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Nutrition and Nutritional Disorders

♦ Normal Nutritional Requirements

       •   Age related requirements

               o   Recognize problems associated with early eating of solid foods

                          Most likely consequence is increased likelihood of GI infections

                          No definitive evidence that early intro of solid foods leads to more respiratory
                           infections, asthma, OM

                          No evidence supports hypothesis that intro of solid foods accelerates development of
                           oral motor skills or helps infants sleep through the night

                          Conflicting evidence regarding early intro of solid foods and development of allergies

                          Inconclusive results of obesity studies

                          Appropriate timing for intro of solid foods depends on development of neuromuscular
                           function and GI maturation

                               ∗      Loss of extrusion reflex and ability to swallow non-liquid foods

               o   Know age related changes in the ability to absorb and digest different nutrients relevant to
                   infant feeding

                          Pancreatic maturity not achieved at birth, takes at least 4 post natal months. Until then
                           salivary gland amylase can achieve a considerable amount of complex carbohydrate
                           digestion. Adding extra starch (i.e. though addition of cereals) may result in increased
                           incomplete starch digestion leading to higher starch load in the intestine and therefore
                           higher bacterial proliferation / gassiness

                          Lactase concentrations reach mature values in the small intestine by 36 weeks GA in
                           healthy infants

               o   Identify what dietary practices place infants at risk for nutritional deficiencies

                          Vegan diet – vitamin B12 deficiency

                          Goat milk – folate deficiency

                          Vegetarians have lower iron stores but no increase in Fe deficiency anemia compared
                           with the general population

               o   Recognize that full term neonates have adequate Fe stores

               o   Know that Fe deficiency anemia is the major nutritional deficiency of American youths and
                   identify the signs and sx associated with this disorder

                          Mild to moderate: usually have no sx and normal PE findings
   Moderate and worse: increasing sx of fatigue, exercise intolerance, tachycardia, poor
            growth, splenomegaly. Also associated with blue sclera, koilonychias, angular stomatitis

           In infancy and early childhood is associated with developmental delays and behavioral
            disturbances; increased susceptibility to infection, pica, increased GI lead absorption

o   Judge the nutritional adequacy of infant formulas in relation to mineral content

o   Know the primary minerals that contribute to the solute load of infant formulas

o   Know the problems associated with inadequate and excessive amounts of phosphorous in the
    diet of the premature infant

           Inadequate = demineralization of bone and metabolic bone disease (osteopenia,
            neonatal rickets)

                ∗   Typically presents after 4 weeks of TPN, often accompanied by normal serum P
                    and Ca concentrations and elevated alk phos activity

           Excessive = uncommon in preterm infants; may result in hypocalcemia, tetany, seizure
            activty

o   Understand the rationales for the use of iron fortified formulas and recognize the misuses of low
    iron formulas

o   Understand the necessity of adequate Ca and phos intake in children and adolescents

           If osteopenia or hypovitaminosis D the initial tx involves Ca supplementation of
            600-1200mg/day and vitamin D of at least 800 IU/day

           Risk factors include living in higher latitudes, inactivity, steroid tx, drinking soda
            (phosphoric acid), and any h/o bone fx

           If suspected check a Ca, PO4, 25-hydroxyvitamin D, PTH concentration and bone mineral
            density

o   Know the absorption, storage and metabolism of fat soluble vitamins (ADEK)

           Vitamin A

                ∗   Absorption: intestinal cell

                ∗   Storage: liver

                ∗   Metabolism: esters must be hydrolyzed to retinol, complexed with retinal-
                    binding protein and then transported to tissues and organs

           Vitamin D

                ∗   Absorption: D2 and D3 are absorbed in the small intestine

                ∗   Storage and Metabolism: D3 is hydroxylated to 25 hydroxyl-D3 in the liver and
then further hydroxylated to 1,25-dihydroxy-D3 (this is the physiologically active
                    from that regulates Ca and PO4 metabolism

                ∗   Metabolism: above

           Vitamin E

                ∗   Absorption:

                ∗   Storage

                ∗   Metabolism

           Vitamin K

                ∗   Absorption: jejunum; comes from the diet or from production by intestinal
                    bacteria

                ∗   Storage: either used rapidly or metabolized

                ∗   Metabolism

o   Understand the necessity of adequate vitamin D intake in children and adolescents

           Risk factors include living in higher latitudes, inactivity, steroid tx, drinking soda
            (phosphoric acid), decreased milk consumption, African American race and any h/o
            bone fx

           All infants should receive a minimum of 200IU/day after age 2 months

           If baby does not get at least 500ml/d of formula or milk they should also be
            supplemented

           Nutritional rickets is treated with daily oral vitamin D3 (cholecalciferol); or 15000
            mcg/day of vitamin D; phosphate levels will increase as early as 4 days and radiologic
            evidence of healing can occur in 1-2 weeks

o   Recognize the importance of the quality of fat in preterm and full term infants formulas

           Preterm infant formulas need to have higher concentrations of medium chain
            triglycerides because of the pretem infant’s decreased ability to digest fats; the MCT can
            be absorbed by a lipase and bile acid independent pathway

o   Recognize the difference in preterm and full term infant’s ability to digest fat and absorb fat
    soluble vitamins

           Preterm infants have reduced pancreatic lipase, decreased enterohepatic circulation of
            bile acids, decreased lipase activity

           Notably human milk contains bile salt stimulated lipase, which is activated in the
            duodenum and aids in infant digestion
o    Recognize that preterm infants may have decreased amounts of intraluminal bile acids and
                  decreased absorption of long chain triglycerides and fat soluble vitamins

             o    Know the protein requirements for full and preterm infants

                         Full term infants need 2.5 to 3 g/kg/day of protein

                         Preterm infants need at leas 3-4g/kg/day of protein

             o    Understand the appropriate age at which cow milk should be introduced into the diet

                         Whole cow’s milk can be added at 12 months of age

                         Adding cow milk can cause increase fecal blood loss in some infants

                         Cow milk has higher content of protein and electrolytes (K and Na) that causes a higher
                          renal solute load that is too high for the infant kidney

                         Iron fortified formula is preferred; it contains 10-12 mg/L of Fe and this is adequate for
                          the first 4-6 months of age, after which iron fortified foods or supplements should be
                          added

                              ∗     Babies iron stores are depleted by this age so they need more given to them

                              ∗     The iron in formula is only 4% absorbed

                              ∗     Iron in breastmilk is less, but has higher bioavailability and therefore better
                                    absorbed

             o    Know the caloric requirements for infants, children and adolescents

                         Infants

                         Children

                         Adolescents

             o    Know the various circumstances in which the caloric requirements of preterm infants vary

                         Higher needs when lower birth weights due to decreased body fat, increased metabolic
                          demands; also higher needs if they have wounds, are ill, etc



♦ Breastfeeding

      •   Recognize that human and cow milk proteins differ in quality and quantity

             o    Human milk is predominantly whey protein (whey:casein = 75:25)

             o    Formula is primarily cow milk protein, higher casein (whey:casein = 22:78)

             o    Human milk also has free amino acids, including essential amino acids, as well as nucelotides
•   Know that human milk contains antibodies against certain bacteria and viruses, including high
    concentrations of secretory IgA antibodies

        o   C. jejuni, c. diff, e. coli, g. lamblia, rotavirus, salmonella typhimurium, shigella sp and vibrio
            cholerae; also h. flu, s. pneumo and RSV

•   Know that ingested antibodies from human colostrum and milk provide local GI immunity against
    organisms entering the body via this route

        o   See above for specifics

•   Understand that the low vitamin K content of human milk may contribute to hemorrhagic disease of the
    newborn infant

        o   By a few days after birth the enteric bacteria can make enough vitamin K, but not until ingestion
            of copious amounts of human milk can promote GI colonization

•   Know the drugs that are contraindicated in breastfeeding

        o   Antineoplastic agents, immunosuppressants, lithium, chloramphenicol, ergot alkaloids,
            radiopharmaceuticals, bromocriptine, iodides

•   Know that maternal ingestion of drugs with sedative properties has the potential to cause sedation in
    breastfeeding infants

•   Understand the qualitative and quantitative differences between human milk and infant formulas

        o   Human milk has higher amounts of lactose; corn syrup is used to add carbs to formula

        o   Protein as in above topic

        o   Human milk has human milk fat with more absorbable TGL; formula has more volatile fatty acids
            and uses soy, cocunut, palm oils

        o   Less iron in human milk, but presence of vitamin C and lactose facilitate its absorption

•   Know that there is a lower incidence of GI infections in infants fed human milk

•   Know the disorders of the breast that interfere with breastfeeding

        o   h/o breast reduction are at risk of insufficient lactation

        o   breast cancer does not disallow breastfeeding, but use of chemo agents does

        o   inverted nipples should not impede breastfeeding

        o   women with acute mastitis should continue breastfeeding

•   Plan the management of a preterm infant with respiratory distress syndrome whose mother wants to
    breast feed her infant

•   Disease and breastfeeding:
o   Contraindicated in women with HIV-1 and 2, HTLV

               o   NOT contraindicated in women with HBV, HCV, HSV (unless active breast lesions), CMV, rubella,
                   west nile

                          If mom has HBV then baby needs to get HBIG and HBV vaccine within 12 hours after
                           delivery regardless of breastfeeding plans

♦ Formula Feeding

       •   Know which infant formulas contain lactose

               o   Cow milk based

       •   Recognize that infants fed goat milk exclusively are prone to megaloblastic anemia due to folate
           deficiency

       •   Know the indications for use of protein hydrolysate formulas

               o   Infants who have food allergies or underlying GI damage

       •   Recognize soy as a potential allergen in GI protein allergy

               o   Up to 30% of babies with a milk protein allergy will have a soy protein allergy (crossreactivity)

       •   Understand the nutritional supplements that can be sued to increase caloric density of formulas and
           their risks

               o   The formula itself can be concentrated to 24-26 cal/oz and be fairly well tolerated with an
                   acceptable solute load

               o   To further increase the caloric density then macronutrient additives may be needed like MCT oil,
                   polycose

       •   Understand the difference between milk protein allergy and lactose intolerance

               o   Lactose intolerance is due to lactase deficiency; in affected children the lactase activity often
                   does not decline to clinically significant levels until after the 6th year

               o   Milk protein allergy is an IgE-mediated reaction that usually develops within the first post natal
                   year. Sx include urticaria, angioedema, atopic dermatitis, anaphylaxis

               o   Milk protein enterocolitis (gastrointestinal protein allergy) is non-IgE mediated and presents
                   with hematochezia within the first few postnatal months

                          These babies often experience the probs with soy based formulas, too, and often need
                           an elemental or amino acid based formula

♦ Vitamin Deficiency States

       •   Know that rickets may develop in rapidly growing premature infants with low intake of either calcium or
           phosphorous
•   Recognize the effects of vitamin D deficiency in children of various ages, including breastfed infants and
    older children

        o   Infants: present with seizures and tetany due to hypocalcemia, hypotonia, FTT, widened cranial
            sutures, frontal bossing, craniotabes

        o   Older children: delayed milestones, potbelly, bowlegs, kyphosis, pelvic deformities, delayed
            dentition, widened wrists, rachitic rosary, Harrison groove (horizontal depression along the
            lower border of the chest at the costal insertion of the diaphragm)

        o   Adolescents and adults: osteomalacia

•   Recognize the clinical manifestations of vitamin K deficiency

        o   Usually seen in newborns with hemorrhagic events

        o   Vit K deficiency leads to absence of vitamin K dependent clotting factors (II, VII, IX and X)

        o   More common in breastfed infants because of low vitamin K content of breastmilk

        o   Can be exaggerated in preterm infants who will present with spontaneous and prolonged
            bleeding between the 2nd and 7th postnatal days

        o   Bleeding is usually GI, nasal, intracranial, or from the circumcision site. Late bleeding can occur
            even several weeks later

        o   Babies need to get a shot of 1mg of vitamin K at delivery

        o   Look for clinical vignette of a baby born at home, breastfed

•   Recognize the presenting signs and symptoms of rickets and manage appropriately

        o   See above for age related findings of rickets

        o   Vitamin D deficient rickets:

                   Decreased calcium and phos, increased alk phos and PTH and normal/decreased 1,25-
                    dihydroxy D3

        o   Vitamin D dependent rickets (2 types)

                   Both have decreased calcium and phos, increased alk phos and PTH but type 1 has low
                    and type 2 has high 1,25 dihydroxy D3;

        o   Vitamin D resistant rickets (X-linked familial hypophosphatemia)

                   Due to a defect in the renal tubular reabsorption of phosphate. Calcium is normal, phos
                    is low. Alk phos is increased and PTH is normal with normal/decreased 1,25 dihydroxy
                    D3

                   Clinically, the kid will have bowed legs but other features of nutritional rickets will be
                    absent
•   Plan the treatment of a patient with rickets

        o   Prevention: All infants should get 200 IU/day of vitamin D beginning during the first 2 postnatal
            months; if baby gets formula then doesn’t need supplementation as long as getting at least
            500ml/day. Preterm babies need 400 IU/day

        o   Also need to correct calcium and phos levels

        o   Once dxed treatment id vitamin D3 150-250 mcg (5000-10000 IU) daily for 2-3 months or until
            healing is complete, then reduce to 10 mcg daily. Alternatively a single dose of 15000mcg
            (600,000 IU) can be given orally or parenterally

•   Recognize that a child with a resected terminal ileum and ileal inflammation requires appropriate B12
    supplementation and plan the regimen

        o   B12 = cobalamine

        o   Need to have intrinsic factor to combine with the vitamin in the stomach for it to be absorbed in
            the terminal ileum

        o   Dietary sources are fish, eggs and cheese; therefore strict vegan diets can cause deficiency

        o   Deficiency of IF leads to pernicious anemia; have irritability, anorexia and a painful red tongue,
            can eventually get ataxia, decreased reflexes, clonus and coma

        o   Older kids can develop B12 deficiency when they have lack of hepatic stores following short
            bowel syndrome or damage to the terminal ileum (i.e. Crohn’s)

        o   Present with dx features of megaloblastic macrocytic anemia, hypersegmented neutrophils, ligh
            LDH, excessive MMA in the urine; Schilling test looks for absorption

        o   Treatment is 100mcg/day IM for 2 weeks, then qweek until hct normalizes, then 60mcg q month
            for maintenance

•   Know that folate deficiency may develop in children with malabsorption syndrome

        o   Clinical signs would be megaloblastic anemia, irritability and failure to gain weight; they;d have
            low serum and RBC folate levels, elevated serum homocysteine and normal MMA

        o   Treatment is 15mcg/kg PO or IM for infants; 1mg/day followed by 0.1mg/day until recovery for
            1-13y/o; 1mg/d for over 13 y/o

•   Know the lab and radiologic findings in children with vit D deficiency rickets

        o   See above for lab

        o   Radiologic findings : knees, wrists and shoulders showed widened distal ends with cupping and
            fraying, uncalcified larger metaphyses, and osteopenia. A line of preparatory calcification that is
            separated from the distal end of the shaft by a zone of decreased calcification suggests initial
            healing
•   Know the signs, sx and causes of vitamin E deficiency

               o   Causes: malabsorption, abetalipoproteinemia, short bowel syndrome, cholstatis liver dz, VLBX
                   infants can become deficient

               o   Si/Sx: muscle weakness, double vision, loss of position sense, hemolytic anemia, reduced
                   reflexes and constricted visual fields

               o   Vit Eis an important antioxidant and helps protect cell membrane damage

               o   Tx: Adding 0.7mg/g of unsaturated fat in the diet is adequate to tx deficiency; if malabsorption is
                   the problem then may need supplements

       •   Know the nutritional complications associated with a strict vegan diet

               o   B12 deficiency as above

♦ Mineral Deficiency States

       •   Know the diseases that are associated with trace mineral deficiency (zinc, copper, magnesium,
           chromium)

               o   Zinc deficiency leads to growth retardation, an eczematous, vesiculbullous and pustular rash
                   over the perioral, perianal and acral areas; alopecia, diarrhea, secondary bacterial and fungal
                   infections and neurologic, behavioral and cognitive impairment. Can result from a zinc deficient
                   diet, malabsorption, increased losses; oral zinc tx reverses most si/sx regardless of cause.

               o   Copper deficiency causes microcytic anemia, osteoporosis, neutropenia, neurologic sx and loss
                   of pigmentation of skin and hair

               o   Magnesium deficiency can occur as part of a refeeding syndrome with protein energy
                   malnutrition; renal disease, malabsorption, or magnesium wasting medications may lead to
                   depletion; can cause a secondary hypoCa and HypoK

               o   Chromium deficiency causes impaired glucose tolerance, peripheral neuropathy and
                   encephalopathy

♦ Protein, calorie deficiency states

       •   Recognize the clinical manifestations of protein deficiency, including edema and malnutrition

               o   Irritability, mild growth failure, developmental delay and edema of the extremities are hallmarks
                   of kwashiorkor

               o   Affected children often have a normsl or near normal weight and height for age; may also have
                   distended abdomens with hepatomegaly and neurologic, hematologic, or immunologic dysfxn

               o   Hair shaft is often frail and depigmented, if they have had intermittient “nutritional rescues”
                   they will have alternating bands of normal pigmentation (“flag sign”)

               o   Pathognomic rash is desquamating areas with hyperkeratosis with a “peeling paint” appearance
o   This is the most common PEM in developing countries and in hospitalized kids in the US

               o   These kids usually have been weaned from breastmilk to a diet rich in carbs and low in proteins

       •   Recognize that marasmus is caused by inadequate caloric intake

               o   Affected children are rvenous, have decreased weight for height, little subQ fat, dry skin, severe
                   constipation, emaciated appearance without edema

       •   Know the causes of protein losing enteropathy

               o   Cardiovascular dysfxn with secondary intestinal lymphangiectasia or a primary intestinal
                   lymphangiectasia

                          Can measure a fecal alpha 1 antitrypsin level, which is a sensitive and specific marker for
                           intestinal protein loss

               o   GI malabsorption

               o   Inflammatory states

♦ Hypervitaminosis

       •   Recognize the signs and symptoms of hypervitaminosis D

               o   Hypotonia, anorexia, polydipsia, polyuria, dehydration, htn, dorneal clouding; xrays show
                   internal calcifications like kidney stones

♦ Principles of Nutritional Support

       •   Understand the differences among categories of formula used for special nutritional support and
           indications for their use

               o   Soy Protein based formula is used for infants with lactose malabsorption (due to hereditary
                   lactase deficiency or following gastroenteritis) and galactosemia; can be used for those with
                   cow’s milk protein allergy, but there is a 30% cross reactivity

               o   Protein hydrolysate formulas are for infants who have food allergies or underlying GI damage
                   and for infants who can’t tolerate cow’s milk and soy protein

       •   Know the advantages of enteral nutrition over parenteral nutrition

               o   Can provide essential fluids and nutrients when the GI tract can’ t be used to maintain needs for
                   extended periods

       •   Know the indications for total or peripheral alimentation

               o   When it is likely needed for 7 or fewer days, a peripheral route can be used

               o   When needed for more than 7 days or in instances when access is an issue then a CVC needs to
                   be used
•   Know the complications of and understand how to monitor parenteral nutrition

               o   When using a peripheral vein do not exceed 12.5% dextrose because it can cause sclerosis of the
                   vessel

               o   Protein (amino acid) should be kept to about 1g/kg/day; adding more might cause excessive
                   protein consumption and increased risk for hyperammonemia and azotemia

               o   Lipid infusion need to start at a lower that targeted amount in order to allow metabolic
                   adaptation and prevent hypertrigluceridemia

♦ Nutritional Problems Associated with Specific Diseases, Conditions

       •   GI disorders

               o   Recognize that secondary lactose intolerance may be caused by acute gastroenteritis

               o   Understand the importance of early refeeding on the nutritional status of a child with
                   gastroenteritis

                            Once the fluid imbalances are addressed then a regular diet should be started in order
                             to ensure adequate caloric and nutrient intake

                            A period without GI intake is unnecessary and may delay nutritional recovery

                            High sugar containing liquids should be avoided because they can increase the osmotic
                             load and worsen the diarrhea

               o   Know the particular nutritional deficiencies associated with Crohn disease

       •   Renal disease

               o   Recognize the importance of adequate nutrition on growth in children with renal insufficiency

       •   Hepatic disease

               o   Understand the causes of growth failure in children with chronic cholestatic disease

                            Cholestasis leads to malabsorption of nutrients, malabsorption of fat soluble vitamins,
                             malabsorption of fat leading to caloric deficiency

               o   Know the general dietary recommendations in hepatic disease

                            Close monitoring of nutritional status, caloric supplements of indicated,
                             supplementation of fat soluble vitamins

               o   Understand the consequences of hepatic disease on nutrient digestion and absorption

                            Hepatic disease can cause bile problems leading to chronic cholestasis can lead to the
                             above problems related to fat and fat soluble vitamin malabsorption

               o   Understand the mechanism of rickets in children with hepatic disease
    Due to vitamin D malabsorption

         o   Recognize that rickets occurs in children with hepatic disorders

•   Cardiac disease

         o   Know the importance of increasing caloric intake in patients with fluid restricted diets

                     Especially babies with cardiac disease will have increased energy expenditures with
                      feedings

                     Feeds can be made more calorie dense (i.e. 24cal/oz) which will give more calories but
                      not increase volume or lead to volume overload

                     Notably babies with CHD often need 110-120 cal/kg/day

•   CF

•   Heme-Onc Dz

         o   Recognize the importance of adequate nutritional intake in children with chronic disease,
             including malignancies

                     Solid tumors (Wilms, neuroblastoma, Ewing) are higher risk of malnutrition

                     Causes include decreased intake due to anorexia, nausea, mucositis and also increased
                      losses like diarrhea, vomiting and malabsorption

                     Also have increased energy requirements, possibly due to tumor factors/hormonal
                      factors

                     Should use arm circumference and skin fold anthropometrics rather than actual weights
                      in kids with solid tumors b/c may not reflect a “true” weight

                     Kids with adequate nutrition have improved survival from their malignancies, better
                      linear growth, better immune function, less susceptibility to infections during tx

•   Neurologically handicapped children

         o   Understand the prevalence, role, and treatment of GER in neurologically impaired children

                 

•   Burns

         o   Know the nutrients required for wound healing

         o   Recognize the nutritional problems in children with burns

•   Allergies

         o   Recognize that artificial flavors and colors have been implicated in causing urticaria and
             angioedema
o   Recognize the effects of restricted diet for multiple food allergies on the nutritional adequacy of
                    a child’s diet

                           Can end up being deficient in nutrients, i.e. calcium and vitamin D if a milk allergy or
                            lactose intolerance

♦ Obesity

      •     Know that patients with moderate exogenous obesity are generally tall for age and that patients with
            endocrine causes of obesity are small for age

      •     Understand the genetic risk factors for obesity

                o   Mutations in several genes like (leptin, leptin receptor, neuropeptide Y, and others) have been
                    linked to obesity, most forms of obesity are likely polygenic

      •     Know the complications of obesity: htn, DM2, metabolic syndrome, PCOS, dyslipidemia

      •     Know that obese adolescents are at risk for DM2

      •     Understand the ddx of obesity

      •     Know how to monitor and manage obesity and understand the importance of early intervention

      •     Know that obesity in adolescence and parental obesity are strong predictors of obesity in adulthood and
            understand the associated morbidity

      •     Know which interventions have been effective and ineffective in managing adolescent obesity

      •     Know the available limitations of treatment for obesity

      •     Understand the lifestyle choices that may contribute to obesity including inadequate physical activity
            and excessive “screen time”

                o   A 2% increase in the prevalence of obesity has been documented for each extra hour of TV
                    viewing in 12-17 yr olds

                o   Food choice and availability, parental education, poverty, parental obesity, decreases in PE
                    activities

      •     Understand the importance of BMI in identifying obesity

                o   Per the CDC obesity is >95th percentile BMI

                o   BMI in the 85-95th percentile considered overweight

                o   If >99th percentile, then severely obese

♦ Weight loss

      •     Recognize the possible adverse effects of fad weight loss diets
Nutrition and nutritional disorders

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Nutrition and nutritional disorders

  • 1. Nutrition and Nutritional Disorders ♦ Normal Nutritional Requirements • Age related requirements o Recognize problems associated with early eating of solid foods  Most likely consequence is increased likelihood of GI infections  No definitive evidence that early intro of solid foods leads to more respiratory infections, asthma, OM  No evidence supports hypothesis that intro of solid foods accelerates development of oral motor skills or helps infants sleep through the night  Conflicting evidence regarding early intro of solid foods and development of allergies  Inconclusive results of obesity studies  Appropriate timing for intro of solid foods depends on development of neuromuscular function and GI maturation ∗ Loss of extrusion reflex and ability to swallow non-liquid foods o Know age related changes in the ability to absorb and digest different nutrients relevant to infant feeding  Pancreatic maturity not achieved at birth, takes at least 4 post natal months. Until then salivary gland amylase can achieve a considerable amount of complex carbohydrate digestion. Adding extra starch (i.e. though addition of cereals) may result in increased incomplete starch digestion leading to higher starch load in the intestine and therefore higher bacterial proliferation / gassiness  Lactase concentrations reach mature values in the small intestine by 36 weeks GA in healthy infants o Identify what dietary practices place infants at risk for nutritional deficiencies  Vegan diet – vitamin B12 deficiency  Goat milk – folate deficiency  Vegetarians have lower iron stores but no increase in Fe deficiency anemia compared with the general population o Recognize that full term neonates have adequate Fe stores o Know that Fe deficiency anemia is the major nutritional deficiency of American youths and identify the signs and sx associated with this disorder  Mild to moderate: usually have no sx and normal PE findings
  • 2. Moderate and worse: increasing sx of fatigue, exercise intolerance, tachycardia, poor growth, splenomegaly. Also associated with blue sclera, koilonychias, angular stomatitis  In infancy and early childhood is associated with developmental delays and behavioral disturbances; increased susceptibility to infection, pica, increased GI lead absorption o Judge the nutritional adequacy of infant formulas in relation to mineral content o Know the primary minerals that contribute to the solute load of infant formulas o Know the problems associated with inadequate and excessive amounts of phosphorous in the diet of the premature infant  Inadequate = demineralization of bone and metabolic bone disease (osteopenia, neonatal rickets) ∗ Typically presents after 4 weeks of TPN, often accompanied by normal serum P and Ca concentrations and elevated alk phos activity  Excessive = uncommon in preterm infants; may result in hypocalcemia, tetany, seizure activty o Understand the rationales for the use of iron fortified formulas and recognize the misuses of low iron formulas o Understand the necessity of adequate Ca and phos intake in children and adolescents  If osteopenia or hypovitaminosis D the initial tx involves Ca supplementation of 600-1200mg/day and vitamin D of at least 800 IU/day  Risk factors include living in higher latitudes, inactivity, steroid tx, drinking soda (phosphoric acid), and any h/o bone fx  If suspected check a Ca, PO4, 25-hydroxyvitamin D, PTH concentration and bone mineral density o Know the absorption, storage and metabolism of fat soluble vitamins (ADEK)  Vitamin A ∗ Absorption: intestinal cell ∗ Storage: liver ∗ Metabolism: esters must be hydrolyzed to retinol, complexed with retinal- binding protein and then transported to tissues and organs  Vitamin D ∗ Absorption: D2 and D3 are absorbed in the small intestine ∗ Storage and Metabolism: D3 is hydroxylated to 25 hydroxyl-D3 in the liver and
  • 3. then further hydroxylated to 1,25-dihydroxy-D3 (this is the physiologically active from that regulates Ca and PO4 metabolism ∗ Metabolism: above  Vitamin E ∗ Absorption: ∗ Storage ∗ Metabolism  Vitamin K ∗ Absorption: jejunum; comes from the diet or from production by intestinal bacteria ∗ Storage: either used rapidly or metabolized ∗ Metabolism o Understand the necessity of adequate vitamin D intake in children and adolescents  Risk factors include living in higher latitudes, inactivity, steroid tx, drinking soda (phosphoric acid), decreased milk consumption, African American race and any h/o bone fx  All infants should receive a minimum of 200IU/day after age 2 months  If baby does not get at least 500ml/d of formula or milk they should also be supplemented  Nutritional rickets is treated with daily oral vitamin D3 (cholecalciferol); or 15000 mcg/day of vitamin D; phosphate levels will increase as early as 4 days and radiologic evidence of healing can occur in 1-2 weeks o Recognize the importance of the quality of fat in preterm and full term infants formulas  Preterm infant formulas need to have higher concentrations of medium chain triglycerides because of the pretem infant’s decreased ability to digest fats; the MCT can be absorbed by a lipase and bile acid independent pathway o Recognize the difference in preterm and full term infant’s ability to digest fat and absorb fat soluble vitamins  Preterm infants have reduced pancreatic lipase, decreased enterohepatic circulation of bile acids, decreased lipase activity  Notably human milk contains bile salt stimulated lipase, which is activated in the duodenum and aids in infant digestion
  • 4. o Recognize that preterm infants may have decreased amounts of intraluminal bile acids and decreased absorption of long chain triglycerides and fat soluble vitamins o Know the protein requirements for full and preterm infants  Full term infants need 2.5 to 3 g/kg/day of protein  Preterm infants need at leas 3-4g/kg/day of protein o Understand the appropriate age at which cow milk should be introduced into the diet  Whole cow’s milk can be added at 12 months of age  Adding cow milk can cause increase fecal blood loss in some infants  Cow milk has higher content of protein and electrolytes (K and Na) that causes a higher renal solute load that is too high for the infant kidney  Iron fortified formula is preferred; it contains 10-12 mg/L of Fe and this is adequate for the first 4-6 months of age, after which iron fortified foods or supplements should be added ∗ Babies iron stores are depleted by this age so they need more given to them ∗ The iron in formula is only 4% absorbed ∗ Iron in breastmilk is less, but has higher bioavailability and therefore better absorbed o Know the caloric requirements for infants, children and adolescents  Infants  Children  Adolescents o Know the various circumstances in which the caloric requirements of preterm infants vary  Higher needs when lower birth weights due to decreased body fat, increased metabolic demands; also higher needs if they have wounds, are ill, etc ♦ Breastfeeding • Recognize that human and cow milk proteins differ in quality and quantity o Human milk is predominantly whey protein (whey:casein = 75:25) o Formula is primarily cow milk protein, higher casein (whey:casein = 22:78) o Human milk also has free amino acids, including essential amino acids, as well as nucelotides
  • 5. Know that human milk contains antibodies against certain bacteria and viruses, including high concentrations of secretory IgA antibodies o C. jejuni, c. diff, e. coli, g. lamblia, rotavirus, salmonella typhimurium, shigella sp and vibrio cholerae; also h. flu, s. pneumo and RSV • Know that ingested antibodies from human colostrum and milk provide local GI immunity against organisms entering the body via this route o See above for specifics • Understand that the low vitamin K content of human milk may contribute to hemorrhagic disease of the newborn infant o By a few days after birth the enteric bacteria can make enough vitamin K, but not until ingestion of copious amounts of human milk can promote GI colonization • Know the drugs that are contraindicated in breastfeeding o Antineoplastic agents, immunosuppressants, lithium, chloramphenicol, ergot alkaloids, radiopharmaceuticals, bromocriptine, iodides • Know that maternal ingestion of drugs with sedative properties has the potential to cause sedation in breastfeeding infants • Understand the qualitative and quantitative differences between human milk and infant formulas o Human milk has higher amounts of lactose; corn syrup is used to add carbs to formula o Protein as in above topic o Human milk has human milk fat with more absorbable TGL; formula has more volatile fatty acids and uses soy, cocunut, palm oils o Less iron in human milk, but presence of vitamin C and lactose facilitate its absorption • Know that there is a lower incidence of GI infections in infants fed human milk • Know the disorders of the breast that interfere with breastfeeding o h/o breast reduction are at risk of insufficient lactation o breast cancer does not disallow breastfeeding, but use of chemo agents does o inverted nipples should not impede breastfeeding o women with acute mastitis should continue breastfeeding • Plan the management of a preterm infant with respiratory distress syndrome whose mother wants to breast feed her infant • Disease and breastfeeding:
  • 6. o Contraindicated in women with HIV-1 and 2, HTLV o NOT contraindicated in women with HBV, HCV, HSV (unless active breast lesions), CMV, rubella, west nile  If mom has HBV then baby needs to get HBIG and HBV vaccine within 12 hours after delivery regardless of breastfeeding plans ♦ Formula Feeding • Know which infant formulas contain lactose o Cow milk based • Recognize that infants fed goat milk exclusively are prone to megaloblastic anemia due to folate deficiency • Know the indications for use of protein hydrolysate formulas o Infants who have food allergies or underlying GI damage • Recognize soy as a potential allergen in GI protein allergy o Up to 30% of babies with a milk protein allergy will have a soy protein allergy (crossreactivity) • Understand the nutritional supplements that can be sued to increase caloric density of formulas and their risks o The formula itself can be concentrated to 24-26 cal/oz and be fairly well tolerated with an acceptable solute load o To further increase the caloric density then macronutrient additives may be needed like MCT oil, polycose • Understand the difference between milk protein allergy and lactose intolerance o Lactose intolerance is due to lactase deficiency; in affected children the lactase activity often does not decline to clinically significant levels until after the 6th year o Milk protein allergy is an IgE-mediated reaction that usually develops within the first post natal year. Sx include urticaria, angioedema, atopic dermatitis, anaphylaxis o Milk protein enterocolitis (gastrointestinal protein allergy) is non-IgE mediated and presents with hematochezia within the first few postnatal months  These babies often experience the probs with soy based formulas, too, and often need an elemental or amino acid based formula ♦ Vitamin Deficiency States • Know that rickets may develop in rapidly growing premature infants with low intake of either calcium or phosphorous
  • 7. Recognize the effects of vitamin D deficiency in children of various ages, including breastfed infants and older children o Infants: present with seizures and tetany due to hypocalcemia, hypotonia, FTT, widened cranial sutures, frontal bossing, craniotabes o Older children: delayed milestones, potbelly, bowlegs, kyphosis, pelvic deformities, delayed dentition, widened wrists, rachitic rosary, Harrison groove (horizontal depression along the lower border of the chest at the costal insertion of the diaphragm) o Adolescents and adults: osteomalacia • Recognize the clinical manifestations of vitamin K deficiency o Usually seen in newborns with hemorrhagic events o Vit K deficiency leads to absence of vitamin K dependent clotting factors (II, VII, IX and X) o More common in breastfed infants because of low vitamin K content of breastmilk o Can be exaggerated in preterm infants who will present with spontaneous and prolonged bleeding between the 2nd and 7th postnatal days o Bleeding is usually GI, nasal, intracranial, or from the circumcision site. Late bleeding can occur even several weeks later o Babies need to get a shot of 1mg of vitamin K at delivery o Look for clinical vignette of a baby born at home, breastfed • Recognize the presenting signs and symptoms of rickets and manage appropriately o See above for age related findings of rickets o Vitamin D deficient rickets:  Decreased calcium and phos, increased alk phos and PTH and normal/decreased 1,25- dihydroxy D3 o Vitamin D dependent rickets (2 types)  Both have decreased calcium and phos, increased alk phos and PTH but type 1 has low and type 2 has high 1,25 dihydroxy D3; o Vitamin D resistant rickets (X-linked familial hypophosphatemia)  Due to a defect in the renal tubular reabsorption of phosphate. Calcium is normal, phos is low. Alk phos is increased and PTH is normal with normal/decreased 1,25 dihydroxy D3  Clinically, the kid will have bowed legs but other features of nutritional rickets will be absent
  • 8. Plan the treatment of a patient with rickets o Prevention: All infants should get 200 IU/day of vitamin D beginning during the first 2 postnatal months; if baby gets formula then doesn’t need supplementation as long as getting at least 500ml/day. Preterm babies need 400 IU/day o Also need to correct calcium and phos levels o Once dxed treatment id vitamin D3 150-250 mcg (5000-10000 IU) daily for 2-3 months or until healing is complete, then reduce to 10 mcg daily. Alternatively a single dose of 15000mcg (600,000 IU) can be given orally or parenterally • Recognize that a child with a resected terminal ileum and ileal inflammation requires appropriate B12 supplementation and plan the regimen o B12 = cobalamine o Need to have intrinsic factor to combine with the vitamin in the stomach for it to be absorbed in the terminal ileum o Dietary sources are fish, eggs and cheese; therefore strict vegan diets can cause deficiency o Deficiency of IF leads to pernicious anemia; have irritability, anorexia and a painful red tongue, can eventually get ataxia, decreased reflexes, clonus and coma o Older kids can develop B12 deficiency when they have lack of hepatic stores following short bowel syndrome or damage to the terminal ileum (i.e. Crohn’s) o Present with dx features of megaloblastic macrocytic anemia, hypersegmented neutrophils, ligh LDH, excessive MMA in the urine; Schilling test looks for absorption o Treatment is 100mcg/day IM for 2 weeks, then qweek until hct normalizes, then 60mcg q month for maintenance • Know that folate deficiency may develop in children with malabsorption syndrome o Clinical signs would be megaloblastic anemia, irritability and failure to gain weight; they;d have low serum and RBC folate levels, elevated serum homocysteine and normal MMA o Treatment is 15mcg/kg PO or IM for infants; 1mg/day followed by 0.1mg/day until recovery for 1-13y/o; 1mg/d for over 13 y/o • Know the lab and radiologic findings in children with vit D deficiency rickets o See above for lab o Radiologic findings : knees, wrists and shoulders showed widened distal ends with cupping and fraying, uncalcified larger metaphyses, and osteopenia. A line of preparatory calcification that is separated from the distal end of the shaft by a zone of decreased calcification suggests initial healing
  • 9. Know the signs, sx and causes of vitamin E deficiency o Causes: malabsorption, abetalipoproteinemia, short bowel syndrome, cholstatis liver dz, VLBX infants can become deficient o Si/Sx: muscle weakness, double vision, loss of position sense, hemolytic anemia, reduced reflexes and constricted visual fields o Vit Eis an important antioxidant and helps protect cell membrane damage o Tx: Adding 0.7mg/g of unsaturated fat in the diet is adequate to tx deficiency; if malabsorption is the problem then may need supplements • Know the nutritional complications associated with a strict vegan diet o B12 deficiency as above ♦ Mineral Deficiency States • Know the diseases that are associated with trace mineral deficiency (zinc, copper, magnesium, chromium) o Zinc deficiency leads to growth retardation, an eczematous, vesiculbullous and pustular rash over the perioral, perianal and acral areas; alopecia, diarrhea, secondary bacterial and fungal infections and neurologic, behavioral and cognitive impairment. Can result from a zinc deficient diet, malabsorption, increased losses; oral zinc tx reverses most si/sx regardless of cause. o Copper deficiency causes microcytic anemia, osteoporosis, neutropenia, neurologic sx and loss of pigmentation of skin and hair o Magnesium deficiency can occur as part of a refeeding syndrome with protein energy malnutrition; renal disease, malabsorption, or magnesium wasting medications may lead to depletion; can cause a secondary hypoCa and HypoK o Chromium deficiency causes impaired glucose tolerance, peripheral neuropathy and encephalopathy ♦ Protein, calorie deficiency states • Recognize the clinical manifestations of protein deficiency, including edema and malnutrition o Irritability, mild growth failure, developmental delay and edema of the extremities are hallmarks of kwashiorkor o Affected children often have a normsl or near normal weight and height for age; may also have distended abdomens with hepatomegaly and neurologic, hematologic, or immunologic dysfxn o Hair shaft is often frail and depigmented, if they have had intermittient “nutritional rescues” they will have alternating bands of normal pigmentation (“flag sign”) o Pathognomic rash is desquamating areas with hyperkeratosis with a “peeling paint” appearance
  • 10. o This is the most common PEM in developing countries and in hospitalized kids in the US o These kids usually have been weaned from breastmilk to a diet rich in carbs and low in proteins • Recognize that marasmus is caused by inadequate caloric intake o Affected children are rvenous, have decreased weight for height, little subQ fat, dry skin, severe constipation, emaciated appearance without edema • Know the causes of protein losing enteropathy o Cardiovascular dysfxn with secondary intestinal lymphangiectasia or a primary intestinal lymphangiectasia  Can measure a fecal alpha 1 antitrypsin level, which is a sensitive and specific marker for intestinal protein loss o GI malabsorption o Inflammatory states ♦ Hypervitaminosis • Recognize the signs and symptoms of hypervitaminosis D o Hypotonia, anorexia, polydipsia, polyuria, dehydration, htn, dorneal clouding; xrays show internal calcifications like kidney stones ♦ Principles of Nutritional Support • Understand the differences among categories of formula used for special nutritional support and indications for their use o Soy Protein based formula is used for infants with lactose malabsorption (due to hereditary lactase deficiency or following gastroenteritis) and galactosemia; can be used for those with cow’s milk protein allergy, but there is a 30% cross reactivity o Protein hydrolysate formulas are for infants who have food allergies or underlying GI damage and for infants who can’t tolerate cow’s milk and soy protein • Know the advantages of enteral nutrition over parenteral nutrition o Can provide essential fluids and nutrients when the GI tract can’ t be used to maintain needs for extended periods • Know the indications for total or peripheral alimentation o When it is likely needed for 7 or fewer days, a peripheral route can be used o When needed for more than 7 days or in instances when access is an issue then a CVC needs to be used
  • 11. Know the complications of and understand how to monitor parenteral nutrition o When using a peripheral vein do not exceed 12.5% dextrose because it can cause sclerosis of the vessel o Protein (amino acid) should be kept to about 1g/kg/day; adding more might cause excessive protein consumption and increased risk for hyperammonemia and azotemia o Lipid infusion need to start at a lower that targeted amount in order to allow metabolic adaptation and prevent hypertrigluceridemia ♦ Nutritional Problems Associated with Specific Diseases, Conditions • GI disorders o Recognize that secondary lactose intolerance may be caused by acute gastroenteritis o Understand the importance of early refeeding on the nutritional status of a child with gastroenteritis  Once the fluid imbalances are addressed then a regular diet should be started in order to ensure adequate caloric and nutrient intake  A period without GI intake is unnecessary and may delay nutritional recovery  High sugar containing liquids should be avoided because they can increase the osmotic load and worsen the diarrhea o Know the particular nutritional deficiencies associated with Crohn disease • Renal disease o Recognize the importance of adequate nutrition on growth in children with renal insufficiency • Hepatic disease o Understand the causes of growth failure in children with chronic cholestatic disease  Cholestasis leads to malabsorption of nutrients, malabsorption of fat soluble vitamins, malabsorption of fat leading to caloric deficiency o Know the general dietary recommendations in hepatic disease  Close monitoring of nutritional status, caloric supplements of indicated, supplementation of fat soluble vitamins o Understand the consequences of hepatic disease on nutrient digestion and absorption  Hepatic disease can cause bile problems leading to chronic cholestasis can lead to the above problems related to fat and fat soluble vitamin malabsorption o Understand the mechanism of rickets in children with hepatic disease
  • 12. Due to vitamin D malabsorption o Recognize that rickets occurs in children with hepatic disorders • Cardiac disease o Know the importance of increasing caloric intake in patients with fluid restricted diets  Especially babies with cardiac disease will have increased energy expenditures with feedings  Feeds can be made more calorie dense (i.e. 24cal/oz) which will give more calories but not increase volume or lead to volume overload  Notably babies with CHD often need 110-120 cal/kg/day • CF • Heme-Onc Dz o Recognize the importance of adequate nutritional intake in children with chronic disease, including malignancies  Solid tumors (Wilms, neuroblastoma, Ewing) are higher risk of malnutrition  Causes include decreased intake due to anorexia, nausea, mucositis and also increased losses like diarrhea, vomiting and malabsorption  Also have increased energy requirements, possibly due to tumor factors/hormonal factors  Should use arm circumference and skin fold anthropometrics rather than actual weights in kids with solid tumors b/c may not reflect a “true” weight  Kids with adequate nutrition have improved survival from their malignancies, better linear growth, better immune function, less susceptibility to infections during tx • Neurologically handicapped children o Understand the prevalence, role, and treatment of GER in neurologically impaired children  • Burns o Know the nutrients required for wound healing o Recognize the nutritional problems in children with burns • Allergies o Recognize that artificial flavors and colors have been implicated in causing urticaria and angioedema
  • 13. o Recognize the effects of restricted diet for multiple food allergies on the nutritional adequacy of a child’s diet  Can end up being deficient in nutrients, i.e. calcium and vitamin D if a milk allergy or lactose intolerance ♦ Obesity • Know that patients with moderate exogenous obesity are generally tall for age and that patients with endocrine causes of obesity are small for age • Understand the genetic risk factors for obesity o Mutations in several genes like (leptin, leptin receptor, neuropeptide Y, and others) have been linked to obesity, most forms of obesity are likely polygenic • Know the complications of obesity: htn, DM2, metabolic syndrome, PCOS, dyslipidemia • Know that obese adolescents are at risk for DM2 • Understand the ddx of obesity • Know how to monitor and manage obesity and understand the importance of early intervention • Know that obesity in adolescence and parental obesity are strong predictors of obesity in adulthood and understand the associated morbidity • Know which interventions have been effective and ineffective in managing adolescent obesity • Know the available limitations of treatment for obesity • Understand the lifestyle choices that may contribute to obesity including inadequate physical activity and excessive “screen time” o A 2% increase in the prevalence of obesity has been documented for each extra hour of TV viewing in 12-17 yr olds o Food choice and availability, parental education, poverty, parental obesity, decreases in PE activities • Understand the importance of BMI in identifying obesity o Per the CDC obesity is >95th percentile BMI o BMI in the 85-95th percentile considered overweight o If >99th percentile, then severely obese ♦ Weight loss • Recognize the possible adverse effects of fad weight loss diets