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Biochem 18

front 1

A karyotype from peripheral blood shows a normal human diploid chromosome count. How many chromosomes are present?
A. 44
B. 45
C. 46
D. 48

back 1

C. 46

front 2

A euploid embryo inherits equal chromosome sets from each parent. Which distribution is correct?
A. 23 maternal, 23 paternal
B. 22 maternal, 24 paternal
C. 24 maternal, 22 paternal
D. 46 maternal, 0 paternal

back 2

A. 23 maternal, 23 paternal

front 3

In humans, the number of autosomal chromosome pairs is:
A. 21 pairs
B. 22 chromosomes
C. 23 pairs
D. 22 pairs

back 3

D. 22 pairs

front 4

A couple asks who determines genetic sex at conception. The father can transmit:
A. X only
B. X or Y
C. Y only
D. Neither X nor Y

back 4

B. X or Y

front 5

A variant disrupts a DNA segment that encodes a functional product. This segment is a:
A. Locus
B. Gene
C. Allele
D. Chromatid

back 5

B. Gene

front 6

A report states “mutation at 7q31.” This describes a chromosomal:
A. Allele
B. Gene
C. Chromosome
D. Locus

back 6

D. Locus

front 7

Two siblings share the same locus but differ in sequence at that site. Each version is an:
A. Allele
B. Centromere
C. Chromatid
D. Telomere

back 7

A. Allele

front 8

A diploid individual’s genotype at one locus includes:
A. One allele
B. Three alleles
C. Two alleles
D. No alleles

back 8

C. Two alleles

front 9

A pedigree shows many affected males with carrier females. Best explanation?
A. X inactivation in males
B. Mitochondrial inheritance pattern
C. Y-linked transmission
D. Single X in males

back 9

D. Single X in males

front 10

A disorder appears in both sexes each generation with no sex bias. This pattern best fits:
A. X-linked dominant
B. X-linked recessive
C. Autosomal recessive
D. Mitochondrial inheritance

back 10

C. Autosomal recessive

front 11

A meiotic error causes sister chromatids to fail separation. This is:
A. Inversion
B. Nondisjunction
C. Translocation
D. Deletion

back 11

B. Nondisjunction

front 12

A newborn has an abnormal chromosome number due to meiotic segregation failure. This is:
A. Polyploidy
B. Mosaicism
C. Aneuploidy
D. Triploidy

back 12

C. Aneuploidy

front 13

Prenatal testing reveals autosomal aneuploidy. The most common outcome is:
A. Spontaneous abortion or disease
B. No phenotype
C. Isolated anemia
D. Always viable, mild symptoms

back 13

A. Spontaneous abortion or disease

front 14

Which is NOT a chromosomal structural alteration?
A. Inversion
B. Translocation
C. Deletion
D. Nondisjunction

back 14

D. Nondisjunction

front 15

A disorder appears in both sexes each generation with no sex bias. This pattern best fits:
A. X-linked dominant
B. X-linked recessive
C. Autosomal dominant
D. Mitochondrial inheritance

back 15

C. Autosomal dominant

front 16

A drug increases transcription by loosening chromatin via histone tail changes. Which mechanism?
A. DNA excision repair
B. Cytosine deamination
C. Histone acetylation
D. Splice-site mutation

back 16

C. Histone acetylation

front 17

A tumor shows stable DNA sequence but reduced gene expression via promoter modification. Most consistent with:
A. Histone ubiquitination
B. Replication slippage
C. Base substitution
D. Cytosine methylation

back 17

D. Cytosine methylation

front 18

A syndrome shows parent-of-origin effects without nucleotide change. This is:
A. Imprinting
B. Duplication
C. Translocation
D. Isochromosome formation

back 18

A. Imprinting

front 19

A disorder differs depending on whether the allele came from mother or father. This reflects imprinting being:
A. Random
B. Tissue-specific only
C. Sex-specific
D. Always paternal

back 19

C. Sex-specific

front 20

In a somatic lineage, an imprint typically:
A. Disappears after mitosis
B. Persists in progeny cells
C. Changes each cell cycle
D. Requires DNA sequence change

back 20

B. Persists in progeny cells

front 21

During gametogenesis, imprint marks are:
A. Reset
B. Permanently fixed
C. Randomly deleted
D. Converted to mutations

back 21

A. Reset

front 22

A researcher uses a population model to infer allele frequencies from genotype frequencies. This is:
A. Founder effect
B. Linkage analysis
C. Genomic imprinting
D. Hardy–Weinberg equilibrium

back 22

D. Hardy–Weinberg equilibrium

front 23

Hardy–Weinberg methods are best suited to analyze:
A. Mitochondrial disorders only
B. Autosomal and XLR disorders
C. X-linked dominant only
D. Chromosomal trisomies only

back 23

B. Autosomal and XLR disorders

front 24

A patient’s condition reflects multiple genes plus environment. This is:
A. Imprinting disorder
B. Single-gene disorder
C. Multifactorial disease
D. Aneuploidy syndrome

back 24

C. Multifactorial disease

front 25

A pedigree shows progressive severity across generations due to repeat expansion. Inheritance is usually:
A. Autosomal dominant
B. Autosomal recessive
C. X-linked recessive
D. Mitochondrial inheritance

back 25

A. Autosomal dominant

front 26

Earlier onset and worsening symptoms in later generations most directly correlates with:
A. Loss of imprinting
B. Decreased penetrance
C. Gene deletion size
D. Increasing repeat number

back 26

D. Increasing repeat number

front 27

A mutation removes cell-cycle “brakes,” enabling uncontrolled proliferation. The gene class is:
A. Growth factor genes
B. Ion channel genes
C. Tumor suppressor genes
D. Spliceosome genes

back 27

C. Tumor suppressor genes

front 28

Which is an epigenetic modification mechanism?
A. Gene translocation
B. Histone phosphorylation
C. Chromosome inversion
D. Nondisjunction

back 28

B. Histone phosphorylation

front 29

A pedigree shows vertical transmission of retinoblastoma-like cancer predisposition. Which statement best fits tumor suppressor inheritance?
A. Autosomal recessive; dominant mechanism
B. X-linked dominant; recessive mechanism
C. Autosomal dominant; recessive mechanism
D. Mitochondrial; dominant mechanism

back 29

C. Autosomal dominant; recessive mechanism

front 30

In a familial cancer workup, a tumor shows deletion of the remaining wild-type allele at a locus. This is:
A. Loss of heterozygosity
B. Variable expressivity
C. Anticipation
D. Imprinting

back 30

A. Loss of heterozygosity

front 31

A prenatal karyotype shows 47 total chromosomes. This abnormality is best termed:
A. Polyploidy
B. Triploidy
C. Tetraploidy
D. Aneuploidy

back 31

D. Aneuploidy

front 32

A newborn has streak ovaries and webbed neck; karyotype: 45,X. Diagnosis?
A. Klinefelter syndrome
B. Turner syndrome
C. Edwards syndrome
D. Patau syndrome

back 32

B. Turner syndrome

front 33

A fetus has 47,XY,+21. The diagnosis is:
A. Turner syndrome
B. Triple X syndrome
C. Patau syndrome
D. Down syndrome

back 33

D. Down syndrome

front 34

A lab orders a test to visualize chromosomes and detect trisomies and translocations. Best test?
A. Karyotype
B. PCR genotyping
C. Southern blot
D. Sanger sequencing

back 34

A. Karyotype

front 35

For karyotyping, cells are arrested when chromosomes are maximally visible. This stage is:
A. Prophase I
B. Mitotic metaphase
C. Anaphase II
D. Interphase

back 35

B. Mitotic metaphase

front 36

A genetics student asks when crossing over occurs. Correct timing is:
A. After metaphase I
B. After metaphase II
C. After replication, before metaphase I
D. Before DNA replication

back 36

C. After replication, before metaphase I

front 37

A traits lecture defines heritability. Which description is correct?
A. Genetic and environmental components
B. Only environmental contribution
C. Only genetic contribution
D. Only mitochondrial contribution

back 37

A. Genetic and environmental components

front 38

A trait has “genetic component 100%.” This implies:
A. Environment fully determines phenotype
B. Genes and environment equally contribute
C. Epigenetics dominates inheritance
D. No environmental influence on phenotype

back 38

D. No environmental influence on phenotype

front 39

A trait has “genetic component 10%.” This implies phenotype is mainly:
A. Mendelian segregation
B. Environmental
C. Chromosomal dosage
D. Imprinted alleles

back 39

B. Environmental

front 40

In counseling, “penetrance” refers to:
A. Disease severity among affected
B. Allele frequency in population
C. Probability phenotype expressed
D. Chromosome separation failure

back 40

C. Probability phenotype expressed

front 41

A mutation has 100% penetrance. This means:
A. All carriers express disease
B. Half of carriers express disease
C. No carriers express disease
D. Severity varies widely

back 41

A. All carriers express disease

front 42

A family shows incomplete penetrance. Which factor can explain penetrance <100%?
A. Nondisjunction events only
B. Modifier genes or epigenetics
C. Tight junction disruption
D. Crossover frequency changes

back 42

B. Modifier genes or epigenetics

front 43

Two relatives carry the same mutant allele and both are affected, but one is mild and one is severe. This is:
A. Anticipation
B. Incomplete dominance
C. Loss of heterozygosity
D. Variable expressivity

back 43

D. Variable expressivity

front 44

A disorder shows 100% penetrance, yet affected relatives have different clinical pictures. This best reflects:
A. Variable expressivity
B. Germline mosaicism
C. Allelic heterogeneity
D. X-inactivation

back 44

A. Variable expressivity

front 45

A clinician asks why expressivity varies within families. Most consistent causes are:
A. Only nondisjunction rates
B. Only imprint resetting
C. Environment and modifier genes
D. Only crossover differences

back 45

C. Environment and modifier genes

front 46

Which disorder is a classic example of variable expressivity?
A. Achondroplasia
B. Marfan syndrome
C. Down syndrome
D. Turner syndrome

back 46

B. Marfan syndrome

front 47

A heterozygous affected parent has an autosomal dominant disorder. Risk each child is affected:
A. 25%
B. 10%
C. 75%
D. 50%

back 47

D. 50%

front 48

A patient has café-au-lait spots and neurofibromas (Neurofibromatosis type 1). Inheritance pattern is most classically:
A. Autosomal recessive
B. X-linked recessive
C. Autosomal dominant
D. Mitochondrial

back 48

C. Autosomal dominant

front 49

Short-limbed dwarfism with FGFR involvement suggests:
A. Achondroplasia
B. Osteogenesis imperfecta
C. Marfan syndrome
D. Neurofibromatosis type 1

back 49

A. Achondroplasia

front 50

An adult develops progressive neurodegeneration with a family history across generations; the listed autosomal dominant example is:
A. Cystic fibrosis
B. Huntington disease type 2
C. Tay-Sachs disease
D. Duchenne muscular dystrophy

back 50

B. Huntington disease type 2

front 51

Achondroplasia is caused by mutation in which gene class?
A. Tumor suppressor gene
B. Ion channel gene
C. FGF receptor gene
D. Collagen gene

back 51

C. FGF receptor gene

front 52

Why is mitotic metaphase optimal for karyotyping?
A. Homologs pair and synapse
B. Spindle absent, DNA relaxed
C. Chromatids segregate to poles
D. Chromosomes most condensed, easily seen

back 52

D. Chromosomes most condensed, easily seen

front 53

A patient develops progressive chorea and cognitive decline. Testing reveals a triplet-repeat expansion in which gene?
A. NF1
B. HTT
C. CFTR
D. FBN1

back 53

B. HTT

front 54

A tall patient with lens subluxation has a mutation in a fibrous protein. The gene product is:
A. Fibrillin
B. Dystrophin
C. Tyrosinase
D. Factor VIII

back 54

A. Fibrillin

front 55

A child with café-au-lait spots has a mutation in a GTPase-activating protein. The mutated gene is:
A. HTT
B. DMD
C. NF1
D. OTC

back 55

C. NF1

front 56

Two carrier parents have a child. In autosomal recessive inheritance, expected outcomes are:
A. 25% affected
B. 50% affected
C. 75% affected
D. 25% carriers

back 56

A. 25% affected

front 57

A drug increases transcription by loosening chromatin via histone tail changes. Which mechanism?
A. DNA excision repair
B. Cytosine deamination
C. Histone acetylation
D. Splice-site mutation

back 57

C. Histone acetylation

front 58

A child has generalized hypopigmentation due to loss of melanin synthesis. Defective enzyme:
A. Phenylalanine hydroxylase
B. Melanocyte tyrosinase
C. Ornithine transcarbamylase
D. Hexosaminidase A

back 58

B. Melanocyte tyrosinase

front 59

A newborn has recurrent lung infections and pancreatic insufficiency. The mutated protein is:
A. Dystrophin
B. CFTR
C. Fibrillin
D. Neurofibromin

back 59

B. CFTR

front 60

A screening test suggests PKU. The most common deficiency is:
A. Homogentisate oxidase
B. Phenylalanine hydroxylase
C. Branched-chain dehydrogenase
D. Porphobilinogen deaminase

back 60

B. Phenylalanine hydroxylase

front 61

A patient has sickle cell disease. The causative mutation is:
A. E6V in β-globin
B. E6K in α-globin
C. V6E in α-globin
D. Frameshift in γ-globin

back 61

A. E6V in β-globin

front 62

A pedigree shows affected males only, with no male-to-male transmission. This suggests:
A. Autosomal dominant
B. Autosomal recessive
C. X-linked recessive
D. X-linked dominant

back 62

C. X-linked recessive

front 63

In X-linked recessive disorders, females are typically:
A. Asymptomatic
B. Always affected
C. More affected than males
D. Embryonic lethal

back 63

A. Asymptomatic

front 64

A conceptus has monosomy of chromosome 7. The expected outcome is:
A. Viable, mild phenotype
B. Embryonic lethal
C. Always mosaic survival
D. Causes only anemia

back 64

B. Embryonic lethal

front 65

Trisomies most compatible with limited survival involve:
A. 1, 2, 3
B. 5, 7, 9
C. 12, 16, 20
D. 13, 18, 21

back 65

D. 13, 18, 21

front 66

To balance gene expression in females, one X chromosome undergoes:
A. Random deletion
B. Inactivation and condensation
C. Complete replication failure
D. Translocation to Y chromosome

back 66

B. Inactivation and condensation

front 67

The condensed inactive X chromosome in female somatic cells is called:
A. Nucleolus organizer
B. Barr body
C. Sex vesicle
D. Chromatid bridge

back 67

B. Barr body

front 68

Which is an X-linked recessive disorder?
A. Hemophilia A
B. Cystic fibrosis
C. Marfan syndrome
D. Huntington disease type 2

back 68

A. Hemophilia A

front 69

Hemophilia A results from mutation in:
A. Factor IX
B. Factor VIII
C. Factor V
D. Factor XI

back 69

B. Factor VIII

front 70

Duchenne muscular dystrophy is most often due to:
A. Point mutation in NF1
B. Large deletions in DMD
C. Trisomy of chromosome 21
D. Expansion in HTT

back 70

B. Large deletions in DMD

front 71

Red-green color blindness most directly reflects:
A. Rod degeneration
B. Cone dysfunction
C. Lens opacification
D. Optic nerve transection

back 71

B. Cone dysfunction

front 72

The most common inborn error of the urea cycle is:
A. CPS1 deficiency
B. OTC deficiency
C. ASS deficiency
D. ASL deficiency

back 72

B. OTC deficiency

front 73

A child has exercise intolerance and lactic acidosis. A pathogenic mtDNA mutation most directly impairs:
A. Glycolysis
B. Oxidative phosphorylation
C. DNA replication
D. Beta oxidation

back 73

B. Oxidative phosphorylation

front 74

A muscle biopsy shows mixed normal and mutant mtDNA within the same cell. This is:
A. Polyploidy
B. Homoplasmy
C. Imprinting
D. Heteroplasmy

back 74

D. Heteroplasmy

front 75

A cell’s mitochondria all carry the same mtDNA genome (normal or mutant). This is:
A. Homoplasmy
B. Heteroplasmy
C. Aneuploidy
D. Mosaicism

back 75

A. Homoplasmy

front 76

A pedigree shows only affected mothers transmit a disorder. Mitochondria are inherited from the:
A. Father
B. Both parents
C. Mother
D. Paternal grandfather

back 76

C. Mother

front 77

A woman with a mitochondrial disorder has children. Expected penetrance among her children is:
A. All children affected
B. Half children affected
C. Only sons affected
D. Only daughters affected

back 77

A. All children affected

front 78

A man with a mitochondrial disorder has children with an unaffected partner. His children are most likely:
A. All affected
B. Half affected
C. Variable by sex
D. Unaffected

back 78

D. Unaffected

front 79

Two siblings from the same affected mother have different severities. Best explanation:
A. Anticipation
B. Reduced penetrance
C. Variable mutant mitochondria load
D. Uniparental disomy

back 79

C. Variable mutant mitochondria load

front 80

A mitochondrial disorder most prominently affects tissues with:
A. Low ATP demand
B. High energy requirement
C. Slow cell turnover
D. High melanin content

back 80

B. High energy requirement

front 81

A young adult has painless central vision loss consistent with Leber’s hereditary optic neuropathy (LHON). The typical genetic lesion is:
A. Mitochondrial rRNA mutation
B. Nuclear tRNA mutation
C. mtDNA large deletion
D. Mitochondrial protein-gene mutation

back 81

D. Mitochondrial protein-gene mutation

front 82

A patient has MERRF myoclonic seizures and ragged-red fibers. The lesion most classically involves:
A. Mitochondrial tRNA gene mutation
B. Mitochondrial protein-gene mutation
C. Autosomal dominant repeat
D. X-linked recessive deletion

back 82

A. Mitochondrial tRNA gene mutation

front 83

A patient has stroke-like episodes and lactic acidosis (MELAS). The mutation most often affects:
A. mtDNA deletion
B. Nuclear transcription factor
C. Mitochondrial tRNA gene mutation
D. PHEX gene mutation

back 83

C. Mitochondrial tRNA gene mutation

front 84

A teen has myopathy, cerebellar findings, and cardiomyopathy (Kearns–Sayre). Most likely mechanism:
A. mt rRNA point mutation
B. mtDNA deletion
C. NF1 loss-of-function
D. CFTR channel mutation

back 84

B. mtDNA deletion

front 85

A child has hypophosphatemic rickets with an X-linked dominant pedigree. The implicated gene is:
A. PHEX
B. HTT
C. NF1
D. CFTR

back 85

A. PHEX

front 86

A girl has blistering rash evolving into hyperpigmented streaks; males in family die early. The causal gene is:
A. PHEX
B. DMD
C. NF1
D. IKBKG

back 86

D. IKBKG

front 87

Incontinentia pigmenti type 1 is often:
A. Benign in males
B. Fatal in males
C. Fatal in females
D. Limited to males

back 87

B. Fatal in males

front 88

The IKBKG gene product primarily regulates a family of:
A. Ion channels
B. Structural collagens
C. Transcription factors
D. Mitochondrial ribosomes

back 88

C. Transcription factors

front 89

A trisomy results from a meiotic error. The process is:
A. Nondisjunction
B. Duplication
C. Insertion
D. Inversion

back 89

A. Nondisjunction

front 90

Nondisjunction is best defined as:
A. Two breaks with reversal
B. Unequal sorting in meiosis I/II
C. Extra segment copied twice
D. Reciprocal chromosome exchange

back 90

B. Unequal sorting in meiosis I/II

front 91

Which is a chromosomal structural alteration?
A. Aneuploidy
B. Penetrance
C. Inversion
D. Heritability

back 91

C. Inversion

front 92

An inversion involves:
A. Two breaks; segment inverted
B. One break; segment lost
C. One break; segment copied
D. Two breaks; chromosomes swapped

back 92

A. Two breaks; segment inverted

front 93

A duplication involves:
A. Segment inverted between breaks
B. Segment deleted entirely
C. Reciprocal exchange between chromosomes
D. Duplicated segment inserted same chromosome

back 93

D. Duplicated segment inserted same chromosome

front 94

A disorder caused by loss of a small chromosomal segment is best termed:
A. Heteroplasmy
B. Aneuploidy
C. Microdeletion syndrome
D. Homoplasmy

back 94

C. Microdeletion syndrome

front 95

A prenatal report notes an “insertion” without net DNA loss. Which description best fits an insertion?
A. Segment flipped within chromosome
B. Whole arm duplicated, other lost
C. Segment inserted into another chromosome
D. Reciprocal exchange between chromosomes

back 95

C. Segment inserted into another chromosome

front 96

A cytogenetics lab suspects an isochromosome. Which structure is most characteristic?
A. Two identical p arms or q arms
B. Two chromosomes mutually exchange arms
C. Small deletion under 5 Mb
D. Segment inverted between two breaks

back 96

A. Two identical p arms or q arms

front 97

In an isochromosome, the two arms are:
A. Randomly different in sequence
B. One maternal, one paternal
C. Complementary but nonidentical
D. Genetically identical to each other

back 97

D. Genetically identical to each other

front 98

A syndrome is linked to a small chromosomal deletion with complex, consistent phenotype. “Microdeletion” typically means:
A. Deletion larger than 20 Mb
B. Deletion under 5 megabases
C. Entire chromosome arm missing
D. Whole chromosome duplicated

back 98

B. Deletion under 5 megabases

front 99

A child’s phenotype suggests a microdeletion syndrome; standard karyotype is normal. A test often needed to localize the deletion is:
A. FISH
B. Karyotype
C. Western blot
D. Gram stain

back 99

A. FISH

front 100

A clinician suspects an even smaller deletion that FISH can miss. Which method is more sensitive for microdeletions?
A. Light microscopy banding
B. Standard karyotype
C. Single-gene PCR
D. Array-based genomic hybridization

back 100

D. Array-based genomic hybridization

front 101

Two main categories of chromosomal translocations are:
A. Inversion and duplication
B. Robertsonian and reciprocal
C. Insertion and deletion
D. Isochromosome and microdeletion

back 101

B. Robertsonian and reciprocal

front 102

A “balanced reciprocal translocation” is best described as:
A. One arm duplicated, other lost
B. Segment inverted within one chromosome
C. Mutual exchange between two chromosomes
D. Small deletion with complex phenotype

back 102

C. Mutual exchange between two chromosomes

front 103

A balanced reciprocal translocation is “balanced” because:
A. Entire chromosome number is normal
B. Extra copy compensates deletion
C. It always causes trisomy 21
D. No net genetic material loss

back 103

D. No net genetic material loss

front 104

A tall man has infertility and small testes. Which karyotype is most likely?
A. 45,X
B. 47,XXY
C. 47,XYY
D. 47,XXX

back 104

B. 47,XXY

front 105

A patient has short stature and amenorrhea; karyotype shows monosomy X. Diagnosis?
A. Turner syndrome
B. Triple-X syndrome
C. Down syndrome
D. Edwards syndrome

back 105

A. Turner syndrome

front 106

A newborn has cleft lip/palate and polydactyly; karyotype is trisomy 13. Syndrome?
A. Down syndrome
B. Edwards syndrome
C. Patau syndrome
D. Turner syndrome

back 106

C. Patau syndrome

front 107

A neonate with low birth weight and a small, abnormally shaped head has trisomy 18. Syndrome?
A. Patau syndrome
B. Edwards syndrome
C. Down syndrome
D. Klinefelter syndrome

back 107

B. Edwards syndrome

front 108

A child has hypotonia, characteristic facies, and congenital heart disease; karyotype shows +21. Syndrome?
A. Patau syndrome
B. Edwards syndrome
C. Turner syndrome
D. Down syndrome

back 108

D. Down syndrome

front 109

A female has learning difficulties but no major physical anomalies; karyotype is 47,XXX. Diagnosis?
A. Klinefelter syndrome
B. Turner syndrome
C. Triple-X syndrome
D. Down syndrome

back 109

C. Triple-X syndrome

front 110

A boy has some learning/behavioral problems; karyotype is 47,XYY. Diagnosis?
A. XYY syndrome
B. Klinefelter syndrome
C. Patau syndrome
D. Turner syndrome

back 110

A. XYY syndrome

front 111

Which aneuploidy is associated with increased leukemia risk?
A. Turner syndrome
B. Down syndrome
C. Triple-X syndrome
D. XYY syndrome

back 111

B. Down syndrome

front 112

Severe CNS anomalies with polydactyly strongly suggests:
A. Edwards syndrome
B. Down syndrome
C. Turner syndrome
D. Patau syndrome

back 112

D. Patau syndrome

front 113

Low birth weight with heart defects and small malformed head most strongly suggests:
A. Down syndrome
B. Patau syndrome
C. Edwards syndrome
D. Klinefelter syndrome

back 113

C. Edwards syndrome

front 114

Turner syndrome is especially common among:
A. Miscarriages and stillbirths
B. Elderly fathers only
C. Premature male births
D. Maternal diabetes pregnancies

back 114

A. Miscarriages and stillbirths