VITAMIN E, VITAMIN K , HYPOPHOSPHATASIA, PSEUDO HYPOPHOSPHATASIA.

1. 1. VITAMIN E
2. VITAMIN K
3. HYPOPHOSPHATASIA
4. PSEUDO HYPOPHOSPHATASIA
Overview

2. VITAMINS
 Vitamins or “vital
amines” are essential
food factors required
in the diet in small
amounts to do
specific biological
functions to maintain
normal growth and
health of an
organism.

3. 1.
2.

4. VITAMIN E
 Tocopherol aka “the alcohol which brings forth offspring”
 Vitamin E consists of 8 naturally occurring tocopherols of
which α-tocopherol is the most active.
 FUNCTIONS:
1. Prevent peroxidation of polyunsaturated fatty
acids .
2. Anti-oxidant.
3. Aids in the formation of RBC’s.
4. Anti-cancer. (γ- tocopherol )

6. Daily Requirements
 Infant 3mg
 Adult 10mg
Deficiency is very rare as our daily intake is approx. 15 mg per day.
Deficiency
 Increased fragility of RBCs
 Degeneration of neurons – chronic cholestatic liver disease
 muscle weakness, degeneration of retina- abetalipoproteinemia
In animals
 decreased male sterility
 vit-E deficient rats -loss of pigmentation and atrophic, degenerative changes
in enamel organ is seen.

7. Treatment
 Replacement therapy
 Dietary supplements

8.  Moisturizing
 healing
 anti inflammatory
 anti aging

9. Vitamin
 Also called as “Koagulation vitamin”.
 Two natural forms of vitamin K
 K1- phylloquinone - derived from vegetables and animal
source
 K2- menaquinone – synthesized by bacterial flora and
found in hepatic tissue.
 One synthetic form K3 – menadione which is water
soluble.

11.  Vitamin K is necessary for the post transitional carboxylation
of glutamic acid necessary for calcium binding to gamma
carboxylated proteins such as prothrombin, factors VII, IX, X,
protein C, protein S and proteins found in bone.

13. Functions
Role of
vitamin K in
coagulation.

14. The ability to bind calcium ions is acquired by the activation of
vitamin K dependent clotting factors or proteins in clotting
cascade.

15. Other functions of vitamin K
 Protects bones from weakening
or fracture
 Prevents calcification of blood
vessels or heart vessels

16. Daily requirements
Deficiency
1. In new-borns : bleeding into skin
blood in stool can be seen.
2. In adults : Secondary hypovitaminosis K- due to
impaired fat absorption or
ulcerative colitis or
obstructive jaundice.
 1-2 mcg per kg
 But our dietary intake is approx. 300-500 mcg ,
which is more than enough to meet daily
requirements

17. Clinical Manifestations
Oral manifestation :
gingival bleeding

18. Diagnosis : elevated prothrombin time
reduced clotting factors.
Treatment
new-borns are given vitamin K injection.
Dierary supplements.

19. Hypophosphatasia
 It is a rare metabolic bone disorder characterised by a
deficiency of tissue non specific alkaline phosphatase.
 The main features include the following:
1. Reduced levels of bone, liver and kidney isoenzyme of alkaline
phosphatase.
2. Increased levels of blood and urinary
phosphoethanolamine.(inhibit mitochondrial function)
3. Bone abnormalities that resemble rickets.

20. I. Perinatal hypophosphatasia
 Most severe
 Infant rarely survives- death is due to respiratory failures.
 Hypo calcification of skeletal structures.

21. II. Infantile hypophosphatasia
 Appear normal up to 6 months of age- then show a failure to grow.
 Vomiting and hypotonia.
 Skeletal malformations – shortened and bowed limbs.
 Deformities of ribs- rachitic rosary- which predisposes patients to
pneumonia.
 Nephrocalcinosis and nephrolithiasis
 H/P: abundant production of poorly mineralised osteoid

22. III. Childhood hypophosphatasia
 Premature loss of primary teeth without evidence of a
significant inflammatory response.
 Enlarged pulp chambers and alveolar bone loss
 Open fontanelles with premature fusion of cranial
sutures- increased intracranial pressure and
subsequent brain damage.
 Short stature, bowed legs and waddling gait.
 H/P: woven bone (less mature form of osseous tissue)
maybe seen, absence of cementum that
covers the root surface.

23. Radiograhically
“beaten copper”
appearance of skull

24. IV. Adult hypophosphotasia
 Many patients are edentulous- loss of permanent and deciduous teeth
 Stress fractures that involve metatarsal bone of the feet
 H/P: woven bone maybe seen

25. Treatment and prognosis
 Symptomatic treatment
 Orthopaedic surgery for fractures, prosthetic appliances for missing teeth
 Genetic counselling
 Perinatal and infantile- poor prognosis
 Childhood and adult- better prognosis
Diagnosis
 Clinical manifestations
 Decreased levels of serum alkaline phosphatase
 Increased amounts of phosphoethynylamine in urine and
blood

26. Pseudohypophosphotasia
 Resembles hypophosphotasia but with a normal serum
alkaline phosphatase level
 Hereditary
 Premature loss of deciduous tooth, hypotonia.
 Treatment – symptomatic