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51 Cards in this Set
- Front
- Back
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When is an APGAR score taken?
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At one and five minutes
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What factors are measured for the APGAR score?
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heart rate
respiratory effort muscle tone response to catheter in nostril skin color |
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What is a good APGAR score?
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9-10 is good
2-3 is bad |
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What factors can cause intrauterine growth retardation?
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Maternal causes most common: hypertension, toxemia, drugs, smoking, alcohol, malnutrition
Fetal development: chromosome disorders (Trisomy 13, 18, 21) congenital malformations congenital infections Placental defects: insufficiency due to diminished maternal blood flow. |
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How does Listeria monocytogenes access the baby?
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Transplacentally
most parasitic, viral and some bacteria use this route |
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What are the TORCH infections?
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Toxoplasma
Other bacterial and viral agents Rubella CMV Herpesvirus |
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What clinical features are caused by TORCH infections?
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Fever, encephalitis, chorioretinitis, HSM, pneumonitis, myocarditis, hemolytic anemia, vesicular/hemorrhagic skin lesions
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What is the most common cause of death in neonates?
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30% of neonatal deaths are caused by respiratory distress syndrome, commonly caused by hyaline membrane disease.
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What maternal conditions increase the risk of respiratory distress syndrome?
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diabetes and cesarean before the onset of labor
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What cells produce surfactant?
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Type II pneumocytes
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What is the effect of corticosteroids on surfactant production? What is the effect of thyroxine? What is the effect of insulin?
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Corticosteroids and thyroxine increase production of surfactant
Hyperinsulinism inhibits production |
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What L:S ratio is protective against respiratory distress syndrome?
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2:1 ratio of lecithin:sphingomyelin
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The administration of high levels of oxygen to treat respiratory distress can cause ____________.
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retrolental fibroplasia of the eyes
bronchopulmonary dysplasia Because oxygen is toxic and causes an increase in reactive oxygen species that cause tissue damage. |
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What causes intraventricular cerebral hemorrhage in neonates?
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anoxia and asphyxia
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What is the primary cause of necrotizing enterocolitis in neonates? What bacterial infection can be found superimposed with this condition?
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Ischemia is the primary cause
associated with C. diff infections. |
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What are the infant risk factors associated with SIDS?
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prematurity
low birth wt male not firstborn, prior SIDS sibling infant botulism (maybe 5% could be caused by C. botulinum toxin) |
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What is erythroblastosis fetalis?
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an antibody induced hemolytic disease caused by ABO/Rh incompatibility between mother and child.
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How much Rh+ fetal blood is necessary to sensitize an Rh- mother? Describe the early and late response.
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more than 1mL
Initial response is IgM, later IgG. The IgG becomes problematic for later gestation. |
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What is more common ABO or Rh HDN? Why?
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ABO HDN because Rh is successfully treated.
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Why can ABO HDN effect the firstborn?
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Does not require sensitization
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What autosomal recessive gene causes cystic fibrosis?
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Chromosome 7 (7q31-32)
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What is the primary defect in cystic fibrosis?
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A primary defect in a chloride ion channel
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How are sodium levels effected by cystic fibrosis?
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Sodium reabsorption is increased, leading to viscous intraluminal secretion in ducts
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Why do patients with cystic fibrosis have malabsorption problems?
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pancreatic abnormalities are present in 85% of pts. When the ducts get plugged it can lead to atrophy of exocrine pancreas called fibrocystic disease of the pancreas. This causes malabsorption of fats and fat-soluble vitamins.
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What rare bacterial lung infection is common among CF pts?
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pseudomonas aeruginosa
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What is meconium ileus and what pts present with this?
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25% of newborns with CF develop a small bowl obstruction with inspissated mucus.
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What is the most common benign tumor of infancy? Where is it found?
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Hemangiomas found on the face and scalp
"port wine stains" Can be a facet of von Hippel-Lindau disease |
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What tissues are most commonly involved in malignant tumors in infancy and childhood?
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hematopoietic
neural soft tissue |
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Why are malignant tumors of infancy and childhood sometimes refer to as small round blue cell tumors?
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They tend to be primitive embryonal cells which exhibit features of organogenesis from the site of origin.
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Where do neuroblastomas occur?
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All in sympathetic nervous system.
Half in adrenals Half in abdominal paravertebral autonomic ganglia |
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What is produced by neuroblastomas?
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90% produce catecholamines
VMA and HVA |
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How does a neuroblastoma present?
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fever, wt loss, and large abdominal mass
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What is the first clue that a child might have a retinoblastoma?
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photography will fail to produce a red-eye effect
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What is the common age group effected with retinioblastomas? How are they treated?
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Most common will effect children under 2
Treatment: enucleation of eye, chemo, radiation. |
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What is Wilms' Tumor?
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nephroblastoma
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What three malformations are characteristic for Wilms' tumor?
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WAGR syndrome
Denys-Drash syndrome Beckwith-Wiedeman syndrome |
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What is WAGR?
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Wilms
Aniridia Genital malformation Retardation |
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What is Denys-Drash? What is Beckwith-Wiedeman?
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DD: male pseudo-hermaphoditism and renal abnormality
BW: enlargement of organs, hemihypertrophy, renal cysts |
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What age group will Wilms' tumor be found in? What is the 2 year survival rate?
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Children 2-5
90% survival rate, which implies a cure |
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What is the abnormality in PKU? How is it inherited?
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Autosomal Recessive
inability to convert phenylalanine to tyrosine due to a lack of homozygous loss of phenylalanine hydroxylase. |
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What is the damage of accumulating phenylalanine?
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by 6 months of age child will develop severe retardation
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How is PKU treated in the child? In a heterozygous mother?
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For the child: special diet to avoid phenylalanine
Heterozygous or homozygous mother: Resume special diet to avoid phenylalanine prior to conception because phenylalanine can cross the placenta and accumulate in baby. |
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Why is it important to diagnose PKU variants?
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Because they cannot be treated with diet
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What is the sequelae of partial lack of phenylalanine hydroxylase?
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child will develop benign hyperphenylalaninemia, but level are not elevated enough to induce neurologic effects.
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What organ systems are at most risk in galactosemia?
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Liver: Cirrhotic
Eyes: Cataracts Brain: Gliosis and edema, loss of neurons |
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What is galactomsemia? How is it inherited?
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Autosomal recessive
Accumulation of galactose-1-phosphate causing tissue damage |
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What is the IQ impact of congenital hypothyroidism?
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10 IQ points lost if TSH is abnormal at week 2
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What is MCADD? What is the clinical presentation? What percent suffer brain damage?
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Medium Chain Acyl CoA DeHase Deficiency
Present with hypoketotic hypoglycemia Autosomal recessive 50% suffer permanent brain damage |
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What is the treatment for MCADD?
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IV glucose for N/V with no fasting periods
High carbo drinks, walnut oil for FA, and carnitine supplementation |
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What is the typical presentation of a neonate with metabolic defects?
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1. born okay--well for up to 72 hrs
2. Food refusal 3. Excessive sleepiness, coma, death 4. Lab: hyperammonemia and acidosis |
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What is the hallmark of Type 1 Glutaric Acidemia?
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Severe life-long dystonia
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