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Sunday, May 15, 2011

A Family Doctor's Tale -G6PD DEFICIENCY

DOC I HAVE G6PD DEFICIENCY

G6PD Deficiency  is an inherited disorder of the Red blood cells which has a lack of the glucose-6-phosphate dehydrogenase enzyme.

This causes the red blood cells to burst (hemolysis) in certain circumstances when certain food, herbs or medicines are taken.

It is a lifelong disease and there is no cure for it.

10 per cent of the world population is believed to have it.


It is  more common in Asians and Africans, less so in Caucasians.

There are 2 types of G6PD Deficiency:
1.G6PD Deficiency major which is a serious illness which occurs as a sex linked genetic disease affecting the males.

2.G6PD Deficiency minor occurs in the females and can also cause red blood cells break up as in the the major form of the disease.

Here the female can pass the gene to the male child(resulting in the major illness) and the female child(resulting in the female child as the carrier of the gene).

Not all mothers with the gene will pass it to the son or daughter.

The  risk of G6PD Deficiency is:
G6PD Deficiency results from a defective gene which provides for the enzyme in the red blood cell which preserve the integrity of the red blood cell.

When the child takes certain food, herbs or chemicals, the absence of the enzyme cause the red blood cell to burst resulting in hemolytic anemia, release of bilirubin and passing of blood through the urine.

If both parents has this faulty gene then the male child will have G6PD Deficiency major and the female child may have the minor illness which allows her to pass the gene to her son.

If the female parent has the faulty gene then the child may have the gene passed to him or her.

If only male parent has the faulty gene then the child will not have the gene passed to him or her.

The red blood cells are normal sized and breaking up easily under certain circumstances to cause a severe anemia.

Triggers which can cause an attack of red blood cells breakup (hemolysis) in G6PD Deficiency are:
1. Certain food - fava beans (also known as broad beans)


2. Chinese herbs especially Huang Lian

3. Medicines
a. Sulphonamides, septrin

b. Antimalaria drugs such as chloroquine, quinine,

c.analgesics such as aspirin,

d.Non-sulphonamide antibiotic such as nalidixic acid, nitrofurantoin, isoniazide, dapsone

4.naphthalene or moth balls

5.some bacterial or viral infections


The symptoms of hemolysis in G6PD Deficiency are:
1.Anemia - pale

2.blood in the urine

3.vomiting

4.abdominal pain

5.Slight jaundice

6.rapid heart beats , lethargy and symptoms of shock


Diagnosis of G6PD Deficiency is often based on
1. blood test for G6PD deficiency - rapid fluorescent spot test detecting the generation of NADPH from NADP

2. microscopic examination of red blood cells(Heinz bodies can be seen in G6PD deficient patients red blood cells)


3. Genetic analysis

The complications of hemolysis in G6PD Deficiency are:

1. Anemia

2. damage to liver

3. shock and death

The treatment of hemolysis in G6PD Deficiency is:
1.Blood transfusion

2.treatment for shock

3.Folic acid to build up the blood


The prognosis of hemolysis in G6PD Deficiency is:

Prognosis is good if treatment is early.

The patient must take care of himself or herself and remembers what are the food, medicines or herbs he cannot take.

Prevention of G6PD Deficiency is by:
1.testing cord blood for G6pd deficiency at birth


2.patient education of his condition and avoidance of certain food or medicines.

Friday, May 13, 2011

A Family Doctor's Tale -EDEMA

DOC I HAVE EDEMA
Edema is a symptom defined as excess of fluid (water and sodium) in extracellular spaces that include a large number of medical conditions.

It usually in the ankle and foot but can spread up the legs to the abdomen where the excessive fluid is known as ascites.

The abdomen will appear bloated.

It can also spread to the lungs causing pulmonary edema and breathlessness.

Of course edema of the face will cause puffiness of the face .

Generalized edema is called anasarca.

The Causes of Edema are:

1.Cardiac edema from right or left heart failure

2.Renal edema from renal failure or nephrotic syndrome


3.Hepatic edema from liver failure or cirrhosis

4.endocrine edema from cushing  syndrome or use of  steroids

5.malnutrition syndrome - lack of protein leads to edema

6.Pregnancy pressure on the lower limbs blood vessels

7.Standing too long resulting in gravity pulling fluid to the feet and ankles

8.local edema from obstruction such as

a.venous obstruction - varicose veins,

b.lymphatic obstruction from filaria or other parasites

c. tight garments

d.local injuries,

e.carcinomatous obstruction

The Signs and Symptoms of Edema are:
Symptoms:

1.The skin of the lower legs may be swollen, stretched and shiny.

2.Gentle pressure on the swollen skin will cause a depression in the swollen area.

3.Abdomen may be swollen or distended due to accumulation of fluid

4.Crepitation can be heard at the bases of the lungs suggesting fluid in the lower prt of the lungs

5. The face may be swollen and puffy

The Complications of edema are :


1.overload of fluid in the body leading to overload on the heart

2.too much fluid in the lungs can cause breathlessness

3.renal failure can be dangerous and may need dialysis

4.Liver failure is also dangerous and may require a liver transplant

The Treatment of Edema is usually by:
1.Medicines like diuretics can force the fluid out through the urine.

The diuretic should be cautiously used in renal cases.


2.Treat the underlying cause whether it is heart , kidney or liver failure.

3.Careful institution of high protein feedings in malnutrition cases

4.surgery may be required  to remove obstruction

5.chemotherapy may also be required in carcinomatous obstruction

General Measures:
1.No strenuous exertion

2.Avoid stress

3.Any breathlessness must be treated quickly


4.Reduce the salt intake

5.Raise the legs when sitting down or lying down

6.Do not stand for long periods

Prognosis of edema:

Mild edema due to excess salts or medicines can be treated easily by reducing the salt or medicine.

Underlying diseases like heart failure, renal failure or liver failure may need specific medication, treatment and may not be completely cured.

Prevention of edema is by:

1.avoid too much salt

2.avoid standing or sitting for too long a period

3.Pregnancy can cause edema of the legs due to pressure of the fetus on the major veins of the legs - avoid over exertion and salty food

Wednesday, May 11, 2011

A Family Doctor's Tale - CARDIOGENIC SHOCK

DOC I HAVE CARDIOGENIC SHOCK
Cardiogenic shock occurs when there is a sudden reduction of cardiac muscle contractibity and blood output from the heart following myocardial infarction or heart attack.

Cardiogenic shock can lead to sudden death.

Cardiogenic shock occurs when
1.Heart attack -there is a sudden interference with the pumping action of the heart from ischemic heart muscle damage ofeten involving 50 per cent of the left ventricle.

2.Injury to the heart muscle from trauma such as knife wounds,direct impact of heavy object on the heart.

3.Drug overdosage such as beta blockers or calcium antagonist affecting the slowing of heart and poor output of blood.

The symptoms of Cardiogenic shock are :
1.Pale, sweaty patient with rapid weak pulse

2.Severe hypotension

3.Urinary output reduced

4.Breathlessness and pulmonary edema

5.Fatique and tiredness

6.Confusion,dizziness,

Diagnosis of Cardiogenic shock is usually confirmed by:
1.History of chest pain, shock and physical examination showing low blood pressure

2.ECG(electrocardiogram)shows typical features of large depression in Q wave, ST segment and raised RS segment. A 12 leads ECG may showed the presence of severe myocardial infarct

3. Blood cardiac enzymes and ESR may be raised.

Any cardiogenic shock is an EMERGENCY!
Immediate treatment is urgent!
Admit to hospital as an emergency.

Severe cases are admitted to Cornary care unit(CCU) for constant monitoring of the heart, blood pressure and abnormal rhythm of heart rate.

Intravenous fluids such as glucose should be given on admission together with insulin to help raise the blood pressure.

Blood pressure can be further raised with dopamine or dobutamine.

Vasodilators like isorbide are give to help dilate the artery to the heart

Diuretics may be necessary to relieve congestion of the lungs and edema.

Any abnormal rhythm of the heart must also be treated with medications or pacemaker if severe as damage to heart may affect the conduction of the electrical impulse of the heart to the cardiac muscles.

Because of the psychological effect of a heart attack on the patient, sometimes antidepressant or tranquilliser may be given.

Interventional Procedures:
--------------------------
Once stable the patient may be requred to have a ballooning of the narrowed artery or a stent inserted in the narrowed artery. This can be done during the cardiac catheterisation.
1.balloon angioplasty
balloon is inflated to compress fatty matter in the wall of narrowed artery and dilate the blood vessel
2.Stent:
balloon angioplasty is performed in combination with placement of a stent which is a small, metal mesh tube that provide support inside the coronary artery.
3.drug eluting stents (DES):
Drug-eluting stents contain a medication that is actively released at the stent implantation site to prevent recurrence of narrowing of the artery
4.rotablation
The Rotoblation special catheter, with an acorn-shaped, diamond-coated tip,  spins around at a high speed and grinds away the heavily calcified  plaque on the arterial walls.
5.cutting balloon
The cutting balloon catheter has a balloon tip with small blades which are activated  when the ballon is inflated. The small blades remove the plaque and the balloon compresses the fatty matter into the arterial wall.

Surgery :
-------------
If the narrowing involved too many arteries, then a coronary artery bypass graft (CABG) surgery will have to be done.

Usually a heart attack patient stays in hospital for 2-4 weeks depending on the severity of his condition.

Mild exercise is started once his condition is stable. Exercise is good for the patient because it helps the blood circulation.


Prognosis of cardiogenic shock depends on the degree of ventricular recovery.

Treatment is aimed at maintaining circulation until some recovery takes place.

Overall prognosis is poor.

Prevention of a cardiogenic shock is the same as prevention of a heart attack  or a stroke as both involve the avoiding the blockage of a major artery to the brain or heart.

1.Control the Blood Pressure


2.Control the Diabetes


3..Control the diet.


4.Check with the doctor regularly.

5.Don't Smoke

6.Regular exercise is good for you.


7.Learn To Relax

8.Time management is important.

Tuesday, May 10, 2011

A Simple Guide to Tropical Sprue

A Simple Guide to Tropical Sprue


A Simple Guide to Tropical Sprue
————————————————–
What is Tropical Sprue?
—————————————
Tropical Sprue is a malabsoption disease of unknown etiology associated with temporary or permanent stay in the tropics and characterized by abnormalities in the lining of the small intestine.
The small bowel mucosa shows
1.villous atrophy
2.columnal to cuboidal changes in absorbing cells
3.infiltration of lamina propia with lymphocytes, plasma cells and eosinophils
Who is affected by Tropical Sprue? ———————————————————————————————-
Tropical Sprue cause is unknown but some possible causes are: 1.Nutritional deficiency
2.intestinal infection
There is a possibility of enzyme deficiency and abnormal immunological response.
Tropical Sprue can be found in all ages but are more common in the 10-30 years age group.
What are the Symptoms and signs of Tropical Sprue? ————————————————————————————-
Symptoms varies from mild to severe:
Early symptoms are:
1.fatigue
2.bulky stools
3.Weight loss despite good appetite and intake
After some months malnutrition is noted with evidence of malnutrition syndrome:
1.weight loss
2.glossitis
3.stomatitis
4.pigmentation
5.edema
5.diarrhea and steatorrhea
6.megaloblastic anemia
7.iron,folic acid and vitamin b12 deficiency
The Diagnosis of Tropical Sprue is confirmed by: ————————————————————————————
1.A history of abdominal pain , diarrhea and weight loss
2.small bowels radiology
a.thickening and coarsening of mucosal folds
b.dilatation of smaa intestinal lumen
c.flocculation and segmentation of barium
3.tests for malabsorption syndrome 4.fecal fat assessment
5.Jejunal biopsy
What are the complications of Tropical Sprue? ————————————————————————————-
1.weight loss
2.Nutrition: malabsorption and vitamin deficiency
What is the treatment of Tropical Sprue? —————————————————————————————————
Treatment is :
Control of Diet
1.Folic acid is given for at least 6 months
2.Vitamin B12 injection is given
3.food containing iron, folic acid and vitamin B12 are are given
4,Enough protein supplement
5.lots of fluids
Medication
1.Antispasmotic medication for abdominal pain
2.lomotil or imodium to suppress diarrhea
3.Tetracycline is given daily for at least 6 months
Start with high doses at first, followed by reduction of dosage.
4.Correction of anemia and nutritional deficiencies is important to enhance the immune system
What is the prognosis of Tropical Sprue? ———————————————————————————————-
The prognosis is usually very good Treatment is very effective in producing weight gain and correcting nutritional deficiency.
Abnomal morphology and bowel function may persist despite treatment
There may be spontaneous remission especially leaving the tropics.
What are preventive measures in Tropical Sprue? ————————————————————————–
A nutritious diet with vitamin supplements can strengthen the body resistance against illness.
Avoid the tropics.

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