CIS Nutrition

Question 1

What BMI is considered underweight?

Answer 1

<18.5

Question 2

What is the normal range for BMI?

Answer 2

18.5-24.9

Question 3

What is considered overweight BMI?

Answer 3

25-29.9

Question 4

Which BMI is considered to be obese?

Answer 4

greater than or equal to 30

Question 5

Obese class I BMI

Answer 5

30-34.9

Question 6

Obese class II BMI

Answer 6

35-39.9

Question 7

Obese class III BMI

Answer 7

Greater than or equal to 40

Question 8

A PE finding of xanthelasma palpebarum and corneal arcus are associated with what??

Answer 8

Elevated cholesterol

Question 9

A PE finding of acanthosis nigricans is associated with what?

Answer 9

Possible elevated blood sugar indicating DM

Question 10

For metabolic syndrome you have to have one of what?

Answer 10

DM, impaired glucose tolerance, impaired fasting glucose and insulin resistance

Question 11

For metabolic syndrome you also have to have two of what?

Answer 11

Blood pressure greater than 140/90 or on BP meds,

Dyslipidemia, central obesity and microalbuminuria

Question 12

Which diseases are cardiac risk factors?

Answer 12

Dyslipidemia, hyperglycemia and HTN

Question 13

Describe the metabolic pathways involved in metabolic syndrome

Answer 13

Peripheral tissues are insulin resistant -> HSL activated -> increased FFAs from adipocytes
Insulin is overproduced
Gluconeogenesis inhibited and FA synthesis stimulated
Increased insulin and insulin resistance increases FA and TAG synthesis in the liver —> fat deposition in the liver —> dyslipidemia

Question 14

What are some general abnormalities suggesting IEM?

Answer 14

Dysmorphic features, deafness, hydrops, self mutilation, abnormal body or urine odor, hepatosplenomegaly and abnormal hair growth

Question 15

Neurologic abnormalities suggesting IEM?

Answer 15

Hypo/hypertonia, coma, persistent lethargy, seizures, not meeting developmental milestones

Question 16

GI abnormalities suggesting IEM?

Answer 16

Poor feeding, recurrent vomiting, jaundice

Question 17

Eye abnormalities suggesting IEM?

Answer 17

Cataracts, cherry red macula, dislocated lens, glaucoma

Question 18

Muscle and joint abnormalities suggesting IEM?

Answer 18

Myopathy and abnormal mobility

Question 19

Describe Hartnup disease

Answer 19

Effects absorption of nonpolar amino acids particularly tryptophan
Treat with a high protein diet
Sx: diarrhea, mood swings, red scaly skin rash usually when exposed to light, photosensitivity, short stature and uncoordinated movements

Question 20

Describe maple syrup urine disease

Answer 20

Sweet smelling urine
Branched chain ketoacid dehydrogenase deficiency (BCAA: leu, Ile, Val)
Treat with low protein diet
Sx: poor feeding, vomiting, lack of energy (lethargy), abnormal movements, delayed development and may lead to seizures, coma or death

Question 21

Describe phenylketonuria (PKU)

Answer 21

Phenylalanine hydroxylase deficiency
Autosomal recessive
Sx: intellectual disorders, seizures, must smelling urine/sweat due to phenylacetate, decreased pigmentation of hair and skin, eczema often accompany the mental retardation in untreated children
Treat with severely decreased protein intake
Phenyllactate and phenylacetate disrupt neurotransmission and block amino acid transport in the brain as well as myelin formation resulting in severe impairment of brain function

Question 22

Describe albinism

Answer 22

Absence or defect of tyrosinase

Sx: sunburns/skin cancer, photophobia, nystagmus and amblyopia

Question 23

Describe the progression seen with PKU

Answer 23

Infants are normal at birth Bc mother metabolizes extra Phe
Usually by 6 months of life severe mental retardation becomes evident
About 1/3 of children are never able to walk and 2/3 cannot talk

Question 24

Describe newborn screenings

Answer 24

Performed between 24-48 hours
Heel stick, hearing screen
Early detection of otherwise devastating diseases
Missouri tests over 70

Question 25

What occurs when galactokinase is defective?

Answer 25

Juvenile cataracts | Decreased conversion of galactose to galactose 1 phosphate by galactokinase

Question 26

Describe what occurs when dihydopterine reductase is defective

Answer 26

Impaired renewal of tetrahydrobiopterin Malignant hyperphenylalaninemia Decreased levels of dopamine, serotonin and folate Sx: psychomotor delay, low muscle tone (hypotonia), seizures, abnormal movements, too much salvation, swallowing difficulties Tx: supplement BH4 and folate No Phe Meds to restore neurotransmitters in the brain

Question 27

Describe what happens when galactosylceramidase is defective

Answer 27

Also called Krabbe’s disease Impairment of the GALC gene Galactosylceramidase hydrolyzes galactosylceramide, an important component of myelin Sx: hypertonia/spasticity, impaired development, optic atrophy and blindness, unexplained fevers, seizures, irritability, eating problems and deafness Tx: anticonvulsant meds to stop seizures, muscle relaxers, PY to help slow deterioration of muscles, OT to help older children with common tasks, bone marrow transplant and cord blood transplant

Question 28

What occurs with GALT deficiency?

Answer 28

Failure to metabolize galactose/lactose into glucose Back up of galactose causes sx Sx: feeding difficulties, lethargy, failure to thrive, jaundice, females have premature ovarian insufficiency, liver damage, abnormal bleeding, sepsis, developmental delay and cataracts Tx: eliminate lactose and galactose from the diet such as dairy products, avocados and sugar beets

Question 29

Describe malnutrition in reference to Kwashiorkor disease

Answer 29

Severe protein deficiency Sufficient calories, but insufficient protein Usually occurs after weaning Sx: edema of hands and feet, light colored skin, thinning hair, distended abdomen, shiny skin, ulcerating dermatoses, anemia, irritable and enlarged fatty liver

Question 30

Describe malnutrition in reference to marasmus

Answer 30

Protein calorie deficiency Sx: emaciated, chronic diarrhea, respiratory infections, intellectual disability, stunted growth, apathy, no energy Weight is less than 62% of expected

Question 31

What are some characteristics of vitamin A deficiency?

Answer 31

Nyctalopia (night blindness), xerophthalmia (failure to produce tears), major player in phototransduction (changing light into electrical signals)

Question 32

What are characteristics of vitamin B1 (thiamine deficiency) deficiency?

Answer 32

Sx: malaise, weight loss, confusion, wet or dry beriberi, and possible Wernicke-Korsakoff syndrome

Question 33

What are sx of wet beriberi?

Answer 33

Fast HR, SOB, difficulty moving the legs and pain

Question 34

What are sx of dry beriberi?

Answer 34

Numbness, confusion, difficulty moving the legs

Question 35

What are characteristics of Wernicke-Korsakoff syndrome?

Answer 35

Vision changes, ataxia, impaired memory

Question 36

What are characteristics of vitamin B12 deficiency?

Answer 36

Sx: fatigue, inflammation of the tongue (decreased taste), numbness/tingling Sign: megaloblastic anemia

Question 37

What are some sx associated with vitamin C deficiency?

Answer 37

Fatigue, SOB, dizziness, pale or yellowish skin, irregular heartbeats, weight loss, numbness or tingling in hands and feet, muscle weakness, personality changes, unsteady movements and mental confusion or forgetfulness

Question 38

What sx are associated with vitamin K deficiency?

Answer 38

Easy bruising, oozing from nose or gums, excessive bleeding from injuries, heavy menstrual periods, bleeding from the GI tract, blood in the urine and/or stool, increased prothrombin time (PT)

Question 39

What sx are associated with vitamin B2 (riboflavin) deficiency?

Answer 39

Stomatitis (inflamed tongue), sore throat, chapped/fissured lips, oily/scaly rashes on the scrotum, vulva, philtrum of the lip and nasolabial folds, itch, watery, blood shot eyes, sensitivity to light and anemia (normocytic, normochromic)

Question 40

What sx are associated with mild vitamin B3 (niacin) deficiency

Answer 40

Indigestion, fatigue, canker sores, vomiting and depression

Question 41

What sx are associated with severe vitamin B3 (niacin) deficiency

Answer 41

Called Pellagra Thick, scaly pigmented rash on skin exposed to sunlight Swollen mouth and tongue, vomiting and diarrhea, HA, apathy, depression, disorientation, memory loss

Question 42

What sx are associated with vitamin B6 (pyridoxine) deficiency?

Answer 42

Microcytic anemia, EEG abnormalities, dermatitis with cheilosis (scaling on the lips and cracks at the corners of the mouth), glossitis, depression, confusion and weakened immune function

Question 43

What sx and risks are associated with vitamin D deficiency?

Answer 43

Sx: subtle to none Risks: increased risk of death from cardiovascular disease, cognitive impairment in older adults, severe asthma in children and cancer

Question 44

Describe vitamin E deficiency

Answer 44

Digestive system requires fat to absorb vitamin E Conditions that impair this: abetaliproprotenemia, Crohn’s disease, cystic fibrosis, AVED (ataxia and vitamin E deficiency) Sx: peripheral neuropathy, ataxia, skeletal myopathy, retinopathy, immune impairment and infertility

Question 45

Normal fasting glucose

Answer 45

70-110

Question 46

Normal total cholesterol

Answer 46

<200

Question 47

Normal HDL

Answer 47

30-75

Question 48

Normal LDL

Answer 48

<130

Question 49

Normal triglycerides

Answer 49

<150