FEN Flashcards
Fatigue, irritability, constipation, cardiac failure.
What deficiency?
B1 = Thiamine
Beriberi
Photophobia, conjunctivitis, FTT, scaling, epiphyseal bone formation, abnormal tooth enamel.
What deficiency?
Vitamin A
A is pointy like teeth
FTT, photophobia, dermatitis, mucositis.
What deficiency?
Associated with:
B2 = Riboflavin
Associated with glutamic aciduria type 1
Dermatitis, mucositis, hypochromic anemia, seizures. What deficiency?
Associated with
B6 = Pyridoxine
Associated with homocystinuria
Alopecia, dermatitis, scaling, seborrhea.
What deficiency?
Associated with
Biotin
Associated with biotinidase deficiency, B-methylcrotonyl glycinuria, propionic acidemia, pyruvate dehydrogenase complex deficiency
Vitamin deficiency: Poor wound healing, bleeding gums.
What deficiency?
Associated with
Vitamin C = Ascorbic acid
Associated with transient tyrosinemia (Tangerines are made of Vitamin C)
Rickets, FTT, tetany.
What deficiency?
Vitamin D
What immunoglobulins are kept and lost during Holder pasteurization?
IgA and G preserved
IgM destroyed
Anemia, osteoporosis, depigmentation of hair and skin, neutropenia, poor weight gain.
What deficiency?
Copper
Critical for production of red blood cells and hemoglobin. Also iron absorption.
Chromium responsible for
Glucose regulation and insulin metabolism
Chromium coats a Hershey’s kiss
Microcytic, hypochromic anemia; FTT.
What deficiency?
Iron deficiency (Vitamin C enhances absorption)
Manganese role
Enzyme activation ie. superoxide dismutase
Normal bone structure
CHO metabolism
Cardiomyopathy. What deficiency?
Selenium
Selena broke your heart
FTT, alopecia, dermatitis, diarrhea. What deficiency?
Zinc –> Acrodermatitis enterohepatica
Commonly perianal dermatitis
Maternal zinc deficiency
IUGR, congenital anomalies
pH in RTA type 1
> 6.5
pH in RTA type 2
<6.5 (can be >6.5 before acidosis is established. Losing bicarb proximally so distal end makes up for it by secreting more H+)
Which type of RTA has hypercalciuria/nephrocalcinosis?
Type 1 (Possibly thought that increased bone breakdown and calcium release to help buffer the extra acid)
When do different disaccharides reach adult levels?
Sucrase, Maltase, isomaltase by 28 weeks
Lactase by 36 weeks
(Carb digestion and absorption adequate because of colonic salvage pathway)
Colonic salvage pathway
Colonic bacteria help ferment malabsorbed CHO to acids which are absorbed in the colon
Colostrum whey to casein ratio
80:20
Mature breast milk whey to casein ratio
55:45
Preterm formula whey to casein ratio
60:40 or 80:20
Effect on ECF, ICF and TBW as gestational age increases
ECF and TBW decrease
ICF increases
When does nephrogenesis start and when is it complete?
5th week;
34-36 weeks
When does urine production begin in the fetus?
10-12 weeks gestation
Urine output at 20 weeks gestation vs term
5 mL/hr –> ~50 mL/hr
How much of amniotic fluid is comprised of urine?
90%
Endothelin effect? Produced by? Stimulated by?
Renal vasoconstrictor
Produced by renal vascular endothelial cells
Stimulated by Ang II, bradykinin, epinephrine, stress
Menkes disease
X-linked recessive disorder; inability of absorption of copper
What is administered with iron to prevent iron induced hemolysis?
Vitamin E (antioxidant)
Hemolytic anemia, reticulocytosis, thrombocytosis, acanthocytosis, neurologic sequelae.
What deficiency?
Vitamin E
Preterm milk has higher __ than term milk
Lower __
Higher protein and electrolytes
Lower lactose
Which is greater in breast milk vs formula? Long chain unsaturated FAs Carnitine Cholesterol DHA Amino acids
Long chain unsaturated FAs- Breast milk Carnitines- Breast milk Cholesterols- Breast milk DHAs- Breast milk Amino acids- Formula
Fat and protein difference in hind and foremilk
Higher fat in hindmilk; Lower CHO; Similar protein
What component of breast milk is the most variable?
Which is most constant?
Triglycerides (vary by GA, time and maternal diet)
Cholesterol (does not vary by maternal diet)
What percentage of calories should protein provide to prevent negative nitrogen balance?
7-15%
What is resting metabolic rate of neonate?
40-60 kcal/kg/day
Energy requirement for enterally vs parenterally fed infants?
Enterally- 100-130 kcal/kg/day
Parenterally- 85-95 kcal/kg/day
Which part of the intestine is responsible for absorption of fat soluble vitamin and zinc?
Ileum
Jejunum is responsible for some fat absorption but not fat soluble vitamins
Proteinuria, hypoproteinemia, hyperlipidemia, edema
Congenital nephrotic syndrome
Finnish type most common
Large placenta + elevated AFP
Congenital nephrotic syndrome
Finnish type
Cataracts, glaucoma, hypotonia, areflexia, mental deficiency, tubular dysfunction, proteinuria, aminoaciduria, cryptorchidism
Lowe syndrome - X-linked recessive ie. oculocerebrorenal syndrome
Elevated AFP and increased nucleotide pyrophosphates in skin fibroblasts
Lowe syndrome- X-linked recessive ie. oculocerebrorenal syndrome
Triangular facies, protruding ears, large eyes with strabismus, drooping mouth
Bartter syndrome
Electrolyte derangements seen in Bartter syndrome
Hypokalemic metabolic alkalosis
Hypercalciuria
What happens when ureteric bud fails to develop?
Renal agenesis
MCC hydronephrosis. First step after birth.
No specific cause, usually resolves before birth.
Do postnatal ultrasound even if resolves before birth (should wait 7-10 days to ensure normal water losses have occurred)
FENa, urine osmolality, urine Na in prerenal vs renal tubular injury
Prerenal: FENa<2%, Uosm: high, UNa: low (dehydration –> increased Na and water reabsorption to try and inc BP)
Renal: FENa>2%, Uosm: low, UNa: high (inappropriate excretion of Na and water –> low urine osmolality and high urine Na)
(explanation pg. 1467 Brodsky)
Which hormone plays the biggest role in fetal growth?
ILGF-2
Which hormone plays the biggest role in placental regulation of fetal nutrient supply?
Placental lactogen
Essential fatty acids
Alpha Linolenic and linoleic acids
Which fatty acids play a role in brain and retinal development?
Long chain polyunsaturated FAs such as omega 6 and 3
DHA and ARA
Contraindications to breastfeeding
HSV lesion on the breast, HIV, active TB, active breast abscess, galactosemia, drugs
How does GFR change with increasing gestational age?
When at adult levels?
Increases
Doubles in first two weeks
Reaches adult levels by 1 year
How do ACS affect insensible water loss?
Decreases IWL at any given age d/t greater skin maturation and better perfusion
Most prevalent fatty acids in human milk
Oleic acid > palmitic acid
Most predominant carbohydrate in breast milk
Lactose
Rapid correction of hyponatremia can lead to
cerebral demyelination
Indications for dialysis
Hyperkalemia Hyponatremia with symptomatic volume overload Hyperphosphatemia metabolic acidosis hypocalcemia uremic symptoms inability to provide nutrition d/t fluid restriction *not creatinine value*
Calories of protein, carbs and fat
4
3.4
9
Hematuria, thrombocytopenia, renal failure, palpable flank mass
Renal vein thrombosis
How do Na, Cl, Ca, phos, Mg, iron change with advancing GA?
Na, Cl decrease
Ca, phos, Mg, iron increase
Liver involvement at birth in ARPKD
~50-60%
MC symptom with ARPKD
Severe hypertension
Which PKD has multi system involvement?
ADPKD
AR only has liver involvement
Where does ADH work?
Late distal tubule and collecting ducts
What is the primary determinant of water excretion in the kidney?
ADH
Side effects of thiazides
HyperGLUC
- glycemia
- lipidemia
- uricemia
- calcemia (vs loops lose calcium)
MC form of PKD
ADPKD
Cilia related disorder
ARPKD
Defect of PKHD1 gene
ARPKD
Scaly dermatitis, alopecia, FTT, increased infection, thrombocytopenia. What deficiency?
Essential fatty acid deficiency (EFAD)
Intralipid includes which FAs?
Omega 6 (linoleum acid) > omega 3 (limolenic acid)
Vitamin D & Calcitriol in relation to calcium prenatally and postnatally
Fetal calcium homeostasis is independent of vitamin D and calcitriol
Postnatally, a deficiency in the above will impair calcium absorption
What happens to calcium levels after birth
Transient drop but they return to normal within 48 hours
Treatment for SIADH
Fluid AND Na restriction
Total body Na is normal, but total body water is elevated, relative hyponatremia
Majority of dietary fat in the form of
Triglycerides
How is calcium transferred from mother to fetus?
Mother to placenta via facilitative diffusion
Placenta to fetus via ATP pump on basal surface of syncytium
Fetus is HYPERcalcemic to mother
Glucogenic amino acids
Alanine, glycine, glutamate
Give rise to glucose or glycogen
Ketogenic amino acids
Leucine and lysine
yield acetyl-CoA or acetoacetyl-coa
Elevated plasma 7-dehydrocholesterol
Smith Lemli opitz syndrome
Triad of Sotos syndrome
Overgrowth, characteristic facial features, learning disabilities and ID
How many kcal/kg/d necessary for proper growth and energy expenditure
105-130 kcal/kg/day
Triene: Tetraene ratio >0.4
EFAD
How maternal glucose transported
facilitated diffusion
Essential Amino acids
Histidine Isoleucine Leucine Lysine Methionine Phenylalanine Threonine Tryptophine Valine "the HILL My Pop TRYd To ski in VAIL"
Conditionally essential amino acids in preterms
Arginine
Cysteine
Taurine
Tyrosine
Intralipid made of
100% soybean oil
Na, Ca, K, Cl, Mg, Phos higher or lower in human vs cow’s milk?
Lower
Osmolarity equation
(Na x 2) + BuN /2.8 + Glucose / 18
When nephrogenesis finished
34-36 weeks
When does fetal urine output begin
9-11 weeks
Peak fetal growth rate
mid 30 weeks (33-35)
Nitrogen intake equation
protein intake (g/day) x 0.16
