GI DISORDERS Flashcards

1
Q

Indirect Hyperbilirubinemia
Increased Production includes

A
  • Sepsis (+dB)
  • DIC
  • Extravasation of blood: hematomas, pulmonary, cerebral or occult hemorrhage
  • Polycythemia
  • Macrosomic infants of DM mothers
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2
Q

Indirect Hyperbilirubinemia
Metabolic includes

A
  • Crigler-NAjjar syndrome (Type I AR & II AR/D)
  • Gilbert syndrome (AR/D)
  • Tyrosinemia, Hypermethioninemia (+dB)
  • Galactosemia (+dB)(vom, exc. wt. loss, HSM)
  • Hypothyroidism
  • Hypopituitarism (+dB)
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3
Q

Indirect Hyperbilirubinemia
Decreased Clearance includes

A
  • prematurity
  • G6PD Deficiency
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4
Q

Indirect Hyperbilirubinemia
Increased Enterohepatic Circulation includes

A
  • Breast milk (breast-feeding) Jaundice
  • Pyloric stenosis
  • Small or large bowel obstruction or ileus
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5
Q

Mixed types

A
  • Sepsis
  • Congenital Syphilis
  • TORCH
  • Coxsackie B Virus
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6
Q

Causes of Prolonged Indirect Hyperbilirubinemia

A
  • Breast milk Jaundice
  • Hemolytic disease
  • Hypothyroidism
  • Extravascular BLOOD
  • PYLORIC STENOSIS
  • Crigler-Najjar Syndrome
  • Gilbert syndrome gentype in breast fed infant
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7
Q
  • 60-80% idiopathic or biliary atresia
  • Clinically jaundiced beyond 3 weeks of life
  • pale stools
  • dark urine
A

Direct Hyperbilirubinemia
(Cholestatic Jaundice)

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8
Q

Direct Hyperbilirubinemia
Ductal disturbances in excretion

  • nonsyndromatic paucity of bile ducts
  • associated with lymphedema
A

Intrahepatic biliary atresia

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9
Q

Direct Hyperbilirubinemia
Ductal disturbances in excretion

  • isolated, trisomy 18, polysplenia-heterotaxia syndrome
A

Extrahepatic biliary atresia

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10
Q

Physiologic Jaundice

A
  • level of indirect bilirubin in umbilical cord serum is 1-3 mg/dL
    > rate of rise - <5 mg/dL in 24 hours
  • Term: visible on the 2° to 3 day of life and resolves
    spontaneously on the 5th and 7th days of life
  • Preterms: rise in serum bilirubin is the same or slower but longer duration;
    > Peak levels: 8-12mg/dL on the 4th-7th day, resolve arounf the 10th day
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11
Q

Pathologic Jaundice

  • Onset:
  • Rate of rise
  • Full term:
  • Pre-term
  • persists after
  • direct bilirubin fraction
A
  • Onset: before 24 to 36 hours after birth
  • Rate of rise > 5 mg/dL/24 hr
  • Full term: serum bilirubin is >12 mg/dL
  • Pre-term 10-14mg/dL
  • persists after 10-14 days after birth
  • direct bilirubin fraction is >2mg/dL at any time
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12
Q

Major Risk Factors for the Development of Severe
Hyperbilirubinemia in Infants > 35 weeks GA

A
  • Pre-discharge TSB in high risk zone
  • Jaundice in first 24 hours
  • Blood group incompatibility with (+) Coombs, other known hemolytic diseases
  • GA 35-36 weeks
  • Hx sibling received phototherapy
  • Cephalhematoma or significant bruising
  • Exclusive breasfeeding particularly if nursing is
    not going well and weight loss is excessive
  • East Asian race
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13
Q

Minor Risk Factors for the Development of Severe
Hyperbilirubinemia in Infants > 35 weeks GA

A
  • Pre-discharge TSB in high intermediate risk ZONE
  • GA 37-38 weeks
  • Jaundice observed before discharge
  • Previous sibling with jaundice
  • Macrosomic infant with diabetic mother
  • Maternal age >/= 25y.o
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14
Q

Decreased Risk Factors for the Development of Severe Hyperbilirubinemia in Infants > 35 weeks GA

A
  • TSB at low risk zone
  • GA >/= 41 wks
  • Exclusive bottlefeeding
  • Discharge from the hospital after 72 hrs
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15
Q
  • current mainstay of treatment
  • proved to be instrumental in containing the rate of rise of TB and lowering the TB
A

Phototherapy

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16
Q

Complications of Phototherapy

A
  • dark grayish-brown discoloration of the skin (bronze baby syndrome)
  • burns, retinal damage, thermoregulatory instability, loose stools, dehydration, skin rash, and tanning of the skin
17
Q

have a role in the mgt of hyperbilirubenemia

A

Phenobarbital therapy

18
Q
  • the most rapid method for lowering serum bilirubin concentrations
  • removes partially hemolyzed and antibody-coated
    erythrocytes and replaces them with uncoated
    donor red blood cells that lack the sensitizing antigen
  • double vol. exchange replaces 85% of the circulating RBC and decreases the bilirubin level to about half of the pre exchange value
A

Exchange transfusion

19
Q
  • adjunctive treatment for hyperbilirubinemia caused by isoimmune hemolytic disease
  • Its use is recommended when serum bilirubin is
    approaching exchange levels despite maximal
    interventions including phototherapy.
  • Dose 0.5-1.0 g/kg/dose; repeat in 12 hr
  • reduces the need for exchange transfusion in both ABO and Rh hemolytic disease, presumably by reducing hemolysis
A

Intravenous Immunoglobulin

20
Q
  • stimulate many hepatic membrane-bound enzyme
    systems and hepatic protein synthesis in general
  • the major effect of this therapy is to increase hepatic UGT activity anf yhe conjugation of bilirubin
  • it also may enhance hepatic uptake of bilirubin in the newborn
A

Phenobarbital

21
Q
  • characterized by lethargy, poor feeding, hypotonia, and a high-pitched cry in a severely jaundiced infant.
  • hyperextension of the extensor muscles and back arching
  • early bilirubin toxicity may be transient and reversible
A

Acute bilirubin encephalopathy

22
Q
  • permanent neurologic impairment
  • used to describe the clinical manifestation of the worsening encephalopathy
  • describe the pathologic findings of bilirubin toxicity within the brain
A

Kernicterus

23
Q

ACUTE FORM OF KERNICTERUS

  • poor suck, stupor, hypotonia, seizures
  • hypertonia of extensor muscles, opisthotonos, retrocollis, fever
  • hypertonia
A
  • Phase 1 (ist 1-2 days)
  • Phase 2 (middie of 1st wk)
  • Phase 3 (after the 1st wk):
24
Q

CHRONIC FORM OF KERNICTERUS

  • hypotonia, active deep tendon reflexes, obligatory tonic neck reflexes, delayed motor skills
  • movement disorders (choreoathetosis, ballisrnus,
    tremor), upward gaze, sensorineural hearing loss
A
  • 1st year
  • After Ist year
25
Q

Bilirubin is thought to enter the brain through multiple mechanisms

A
  • bilirubin produtcion that overwhelms the normal buffering capacity of the blood and tissues
  • alterations in the bilirubin-binding capacity of albumin and other proteins
  • increased CNS permeability to bilirubin
  • other factors that may affect those previously mentioned or act through novel independent mechanisms
26
Q

Potentially preventable causes of Kernicterus

A
  1. early discharge (<48 hr) with no early follow-up
    (within 48 hr of discharge); this problem is particularly important in near-term infants (35-37 wk of gestation)
  2. failure to check the bilirubin level in an infant noted to be jaundiced in the 1st 24hr
  3. failure to recognize the presence of risk factors for hyperbilirubinemia
  4. underestimation of the severity of jaundice by
    clinical (visual) assessment ‘
  5. lack of concern regarding the presence of jaundice
  6. delay in measuring the serum bilirubin levels despite marked jaundice or delay in initiating phototherapy in the presence of elevated bilirubin levels
  7. failure to respond to parental concern regarding jaundice, poor feeding or lethargy
27
Q

Recommendations

A
  1. Any infant who is jaundiced before 24 hr requires measurement of total and direct serum bilirubin levels and, if it is elevated, evaluation for possible hemolytic disease
  2. Follow-up should be provided within 2-3 days of discharge to all neonates discharged earlier than 48 hr after birth.
  3. Early follow-up is particularly important for infants younger than 38 wk of gestation.
  4. Timing of follow-up depends on the age at discharge and the presence of risk factors
  5. In some cases, follow-up w/in 24hr is necessary
  6. Poat discharge follow-up is essential for early recognition of problems related to hyperbilirubinemia and disease progression
28
Q

Risk Assessment before Discharge

A
  • Evaluate each infant for the risk of developing
    severe hyperbilirubinemia
  • Especialy for infants who are discharged before
    72 hours of age
  • Measure TSB and plot in the nomogram. If px is in the low risk-zone, possibility of severe jaundice is remote
29
Q

Timing of follow up

Infant discharged
- Before 24 hours
- 24-49.9 hrs
- 48-72 hrs

A

Should be seen by age
- Before 24 hours: 72 hrs
- 24-49.9 hrs: 96 hrs
- 48-72 hrs: 120 hrs

30
Q

What to assess on follow up?

A
  • Assess adequacy of intake in breastfed infants
  • Weight loss not be > 10% of birth weight by day 3
  • 4-6 wet diapers a day
  • 3-4 stools per day
  • stools should change from meconium to mustard yellow on the 3rd to 4th day of life
31
Q
  • failure in the folding mechanism that converts the flat trilaminar germ disc into a complex “tubular” structure starting at about 5 weeks’ gestation
  • The lateral body folds and craniocaudal folds converge at the umbilical ring, w/c contracts, closing the ventral abdominal wall
  • this ring fails to ctract and leaves a round defect of variable size and a corresponding sac composed of amnion
A

Omphalocoele

32
Q

Syndromes most often associated w/ Omphalocoele

A
  • VACTERL association
  • Beckwith- Wiedemann syndrome (macrosomia,
    macroglossia, visceromegaly, hemihypertrophy,
    hypoglycemia, renal pathology)
  • EEC syndrome (ectrodermal dysplasia, ectro-dactyly, cleft palate)
  • OEIS complex (omphalocele, exstrophy, imperforate anus, spinal defects)
  • Chromosomal abnormalities: trisomies 12, 18, 21
33
Q
  • Abdominal wall defect, to the right of a normally
    inserted umbilical cord, without membranous
    covering of the extruded organs.
  • failure in the normal attachment between umbilical cord and umbilical ring
A

Gastroschisis

34
Q

2major insults of Gastroschisis

A
  1. exposure to the amniotic fluid
  2. partial closure of the defect around the base of the eviscerated intestinal mass
35
Q

2 types of Impertorate Anus

A
  • high
  • low
36
Q
  • Rectovesical-
  • Rectoprostatis-
  • Rectobulbar-
  • Perineal fistula-
A
  • Rectovesical- from the rectum to the bladder
  • Rectoprostatis- to the prostatic urethra
  • Rectobulbar- to the bulbar urethra
  • Perineal fistula- rectal fistula opens onto the perineal skin
37
Q

three main types of malformations:

  • the rectum opens on the perineal skin anterior to the anal dimple.
  • opens in the posterior aspect of the introitus but outside the hymen
  • malfomation in w/c the rectum, vagina and urethra all open into a common channel of variable length, w/c then opens onto the perinem
A
  • perineal fistulas
  • vestibular fistulas
  • cloacae
38
Q

Surgical Management imperforate anus

  • high type
  • low type
A
  • colostomy
  • perineal anoplasty