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Pathology > Diseases of Infancy and Childhood > Flashcards

Diseases of Infancy and Childhood Flashcards

1
Q

LBB born preterm at 27 weeks AOG via SVD, observed TACHYPNEIC a few minutes after birth w/ associated GRUNTING, ALAR FLARING and INTERCOSTAL RETRACTIONS.

CXR: GROUND GLASS appearance of lung field w/ air bronchograms

Patient eventually expired 2 days after despite appropriate treatment measures.

Autopsy: EOSINOPHILIC membranes lining the alveolar walls

A

Respiratory Distress Syndrome (Hyaline Membrane Disease)

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2
Q

LBB born preterm at 27 weeks AOG via SVD, observed TACHYPNEIC a few minutes after birth w/ associated GRUNTING, ALAR FLARING and INTERCOSTAL RETRACTIONS.
CXR: GROUND GLASS appearance of lung field w/ air bronchograms

Excessive oxygen administration
Fundoscopy showed - RETINAL NEOVASCULARIZATION

A

Retinopathy of Prematurity (ROP)/ Retrolental fibroplasia

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3
Q

CXR: GROUND GLASS appearance of lung field w/ air bronchograms

Admitted at the NICU for 7 weeks maintained on > 21% FI at discharge but expired several days after.

Autopsy: DECREASE in ALVEOLAR SEPTATION

A

Bronchopulmonary Dysplasia (Moderate)

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4
Q

LBG, born preterm at 29 weeks AOG via VSD, hematochezia and abdominal distention.
Babygram: PNEUMOPERITONEUM
Autopsy: TRANSMURAL COAGULATIVE NECROSIS, ULCERATION and SUBMUCOSAL GAS BUBBLES in the COLON

A

Necrotizing Enterocolitis (NEC)

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5
Q

7/F with salty sweat and hx of recurrent pulmonary infections presented w/ dyspnea and cyanosis and was intubated. ETA CS showed Pseudomonas aeruginosa. Patient eventually expired. Autopsy showed no diagnostic abnormality on the skin but lung shoe dilation of airways w/ intense acute and chronic inflammation and granulation tissue formation

Cause of death
Diagnosis for pulmonary findings

A

Cystic Fibrosis (Mucoviscidosis)
Bronchiectasis

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6
Q

6/M with an enlarging ABDOMINAL mass that CROSSES the midline and generalized DUSKY MACULES resembling the surface of BLUEBERRY MUFFIN.
Autopsy: MASS in the ADRENAL GLAND
sheets of small round blue cells with occasional formation of Homer-Wright Rosettes

A

Neuroblastoma

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7
Q

5/F w/ HEMATURIA and large ABDOMINAL mass mainly located on the RIGHT but appears to cross the midline.
Biopsy: MIXTURE of glandular structures, sheets of spindle cells and small round cells

A

Wilms tumor

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8
Q

Primary errors of morphogenesis

Intrinsically abnormal developmental process

A

MALFORMATION

anencephaly
congenital heart disease

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9
Q

Secondary destruction of a normally developed organ

A

DISRUPTIONS

amniotic bands compressing parts of fetus

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10
Q

Extrinsic disturbance of development

UTERINE CONSTRAINT - MCC

A

DEFORMATIONS

club feet

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11
Q

Cascade of anomalies triggered by one initiating aberration

A

SEQUENCE

Oligohydramnios (Potter Sequence)

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12
Q

Constellation of congenital anomalies that are pathologically related and cannot be explained by single, localized, initiating defect

A

MALFORMATION SYNDROME

Congenital Rubella Syndrome

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13
Q

Embryo most susceptible during early during embryonic period

A

1st 3 weeks post fertilization

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14
Q

EXTREMELY susceptible to teratogenesis

A

3rd - 9th week

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15
Q

PEAK SENSITIVITY (height organogenesis)

A

4th - 5th week

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16
Q

MCC of respiratory distress in newborns

A

Neonatal RDS (Hyaline Membrane Disease)

ASSOCIATIONS:
prematurity
male
maternal diabetes
CS delivery

17
Q

Hyperoxia causes vasoconstriction and hypoxia that increases VEGF –> neovascularization

A

Retinopathy of Prematurity (ROP)

18
Q

Impairment of lung development at saccular stage

striking decrease in alveolar septations

A

Bronchopulmonary Dysplasia

19
Q

Impairment of lung development at saccular stage

striking decrease in alveolar septations

A

Bronchopulmonary Dysplasia

20
Q

Hematochezia, abdominal distention, circulatory collapse

PNEUMATOSIS INTESTINALIS - gas within intestinal wall

PNEUMOPERITONEUM - in severe NEC

A

Necrotizing Enterocolitis (NEC)

ASSOCIATIONS:
prematurity
VLBW
aggressive enteral nutrition

21
Q

Mediator implicated in NEC

increases mucosal permeability

A

Platelet Activating Factor (PAF)

21
Q

Mediator implicated in NEC

increases mucosal permeability

A

Platelet Activating Factor (PAF)

22
Q

MCC of hemolytic disease of the newborn

A

ABO incompatibility

23
Q

MOST SEVERE cause of hemolytic disease of the newborn

A

Rh incompatibility

24
Q

MCC of non-immune hydrops

A

Homozygous a-thalassemia

25
Q

The most serious complication of fetal hydrops (CNS) damage

A

Kernicterus

26
Q

MC lethal genetic disease affecting Caucasian populations

salty sweat
respiratory and intestinal complications
pancreatic insufficiency
atrophy and fibrosis of exocrine pancreas
meconium ileus
bile plugs
steatosis

A

CYSTIC FIBROSIS (Mucoviscidosis)

CFTR gene in chromosome 7

27
Q

MOST serious manifestation of Cystic Fibrosis

A

LUNGS

mucus plugging

S. aureus
H. influenza
P. aeruginosa

28
Q

MC tumors of infancy

A

HEMANGIOMA

29
Q

MC teratomas of childhood

A

Sacrococcygeal teratomas

30
Q

MC neoplastic disease

A

Leukemia

31
Q

MC solid tumors

A

CNS (INFRAtentorial)

32
Q

MC extracranial solid tumor of childhood

Most frequently diagnosed
tumor of infancy

A

Neuroblastoma

abdominal mass CROSSES midline
blueberry muffin
elevated catecholamines
OPSOCLONUS-MYOCLONUS SYNDROME

33
Q

MC site of neuroblastoma

A

ADRENAL MEDULLA (40%)

34
Q

Morphology of Neuroblastoma

A

BLASTEMAL CELLS
HOMER WRIGHT PSEUDOROSETTES
SCHWANNIAN STROMA - favorable prognosis

35
Q

MC primary renal tumor of childhood

chromosome 11 (WT1, WT2)

A

Nephroblastoma (Wilms tumor)

ASSOCIATED WITH:
WAGR syndrome (WT-1 deletion)
Denys-Drash syndrome (WT-1 dysfunction)
Beckwith-Wiedemann syndrome (WT2 imprinting)

36
Q

Triad of Wilms Tumor

A

unilateral mass (does NOT cross the midline)
hematuria
HPN

37
Q

Morphology of Wilms Tumor

A

CLASSIC TRIPHASIC TUMOR

BES!

Blastema - small round blue cells
Epithelial - glandular, glomerular
Stromal - myxoid, fibroblasts

Pathology (24 decks)

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