Nutrition and GI Flashcards
1
Q
List the essential amino acids?
A
branched chain (leucine, isoluecine, valine), histadine, lysine, methionine, phenylalanine, threonine, tryptophan
2
Q
What amino acids are considered essential in the premature newborn
A
cysteine, arginine, tyrosine, taurine (CATT)
3
Q
What are the conditionally essential amino acids in infants?
A
arginine, glutamine, glycine, proline, taurine, tyrosine
4
Q
Where are B12, bile salts and acids absorbed?
A
ileum
5
Q
where are lipids aborbed?
A
jejunum
6
Q
Where are sodium, Cl and K absorbed?
A
Large intestine
7
Q
At what GA, are sucrase, maltase and lactase at adult levels?
A
sucrase and maltase at 28 weeks and lactase at 36 weeks
8
Q
Compare infant with SIP to those with surgical NEC
A
SIP occurs earlier (usually first week of life) compare to the timing and presentation of NEC. infants with SIP are more likely to be smaller, more premature, unlikely to be fed, have a PDA s/p indocin, receive vasopressor support, receive surf and vent support. a/w chorio
9
Q
What meds are a/w SIP?
A
indomethacin, hydrocortisone, vasopressors
10
Q
Where is a SIP most likely to occur?
A
terminal/ distal ileum
11
Q
What is the most common to least common sites for jejunal-ileal atresia?
A
distal ileum, proximal jejunum, distal jejunum, proximal ileum
12
Q
What % of patients with meconium ileus have CF?
A
90%
13
Q
What % of patient with CF have meconium ileus?
A
10-15%
14
Q
What other things can malrotation be associated with?
A
gastroschisis, Beckwith-Weidemann syndrome, jejunal atresia, CDH
15
Q
Is Jejunal atresia more common in males or females?
A
its equally common in both; same for gastroschisis
16
Q
pancreatic insufficiency (watery diarrhea, steatorrhea), bone marrow dysfunction (neutropenia), short stature, normal bicarb secretion, normal sweat test. Whats the diagnosis?
A
Schwachmann-Diamond syndrome
17
Q
List the defects seen in Pentalogy of Cantrell
A
omphalocoele, CDH, sternal cleft, extopia cordis, intracardiac defect (VSD or diverticulum of the LV)
18
Q
What are the abnormalities in OEIS complex?
A
omphalocoele, extropohy of the bladder, imperate anus, spinal deformity
19
Q
What syndromes are a/w omphalocoele?
A
pentalogy of Cantrell, Beckwith-Wiedemann Syndrome, OEIS complex
20
Q
Is omphalocoele more common in males or females?
A
males
21
Q
True or FalsePreterm infants fed standard infant formulas gain a higher proportion of their weight as fat when compared with a fetus of the same maturity
A
TRUE
22
Q
T or F The use of special pretmer formulas and preterm HMF results in a compositon of weight gain and bone mineralization closer to that of the reference fetus when compared to infans fed standards term formulas or straight human milk
A
TRUE
23
Q
What fatty acids are essential in neonates?
A
linoleic and linolenic acid
24
Q
Infant with hemorrhagic dermatitis, skin atrophy, scaly dermatitis, weakness, impaired vision, edema, high BP, and impaired growth. Name the nutritional deificiency
A
essential fatty acid deficiency
25
How is essential fatty acid deficiency diagnosed?
Triene:Tetraene ratio > 0.4 (AKA Holman index)
26
What oils are in preterm formulas to help with fat absorption?
MCT and long chain polyunsaturated triglycerides
27
Name the components of fetal composition that decrease with advancing GA and birth weight
Total body water, extracellular water, sodium and chloride content,
28
Name the components of fetal composition that increase with advancing GA and weight
intracellular water, protein, fat, Ca, phos, mag, iron
29
What the goals for nutrition content : %fat, % carbs, % protein
30-50% fat (no more than 60%), 35-65% as carbs, and 7-15% protein; 3-4 g.kg.day fat, 9.7-17 g/kg/day carbs and 3.5-4 grams/kg/day protein
30
What macronutrient provides 2/3 of the fetal energy source?
maternal glucose
31
What are the gluconeogenic substrates for in the fetus?
alanine, glutamic acid, aspartic acid
32
What makes up 30% of all milk protein?
secretory igA and lactoferrin
33
Whey to Casein ratios in colostrum, mature human milk, hydrolyzed protein based formulas, 14 day post-delivery pumped human milk from mother of 26 weeker, predominantly whey formulas: preterm formulas
colostrum 80:20; mature human milk is 50:50; hydrolyzed protein terms based fomulas are all hydrolyzed casein; 14 day post deliver in 26 weeker mom is 60:40 and predominantly whey formulas are 60:40: preterm formulas are 60:40
34
What is the most important amino acid that assists the metabolism of fat?
carnitine
35
What are the most abdundant fatty acids in human milk?
Oleic acid (18:1) and palmitic acid (16:0)
36
Where is iron absorbed?
duodenum and proximal jejunum
37
How much iron do preterms and low BW infants need?
at least 2 mg/kg/day
38
hemorhagic diseae of the newborn- name the vitamin deficiency
Vit K
39
BPD, abnormal epiphyseal formation and tooth enamel- name the vit deficiecy
Vit A
40
a/w pyruvate dehydrogenase complex deficiency, MSUD, and beriberi (fatigue, irritability, constipation, cardiac failure). Name the vit deficiency
B1 (thiamine defiency)
41
FTT, dermatitis, mucositis, a/w glutaric aciduria type 1. Name the vit deficiency
Vit B 2 (riboflavin)
42
dermatitis, mucositis, seizures, a/w homocystienuria. Name the vit deficiency
Vit B6 (pyridoxine)
43
scalring dermatitis, a/w biotinidase deficiency, proprionic acidemia, pyruvate dehydrogenase complex deficiency, and beta- methylcrotonyl glycinuria. Name the vit deficiency
biotin
44
poor wound healing, transient tyrosinemia. Name the vit deficiency
Vit C
45
osteopenia/rickets, FTT, tetany. Name the vit deficiency
Vit D
46
Inuslin metabolism involves which trace element?
chromium
47
this trace element is critial for RBC production and Hgb formation
copper
48
This trace element activates super oxide dismutase, and involved in normal bone structure as well as carbohydrate metabolism
Manganese
49
This trace element is a cofactro in glutathione peroxidase (RBC membrane stabilization vs oxidative stress).
Selenium
50
Deficiency in this trace element results in cardiomyopathy
selenium
51
Which trace element is deficient in acrodermatitis enteropathica and what is the inheritance
zinc and AR
52
FTT, alopecia, diarrhea, perianal dermatitis, ocular changes, rash ( crusted erythematous, involving the face, extremities, and anogenital areas), nail hypoplasia or dysplasia
acrodermititis enteropathica
53
Preterm neonates with this deficiency present with poor weight gain, malabsorption, and poor wound healing.
zinc
54
What happens to the fetus when there is a maternal zinc deficiency?
fetal growth restriction and congenital anomalies
55
delay or failure pass meconium, bilious emesis, abdominal distension with x-ray findings that show distension of small and large bowel with lack of rectal air. Name the diagnosis
Hirschsprungs
56
What is the most likely long term outcome of an infant with medical NEC?
intestinal stricture; most commonly in the L colon
57
Which maternal disease are a/w small left color or microcolon?
DM and hypothyroid
58
What are risk factors for gastroschisis?
young maternal age, maternal smoking, alcohol use, illicit drug use, salicylates, and over the counter vaso-active medications
59
Are the following gene polymorphisms a/w gastroschisis or omphalocoele? Endothelial NO synthase, intrcellular adhesion molecule 1, and atrial natriuretic peptide.
Gastroschsis. Having these increaes your risk 2 fold
60
What is the most common cause of neonatal liver failure?
neonatal hemochromatosis aka gestational alloimmune liver disease
61
What are the two most common causes of conjugated hyperbili and what is the pathology?
biliary atresia (extrahepatic obstruction) and idiopathic neonatal hepatitis (intrahepatic); Biliary atresia pathology: bile duct proliferation and idiopathic neonatal hepatits is mult-nuleated giant cells and increased AFP
62
What are the intrahepatic obstructive causes of conjugted hyperbili?
neonatal idiopathic hepatitis, Alagilles (paucity of intrahepatic bile ducts with extrahepatic ducts, unusal facies, vertebral anomalies), non-syndromic paucity of bile ducts
63
What the extra-hepatic obstructive cause of direct hyperbili?
biliary atresia, sclerosing cholangitis, bile duct stenosis, choledocal cyst (cystic dilation of the common bile duct), bile plug syndrome
64
What are the metabolic causes of direct hyperbili?
galactosemia, glycogen storage disease, tyrosinemia, alpha 1 antitrypsin deficiency, hypopituitarism, hypothyroidism, CF, and Zellwger syndrome
65
Lis the genetic causes of conjugated hyperbili?
Turner, T21
66
What percent of the Total bilrubin should the direct component be?
no more than 15%; conjugated hyperbili is when direct is >1 when TB is <5 or more than 20% of the TB if > 5 OR > 1.5 if > 10% of TB
67
Is polyhydramnios more common in jejunal atresia or ileal atresia?
polyhydramnios occurs in in ~1/3 of fetuses with jejunal atresia and is less common in fetuses with ileal atresia
68
How the pathology of duodenal atresia differ from jejunal/ileal atresia?
duodenal atresia occurs due to fialure of recanalization of the intestinal tube durinr the 8th and 10th week of gestation after obliteration of thje lumen by epithelial proliferation (6-7 weeks). Jejunal-ileal atresia occurs b/c of the presence of bile droplets, meconium, and lanugo distal to the atresia and occlusion occurs after intestinal development
69
What is the most common etiology behind jejunal-ileal atresia
intrauterine ischemic injury 2/2 volvulus, malrotation, intestinal strangulation
70
What the genetic inheritance and gene mutation for Schwachman Diamond syndrome
rare autosomal recessive disorder that is most commonly due to a mutation in the SDS gene on chromosome 7q11. This gene plays are role in the maturation of the ribosomal subunit
71
What is the most common cause of an abdominal flank mass?
hydronephrosis
72
What are the infectious causes of conjugated hyperbili?
viral (m ost common), toxo, syphilis, TB and listeria
73
What is the most likely etiology behind biliary atresia?
perinatal viral infection followed by exaggerated inflammation and bile duct obstruction
74
For infants with syndromic or complex CDH, what is the most common other organ system that is affected?
cardiac (60%)
75
What is the most common associated anomaly with tracheoesophageal atresia?
congenital heart disease.associated anomalies occure in >50% of cases of TE artresia and esophageal atresia
76
In patiuent with short gut that retain their colon, what hormone if important for intestinal transit
Glucagon-like peptide -2 ; secreted by L cells in the terminal ileum and colon and increases intestinal blood flow.; it is an anti-secretory hormone that regulates intestinal transit
77
What is the most common inherited RBC enzyme defect and what is the inheritance pattern?
G6PD, x-linked recessive
78
Heinz bordies on a periheral smear
G6PD
79
What is the most common hereditary hemoolytic anemia among people of Northern European background?
Hereditary spherocytosis; autosomal dominant,
80
What hemophilia is a/w Noonan's syndrome
Hemophilia C, AR, Factor XI deficiency
81
What is the timing of presentation of HDN for early, classic, and late>
early within 24 hours of life, classic 2-7 days, late 2w-6 months
82
What are the causes of early HDN?
maternal medications like anticonvulsants, cephalosporin, nd wafarin
83
What the causes of classic HDN? How does it present?
inadequate Vit K; p/w GI bleeding, ICH, oozing
84
What are the causes of late HDN?
inadequate intake, hepatobiliary disease. ( high risk for ICH and death, more common in boys)
85
What components of breastmilk are destroyed in the paturization proess?
IgM, lymphocytes, alk phos, cytokines, lipoprotein and some lipases, lactoferrin concentration decreased, lysozyme concentration decreased
86
What components of breastmilk are preserved in the paturization proess?
oligosaccharides, Vit A,D,E, lactose, long chain polyunsaturated fatty acids and epidermal groth factor; IgA and IgG
87
infant with Pierre Robin sequence changed from breastfeeding to formula. Developed poor weight gain, severe diaper dermatitis, hypoalbinemia, crusted erythematous rash around peri-oral area. Name the diagnosis and vit deficiency?
acrodermititis enteropathica; zinc deficiency
88
What drug is prescribed to moms with mastitis?
dicloxicillin, if resistant to Diclox, then erythromycin and is resistant to both, Vanco
89
At how many months of age does ECF = ICF?
3 months, 35% each
90
How much of the cardiac output goes to infant kidneys? Adults?
infant is 10% while adults is 25%
91
What are the differences between cows milk formula and breastmilk?
human milk contains more long chain polyunsaturated fatty acids, more carnitine, cholesterol, DHA; Cows milk formulas have more amino acids
92
What are the differences between preterm human milk and term milk?
preterm milk contains more protein, sodium, Cl but less lactose. Protein amt is still insufficient so it needs to be fortified
93
Compared to term infant formula, what are the contents of protein, lactose, sodium and iron?
compared to term formula, preterm formula has a high protein content, lower lactose, higher sodium, less iron. Preterm formulas are iso-osmolar until fortified beyond 24 kcal/oz
94
What portion of the small intestine is responsible for protein, fat and carbohydrate absoprtion as well as iron, calcium, and magnesium
jejunum
95
What portion of the small intestine is responsible for uptake of Vit b12, release of neurologically important hormones, and absorption of bile salts, fats, and fat solube vitamins and zinc?
ileum
96
True or false. The ileum has the ability to compensate for some of the functions of the jejunum
TRUE
97
What hormone plays the largest role in fetal growth?
IGF-2
98
What hormone plays the biggest role in placental regulation of fetal nutrient supply?
placental lactogen
99
What fatty acid has been found to play a role in brain and retinal development?
long chain poly unsaturated fatty acids
100
What are contraindications to breastfeeding?
maternal HIV, mother with active HSV lesion on the breast, symptomatic mother with a pos PPD and CXR (presumed active TB), and active breast abscess
101
What are relative contraindications to breastfeeding?
mother who is CMV sero-positive with preterm infant; maternal oral HSV lesions (mother to cover lesions)
102
What are indications for dialysis in a newborn?
Acidosis (metabolic), electrolyte issues (hyperkalemia, hyperphosphatemia, hypocalcemia, hyponatremia with volume overload), inability to provide adequate nutrition, overload with fluids, uremia. Notice NOT elevated Cr but that will often coincide with these indications
103
compared to LGA infants, SGA infant have an decrease in these 3 body composition metrics
reduced total body mass, lean mass, and bone mineral content and therefore less nitrogen
104
Preterm neonates loss weight in the first several days of life. What happens to TBW, ECF, and ICF?
TBW and ECF decreases and specifically the interstitial compartment of the ECF decreases; ICF increases
105
What are the main glucogenic amino acids?
alanine, glycine, glutatmate. Others include aspartate, cysteine, and proline
106
What are the amino acids that are both glucogenic and ketohgenic?
isoluecine, phenylalanine, tyrptophan, tyrosine
107
What amino acids are ketogenic?
leucine and lysine
