Term
What are the 4 time spans of infancy and childhood?
What i |
|
Definition
Infancy (1st year of life)
Childhood
Juvenile
Adolescence |
|
|
Term
What is the most hazardous/critical period of life?
Why is that? |
|
Definition
The neonatal period
infant must make a transition from a dependent intrauterine existence to an independent postnatal life = requires adaptations of the respiratory and circulatory systems and the maintenance of homeostatic mechanisms |
|
|
Term
| Why is infant mortality declining in the U.S.? |
|
Definition
| include better prenatal care, fetal and infant monitoring, and the use of Cesarean section |
|
|
Term
| What is the most common cause of death in children under 1 year of age? |
|
Definition
| Major congenital anomalies (malformations, deformities, and chromosomal syndromes) |
|
|
Term
A premature baby is ____ weeks.
Survival in the neonatal period is directly correlated with ______. |
|
Definition
<37
birth weight (low birthweight=more likely to experience long-term disability or die) |
|
|
Term
| What are the 3 factors that result in undergrown infants? |
|
Definition
Fetal (results in symmetric/all organs growth restriction)
Placental (commonly in 3rd trimester)
Maternal (MOST COMMON-disproportionate growth restriction) |
|
|
Term
| What score is used to evaluate the physiologic condition and responsiveness of a baby at birth? |
|
Definition
| APGAR (Activity, Pulse, Grimace, Appearance, Respiration from 1 minute(asphyxia) to 5 minutes(survival); 10 being best condition) |
|
|
Term
What are large-for-gestational age infants at greater risk for?
Low birthweight babies have higher associated ____ because they have the greatest difficulty in adapting |
|
Definition
|
|
Term
| What is the most common important injury that is related to excessive molding of the head or sudden pressure-induced changes in head shape? |
|
Definition
|
|
Term
| What is the common cause of respiratory distress in premature infants? |
|
Definition
| idiopathic respiratory distress syndrome or hyaline membrane disease (chest x-ray reveals a uniform hazy "ground-glass" appearance) |
|
|
Term
| What is given to infants to treat Respiratory distress syndrome? |
|
Definition
| Surfactant (oxygen cannot reverse situation) |
|
|
Term
| What reduces surface tension within alveoli so that less pressure is required to hold alveoli open, and maintains alveolar expansion by varying surface tension with alveolar size |
|
Definition
|
|
Term
What are the major complications of respiratory distress syndrome?
What complication happen when giving too much oxygen?
Giving too much oxygen at too high a pressure? |
|
Definition
anoxia and acidosis
oxygen toxicity with fibroplasia of the eyes and/or lung injury (bronchopulmonary dysplasia)
Iatrogenic Pneumothorax (tear in pleura) |
|
|
Term
| What is it when an infant has feeding intolerance, abdominal distention, and bloody stools. The lesion primarily involves the ileum and colon and grossly looks like dead bowel with bubbles. |
|
Definition
| Neonatal necrotizing enterocolitis |
|
|
Term
| What is the most common lethal genetic disease on chromosome 7 in caucasian populations and is an autosomal recessive trait? |
|
Definition
|
|
Term
| What disease affects the exocrine glands, both mucous-secreting and sweat glands, throughout the body --> chronic lung disease, pancreatic insufficiency, malnutrition, hepatic fibrosis, and intestinal obstruction |
|
Definition
|
|
Term
| Cystic Fibrosis results in an abnormal _____ channel |
|
Definition
| chloride channel (doesn't allow chloride to cross the epithelial cells of exocrine glands) |
|
|
Term
| What test is used to test cystic fibrosis? |
|
Definition
| "Sweat test" (Iontophoresis) - measures Na and Cl in sweat, kid is very salty |
|
|
Term
In Cystic Fibrosis, what internal organ is most affected?
What are the common organisms that infect this organ? |
|
Definition
lungs
staph aureus and pseudomonas (green color) |
|
|
Term
| What is the sudden and unexpected death of a child under one year of age, with the onset of the fatal episode apparently occuring during sleep and with death remaining unexplained after a thorough investigation, including complete autopsy, and review of the circumstances of death and the clinical history |
|
Definition
| Sudden Infant Death Syndrome (SIDS) |
|
|
Term
| What is often seen on the thymus and on the lungs of SIDS babies? |
|
Definition
| Petechiae (little red areas) |
|
|
Term
| Some factors associated with Sudden Infant Death Syndrome |
|
Definition
| they are premature with a lower birthweight, more often male, from minority and/or low socioeconomic groups, belong to large families living in over-crowded homes, and have young unmarried mothers. The risk is increased if the mother smoked cigarettes or abused narcotics (particularly methadone and cocaine) during pregnancy, if either parent used marijuana, or if the infant sleeps in a prone or side position or on a soft surface. |
|
|
Term
| What are some things that are shown to reduce SIDS risk? |
|
Definition
| infants sleep on back, pacifier, avoid bed sharing between infants and parents, using a fan |
|
|
Term
| What disease is an *absence of ganglion cells in the bowel wall due to lack of migration of neural crest cells into the bowel during development causing constipation |
|
Definition
| Hirschsprung Disease (congenital aganglionic megacolon) |
|
|
Term
| What test is used to determine Hirschsprung Disease? |
|
Definition
| Barium enema (shows transition zone from normal bowel function to little or no bowel function) |
|
|
Term
| What is the most common benign tumor of infancy mostly located in the skin, on the face and scalp, and composed of dilated vascular channels filled with blood? |
|
Definition
|
|
Term
| What is the leading cause of death from disease in children 4-14 years in the U.S? |
|
Definition
|
|
Term
| Where are the usual origins of childhood cancers? |
|
Definition
| hematopoietic system, the central and peripheral nervous systems, soft tissues, bone, and kidney |
|
|
Term
| What is a tumor that has the potential to develop disorganized tissues from all three germ layers (may be cystic and solid, contained teeth, skin with hair and sebaceous glands, brain tissue, intestine, and eye tissue). They often arise in the testes or ovary but may occur anywhere in the midline from the buttock to the brain. |
|
Definition
| teratoma (Sacrococcygeal or oropharyngeal) |
|
|
Term
Benign teratomas occur more often in (males or females)?
Malignant teratomas are more common in (males or females)? |
|
Definition
| The benign occur more often in females, while the malignant teratomas are more common in males |
|
|
Term
| (Malignant or Benign) teratomas often contain immature brain tissue in the form of ependymal rosettas |
|
Definition
| Malignant teratomas often contain immature brain tissue in the form of ependymal rosettes. |
|
|
Term
| What is the most common malignancy in children and alone accounts for more deaths in children under 15 years of age than all the other tumors combined? |
|
Definition
|
|
Term
| In a malignant cell there will be a high ratio of what? |
|
Definition
| High nuclear to cytoplasmic ratio (more nucleus than cytoplasm) |
|
|
Term
| Where are 2 sites where leukemia may relapse commonly? |
|
Definition
| cerebrospinal fluid and testis |
|
|
Term
What is the most common malignant eye tumor in children?
What chromosome is the mutation in? |
|
Definition
Retinoblastoma
Chromosome 13 |
|
|
Term
| What is the second most common solid tumors of childhood |
|
Definition
| Brain tumors are the second most common solid tumors of childhood (after lymphomas) |
|
|
Term
| What is one of the less aggressive brain tumors that is found most often in the cerebellum, floor and walls of the third ventricle, optic nerves, and occasionally, the cerebral hemispheres. These are slow-growing tumors, and often there is long survival even with incomplete resection. The tumor is composed of neoplastic astrocytes, which display long hair-like cytoplasmic processes. |
|
Definition
|
|
Term
| What is a highly malignant invasive primitive neuroectodermal tumor that arises in the cerebellum. The prognosis is worse if the child is less than 3 years old, there is incomplete resection, or if there is CSF, spinal, supratentorial or systemic spread |
|
Definition
|
|
Term
| What tumor may be found anywhere along the vertebral column column since they arise from sympathetic nervous tissue. More than 90% of these tumors secrete catecholamines, principally norepinephrine. They form rosettas (a characteristic feature) |
|
Definition
|
|
Term
| What tumor arises from deletions of chromosome 1p, 3p, and 11q, and gains in 17q. |
|
Definition
|
|
Term
| What is it called when neuroblastic tumors undergo spontaneous or chemotherapy-induced maturation and differentiation (less lethal), with neuroblasts transforming into ganglion cells and the stroma maturing to nerve-like Schwannian tissue |
|
Definition
|
|
Term
| What are the 2 types of malignant skeletal muscle tumor in childhood? |
|
Definition
| alveolar and embryonal rhabdomyosarcoma |
|
|
Term
| What is the most common malignant renal (kidney) tumor of childhood? They are bulky and gelatinous. Microscopically they often display primitive tubules, undifferentiated blastema and fibromyxoid stroma |
|
Definition
| Wilms tumor (nephroblastoma) |
|
|
Term
|
Definition
|
|
Term
| What gene deletion may result in Wilms tumor associated with aniridia, genitourinary abnormalities, and mental retardation? |
|
Definition
|
|
Term
| What syndrome is children present with overgrowth of body parts and/or organs, omphalocele, mental retardation, and a propensity to develop a variety of childhood tumors, including Wilms tumor |
|
Definition
| Beckwith-Wiedemann syndrome |
|
|
Term
| What gene deletion is associated with Beckwith-Wiedemann syndrome? |
|
Definition
| deletions at WT2 on chromosome 11 |
|
|
Term
| The cancer survival rate is (increasing or decreasing) in (some or all) cancers? |
|
Definition
| The cancer survival rate is decreasing in ALL cancers |
|
|
Term
Which of the following is NOT a major cause of death in children under the age of one?
-Malignant neoplasms
-congenital anomalies
-short gestation
-SIDS. |
|
Definition
Malignant neoplasms
The others are top 3 |
|
|
Term
| What is the complete absence of an organ? |
|
Definition
|
|
Term
| What is the absence of an opening? |
|
Definition
|
|
Term
| What is an incomplete development or a decrease in size |
|
Definition
|
|
Term
| What is an increase in size? |
|
Definition
|
|
Term
| What is an abnormal organization of cells? |
|
Definition
| dysplasia (may become malignant in the future) |
|
|
Term
| What is the most common teratogen leading to growth retardation, microcephaly, atrial septal defect, short palpebral fissures, and maxillary hypoplasia? |
|
Definition
| Alcohol (fetal alcohol syndrome) |
|
|
Term
Which of the following is NOT associated with TORCH group of infections and fetal growth restriction (FGR)?
-other, syphilis and parvo 319
-Rubella
-Haemophilus influenza
-Herpes simplex virus
-Toxoplasmosis
-Rubella
-Cytomegalovirus |
|
Definition
Haemophilus influenze
(T-toxoplasmosis, O-other, syphilis and parvo319 are in this category, R-rubella, C-cytomegalovirus, & H-herpes simplex virus) |
|
|
Term
While the pathogenesis of NEC is likely multifactorial, which of the following is associated with NEC?
-smoking
-enteral feeding
-maternal alcohol |
|
Definition
|
|
Term
Phenylketonuria (PKU) is an autosomal recessive condition due to the inability to convert phenylalanine to tyrosine
True or False? |
|
Definition
| True (Cystic fibrosis is also autosomal recessive.) |
|
|
Term
Galactosemia is an autosomal recessive disorder of galactose metabolism.
True or False? |
|
Definition
|
|
Term
Which of the following is a risk factor for Sudden Infant Death Syndrome (SIDS)?
-Hypothermia
-Maternal smoking during pregnancy
-Amniocentesis
-Caucasian ethnicity |
|
Definition
Maternal smoking during pregnancy
Hypothermia is not. Actually hyperthermia, when you bundle up the kids and you put all these blankets around them and they get too hot they lose that ability to regulate. |
|
|
Term
| Leukemia, Neuroblastoma, Wilms tumor, Hepatoblastoma, and Teratomas are most common in which age group? |
|
Definition
| 0 to 4 years (The ones that have “blasts”, leukemia is actually a blast, they are most common in the 0-4 age range.) |
|
|
Term
| The primary defect in cystic fibrosis results from abnormal function of an epithelial chloride channel protein encoded by what gene? |
|
Definition
| Cystic fibrosis transmembrane conductance regulator (CFTR) |
|
|
Term
Nephrogenic rests are precursor lesions of Wilms tumors.
True or False? |
|
Definition
True
nephrogenic rests are right next to the Wilms tumor and we have to look for these because if we find them that mean that the kid may get Wilms on the other side. |
|
|
