NUTRITION II DISEASES OF MALNUTRITION

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NUTRITION II DISEASES OF MALNUTRITION

• PROF.DR. SHAHENAZ M. HUSSIEN

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DISEASES OF MALNUTRITION

• OBJECTIVES
• By the end of this lecture you will be able to
  know the followings
• -Protein energy malnurition diseases ( marasmus
  and Kwashiokor) definition causes clinical
  picture, complications, investigations,
  prevention and management.
• -Vitamin D- defficiency Rickets and review other
  types of rickets definition causes clinical
  picture complications investigations
  prevention and management.

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• Classification of P.E.M.-
• 1-Welcome classification
• -This classification depends mainly on body
  weight for age and the presence or absence of
  oedema.
• Body weight less than 60
• Without oedema (marasmus)
• With oedema (marasmic kwashiorkor )
• Body weight 60-80
• Without oedema (under weight)
  
  With oedema
  (kwashiorkor)

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•  2- Water-law classification
• This classification gives an idea about the
  duration of occurrence of the disease.
•  
• Weight for length gt 80 of the standard Wasted
  -This means acute malnutrition
  (within 6 months). 
• Length for age gt 90 of the standard Stunted.  
• - This means chronic malnutrition (more than 6
  months).
• Weight for length gt 80 of the standard length
  for age gt 90 of the standard Wasted and
  stunted.  

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• Kwashiorkor
• Kwashiorkor results from lack of protein in diet.
• Age- Mainly 6months ? 3years.
• Aetiology-
• General causes 1- Maternal ignorance.
  2- Poverty.
• Dietetic errors Excess starchy feeding.
• Infections
• 1- Diarrhea 2- Measles. 3- T.B.
• Clinical pictures
• Constant features
• 1- Growth failure 1-Wheight at 60 80 of
  standard age.
• 2- Failure to gain wheight then followed by
  weight loss.
• 3- Wasted ? decreased weight for length.
• 4- Lastely, height and head circumference may
  be affected.

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• 2- Muscle wasting
• - Mostly defects biceps and triceps.
• - Leading to hypotonia and weakness.
• Muscle wasting detected by
• Decreased mid-arm circumference.
  
• Decreased muscle / fat ratio.
• Decreased skin fold thickness.
• 3- Oedema (nutritional oedema)
• - Mainly due to hypoproteinemia.
• - Starts early at the dorsum of feet and
  legs, then becomes generalized
• bilateral, pitting and painless. Buffy checks
  with moon face appearance.
• -Not associated with ascites.
• 4- Mental changes
• - The patient becomes apathetic, disoriented
  with his surroundings

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• Variable features
• 1- Hair changes - Colour- lighter
  progressively, black to dark brown
• Light brown to orange to yellow.
• - Texture- soft and easily broken.
  Distribution- sparse.
• - Attachment - loose easily epilated without
  pain.
• - Flag sign (diagnostic sign) due to
  repeated attacks and affection of hairs in
  segmental manner which leads to bands of light
  colour alternating with bands of darkening in the
  same hair.
• 2- Skin changes - Dry scaly skin followed
  by erythema.
• - Areas of hyperpigmentations which is followed
  by exfoliated skin.
• Areas of hypopigmentation
• - Fissuring and cracking of the skin.
• - Purpura. - Secondary bacterial infection.
• 3- Hepatomegally (fatty liver).- Soft to
  firm and smooth with rounded border. - Caused by
  increased fat mobilization to the liver from the
  body.

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• 4- Gastro-intestinal manifestations
• - Anorexia caused by infections and mental
  changes.
• - Diarrhea, caused by Infection, maldigestion,
  malabsorption and lactose intolerance
• - Vomiting.
• 5- Anaemia may be
• - Microcytic hypochromic anaemia- due to iron
  deficiency anemia
• - Normocytic normochromic anaemia- due to
  infection and
• hypoproteinemia.
• - Megaloblastic anaemia- due to folic acid and
  vitamin B12 deficiency.
• 6- Infections occurs due to
• - Epithelial cells linning of the
  gastro-intestinal tract and respiratory tract
  were laible to invasion by micro-organisms.
• - Impaired immunity.

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• Infection may be masked by absence of fever due
  to
• Oedema leading to increase heat loss.
• Hypoglycemia leading to decrease heat
  production.
• Impaired shivering due to muscle wasting.
• - Most common infections are gastro enteritis,
  pneumonia,
• otitis media, T.B., urinary tract infection,
  Candida infections.
• 7- Signs of vitamin deficiencies
• - Vitamin A deficiency xerosis, keratomalacia,
  night blindness, corneal opacities.
• - Vitamin B deficiency glossitis, angular
  stomatitis. 
• - Vitamin C deficiency scurvy, bleeding gums.
• - Vitamin D deficiency rickets.
• 8- Hemorrhagic manifestations mainly due to
• - Vitamin K deficiency - Protein deficiency
  and increased capillary fragility.

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• Investigations-
• Plasma proteins 1-
  Decreased total serum proteins. 2-
  Decreased serum albumin 3- Decreased ?
  and ß globulins.
• 4- Decreased essential aminoacids. 5- Reversed
  albumin / globulin ratio .  
• 6- Normal or increased non-essential aminoacids.
  
• 7- Special proteins
• - Decreased transferrin (used to transfer iron).
• - Decreased ceruloplasmin (used to
  transfer copper).
• - Decreased haptoglobulin.
• Blood sugar Hypoglycemia.
• Water and electrolytic disturbances
• 1- Increased total body water (intra and extra
  cellular).
• 2- Increased sodium level but water retention is
  excessive lead to hyponaetremia (dilutional).
• 3- Decreased potassium level, mainly due to
  vomiting and diarrhea.
• 4- Decreased calcium level.

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• Minerals Mg, Fe, Cu, Zn and all other trace
  elements are reduced. Hematological changes
• 1- Anaemia.
• 2- Leucocytosis, may be leucopenia.
• 3- Thrombocytosis.
• Tuberculin test.
  Chest X-ray.
• Complications of kwashiorkor-
• Infections 1- Bronchopneumonia is the most
  common cause of death.
• 2- Others otitis media, U.T.I., T.B., monilial
  infections.
• 3- Gastroenteritis diarrhea, malabsorption
  and dehydration.
• Hypoglycemia.
• Heart failure due to1- Anaemia. 2- Volume
  overload (fluid or blood).
• 3- Weak
  myocardial contractility.

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MARASMUS
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• Marasmus
• Definition-
• It is a state of chronic malnutrition due to
  deficiency of total caloric requirements.
  - Commonly seen in the first 2years of life.
• Etiology-
• 1- Socio-economic causes Ignorance,
  poverty, depression.
• 2- Dietetic errors (nutritional marasmus)
• 1- Quantitative disorders- Scanty breast milk (in
  amount or number of feeds).
• - Small amount of feed. - Delayed weaning.
• 2- Qualitative disorders - Over dilutional
  formula in artificial feeding.
• - Cows milk protein allergy.
• 3- Non-dietetic errors (secondary marasmus)
  1- Gastroenteritis.
• 2- Malabsorption syndromes. 3- Infections.
  as T.B., pyelonephritis, chronic suppurative
  lung disease.

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• 4 - Congenital abnormalties
• - G.I.T.- congenital pyloric stenosis, cleft lip
  and palate.
• - Liver- congenital hepatic cirrhosis.
• - CVS- fallots tetralogy, V.S.D.
• - Chest - congenital interstitial fibrosis.
• - Renal- renal agenesis, obstructive uropathy.
• CNS- defective cerebral development.
• 5 - Metabolic disorders
• - Renal tubular acidosis. - Fructosemia. - Urea
  cycle defects.
• - Galactosemia. - Amino acid defects.
• 6 - Endocrinal disorders
• - Juvenile D.M. - Adrenal insufficiency.

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• Clinical picture of marasmus-
• Growth failure
• 1- At first, there is failure to gain weight then
  loss of weight occurs.
• 2- Weight less than 60 of the ideal weight for
  age.
• Loss of subcutaneous fat
• 1- First degree loss of subcutaneous fat in the
  abdominal wall.
• 2- Second degree loss of subcutaneous fat in
  limbs, buttocks and
• abdominal wall.
• 3- Third degree loss of subcutaneous fat in face
  limbs and abdominal.
• Muscle wasting- Detected by decreased mid-arm
  circumference.
• - Marked pallor due to associated anaemia.
• - Subnormal temperature due to loss of
  subcutaneous fat.
• Gastro-intestinal manifestations 1- Anorexia
  2- Constipation .
• 3- Diarrhea due to gastroenteritis and
  malabsorption.

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• 4- Signs of vitamin deficiencies.
• 5- Infections- Pneumonia, Gastroenteritis,
  Otitis media, U.T.I and T.B.
• Investigations-
• -Complete blood picture
• 1- RBCs- anaemia (all types of anaemia can be
  found).
• 2- WBCs- leucocytosis or leucopenia.
• 3- Platelets- thrombocytopenia.
• -Total proteins and serum albumin
  Slightly reduced.
• -Urine analysis -1- Culture in case of U.T.I.
  2- Glucosuria in case of D.M.
• -Stool analysis For parasites or steatorrhea.
• -Chest X-ray For bronchopneumonia or
  congenital heart disease.
• -Tuberculin test. -Intestinal biopsy
  If there is malabsorption.
• Complications- 1- Oedema- marasmus
  kwashiorkor. 2- DIC.
• 3- Pressure
  sores. 4- Fatal hypothermia.

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• Management of P.E.M.
• Management of simple undernutrition
• 1- Can be managed at home without hospital
  admission.
• 2- Diet should provide120-150 kcal/ kg / day,
  proteins 2-4 gm/ day.
• 3- Frequency - Small frequent feeds (every
  2-4 hours).
• 4- Types of foods which can be used
• - Choose suitable, locally available,
  economically feasible weaning foods as milk,
  eggs, cereals, vegetables, beans and if feasible
  animal proteins.
• 5- Regular follow up of weight is very important.

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• Treatment of the complications.
• 1- Treatment of dehydration
• -ORS is the golden treatment for dehydration due
  to P.E.M. we must avoid I.V. fluid therapy as
  possible for the risk of heart failure due to
  overload except if there is shock. - The
  recommended regimen for the treatment of
  dehydration due to malnutrition as follow
• - ORS given slowly in amount of 70-100 ml / kg
  over 12 hours.
• - At the first 2 hours, the patient receives 10
  ml / kg.
• - The remaining amount given over the following
  10 hours.
• - Add 50-100 ml after each watery stool.
• - I.V. fluids ringers lactate plus glucose in a
  percentage of 11 and add 2.5 ml of 15 kcl for
  each one litter. 
• 2- Treatment of infections
• - Appropriate antibiotics even if the signs of
  infection is not present.
• - In manifest infection- according to culture
  and sensitivity.

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• 3- Treatment of electrolyte disturbances
• - Hypoglycemia- glucose 10 2 ml / kg I.V. and
  regular feeding.
• - Hypocalcemia- Ca gluconate 10 slowely I.V
  (2ml / kg).
• - Hypokalemia- Add K to the I.V. fluids (2mEq /
  kg).
• 4- Treatment of hypothermia- Proper wrappings. -
  Put under radient warmth.
• 5-Treatment of anaemia If severe give
• - Fresh blood in amount of 20 ml / kg. - Fresh
  packed RBCs in case of anaemic heart failure in
  amount of 5-10 ml / kg.
• - Fresh frozen plasma 10 ml / kg in case of
  hypoprothrombinemia .
• Dietetic management Route - Oral. -
  Nasogastric tube.
• - Small feeds (every 2-3 hours).
• - Half strength and half amount in the first
  2days then increased gradually until we
  reach full strength .
• - Start with 120-150 kcal / kg / day, and after
  1-2 weeks we increase calories gradually up to
  200 kcal / kg / day.

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• 3- Food used in management
• - Milk, youghurt, rice, beans, lentils, fish,
  meat, eggs and
• chicken. Oils, sugar may be added for food to
  increase calories content.
• 4- Vitamins and minerals
• - Vitamin A, vitamin D and vitamin B complex.
• - Calcium, zinc and iron.
• If no satisfactory response to good dietary
  management, look for
• Hidden infections as U.T.I. Hidden
  disease as anaemia, C.H.D and, metabolic
  disorders.
• Prevention of PEM1- Breast milk feeding
  promotion. 2- Proper weaning.
• 3- Early detection of P.E.M. by using weight
  charts and MAC. 4-Immunization. 5- Control of
  most common diseases such as diarrhea.
• 6- Food supplementation programmes - Iron
  to treat anaemia.
• Iodine to treat and prevent hypothroidism.
• 7- Health education

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RICKETS

• Vitamin D
• Vitamin D is one of the group of fat soluble
  vitamins which are essential for calcium and
  phosphate metabolism.
• Sources of Vitamin D are
• - Animal source as Vitamin D3 (cholecalciferol)
  is present in fish liver oil and egg yolk,
  Powdered milk and breast milk.
• - Plant source as Vitamin D2 (Ergocalciferol)
  present in irradiated green plants by
  ultraviolet irradiation. -Endogenous source
  ultraviolet irradiation converts 7
  dehydrocholesterol in the skin to Vitamin D3.
• Metabolism - Vitamin D is absorbed from upper
  part of intestine aided with bile salts which
  form micelles transported by lymphatics to liver.
• Also ultraviolet rays converts
  (7-dehydrochocholesterol) in skin to Vitamin D3
  that transported to liver.

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• In the liver
• Both Vitamin D2, D3 are hydroxylated to 25-OH
  cholecalciferol by 25 hydroxylase enzyme.
• In the kidney
• -In renal cortex 25 (OH) D is converted to 1,
  25 (OH)2 D (1.25 Dihdoxycholecalciferol) by 1 ,25
  hydroxylase enzyme, which is the active form and
  function as hormone.
• -If Ca and Ph are normal or high, 24
  hydroxylase enzyme is activated to form 24, 25
  (OH)2 D which is less active form. This pathway
  is essential for removal of excess Vitamin D.
• Function Receptors for 1,25 (OH)2 D are
  present in most tissues .
• -Intestine When serum level of calcium falls
  leads to stimulation of parathormone hormone
  secretion ,and stimulation of 1.25 hydroxylase
  in the kidneys that enhance production of 1,25
  (OH)2 D3. This induces synthesis of calcium
  binding protein (calbindin D) in the intestine
  that leads to absorption of calcium.
  -Kidney tubular reabsorption of Ca and Ph.
  -Bone enhance mineralization of bone matrix.

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• Vitamin D deficiency rickets.
• Epidemiology
• Age 6months 2years peak at 18months.
• Sex more in males.
• Race negros are more susceptible.
• Growth more in rapidly growing infants twins,
  preterms.
• Environment smoke, dust, clouds and ordinary
  window glass prevent ultraviolet rays to reach
  the skin.
• Rachitogenic diet
• - High phosphate content in unmodified animal
  milk ? decrease calcium absorption.
• - Cereals rich in phytates and phosphates
  interfer with calcium absorption due to formation
  of insoluble salts with calcium.

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• Clinical picture
• Early manifestation - Anorexia. Irritability,
  sweating.
• Craniotabes caused by thinning of the outer
  table of the skull.
• Rachitic rosaries It is a prominant enlargement
  of the costochondral junction and felt as a raw
  of beads.
• Broadening of wrists and ankles due to epiphysial
  enlargement.
• Advanced manifestation
• Head
• Large if rickets develops early in the 1st year.
• Large anterior fontanell with delayed closure.
• Bossing of the skull ---- due to thickening of
  the central parts of parietal and frontal bone.
• Delayed teething with enamel defect and caries
  may occur.

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• Thorax
• Rachitic rosaries.
• Longitudinal grooves developed posterior to the
  rosaries with flattening of sides of chest cage.
• Harrison sulcus a horizontal depression at the
  lower part of the chest along the costal
  attachment of the diaphragm which is dragged in
  during inspiration.
• Pigeon breast deformity the sternum with its
  adjacent cartilage appears to project forward.
• Vertebral spine
• Kyphosis dorso-lumber and is apparent while
  sitting due to laxity of spinal muscles and
  ligament.
• Scoliosis lateral curvature of the spine.
• Lordosis may be seen in the lumber region while
  standing.
• Pelvis concomitant deformity (contracted inlet
  and outlet)

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• Extremities
• Broadening of epiphysis of long bone especially
  at wrist and ankle.
• Marfan sign a transverse groove over the medial
  malleolus due to defect in osteoid deposition in
  the centres of ossification
• Deformities due to weight bearing at the shaft
  of bones leading to
• Bowing of forearm in creeping infants.
• Bowlegs or knock-knees (genuvarus, genuvalgum).
• Genue recruvature (over extension) ? during
  walking.
• Non skeletal signs
• Hypotonia and laxed ligament lead to-
• Delayed motor milestones as delayed sitting,and
  walking with waddling.
• Pot belly abdomen due to weakness of abdominal
  muscles.
• Ptosis of liver and spleen due to chest
  deformity and weak abdominal muscles.

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• Complications
• -Respiratory tract infection due to chest
  deformity.
• -Tetany is uncommon with nutritional rickets.
  -Iron deficiency anemia.
• -bone deformities and fracture. -Dental
  caries.
• Diagnosis Clinical manifestations ,
  Laboratory, and Radiology
• Laboratory investigations
• -Serum calcium may be normal or low (9 -11
  mg/dl).
• -Serum phosphate level almost always is less
  than 4mg/dl (n 4.5 6.5 mg).
• -Serum alkaline phosphatase level is increased lt
  500 IU/1 (N 50Iu 200Iu /dl) due to increased
  osteoblastic activity.
• -Serum parathormone hormone (PTH) ? high. -Serum
  25 (OH)2 D ? is low.
• Non specific findings
• -Generalized aminoaciduria. - Low bone
  citrate level.
• -Elevated urinary citrate excretion. -
  Impaird renal acidification.
• -Phosphaturia and occasionaly glucosuria.

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• Radiological changes
• By X rays best seen at the lower end of long
  bone especially wrist and ankle.
• Active rickets
• -Lower end broadening -Cupping (concavity).
  - Wide joint space.
• Fraying and epiphyseal line (faint, irregular)
  indistinct.
• Shaft - Rarefaction ? decreased bone
  density.
• - Greenstick fracture may occur in the
  long bone with no symptoms.
• Healing rickets (2 3 weeks after treatment).
• - A line of preparatory calcification
  appears with no fraying.
  - Other features of active rickets are less
  evident.
• Heald rickets (after 4 weeks)
• The lower end becomes straight, thick and
  slightly irregular than normal.
• Prevention- Vitamin D supplementation Full
  term ----- 400 IU /day from the 3rd
  month.Premature ----- 1000 IU /day from 2nd week.

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• Treatment
• Both natural (sunlight) and artificial light of
  the appropriate wave length are effective
  therapeutically but administration of Vitamin D
  either orally in daily doses or in a single large
  dose is preferable (1500 I.U)for 6-8 weeks.
• Daily oral administration of 500- 1500 iu of
  Vitamin D or 0.5 2 mg of 1.25
  (OH)2 D can produce healing within 2 4 weeks
  except in Vitamin D refractory rickets.
• Alternatively, single large dose (shock therapy)
  600.000 IU given once IM and can be repeated
  every 2 4 weeks until healing occurs (maximum
  2-3 doses).

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• Other variants of rickets
• 1- Familial hypophosphatemia (Vitamin D resistant
  rickets, X linked hypophophatemia).
• Pathogenesis
• Defect in proximal tubular reabsorption of
  phosphate.
• Defect in the conversion of 25 (OH) D to 1,25
  (OH)2 D.
• Diagnosis
• Develop after 2 years of age.
• Retarted growth and bone deformities are marked.
• Laboratory finding
• Normal or slightly reduced serum calcium.
• Moderately reduced serum phosphate.
• Increased urinary excretion of phosphate.

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• 2- Renal osteodystrophy (ROD)
• - In patients developing chronic renal failure
  from glomerular or hereditary disease, may also
  have growth retardation and rickets.
• Pathophysiologic manifestations of chronic renal
  failure
• - Decreased 1 hydroxylase enzyme leading to
  impared renal synthesis of 1.25 (OH)2 D that
  lead to mineralization defect.
• - phosphate retention cause? serum calcium
  and 2ry hyper parathyoidism that lead to more
  bone resorption.
• Investigations
• - Serum calcium- decreased .
  
  
  -Serum phosphate - increased.
• - 25 (OH)2 D - increased

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• 3- Vitamin D Dependent rickets
• (pseudo Vitamin D deficiency).
• This type appears at age 3-6 months , and is a
  type of calcium deficiency
• Type I - Defect in enzyme necessary for
  formation of 1.25(OH) 2D.
• Type II (hereditary resistance) End organ
  resistance to 1.25 (OH)2 D some patients have ?
  short stature and alopecia totals.
• 4- Fanconi syndrome.
• A generalized defect in proximal tubular
  transport characterized by proteinuria,
  glucosuria, phosphatasia, aminoaciduria, and
  proximal renal tubular acidosis.

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• 5- Low's syndrome (oculocerebro renal syndrome).
• -X linked recessive disorder.- Characterized by
  congenital cataract, sometimes glaucoma, mental
  retardation and fanconi syndrome.
  
• -Blindness and renal insufficiency may develop.
• 6- Hypophosphatasia AR disorder.
• It is an inborn error of metabolism in which the
  activity of tissue (liver, bone, kidney) alkaline
  phosphatase is deficient.
• Types
• Severe infantile form (congenital lethal
  hypophosphatasia)
• Characterized by moth-eaten appearance at end of
  longs bone and marked shortening of longs bone
• Milder form (hypophosphatasia tarda) occurs in
  childhood or late adolescence may present with
  bowing of the legs with short stature.
• - Hypercalcemia in neonatal and infantile forms.
•