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MNI (bones and joints path) > L47- Pediatric Pathology III > Flashcards

L47- Pediatric Pathology III Flashcards

1
Q
  • (1) list types of neonatal jaundice

- (2) is the first physical exam sign used to determine jaundice in a newborn; once bilirubin levels reach (3)

A

1- physiological and pathological

2- blanching of skin with digital pressure
3- >5 mg/dl

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2
Q

describe process of physiological jaundice

A

(Note: cause is normal hemolytic activity)
-Defined as elevation of unconjugated bilirubin during 1st wk of life –> always starting from the 2nd day

Phase I: lasts 5 days (term infants) or 7 days (premature) – serum bilirubin ~12-15 mg/dl

Phase II: decline in bilirubin for 2 wks –> normal adult bilirubin values achieved

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3
Q

list the main features of pathological jaundice

A

-clinical jaundice appears w/in 24hrs of birth

Total Bilirubin: >15 mg/dl
Conjugated Bilirubin: >2 mg/dl

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4
Q

list the main examples for each type of pathological jaundice

A

Unconjugated:

  • fetomaternal blood group incompatibility // hemolytic disease of newborn
  • Crigler-Najjar syndrome Type I, II

Conjugates:

  • biliary atresia
  • neonatal hepatitis
  • Dubin-Johnson syndrome
  • Rotor syndrome
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5
Q

Erythroblastosis fetalis:

  • (1) is the most extreme form of disease
  • (2) is the classic form of disease in newborns
A
  • death in utero = hydrops fetalis

- Kernicterus (via hyperbilirubinemia)

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6
Q

Hemolytic disease of the newborn, aka (1)- (2) describe pathogenesis

A

1- Erythroblastosis Fetalis

2:

  • mother sensitization to fetal Ag via previous exposure (transfusion, previous birth)
  • Ig against fetal RBC Ag produced –> crosses into placenta
  • Ig attacks fetal RBCs in utero => hemolysis
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7
Q

describe the effects and results of in utero hemolysis (via erythroblastosis fetalis)

A

Anemia:

  • extramedullary hematopoeisis (hepatosplenomegaly)
  • cardiac decompensation (cardiac failure) –> hydrops (death)

Hb degradation –> inc bilirubin:

  • jaundice
  • kernicterus
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8
Q

discuss the important gross and microscopic findings seen on autopsy of Hydrops Fetalis (erythroblastosis fetalis)

A
  • hepatosplenomegaly
  • bile stained organs
  • erythroblastic hyperplasia of BM
  • extramedullary hematopoiesis in liver, spleen
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9
Q

Kernicterus is the result of high levels of (un-/conjugated) bilirubin [include bilibrubin values and solubility] crossing BBB because of (2) status of BBB. As a result, (3) change is noted in the brain, predominately in (4) area.

A

1- unconjugated bilirubin (lipid soluble – conjugated is water soluble) – at total bilirubin levels >20 mg/dl

2- BBB not as strong or resistant since its is still maturing
3- bile staining
4- basal ganglia, brainstem

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10
Q

describe phototherapy as a treatment for hyperbilirubinemia

A
  • for elevated unconjugated bilirubin levels
  • conjugates bilirubin from liposoluble form into water soluble form
  • bilirubin excretion via urine
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11
Q

Crigler-Najjar Syndrome:

  • (1) type inheritance pattern
  • (2) is affected enzyme with (3) types of disease
A

1- AR
2- UDP-glucuronyltransferase

3:

  • type I, complete absence of enzymatic activity
  • type II, partial decrease in enzymatic activity
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12
Q

Type I Crigler-Najjar Syndrome:

  • (complete/partial) loss of UDP-gluconyltransferase activity
  • (2) resulting symptoms
  • (3) Tx
A

1- complete loss

2- unconjugated hyperbilirubinemia –> bilirubin encephalopathy –> kernicterus –> most Pts die w/in 1yr

3- phototherapy

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13
Q

Type II Crigler-Najjar Syndrome:

  • (complete/partial) loss of UDP-gluconyltransferase activity
  • (2) resulting symptoms
  • (3) Tx
A

1- partial loss

2- less severe form, jaundice may or may not be evident

3- Phenobarbital induces enzyme activity and upregulation + phototherapy

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14
Q

Embryonic / Fetal type biliary atresia:

  • results from (1), associated with congenital (2)
  • (3) is evident at birth, followed by (4)- include timeframe
A

1- aberrant intrauterine development of extraheptic biliary tree in utero
2- anomalies: malrotation of abdominal viscera, congenital heart disease

3- early onset neonatal cholestasis
4- jaundice free period after physiological jaundice period – 1wk

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15
Q

Perinatal type biliary atresia:

  • (1) cause, w/o association with (2)
  • (3) is evident at birth, followed by (4)- include timeframe
A

1- normal extra-hepatic biliary tree development (in utero) –> destroyed following birth via viral infection (or teratogen) –> bile duct resorption
2- congenital anomalies

3- normal fetus, jaundice free interval
4- late onset neonatal cholestasis (4-8 wks)

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16
Q

describe the morphology of the entire biliary tree in biliary atresia

A

Extrahepatic:

  • inflammation and fibrosis of hepatic and common bile duct
  • bile ductular proliferation
  • portal tract edema, fibrosis
  • parenchymal cholestasis

Intrahepatic:

  • periductal inflammation
  • progressive destruction of intrahepatic biliary tree
17
Q

describe prognosis of biliary atresia

A
  • 75% survival, operated w/in 60 days
  • 20-30% survivial, operated w/in 90 days

-80% survival with transplantation

18
Q

Idiopathic neonatal hepatitis:

  • (1)% of neonatal hepatitis cases
  • important to rule out (2) factors (secondary causes)
  • affects (males/females) more
A

1- 10-15%

2- AAT-deficiency, extrahepatic biliary atresia, infectious agents

3- males, 2:1

19
Q

describe microscopic features of idiopathic neonatal hepatitis

A

-hepatocytes form multi-nucleated Giant cells, diffusely distributed and prominent

  • ballooning of cells
  • acidophilic / eosinophilic degeneration
  • cholestasis
20
Q

Rotor Syndrome = (1):

  • results from (2) defect
  • (3) clinical presentation
  • (4) microscopic presenatation
A

1- familial conjugated hyperbilirubinemia
2- defect of excretion of bilirubin into bile canaliculi from hepatocytes –> reabsorbed in blood

3: (mild symptomatic jaundice)
- jaundice
- intermittent attacks of epigastric discomfort
- occasional abdominal pain and fever

4:

  • low grade pigment deposition
  • dissociation of liver cells
  • occasional necrotic foci
  • fibrin precipitation
21
Q

Dubin-Johnson syndrome:

  • (1) type of inheritance pattern of (2) defect
  • (3) is another associated defect
A

1- AR
2- defective transport of conjugated bilirubin out of hepatocytes into canalicular lumen

3- hepatic excretion of coproporyphrins (=> black liver appearance)

22
Q

discuss the general characterization and diagnosis of Dubin-Johnson syndrome (include clinical features)

A

-chronic or intermittent jaundice with ‘black liver’ (coproporyphrin lysosomal accumulation)

  • most patients are asymptomatic beyond mild, intermittent jaundice
  • usually diagnosed in teenage or young adulthood age, few may be evident in neonates

Serum Bilirubin ~2-5 mg/dl

23
Q

Cystic Fibrosis:

  • (1) type of inheritance pattern for (2- most common) mutation of (3) gene on chromosome (4)
  • (5) describe normal function of (3) protein
A

1- AR
2- ΔF508 deletion (70%, 3 base pair deletion) or one of 800 other mutations

3- CF transmembrane conductance receptor (CFTR)
4- chr.7

5- CFTR phosphorylated by protein kinase A via cAMP –> Cl- APC transporter (influx and efflux) in apical membranes, eccrine glands

24
Q

CF:

  • (1) describe changes seen in sweat gland
  • (2) changes in bronchi / bronchioles
A

1:

  • Normal: Cl- influx from duct lumen via CFTR drives Na+ resorption
  • CF: inc [Cl-], [Na+] in sweat (less influx)

2:

  • Normal: Cl- efflux into respiratory lumen balances / limits Na+, H2O influx/resorption to maintain mucosal hydration
  • CF: no Cl- efflux –> massive Na+, H2O influx => mucosal dehydration
25
Q

CF in the lung:

  • (1) is the predominant consequence with associated (2) changes around it
  • (1) will result in (3) disease processes
  • (4) are the usual infections that can result
A

1- (mucosal dehydration) plugging of submucosal tracheobronchial mucous glands and ducts –> obstruction of bronchioles with mucus
2- hyperplasia, hypertrophy of mucus secreting cells

3- atelectasis (lobular lung collapse), emphysema (due to parenchymal destruction)

4- chronic bronchitis, bronchiectasis, lung abscess

26
Q

CF in the pancreas:

  • (1) is the predominant consequence
  • (2) are the secondary changes
  • (3) is the end result
A

1- mucous obstruction of pancreatic ducts

2:

  • dilatation and cystic changes in distal ducts
  • atrophy of secretory cells
  • fibrosis
  • parenchymal destruction

3- (85% Pts) chronic pancreatitis

27
Q

list the main organ systems affected by CF

A
  • Lungs
  • Pancreas
  • liver
  • reproductive tract (males)
  • GIT
28
Q

______ may be evident in some CF patients immediately at birth (or up to 24hrs)

A

(15% Pts)

Meconium Ileus: impaction of meconium in terminal ileum –> subseqent risk of perforation, peritonitis

29
Q

describe hepatic effects usually seen in CF

A

late focal biliary cirrhosis via obstruction and bile duct hyperplasia

30
Q

describe CF testing / diagnosis

A

Pilocarpine Sweat Test:

  • Normal sweat, Cl = 10 mEq/L
  • severe CF sweat, Cl = >60 mEq/L
  • mild CF sweat, Cl = 40-60 mEq/L

Genetic testing

31
Q

list the CF clinical features found in the 2-12 month old period + recurrent issues if survival past this age

A

Pancreatic Insufficiency:

  • foul-smelling steatorrhea
  • malnutrition –> edema, hypoalbuminemia
  • failure to thrive

If survived:

  • recurrent URIs and sinus infections
  • 80-90% deaths due to Pulmonary disease
32
Q

______ are the signs in children that should clue physician to check for CF (note- not Sxs)

A
  • Nasal Polyps (uncommon before 5 y/o)

- Rectal Prolapse

MNI (bones and joints path) (10 decks)

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