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childhood disease II Flashcards

1
Q

describe Ascending (transcervical) infections

A
  • bacterial invasion through the cervix, following or triggering premature rupture of the membranes (PROM)
  • causes INFLAMMATION of the placental and extraplacental membranes (chorioamnionitis) and the umbilical cord (funisitis) and VILLITIS (lymphocytic infiltrate of chronionic villi)
  • may result in PRETERM BIRTH neonatal sepsis, pneuomonia and meningitis
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2
Q

Describe Neonatal sepsis

A
  • invasive bacterial infection occurring in the first week of life (early onset) or during the next 3 months (late onset)
  • More common in PREMATURE NEWBORNS 12-24 h before birth, maternal bleeding or infection
  • common cause for early-onset sepsis = Group B streptococcus (GBS)
  • complications = pneumonia and meningitis
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3
Q

describe Transplacental infections

A
  • TORCH (Toxoplasma, Other infectious agents, Rubella, Cytomegaloirus, and Herpesvirus) cause villitis and fetal infection
  • manifestations = pneumonitis, chorioretinitis, myocarditis, encephalitis, hepatosplenomegaly, anema, and thrombocytopenia
  • parvovirus B19 infection causes abortion. stillbirth, nonimmune hydrops fetalis, and anemia in the newborn.
  • -> erythroid precursors in the infant bone marrow and spleen develop typical inclusions
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4
Q

Neonatal resiratory distress syndrome causes

A
  • prematurity (60% of infants born less than 28weks)
  • lack of surfactant
  • fetal head injury
  • sedation
  • aorta anomalies
  • umbilical cord coiling
  • amniotic fluid aspiration
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5
Q

describe the pathogenesis of hyaline membrane disease (type of RDS)**

A

1) prematurity –> reduced sufactant synthesis, storage and release
2) decreased alveolar surfactant –> increased alveolar surface tension –> Atelectasis
3) atelectasis leads to uneven perfusion and hypoventilation –> hypoxemia and CO2 retention
4) leads to Acidosis –> pulmonary vasoconstriction –> pulmonary hypoperfusion
5) pulmonary hypoperfusion causes endothelial damage and epithelial damage
6) eventually leads to fibrin + necrotic cells (hyaline membrane)

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6
Q

describe bronchopulmonary dysplasia (chronic lung disease)

A
  • occurs in preterm neonates treated oxygen therapy >4wks and positive pressure ventilation
  • Sponge-like lung radiology*
  • interstitial fibrosis*
  • epithelial hyperplasia, squamous metaplasia (causes COBBLESTONE EXTERIOR SURFACE)
  • reduced total numbers of alveoli
  • predisposition to respiratory infection
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7
Q

describe necrotizing enterocolitis*

A
  • complication of prematurity and low birth weight
  • pathogenesis = ischemia results in focal to confluent areas of bowel necrosis, most often in the terminal ileum
  • abdominal distension, ileum and blood stools
  • abdominal radiographs = gas in the bowel wall*
  • increased chance perforation
  • strictures in intestines
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8
Q

define fetal hydrops

A
  • edema in fetus
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9
Q

hydrops fetalis

A
  • generalized edema
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10
Q

describe the development of immune hydrops fetalis **

A

1) Mother Rd D-; Father Rh D+
2) maternal immunization to Rh D antigen
3) transplacental passage of maternal anti-D IgG antibodies
4) binding anti-D IgG to fetal Rh+ RBC
5) destruction of anti-D IgG-RBC complex
- -> causes miscarriages etc
- -> first baby causes memory B cells
- -> second baby leads to immune response targeting second baby

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11
Q

describe Sudden Infant death syndrome (SIDS)

A
  • unexplained death under 1 year of age

- 90% of cases infant is less than 6 months

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12
Q

what are the paternal risk factors of SIDS**

A
  • young maternal age
  • maternal SMOKING during pregnancy
  • drug abuse
  • late or no prenatal care
  • short intergestational intervals
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13
Q

What are the infant risk factors of SIDS**

A
  • brain stem abnormal
  • prematurity/SGA
  • MALE (also Hyaline disease risk factor)
  • antecedent respiratory infections
  • multiple birth pregnancy
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14
Q

what are the environmental risk factors of SIDS**

A
  • PRONE sleep position
  • sleeping on SOFT surfaces
  • hyperthermia (too hot)
  • postnatal passive smoking
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15
Q

cystic hygroma

A

localized edema

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16
Q

what causes immune hydrops

A
  • blood group incompatibility
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17
Q

what causes nonimmune hydrops

A
  • infections
  • chromosomal anomalies
  • twin pregnancy
  • cardiovascular defects
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18
Q

describe the development of immune hydrops fetalis

A

1) Mother Rd D-; Father Rh D+
2) maternal immunization to Rh D antigen
3) transplacental passage of maternal anti-D IgG antibodies
4) binding anti-D IgG to fetal Rh+ RBC
5) destruction of anti-D IgG-RBC complex

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19
Q

what is the pathology of SIDS

A
  • multipe petechiae (80% of cases)
  • lungs congestion - vascular engorgement
  • Hypoplasia of arcuate nucleus and decreased brain stem neuronal populations
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20
Q

Capillary hemangiomas

A
  • “birthmarks”
  • have only cosmetic importance, and the juvenile hemangiomas that show rapid growth during the 1st year of life, slowing in the next 5 years and disappearing by age 10-15
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21
Q

cavernous hemangiomas

A
  • “port-wine stains”
  • do not regress
  • Port-wine stains in the TRIGEMINAL NERVE area as part of Sturge-weber syndrome is associated with hemangiomas of the leptomeninges, hemiplegia and mental retardation
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22
Q

Von Hippel-lindau disease

A
  • skin hemangiomas associated with hemangiomas in the cerebellum and retina, cysts renal cell carcinoma and pheochromocytoma
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23
Q

Lymphangiomas

A
  • occur in the neck, axilla, mediastinum and retroperitoneal tissue
  • most common is cystic hygroma = a cavernous lymphangioma of the neck or the axilla
  • lesions are progressive, do not regress and should be resected
24
Q

Lymphangiectasis

A
  • dilated lymph channels presenting as a diffuse non-progressive swelling of an extremity with cosmetic consequences
25
Q

Infantile myofibromatosis

A
  • most prevalent fibrous tumor of infancy
  • fibrous tumor in which the cells express muscle-specific actin
  • myofibromas are solitary or generalized soft tissue tumors
  • Solitary form and the multicentric form without visceral involvement undergo spontaneous regression
  • Multicentric form with visceral involvement produces varied symptoms and death within the first 4 months of life
26
Q

Fibromatosis

A
  • is a condition characterized by the occurence of multiple fibromas, subcutaneous nodules that sometimes may grow rapidly
27
Q

congenital-infantile fibrosarcoma

A
  • densely cellular tumor with good prognosis
28
Q

Sacrococcygeal teratomas (SCT)***

A
  • most frequently recognized neoplasm of fetuses
  • benign
  • malignancy occur in 10% of teratomas with immature tissue microscopy
  • may develop nonimmune hydrops in utero and die
  • Complications = polyhydramnios, premature rupture of membranes, cardiac failure, and coagulopathy
29
Q

histologic appearance of SCT**

A
  • most SCTs contain solid and cystic elements
  • Three main categories:
    1) benign teratomas containing well-differented, adult tissue
    2) immature teratomas containing embryonic tissue that is not frankly malignant
    3) malignant teratomas
30
Q

CHildhood malignancy

A
  • malignancy is the second leading cause of death from disease in age 5-14
  • origin from hematopoietic, nervous, renal, adrenal gland, soft tissues, bone
  • has relationship with devleopmental aberrations
  • tendency for spontaneous regression
  • may have favorable prognosis
31
Q

describe the composition of NB***

A
  • composed of “small, blue, round cells” (neuroblasts) forming rosettes
  • dense core neurosecretory granules (contain catecholamine)
  • Grossly = Blueberry muffin baby
32
Q

define Neuroblastoma (NB)

A
  • malignant tumor arising from primitive sympathetic cells in the adrenal medulla, the mediastinal and abdominal sympathetic chain, in the pelvis, neck or brain
33
Q

describe clinical manifestation of neuroblastoma

A
  • abdominal mass
  • fever
  • pain
  • weight loss
  • ascites
  • respiratory distress
  • gait disturbances
  • diarrhea
  • proptosis
  • periorbital ecchymosis
34
Q

describe Stage 1, 2A and 2B of the International neuroblastoma staging system (INSS)

A
  • Stage 1, 2A and 2B = tumor is localized and contralateral lymph nodes are not invaded
35
Q

describe stage 4A of the international neuroblastoma staging system (INSS)

A
  • Stage 4S defined as a small primary tumor in the abdomen or thoracic cavity, with metastasis in the liver or bone marrow and skin
  • patients are at low risk if there are no N-myc amplification or chromosome changes
36
Q

describe the microscopic appearance of wilms tumor

A
  • tightly packed blue cells consistent with blastemal component and interspersed primitive tubules, reprentin the epithelial component
37
Q

describe stages 3 and 4 of the international neuroblastoma staging system (INSS)

A
  • Stage 3 = unilateral tumor extending across midline or localized with invaded contralateral lymph nodes
  • Stage 4 = disseminated tumor
  • no N-myc amplification, low TRK-A expression
38
Q

what is the high-risk group of international neuroblastoma staging system

A
  • high-risk group includes children >1 year with S3 or S4 tumors and unfavorable histology, N-myc amplification, chromosome abnormalities, minimal TRK-A expression, near-diploid or tetraploid karyotype
39
Q

Pleomorphic rhabdomyosarcoma

A
  • elderly
  • located deep within muscle of extremities and trunk
  • very aggressie (WORST)
  • numerous large large, sometimes multinucleated, bizarre eosinophilic tumor cells
  • ugly looking (lots of eyes starring at you)
40
Q

alveolar rhabdomyosarcoma

A
  • adolescents
  • located in muscles of extremities form cords or alveoli infibrovascular stroma
  • very aggressive
  • crude resemblence of pulmonary alveoli
  • Tumor is travered by a network of fibrous septae that divide the cells into cluster or aggregates
  • cells in periphery adhere to septae; cell sin center of aggregates are discohesive
41
Q

embryonal rhabdomyosarcoma

A
  • infancy or childhood
  • -> most often located in head and neck tissue
  • -> less aggressive than other forms
  • sarcoma botryoides-embryonal rhabdomyosarcoma with grape-like, soft polypoid gross appearance
  • -> located in genitourinary, upper respiratory or biliary tract
  • -> BEST PROGNOSIS
42
Q

describe the appearance of Wilms tumor (WT) (nephroblastoma)

A
  • WTs are unicentric lesions, but many are multifocal or bilateral
  • pseudocapsule, areas of hemorrhage and necrosis
  • consists of epithelial, blastemal, and stromal elements
  • anaplasia correlates with chemotherapy resistance and poor prognosis
43
Q

neural tube defects (NTDs)

A
  • opening in the spinal cord or brain that occurs very early in human development
  • supplementing the maternal diet with FOLIC ACID prior to pregnancy can reduce the incidence of NTD
  • prenatal screening test for neural NTDs: maternal serum AFP**
44
Q

Describe the clinical appearance of Wilms tumor

A
  • abdominal mass
  • hematuria
  • fever
  • hypertension
45
Q

describe the cause of Wilms tumor

A
  • mutation of the WT-1 gene located on chromosome 11p13 are found in 15% of cases
  • associated with beta-catenin mutations
  • also associated with WT-2 gene on chromosome 11p15
46
Q

describe the types of rhabdomyosarcoma pathology

A
  • Embryonal rhabdomyosarcoma
  • alveolar rhabdomyosarcoma
  • pleomorphic rhabdomyosarcoma (prognosis is the worst)
47
Q

Fetal alcohol syndrome (FAS)

A
  • a pattern of physical and mental defects that can develop in a fetus in association with high levels of alcohol consumption during pregnancy
  • alcohol crosses the placental barrier
48
Q

alcohol causes

A
  • growth retardation
  • microcephaly
  • short palpebral fissures
  • maxillary hypoplasia
  • atrial septal defect
  • small head, epicanthal folds, flat midface, smooth philtrum, thin upper lip
49
Q

what is an indicator of fetal lung maturity

A
  • sulfactant is an indicator
  • surfactant is a mixture of lipids, proteins, and glycoproteins, lecithin and sphingomyelin being two of them
  • Lecithin-sphingomyelin ratio (L/S ratio) is a test of fetal amniotic fluid to assess for fetal lung immaturity
50
Q

What are the risk factors for early-onset neonatal sepsis

A
  • previous infant with GBS disease*
  • GBS bacteriuria during pregnancy*
  • delivery before 37 wk gestation*
  • Ruptured membrans > 18h
  • intrapartum temperature > 38 C
51
Q

what are the risk factors of Hyaline membrane disease

A
  • mother has diabetes
  • C section
  • Male gender
  • preterm AGA
52
Q

what is the clinical presentation of hyaline membrane disease

A
  • respiratory distress
  • cyanosis**
  • hypoxemia
  • hypercarbia
  • metabolic acidosis
53
Q

Kernicterus

A
  • due to immune hydrops fetalis
  • -> hemolytic anemia
  • -> hyperbilirubinemia leads to jaundice and kernicterus
54
Q

Fibrosarcoma

A
  • Grade III fibrosarcoma has high grade atypia and high mitotic index
55
Q

describe WAGR syndrome

A
  • 33% risk to have Wilms tumor
  • aniridia (no iris)
  • genital anomalies
  • retardation (mental)
  • Germline Del 11p13 (WT1)
56
Q

describe Denys-Drash syndrome

A
  • 90% risk to have wilms tumor
  • nephropathy
  • gonadal dysgenesis
  • gonadoblastoma
  • WT1 MUTATION

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