Nutrition

Question 1

Useful for assessing the nutritional status of populations, because this measure of skeletal growth reflects the cumulative impact of events affecting nutritional status that result in stunting and is also referred to as chronic malnutrition

Answer 1

Height for age

Question 2

What is the measure of acute malnutrition (wasting)?

Answer 2

weight for height (WFA)

NOT weight for age, which is an additional commonly used measurement of nutritional status (easier to measure, butcombines stature with current health conditions)

In emergencies and in some field settings, mid-upper arm circumference (MUAC) is often used for screening in lieu of weight for height.

Question 3

Type of malnutrition characterized by failure to gain weight and irritability, followed by weight loss and listlessness until emaciation results

Answer 3

Nonedematous malnutrition (marasmus)

Question 4

Syndrome that complicates the acute nutritional rehabilitation of children who are undernourished, marked by the development of severe hypophosphatemia after the cellular uptake of phosphate during the 1st week of starting to refeed

Answer 4

REFEEDING SYNDROME

• Serum phosphate levels of ≤0.5 mmol/L can produce weakness, rhabdomyolysis, neutrophil dysfunction, cardiorespiratory failure, arrhythmias, seizures, altered level of consciousness, or sudden death
• prior to refeeding, get baseline K, Ca, Mg, PO4

Question 5

How is pediatric obesity defined?

Answer 5

for children > 2y/o, obesity is defined as a BMI >= 95th percentile

overweight: BMI between 85-95th percentile

Question 6

For obese children, when should bariatric surgery be considered?

Answer 6

surgery should be considered only in children with complete or near-complete skeletal maturity,
a BMI ≥40, and
a medical complication resulting from obesity, after they have failed 6 mo of a multidisciplinary weight management program

Question 7

Name the vitamin deficiency based on the symptoms below:

night blindness
xerophthalmia
Bitot spots
poor overall growth, diarrhea, susceptibility to infections, anemia, apathy, mental retardation, and increased intracranial pressure, with wide separation of the cranial bones at the sutures

Answer 7

Vitamin A deficiency

night blindness - when vitamin A deficiency is more advanced, due to the absence of retinal in the visual pigment, rhodopsin, of the retina

xerophthalmia - cornea keratinizes and becomes opaque
Bitot spots - conjunctiva keratinizes and develops plaques

Question 8

What are the three useful indicators of marginal vitamin A status?

Answer 8

conjunctival impression cytology
relative dose response
modified relative dose response

Question 9

Patient was brought in with the following symptoms: headache; vomiting; anorexia; dry, itchy desquamating skin; seborrheic cutaneous lesions; fissuring at the corners of the mouth; alopecia and/or coarsening of the hair; bone abnormalities; swelling of the bones; enlargement of the liver and spleen; diplopia; increased intracranial pressure; irritability; stupor; limited motion; and dryness of the mucous membranes.

Radiographs show hyperostosis affecting several long bones, especially in the middle of the shafts

What should you consider?

Answer 9

acute hypervitaminosis A toxicity

Symptoms include nausea, vomiting, and drowsiness; less-common symptoms include diplopia, papilledema, cranial nerve palsies, and other symptoms suggesting pseudotumor cerebri

Question 10

Rare autosomal recessive disorder characterized by megaloblastic anemia, diabetes mellitus, and sensorineural deafness, responding in varying degrees to thiamine treatment

Answer 10

Thiamine-responsive megaloblastic anemia (TRMA) syndrome

Question 11

Patient on a diet consisting nostly of polished rice was brought in for consult due to the ff symptoms: peripheral neuritis (manifesting as tingling, burning, paresthesias of the toes and feet), decreased deep tendon reflexes, loss of vibration sense, tenderness and cramping of the leg muscles, congestive heart failure, and psychic disturbances.

What is your initial impression.

Answer 11

thiamine deficiency (beriberi)

Question 12

Patient has the following complaints: Hoarseness or aphonia, ptosis, muscle atrophy and tenderness of the nerve trunks followed by ataxia, loss of coordination, and loss of deep sensation

What should you consider?

Answer 12

Thiamine deficiency

- Hoarseness or aphonia caused by paralysis of the laryngeal nerve is a characteristic sign

Question 13

What are the two clinical types of thiamine deficiency?

Answer 13

dry (neuritic) type and a wet (cardiac) type

• Many cases of thiamine deficiency show a mixture of the 2 main features and are more properly termed thiamine deficiency with cardiopathy and peripheral neuropathy.

Question 14

What is the classic clinical triad of Wernicke encephalopathy? What is the vitamin deficiency associated with this disorder?

Answer 14

mental status changes, ocular signs, ataxia

Thiamine deficiency (Vitamin B1)

Question 15

What is the most common cause of death in patients with thiamine deficiency?

Answer 15

cardiac involvement

initial signs are slight cyanosis and dyspnea, but tachycardia, enlargement of the liver, loss of consciousness, and convulsions can develop rapidly. The heart, especially the right side, is enlarged and dilated; fatty degeneration of the myocardium is common.

Question 16

What are the biochemical markers diagnostic of thiamine deficiency?

Answer 16

LOW erythrocyte transketolase activity (ETKA) and the HIGH thiamine pyrophosphate effect (TPPE).

Question 17

What are the symptoms of dry beriberi (neuritic type)?

Answer 17

Irritability, peripheral neuritis, muscle tenderness, ataxia

Question 18

What are the symptoms of wet beriberi (cardiac type)?

Answer 18

tachycardia, edema, cardiomegaly, cardiac failure

Question 19

A malnourished child came in with the following symptoms: glossitis, photophobia, lacrimation, corneal vascularization, poor growth, cheilosis

What vitamin deficiency should you suspect?

Answer 19

riboflavin (vitamin b2)

riboflavin deficiency are mainly related to malnourished and malabsorptive states, including GI infections. Treatment with some drugs, such as probenecid, phenothiazine, or oral contraceptives, can also cause the deficiency

clinical features: cheilosis, glossitis, keratitis, conjunctivitis, photophobia, lacrimation, corneal vascularization, and seborrheic dermatitis

diagnosis is based on the clinical features of angular cheilosis in a malnourished child, which responds promptly to riboflavin supplementation

Question 20

Associated vitamin deficiency with the ff symptoms: Pellagra manifesting as diarrhea, symmetric scaly dermatitis in sun-exposed areas, and neurologic symptoms of disorientation and delirium (dermatitis, diarrhea, dementia)

Answer 20

Niacin (vitamin B3) deficiency

• caused by predominantly maize-based diets, anorexia nervosa, carcinoid syndrome

Question 21

Associated vitamin deficiency with the ff symptoms: Irritability, convulsions, hypochromic anemia, failure to thrive, oxaluria

Answer 21

pyridoxine deficiency (vitamin B6)

• usually caused by prolonged treatment with INH, penicillamine, OCPs

Question 22

Associated vitamin deficiency: Scaly periorificial dermatitis, conjunctivitis, alopecia, lethargy, hypotonia and withdrawn behavior, usually caused by consumption of raw eggs for prolonged periods, parenteral nutrition with infusates lacking this vitamin, valproate therapy

Answer 22

biotin deficiency

*Avidin found in raw egg whites acts as a biotin antagonist.

Question 23

Associated vitamin deficiency: irritability, fatigue, numbness, paresthesias (burning feet syndrome), muscle cramps; Isolated deficiency extremely rare in humans

Answer 23

Pantothenic acid (vitamin B5)

Question 24

Associated vitamin deficiency: Megaloblastic anemia, growth retardation, glossitis, neural tube defects in progeny; usually caused by malnutrition, malabsorptive states, malignancies, hemolytic anemias, anticonvulsant therapy

Answer 24

Folic acid deficiency

Question 25

Associated deficiency: Megaloblastic anemia, irritability, developmental delay, developmental regression, involuntary movements, hyperpigmentation; usually caused by vegan diets, malabsorptive states, Crohn disease, intrinsic factor deficiency (pernicious anemia)

Answer 25

Cobalamin (vitamin B12)

Question 26

Pellagra is associated with what vitamin deficiency? Define the classic clinical triad of pellagra

Answer 26

Niacin deficiency (vitamin B3)
dermatitis, diarrhea, dementia

occurs chiefly in populations where corn (maize), a poor source of tryptophan, is the major foodstuff

Question 27

Sharply demarcated lesions around the neck associated with niacin deficiency

Answer 27

Casal necklace

The lesions first appear as symmetric areas of erythema on exposed surfaces, resembling sunburn, and might go unrecognized. The lesions are usually sharply demarcated from the surrounding healthy skin, and their distribution can change frequently.

Question 28

Persons on a strict vegetarian/vegan diet, those who have pernicious anemia or Crohn’s disease and pxs who underwent ileal resection are typically at risk for developing what vitamin deficiency?

Answer 28

Vitamin B12 (Cobalamin)

• Dietary sources of vitamin B12 are almost exclusively from animal foods

Question 29

A disease of growing bone that is due to unmineralized matrix at the growth plates and occurs in children only before fusion of the epiphyses

Answer 29

rickets

• Osteomalacia is present when there is inadequate mineralization of bone osteoid and occurs in children and adults.

Question 30

The following radiographic findings are characteristic of which vitamin deficiency?

• shafts of the long bones have a ground-glass appearance due to trabecular atrophy
• pencil outlining of the diaphysis and epiphysis
• subperiosteal hemorrhages
• white line of Frankel
• Trumerfeld zone
• Pelkan spur

Answer 30

Scurvy (vitamin C deficiency)

white line of Frankel - an irregular but thickened white line at the metaphysis, represents the zone of well-calcified cartilage

Trumerfeld zone- zone of rarefaction, a linear break in the bone that is proximal and parallel to the white line, represents area of debris of broken-down bone trabeculae and connective tissue

Pelkan spur- a lateral prolongation of the white line and may be present at cortical ends.

Question 31

Which vitamin deficiency should be suspected with the following classic clinical signs:

craniotabes
rachitic rosary
Harrison groove
enlargement at the wrists and ankles

Answer 31

Vitamin D deficiency (rickets)

• rachitic rosary - results from the widening of the costochondral junctions
• enlargement at the wrists and ankles - due to growth plate widening
• Harrison groove - horizontal depression along the lower anterior chest, results from pulling of the softened ribs by the diaphragm during inspiration

Question 32

How is vitamin D deficiency diagnosed?

Answer 32

Most are diagnosed based on the presence of classic radiographic abnormalities, supported by PE findings

The child with rickets has metaphyseal fraying and cupping of the distal radius and ulna.

Question 33

What is the most important source of vitamin D?

Answer 33

the skin (cutaneous synthesis)

Question 34

What vitamin deficiency may result in children receiving anticonvulsants such as phenobarbital or phenytoin or antituberculosis medications such as isoniazid or rifampin?

Answer 34

Vitamin D deficiency (rickets)

• these drugs induce cytochrome P450 in the liver, which increases the degradation of vitamin D

Question 35

An autosomal recessive disorder with mutations in the gene encoding renal 1α-hydroxylase, preventing conversion of 25-D into 1,25-D

Answer 35

Vitamin D dependent Rickets Type 1

• since 1α-hydroxylase is not present, 1,25-D cannot be produced, leading to decreased absorption of Ca in the gut

Question 36

Patients with this type of rickets have mutations in the gene encoding the vitamin D receptor, preventing a normal physiologic response to 1,25-D

Answer 36

Vitamin D-dependent rickets, Type 2

Type 1- no enzyme that converts 25-D to 1,25-D, which promotes Ca absorption

Type 2 - receptors for 1,25-D are defective, normal response not attained

Question 37

Which population is particularly at risk for vitamin E deficiency?

Answer 37

Premature infants

• Vitamin E deficiency in premature infants causes thrombocytosis, edema, and hemolysis potentially causing anemia
• risk increased by use of preterm formulas containing high PUFAs and aggressive use of iron, which can lead to further oxidative stress

Question 38

What are the symptoms of vitamin E deficiency?

Answer 38

in patients with prolonged vitamin E deficiency, a severe, progressive neurologic disorder occurs

• symptoms occur only after 1 yr old
• may have cerebellar disease, posterior column dysfunction, and retinal disease
• initial finding : loss of DTRs
• in premature infants, vitamin E deficiency causes hemolysis usually on the 2nd month

Question 39

What vitamin deficiency should be screened in children with unexplained ataxia?

Answer 39

Vitamin E

consider AVED (ataxia with isolated vitamin E deficiency)

• Patients with this disorder are unable to incorporate vitamin E into lipoproteins before their release from the liver, leading to reduced serum levels of vitamin E
• no symptoms of malabsorption
• diagnose with high index of suspicion

Question 40

What are the two types of vitamin K?

Answer 40

Vitamin K1 - phylloquinone (plant based)

Vitamin K2 - menaquinone (produced by intestinal bacteria)

Question 41

What are the classic Gla-containing proteins involved in blood coagulation that are decreased in vitamin K deficiency?

(Gla - carboxyglutamate)

Answer 41

Factors II (prothrombin), VII, IX, X, protein C, S, and Z

Question 42

3 forms of VKDB (vit K dependent bleeding) of the newborn?

Answer 42

1. Early VKDB, formerly classic hemorrhagic disease of the newborn (occurs 1-2 wks of age)
2. Late VKDB - 2-12 wks of age
3. VKDB due to maternal medications - occurs at birth, eg maternal use of phenobarbital, phenytoin, and warfarin may cause bleeding bec it crosses placenta and interferes with vit K function

Question 43

What is the lab parameter being monitored in the correction of vitamin K deficiency?

Answer 43

prothrombin time(PT)

• PT should decrease in the first 6 hrs after treatment and normalize in 24 hrs
• PTT may be prolonged, but may be normal in the early stage of the disease
• PIVKA-II (proteins induced by vitamin K absence) can be measured to detect mild vit K deficiency

Question 44

What is the known effect of dietary selenium deficiency?

Answer 44

Cardiomyopathy (Keshan disease)

Question 45

Menkes disease is associated with what trace element deficiency?

Answer 45

Copper

• due to a mutation in the gene coding for a protein that facilitates intestinal copper absorption
• results in severe copper deficiency

Question 46

Patients with the recessive disorder acrodermatitis enteropathica will need this trace element supplementation

Answer 46

Zinc

• the disorder is secondary to malabsorption if zinc

Question 47

This micronutrient is helpful in shortening the course of diarrhea and helps improve mortality and morbidity

Answer 47

Zinc

• 20 mg/day orally for 14 days
• also reduces the incidence and severity of diarrhea, pneumonia, and possibly malaria

Question 48

Th WHO definition of moderate stunting is measured through what parameter and what cut-off value?

Answer 48

Z-score below median HFA

-3< -2

severe stunting: zscore < -3