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Tuesday, April 19, 2011

A Family Doctor's Tale - ADDISON DISEASE

DOC I HAVE ADDISON DISEASE

Addison Disease is a  disease which is caused by the deficiency of  circulating glucocorticoids steroids and mineralocorticoids (aldosterone) hormones as a result of disease of the adrenal glands.

The causes are believed to be:
1. Auto-immune disease of the adrenals with destruction of the adrenal parenchyma is associated with circulating antibodies and decreased production of glucocorticosteroids.

2.Tuberculosis of the adrenal glands

3.Adrenal tumors which may be benign or malignant can reduce production of corticosteroids.

4.Rare causes are metastatic carcinoma, amyloidosis and hemachromatosis

5.Iatrogenic -sudden cessation of chronic corticosteroids or failure to increase therapy in corticosteroid dependent patients undergoing stress, surgery or infection.

Symptoms of Addison Disease are:

A.Acute Addison Crisis
The symptoms are :
1.vomiting


2.abdominal pain


3.back and extremity pain


4.extreme muscle weakness


5.dehydration leading to hypotension


6.shock


7.confusion


8.coma


Addison crisis is fortunately rare

B.Chronic Addison disease:
1.Chronic adrenal hypofunction begins gradually

2.fatique

3.loss of appetite and weight

4.abdominal pain.

5.vomiting

6.dizziness

7.craving for salty food

8.increased pigmentation especially palmar flexures, nipples, genitalia, mouth lining, scars and exposed areas.

9.postural hypotension is common with giddiness

10.occasionally hypoglycemic symptoms may occur in early morning or when a meal is missed

Diagnosis of Addison Disease:
1.The increased pigmentation and loss of weight can suggest the diagnosis of Addison Disease

2.Electrolytes especially sodium is low, potassium and urea nitrogen is high

3.ACTH stimulation test with impaired corticol response is required to confirm diagnosis

4.Plasma adrenal antibodies may be present

5.Chest and abdominal X-rays for tuberculosis

Complications of Addison Disease:
1.coma from Addison's crisis

2.Hypotension

Treatment of Addison Disease:
1.Addison crisis is treated by intravenous normal saline and hydrocortisone 100mg every eight hours
Treatment must be done before laboratory confirmation of
diagnosis because of the danger of coma

2.Chronic Addison disease requires replacement of glucocorticoid:
a.hydrocortisone 30mg daily or
b.cortisone acetate 37.5 mg daily or
c.fludrocortisone 0.05 to 0.2 mg daily

Prognosis of Addison Disease:
The prognosis of Addison Disease is usually very good.

Addison crisis patients have excellent recovery with appropriate treatment

Chronic Addison disease patients also recovered well with replacement therapy.

Care is needed when there is trauma, infection and surgery.

Sunday, April 17, 2011

A Family Doctor's Tale - CUSHING SYNDROME

DOC I HAVE CUSHING SYNDROME

Cushing Syndrome is a  disease which is caused by the exposure of the body to excessive quantities of glucocorticoids steroids.

People of all ages are affected but more are seen in females between 30 to 50 years of age.

The causes are believed to be:
1. Cushing Disease -excessive production of adrenocorticotrophin hormone(ACTH) by the pituary gland causes bilateral adrenal(gland above kidney)hyperplasia(overgrowth of gland tissue).The enlarged adrenal gland will produce excessive corticosteroids.
A pituitary basophil or chromphobe adenoma may be present.

2.Adrenal tumors which may be benign or malinant can produce excessive corticosteroids

3.Ectopic ACTH syndrome
Production of ACTH by a tumor such as cancer of the lungd leads to adrenal hyperplasia

4.Iatrogenic -patients receiving excessive doses of corticosteroids may present as Cushing syndrome

Symptoms of Cushing Syndrome:
1.Obesity predominally truncal often with pad of fat between shoulders(buffalo hump)

2.Round flat plethoric(red) face (moon face)

3.Skin is thin and bruises easily

4.Purole striae on abdomen, thighs, and shoulders.

5.Proximal myopathy leads to difficulty in getting out of chairs and walking upstairs

6.Excessive adrenal androgen secretions leads to hirsutism, amenorrhea and acne

7.Osteoporis can lead to back pain

8.Hypertension and glycosuria(glucose in urine) are common

9.Depression and other psychiatric disturbance common

10.In children there may stunting of growth

Diagnosis of Cushing Syndrome:
1.The typical moon face and buffalo hump can suggest the diagnosis of Cushing Syndrome

2.24 hour urine cortisol tests

3.Overnight dexamethasone suppression test

4.Plasma ACTH level

5.Chest X-ray to exclude lung cancer

6.X-ray of pituitary fossa to exclde pituitary tumors

7.Ultrasound of adrenal glands for enlargement

Complications of Cushing Syndrome:
1.Hypertension

2.fractures from osteoporosis

3.stunted growth in children

Treatment of Cushing Syndrome:
1.Anti-sdrenal drug such as aminogluthemide

2.bilateral adrenalectomy

3.Surgery to remove pituitary tumor

4.surgery to remove primary neoplasm in lungs or pancreas

Prognosis of Cushing Syndrome:
The prognosis of Cushing Syndrome varies with cause.

The response is often excellent in cases of pituitary tumor removal. However recurrence of tumor or increase in size of the tumor will affect the prognosis.

Benign adrenal tumors have excellent response to treatment.

Iatrogenic Cushing syndrome often responds well to decreasing doses of corticosteroids.

In cases of lung cancer, treatment may have poor response.

Friday, April 15, 2011

A Family Doctor's Tale - NEPHROTIC SYNDROME

DOC I HAVE NEPHROTIC SYNDROME

Nephrotic Syndrome is a rare disease characterized by the edema(accumulation of fluid in the body) and hypo-albumemia (low blood protein due to protein loss through the kidneys).

The cause is the increased capillary permeability of the glomeruli to plasma protein  due to the inflammation and allows loss of blood and protein in the urine and retention of salt, water and nitrogen.

Loss of negative charges on the filtering membrane allows negatively charged molecules including albumin to enter the urinary tract.

Reduced protein in the blood leads to edema.

There are 3 types of Nephrotic Syndrome:
1.Minimal Change Nephropathy (MCN) occurs in children with very little microscopic changes in the glomerular tissues of the kidney.

2.Focal glomerulonephritis has appearance of MCN above but develop sclerosis of the glomeruli later.

3.Diffuse proliferative glomerulonephritis and membranous nephropathy are more common in adults and be secondary to other diseases such as:


a.SLE and other connective tissues
b.diabetes
c.allergies to drugs such as penicillinamine, tolbutamide, probenecid
d.certain infections
e.cancer (carcinomas and lymhomas)

Symptoms:
1.Urine may show the presence of protein at least 3.5g/day

2.low protein in blood causes decreased plasma mass pressure resulting in retention of water and salts

3.swelling of the face and ankle occurs as a result

4.edema ranges from puffiness of eyes, ascites (water in abdominal cavity), pleural effusions(water in the lungs) to generalized edema

5.With increasing edema and nitrogen retention, headaches, nausea and vomiting may occur.

6.high blood pressure may occur in one third of patients

7.The blood in nephrotic syndrome tend to clot easily and the patient may develop blood clots in his leg veins.

8.Lipids may be raised in the blood especially triglicerides and cholesterol

9.Symptoms and signs of underlying diseases such as diabetes, SLE etc may be present

Diagnosis:
1.A history of recent kidney infection followed by swelling of face ang legs

2.Urinary protein loss of 15g per day

3.Full examination of fresh urine for red blood cells and casts(indicating glomerulonephritis) and lipid

4.Full blood count and tests for kidney function

5.Ultrasound of kidneys

6.Biopsy of kidney and histology of specimen

Complications:
1.Renal failure

2.hemolytic uremic stndrome

Treatment should be in hospital:
1.Minimal change nephropathy in children are treated with high doses of corticosteroids up to 1mg prednisolone per kg per day.
In most cases within 10 days there should be reduction of fluid in the body with increased urinary passage of urine.
Relapses may occur and should be treated with more corticosteroids or cyclophosphamide

2.In adults treatment with corticosteroids should help to treat the edema.

3.In addition thiazide diuretics or frusemide to reduce edema.
Spironolactone and amiloride may also help

4.Hypertension may require anti hypertensive. The use of corticosteroids may aggravate hypertension.

5.Diet with low salt and protein

6.Intake of fluids are restricted
These 2 measures will reduce the strain on the kidneys and prevent accumulation of fluids

Prognosis:
1.In most cases of MCN the illness will clear up with proper treatment.

Some may have relapses.

After 3 years of remission 99% of MCN will not have relapses
MCN cases seldom progress to renal failure.

2.In focal glomerulonephritis cases nephrotic syndrome may develop into chronic renal failure.

3.In Membranous nephropathy
25% complete remission
25% improves in renal function
25% have persistent nephrotic syndrome
25% will have chronic renal failure

Wednesday, April 13, 2011

A Family Doctor's Tale -GLOMERULONEPHRITIS

DOC I HAVE GLOMERULONEPHRITIS

Glomerulonephritis is a disease characterized by the inflammation of the glomeruli, the filtering units of the kidneys.

The glomeruli are unable to function properly due to the inflammation and allows loss of blood and protein in the urine and retention of salt, water and nitrogen.

Both kidneys are affected usually.

It is not contagious.

The exact cause of Glomerulonephritis is not known.

1.It has been suggested that an autoimmune disease is the main cause of Glomerulonephritis disease.

The antibodies produced by the body to fight germs starts to attack the body's own healthy kidney tissue.

2.Sometimes Glomerulonephritis disease may follow an infection by the streptococcus bacteria or by viruses.

Symptoms of Glomerulonephritis disease:
1.Urine may show the presence of protein, red blood cells, casts.

2.less urine is passed than normal

3.swelling of the face and legs is quite common

4.high blood pressure may occur in rare cases

5.With increasing edema and nitrogen retention, headaches, nausea and vomiting may occur.

Diagnosis of Glomerulonephritis disease is made by:

1.A history of recent infection followed by swelling of face and legs

2.Urine test for blood, protein and casts

3.Full blood count and tests for kidney function

4.Blood Tests for raised ESR,  anti-streptolysin titer, C reactive protein

5.Ultrasound of kidneys

6.Biopsy of kidney if required

Complications of Glomerulonephritis disease are:
1.Nephrotic syndrome

2.hemolytic uremic syndrome

3.renal failure

Treatment of Glomerulonephritis disease is by:
1.Diet with low salt and protein

2.Intake of fluids are restricted
These 2 measures will reduce the strain on the kidneys and prevent accumulation of fluids

3. antibiotics are given if the glomerulonephritis is suspected to be caused by a bacteria

4.hypertension is usually controlled with drugs until the blood pressure returns to normal

5.Corticosteroids and cytotoxic drugs have not been found to be effective against glomerulonephritis

Prognosis of Glomerulonephritis disease:
In most cases the illness will clear up with proper treatment.

In rare cases nephrotic syndrome may develop with ensuing chronic renal failure.

Monday, April 11, 2011

A Family Doctor's Tale - THREADWORMS

DOC I HAVE THREADWORMS


Thread worms are parasitic worms (also called pin worms) which are found in the intestines of children in undeveloped countries.

It is rare in developed countries because of the modern sanitary conditions and better hygiene.

Several members of the same household may be infested at the same time so all the family should be treated together.

The cause of Threadworms infection is:
Threadworms eggs or larva are present on the hands or food prepared by people who has threadworms infestations.

When the eggs or larvae are swallowed  they developed into adult worms in the intestines.

The adult worms will cling on to the mucosal lining of the intestine and absorb nutrients from the food taken into the intestines by the affected person.


The female worms emerge from the rectum at night to lay their eggs on the skin around the anus.

The main symptoms of Thread worms are
1. Itch in the anal region especially at night when the worms lay the eggs.

2. Itch in the vulva in girls

3. inflammation of the anus as a result of constant scratching.

4.Rarely tiny white worms can be seen wriggling in the feces.

Doctors generally diagnose Thread worms based on:

1.sticky tape pressed to the anal area in the morning before the patient bathes or go to the toilet will collect the eggs for microscopic examination and confirmation of the presence of thread worms

2.Stool examination and culture

Treatment of thread worm infestation is by the use of anti-parasitic medicine:
1.Zentel or Abendazole 400mg in a single dose

2.Pyrantel pamoate 10mg/kg in a single dose

3.Mebendazole 100mg in a single dose

A single dose will usually cure the patient of thread worms but to prevent re-infection a second dosage is taken 2 weeks later.

All the family members should also be treated.

Prevention of thread worms is through:
1.good personal hygiene and hand washing

2. good food hygiene

Prognosis of thread worms treatment is excellent.

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