphism and nonsyndromic cleft lip and palate
Two novel SLC2A10/GLUT10 mutations in a patient with arterial tortuosity syndrome
Clinical‐etiologic correlation in children with Prader‐Willi syndrome (PWS): An interdisciplinary study
Subtelomeric analysis detects a familial 10p;12p rearrangement in two relatives with a distinct syndrome
Reply to Henthorn and Deutsch: Ethnicity versus early environment: Comment on ‘Early Childhood Music Education and Predisposition to Absolute Pitch: Teasing Apart Genes and Environment’ by Peter K. Gr
Trisomy 13 mosaicism in a phenotypically normal child: Description of cytogenetic and clinical findings from early pregnancy beyond 2 years of age
A novel patient with Cooks syndrome supports splitting from “classic” brachydactyly type B
Prevalence of duplications and deletions of the 22q11 DiGeorge syndrome region in a population‐based sample of infants with cleft palate
A syndrome of immunodeficiency, autoimmunity, and spondylometaphyseal dysplasia
Candidate loci for Zimmermann–Laband syndrome at 3p14.3
Novel risk factor in gastroschisis: Change of paternity
Pericentric inversion causing duplication and deletion of chromosome region 13q22 → qter in the offspring
Non‐chromosome 11‐p syndromes in Wilms tumor patients: Clinical and cytogenetic report of two Down syndrome cases and one Turner syndrome case
A patient with duplication (7)(p22.1pter) characterized by array‐CGH
COL2A1–related skeletal dysplasias with predominant metaphyseal involvement
A family‐based association study in Central Europeans: No evidence for the cystathionine beta‐synthase c.844ins68 gene variant as a risk factor for non‐syndromic cleft lip and palate Central Europeans
Recurrent insertional polydactyly and situs inversus in a Bardet‐Biedl syndrome family
Ethnicity versus early environment: Comment on ‘Early Childhood Music Education and Predisposition to Absolute Pitch: Teasing Apart Genes and Environment’ by Peter K. Gregersen, Elena Kowalsky, Nina K
Fraser and Ablepharon macrostomia phenotypes: Concurrence in one family and association with mutated<i>FRAS1</i>
Hematological abnormalities during the first week of life among neonates with Down syndrome: Data from a multihospital healthcare system
A male infant with a 9.6 Mb terminal Xp deletion including the <i>OA1</i> locus: Limit of viability of Xp deletions in males
Characterization of a 5.3 Mb deletion in 15q14 by comparative genomic hybridization using a whole genome “tiling path” BAC array in a girl with heart defect, cleft palate, and developmental delay
Obesity, hypothyroidism, craniosynostosis, cardiac hypertrophy, colitis, and developmental delay: A novel syndrome
A girl with neurofibromatosis type 1, atypical autism and mosaic ring chromosome 17
Duplication/deletion mosaicism of the 7q(21.1 → 31.3) region
Gray matter heterotopias and brachytelephalangic chondrodysplasia punctata: A complication of hyperemesis gravidarum induced vitamin K deficiency?
Sibling cases of Vici syndrome: Sleep abnormalities and complications of renal tubular acidosis
Hans‐Rudolf Wiedemann (1915–2006)
A new chromosome anomaly in a patient with apparent C (trigonocephaly) syndrome
A novel recurrent mitochondrial DNA mutation in <i>ND3</i> gene is associated with isolated complex I deficiency causing Leigh syndrome and dystonia
Angelman syndrome caused by an identical familial 1,487‐kb deletion
Terminal osseous dysplasia with pigmentary defects: Clinical description of a new family
Neuropathic features in fragile X premutation carriers
Unusually severe expression of craniofacial features in Aarskog‐Scott syndrome due to a novel truncating mutation of the <i>FGD1</i> gene
Two novel point mutations in the long‐range SHH enhancer in three families with triphalangeal thumb and preaxial polydactyly
Anophthalmia‐plus syndrome: A clinical report and review of the literature
Frontometaphyseal dysplasia: Mutations in <i>FLNA</i> and phenotypic diversity (Am J Med Genet 140A: 1726–1736)
The new bone biology: Pathologic, molecular, and clinical correlates
Two years of growth hormone therapy in young children with Prader–Willi syndrome: Physical and neurodevelopmental benefits
Neuropsychological evaluation in Lujan–Fryns syndrome: Commentary and clinical report
Immunoglobulin deficiency in Stickler syndrome
Concordant partial urorectal septum malformation sequence in monozygotic twins
The G397A (E133K) change in the <i>AGGF1</i> (<i>VG5Q</i>) gene is a single nucleotide polymorphism in the Spanish population Spanish population
A report of pure 7p duplication syndrome and review of the literature
A syndrome of holoprosencephaly, recurrent infections, and monocytosis
Karyotype–phenotype insights from 11q14.1‐q23.2 interstitial deletions:<i>FZD4</i>haploinsufficiency and exudative vitreoretinopathy in a patient with a complex chromosome rearrangement
Clinical features and management issues in Mowat–Wilson syndrome Northern Europe
A microdeletion 22q11.2 can resemble Shprintzen–Goldberg omphalocele syndrome
Hepatoblastoma in a patient with Goldenhar syndrome born to a diabetic mother
Multiple primary tumors associated with chromosome 9p deletion
A variable combination of features of Noonan syndrome and neurofibromatosis type I are caused by mutations in the <i>NF1</i> gene
A new genomic mechanism leading to cri‐du‐chat syndrome
Unusual dicentric chromosome 22 associated with a 22q13 deletion
Intracranial abnormalities detected by three‐dimensional magnetic resonance imaging in Prader–Willi syndrome
Sudden infant death in a patient with <i>FGFR3</i> P250R mutation
In memoriam: Hooshang Taybi, M.D. (1919–2006)*
Nijmegen breakage syndrome (NBS) due to maternal isodisomy of chromosome 8
High‐throughput analysis of chromosome abnormality in spontaneous miscarriage using an MLPA subtelomere assay with an ancillary FISH test for polyploidy
Nosology and classification of genetic skeletal disorders: 2006 revision
Robert J. Gorlin (1923–2006): Teacher, colleague, and friend
A new distal arthrogryposis syndrome characterized by plantar flexion contractures
Blepharophimosis, corneal vascularization, deafness, and acroosteolysis: A “new” syndrome?
Detection of low‐level mosaicism by array CGH in routine diagnostic specimens
Energy expenditure and physical activity in Prader–Willi syndrome: Comparison with obese subjects
Prenatal detection of subtelomeric rearrangements by multi‐subtelomere FISH in a cohort of fetuses with major malformations
Phenotypic definition of Chiari type I malformation coupled with high‐density SNP genome screen shows significant evidence for linkage to regions on chromosomes 9 and 15
<i>GLI2</i> mutations in four Brazilian patients: How wide is the phenotypic spectrum?
PVRL1 variants contribute to non‐syndromic cleft lip and palate in multiple populations
In memoriam: Robert J. Gorlin, 1923–2006
Robert J. Gorlin, 1923–2006: A remembrance
High incidence of short rib‐polydactyly syndrome type IV in a Hungarian Roma subpopulation
Trismus‐pseudocamptodactyly syndrome is caused by recurrent mutation of <i>MYH8</i> North American and European TPS pedigrees; Dutch; Dutch‐Kentucky; Dutch family
Tracking rare incidence syndromes (TRIS) project
Frontonasal malformation, first branchial arch anomalies, congenital heart defect, and severe central nervous system involvement: A possible “new” autosomal recessive syndrome?
Further phenotypic and genetic variation in ADULT syndrome
Microdeletion and microduplication 22q11.2 screening in 295 patients with clinical features of DiGeorge/Velocardiofacial syndrome
<i>DLL3</i> as a candidate gene for vertebral malformations
DNA sequence analysis of <i>GJB2</i>, encoding connexin 26: Observations from a population of hearing impaired cases and variable carrier rates, complex genotypes, and ethnic stratification of alleles multi-ethnic control group; ethnic groups; Asians; ethnic stratification
A novel G106D alteration of the <i>SDHD</i> gene in a pedigree with familial paraganglioma Japanese
Family‐based association study of the <i>MTHFR</i> polymorphism C677T in the bladder‐exstrophy‐epispadias‐complex
<i>RAI1</i> point mutations, CAG repeat variation, and SNP analysis in non‐deletion Smith–Magenis syndrome
Antenatal screening tests: Knowledge and practice patterns of obstetricians in Utah
No association between periconceptional multivitamin supplementation and risk of multiple congenital abnormalities: A population‐based case‐control study
A case surviving for over a year of renal tubular dysgenesis with compound heterozygous angiotensinogen gene mutations
Attitudes of genetic counselors towards expanding newborn screening and offering predictive genetic testing to children
<i>CRELD1</i> mutations contribute to the occurrence of cardiac atrioventricular septal defects in Down syndrome
Whole genome microarray analysis of gene expression in an imprinting center deletion mouse model of Prader–Willi syndrome
Risks of human limb deficiency anomalies associated with 29 SNPs of genes involved in homocysteine metabolism, coagulation, cell–cell interactions, inflammatory response, and blood pressure regulation
Trigonocephaly in Muenke syndrome
Associated malformations in congenital diaphragmatic hernia cases in the last 15 years in a tertiary referral institute
Atypical cases of Angelman syndrome
Daughter and her mildly affected father with Keipert syndrome
Response to letter to the editor: “Hypoparathyroidism‐Retardation‐Dysmorphism Syndrome in a Female Child: A New Variant Not Caused by a <i>TBCE</i> Mutation—Clinical Report and Review”
Psychopathology in the Lujan–Fryns syndrome: Report of two patients and review
X‐chromosome inactivation patterns in females with Prader–Willi syndrome
FISH and array‐CGH analysis of a complex chromosome 3 aberration suggests that loss of <i>CNTN4</i> and <i>CRBN</i> contributes to mental retardation in 3pter deletions
Polymicrogyria and deletion 22q11.2 syndrome: Window to the etiology of a common cortical malformation
Association between the NAT1 1095C &gt; A polymorphism and homocysteine concentration
Analysis of a Scottish founder effect narrows the TAPVR‐1 gene interval to chromosome 4q12
Parietal bone agenesis with gracile bones and splenic hypoplasia/aplasia: Clinico‐pathologic report and differential diagnosis with review of cranio‐gracile bone syndromes, “osteocraniostenosis” and K
V37I connexin 26 allele in patients with sensorineural hearing loss: Evidence of its pathogenicity
Deaths due to choking in Prader–Willi syndrome
Core binding factor beta (<i>CBFB</i>) haploinsufficiency due to an interstitial deletion at 16q21q22 resulting in delayed cranial ossification, cleft palate, congenital heart anomalies, and feeding d
Evolutionary pathways in nature: A phylogenetic approach. By John C. Avise. Cambridge University Press, New York, 2006, 286 p.
RE: Correspondence from Wieczorek &amp; Gillessen‐Kaesbach and Hing &amp; Parisi
ADHD symptoms in children with FXS
Trends and racial disparities in muscular dystrophy deaths in the United States, 1983–1998: An analysis of multiple cause mortality data Whites; Blacks
Trisomy 18: Changes in sex ratio during intrauterine life
Diagnostic yield of chromosome analysis in patients with developmental delay or mental retardation who are otherwise nondysmorphic
Oculo‐oto‐facial dysplasia (OOFD) versus Burn–McKeown syndrome
Recurrent pancreatitis in mitochondrial cytopathy
Head circumference and height in autism: A study by the collaborative program of excellence in autism
Response to Wieczorek and Gillessen‐Kaesbach letter addressing “A Novel Oculo‐Oto‐Facial Dysplasia in a Native Alaskan Community With Autosomal Recessive Inheritance”
Sensenbrenner syndrome: A new member of the hepatorenal fibrocystic family
Terminal 14q32.33 deletion: Genotype–phenotype correlation
Attitudes and beliefs of pediatricians and genetic counselors regarding testing and screening for CF and G6PD: Implications for policy
<i>PTCH</i> mutations in four Brazilian patients with holoprosencephaly and in one with holoprosencephaly‐like features and normal MRI
Single maxillary central incisor, holoprosencephaly, and holoprosencephaly‐like phenotype
Robert J. Gorlin and the dysmorphology conferences
Craniofacial and dental phenotype of Smith–Magenis syndrome
A newly recognized polyosteolysis/hyperostosis syndrome
<i>SIX3</i> mutations with holoprosencephaly
Holoprosencephaly‐like phenotype: Clinical and genetic perspectives
Contiguous gene syndrome of holoprosencephaly and hypotrichosis simplex: Association with an 18p11.3 deletion
Scientific abstract submissions presented at the 27th Annual PWSA (USA) National Conference in Orlando, Florida
Mitochondrial dysfunction in Stüve–Wiedemann syndrome in a patient carrying an <i>ND1</i> gene mutation
Elejalde syndrome—A case report
Chromosome 10q24.3‐qter deletion associated with left‐sided first branchial arch defect, diaphragmatic eventration, and duplicated renal pelvis
Early‐onset low‐grade papillary carcinoma of the bladder associated with Apert syndrome and a germline <i>FGFR2</i> mutation (Pro253Arg)
Festschrift for Dr. John M. Opitz: Pathogenesis of cardiac conduction disorders in children genetic and histopathologic aspects
A fourth locus for hereditary hemorrhagic telangiectasia maps to chromosome 7
Bladder exstrophy and Epstein type congenital macrothrombocytopenia: Evidence for a common cause?
<i>BRCA1/2</i> testing in hereditary breast and ovarian cancer families III: Risk perception and screening
A family with X‐linked optic atrophy linked to the OPA2 locus Xp11.4‐Xp11.2
Somatic mosaicism for an <i>HRAS</i> mutation causes Costello syndrome
ATRX syndrome in a girl with a heterozygous mutation in the <i>ATRX</i> Zn finger domain and a totally skewed X‐inactivation pattern
Bifid ribs and unusual vertebral anomalies diagnosed in an anatomical specimen. Gorlin syndrome?
Additional chromosomal abnormalities in patients with a previously detected abnormal karyotype, mental retardation, and dysmorphic features
Molecular basis of human dentin diseases
Variants in mitochondrial tRNA<sup>Glu</sup>, tRNA<sup>Arg</sup>, and tRNA<sup>Thr</sup> may influence the phenotypic manifestation of deafness‐associated 12S rRNA A1555G mutation in three Han Chinese
Novel mitochondrial DNA mutations associated with myopathy, cardiomyopathy, renal failure, and deafness
Vascular update: Morphogenesis, tumors, malformations, and molecular dimensions
Significance of case reports in the advancement of medical scientific knowledge
4q35 deletion and 10p15 duplication associated with immunodeficiency
Introduction to John M. Opitz Festschrift
Familial occurrence of multiple pterygium syndrome: Expression in a heterozygote of the recessive form or variability of the dominant form?
Consanguineous marriage and congenital heart defects: A case‐control study in the neonatal period
Colophon: Vere dignum et justum est … An unedited MS
Diagnostic yield of various genetic approaches in patients with unexplained developmental delay or mental retardation
Linking Antley–Bixler syndrome and congenital adrenal hyperplasia: A novel case of P450 oxidoreductase deficiency
Risk of limb deficiency defects associated with <i>NAT1</i>, <i>NAT2</i>, <i>GSTT1</i>, <i>GSTM1</i>, and <i>NOS3</i> genetic variants, maternal smoking, and vitamin supplement intake
High cognitive functioning and behavioral phenotype in Pallister‐Killian syndrome
Prenatal diagnosis of episodic tachypnea in an infant with OFD VI
Expanding the clinical spectrum of <i>MYCN</i>‐related Feingold syndrome
<i>DHCR7</i> mutation carrier rates and prevalence of the RSH/Smith‐Lemli‐Opitz syndrome: Where are the patients? Central European populations
Clinical experience with array CGH: Case presentations from nine months of practice
Prevalence of Angelman syndrome and Prader–Willi syndrome in Estonian children: Sister syndromes not equally represented
Tetralogy of Fallot with absent pulmonary valve in a de novo derivative chromosome 9 with duplication of 9p13 → 9pter and deletion of 9q34.3
Atrioventricular block and wiry hair in Teebi hypertelorism syndrome
<i>Patched</i> mutations and hairy skin patches: A new sign in Gorlin syndrome
Response to: Multiple aneuploidy recurrence risk
Cornelia de Lange syndrome: Parental preferences regarding the provision of medical information
Autism spectrum disorder in Fragile X syndrome: Differential contribution of adaptive socialization and social withdrawal
Maternal 21‐hydroxylase deficiency and uniparental isodisomy of chromosome 6 and X results in a child with 21‐hydroxylase deficiency and Klinefelter syndrome
A non‐obese boy with Prader‐Willi syndrome shows cardiopulmonary impairment due to severe kyphoscoliosis
Epiphyseal dysplasia and other skeletal anomalies in a patient with the 6p25 microdeletion syndrome
Is there a higher incidence of maternal uniparental disomy 14 [upd(14)mat]? Detection of 10 new patients by methylation‐specific PCR
The Helena syndromes
A previously unreported mutation in a Currarino syndrome kindred
Array comparative genomic hybridization analysis in first‐trimester spontaneous abortions with ‘normal’ karyotypes
Impact of neurofibromatosis 1 on Quality of Life: A cross‐sectional study of 176 American cases
Dwarfism in the ancient Mediterranean world
Clinical phenotype and molecular characterization of 6q terminal deletion syndrome: Five new cases
XX male with sex reversal and a de novo 11;22 translocation
Mutation analysis of the <i>FRAS1</i> gene demonstrates new mutations in a propositus with Fraser syndrome
Multiple aneuploidy recurrence risk
Bannayan–Riley–Ruvalcaba syndrome with reactive nodular lymphoid hyperplasia and autism and a PTEN mutation
Lethal congenital contracture syndrome (LCCS) and other lethal arthrogryposes in Finland—An epidemiological study Finnish
<i>SALL1</i> mutations in sporadic Townes–Brocks syndrome are of predominantly paternal origin without obvious paternal age effect
Clinical outcome of infants with confined placental mosaicism and intrauterine growth restriction of unknown cause
Additional evidence that <i>PTPN11</i> mutations play only a minor role in the pathogenesis of non‐syndromic atrioventricular canal defect
Contiguous hemizygous deletion of <i>TBX5</i>, <i>TBX3</i>, and <i>RBM19</i> resulting in a combined phenotype of Holt‐Oram and ulnar‐mammary syndromes
Determination of the sexual phenotype in a child with 45,X/46,X,Idic(Yp) mosaicism: Importance of the relative proportion of the 45,X line in gonadal tissue
A novel mutation in <i>GDF5</i> causes autosomal dominant symphalangism in two Chinese families Chinese
Sedaghatian spondylometaphyseal dysplasia with pachygyria and absence of the corpus callosum
Anophthalmia‐esophageal atresia syndrome caused by an <i>SOX2</i> gene deletion in monozygotic twin brothers with markedly discordant phenotypes
Identification of a novel polymorphism—the duplication of the <i>NPHP1</i> (nephronophthisis 1) gene
A father of four consecutive trisomic pregnancies with elevated frequencies of associated aneuploid sperm
A novel 8.5 MB dup(1)(p34.1p34.3) characterized by FISH in a child presenting with congenital heart defect and dysmorphic features
Major feeding difficulties in the first reported case of interstitial 20q11.22‐q12 microdeletion and molecular cytogenetic characterization
Errors in manuscript classification
Invited comment: Introductory comments special section: Trisomy 18
Complete trisomy 17p syndrome in a girl with der(14)t(14;17)(p11.2;p11.2)
Comprehensive genetic analysis of relevant four genes in 49 patients with Marfan syndrome or Marfan‐related phenotypes
Hutchinson–Gilford progeria syndrome: Review of the phenotype
Cantu syndrome in a woman and her two daughters: Further confirmation of autosomal dominant inheritance and review of the cardiac manifestations
Extensive acrochordons and pancreatic islet‐cell tumors in tuberous sclerosis associated with <i>TSC2</i> mutations
The genetic basis of tooth development and dental defects
Evidence for an additional locus for split hand/foot malformation in chromosome region 8q21.11–q22.3
An efficient chemical method to generate repetitive sequences depleted DNA probes
Mosaic ring 20 with no detectable deletion by FISH analysis: Characteristic seizure disorder and literature review
Deletion of chromosome 1p36 is associated with periventricular nodular heterotopia
Deletion at 14q22‐23 indicates a contiguous gene syndrome comprising anophthalmia, pituitary hypoplasia, and ear anomalies
Opitz trigonocephaly C syndrome in a boy with a de novo balanced reciprocal translocation t(3;18)(q13.13;q12.1)
Normal values for morphological abnormalities in school children mixed ethnic backgrounds
The molecular etiologies and associated phenotypes of amelogenesis imperfecta
Looking different: Understanding diversity in facial form
Clinical dividends from the molecular genetic diagnosis of craniosynostosis
<i>RMRP</i> mutations in cartilage‐hair hypoplasia ethnically heterogeneous; ethnically homogenous groups
Microdissection‐based high‐resolution genomic array analysis of two patients with cytogenetically identical interstitial deletions of chromosome 1q but distinct clinical phenotypes
Trisomy 8q and partial trisomy 22 in a 43‐year‐old man with moderate intellectual disability, epilepsy and large cell non‐Hodgkin lymphoma
Congenital malformations among liveborn infants with trisomies 18 and 13
Male pseudohermaphroditism and gonadal mosaicism in a 47,XY,+22 fetus
Origin and mechanisms of formation of fetus‐in‐fetu: Two cases with genotype and methylation analyses
Paternal isodisomy of chromosome 7 with cystic fibrosis and overgrowth
Frontometaphyseal dysplasia: Mutations in <i>FLNA</i> and phenotypic diversity
Identification of a novel <i>COCH</i> mutation, G87W, causing autosomal dominant hearing impairment (DFNA9)
Mild craniosynostosis with 1p36.3 trisomy and 1p36.3 deletion syndrome caused by familial translocation t(Y;1)
Two sisters with IMAGe syndrome: Cytomegalic adrenal histopathology, support for autosomal recessive inheritance and literature review
Muscle involvement and motor function in amyoplasia
Letter from Baghdad: Coffin–Siris syndrome in a girl with absent kidney
A case of mosaic supernumerary ring chromosome 15 with two copies of the segment 15p11.1–q14
Familial congenital non‐immune hydrops
Genetic disorders among Palestinian Arabs. 4: Genetic clinics in the community
Sudden infant death syndrome: Case‐control frequency differences in paired like homeobox (<i>PHOX</i>) <i>2B</i> gene
First report of prevalence of non‐syndromic hereditary prosopagnosia (HPA)
Pre‐ and postnatal findings in trisomy 17 mosaicism
Distinguishing Costello versus cardio‐facio‐cutaneous syndrome: <i>BRAF</i> mutations in patients with a Costello phenotype
Growth hormone therapy and scoliosis in patients with Prader–Willi syndrome
Mutational and genotype–phenotype correlation analyses in 28 Polish patients with Cornelia de Lange syndrome Polish
A newly recognized autosomal recessive syndrome with short stature and oculo‐skeletal involvement
Further cases of “neighbor” mutations in mucopolysaccharidosis type II
Osteocraniostenosis–hypomineralized skull with gracile long bones and splenic hypoplasia. Four new cases with distinctive chondro‐osseous morphology
Non‐random associations and vascular fields in neurofibromatosis 1: A pathogenetic hypothesis African Americans
Novel mutations in dihydrolipoamide dehydrogenase deficiency in two cousins with borderline‐normal PDH complex activity Ashkenazi Jewish population
Congenital diaphragmatic hernia associated with duplication of 11q23‐qter
Constitutional UPD for chromosome 11p15 in individuals with isolated hemihyperplasia is associated with high tumor risk and occurs following assisted reproductive technologies
Additional mitochondrial DNA mutations may explain extra‐ocular involvement in LHON
Chromosome 3p25 deletion in mother and daughter with minimal phenotypic effect
Molecular cytogenetic analysis of de novo dup(5)(q35.2q35.3) and review of the literature of pure partial trisomy 5q
Book review
Response to letter by Arti Nanda et al.
Frequency of genomic rearrangements involving the SHFM3 locus at chromosome 10q24 in syndromic and non‐syndromic split‐hand/foot malformation
Mosaicism for a full mutation, premutation, and deletion of the CGG repeats results in 22% FMRP and elevated <i>FMR1</i> mRNA levels in a high‐functioning fragile X male
The near universal presence of autism spectrum disorders in children with Smith–Lemli–Opitz syndrome
A newborn with anophthalmia and pulmonary hypoplasia (the Matthew–Wood syndrome)
Book review
Antenatal presentation of the oculo‐auriculo‐vertebral spectrum (OAVS)
Atypical facial clefting in a patient with Goltz syndrome
Genitopatellar syndrome: Expanding the phenotype and excluding mutations in <i>LMX1B</i> and <i>TBX4</i>
An autosomal recessive form of spastic cerebral palsy (CP) with microcephaly and mental retardation European populations
Renal malformations in deletion 22q11.2 patients
The intrafamilial variability of the 22q11.2 microduplication encompasses a spectrum from minor cognitive deficits to severe congenital anomalies
Nicotine metabolizing genes <i>GSTT1</i> and <i>CYP1A1</i> in sudden infant death syndrome
Consanguineous marriage and congenital heart defects: A case‐control study in the neonatal period
A new cohort of <i>MECP2</i> mutation screening in unexplained mental retardation: Careful re‐evaluation is the best indicator for molecular diagnosis
The expanding panorama of split hand foot malformation
A new detection method for <i>ATRX</i> gene mutations using a mismatch‐specific endonuclease
<i>CHD7</i> gene and non‐syndromic cleft lip and palate
Language skills and neuropsychological performance in patients with <i>SHH</i> mutations and a holoprosencephaly‐like phenotype
A germline <i>PTEN</i> mutation with manifestations of prenatal onset and verrucous epidermal nevus
Thanatophoric dysplasia type 2 with encephalocele during the second trimester
A study of familial stuttering
Book review
Problems in the naming of genes
Holoprosencephaly: Clinical evaluation on audiological and brainstem electrophysiological profiles
Autosomal dominant atretic cephalocele with phenotype variability: Report of a Brazilian family with six affected in four generations
Using the TBX5 transcription factor to grow and sculpt the heart
The use of inappropriate, demeaning, and pejorative terminology in gene nomenclature: A comment on Feingold
Craniofacial dyssynostosis in two boys with apparently normal cognitive development
Blepharophimosis‐mental retardation (BMR) syndromes: A proposed clinical classification of the so‐called Ohdo syndrome, and delineation of two new BMR syndromes, one X‐linked and one autosomal recessi
A new case of Grange syndrome without cardiac findings
Metaphyseal dysplasia of Braun–Tinschert type: Report of a Japanese girl Japanese; Germans; Bohemia
Nablus mask‐like facial syndrome is caused by a microdeletion of 8q detected by array‐based comparative genomic hybridization
Pattern of <i>p63</i> mutations and their phenotypes—update
Overgrowth with severe developmental delay following IVF/ICSI: A newly recognized syndrome?
PHOX2B analysis in non‐syndromic neuroblastoma cases shows novel mutations and genotype–phenotype associations
Book review
Identification of a novel <i>EYA1</i> mutation presenting in a newborn with laryngomalacia, glossoptosis, retrognathia, and pectus excavatum
Autosomal dominant syndrome resembling Coffin–Siris syndrome
Comprehensive screening of multiple epiphyseal dysplasia mutations in Japanese population Japanese; Western populations
Epilepsy and deletions at chromosome 2q24
Split‐hand/split‐foot malformation 3 (SHFM3) at 10q24, development of rapid diagnostic methods and gene expression from the region
Report of a del22q11 in a patient with Mayer‐Rokitansky‐Küster‐Hauser (MRKH) anomaly and exclusion of <i>WNT‐4</i>, <i>RAR‐gamma</i>, and <i>RXR‐alpha</i> as major genes determining MRKH anomaly in a
Periventricular nodular heterotopia and Williams syndrome
New cases of Bohring–Opitz syndrome, update, and critical review of the literature
Intracranial anomalies detected by imaging studies in 30 patients with Apert syndrome
Trigonocephaly in a boy with paternally inherited deletion 22q11.2 syndrome
Re: Majewski osteodysplastic primordial dwarfism type II (MOPD II) complicated by stroke: Clinical report and review of cerebral vascular anomalies [Brancati et al., 2005: Am J Med Genet 139A:212–215]
Evaluation of <i>SLC35A3</i> as a candidate gene for human vertebral malformations
Hydrocephaly, penoscrotal transposition, and digital anomalies associated with de novo pseudodicentric rearranged chromosome 13 characterized by classical cytogenetic methods and mBAND analysis
A newly recognized, likely autosomal recessive syndrome comprising agammaglobulinemia, microcephaly, craniosynostosis, severe dermatitis, and other features
Book review
Unexpected resiliency
Autistic behavior in children with fragile X syndrome: Prevalence, stability, and the impact of FMRP
The FG syndrome: Report of a large Italian series Italian
Clinical and molecular characterization of individuals with 18p deletion: A genotype–phenotype correlation
3′ UTR polymorphism of the serotonin transporter gene and sudden infant death syndrome: Haplotype analysis
Book review
Reflections of our past: How human history is revealed in our genes
Skewed X‐chromosome inactivation is not associated with premature ovarian failure in a large cohort of Italian patients
A novel <i>RSK2</i> (<i>RPS6KA3</i>) gene mutation associated with abnormal brain MRI findings in a family with Coffin–Lowry syndrome
A constitutional telomeric translocation showing meiotic instability
Array‐based comparative genomic hybridization facilitates identification of breakpoints of a novel der(1)t(1;18)(p36.3;q23)dn in a child presenting with mental retardation
Genotype–phenotype correlations in mapped split hand foot malformation (SHFM) patients
Camptodactyly, joint contractures, facial, and skeletal defects: Further delineation of the Rozin camptodactyly syndrome
Ectrodactyly with aplasia of long bones (OMIM; 119100) in a large inbred Arab family with an apparent autosomal dominant inheritance and reduced penetrance: Clinical and genetic analysis Arab family from the United Arab Emirates (UAE)
Gastrointestinal phenotype of ATR‐X syndrome
Split hand foot malformation (SHFM): An introduction
A missense mutation in the <i>ZFHX1B</i> gene associated with an atypical Mowat–Wilson syndrome phenotype
T‐genes and limb bud development
Neurobehavioral disorders in patients with Aarskog–Scott syndrome affected by novel <i>FGD1</i> mutations
<i>Dlx</i> homeobox gene control of mammalian limb and craniofacial development
Clinical and epidemiological findings in patients with central ray deficiency: Split hand foot malformation (SHFM) in Manitoba, Canada
De novo pure 12q22q24.33 duplication: First report of a case with mental retardation, ADHD, and Dandy‐Walker malformation
Intrachromosomal triplication 12p11.22–p12.3 and gonadal mosaicism of partial tetrasomy 12p<sup>,</sup>**
Pregnancy outcome of fetuses with trisomy 18 identified by prenatal sonography and chromosomal analysis in a perinatal center
A population‐based case‐control study of isolated primary congenital glaucoma Gypsy origin; Hungarian Gypsy population
Response to “Letter: Fibular Aplasia, Tibial Campomelia and Oligosyndactyly” by Evans and Elliott
Familial thoracic aortic aneurysms and dissections: Three families with early‐onset ascending and descending aortic dissections in women
WAGR(O?) syndrome and congenital ptosis caused by an unbalanced t(11;15)(p13;p11.2)dn demonstrating a 7 megabase deletion by FISH
Pervasive developmental disorders in Prader–Willi syndrome: The Leuven experience in 59 subjects and controls
Sensorineural deafness, hydrocephalus and structural brain abnormalities in two sisters: The Chudley–McCullough syndrome
Chilean primary health workers' knowledge about folic acid supplementation for the prevention of neural tube defects
Response to the Alvarez Nava and Puerta “Y‐chromosome microdeletions in 45,X/46,XY patients”
Keutel syndrome with overlapping features of cutis laxa: A new variant
Schizophrenia in an adult with 6p25 deletion syndrome
Desbuquois syndrome in three sisters with significantly different lengths of survival
The neonatal phenotype of Prader–Willi syndrome
A compound heterozygote harboring novel and recurrent <i>DTDST</i> mutations with intermediate phenotype between atelosteogenesis type II and diastrophic dysplasia
Oto‐spondylo‐megaepiphyseal dysplasia (OSMED): Clinical and radiological findings in sibs homozygous for premature stop codon mutation in the <i>COL11A2</i> gene Egyptian
Congenital chylothorax in Opitz G/BBB syndrome
Molecular cytogenetic analysis of a de novo interstitial chromosome 10q22 deletion
A novel duplication/insertion mutation of <i>NEFL</i> in a patient with Charcot‐Marie‐Tooth disease
Findings from aCGH in patients with congenital diaphragmatic hernia (CDH): A possible locus for Fryns syndrome (Am J Med Genet 140A:17–23, 2006)
Y‐chromosome microdeletions in 45,X/46,XY patients
del5p/dup5q in a ‘cri du chat’ patient without parental chromosomal rearrangement
Letter re: Fibula aplasia, tibial campomelia, and oligodactyly
Introductory comments special section: Trisomy 18
A balanced reciprocal translocation in a case of hypomelanosis of Ito with confirmation of mosaicism using buccal cell interphase FISH
Bilateral periventricular heterotopias in an X‐linked dominant transmission in a family with two affected males
<i>FBN1</i>, <i>TGFBR1</i>, and the Marfan‐craniosynostosis/mental retardation disorders revisited
Lack of meiotic crossovers during oogenesis in an apparent 45,X Ullrich–Turner syndrome patient with three children
Literature searches of double trisomy citations are inadequate
Thyroid anomalies in Williams syndrome: Investigation of 95 patients
Linkage analysis of genetic loci for kyphoscoliosis on chromosomes 5p13, 13q13.3, and 13q32
Maternal genotype for the monocyte chemoattractant protein 1 A(‐2518)G promoter polymorphism is associated with the risk of spina bifida in offspring
Meiotic segregation analysis of reciprocal translocations both in sperms and blastomeres
Genotype–epigenotype–phenotype correlations in females with frontometaphyseal dysplasia
Multicolor banding detects a complex three chromosome, seven breakpoint unbalanced rearrangement in an ICSI‐derived fetus with multiple abnormalities
Folate gene polymorphisms and the risk of Down syndrome pregnancies in young Italian women
Aplasia and duplication of the thumb and facial clefts associated with fetal trisomy 18
Prenatal diagnosis of hypochondroplasia: Report of two cases
Can we exclude the <i>TXNIP</i> gene as a candidate gene for familial combined hyperlipidemia?
Familial Klippel–Feil anomaly and t(5;8)(q35.1;p21.1) translocation
Trisomy 18 in a second 20‐year‐old woman
Clarification of data reported in “Cleidocranial Dysplasia: Molecular Genetic Analysis and Phenotypic‐Based Description of a Middle European Patient Group” (AJMG 139A:78–85) Middle European
Behavioral and temperamental features of children with Costello syndrome
Fumarase deficiency caused by homozygous P131R mutation and paternal partial isodisomy of chromosome 1
A prospective study of PHACE syndrome in infantile hemangiomas: Demographic features, clinical findings, and complications
Reporting genetic results in research studies: Summary and recommendations of an NHLBI working group
Maternal polymorphisms 677C‐T and 1298A‐C of MTHFR, and 66A‐G MTRR genes: Is there any relationship between polymorphisms of the folate pathway, maternal homocysteine levels, and the risk for having a
Screening for conditions that do not meet the Wilson and Jungner criteria: The case of Duchenne muscular dystrophy
HOXA1 mutations are not a common cause of Duane anomaly
No major contribution of the TGFBR1- and TGFBR2-mediated pathway to Kabuki syndrome
Regarding trisomy 18
A species not extinct: Publication of case reports and scientific knowledge
Metabolic disorders detectable by tandem mass spectrometry and unexpected early childhood mortality: A population-based study
De novo isodicentric X chromosome: 46,X,idic(X)(q24), and summary of literature
Non-lethal congenital hypotonia due to glycogen storage disease type IV
Survival with trisomy 18—data from Switzerland
Clinical characteristics and survival of trisomy 18 in a medical center in Taipei, 1988–2004
Neonatal management of trisomy 18: Clinical details of 24 patients receiving intensive treatment
Neonatal-onset multisystem inflammatory disease (NOMID) due to a novel S331R mutation of theCIAS1 gene and response to interleukin-1 receptor antagonist treatment
Malignancy in chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome)
A novel 8 Mb interstitial deletion of chromosome 8p12-p21.2
A Japanese boy with apparent Bohring-Opitz or “C-like” syndrome Japanese
Outcomes of clinical examination and genetic testing of 500 individuals with hearing loss evaluated through a genetics of hearing loss clinic
The perceived personal control (PPC) questionnaire as an outcome of genetic counseling: Reliability and validity of the instrument
Rapp–Hodgkin ectodermal dysplasia syndrome: The clinical and molecular overlap with Hay–Wells syndrome
Phenotype resembling Donnai–Barrow syndrome in a patient with 9qter;16qter unbalanced translocation
Genotype–phenotype correlation of the Wilson diseaseATP7B gene
Association of Adams–Oliver syndrome and hepatoportal sclerosis: An additional case
CHARGE syndrome: Relations between behavioral characteristics and medical conditions
AtypicalZFHX1B mutation associated with a mild Mowat–Wilson syndrome phenotype
Bioethics and the new embryology: Springboards for debate. By Scott F. Gilbert, Anna L. Tyler, and Emily J. Zackin. Sinauer Associates Inc., Sunderland, 2005
Malformations in children with cancer
Clinical and biochemical characteristics in patients with a high mutant load of the mitochondrial T8993G/C mutations
Progressive osseous heteroplasia controlled by intravenous administration of pamidronate
The <i>ARX</i> mutations: A frequent cause of X‐linked mental retardation
Tracheobronchial anomalies in chromosome 22q11.2 microdeletion
Familial dilated cardiomyopathy hypergonadotrophic hypogonadism associated with thyroid hemiagenesis
Delineation of a 2q deletion in a girl with dysmorphic features and epilepsy
Oculocerebrocutaneous and encephalocraniocutaneous lipomatosis syndromes: Blind men and an elephant or separate syndromes?
Fetus with two identical reciprocal translocations: Description of a rare complication of consanguinity
Familial recurrence of anomalous origin of right pulmonary artery from the aorta
Thirty‐two year follow‐up of the first patient reported with the Floating‐Harbor syndrome
Acceptance of genetic testing for hereditary breast ovarian cancer among study enrollees from an African American kindred
Phosphatidylethanolamine <i>N</i>‐methyltransferase (<i>PEMT</i>) gene polymorphisms and risk of spina bifida
Correspondence concerning Hunter and Yotsuyanagi's “The External Ear: More Attention to Detail May Aid Syndrome and Contribute Answers to Embryological Questions”
Response to Bader et al. letter addressing “The External Ear: More Attention to Detail May Aid Syndrome Diagnosis and Contribute Answers to Embryological Questions”
LEOPARD syndrome: Clinical diagnosis in the first year of life
Dural ectasia in children with Marfan syndrome: A prospective, multicenter, patient‐control study
Prader–Willi syndrome: Development and manifestations. By Joyce Whittington and Tony Holland
Research on stored biological samples: Views of African American and White American cancer patients African American; White American
A novel oculo-oto-facial dysplasia in a Native Alaskan community with autosomal recessive inheritance
Arithmetic difficulties in females with the fragile X premutation
The principles of clinical cytogenetics, second edition. Edited by S.L. Gersen and M.B. Keagle. Humana Press, Totowa, NJ, 2005, 596p.
Nonimmune idiopathic hydrops fetalis and congenital lymphatic dysplasia
The von Hippel–Lindau (<i>VHL</i>) germline mutation V84L manifests as early‐onset bilateral pheochromocytoma
<i>NTNG1</i> mutations are a rare cause of Rett syndrome
The natural history of trisomy 12p
Orofaciodigital syndrome with cerebral dysgenesis
Molecular characterization of an 11q interstitial deletion in a patient with the clinical features of Jacobsen syndrome
A new syndrome of microtia with mixed type hearing loss, renal agenesis, and multiple skeletal anomalies
Cognitive‐behavioral profiles of females with the fragile X mutation
Discordant encephalocele in monozygotic twins
Spondyloenchondrodysplasia with spasticity, cerebral calcifications, and immune dysregulation: Clinical and radiographic delineation of a pleiotropic disorder
Case of chromosome 6p25 terminal deletion associated with Axenfeld–Rieger syndrome and persistent hyperplastic primary vitreous
Delineation of a 2.2 Mb microdeletion at 5q35 associated with microcephaly and congenital heart disease
Clinical findings in 33 subjects with large supernumerary marker(15) chromosomes and 3 subjects with triplication of 15q11‐q13
Molecular cytogenetic analysis of a de novo interstitial deletion of 5q23.3q31.2 and its phenotypic consequences
Chimerism in twins: Caution is needed in interpretation of karyotypes
Spondylo‐ocular syndrome: A new entity involving the eye and spine
Variable phenotype and associations in chromosome 22q11.2 microdeletion
Primary palmar hyperhidrosis locus maps to 14q11.2‐q13
Genetics and life insurance: Medical underwriting and social policy. Edited by Mark A. Rothstein. The MIT Press, 2004, 293p.
Genetics of developmental disabilities. Edited by Merlin G. Butler and F. John Meaney.
Angelman syndrome 2005: Updated consensus for diagnostic criteria
Dyggve–Melchior–Clausen syndrome and Smith–McCort dysplasia: Clinical and molecular findings in three families supporting genetic heterogeneity in Smith–McCort dysplasia
Severe, fetal‐onset form of olivopontocerebellar hypoplasia in three sibs: PCH type 5?
Minimal phenotype in a girl with trisomy 15q due to t(X;15)(q22.3;q11.2) translocation
Inverse association between severe nausea and vomiting in pregnancy and some congenital abnormalities
Genotype–phenotype correlation in hereditary hemorrhagic telangiectasia: Mutations and manifestations*
The biallelic expression pattern of X‐linked genes in Klinefelter syndrome by pyrosequencing
Klinefelter syndrome and mediastinal germ cell tumors
Characterization of two supernumerary marker chromosomes in a patient with signs of Klinefelter syndrome, mild facial anomalies, and severe speech delay*
Extended mutational analyses of <i>FGFR1</i> in osteoglophonic dysplasia
A truncating mutation in the <i>IL1RAPL1</i> gene is responsible for X‐linked mental retardation in the MRX21 family
Speech and language impairment and oromotor dyspraxia due to deletion of 7q31 that involves <i>FOXP2</i>
<i>POR</i> R457H is a global founder mutation causing Antley–Bixler syndrome with autosomal recessive trait
Recurrence of <i>SOX2</i> anophthalmia syndrome with gonosomal mosaicism in a phenotypically normal mother
Variable contribution of the <i>MTHFR</i> C677T polymorphism to non‐syndromic cleft lip and palate risk in China
Mitochondrial dysfunction in Brooks–Wisniewski–Brown syndrome
Healthy 12‐year‐old boy with mosaic inv dup(15)(q13)
Eponymous Jacobsen syndrome: Mapping the breakpoints of the original family suggests an association between the distal 1.1 Mb of chromosome 21 and osteoporosis in Down syndrome (<i>Am J Med Genet</i>
Interstitial 2.2 Mb deletion at 9q34 in a patient with mental retardation but without classical features of the 9q subtelomeric deletion syndrome
Systematic search for single nucleotide polymorphisms in a lymphoid tyrosine phosphatase gene (<i>PTPN22</i>): Association between a promoter polymorphism and type 1 diabetes in Asian populations European descent
A novel mutation in the DNA‐binding domain of <i>MAF</i> at 16q23.1 associated with autosomal dominant “cerulean cataract” in an Indian family
Wiedemann–Rautenstrauch syndrome's fibroblasts display a normal in vitro lifespan
Mosaic trisomy 6 and maternal uniparental disomy 6 in a 23‐week gestation fetus with atrioventricular septal defect
Twins with mental retardation and an interstitial deletion 7q34q36.2 leading to the diagnosis of long QT syndrome
Ring chromosome 4 and Wolf–Hirschhorn syndrome (WHS) in a child with multiple anomalies
A 9‐year‐old male with a duplication of chromosome 3p25.3p26.2: Clinical report and gene expression analysis
A nonsense mutation of <i>PEPD</i> in four Amish children with prolidase deficiency Amish
Mosaic trisomy 8 and Townes–Brocks syndrome due to a novel <i>SALL1</i> mutation in the same patient
Welcome to the genome. A user's guide to the genetic past, present, and future. By Rod DeSalle and Michael Yudell. Wiley‐Liss, Hoboken, NJ, 2005, 215 p.
Molecular cytogenetic analysis of a familial interstitial deletion Xp22.2‐22.3 with a highly variable phenotype in female carriers
Hypoparathyroidism‐retardation‐dysmorphism syndrome in a girl: A new variant not caused by a <i>TBCE</i> mutation—clinical report and review
AGTR2 in brain development and function
Role of leptin in regulating appetite, neuroendocrine function, and bone remodeling
18q deletions: Clinical, molecular, and brain MRI findings of 14 individuals
The use of inappropriate, demeaning, and pejorative terminology to describe syndromes
Manifestations in a family with autosomal dominant bone fragility and limb‐girdle myopathy
A subterminal deletion of the long arm of chromosome 10: A clinical report and review
Obstructive sleep apnea in Costello syndrome
BAC array CGH reveals genomic aberrations in idiopathic mental retardation
A microdeletion in Xp11.3 accounts for co‐segregation of retinitis pigmentosa and mental retardation in a large kindred
Association of Jacobsen syndrome and bipolar affective disorder in a patient with a de novo 11q terminal deletion
Schimke immuno‐osseous dysplasia: A cell autonomous disorder?
Andersen‐Tawil syndrome: Prospective cohort analysis and expansion of the phenotype
Narrowing candidate region for monosomy 9p syndrome to a 4.7‐Mb segment at 9p22.2‐p23
Response to Feingold's: The use of inappropriate, demeaning, and perjorative terminology to describe syndromes
Cleidocranial dysplasia plus vascular anomalies with 6p21.2 microdeletion spanning <i>RUNX2</i> and <i>VEGF</i>
Familial congenital non‐immune hydrops, chylothorax, and pulmonary lymphangiectasia
Restrictive dermatopathy: A lethal congenital dermatosis and review of literature
Familial adenomatous polyposis (FAP): Genotype correlation to FAP phenotype with osteomas and sebaceous cysts Danish
Diagnosis and management of heterokaryotypic monochorionic twins
Pure direct duplication (12)(q24.1 → q24.2) in a child with Marcus Gunn phenomenon and multiple congenital anomalies
Behavioral management of a long‐term survivor with tetrasomy 18p
De novo pericentric inversion of chromosome 5 in a girl with mental retardation and unilateral ear malformation
A family with features overlapping Okihiro syndrome, hemifacial microsomia and isolated Duane anomaly caused by a novel <i>SALL4</i> mutation
Finlay–Marks (SEN) syndrome: A sporadic case and the delineation of the syndrome
Shprintzen–Goldberg omphalocele syndrome: A new patient with an expanded phenotype
Karyotype–phenotype analysis and molecular delineation of a 3p26 deletion/8q24.3 duplication case with a virtually normal phenotype and mild cognitive deficit
Monoallelic <i>BUB1B</i> mutations and defective mitotic‐spindle checkpoint in seven families with premature chromatid separation (PCS) syndrome
Yellow teeth, seizures, and mental retardation: A less severe case of Kohlschütter–Tönz syndrome
Two cases of partial trisomy 21 (pter‐q22.1) without the major features of Down syndrome
Characterization of mosaic supernumerary ring chromosomes by array‐CGH: Segmental aneusomy for proximal 4q in a child with tall stature and obesity
A father and son with mental retardation, a characteristic face, inv(12), and insertion trisomy 12p12.3‐p11.2
A novel mutation in the gene encoding TIMM8a, a component of the mitochondrial protein translocase complexes, in a Spanish familial case of deafness‐dystonia (Mohr–Tranebjaerg) syndrome Spanish
Risk evaluation of carriers with chromosome reciprocal translocation t(7;13)(q34;q13) and concomitant meiotic segregation analyzed by FISH on ejaculated spermatozoa
Severe complications in a child with achondroplasia and two <i>FGFR3</i> mutations on the same allele
High heritability of fingertip arch patterns in twin‐pairs
Novel clinical features in a child with partial deletion of chromosome 11 [del(11)(q24.2)]: Further evidence for phenotypic heterogeneity