Exam 2 pt2 Flashcards
1
Q
The presence of bacteria in the bloodstream
-This can be a transient, self-limited phenomenon, cleared by immune system without event but can also progress to sepsis
A
bacteremia
*can seed other sites such as bone, lung, and meninges
2
Q
Bacteremia coupled with inadequate perfusion and end-organ involvement
Meaning, decreased blood flow
Causing damage to important organs
A
sepsis
3
Q
Sepsis which occurs in a neonate within the first 3 days of life
A
early-onset sepsis (EOS)
*Vertical transmission
4
Q
Sepsis which occurs after the first 3 days until 2 to 3 months of life (definitions vary)
A
Late-onset (LOS)
*Horizontal transmission
5
Q
sx:
- Temperature may be elevated or depressed. Recall that normal range is 36.5 to 37.5
- Tachypnea; ie, respiratory rate > 60/min
- Other signs of respiratory distress (retractions)
- Poor color (cyanosis, poor perfuson= mallor)
- Decreased responsiveness/lethargy
- Poor feeding
- Irritability or sleepiness
A
sepsis
6
Q
Babies have a limited repertoire. Symptoms of ___ may be indistinguishable from non-infectious illness.
A
sepsis
7
Q
mortality rate of EOS
A
15%
*Higher mortality in pre-term babies and Lower in full-term
8
Q
what type of sepsis is even more likely to be complicated by meningitis with its attendant morbidity
A
late onset sepsis
9
Q
Which bacterial organisms are involved with sepsis
A
- Group B Strep
- E. coli- actually #1 in preterm babies
- Other strep species, most commonly Pneumococcus
- Enterococcus (Group D strep)
- Staph (usually iatrogenic)
- Listeria
- Klebsiella
* These organisms all come from vagina or its neighbor, the GI tract
10
Q
How does transmission of sepsis occur?
A
- During labor membranes rupture or become leaky
- Organisms can ascend from the birth canal (vagina)
- Fluid becomes infected, fetus inhales or swallows it and also becomes infected
11
Q
if mom goes into labor early for no known reason think…
A
possible infection like sepsis (Premature babies are more vulnerable because of less adequate immune system)
*Very high risk for EOS
Also higher risk for mortality associated with sepsis
It may be that the premature labor was prompted by brewing infection
12
Q
MAJOR RISK FACTORS of EOS
A
*Chorioamnionitis
Maternal Group B Strep carriage
Prolonged rupture of the membranes (18 hrs)
Prematurity
13
Q
MINOR RISK FACTORS of EOS
A
Ethnicity (black women are at higher risk of GBS colonization)
Low socioeconomic status
Male sex
Low birth weight (>2500 grams)
14
Q
Definition of Chorioamnionitis
A
Maternal temperature during labor > 38.o C or 1oo.4 F
And at least 2 other features
1. maternal leukocytosis- WBC > 15,000 in blood
2. maternal tachycardia- > 100 beats/min
3. fetal tachycardia- baseline > 160 beats/min
4. uterine tenderness (tenderness=elicited, pain=subjective)
5. foul smelling amniotic fluid
15
Q
Gram positive bacteria
Carrier state in mother (in her normal flora)
A
GBS
*6-30% prevalence internationally
16
Q
how is GBS transmitted
A
- Not sexually transmitted
- 50% vertical transmission
- Late onset disease is horizontal transmission
17
Q
____% of babies born to mothers with GBS will develop early onset sepsis
A
1%
18
Q
___% reduction in GBS sepsis from 1990s
A
87%
*Currently- all moms are cultured for GBS at 36 weeks and treat when they go into labor
19
Q
risk factors for GBS
A
- Previous child who had early-onset GBS sepsis
- GBS bactiuria during current pregnancy
- Maternal fever/chorio
- Preterm labor
- We revert to these criteria now when GBS culture is not available
20
Q
Definition of prolonged rupture
A
> 18 hrs
*The longer the membranes are ruptured, the higher the likelihood of ascending infection
21
Q
definition of Prematurity
A
gestational age less than 37 yrs
22
Q
sepsis workup includes
A
- Blood culture is mandatory (1-2ml from 1 site)
- Chest X-ray (almost always done)
- Lumbar puncture, aka “spinal tap” (look for WBC in CSF)
- Complete blood count (CBC)
- C-reactive protein
- Urine culture is NOT indicated in septic workup of a newborn in the first 3 days of life. It should be included in workup of late onset sepsis
23
Q
blood culture of spesis characteristics
A
1-2ml
- Almost all pathogens will grow within 48hrs
- Positive blood culture is diagnostic of neonatal sepsis but negative culture does not rule it
24
Q
When to include Spinal Tap for sepsis dx
A
- positive blood culture (Best performed before starting antibiotics, can be delayed if baby is unstable)
- sx referable to CNS (Neonates with symptoms of meningitis -TRUE lethargy, abnormal tone, excessive irritability, bulging fontanel, or septic shock)
- being proactive before starting antibiotics
25
sx of meningitis
lethargy, abnormal tone, excessive irritability, bulging fontanel, or septic shock
26
important features of CBC to consider
1. Total WBC count (but can be caused by stress too ie. labor)
2. Differential
3. Absolute neutrophil count
4. Immature/total ratio (I/T)
5. Immature leukocyte count
6. Normal ranges are broad and depend upon timing **best time is at least 6 hours after birth
*can be a clue but not diagnostic
27
hematopoiesis
maturation and differentiation of WBC
28
the cell line most responsible for managing bacterial infections.
neutrophils
* Mature cells of the granulocyte line
* *Also known as polymorphonucleocytes or “polys”
29
Immature forms of neutrophils include:
Bands
Metamyelocytes
Myelocytes
Promyelocytes and myeloblasts are not in circulation so not on CBC
30
Hematopoiesis of neutrophils
myeloid stem cell--> myeloblast --> N. promyelocyte --> N. myelocyte--> N. Metamyelocytes ---> N. Band---> Neutrophil
31
- Generally make up about 60% of circulating white blood cells.
- Their numbers can increase in times of stress, such as infection. Normal values for a newborn are 16,000 to 31,000!
neutrophil
| *A low neutrophil count may be more ominous as it reflects an overwhelmed immune system.
32
how do u calculate absolute neutrophil count
the total percentage of all neutrophils x total white blood cell count
*NOTE: total neutrophil count = polys + bands+ myelocytes + metamyelocytes
WBC x % of all neutrophils
Ex. 15% poly + 5% bands = 20%
WBC x 20% = ANC
33
abnormal absoulte neutrophil count
less than 1750
34
First few hours of life, ANC is approximately ___ and what happens to the number
1800
*Rises to approximately 8000/cu.mm by 12hrs
Returns to 1800 by 3 days.
35
what is released in times of stress and to fight infection
immature neutrophils
36
how do u calculate Immature neutrophils
adding all immature forms of neutrophil line and dividing by total neutrophil count
*Upper limit of normal of 0.2
37
a protein synthesized by the liver in response to, and as part of, the inflammatory response
CRP
| *Poor specificity- other perinatal conditions confound- asphyxia, fetal distress, MAS
38
normal CRP
less than 1.0
39
pattern of CRP release
- Released 4-6hr after stress
- Peaks at 24-48 hrs
- Diminishes over time as inflammation resolves
40
The only truly specific laboratory test in the workup of neonatal sepsis is __
blood culture
*However, many times, we make a PRESUMPTIVE diagnosis of sepsis based upon “clues” of suggestive history and suggestive lab tests.
41
prophylaxis tx of GBS
penicillin-mother needs 2 doses of PCN prior to delivery
*Acceptable: at least one dose, at least 4 hours prior to delivery
*If mother is Penicillin-allergic, other antibiotics are used:
Clindamycin- sensitivities are checked during pregnancy
Vancomycin- very broad spectrum
*These probably work but there is not evidence for specific parameters
42
If adequate prophylaxis, eliminates risk of EOS GBS sepsis, can go home when?
If inadequate, needs how long of an observation period to watch for evidence of EOS?
If adequate prophylaxis, eliminates risk of EOS GBS sepsis, can go home in 24 hours
If inadequate, needs a 48 hours observation period to watch for evidence of EOS.
43
Mothers who have fever or signs of chorio are treated with
antibiotics during labor to prevent sepsis in newborn
(prophylaxis for mothers with chorio)
*baby must be observed for 48 hrs after birth
44
Treatment for Newborns with Sepsis
-need IV antibiotics (best choice is ampicllin and gentamicin)
-Duration
10 full days for sepsis
2 weeks if meningitis
45
AAP and CDC recommend babies born to mothers with diagnosis of chorio should have ___
- sepsis workup
| - i.v. antibiotics: Ampicillin and Gentamicin for 48 hours until cultures are negative
46
Current incidence of EOS is estimated __% and
| Using current guidelines necessitates treating up to ___% of all newborns as if they are ill
0.05-0.12%
10%
*can use Kaiser spesis risk calculator to determine risk
47
by-product of RBC breakdown
bilirubin
*Bilirubin pigments are deposited in the skin and mucous membranes, causing the classic yellow or jaundiced appearance of the skin
48
when does jaundice occur
when an infant is hyperbilirubinemic
49
Jaundice first becomes visible____ and____ and progresses caudally to the ____
in the face
forehead
trunk and extremities
50
how to look for jaundice in a baby
Blanch the skin and look for an underlying yellow tone
51
average length of breastfeeding in the US and worldwide
and when are the biggest drop off in the US
- US is 3-6 months (bc women have to get back to work) and 5 yrs worldwide
- Biggest drop off is first leaving the hospital and the second is at 6 weeks when women go back to work
52
what % of women in CO exclusively b.f. at 3 months and 6 months and ever b.f
3 months- 50.3%**
6 months- 25.8%**
ever b.f- 81%
**meets US dept. of Heatlh goals
53
Breastfeeding initiation is less common in moms who:
Have lower education levels
Are single
Are teen
*mostly due to having to return to work, and these are the ones that would most benefit from b.f.
54
Five hospital maternity care practices that will help with successful breastfeeding:
1. Infants are breastfed in the first hour after birth.
2. Infants stay in the same room as their mothers.
3. Infants are fed only breast milk and receive no supplementation (use human donor milk if need to supplement)
4. No pacifier is used.
5. Staff gives mothers a telephone number to call for help with breastfeeding.
55
provides Federal grants to States for supplemental foods, health care referrals, and nutrition education for low-income pregnant, breastfeeding, and non-breastfeeding postpartum women, and to infants and children up to age five who are found to be at nutritional risk.
WIC (women infants and children)
*have to choose bf or formula up front and can't switch
56
Baby loses ___% of weight and then regains birth weight by day __
10%
10
*colostrum is less caloric
57
baby should have a yellow stool by day ___
5
58
what is the asymmetical latch
the babys nose should be pointed right at the nipple
-lower lip covers much more of the nipple and aerolar, babys tongue messages nipple to get milk out
*Nipple should not be latched (should Compress areolar and breast tissue)
59
___% of women in the United States elect to breastfeed and only about ___% are still breastfeeding at 6 months
75%
35%
60
indications for formula
1. a substitute or supplement for human milk in infants whose mothers choose not to exclusively breastfeed
2. For infants in whom breastfeeding is contraindicated
3. As a supplement for breastfed infants whose intake of human milk is inadequate to support appropriate weight gain
61
Whey in breast milk is primarily ____, in cow’s milk it is ____
lactalbumin
lactoglobulin
62
use thawed room temp breast milk within ___hours
4-6 hrs
63
Use refrigerated breast milk within ___ hours (formula ___hrs).
48 hrs
24 hrs
*Do not freeze formula (breast milk can be frozen for 3-6 months)
64
Normal hematocrit in newborn is __-__%
45 - 60%
| 40-50 in an adult
*Newborns have a higher mean hematocrit than adults
65
pathology of jaundice
- In utero, baby is relatively hypoxic
- Hypoxia triggers production of erythropoietin, a hormone which stimulates red blood cell production
- Jaundice occurs when this relatively large pool of RBCs are broken down, a process that can either be physiologic or pathologic in origin
66
As extra RBCs are broken down, bilirubin is released
physiological jaundice
67
Step 1:
RBC’s are destroyed in the _____.
-There, hemoglobin is separated into _____ and a ____
reticuloendothelial system
globin and a heme pyrrole ring
68
Step 2:
| Heme is converted to ______
uncongugated bilirubin
69
Step 3:
| Unconjugated bilirubin is released from the RES into the circulation where it is _____.
tightly but reversibly bound to albumin
70
Step 4:
| The bilirubin-albumin complex is carried through the splenic vein to the _____
portal vein to the liver.
71
Step 5:
| In the liver, the albumin- bilirubin complex is combined with glucuronic acid to make ______ or _____
a bile salt or conjugated bilirubin
72
Step 6:
| Conjugated bili is pumped out of hepatocyte into ____
canalicular (bile duct) system
73
Step 7-10:
7. Leaves liver through common bile duct
8. Enters Duodenum
9. Leaves body in
stool
10. Or is deconjugated and reabsorbed back into enterohepatic circulation
74
- The fat soluble product of hemoglobin metabolism (must be secreted in stool)
- Binds tightly and readily to albumin in serum; neither is changed by the union
Unconjugated bilirubin
*Known as “free bilirubin” regardless of whether it is bound to albumin or not
Unconjugated= indirect/prehepatic
75
- Salts of glucuronic or sulfonic acids
| - Water soluble
Conjugated bilirubin
*(essentially) Conjugated = Direct/post hepatic
76
Bilirubin is conjugated where
the Liver
77
Incorporated in to bile acids which act as a detergent to help in digestion of fats
conjugated bilirubin
78
In a normal newborn the direct bilirubin should be __ and ___ of the total bilirubin in the serum
less than 2mg/dl and less then 10% of the total bilirubin in the serum
79
Measures all bilirubin fractions in serum
total bili
*should be mostly unconjugated bili
80
measures 90% of conjugated bili.
direct bili
81
how to get value of indirect bili
- Ordering “fractionated” bili will get you total and direct values
- Indirect is a calculated value: Total – direct = indirect
82
causes of unconjugated hyperbilirubinemia
- Hemolytic (physiological, pathologic, immune mediated, enzyme defects, pool of blood outside vascular system)
- Prematurity
- Associated w/ breastfeeding (breast milk jaundice, b.f jaundice)
- Defects in Conjugation step (Crigler-Najjar, Gilberts, Asian descent)
83
-Normal hematocrit of term baby is 45- 60%
-begins to fall after delivery
-Nadir occurs at 2 months, with hematocrit as low as 28-30%
-Large # of RBCs destroyed in the process
-Hemolysis begins in utero, jaundice occurs
during the first 2 hours
Physiologic Hemolytic hyperbilirubemia
*peaks at 3-5 days (timing of 1st visit)
**UNCONJUGATED
84
-Occurs when a maternal antibody crosses
the placenta to attack fetal RBCs
-ABO most common, occurs in 20% of all births
-Begins in utero, therefore jaundice present during first few days of life
Pathologic Hemolytic hyperbilirubemia
-usually present 24-48 hrs of life
**UNCONJUGATED
85
___ antibody in ABO system
- Works in the same way - if MOC is Rh- then she can make ____ antibodies
- Rho-gam is ____ given at 28 weeks and after birth
D
anti-D antibodies
anti-D IgG
86
when mom is Rh- and baby is Rh+
Rh incompatibility
87
- Breakdown of extravascular blood, eg. bruising or cephalohematoma
- blood trapped between periostium and skull, caused by birth trauma
Hemolytic
UNCONJUGATED
88
Sex-linked disorder of cell membranes which makes them
vulnerable to hemolysis (More severe in males, Variable presentation in females)
-Usually occurs upon exposure to oxidants, especially medications or infections
-Protective against malaria
-Occurs in Mediterranean region, China, Africa
-Occurs in 10% of African Americans
Hemolytic: G-6PD Deficiency
*unconjugated
89
___% of premature infants become jaundiced
80%
*Premature infants conjugate more slowly bc their livers don't fxn as well
90
- Higher risk for BIND (bilirubin induced neurologic dysfunction) at lower levels of bilirubin.
- Leaky blood-brain barriers
prematurity jaundice
91
- Caused by inadequate oral intake
- ncreases enterohepatic circulation
- Essentially, dehydration
- Occurs on days 2 to 5
- baby’s hydration is the urgent problem
Breastfeeding jaundice
* UNCONJUGATED
* consider supplementation with pumped milk formula
92
- Begins day 4
- Can peak weeks 2 to 4
- Persists up to 3 months
breastmilk jaundice
* UNCONJUGATED
- Totally benign– don’t interrupt nursing (Etiology unclear)
93
- Defect in glucuronyl transferase (enzyme important in creating bile salts)
- Typically, mild jaundice after puberty
- Possibly related to breastmilk jaundice
- Possibly related to increased jaundice in certain ethnic groups
- Autosomal dominant
Gilbert's
*UNCONJUGATED
**turns yellow when get stressed
94
Severe, often lethal form of hyperbili.
- Clinically looks like kernicterus
- Autosomal recessive
- Occurs in one in one million live births
- Virtual absence of glucuronyl transferase enzyme
- Two Types (I, II)
Crigler-Najjar Syndrome
**UNCONJUGATED
95
requires lifelong PTX to avoid BIND and liver transplantation
Type I Crigler-Najjar Syndrome
96
treatable with Phenobarbital
Type II Crigler-Najjar Syndrome
97
Causes of Conjugated Hyperbilirubinemia
Hepatitis
Inborn Errors of Metabolism
Biliary atresia
98
- Agents have a predilection for cannicular cells (cells of the ductal system)
- Majority are “idiopathic”, self-limited without permanent damage
Infectious hepatitis
(Various infectious etiologies: Hep B, Rubella, CMV, Toxoplasmosis)
*CONJUGATEd
99
- Inborn error of galactose metabolism caused by congenital enzyme deficiency
- FTT,hepatosplenomegaly/cirrhosis, MR, cataracts
Galactosemia
* CONJUGATED
- Clinically, disease looks like hepatitis
100
____ jaundice implies that conjugated bilirubin flow is obstructed
Cholestatic
101
-Presents weeks 2 to 8 after birth
- Light stools***, dark urine, jaundice, weight loss and
irritability are hallmarks
-congenital absence of all or part of the biliary tree (ducts that drain bile from liver)
Biliary Atresia
* *WORRY ABOUT THIS MOST
* etiology is uncertained, may be viral
*CONJUGATED
102
treatment of biliary atresia
- Initially treated with Kasai procedure– intestine attached to liver
- Ultimately need liver transplant
103
History and physical questions to ask surrounding jaundice
- Know MOC’s blood type and Rh
- Any family history of hemolytic disease?
- Any maternal history of infections during pregnancy? Hepatitis B status?
- Prematurity?
- Medications?
- Baby’s diet history and stooling patterns
- Physical findings consistent with intrauterine infections?
- Hepato- or splenomegaly?
- Hydration status
104
Generally, in the first week of life, if there is no cause to suspect hepatitis, obtain a __
transcutaneous bilirubin level
*ALL babys are screened at 24 hrs of life
105
indications for a bilirubin level
1. baby appears jaundiced
2. compelling risk factors for hyperbilirubinemia (ABO or rh “set-up;” ie. Risk for immune-mediated hemolysis, Family history to suggest genetic causes, History of, or signs which suggest viral infection, Prematurity, Asian)
106
pros and cons of transcutaneous bilirubin monitoring
Pros:
inexpensive
good screening tool
cons:
require developing own guidelines
107
In general–if the Tcb is in the __ we will draw a serum bilirubin
HIRZ (high intermediate risk zone)
*allow higher levels overtime
108
```
Interpreting Serum Values:
high risk zone:
high intermediate:
low intermediate:
low risk:
```
High risk zone: begin phototherapy
High intermediate: recheck 6-12 hrs +/- phototherapy
Low intermediate: recheck based on clinical progress
Low risk: no need to follow up
*get serum values for high and high-intermediate
109
phototherapy: Blue light with wavelengths between 440 and 470 causes the outer ring of bilirubin to be turned 180 degrees.
how does this treatment help?
In this form it is less tightly bound to albumin, is more water soluble and can be eliminated from the body via the kidneys without conjugation
110
Phototherapy is only effective in ____
unconjugated hyperbilirubinemia
*Use of PTX in conjugated hyperbili results in “bronze baby”, an unappealing discoloration of skin
111
- ________ is used to describe acute manifestations of BIND
| - _____is used to describe chronic and permanent sequelae of BIND
Acute bilirubin encephalopathy or ABE
Kernicterus
112
- Form of brain damage associated with greatly elevated unconjugated bili
- abnormalities of tone, high pitched cry, arching of back
Kernicterus
113
Pathology: Bilirubin staining of basal ganglia and hippocampus
-Had occurred in babes with erythroblastosis fetalis and has become rare since the advent of rho-gam
Kernicterus
114
protects skin from utero environment
| helps baby regulate its temp and keep skin soft
vernix
115
when normal: non-blanching lesions secondary to birth trauma or vigorous resuscitation
-bluebery muffin appearance
Petechiae
*When to be concerned
Mom has a hx of low platelets or infection risk

116
downy hair present to varying degrees at birth
| 
lanugo
*gets rubbed off in first 2-3 week of life
117
- Lace-like pattern of dusky erythema over trunk and extremities.
- Normal in newborns who are cold - disappears on re-warming.
- Can be a sign of poor perfusion in illness - look for other physical exam finding.
Mottling (cutis marmora
118
- Rare congenital vascular malformation.
- Usually presents on one limb**
- Associated with underlying muscular defects (50%).
- Usually resolve with time - but should be followed for the risk of ulceration**
Cutis marmorata telangiectatica congenita (CMTC)
119
Transient skin lesions
-Erythema Toxicum
-Milia
-Sebaceous Gland Hyperplasia
-Pustular Melanosis
-Normal Peeling
-Sucking Blisters

120
- White to yellow papule on a blotchy erythematous base.
- Usually start on DOL 1 or 2.
- Contain eosinophils - but etiology poorly understood.
- Come and go over first 10 days of life.
- Located everywhere except palms and soles.
- Worse in areas of high friction and heat (such as back)
Erythema Toxicum
121
- Keratin filled epithelial cysts
| - Often mistaken for neonatal acne which does not appear until 2 weeks of life
Milia
122
- More yellow than milia.
- Caused by maternal androgen exposure.
- Also spontaneously resolve
Sebaceous Gland Hyperplasia
123
- Fragile pustular rash that begins in-utero.
- When pustules break they leave behind a white collarette with a central hyper-pigmented macule.
- No inflammatory component.
- Pustules usually removed during resuscitation or bath
Transient pustular melanosis
124
do not use lotion for the first ____ of life because it will get into the blood stream
3 months
125
immature thalmus
| cause 1 side of body to be red
Harlequin Phenomenon
126
____ represent an area of excess of one or more of the normal components of skin per unit areas.
Blood vessels, pigment cells, sebaceous glands, epidermis…
Birthmarks
127
____ are blue-black macules commonly located over lumbosacral area
-Important to clearly record location of macules as they are easily confused for bruising.
Mongolian Spots
-Fade during first or second year of life. Most have disappeared by age 6-10.
128
Light brown oval macules.
Single lesions occur.
Multiple lesions associated with neurofribromatosis.
Measure size and draw location.
Cafe Au Lait Spots
129
Occurance: 99% present at birth- clinically subtle
Location: common on limbs, anywhere
Appearance: poorly circumscribed
Course: no change in size, grows with child, no involution
Vessel Type: predominately venous and capillary
Malformations
| congenital vascular lesion
130
```
Occurance: 40% present at birth
Location: 40% present at birth
Appearance: well delineated
Course: rapid neonatal growth with slow involution
Vessel Type: predominately arterial
```
Hemangiomas
| congenital vascular lesion
131
Benign neoplasm resulting from rapid proliferation of endothelial cells.
Superficial (strawberry), deep or mixed.
Proliferate for 8-18 months then regress
hemangiomas
Regress by rule of 10:
50% gone by age 5, 90% gone by age 9 (10% per year).
132
Indications for treatment of hemangiomas
- Obstructed vision.
- High-output heart failure.
- Hemangiomas in diaper area because tend to ulcerate.
- Facial lesions because they can disfigure even after they regress
**treatment is painful and not that helpfu
133
Vascular Malformations
- Nevus simplex (salmon patch or macular stain)
- Port Wine statins
- Cutis Marmorata
134
Light red blanching macule on nape of neck (stork bite) or glabella/eyelids (angel kiss).
-Fade with time but always present - more pronounced when flushed
Nevus simplex
135
-Flat dark pink to red macule found on face or limbs.
-Typically do not cross midline.
On face and involving an eye, may be associated with Sturge-Weber syndrome
-On legs can be associated with Klippel-Trenaunay-Weber syndrome
Port wine stain or Nevus Flameus (capillary)
136
Hemangioma of face and meninges.
| Associated with seizures
Sturge-Weber syndrome
137
Port wine stain or Nevus Flameus on the legs
Associated with hemihypertrophy
*It is characterized by a triad of port wine stain, varicose veins and bony and soft tissue hypertrophy involving an extremity
Klippel-Trenaunay-Weber syndrome
138
Light red blanching macule on nape of neck
stork bite
*nevus simplex
139
Light red blanching macule on glabella/eyelids
angel kiss
*nevus simplex
140
_____ must be considered if vesiculobullous or pustular lesions are found in a newborn
Infection
141
viral infectious lesions and bacterial infectious lesions
Viral: Herpes Simplex, CMV, varicella zoster
Bacterial: Staphylococcus, bullous impetigo
142
- Slightly raised oval or linear area, yellow or orange.
- Common on scalp, associated with alopecia.
- Consist of hypertrophy of sebaceous glands (glands which produce acne later).
- Malignant potential later in life - consider removal (basal cell carcinoma).
Sebaceious Gland Nevi
143
- Hamartomas arising from the embryonic ectoderm.
- May be deeply or slightly pigmented.
- Have either a unilateral or bilateral distribution, and often favor the extremities in what appears to be a dermatomal distribution.
- Onset is usually at birth but may also occur in the second or third decade.
Epidermal Nevi
144
Special Qualities of Newborn Skin
Thinner
| Large Surface area-to-body ratio
145
bathing a newborn
- Sponge baths until cord separates.
- Then bathe in water without soaps or bubbles no more than a couple times a week. Use soap only if baby very dirty or smelly.
- Regular neck/skin fold cleanings with a burp cloth enough to keep an infant clean.
- Water temp
146
water temp when bathing a newborn
less than 120 degrees F
147
how to prevent diaper rash
- Change diaper with every feed.
- Use mild wipes - without additives. (water and washcloth best!)
- Allow area to fully dry before putting on new diaper.
- Apply a thick layer of protective ointment or cream (such as one that contains zinc oxide or petroleum jelly)
148
diaper rash
Has satellite lesions.
Most prominent in skin folds.
Candidal diaper rash
*caused by Candida albicans
149
treatment of Candidal diaper rash
topical antifungal cream, eg, mycostatin
150
Caused by chemical irritant.
20% of babies will develop by 2 yrs.
Most pronounced on convex areas, spares skin folds.
contact diaper rash
*tx by:
remove irritant, air dry, use petroleum barrier.
151
Why not use cow’s milk?
- Infants fed cow’s milk develop anemia
- Higher concentrations of protein, sodium, potassium and chloride
- Inadequate essential fatty acids, Vit E and zinc
152
Powdered: one scoop of powder to___ oz of water = ___ cal/oz
2
20 cal/oz
153
Formula should be fed ____
ad libitum, on demand
154
Guidelines for intake:
Usually, for the first day, they will take __ml every 3 hrs
Rapidly increase to about __oz by day 3
Usual intake will be __ml/kg/day for first 3 months
Usually, for the first day, they will take 10-15ml Q 3 hours
Rapidly increase to about 1-1.5 oz Q 3-4 hours by 3 days
Usual intake will be 140-200 ml/kg/day for first 3 months
155
For growth, baby needs a minimum of ____ cal/kg/day to thrive and grow
*Standard formula is 20cal/oz
100cal/kg/day
156
```
Goals for Growth:
Surpass birth weight by ___
0-3 months gain ____
3-6 months gain ____
6-12 months gain
```
Surpass birth weight by 2 weeks.
0-3 months gain 15-30 grams/day
3-6 months gain 15-20 grams/day
6-12 months gain 10-15 grams/day
157
Vomiting or spitting up is common and does not require formula change unless:
- there is inadequate growth
- Due to weak sphincters
*Only change formula if blood in stool, weight loss, projective vomiting, extensive rash
158
Stools tend to be ____ in color, occur ____ daily
pasty and brown
one to twice/day
159
Constipation with slow weight gain may indicate _____
inadequate calories (volume or concentration – check history)
*constipation with good wt gain is common with formula!
160
Begin complementary solids at ___
4-6 months
161
____ formulas should be given to infants who have had a severe gastrointestinal reaction (abdominal pain with bloody diarrhea) to cow's milk, since anywhere from 30% to 64% of babies will also have a similar reaction to soy
Hypoallergenic
162
According to the AAP, the only formula-bed babies who should get soy formula are those with:
- Parents who are strict vegans
- A true diagnosis of lactose intolerance, which is actually rare in babies but more common among older kids and adults.
- Congenital galactosemia (in which babies lack the enzyme that converts galactose, one of two sugars found in lactose, into glucose, a sugar the body is able to use).
163
is the inability to digest the sugar lactose, found in cow's milk and cow's milk infant formulas
Lactose intolerance
*not the same as a “milk allergy”
milk allergy kid will not tolerate soy either
164
If these children consume breast milk, cow's milk, or other dairy products, galactose can build up in the system and damage the body's cells and organs, leading to blindness, severe mental retardation, growth deficiency, and even death.)
Congenital galactosemia
165
inflammation of mucosa resulting in GI discomfort and blood in stool
colitis
166
it's more common among infants (about 2% to 3% of babies), though most outgrow it. (80% by 4)
- Diarrhea, vomiting, failure to thrive, colitis, eczema and respiratory symptoms
- IgE mediated
milk allergy
*Skin prick test not accurate until 12 months.
167
______ formulas have cow's milk proteins that are broken down into small particles so they're less allergenic than the whole proteins in regular formulas
Extensively hydrolyzed formulas
*Most infants who have a milk allergy can tolerate these formulas, but in some cases, they still provoke allergic reactions
168
____ formulas, which contain protein in its simplest form (amino acids are the building blocks of proteins).
Amino acid-based infant formulas
169
______ formulas also are on the market, but aren't considered truly hypoallergenic and can still provoke a significant allergic reaction.
- Use corn sugars rather than lactose sugars and partially digested proteins
* **Marketed to treat colic
"Partially hydrolyzed" formulas
170
The final common pathway of all of the pathology in this system is the ____ of passage of material along the tract because of:
obstruction
1. atresia
2. stenosis
3. blockage
4. dysmotility
171
non-formation resulting in discontinuous lumen and complete obstruction
atresia
172
contents of lumen harden to cause obstruction or external compression of tract occurs
blockage
173
formation with restriction; i.e., limitation of diameter of lumen with partial obstruction
stenosis
174
peristalsis, the rhythmic contraction of tract is abnormal leading to functional obstruction
dysmotility
175
sx:
Presents with vomiting
Belly will be flat or scaphoid (concave
upper GI tract problem
* From mouth to end of duodenum
* Closer to mouth, sooner the emesis
176
sx:
Presents with decreased or no stooling.
Belly will be distended
lower GI tract problem
*duodenum to anus
177
"Air-fluid levels” reflect
decrease in peristalsis
178
Radiology studies used to define location of a problem
1. xray
2. us
3. contrast studies
*Air is present in the stomach immediately after birth.
By 24 hours of life air should appear in the rectum
179
decreased swallowing, decreased GI motility, vomiting or gastric decompression
gassless abdomen
*Note: Resuscitation may increase the amount of bowel gas seen on x-ray
180
perforation: contents of the bowl have gotten outside the bowel causing peritonitis --> pain w/ movement
get abdominal distension
abdominal tenderness
tympanic
acute abdomen
181
Immediate interventions for GI problems
1. Make baby N.P.O. – nothing by mouth until you have identified problem and have decided upon intervention
2. Place nasogastric/oralgastric tube to decompress stomach of air and stomach contents
3. Start I.V. to provide fluid maintenance
4. Call surgeons
182
The gastroesophageal sphincter in a newborn is present but does not have sufficient tone to prevent emesis. What goes down, easily returns.
wet burp or
the clincally significant version is known as gastroesophageal reflux
183
Normal newborns all spit up for a variety of reasons:
“wet burp”, swallowed materials, overfeeding, stooling.
184
sx:
-When mild, nuisance
-Pain of esophagitis
-Poor growth because of inadequate intake**
(Losing too much of feed, Refusal to eat b/o pain)
-Risk of aspiration and pneumonia
-commonly arch their back
Gastroesophageal reflux
GERD
185
management for GERD
- Feed small amounts frequently
- Positioning during and after feeding (keep head above stomach)
- Medications to prevent pain- H2 Blockers, PPIs
- Motility agents (ex. Reglan)
- Surgery
186
In severe cases of gastroesphageal reflux, ______ may be warranted
fundoplication with gastrostomy feedings
187
take fundus of the stomach and create an new sphincter
fundoplication
188
Esophagus is discontinuous and there is communication between GI and respirtory tracts.
*most common malformation of the esophagus
esophageal atresia
189
sx:
- Drooling
- Immediate vomiting after eating
- Coughing
- Abdomen may be distended or scaphoid
esophageal atresia
*usually present in the first few hours of life
190
Occurs with tracheoesophageal fistula (TEF) in 85-90% of cases
_____ most common presentation
Proximal EA with distal TEF
191
Malformation: Disordered elongation / separation of esophagus from trachea
- 30-40% have associated anomalies (especially VACTERL)
- May have history of polyhydramnios
esophageal atresia
192
type of EA:
atresia only, 5-10%
belly will be concave
Type I
193
type of EA:
- Fistula only
- presents later because esophagus is still intact, 5%
- chronic cough, poor growing, repeated pneumonia
- normal belly
Type II (H type)
194
type of EA:
-Atresia plus lower fistula
belly is distended: fill up with air w/ each breath
85-95%
Type III B
195
Type of EA:
rare
Atresia + upper fistula
Type III A
196
type of EA:
rare
atresia + double fistula
Type III C
197
Evaluation of Suspected Esophageal Atresia
Attempt to pass catheter from mouth to stomach
198
xray:
| Shows catheter in proximal esophagus with no air in stomach
EA
indicating no connection between distal esophagus and trachea
199
VACTERL Association features:
```
Vertebral anomalies
Imperforate Anus
Cardiac defects (PDA,ASD,VSD)
Tracheoesophageal fistula
Renal anomalies
Limb anomalies (most often of radius)
```
*Present in 25- 30% of children with EA/TEF
200
```
sx:
Vomits a few minutes after each feeding
Seems hungry
Gets worse as days pass
Often presents with dehydration
Normal stool
No fever
No ill contacts
-Increasing non-bilious emesis, after day 4, average 3 weeks
```
Pyloric Stenosis
*children 2 days to 2 months
201
pyloric stenosis can result in ____
PE may show palpable firm mass in ____
Hypertrophy of muscular layer of pyloric channel
*PE may show palpable firm mass RUQ
"olive"
202
treatment of pyloric stenosis
Very minor surgery
| “Pyloromyotomy”- consists of incising the hypertrophic muscle from the outside to essentially loosen it up
203
Bilious vomiting implies ___
obstruction distal to the ampulla
*Usual spitting up or feeding intolerance is not bilious; therefore, consider pathologic until proven otherwise
204
sx:
- vomiting (Vomiting begins soon after birth)
- Abdominal distention may be present soon after birth, peaks at 24 to 48 hrs.
- May pass meconium
Duodenal Obstruction
205
Malformation:
| Caused by incomplete re-canalization of gut during 8th to 10th week of fetal life
Duodenal Obstruction
206
Less common
| Caused by extrinsic compression of duodenum by a pancreas which encircles it
annular pancreas
207
prenatal clues to duodenal obstruction
1. polyhydramnios
2. SGA
3. Preterm (secondary to stress)
4. Prenatal US (20 weeks) shows "double bubble"
208
30% of babes with duodenal obstruction have ______, many have other anomalies as well
Down syndrome
*DS baby vomitting think duodenal obstruction
209
If distal gas present, on abdominal film
duodenal stenosis rather than atresia
210
50% of all atresias occur in ___-
jejunum or ileum
211
midgut obstructions
atresia
stenosis
Malrotation/midgut volvulus
212
Mesenteric attachments are posterior only
Midgut is free to move
Duodenum and proximal colon are fixed
Malrotation/midgut volvulus
213
```
Abnormal rotation (in ______ fashion) results in abnormal position, known as malrotation – as such, does not cause obstruction BUT
May twist in either direction, causing a ___
```
clock-wise fashion
volvulus
214
In addition to obstruction, malroatation with volvulus causes compromise of vascular system, resulting in:
resulting in necrosis, peritonitis, sepsis and shock
*THIS IS A SURGICAL EMERGENCY
215
sx:
- Apparently well child who presents with sudden onset of bilious vomiting
- Initially intermittent
- Abdominal distention (tenderness common)
- Vascular compromise leads to acute abdomen – peritonitis, sepsis, shock, metabolic acidosis
Malrotation with Midgut Volvulus
*May occur at any time postnatally but 80% occur in neonatal period (first month)
216
Clue to:
| Associated with abdominal wall defects, intestinal atresias, congenital heart defect, situs inversus
Clue to presence of malrotation
217
Abdominal X-ray shows dilated bowel with air fluid levels
Malrotation with Midgut Volvulus
218
Upper GI shows abnormal ligament of Treitz
Barium enema show abnormal cecum position
Malrotation with Midgut Volvulus
219
All bilious vomiting in the newborn is_____ until proven otherwise!
Volvulus
**this is a surgical emergency
220
“Ladd Procedure” done to divide the peritoneal bands, unwind twisted portion and tack down the bowel to prevent it from happening again
tx in Malrotation with Midgut Volvulus
*done prior to volvulus is possible
221
Large amount of intestinal loss results in _____
short bowel syndrome or death
*have removed a lot of internal surface area= poor absorption = failure to thrive
222
distal bowel obstruction dx
meconium ileus
Hirschprung's disease
Anal atresia
223
sx:
| A term newborn eats well for the first day, but then develops abdominal distention.
Typical presentation of lower tract obstruction
224
presents with:
- passage of little or no meconium in the newborn period
- Abdominal distension
- Air-fluid levels seen on x-ray, indicating decrease in peristalsis
lower tract obstruction
225
sx:
- Abdominal distention at or after birth
- Viscous, inspissated meconium obstructs the mid-ileum
- Simple obstruction (2/3) or complicated by volvulus, atresias, perforation, peritonitis
Meconium Ileus
*Associated with Cystic Fibrosis
226
Diagnosis and treatment of Meconium Ileus by
gastrograffin enema
227
Untreated, meconium ileus can cause ____
rupture of bowel, spilling intestinal contents into peritoneal space
*This results in peritonitis, infection of that space.
228
```
Presents as an acute abdomen:
Rigidity
Tenderness
Fever
Shock
Death if untreated
```
Meconium Ileus with Perforation
229
sx:
- Constipation, delayed stooling, meconium plug
- Abdominal distention
- Congenital but rarely diagnosed in neonatal period- diagnosed by 4 months
- Extreme cases: “Toxic megacolon”
Hirschprung’s Disease
* 75- 80% male
- Accounts for 25% of all congenital GI obstruct
230
he appears constipated and strains excessively before passing stool. He has a stool only every 3-4 days. He sometimes vomits after meals. On exam his abdomen is mildly distended. He was noted to pass a hard rubbery plug of meconium with his first stool at 2 days of life.
Hirschprung’s Disease
*abnormal to have 1st stool on 2nd day of life
231
- Congenital absence of nervous plexuses of the colon
- Always involves anus along with proximal segment of variable length
- Peristalsis is abnormal in affected segment (skinny), causes functional obstruction with dilated bowel proximally
Hirschprung’s Disease
232
The nervous plexus is missing in the ____, not permitting it to relax.
small section
233
- Abdominal films may show lack of gas in rectum
| - Barium enema shows “transition zone”
Hirschprung’s Disease
234
- Suction rectal biopsy shows lack of ganglion cells
| - Full thickness biopsies occasionally needed
Hirschprung’s Disease
235
treatment of HD
NG decompression, IV fluids, antibiotics
236
____ to promote stooling in HD
Initial enemas
237
Laparoscopic or open “pull-through” of colon containing _____
ganglion cells to the anal verge
238
sx:
- PE shows no / abnormal anus
- 75% have fistula to urethra, vagina, or perineum and may pass meconium
- Associated with VACTERL, chromosomal, cardiac, and CNS anomalies
imperforate anus
239
treatment of imperforated anus
surgially
| longterm outcome good
240
Although the causes of intestinal obstruction are______ rather than _____, it is often wise to begin antibiotics prophylactically
mechanical
infectious
241
types of vental wall defects
Gastroschisis
| Omphalocele
242
sx:
- Intestine present outside abdominal wall
- Risks include motility problems, fluid loss, infection
- Present at birth
ventral wall defects
243
Immediate response for ventral wall defects
1. Cover with saran wrap to prevent fluid losses
2. NPO
3. Start I.V.
4. Start antibiotics
5. Surgical intervention
244
- Malformation*
- Failure of intestine to fully return to abdomen during fetal life
* *Always associated with malrotation
omphalocele ventral wall defect
245
- Disruption**
- Caused by vascular accident during fetal life
- Dysmotility because has a thick peel on surface as result of contact with amniotic fluid
Gastroschisis ventral wall defect
246
What is the conjunction step in the conjugation of bilirubin and where does it occur?
- addition of glucuronic acid and requires glucuronyl transferase
* occurs in the liver
