- Fanconi's syndrome.

"Fanconi's syndromes"

* There are many types of Fanconi's syndrome according to
etiology :-

-Primary type

-Hereditary:-

• Abderhalden-Kaufmann-Lignac syndrome "cystinosis" (click here).
• Oculo-cerebro-renal Syndrome (Lowe's Syndrome) (click here).
• Galactosemia.
• Wilson disease.

-Drug induced:-

• Out dated tetracycline.
• Cyclosporin.
• Heavy metals (e.g lead poisoning).

*Fanconi's syndrome ( primary type)*

-Def:- it is an autosomal recessive disorder due to multiple defects in proximal renal tubules which decrease the urinary re-absorption of phosphate,bicarbonate,glucose, amino acids and may be Potassium ( they all are lost in urine).

-It is named after Guido Fanconi, a Swiss pediatrician.

-Clinical picture:-

- Nutritional problems in childhood.

" DO in pairs"

Dental caries (Ca,fluorine deficiency).
Obesity

PEM (protein energy malnutrition).
Pellgra.

Aribofilavinosis.
Vit A deficiency.

Iron deficiency anaemia.
Iodine deficiency.

Rickets (Vit D deficiency).

scurvy(Vit c deficiency).

Enjoy...
Dr Ibrahim Samaha

- Manifestations of Vit A toxicity.

" Haem + CL "

Hepatosplenomegaly.
Hair (sprase and coarse).
Hypoplastic anaemia.

Alopecia.
Abnormal bone growth.

Eczema.
Erythema.

Mitosis stop,Mortality of cell ( with dose of 100000 microgram/Kg/day)
+
Clubbing of fingers.
Congenital abnormalities.

Leucopenia.

Enjoy...
Dr Ibrahim Samaha

- Important Points in Nutrition.

- Animal milk is poor in:-

*Vitamins:-

Vit D

Vit C

Thiamine (Vit B1).

*Minerals:-

Zinc

Iron


*Essential fatty acids

--------------------------------

- Vitamins syenthesized by Intestinal flora:-

Vit K2

Vit B6

Folic acid

Niacin

--------------------------------

- Nutritional Bone diseases:-

Rickets (Vit D deficiency)

Osteomalacia (Ca deficiency)

Osteoprosis

Dental Caries (Ca,fluorine deficiency)

--------------------------------

- Nutritional Anaemia:-

Iron ( hypochromic microcytic anaemia)

Vit B12 (megaloblastic anaemia)

Folic acid (megaloblastic anaemia)

Enjoy...

Dr Ibrahim Samaha

-Ten ways to reduce risk of cancer.

1- Balanced diet ( which supply the body with sufficient food stuffs,satisfy body requirement,free of toxins or infection,selected in proper portion 50-60% cHO,15-20% proteins,20-30% fat).

2-choose diet rich in fibers.

3-Increase vegetables and fruits for more than 2 servings /day.

4-Control weight,avoid obesity.

5-Excercise regularly.

6-limit highly salted food.

7-limit smoky and  pickled food.

8-Decrease fat intake.

9-Stop alcohol.

10-Stop smoking with its different forms (cigarettes,Bidi,Cigar,Goza ,Hook-ah,Hubble-bubble,Tobacco chewing,Snuff,pipe).

Dr Ibrahim samaha

- Dietary Protein.

Dietary Protein

Chemistery :-

Proteins are made of strings of amino acids that form chains known as peptides.
Proteins differ by the types and order of amino acids they contain. Even though there are only 20 amino acids, they create almost endless variations in chains as long as 500 links.

site of synthesis:-

Proteins form inside animals (including humans) and plants through processes that synthesize peptides. For humans, we cannot synthesize certain "essential protein," and so we must ingest them through food. These essential proteins are made of phenylalanine, threonine, methionine, tryptophan, leucine, isoleucine, lysine, and valine amino acids.

Functions pf proteins:-
Our bodies need dietary protein to accomplish many basic functions, such as:-
1- building bones.
2- moving muscles.
3- repairing tissue.
4- a catalyst in metabolism.
5- They're involved in the nervous system function.
6- bringing energy to cells.

Dietary protein, an essential nutrient, comes from meat, dairy, and certain grains and beans.

Food from plants, like corn, have incomplete protein, which means they do not contain all the necessary amino acids. Only food from animals, such as cheese and fish, provide complete protein, and don't need to be combined with other protein sources.

Examples of complete protein foods are :-

milk, cheese, yogurt, butter, poultry, pork, or any meat. Incomplete proteins include oats, wheat, pasta, lentils, nuts, rice, soy, pears, and seeds. Eating a combination of complementary protein sources, such as grains mixed with legumes, results in a diet of essential protein. This is how vegetarians and vegans maintain health without eating meat or dairy.

Dietary requirements:-
vary from 40-70 gm of protein per day.

Disturbances:-
1- Too much protein might deplete calcium,
2- while too little causes a form of malnutrition called kwashiorkor. Insufficient protein weakens the heart and other muscles, eventually leading to death.
3- Incidentally, proteins are responsible for most food allergies.

With my best wishes

Dr-hema