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Nutritional Disorders
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Nutritional Disorders
Nutritional Disorders
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Nutritional Disorders

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  • PEM describes certain signs & symptoms in infants & young children which result from a deficiency of calories &/or proteins in their diets.
  • *Wasting : acute, current, short-duration undernutrition, where weight-for-age & weight-for-height are low but height-for-age is normal *Stunting : past, chronic undernutrition, where weight-for-age and height-for-age are low but weight-for-height is normal *Wasting and stunting : acute and chronic or current long-duration undernutrition, where weight-for-age, height-for-age and weight-for-height are all low. This classification makes a distinction between current and past influences on nutritional status. It helps the examiner assess the likelihood that supplementary feeding will markedly improve the nutritional status of the child, and it gives the clinician some clue as to the history of the malnutrition in the patient. It also has advantages for nutritional surveys and surveillance. In general, stunting is more prevalent than wasting worldwide.
  • *Another factor that is attributed to the development of kwashiorkor is aflatoxin poisoning.  Aflatoxins are produced by certain molds, and are ingested in connection with moldy foods.  They can damage the liver DNA and subsequently interfere with the production of serum albumin. 
  • *Thus, it affects many physiologic processes, including reproduction, growth, embryonic and fetal development, and bone development, in addition to respiratory, gastrointestinal, hematopoietic, and immune functions.
  • *The risk of symptomatic vitamin E deficiency increased by the use of formulas that has a high content of PUFAs  high content of PUFAs in RBCs + Fe (increases the production of oxygen radicals)  oxidative stress ** Loss of deep tendon reflexes usually initial finding Subsequent include limb ataxia (intention tremor, dysdiadochokinesia), truncal ataxia (wide-based, unsteady gait), dysarthria, ophthalmoplegia (limited upward gaze), nystagmus, decreased proprioception (positive Romberg test), decreased vibratory sensation, and dysarthria Pigmentary retinopathy may progress to blindness Cognition and behavior may also be affected Myopathy and cardiac arrhythmias less common
  • Thiamine, Riboflavin, Niacin, Pyridoxine are cofactors to enzymes in energy metabolism, hence, deficiencies show up in quickly growing tissues such as epithelium as well as nerve cells that use lots of energy .
  • A block in DNA synthesis due to folate deficiency slows down the maturation of red blood cells, causing production of abnormally large "macrocytic" red blood cells with fragile membranes. The rapid hemolysis of these macrocytes leads to a hemolytic anemia. A macrocytic anemia associated with megaloblastic changes in the bone marrow is fairly characteristic of folate deficiency.
  • *The megaloblastic anemia associated with the B12 deficiency is thought to be due to the effect of B12 on folate metabolism. High levels of supplemental folate can overcome the megaloblastic anemia but not the neurological problems. **The neurological disorders seen in B12 deficiency are due to progressive demyelination of nervous tissue. It has been proposed that the methylmalonyl- CoA which accumulates in B12 deficiency interferes with myelin sheath formation.
  • Transcript

    • 1. NUTRITIONAL DISORDERS
      • Myrna DC San Pedro, MD, FPPS
    • 2. MALNUTRITION
      • A pathological state resulting from
        • Relative or absolute deficiency
        • Or excess of one or more nutrients and
        • Clinically detected only by biochemical, anthropometric or physiological tests
    • 3. Forms of Malnutrition
      • Undernutrition: Marasmus
      • Overnutrition: Obesity, Hypervitaminoses
      • Specific Deficiency: Kwashiorkor, Hypovitaminoses, Mineral Deficiencies
      • Imbalance: Electrolyte Imbalance
    • 4. Etiology
    • 5.  
    • 6.  
    • 7. Protein Energy Malnutrition (PEM)
    • 8. Classification of Undernutrition
      • Gomez Classification
        • Uses weight-for-age measurements
        • Provides prognosis
      • Weight-for-Age% Status
      • 91-100 Normal
      • 76-90 1 st degree
      • 61-75 2 nd degree
      • <60 3 rd degree
    • 9.
      • Wellcome Classification
        • Based on 2 criteria only: weight loss (wt-for-age%) & edema (present or absent)
      • Wt-for-Age% Edema No Edema
      • 80-60 Kwashiorkor Undernutrition
      • <60 Marasmic- Marasmus
      • Kwashiorkor
    • 10.
      • Waterlow Classification
        • Distinguishes between deficits of weight-for-height% (wasting*) & height-for-age% (stunting*)
        • Adopted by WHO
      • N Mild Mod Severe
      • Wt-for-Ht% >90 89-80 79-70 <70
      • Ht-for-Age% >95 95-90 90-80 <80
    • 11. A 6-yr-old boy has an actual weight of 15 kg & height of 105 cm. Compute for the wt-for-ht%. Actual Wt = 15 kg Actual Ht = 105 cm IBW based on actual age (6 yr) = 20 kg IBW based on plotted Ht = 17 kg Wt-for-Ht% = 15/ 17 = 0.88 Wt-for-age% = 15/ 20 = 0.75 Ht-for-age% = 105 / 110 = 0.95
    • 12. Marasmus
      • Common in the 1st year of life
      • Etiology:
      • “ Balanced starvation”
      • Insufficient breastmilk
      • Dilute milk mixture or lack of hygiene
    • 13. Marasmus
      • Clinical Manifestations:
      • Wasting
      • Muscle wasting
      • Growth retardation
      • Mental changes
      • No edema
      • Variable-subnormal temp, slow PR, good appetite, often with diarrhea, etc.
      • Laboratory Data:
      • Serum albumin N
      • Urinary urea/g creatinine N or low
      • Urinary hydroxyproline/g creatinine low, early
      • Serum essential amino acid index N
      • Anemia uncommon
      • Glucose tolerance curves diabetic type
      • K + deficiency present
      • Serum cholesterol low
      • Diminished enzyme activity
      • Bone growth delayed
      • Liver biopsy N or atrophic
    • 14. Marasmus
    • 15. Kwashiorkor
      • Between 1-3 yrs old
      • Etiology:
      • Very low protein but with calories from CHO
      • In places where starchy foods main staple
      • Never exclusively dietary*
    • 16. Kwashiorkor
      • Clinical Manifestations:
      • Diagnostic Signs
        • Edema
        • Muscle wasting
        • Psychomotor changes
      • Common Signs
        • Hair changes
        • Diffuse depigmentation of skin
        • Moonface
        • Anemia
      • Occasional Signs:
        • Flaky-paint rash
        • Noma
        • Hepatomegaly
        • Associated
      • Laboratory:
      • Decreased serum albumin
      • EEG abnormalities
      • Iron & folic acid deficiencies
      • Liver biopsy fatty or fibrosis may occur
    • 17. Kwashiorkor
    • 18. Treatment of PEM
      • Severe PEM is an emergency, 1-3 months hospitalization desirable
      • On admission, treat dehydration, infections & vitamin deficiencies
      • During 1 st wk, the acute phase, stabilize & start feeding as soon as rehydrated
      • When edema subsides, give full-strength feeds of maintenance calories & protein, recovery after 2-3 wk
      • Once full-strength feeds tolerated, start rehabilitation with high energy feeds (150-200 kcal/kg/day), recovery expected within 4-6 wk
    • 19. Prognosis of PEM
      • If severe & early, <6 months old, permanent impairment of physical & mental growth
      • First 48 hours critical, with poor treatment mortality may be >50%
      • Even with thorough treatment, 10% mortality may still occur
      • Some mortality causes: endocrine, cardiac or liver failure, electrolyte imbalance, hypoglycemia & hypothermia
    • 20. Obesity
    • 21. Obesity
      • Definition: Generalized, excessive accumulation of fat in subcutaneous & other tissues
      • Classification according to “desirable” weight standard:
        • Overweight = > 10% IBW or “desirable” weight
        • Obese = > 20% IBW or “desirable” weight
      • The Centers for Disease Control (CDC) avoids using &quot;obesity&quot; instead suggest two levels of overweight:
        • “ At risk&quot; level = BMI 85 th %
        • More severe level = BMI 95 th %
      • The American Obesity Association:
        • Overweight = BMI 85 th % because ~ BMI of 25, overweight for adults
        • Obesity = BMI 95 th % because ~ BMI of 30, obesity in adults
    • 22. Obesity
      • Appears most frequently in the 1 st yr, 5-6 yr & adolescence
      • Etiology:
      • Excessive food intake compared with use
      • Genetic constitution
      • Psychic disturbance
      • Endocrine & metabolic disturbances rare
      • Insufficient exercise or lack of activity
    • 23. Obesity
      • Clinical Manifestations:
      • Fine facial features on a heavy-looking taller child
      • Larger upper arms & thighs
      • Genu valgum common
      • Relatively small hands & fingers tapering
      • Adiposity in mammary regions
      • Pendulous abdomen with striae
      • In boys, external genitalia appears small but actually average in size
      • In girls, external genitalia normal & menarche not delayed
      • Psychologic disturbances common
      • Bone age advanced
    • 24. Obesity
    • 25. Treatment of Obesity
      • 1 st principle: Decrease energy intake
        • Initial med exam to R/O pathological causes
        • 3-day food recall to itemize child’s diet
        • Plan right diet
          • Avoid all sweets, fried foods & fats
          • Limit milk intake to not >2 glasses/day
          • For 10-14 yrs, limit to 1,100-1300 kcal diet for several months
        • Child must be properly motivated & family involvement essential
      • 2 nd principle: Increase energy output
        • Obtain an activity history
        • Increase physical activity
        • Involve in hobbies to prevent boredom
    • 26. Complication of Obesity
      • Pickwickian Syndrome
      • Rare complication of extreme exogenous obesity
      • Severe cardiorespiratory distress & alveolar hypoventilation
      • Includes polycythemia, hypoxemia, cyanosis, CHF & somnolence
      • High O 2 dangerous in cyanosis
      • Weight reduction ASAP & quick
    • 27. The Fat-Soluble Vitamins
    • 28. Vitamin A (Retinol)
      • Active forms: retinol, retinaldehyde & retinoic acid
      • Plants synthesize carotenoids  cleaved to retinol by most animals  stored in liver as retinyl palmitate
      • Retinol, alcohol form of vitamin A: N plasma values = 20–50 μg/dl in infants & 30–225 μg/dL in older children & adults
      • Retinal , the aldehyde form, functions in vision
      • Retinoic acid , the physiologically most important vitamin A metabolite, regulates many genes involved in fundamental biologic activities of cells*
    • 29. Vitamin A (Retinol) Deficiency
      • Functions:
      • Retinal is in photosensitive pigment in both rods (rhodopsin) & cones (iodopsin)
      • Needed in lysosomal membrane stability
      • Involved in keratinization, cornification, bone development & cell growth & reproduction
      • Absence in the diet common by 2-3 yrs old
      • Etiology:
      • Poor fetal storage
      • Poor absorption as in low-fat diet, malabsorption syndromes, etc.
      • Low protein intake resulting in deficient carriers
      • Increased excretion as in cancer & UTI
    • 30. Hypovitaminosis A
      • Eye signs & symptoms
        • Nyctalopia or night blindness, an early symptom  photophobia  pain insensitivity
        • Xerosis conjunctivae, 1 st clinical sign
        • Bitot’s spots
        • Corneal xerosis or xeropthalmia
        • Corneal ulcers
        • Keratomalacia
        • Blindness
    • 31. Hypovitaminosis A
      • Skin signs: Xerosis of the skin & follicular hyperkeratosis or phrynoderma
      • Others: Apathy, physical & mental growth retardation, faulty epiphyseal bone formation, defective teeth enamel & signs of benign increased ICP
      • Diagnosis:
      • Routine PE
      • Dark adaptation test to detect nyctalopia
      • Absorption test for vitamin A
      • Conjunctival impression cytology to evaluate early xeropthalmia
    • 32. Hypovitaminosis A
      • RDA: 1800 IU/day (1 IU vitamin A = 0.3 mcg retinol)
      • Prevention:
      • Pregnant in last trimester be given 5000 IU p. o.
      • Every 6 months, infants <1 yr retinol palmitate 55mg or 33mg retinol acetate (100,000 IU) p. o.
      • Every 4-6 months, older children 110mg retinol palmitate or 66 mg retinol acetate (200,000 IU) p. o.
      • In prevalent areas, 100,000 IU p. o. q 3 mo
      • For malnourished children 1-6 yrs, 250,000 IU p. o. q 6 mo
      • Treatment:
      • > 1 yr: 110mg retinol palmitate or 66mg retinol acetate (200,000 IU) orally or preferably 33mg (100,000 IU) of retinyl palmitate by IM
      • The oral dose should be repeated on 2nd day and on discharge from hospital 7-30 days after 1st dose
      • Above doses halved for infants
      • For corneal involvement, apply antibiotic ointment like topical bacitracin to both eyes 6x/day and give also systemic antibiotics
    • 33. Hypovitaminosis A
    • 34. Hypervitaminosis A
      • Acute Intoxication:
      • Excessively large single doses > 300,000 IU
      • Infants: nausea & vomiting, drowsiness or irritability w/ signs of increased ICP
      • Adults: drowsiness, irritability, headache & vomiting
      • Serum vitamin A values = 200-1000 IU/dl (N: 50-100 IU/dl)
      • Chronic Intoxication:
      • > 50,000 IU/day for several wk
      • Signs & symptoms in infants:
        • Early: anorexia, pruritus, irritability, tender swollen bones w/ motion limitation
        • Alopecia, seborrhea, cheilosis & peeling palms & soles
        • Hepatomegaly & hypercalcemia
        • Craniotabes & hyperostosis of long bones (differentiate from Caffey’s disease)
      • Elevated serum vitamin A
      • Reversible manifestations when vitamin A discontinued
    • 35. Vitamin D (Cholecalciferol)
      • 90% Vitamin D 3 , cholecalciferol , produced in skin by UV irradiation of 7-dehydrocholesterol (mainly an animal sterol)  blood  25 hydroxylation to calcidiol in liver + PTH  di-hydroxylation in kidney to calcitriol 1,25 (OH) 2-cholecalciferol
      • 1,25-dihydroxycholecalciferol most active form
      • Vitamin D 2 , ergocalciferol , taken orally from plants (ergosterol) then irradiated as above
      • Animal derived vitamin D 3 and plant derived vitamin D 2 biologically equal
    • 36. Vitamin D (Cholecalciferol) Deficiency
      • Functions: Vitamin D enhances Ca +2 absorption from gut, removal of Ca +2 from bone & PO 4 -3 reabsorption in kidney
      • Rickets appear towards end of 1-2 yr
      • Etiology:
      • Lack in diet or lack of sunlight exposure
      • Rapid growth as in prematures & adolescents
      • Disorders of absorption such as celiac disease, steatorrhea or cystic fibrosis
      • In children with hepatic disease
      • Maternal malnutrition
      • Poverty or ignorance
    • 37. Hypovitaminosis D (Rickets)
      • Rickets: Deficient calcification or softening bones in a growing child resulting in deformation of bones
        • Head manifestations
          • Craniotabes: Thinning of skull outer table & feeling ping-pong ball sensation over occiput or posterior parietal bones  may disappear before end 1 yr  continues as flattening & at times permanent head asymmetry
          • Anterior fontanel larger & closure delayed
          • Caput quadratum: Box-like head due to thickened & prominent central parts of parietal & frontal bones
          • Delayed eruption of temporary teeth while permanent with enamel defects
    • 38. Hypovitaminosis D (Rickets)
      • Thorax signs
        • Palpable enlargement of costochondral junctions called “rachitic rosary”
        • Flattened sides of the thorax with posterior longitudinal grooves
        • Pigeon-breast deformity
        • Harrison groove
      • Spinal column signs
        • Scoliosis common
        • Kyphosis when sitting
        • Lordosis in the erect position
      • Pelvis narrowed due to changes in promontory, sacrum & coccyx which if permanent, add to hazards of childbirth in female
    • 39. Hypovitaminosis D (Rickets)
      • Extremities in children >2 years
        • Thickened & enlarged wrists & ankles
        • Bowlegs or knock-knees as a result of the bending of the softened shafts of the femur, tibia & fibula
        • Coxa vara or pronated feet
        • Greenstick fractures
      • Muscles poorly developed & lack tone
        • Delay in sitting, standing & walking
        • Potbelly due to weakness of abdominal muscles
      • Other manifestations
        • Underweight
        • Mental retardation
    • 40. Rickets
    • 41. Rickets
    • 42. Rickets
    • 43. Rickets A teenage male w/ rickets. Note bow legs & compromised height. Distal femur, proximal tibia and fibula in rickets. Note widening epiphysis, resorption of provisional zone of calcification, flaring metaphysis & bone deformity.
    • 44. Hypovitaminosis D (Osteomalacia)
      • Osteomalacia: Accumulation of uncalcified osteoid tissue in rib joints of an adult resulting in
        • Pain in pelvis, lower back and legs
        • Tenderness in shins and in other bones
        • Waddling gait
        • Deformities of pelvis
        • Tetany may occur manifested by involuntary twitching of facial muscles or by carpopedal spasm
        • Spontaneous fractures may occur
      • Osteomalacia should not be confused with osteoporosis , a disease of ageing, in which decalcification is also a feature
    • 45. Osteomalacia A young male w/ osteomalacia. Note a pseudofracture in the medial edge of the upper femoral shaft (arrow). Xray showing a pseudofracture (red arrow) from an adult who has x-linked hypophosphatemic rickets. This sign is seen only in osteomalacia, but not in many of the cases. AP pelvis in a patient w/ osteomalacia. The film shows diffuse osteopenia, & a Looser zone (arrow) in the superior ramus of the right obturator ring.
    • 46. Hypovitaminosis D
      • Diagnosis:
      • History & clinical observation
      • Laboratory findings:
        • Serum Ca +2 may be normal or low
        • Serum phosphorus level <4 mg/dl (N serum phosphorus 4.5-6.5 mg/dl but in rachitic infants reduced to < 1.5-3.5 mg/dl)
        • Serum alkaline phosphatase elevated (N serum phosphatase 5-15 Bodansky units per 100 ml but elevated to 20-30 in mild rickets & to > 60 in severe)
        • Serum 25-hydroxycholecalciferol decreased
        • Urinary cyclic AMP elevated
    • 47. Hypovitaminosis D
      • Roentgenographic changes
        • X-ray of wrist, cupping & fraying of proximal ends of ulna & radius, best for early diagnosis
        • Humeral ossification centers barely visualized
        • Shafts osteoporotic or density decreased
        • Rosary beading of sternal ends of the ribs due to deposited uncalcified osteoid tissue becoming compressed & bulges laterally
        • Initial healing indicated by appearance of line of preparatory calcification
    • 48. Hypovitaminosis D
      • Differential Diagnosis:
      • Craniotabes in hydrocephalus & osteogenesis imperfecta
      • “ Rosary” at costochondral junctions in scurvy & chondrodystrophy
      • Epiphyseal lesions in congenital epiphyseal dysplasia, cytomegalic inclusion disease, syphilis, rubella & copper deficiency
      • Congenital pigeon breast deformity
      • Familial bowlegs
      • Metabolic disturbances with osseous lesions
      • Complications:
      • Respiratory infections
      • Chronic gastroenteritis
      • Iron deficiency anemia
    • 49. Hypovitaminosis D
      • Prognosis:
      • In tropics, usually has tendency to heal spontaneously
      • A possibly deforming disorder
      • Not fatal but complications & intercurrent infections may cause death
      • RDA: 400 IU (1 IU vitamin D = 0.025 mcg cholecalciferol/ ergocalciferol)
      • Prevention:
      • Sunlight prophylaxis effective only in temperate zones during summer months in haze-free areas
      • Daily requirement of vitamin D in 1 quart of fresh whole milk or a can of evaporated milk
      • Natural vitamin D present only in animal foods like egg yolk, liver, cod-liver & other fish-liver oils & fishbody oils
    • 50. Hypovitaminosis D
      • Prematures or breast-fed infants should receive supplemental vitamin D daily because milk is a poor source unless fortified
      • Vitamin D should also be administered to pregnant & lactating mothers
      • Treatment:
      • Daily administration of 50-150 mcg of vitamin D 3 or 0.5-2 mcg of 1,25-dihydroxycholecalciferol will produce healing seen on X-ray within 2-4 wks
      • Vitamin D 15,000 mcg in a single dose w/o further therapy for several months may be advantageous
      • After healing is complete, the dose of vitamin D should be lowered to 10 mcg/day
      • If no healing occurs, rickets is probably resistant to vitamin D or non-nutritional rickets
    • 51. Hypervitaminosis D
      • Etiology: Excessive intakes from
      • Inadvertently substituting concentrated form for dilute
      • Parents’ increasing prescribed dose
      • Inadequately controlling dosages for children receiving large amounts of vitamin D for chronic hyperphosphatemia
      • Clinical Manifestations: Symptoms after 1-3 months
      • Anorexia, irritability, hypotonia & constipation
      • Polydipsia, polyuria & pallor
      • Dehydration usually present
      • Aortic valvular stenosis, vomiting & hypertension
      • Retinopathy & clouding of cornea & conjunctiva may occur
    • 52. Hypervitaminosis D
      • Laboratory Data:
      • Proteinuria
      • Hypercalcemia & hypercalciuria
      • With continued excess, renal damage & metastatic calcifications may occur
      • Roentgenograms of long bones reveal metastatic calcification & generalized osteoporosis
      • Differential Diagnosis:
      • Chronic nephritis
      • Hyperparathyroidism
      • Idiopathic Hypercalcemia
      • Treatment:
      • Discontinue vitamin D & decrease Ca +2 intake
      • For severe: Al(OH)3 p. o., cortisone or sodium versenate may be used
    • 53. Vitamin K
      • Naturally occurring vitamin K abundant in pork, liver, soybeans & green leafy vegetables
      • Synthesize by intestinal microorganisms
      • Required for normal clotting of blood
      • Vitamin K-dependent clotting factors:
        • Prothrombin (Factor II)
        • Proconvertin (Factor VII)
        • Plasma thromboplastin component or PTC (Factor IX)
        • Stuart-Prower factor (Factor X)
    • 54. Vitamin K Deficiency (Hypoprothrombinemia)
      • Etiology:
      • The fetus depends on mother for supply & at birth, bacterial flora of GIT not yet produce
      • Exclusively breast-fed infants lower vitamin K compared to formula-fed
      • Faulty intestinal absorption as in diarrhea, celiac disease, gastrointestinal malformation & steatorrhea
      • Obstructive jaundice, biliary fistula, insufficient production of bile acids or pancreatic insufficiency lead to inadequate intestinal absorption
      • Administration of antibiotics inhibit intestinal bacteria
      • In sepsis, deficiency from disease affecting hepatobiliary functions & therapy
      • Drugs like coumarin, salicylates & anticonvulsants
    • 55. Hypoprothrombinemia
      • Clinical Manifestations:
      • Hemorrhagic manifestations are the hallmark
      • Bleeding in the newborn from the cord or circumcision site
      • GIT bleeding, hematuria & intracranial hemorrhage
      • Anemia & shock may ensue from severe blood loss
      • Laboratory Test: Most useful test is 1-stage prothrombin time test (Quick) , prolongation presumptive evidence deficiency
    • 56. Hypoprothrombinemia
      • Prevention and Treatment:
      • 4 requirements to prevent & control a potentially fatal hemorrhagic state:
        • Bile of normal composition in the GIT
        • Adequate diet
        • Normal absorptive surface in the small intestines
        • Functioning liver capable of synthesizing
      • In the newborn, vitamin K 1 is being used because:
        • Greater margin of safety
        • Acts rapidly with therapeutic levels within 2-4 hours
    • 57. Hypoprothrombinemia
      • Prevention and Treatment:
      • The AAP Committee on Nutrition recommends:
        • Prophylactic dose: Vitamin K 0.5-1 mg as single parenteral dose or 1-2 mg single p. o. dose
        • Mild prothrombin deficiency: Vitamin K 1-2 mg p. o. OD
        • In severe cases with hemorrhages:
          • Vitamin K 1 5 mg daily parenterally
          • Whole blood if due to liver damage
      • Avoid excessive doses in prematures & G-6-PD deficient newborns due to hemolytic action & hyperbilirubinemia
      • Vitamin K prophylaxis to woman in labor may be followed by hemolytic anemia, hyperbilirubinemia, kernicterus & death in infant
    • 58. Hypoprothrombinemia
    • 59. Vitamin E (Tocopherol)
      • Denotes a group of 8 compounds with similar structures and antioxidant activity
      • The most potent member is  tocopherol , the main form in humans
      • Functions as antioxidant mainly located within cell membranes where it prevents lipid peroxidation and formation of free radicals
      • Best dietary sources: vegetable oils, seeds, nuts, green leafy vegetables and margarine
    • 60. Hypovitaminosis E
      • Etiology:
      • Poor absorption in premature infants*
      • In children with fat malabsorption, most common in cholestatic liver disease & a complication of autosomal recessive abetalipoproteinemia
      • Other malabsorptive states such as cystic fibrosis, acanthocytosis, celiac disease, short-bowel syndrome or Crohn disease
      • In a rare autosomal recessive disorder, Ataxia with isolated Vitamin E Deficiency (AVED), that affects vitamin E transport
      • Clinical Manifestations:
      • Appear after 1 yr of age in prolonged deficiency: degenerative neurologic syndrome of cerebellar disease, posterior column dysfunction and retinal disease**
      • In premature infants, hemolysis typically develops during 2 nd mo with edema and thrombocytosis potentially causing anemia
      • Diagnosis:
      • Best by measuring ratio of vitamin E to serum lipids: abnormal ratio <0.8 mg/g
    • 61. Hypovitaminosis E
      • Electroretinography abnormalities may precede PE findings in those with retinal involvement
      • Premature infants with unexplained hemolytic anemia after 1 st mo, especially if thrombocytosis present
      • Prognosis:
        • Hemolytic anemia in infants resolves with correction of deficiency
        • Treatment prevents progression of neurologic manifestations
      • Prevention & Treatment:
      • Prevention in premature infants: sufficient vitamin E, 7mg/g of unsaturated fat in the diet, and formula without high content PUFAs
      • Treatment dose in neonates: 25–50 units/day for 1 wk, follow with adequate dietary intake
      • Severe malabsorption:  -Tocopheryl polyethylene glycol succinate (TPGS), a water-soluble preparation absorbed in absence of bile salts 20–25 units/kg/day
      • Children with AVED: high doses of vitamin E
    • 62. The Energy-Releasing Vitamins
    • 63. Thiamine (Vitamin B1) Deficiency Beriberi
      • Pathology: Biochemical accumulation of pyruvic and lactic acid in body fluids causing:
      • Cardiac dysfunction such as cardiac enlargement esp. right side, edema of interstitial tissue & fatty degeneration of myocardium
      • Degeneration of myelin & axon cylinders resulting in peripheral neuropathy
      • In chronic deficiency states, vascular dilatation & brain hemorrhages of Wernicke’s Disease resulting in weakness of eye movement, ataxia of gait and mental disturbance
    • 64. Beriberi
      • Three forms:
      • Wet beriberi: generalized edema, acute cardiac symptoms and prompt response to thiamine administration
      • Dry beriberi: edema not present, condition similar to peripheral neuritis w/ neurological disorders present
      • Infantile beriberi divided into:
        • Acute cardiac: Ages 2-4 months; sudden onset of cardiac s/sx such as cyanosis, dyspnea, systolic murmur & pulmonary edema w/ rales
        • Aphonic: Ages 5-7 months; insidious onset of hoarseness, dysphonia or aphonia
        • Pseudomeningeal: Ages 8-10 months; signs of meningeal irritation w/ apathy, drowsiness & even unconsciousness; occurs more often
    • 65. Beriberi
      • Diagnosis:
      • Clinical manifestations not conclusive
      • Therapeutic test, parenteral thiamine = dramatic improvement
      • Blood lactic & pyruvic acid levels elevated after oral glucose load
      • Decreased red cell hemolysate transketolase
      • RDA: Infants 0.4mg
      • Older children 0.6-1.2mg
      • Nursing mothers 1.5mg
      • Adults 1-1.3mg
      • Prevention:
      • Richest sources: pork, whole grain, enriched cereal grains and legumes
      • Improved milling of rice conserve thiamine
      • Excessive cooking of vegetables or polishing of cereals destroy it
      • In breast-fed infants, prevention achieved by maternal diet w/ sufficient amounts
      • Treatment:
      • Children: 10mg p. o. daily for several weeks
      • Adults: 50mg
    • 66. Beriberi
    • 67. Beriberi
    • 68. Riboflavin (Vitamin B2) Deficiency
      • Functions:
      • Coenzyme of flavoprotein important in a. a., f. a. & CHO metabolism & cellular respiration
      • Needed also by retinal eye pigments for light adaptation
      • Clinical Manifestations:
      • Lesions of the lips characteristic, most common are angular stomatitis and cheilosis
      • Localized seborrheic dermatitis of the face such as nasolabial seborrhea or dyssebacia & angular palpebritis
      • Scrotal or vulvar dermatosis may also occur
      • Ocular s/sx are photophobia, blurred vision, itching of eyes, lacrimation & corneal vascularization
    • 69. Riboflavin Deficiency
      • Diagnosis:
      • Urinary riboflavin determination
      • RBC riboflavin load test
      • RDA: Infants & children <10yrs 0.6-1.4mg
      • Children >10yrs 1.4-2mg
      • Adults 0.025mg/gm dietary protein
      • Prevention:
      • Best sources: eggs, liver, meat, fish, milk, whole or enriched ground cereals, legumes, green leafy vegetables
      • Also present in beer
      • Impaired absorption in achlorhydria, diarrhea & vomiting
      • Treatment:
      • Riboflavin 2-5mg p. o. daily w/ increased B complex
      • Parenteral administration if relief not obtained
    • 70. Riboflavin Deficiency
    • 71. Niacin (Vitamin B3) Deficiency Pellagra
      • Etiology:
      • Diets low in niacin &/or tryptophan
      • Amino acid imbalance or as result of malabsorption
      • Excessive corn consumption
      • Clinical Manifestations:
      • Starts w/ anorexia, weakness, irritability, numbness & dizziness
      • Classical triad of dermatitis, diarrhea & dementia
      • Dermatitis may develop insidiously to sunlight or heat
        • First as symmetrical erythema
        • Followed by drying, scaling & pigmentation w/ vesicles or bullae
        • Predilection for back of hands, wrists, forearms (pellagrous glove), neck (Casal’s necklace) & lower legs (pellagrous boot)
      • GIT s/sx are stomatitis, glossitis or diarrhea w/ feces pale, foul milky, soapy or at times steatorrheic
      • Mental changes include depression, irritability, disorientation, insomnia & delirium
    • 72. Pellagra
      • Diagnosis:
      • History & manifestations of diet poor in niacin or tryptophan
      • In niacin deficiency, urinary levels of N-methyl-nicotinamide low or absent
      • Differential diagnoses: Kwashiorkor, Infantile Eczema, Combination deficiencies of amino acids & trace minerals such as zinc
      • RDA: Infants & children <10yrs 6-10mg
      • Older individuals 10-20mg
      • Prevention:
      • Rich sources include meat, peanuts and legumes, whole grain and enriched breads and cereals
      • Avoid too large a proportion of corn
      • Treatment:
      • Niacin 50-300mg daily may be taken for a long time
      • Skin lesions may be covered w/ soothing lotions
    • 73. Pellagra
    • 74. Pyridoxine (Vitamin B6) Deficiency
      • Functions:
      • In the synthesis and catabolism of a. a., synthesis of neurotransmitters, porphyrins and niacin
      • Important role in clinical conditions such as anemia, hyperemesis gravidarum, cardiac decompensation, radiation effects, skin grafting, INH therapy & seborrheic dermatitis
      • Etiology:
      • Losses from refining, processing, cooking & storing
      • Malabsorptive diseases such as celiac disease
      • Direct antagonism between INH & pyridoxal phosphate at the apoenzyme level
    • 75. Pyridoxine Deficiency
      • Clinical Manifestations:
      • Three different types
        • Neuropathic , due to insufficient neurotransmitter synthesis such as irritability, depression & somnolence
        • Pellagrous , due to low endogenous niacin synthesis such as seborrheic dermatitis, intertrigo, angular stomatitis & glossitis
        • Anemic , due to low porphyrin synthesis such as microcytic anemia & lymphopenia
      • In genetic diseases involving pyridoxal phosphate enzymes: xanthurenic aciduria, cystathioninuria & homocystinuria
    • 76. Pyridoxine Deficiency
      • Diagnosis: As screening test, tryptophan load test -100mg/kg BW tryptophan gives large amount of xanthurenic acid in urine
      • Prevention:
      • Firm requirement not established but usual recommendation: Infant 0.1-0.5mg, Child 0.5-1.5mg & Adult 1.5-2mg
      • Rich sources: yeast, whole wheat, corn, egg yolk, liver and lean meat
      • Toxicity at extremely high doses described; infants whose mothers received large doses during pregnancy should be observed for seizures due to dependency
      • Children receiving INH therapy should be observed for neurologic s/sx in w/c case pyridoxine should be given
      • Treatment:
      • Pyridoxine 100mg IM injection for seizures due to deficiency
      • Children w/ pyridoxine dependency 2-10mg IM injection or 10-100mg oral vitamin B6
    • 77. The Hematopoietic Vitamins
    • 78. Folic Acid (Vitamin B9) Deficiency
      • Functions:
      • Needed for RBC & DNA formation & cell multiplication esp. GI cells
      • Newly discovered functions:
        • Prevents neural tube defects
        • Prevents heart disease (reduces homocysteine levels)
        • Prevents colon cancer
      • Peak incidence 4-7 months
      • Etiology:
      • Deficient dietary intake: goat’s milk deficient & powdered milk poor source
      • Deficient absorption in celiac disease, achlorhydria, w/ anticonvulsant drugs, zinc deficiency & bacterial overgrowth
      • Impaired metabolism in ascorbic acid deficiency, hypothyroidism, w/ drugs like trimethoprim
      • Increased requirement during rapid growth & infection
      • Increased excretion/loss may occur due to vitamin B12 deficiency
    • 79. Folic Acid Deficiency
      • Clinical Manifestations:
      • Megaloblastic anemia w/ irritability, failure to gain wt & chronic diarrhea
      • Thrombocytopenic hemorrhages advanced cases
      • Scurvy may be present
      • Laboratory Findings:
      • Anemia macrocytic
      • Serum folic acid <3ng/ml (Normal = 5-20ng/ml)
      • RBC folate levels indicator of chronic deficiency (Normal = 150-600ng/ml)
      • Serum iron & vitamin B12 normal or elevated
      • Formiminoglutamic acid in urine esp after oral histidine
      • Serum LDH markedly high
      • Bone marrow hypercellular
      • RDA: 20-50mcg/24 hrs
      • Treatment:
      • Parenteral folic acid 2-5mg/24 hr response in 72 hrs, for 3-4 wks
      • Transfusions only if anemia severe
      • Satisfactory responses even w/ low doses of 50mcg/24 hr, no effect on primary vitamin B12 deficiency
      • If pernicious anemia present, prolonged use of folic acid should be avoided
    • 80. Folic Acid Deficiency
    • 81. Cobalamine (Vitamin B12) Deficiency
      • Absorption: Vitamin B12 + glycoprotein (intrinsic factor) from parietal cells of gastric fundus  terminal ileum absorption + intrinsic factor + Ca++  blood
      • Function: Needed in reactions affecting production of methyl groups
      • Etiology:
      • Congenital Pernicious Anemia : Lack of secretion of intrinsic factor by stomach manifest at 9 mo-10 yr as uterine stores become exhausted
      • Inadequate intake or dietary deficiency rare
        • Strict vegetarian diet
        • Not commonly seen in kwashiorkor or marasmus
        • Breast-fed infants whose mothers had deficient diets or pernicious anemia
      • Consumption or inhibition of B12-intrinsic factor complex
      • Vitamin B12 malabsorption from disease of ileal receptor sites or other intestinal causes
    • 82. Cobalamine Deficiency
      • Clinical Manifestations:
      • Megaloblastic anemia that becomes severe*
      • Neurological includes ataxia, paresthesias, hyporeflexia, Babinski responses, clonus & coma**
      • Tongue smooth, red & painful
      • Laboratory Findings:
      • Anemia macrocytic
      • Serum vitamin B12 <100pg/ml but serum iron & folic acid normal or elevated
      • Serum LDH activity markedly increased
      • Urinary excess of methylmalonic acid, a reliable & sensitive index
    • 83. Cobalamine Deficiency
      • Schilling test to assess the absorption of vitamin B12:
        • Normal person ingests small amount of radioactive vitamin B12  none in urine **If flushing dose injected parenterally, 1000mcg of non-radioactive vitamin B12  10-30% of previous radioactive vitamin B12 appears in urine
        • Pernicious anemia  2% or less **If modified: 30 mg intrinsic factor administered along  normal amounts
        • Disease of ileal receptor sites or other intestinal causes  no improvement even w/ intrinsic factor
      • RDA: Infants 0.5 mcg/day
      • Older children & adults 3mcg/day
      • Treatment:
      • Prompt hematological response w/ parenteral vitamin B12 1-5mcg /24hr
      • If there is neurological involvement 1mg IM daily for at least 2wks
      • Pernicious Anemia: Monthly vitamin B12 1mg IM necessary throughout patient’s life
    • 84. Cobalamine Deficiency
    • 85. Ascorbic Acid (Vitamin C) Deficiency Scurvy
      • Functions:
      • Hydroxyproline, found only in collagen, major connective tissue in body, forms from proline requiring ascorbic acid
      • If defective collagen formation, endochondral bone formation stops since oste, intercellular substance, not formed
      • In hydroxylation reactions in synthesis of steroids and epinephrine
      • Aids iron absorption by reducing it to ferrous state in stomach
      • Spares vitamin A, vitamin E and some B vitamins by protecting them from oxidation
      • Enhances utilization of folic acid by aiding conversion of folate to tetrahydrofolate
    • 86. Ascorbic Acid (Vitamin C) Deficiency Scurvy
      • More common 6-24 months
      • Etiology:
      • May develop in breastfed infant if mother’s diet deficient
      • Improper cooking practices produce significant losses
      • Clinical Manifestations:
      • Early: irritability, digestive disturbances & anorexia
      • Mild deficiency: ecchymoses, corkscrew hairs & petechiae due to increased capillary fragility from weakened collagen
      • Severe deficiency: decreased wound healing, osteoporosis, hemorrhaging, bleeding into skin and friable bleeding gums with loosened teeth
    • 87. Scurvy
      • Clinical Manifestations:
      • A presenting feature: an infant w/ painful, immobile legs (pseudoparalysis), in “frog position” & occasionally w/ mass
      • Depression of sternum w/ a “rosary of scorbutic beads” at costochondral junction due to subluxation of sternal plate
      • Orbital or subdural hemorrhages, melena & hematuria
      • Low grade fever & anemia usually present
      • Impairment of growth & development
      • Diagnosis:
      • History of vitamin C-deficient diet
      • Clinical picture
      • Therapeutic test
    • 88. Scurvy
      • Diagnosis:
      • X-ray findings in long bones:
        • Most prominent & early change: knee atrophy
        • Shaft trabeculae cannot be distinguished giving “ground glass appearance”
        • Cortex reduced to “pencil-point thinness”
        • Zone of well-calcified cartilage, white line of Fraenkel irregular & thickened white line
        • Zone of rarefaction, a linear break in bone proximal & parallel to white line under at metaphysis
        • Calcifying subperiosteal hemorrhages cause bone to assume a dumb-bell shape
    • 89. Scurvy
      • Laboratory tests not helpful:
        • Ascorbic acid concentrate of buffy layer of centrifuged oxalated blood = zero in latent scurvy
        • Diminished urinary excretion of vitamin C after loading
      • Differential Diagnosis:
      • Bleeding, swollen gums: Chronic gingivitis & pyorrhea w/ pus & respond to good dental hygiene
      • Pseudoparalysis: Syphilis negative x-ray; Poliomyelitis absent tenderness of extremities
      • Tenderness of limbs: RF age >2 yrs; Suppurative arthritis & osteomyelitis positive blood cultures
      • Bleeding manifestations: Blood dyscracias positive blood exams
      • “ Rosary of scorbutic beads”: Rickets
    • 90. Scurvy
      • Prognosis:
      • Recovery rapid w/ adequate treatment & permanent deformity rare
      • Pain ceases in a few days but swelling may last months
      • Prevention :
      • Minimum daily intake 30mg as recommended by WHO for all ages
      • Every infant should receive supplement starting 2nd wk
      • Lactating mothers at least 50mg vitamin C daily
      • Guava & papaya richer than citrus fruits, also in most green leafy vegetables, tomatoes & fresh tubers but absent in cereals, most animal products & canned milk
      • Treatment:
      • Ascorbic acid 200-500mg daily or 100-150ml of fruit juice
    • 91. Scurvy
    • 92. Scurvy
    • 93. Be master of your habits, Or they will master you. Thank You!

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