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
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)
3
Q
Indirect Hyperbilirubinemia
Decreased Clearance includes
A
- prematurity
- G6PD Deficiency
4
Q
Indirect Hyperbilirubinemia
Increased Enterohepatic Circulation includes
A
- Breast milk (breast-feeding) Jaundice
- Pyloric stenosis
- Small or large bowel obstruction or ileus
5
Q
Mixed types
A
- Sepsis
- Congenital Syphilis
- TORCH
- Coxsackie B Virus
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
7
Q
- 60-80% idiopathic or biliary atresia
- Clinically jaundiced beyond 3 weeks of life
- pale stools
- dark urine
A
Direct Hyperbilirubinemia
(Cholestatic Jaundice)
8
Q
Direct Hyperbilirubinemia
Ductal disturbances in excretion
- nonsyndromatic paucity of bile ducts
- associated with lymphedema
A
Intrahepatic biliary atresia
9
Q
Direct Hyperbilirubinemia
Ductal disturbances in excretion
- isolated, trisomy 18, polysplenia-heterotaxia syndrome
A
Extrahepatic biliary atresia
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
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
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
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
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
15
Q
- current mainstay of treatment
- proved to be instrumental in containing the rate of rise of TB and lowering the TB
A
Phototherapy
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
- 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) - failure to check the bilirubin level in an infant noted to be jaundiced in the 1st 24hr
- failure to recognize the presence of risk factors for hyperbilirubinemia
- underestimation of the severity of jaundice by
clinical (visual) assessment ‘ - lack of concern regarding the presence of jaundice
- delay in measuring the serum bilirubin levels despite marked jaundice or delay in initiating phototherapy in the presence of elevated bilirubin levels
- failure to respond to parental concern regarding jaundice, poor feeding or lethargy
27
Q
Recommendations
A
- 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
- Follow-up should be provided within 2-3 days of discharge to all neonates discharged earlier than 48 hr after birth.
- Early follow-up is particularly important for infants younger than 38 wk of gestation.
- Timing of follow-up depends on the age at discharge and the presence of risk factors
- In some cases, follow-up w/in 24hr is necessary
- 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
- exposure to the amniotic fluid
- 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
