1. VITAMIN E2. VITAMIN K 3. HYPOPHOSPHATASIA4. PSEUDO HYPOPHOSPHATASIA

Overview

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.

1.

2.

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 )

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.

Treatment Replacement therapy Dietary supplements

Moisturizing healing anti inflammatory anti aging

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.

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.

FunctionsRole of vitamin K in coagulation.

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

Other functions of vitamin K Protects bones from

weakening or fracture Prevents calcification of

blood vessels or heart vessels

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

Clinical Manifestations

Oral manifestation : gingival bleeding

Diagnosis : elevated prothrombin time reduced clotting factors.

Treatment new-borns are given vitamin K injection.Dierary supplements.

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.

I. Perinatal hypophosphatasia Most severe Infant rarely survives- death is due to respiratory

failures. Hypo calcification of skeletal structures.

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

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.

Radiograhically “beaten copper” appearance of skull

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

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

Pseudohypophosphotasia Resembles hypophosphotasia but with a normal

serum alkaline phosphatase level Hereditary Premature loss of deciduous tooth, hypotonia. Treatment – symptomatic