Genetics in Medicine - 2000

292 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
Subject Index
Mutation in the CSB gene in a patient with Cerebro-Oculo-Facio-Skeletal syndrome
Evaluating data from newborn screening programs - Georgia, 1998
Detection of a de novo mutation in a family with SMA Type I: The importance of dosage testing
Mohr-Tranebjaerg Syndrome is an X-linked Recessive Disorder Characterized by Mitochondrial Dysfunction Associated with Neuronal Cell Death
The molecular genetics of muscular dystrophy
THE ROLE OF FETAL TELEMEDICINE IN OBSTETRIC ULTRASONOGRAPHY
Adult Genetic Screening: Are we ready for hemochromatosis?
A retrospective review of neurobehavioral and psychosocial issues in adults with putative Sotos syndrome
Familial aortic aneurysms
Lethal Pallister-Killian syndrome and Fryns syndrome: diagnostic considerations
An unexpected female patient within a classical Lesch-Nyhan family
Molecular analysis of chromosome 6p rearrangement in retinoblastoma
Prenatal and post-mortem features of a case of Ritscher-Schinzel syndrome
poster listings
Genetics and Public Health in 2121 Century: A Scientific Foundation for Using Genetic Information to Improve Health and Prevent Discease?
Is Assortative Mating the Cause for the High Frequency of Connexin 26 (Cx 26) Deafness?
Concordance among monozygotic and dizygotic twins from a population-based sample for self-reported atopic triad, syndrome x, and psychiatric conditions
mission statement, CMEs and CEUs
Clinical presentation and management in children with mitochondria) disorders
What's new in newborn screening?
Connexin-26 deafness in the United States: Are we ready for the next Millennium?
Focal abdominal wall hernias and pre-axial toe polydactyly define distinct phenotypes and etiologies for infants of diabetic mothers and the VATER Association
An Integrated BAC/PAC Resource for identifying chromosomal abnormalities in solid tumors
FGFR3 Mutations K650N and K650Q Cause Hypochondroplasia
Knowledge and attitudes about carrier testing for hemophilia A among patients and their relatives
Minimal Phenotypic Findings of Down Syndrome in a Patient with True Trisomy 21
Identification of mitochondria) mutation (tRNAlys) & genetic counseling in a family with MERRF syndrome
Clinical and Molecular Studies in a large unique family with Limb-Girdle Muscular Dystrophy and Paget Disease of Bone
A close relationship to Smith-Lemli-Opitz (RSH) patients positively correlates with an increased incidence of high cholesterol, late-onset diabetes, and infertility; and negatively correlates with alc
BRCA testing uptake and participation in ovarian cancer prevention in women at risk for an inherited ovarian cancer susceptibility
Natural history of the Adams-Oliver Syndrome: A report of progressive Central and Peripheral nervous system involvement in a mother and son
Acampomelic campomelic dysplasia with SOX9 mutation
Isochromosome 9p and choroid plexus papilloma: Coincidence or cause?
Ring Chromosome 14 Syndrome: Prenatal diagnosis of two cases with 45,XY,-14/46,XY,r(14)(p11.2q32)
Familial Report of Duplication 9p Syndrome
Public acceptance of an interactive kiosk to educate about folic acid to prevent neural tube defects
Genetic risk assessment in women over 35: natural pregnancies compared to assisted reproductive technology pregnancies
Mitochondria, DNA mutations in chilean patients with Leber Hereditary Optic Neuropathy (LHON)
The HFE 5569A allele defines a low-risk haplotype for hereditary hemochromatosis
Prune belly syndrome in a patient with only a mildly distended bladder
Evaluation of Prenatal Screening Program for Down Syndrome analysis of 96 cases of Down syndrome for last 10 years
Congenital mesoblastic nephroma: perinatal, surgical and genetic features and associated 2D and 3D imaging
Prenatal diagnosis of MIDAS/MLS syndrome associated with a deletion at Xp22.1
Prevalence of Methylenetetrahydrofolate reductase (MTHFR) C677T polymorphism in Northwest Louisiana newborn population
general information
program information
EXHIBITORS AS OF 2/1/00
invited speakers, moderators and program committee members
Historical perspective of informed consent in ethics and the law
The UCLA experience in developing protocols for informed consent
Congenital Disorders of Glycosylation (CDG): Have you seen them?
The Impact of Recombinant DNA on Inborn Errors of Metabolism
One Clinician's View of Clinician/Researcher Interaction
Overview of telegenetics
Experience with Telemedicine Genetic Services at the Medical College of Georgia
Genetic counseling and video phones
Implementing Telemedicine & Principles, Practices, and Issues – A Perspective from Georgia
The Specialty of Genetics: Communicating Our Value to Medical Colleagues
Options and Challenges in the Treatment of Mitochondria) Disease
Prenatal dysmorphology: Evaluation and management
Application of Tandem Mass Spectrometry to Newborn Screening
Maternal complications and sudden infant death in families with mutations in mitochondrial trifunctional protein
Evaluation and management of urea cycle disorders using stable isotope infusions
Absence of congemla1 myotomc dystrophy (CDM) in a baby wth 1000 DMPK CTG repeats born afler a slblmg with CDM
The A986S polymorphism of the calcium-sensing receptor (CASR) gene is a significant contributor to inter-individual variability of serum calcium
Trends in a clinical genetics program for adults
Cyto 2000 – A Collaborative study for the evaluation of blood chromosome mosaicism
Are we making a difference? Genetics in Undergraduate Medical Education
Expanded metabolic screening utilizing tandem mass spectrometry: The Massachusetts experience in the first year
Newborn screening for sickle cell disease: assessing program effectiveness
A Practical Theory-Based Method to Improve Lay Decision-Making for Genetic Testing
MTHFR mutation and a NOS3 polymorphism influence blood pressure characteristics in preeclampsia
Severe Conradi-Hünermann Syndrome (CDPX2) is a phenocopy of peroxisomal Rhizomelic Chondrodysplasia Punctata (RCDP)
Methylmalonic aciduria, hyperhomocysteinemia, hematologic and/or neurologic abnormalities in 3 infants born to mothers with asymptomatic B12 deficiency
RadioHPLC profiles of acyl-camitines improve detection of mild Glutaric Acidemia type II and Short Chain Acyl-CoA Dehydrogenase deficiency
Vitamin B 12-responsive methylmalonic aciduria due to a new inborn error of adenosylcobalamin synthesis, cblH
Clinical and Molecular diagnosis of Nager syndrome in a preterm Infant (27 week gestation) – A case report
BRCAI and BRCA2 Mutation Analysis in At-risk African-American Families: Results and Implications African-American
Mitochondrial disease and disorders of energy metabolism: A recognizable pattern of systemic disease
Clinical Findings in mitochondnal neurogastrolntestinal encephalomyopathy syndrome (MNGIE)
The Scapuloiliac Dysostosis (Kosenow Syndrome) Spectrum – Two Additional Cases
Retinitis pigmentosa, growth hormone deficiency and acromelic skeletal dysplasia in two male siblings: possible familial RHYNS syndrome
Cervical spine anomalies in Weaver syndrome; a diagnostic clue in adults
Evaluation of telemedicine use for clinical genetics services in Iowa
Microgastria in the Genetic Clinic
Ovarian Cancer Screening in Women from Hereditary Breast/Ovarian Cancer Families
Ruvalcaba Syndrome (a rare progeroid syndrome). A new case and review of the literature
A new genetic syndrome among the Old Order Amish of Smicksburg Pennsylvania Old Order Amish
Unique skeletal dysplasia with cataracts, ataxia, learning disability and mild facial dysmorphism
Anonychia and absence of distal phalanges in a patient with apparently balanced t(17;21)(q24.2;g11.2)
Oral clefting, cartilaginous auricular malformations and other anomalies: A provisionally unique autosomal dominant syndrome
Partial Monosomy of Chromosome 5 in 2 Male Siblings – Phenotypic Correlates
Marden-Walker syndrome: case report and review
Severe Saethre-Chotzen syndrome in an infant with a complex chromosome rearrangement
Seckel syndrome phenotype in a live-born with ring 4/monosomy 4 chromosomal mosaicism
An infant with trisomy 9 mosaicism and features of CHARGE association
A third Prader-Willi syndrome phenotype due to maternal uniparental disomy 15 with mosaic trisomy 15
Phenotypic recognition of maternal (mosaic) and paternal (segmental) isodisomy for chromosome 14 without a Robertsonian translocation
DOUBLE TRISOMY IN SPONTANEOUS ABORTIONS—AN 11 YEAR REVIEW
Can pericentric inversion and C-heterochromatin cause interchromosomal effect leading to an increased aneuploidy in sperm nuclei?
FISH delineation of multiple chromosome abnormalities in a mentally retarded patient with severe chronic disabilities
Smith-Magenis Syndrome Diagnosed at Birth
Double and triple trisomy in spontaneous abortions: an older maternal age and earlier gestational age than seen in single trisomies
Further clinical & cytogenetic delineation in Ip36 deletion syndrome
Partial Monosomy 12p and Ring Chromosome 12 Mosaicism in a Male with Developmental Delay and Mild Dysmorphism
The genetic counselor as a resource for families with a medical indication for cord blood banking
An assessment of genetic knowledge and utilization among mental health care providers and consumers
MCHB SPRANS Projects: Collaborative efforts to guide primary care providers to access and use medical genetic information electronically
Ethical Issues Encountered in Establishment of the Texas Birth Defect Research Center (TBDRC)
Visual presentations at 1998 ACMG meeting suggests insensitivity of geneticists to a common genetic disorder
Attitudies reguarding genetic counseling issues of the pakistani population at Maimonides Medical Center in Brooklyn
Patient perspectives on the process of informed consent for DNA testing
Genetic counseling for mitochondrial disorders
Constructing rapport in televideo genetic counseling
Practical use of Three-Dimensional Imaging in Genetic Counseling: Patient Perception of Usefulness as a counseling tool
Prenatal diagnosis of fragile X syndrome: identification of a male fetus mosaic for a premutation on chorionic villus sampling - management and follow-up
Interactive web-based genetic screening questionnaires in a primary care and obstetrics practice: A pilot study
Preconceived Ideas about Second Trimester Screening: a Guide for Counseling
Low maternal serum estriol as a marker for steroid sulfatase deficiency
X-Linked Corneal Dermoids Maps to Xq24-Xter
Primary Ciliary Dyskinesia: A search for the responsible genes through linkage and candidate gene approaches
The frequency and clinical significance of the S1235R mutation in the Cystic Fibrosis Transmembrane Conductance Regulator gene: results from a collaborative study
A novel nonsense mutation of the GTP cyclohydrolase I gene in a family with doparesponsive dystonia
The locus for autosomal dominant renal Fanconi Syndrome maps to the long arm of chromosome 15
Genetic testing for Niemann-Pick Type C disease
Is an elevated maternal serum uE3 MoM value in the second trimester associated with a poor pregnancy outcome?
Prenatal diagnosis of otocephaly
The extreme Spectrum of Pallister-Hall syndrome
Cystic lymphangioma of the axilla and chest wall
Recurrence of triploidy in a woman with low level 45,X mosaicism
Characteristics of fetuses with polyhydramnios and abnormal chromosome study
Do X- or Y-chromosome bearing spermatozoa competes with older eggs in human?
session listings
board and committee meetings
Genetic mechanisms of cancer development
Nramp proteins, susceptibility to infection and divalent cations transport
Genetics and hearing loss
The emerging and empowering roles of online mutual help groups in patient care
CF population screening 2000: The end is near
Gene Therapy and Futhure Directions in the Treatment of Cystic Fibrosis
Precision and uncertainty in mitochondrial genetics: clinical approach and a parent's perspective
Human telomere probes in clinical cytogenetics: probe characteristics, methods, applications and limitations
Abnormal brain structure and function in adult males with isolated clefts of the lip and/or palate
I don't want to hear you, but I'd like to see you
Optimal cancer risk assessment program professional roles; analysis of 30 American centers
Riboflavin responsive ethylmalonic encephalopathy in two Korean sibs
Oculoauriculofrontonasal syndrome in a nine-month-old male
A BRCA1 mutation carrier with three breast primaries and childhood ionizing radiation treatments: A possible gene/environment interaction
Analysis of 70 adult patients referred for genetics evaluation
Circadian rhythm abnormalities of melatonin and haploinsufficiency of COPS3 in Srnith-Magenis Syndrome
Craniosynostosis, ectopia lentis and congenital heart defect: further delineation of an autosomal dominant syndrome with reduced penetrance
Spondyloepimetaphyseal dysplasia with multiple joint dislocations
A family with multiple chromosome anomalies
Terminal deletion of 11q in two brothers: Clinical, cytogenetic, molecular genetic and counseling issues
Proximal 6q deletion phenotype: findings in de novo interstitial deletion 6g14.1g15
The state of public health genetics in Rhode Island
BRCA buccal immunoassay predicts BRCA1 and BRCA2 mutations
Sonogram findings with Brachmann-de Lange syndrome
Outcomes of a prenatal cytogenetic screening program in an urban state university medical center
ACMG board of directors
Orphan Genetic Disease – making testing a reality
The genetics of resistance and susceptibility to HIV-1 infection
Skeletal abnormalities/short bones
Potential for clinical misdiagnosis of combined methylmalonic aciduria/homocysteinemia (MMA/HCYS) due to absence of acute metabolic derangement
The clinical expression of Gaucher disease correlates with genotype: Data from 570 patients
Aortic root dilatation complicates Ehlers-Danlos syndrome
Neonatal cholestasis: A new presentation of X-linked adrenoleukodystrophy
Unilateral congenital lymphedema with intestinal lymphangiectasia, elevated liver transaminases, and hypopigmentation
Rubinstein-Taybi syndrome with hepatoblastoma
Testing for the Jewish BRCA founder mutations in archived tissue Jewish
Syndrome of ocular, skeletal & abdominal abnormalities
Utility of both bone marrow and renal transplants in the management of individuals with Schimke immunooseous dysplasia
Osteopetrosis, progressive sensorineural deafness, glaucoma, alopecia and cardiomyopathy in a 13 year old female: New syndrome or mild variant of YunisVaron syndrome?
The molecular pathogenesis of Schmid metaphyseal chondrodysplasia
Sudden death in Proteus syndrome
Clinical manifestations of NF1 in African-Americans and Caucasians
Chlari I malformation associated with a P250R mutationv of FGFR3
Molecular and cytogenetic analysis of Y;1 familial translocation
Chromosomal nosology in referred populations
The public's quest for genetic information: The role of a telephone helpline
Williams Syndrome: on the genetic basis of human cognition
Association of G-protein β3 subunit C825T variant (Gβ3S) and heart valve abnormalities in obese patients treated with fenfuramine-phentermine
CFTR Mutations in Chilean patients with cystic fibrosis
THE CLINICAL SIGNIFICANCE OF ISOLATED FETAL ECHOGENIC BOWEL
Long-term follow-up of amniocentesis
The relationship between the Glu298Asp and intron 4 polymorphisms of endothelial nitric oxide synthase in an Hispanic population with preeclampsia
Author Index for contributed papers
CONFERENCE/MEETING ROOMS
Meetings Calender
Mitochondrial Diseases in Men and Mice
march of dimes/colonel harland sanders award
45 CFR 46: Federal Regulations and Institutional Review Boards
Genetics and Informed Consent: Process and Content at the Millennium
Beyond the Human Genome Project: Genetics and Ethics, Human Nature and Society
The Consumer's View of Research
Mitochondrial Deafness
Prenatal Dysmorphology – Additional Laboratory Evaluations
Comparing satisfaction with clinical genetic services to other health services using a standardized survey, the CSQ-8©
Sensitivity of multiple color spectral karyotyping assessed by small constitutional translocations
Lack of a cardiac bulge in human growth disorganized embryos: evidence for cardiac malformation leading to pregnancy failure
Impracticality and inefficiency of first trimester population screening for birth defects
2-Methylbutyryl-CoA dehydrogenase (2-MBCDase) deficiency: a new inborn error of L-isoleucine metabolism
The detection of carbohydrate deficient glycoprotein syndrome by capillary electrophoresis
Correction of the Cystinotic Phenotype in Cultured Cells by an Aminoglycoside
Say-Meyer syndrome: A new case with magnetic resonance imaging of the brain, cardiac abnormality and X-linked dominant inheritance pattern
A new caw of hepatic glycogen snthase deficiency. biochemical findings and comparison with reported cases
Autosomal recessive syndrome due to amazing consanguinity
AREDYLD syndrome with focal segmental glomerulosclerosis
Genetic study of two Tunisian Ehlers Danlos syndrome type VI
Incidence and severity of pain in Stickler and Ehlers-Danlos syndromes
A comparison of the Berlin and Gent Nosologies in the diagnosis of Marfan syndrome: the NIH experience
Early clinical features of Angelman Syndrome in infants with chromosomal deletion of 15g11-q13
A Case of 46,XX African American male
Electronic karyotype transmission
Chromosome analysis of spermatozoa extracted from testes of men with non-obstructive azoospermia
Zeroing-in-on breakpoint susceptibility regions on chromosomes in breast carcinoma
Interphase Spectral FISH: Tailoring a diagnostic and minimal residual disease (MRD) assay for oncology
Tandem duplication of bands g13.13g13.33 resulting in partial trisomy of long arm of chromosome 19
Maternal age specific chromosomal abnormalities at amniocentesis
Severe growth retardation and limb anomalies in a boy with 47,XY,+r(7) and maternal uniparental disomy for chromosome 7
Tetraploidy in Prenatal Diagnosis: “culture artifact” or clinical diagnosis?
Factors affecting the incidence of Down syndrome live births in Illinois from 1989 to 1997
BRCA genetic testing: where are physicians in the decision process?
Prenatal genetic counseling by telemedicme: a feasibility study
Sequence characterization of the –THAIallele of athalassemia and rapid detection using a single-tube multiplex-PCR assay
Variant chromosome 1 reveals centromeric DNA sequences within the lqh region
Genome-wide linkage study for ossification of the posterior longitudinal ligament of the spine reveals a major susceptibility locus on chromosome 21q
A PATTERN ANALYSIS FOR ULTRASOUND ANOMALIES IN FETUSES WITH NORMAL KARYOTYPE
Antenatal diagnosis of mixoploidy: a case report
Prominent amnion-chorion separation between 13 and 15 weeks gestation is associated with increased risk for fetal chromosome abnormalities
Fetal methotrexate syndrome
Lessons from genetic discrimination
College women's awareness and consumption of folic acid for the prevention of neural tube defects
Patients' fear of genetic discrimination by health insurers: the impact of legal protections
Fragile X full mutations are more similar in siblings than in unrelated patients: further evidence for a familial factor in CGG repeat dynamics
Some major milestones and future directions
Forward Motion: Strategic Plan
A comparison of the Berlin and Ghent nosologies and the influence of dural ectasia in the diagnosis of Marfan syndrome
Clinical heterogeneity in autosomal dominant optic atrophy in two 3q28-qter linked central Illinois families
Acute intermittent porphyria: novel missense mutations in the human hydroxymethylbilane synthase gene
Genetics Resources on the Web (GROW)
To the Editor
To the Editor
College News: American College of Medical Genetics
Meetings Calendar
A familial risk profile for osteoporosis
The gap between practice and genetics education of health professionals: HuGEM survey results
Clinical and molecular studies in a unique family with autosomal dominant limb-girdle muscular dystrophy and Paget disease of bone
Gender verification of female athletes
Tandem mass spectrometry in newborn screening
Meetings Calendar
Contribution of different HFE genotypes to iron overload disease: a pooled analysis
In Response
ACMG recommendations for standards for interpretation of sequence variations
The interactivity between the CFTR gene and cystic fibrosis would be limited to the initial phase of the disease
Statement of the American College of Medical Genetics on Universal Newborn Hearing Screening
Erratum
Meetings Calendar
Clinical validation of genetic tests
Heterotaxy: Associated conditions and hospital-based prevalence in newborns
To the Editor
Mammography behavior after receiving a negative BRCA1 mutation test result in the Ashkenazim: A Community-based study Ashkenazim
Molecular diagnosis and inborn errors of metabolism: A practitioner’s view
College News: American College of Medical Genetics
From the Editor’s Desk
Characteristics of two cases with dup(15) (q 11.2-q 12): one of maternal and one of paternal origin
Challenges in communicating genetics: A public health approach
In memoriam
Positions Available
Congenital disorders of glycosylation: Have you encountered them?
Informed consent for medical photographs
Molecular determination of X inactivation pattern correlates with phenotype in women with a structurally abnormal X chromosome
Positions Available
Family history-taking in community family practice: Implications for genetic screening
Some ethical implications of The Human Genome Project
In Response
Meetings Calendar
Heterogeneity of late-infantile neuronal ceroid lipofuscinosis
The GM2 gangliosidoses datanases: Allelic variation at the HEXA, HEXB, and GMWA gene loci
Disorders of fatty acid transport and mitochondrial oxidation: Challenges and dilemmas of metabolic evaluation
Technical and clinical assessment of fluorescence in situ hybridization: An ACMG/ASHG position statement. I. Technical considerations: Test and Technology Transfer Committee
Guidelines for expert witness testimony for the specialty of medical genetics: Social, Ethical and Legal Issues Committee, American College of Medical Genetics
Author Index
Outcomes analysis of verbal dyspraxia in classic galactosemia
On the use of population-based registries in the clinical validation of genetic tests for disease susceptibility
To the Editor
Genetics testing for colon cancer: Joint statement of the American College of Medical Genetics and American Society of Human Genetics: Joint Test and Technology Transfer Committee Working Group
Positions Available
Dural ectasia in the Marfan syndrome: MR and CT findings and criteria
Biochemical genetics: From mechanisms to diagnosis and management of genetic disease
To the Editor