Infant / Childhood Diseases Flashcards
(87 cards)
1
Q
Most common congenital malformation in the US
25.7 per 10,000 births
A
CLUBFOOT. Internal rotation at ankle.
2
Q
What are the four most common congenital malformations in the US?
A
- Clubfoot w/o CNS anomalies
- Patent Ductus Arteriosus (PDA)
- Ventricular Septal Defect (VSD)
- Cleft Lip w/ or w/o cleft palate
3
Q
What are the four most common congenital malformations in the US?
A
- Clubfoot w/o CNS anomalies
- Patent Ductus Arteriosus (PDA)
- Ventricular Septal Defect (VSD)
- Cleft Lip w/ or w/o cleft palate
4
Q
Neonate (newborn)
A
0-28 days (4wks)
5
Q
Normal birth term
A
38-42 wks
6
Q
Pre-term birth (prematurity)
A
less than 37 wks
Second most common cause of neonatal mortality
12% of infants born prematurely in US
7
Q
Post-term birth
A
longer than 42 wks
8
Q
Infant
A
28days - 1yr (less than 1yr old)
9
Q
Child
A
1yr - 17yrs
10
Q
Risk factors for pre-mature birth
A
PPROM: preterm premature rupture of membranes
Smoking, previous preterm delivery, vaginal bleeding
Intrauterine infection, abnormal uterus, cervix, placenta
Multiple gestations
11
Q
Risk factors for pre-mature birth
A
PPROM: preterm premature rupture of membranes
Smoking, previous preterm delivery, vaginal bleeding
Intrauterine infection, abnormal uterus, cervix, placenta
Multiple gestations
12
Q
Complications of pre-mature birth
A
Hyaline membrane disease Necrotizing enterocolitis Sepsis Intraventricular hemorrhage Developmental delay
13
Q
Sx of prematuirty
A
Small infant (
14
Q
Fetal Growth Restriction
A
Fetal wt below 10th percentile for gestation age as determined thru ultrasound
15
Q
Fetal Growth Restriction Complications
A
Perinatal asphyxia, meconium aspiration, hypoglycemia, polycythemia, brain dysfunctions, hearing and visual impairment, learning disability.
16
Q
Fetal Growth Restriction Complications
A
Perinatal asphyxia, meconium aspiration, hypoglycemia, polycythemia, brain dysfunctions, hearing and visual impairment, learning disability.
17
Q
Predisposing factors for birth injury
A
LGA, small pelvis, precipitous delivery, abnormal presentation, forceps
18
Q
Caput cuccedaneum
A
Birth Injury: edema of presenting part of scalp. Significant only if there is an underlying skull fracture
19
Q
Cephalhematoma
A
Birth Injury: hemorrhage under periosteum, sometimes underlined by skull fracture
20
Q
Subgaleal hemorrhage
A
Birth Injury: hemorrhage that extends over entire scalp
21
Q
Causes of intracranial Hemorrhage
in newborns - d/t birth injury
A
Hypoxia/ischemia, variation in blood pressure, pressure on head during labor, common in small premature infants
22
Q
Subarachnoid hemorrhage (in newborns - d/t birth injury)
A
Most common, related to forceps or precipate expulsion, presents w/ apnea, seizures, may lead to hydrocephaly
23
Q
Subdural hemorrhage (in newborns - d/t birth injury)
A
presents w/ seizures and enlarging head
24
Q
Germinal Matrix Hemorrhage
in newborns - d/t birth injury
A
Occurs in premature infants. Can be fatal
25
Ascending (Transcervical) Infections - Perinatal Infections
Bacterial invasion thru cervix, following/triggering premature rupture of membranes (PROM). May cause inflammation of placental and extraplacental membranes (chorioamnionitis)
And inflammation of the umbilical cord (funisitis). May result in neonatal sepsis/pneumonia/meningitis
26
Ascending (Transcervical) Infections - Perinatal Infections
Bacterial invasion thru cervix, following/triggering premature rupture of membranes (PROM). May cause inflammation of placental and extraplacental membranes (chorioamnionitis)
And inflammation of the umbilical cord (funisitis). May result in neonatal sepsis/pneumonia/meningitis
27
Neonatal Sepsis
Early vs Late Onset
Most common cause of early onset?
Invasice bacterial infection in first week of life (early onset) vs next 3mo (late onset). More common in premature newborns, PROM 12-24hrs b4 birth, maternal bleeing/infection. Group B streptococcus (GBS). Complications: pneumonia and meningitis.
28
Ascending (Transcervical) Infections - Perinatal Infections
Bacterial invasion thru cervix, following/triggering premature rupture of membranes (PROM). May cause inflammation of placental and extraplacental membranes (chorioamnionitis). And inflammation of the umbilical cord (funisitis). May result in neonatal sepsis, pneumonia, meningitis.
E-coli, GBS, Herpes Simplex II
29
Neonatal Sepsis
Early vs Late Onset
Most common cause of early onset?
Invasice bacterial infection in first week of life (early onset) vs next 3mo (late onset). More common in premature newborns, PROM 12-24hrs b4 birth, maternal bleeing/infection. Group B streptococcus (GBS). Complications: pneumonia and meningitis.
30
Risk Factors for Early-onset Neonatal Spesis
```
Previous infant w/ GBS
GBS bacteriuria during pregnancy
Delivery b4 37wks
Ruptured membranes >18hrs
Intrapartum temp > 38
```
31
Risk Factors for Early-onset Neonatal Spesis
```
Previous infant w/ GBS
GBS bacteriuria during pregnancy
Delivery b4 37wks
Ruptured membranes >18hrs
Intrapartum temp > 38
```
32
What are the most common transplacental infections?
TORCHeS
| Toxoplasmosis, Others (listeriosis, HIV, HBV, Parovirus B19) Rubella, Cytomegalovirus, Herpesvirus, Syphilis
33
Manifestations of transplacental infections
Pneumonitis, chorioretinitis, myocarditis, encephalitis, hepatosplenomeagaly, anemia, thrombocytopenia
All can be caused by the TORCHeS infections
34
Parovirus B19
Part of the TORCHeS (kind of, its under 'O' for 'other')
Causesabortion, stillbirth, nonimmune hydrops fetalis, anemia. Erythroid precursors in infant bone marrow and spleen develop typical inclusions.
35
Parovirus B19
Part of the TORCHeS (kind of, its under 'O' for 'other')
Causesabortion, stillbirth, nonimmune hydrops fetalis, anemia. Erythroid precursors in infant bone marrow and spleen develop typical inclusions.
36
What are some causes of neonatal Respiratory Distress Syndrome (RDS)?
HMD is most common cause. Others: aortic stenosis, coarctation of aorta, pneumonia, pneumothorax, hypoglycemia, hypo/hyperthermia, drugs, excessive maternal sedation, fetal head injury, aspiration of blood/amniotic fluid, umbilical cord coiling, lack of surfactant.
37
RDS risk factors
Prematurity, diabetic moms, C-section, male infants
38
Pathology of Neonatal RDS / HMS
lack of surfactant results in atelectasis, hypoxemia, hypercarbia: these trigger pulm artery vasocx and increased R2L shunting leading2 acidosis, pulm vasocx and hypoperfusion. Ischemia damages alveolar/endothelial cells, plasma proteins (fibrin) leak into alveolar spaces. Proteins/cellular debris in alveolar ducts form hyaline membranes. Hyaline Membrane Disease.
39
RDS Sx
rapid grunting respirations, respiratory distress, cyanosis
40
RDS Dx
Amniotic fluid phospholipids: at maturity the amniotic fluid lectin/sphingomyelin ration is >2 and phosphatidylglycerol is present. Arterial blood gas show hypoxemia and hypercarbia. CXR shows atelectasis (ground-glass appearance)
41
RDS Tx
antenatal administration of steroids, surfactant replacement therapy, O2
42
RDS acute and long term complications
Acute: infection, air leaks, cerebral hemorrhage, necrotiing enterocolitis
Chronic: O2 toxicity causes bronchopulmonary dysplasia and retrolental fibroplasia (retinopathy of prematurity)
43
RDS acute and long term complications
Acute: infection, air leaks, cerebral hemorrhage, necrotiing enterocolitis
Chronic: O2 toxicity causes bronchopulmonary dysplasia and retrolental fibroplasia (retinopathy of prematurity)
44
Necrotizing Enterocolitis
Complication of prematurity and low birth wt
| Multifactorial etiology: ischemia causes bowel necrosis in terminal ileum. Gas in bowel wall, perforation/strictures
45
Bronchopulmonary Dysplasia
Complication of preterm neonate tx w/ longerm O2 therapy and pos pressure ventilation. Sponge-like lung radiology, interstitial fibrosis: thick septa, epithelial hyperplasia, squamous hyperplasia, decreased alveoli, predisposition to resp. infection. Cobblestone exterior surface of lung d/t hyperinflation and collapse.
46
Necrotizing Enterocolitis
Complication of prematurity and low birth wt
Multifactorial etiology: ischemia, infection, or oral feeding (intraluminal substrate) causes bowel necrosis in terminal ileum. Gas in bowel wall, perforation/strictures
47
Bronchopulmonary Dysplasia
Premature newborns
48
Bronchopulmonary Dysplasia
Premature newborns
49
Fetal Hydrops
| hydrops fetalis) vs (cystic hygroma
Accumulation of fluid (edema) in thefetus, generalized edema of the fetus is called hydrops fetalis. Localized edema in soft tissues of neck is called cystic hygroma.
50
Fetal Hydrops
| hydrops fetalis) vs (cystic hygroma
Accumulation of fluid (edema) in thefetus, generalized edema of the fetus is called hydrops fetalis. Localized edema in soft tissues of neck is called cystic hygroma.
51
Anasarca
Generalized Edema
52
Kernicterus
Hyperbilirubinemia, deposition in brain parenchyma when bilirubin >20mg/dL. Brain damage results in bilirubin encephalopathy.
53
Immune vs non-immune hydrops
Inflammation of the butt-hole
54
Immune vs non-immune hydrops
Inflammation of the butt-hole
55
Parovirus B19 Infection
tropic for erythroid progenitor cells. Viremia, rash, 33% o fetal transmission. Fetal infection may cause abortion during 1st trimester, in 2nd/3rd trimesters B19 accounts for
56
SIDS
Most common cause of mortality b/t 4wks and 1yr. Assoc w/ parental, infant, environmental risk factors: maternal smoking, drugs, young mom, freq. childbirths, prone sleeping on soft surface, hyperthermia, preterm, male, low birth wt, prior sibling, recent resp tract infection. Multifactorial condition of unknown cause. Brain stem abnormality in arousal and cardioresp control in arcuate nucleus.
57
SIDS
Most common cause of mortality b/t 4wks and 1yr. Assoc w/ parental, infant, environmental risk factors: maternal smoking, drugs, young mom, freq. childbirths, prone sleeping on soft surface, hyperthermia, preterm, male, low birth wt, prior sibling, recent resp tract infection. Multifactorial condition of unknown cause. Brain stem abnormality in arousal and cardioresp control in arcuate nucleus.
58
Hemangioma
Most common (vascular) tumor of infancy. Include capillary hemangioma (strawberry type) and Cavernous Hemangioma
59
Capillary Hemangioma
Strawberry type. On skin, spontaneously regress, leaves scarring and hemosiderin pigment, thin walled capillaries w/ scant stroma. Birthmarks w/ only cosmetic importance. Rapid growth during 1st yr, slowing in next 5yrs, regressing by age 10-15. Prognosis better than cavernous hemangiomas.
60
Cavernous Hemangiomas
Component of vonHippel-Lindau disease (cerebellum, retina, cysts, renal cell carcinoma, pheochromocytoma) or independent. Prognosis not as good as capillary hemangioma. Large cavernous blood-filled vascular spaces separated by CT stroma. "port-wine stains" that don't regress. In trigeminal nerve area as part of Sturge-Weber syndrome assoc w/ tumors in leptomeninges, hemiplegia, MR
61
Hemangiomas
Most common (vascular) tumor of infancy. Include capillary hemangioma (strawberry type) and Cavernous Hemangioma
62
Capillary Hemangiomass
Strawberry type. On skin, spontaneously regress, leaves scarring and hemosiderin pigment, thin walled capillaries w/ scant stroma. Birthmarks w/ only cosmetic importance. Rapid growth during 1st yr, slowing in next 5yrs, regressing by age 10-15. Prognosis better than cavernous hemangiomas.
63
Cavernous Hemangiomas
Component of vonHippel-Lindau disease (cerebellum, retina, cysts, renal cell carcinoma, pheochromocytoma) or independent. Prognosis not as good as capillary hemangioma. Large cavernous blood-filled vascular spaces separated by CT stroma. "port-wine stains" that don't regress. In trigeminal nerve area as part of Sturge-Weber syndrome assoc w/ tumors in leptomeninges, hemiplegia, MR
64
Lymphangiomas
Occur in neck, axilla, mediastinum, retroperitoneal tissues. Most common is cystic hygroma: a cavernous lymphangioma of the neck or the axilla. These lesions are progressive, don't regress, should be resected.
65
Lymphangiectasis
Dilated lymph channels presenting as a diffuse non-progressive swelling of an extremity w/ cosmetic consequences.
66
Infantile Myofibromatosis
Most prevalent fibrous tumor of infancy. Multiple subcutaneous myofibromas (benign fibrous tumors). Cells express muscle-specific actin, spindle shape, mitotic figures. Solitary form and multicentric form w/o visceral involvement spontaneously regress. Multicentric form w/ visceral involvement = varied sx & death w/in first 4mo of life.
67
Infantile Myofibromatosis
Most prevalent fibrous tumor of infancy. Multiple subcutaneous myofibromas (benign fibrous tumors). Cells express muscle-specific actin, spindle shape, mitotic figures. Solitary form and multicentric form w/o visceral involvement spontaneously regress. Multicentric form w/ visceral involvement = varied sx & death w/in first 4mo of life.
68
Aggressive Infantile Myofibromatosis
Myofibroblast cells infiltrate skeletal muscle, does not metastasize
69
(Congenital-infantile) Fibrosarcoma
Malignant. High grade atypia and high mitotic index. Very dark/dense spots - "ugly cells"
Densely cellular tumor w/ good prognosis
70
Fibromatosis
A condition characterized by the occurrence of multiple fibromas, subcutaneous nodules that sometimes may grow rapidly.
71
Fibromatosis
A condition characterized by the occurrence of multiple fibromas, subcutaneous nodules that sometimes may grow rapidly.
72
Teratomas
Germ cell neoplasm. Benign. Most in sacrococcygeal region. Most frequently recognized neoplasm of fetuses. More common in girls. Most common solid tumor of newborn (benign). A significant number w/ this develop non-immune hydrops in utero and die.
73
Teratomas
Germ cell neoplasm. Benign. Most in sacrococcygeal region. Most frequently recognized neoplasm of fetuses. More common in girls. Most common solid tumor of newborn (benign). A significant number w/ this develop non-immune hydrops in utero and die.
74
Three cateogries of teratomas
Cure rate
Complications
1. benign, well-differentiated aldult tissue
2. immature, embryonic tissue, not malignant
3. malignant
All types have malignant potential: 10%males, 7%females
95% cure rate of benign teratoma
Polyhydraminos, PROM, cardiac failure, coagulopathy
75
Three cateogries of teratomas
Cure rate
Complications
1. benign, well-differentiated aldult tissue
2. immature, embryonic tissue, not malignant
3. malignant
All types have malignant potential: 10%males, 7%females
95% cure rate of benign teratoma
Polyhydraminos, PROM, cardiac failure, coagulopathy
76
Most common malignancies in children under 4yo
Leukemia, brain tumors, neuroblastoma, wilms tumor, hepatoblastoma, retinoblastoma, rhabdomyosarcoma
Tend to regress, differentiate and respond to tx better
77
Neuroblastoma
Malignant, primitive sympathetic cells of adrenal medulla
sympathetic chain also affected. Most common extra cranial solid malignancy of childhood. Sx: abdominal mass & wt loss, blueberry muffin baby. Dx: catecholamines in blood, neuron specific enolase, VMA/HVA in urine. Morphology: small blue round cells = rosette structure. Dense core neurosecretory granules on EM.
78
Neuroblastoma
Malignant, primitive sympathetic cells of adrenal medulla
sympathetic chain also affected. Most common extra cranial solid malignancy of childhood. Sx: abdominal mass & wt loss, blueberry muffin baby. Dx: catecholamines in blood, neuron specific enolase, VMA/HVA in urine. Morphology: small blue round cells = rosette structure. Dense core neurosecretory granules on EM. Oncogene: N-myc
79
Wilms Tumor
Most common primary malignancy of kidney in kids. Sx: abdominal mass, hematuria, fever, HTN. Dx: r/o neuroblastoma. Morphology: primitive tubules. Prognosis good w/ nephrectomy. DIffuse anaplasia in extrarenal mets = poorer prognosis. Associated w/ two syndromes/malformations: WAGR and Denys-Drash
80
Wilms Tumor (WT) Congenital Malformations
WAGR Syndrome: 33% risk for WT, aniridia, genital anomalies, MR, germline DELETION of 11p13 (WT1)
Denys-Drash: 90% risk for WT, nepropathy, gonadal dysgenesis, gonadoblastoma, WT1 MUTATION
81
Rhabdomyosarcoma
Most common sarcoma (soft tissue) of childhood. Malignency of skeletal muscle. Most near head/neck and around EYES.
Three types: Embryonal, Alveolar, Pleomorphic
82
Embryonal rhabdomyosarcoma
60% of all rhabdomyosarcomas. Best prognosis. Sarcoma botryoides: develops in walls of hollow mucosal lined structures: nasopharynx, common bile duct, bladder, vagina
83
Alveolar Rhabdomyosarcoma
20% of all rhabdomyosarcomas, tumor cells separated by fibrous septae: divide into clusters that look like alveoli
84
Pleomorphic Rhabdomyosarcoma
20% of all rhabdomyosarcomas, worst prognosis. Mostly in adults. Numerous, large, multinucleated, hyperchromatic, myogenin is a marker for rhabdomyocytes
85
Pleomorphic Rhabdomyosarcoma
20% of all rhabdomyosarcomas, worst prognosis. Mostly in adults. Numerous, large, multinucleated, hyperchromatic, myogenin is a marker for rhabdomyocytes
86
Fetal Alcohol Syndrome
Pattern of physical/mental defects d/t alcohol crossing placenta: growth retardation, microcephaly, short palpebral fissures, maxillary hypoplasia, atrial septal defect
87
Neural Tube Defects
Improper closing of spinal cord/brain in early development. Folic acid greatly reduces the incidence. To screen: test mom's senum AFP levels
