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Cholangitis

Definition:
Cholangitis is an infection of the common bile duct, the tube that carries bile from the liver to the gallbladder and intestines. Bile is a liquid made by the liver that helps digest food.

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Cholangitis can be life-threatening, and is regarded as a medical emergency. Characteristic symptoms include yellow discoloration of the skin or whites of the eyes, fever, abdominal pain, and in severe cases, low blood pressure and confusion. Initial treatment is with intravenous fluids and antibiotics, but there is often an underlying problem (such as gallstones or narrowing in the bile duct) for which further tests and treatments may be necessary, usually in the form of endoscopy to relieve obstruction of the bile duct.
Symptoms:
The following symptoms may occur:

*Pain on the upper right side or upper middle part of the abdomen. It may also be felt in the back or below the right shoulder blade. The pain may come and go and feel sharp, cramp-like, or dull.

*Fever and chills

*Dark urine and clay-colored stools

*Nausea and vomiting

*Yellowing of the skin (jaundice), which may come and go
Physical examination findings typically include jaundice and right upper quadrant tenderness.Charcot’s triad is a set of three common findings in cholangitis: abdominal pain, jaundice, and fever. This was assumed in the past to be present in 50–70% of cases, although more recently the frequency has been reported as 15–20%.Reynolds’ pentad includes the findings of Charcot’s triad with the presence of septic shock and mental confusion. This combination of symptoms indicates worsening of the condition and the development of sepsis, and is seen less commonly still.

In the elderly, the presentation may be atypical; they may directly collapse due to septicemia without first showing typical features. Those with an indwelling stent in the bile duct (see below) may not develop jaundice.

Causes:
Cholangitis is most often caused by a bacterial infection. This can occur when the duct is blocked by something, such as a gallstone or tumor. The infection causing this condition may also spread to the liver.

Bile duct obstruction, which is usually present in acute cholangitis, is generally due to gallstones. 10–30% of cases, however, are due to other causes such as benign stricturing (narrowing of the bile duct without an underlying tumor), postoperative damage or an altered structure of the bile ducts such as narrowing at the site of an anastomosis (surgical connection), various tumors (cancer of the bile duct, gallbladder cancer, cancer of the ampulla of Vater, pancreatic cancer, cancer of the duodenum), anaerobic organisms such as Clostridium and Bacteroides (especially in the elderly and those who have undergone previous surgery of the biliary system). Parasites which may infect the liver and bile ducts may cause cholangitis; these include the roundworm Ascaris lumbricoides and the liver flukes Clonorchis sinensis, Opisthorchis viverrini and Opisthorchis felineus. In people with AIDS, a large number of opportunistic organisms has been known to cause AIDS cholangiopathy, but the risk has rapidly diminished since the introduction of effective AIDS treatment. Cholangitis may also complicate medical procedures involving the bile duct, especially ERCP. To prevent this, it is recommended that those undergoing ERCP for any indication receive prophylactic (preventative) antibiotics.

The presence of a permanent biliary stent (e.g. in pancreatic cancer) slightly increases the risk of cholangitis, but stents of this type are often needed to keep the bile duct patent under outside pressure

Diagnosis:
Routine blood tests show features of acute inflammation (raised white blood cell count and elevated C-reactive protein level), and usually abnormal liver function tests (LFTs). In most cases the LFTs will be consistent with obstruction: raised bilirubin, alkaline phosphatase and ?-glutamyl transpeptidase. In the early stages, however, pressure on the liver cells may be the main feature and the tests will resemble those in hepatitis, with elevations in alanine transaminase and aspartate transaminase.

Blood cultures are often performed in people with fever and evidence of acute infection. These yield the bacteria causing the infection in 36% of cases, usually after 24–48 hours of incubation. Bile, too, may be sent for culture during ERCP (see below). The most common bacteria linked to ascending cholangitis are gram-negative bacilli: Escherichia coli (25–50%), Klebsiella (15–20%) and Enterobacter (5–10%). Of the gram-positive cocci, Enterococcus causes 10–20%.

You may have the following tests to look for blockages:

*Abdominal ultrasound

*Endoscopic retrograde cholangiopancreatography (ERCP)

*Magnetic resonance cholangiopancreatography (MRCP)

*Percutaneous transhepatic cholangiogram (PTCA)

*You may also have the following blood tests:

#Bilirubin level
#Liver enzyme levels
#Liver function tests
#White blood count (WBC)
Treatment:
Quick diagnosis and treatment are very important.Antibiotics to cure infection is the first treatment done in most cases. ERCP or other surgical procedure is done when the patient is stable.Patients who are very ill or are quickly getting worse may need surgery right away.

Cholangitis requires admission to hospital. Intravenous fluids are administered, especially if the blood pressure is low, and antibiotics are commenced. Empirical treatment with broad-spectrum antibiotics is usually necessary until it is known for certain which pathogen is causing the infection, and to which antibiotics it is sensitive. Combinations of penicillins and aminoglycosides are widely used, although ciprofloxacin has been shown to be effective in most cases, and may be preferred to aminoglycosides because of fewer side effects. Metronidazole is often added to specifically treat the anaerobic pathogens, especially in those who are very ill or at risk of anaerobic infections. Antibiotics are continued for 7–10 days. Drugs that increase the blood pressure (vasopressors) may also be required to counter the low blood pressure.
Prognosis:
Acute cholangitis carries a significant risk of death, the leading cause being irreversible shock with multiple organ failure (a possible complication of severe infections). Improvements in diagnosis and treatment have led to a reduction in mortality: before 1980, the mortality rate was greater than 50%, but after 1980 it was 10–30%. Patients with signs of multiple organ failure are likely to die unless they undergo early biliary drainage and treatment with systemic antibiotics. Other causes of death following severe cholangitis include heart failure and pneumonia.

Risk Factors:
Risk factors include a previous history of gallstones, sclerosing cholangitis, HIV, narrowing of the common bile duct, and, rarely, travel to countries where you might catch a worm or parasite infection.

Risk factors indicating an increased risk of death include older age, female gender, a history of liver cirrhosis, biliary narrowing due to cancer, acute renal failure and the presence of liver abscesses. Complications following severe cholangitis include renal failure, respiratory failure (inability of the respiratory system to oxygenate blood and/or eliminate carbon dioxide), cardiac arrhythmia, wound infection, pneumonia, gastrointestinal bleeding and myocardial ischemia (lack of blood flow to the heart, leading to heart attacks).

Prevention:
Treatment of gallstones, tumors, and infestations of parasites may reduce the risk for some people. A metal or plastic stent that is placed in the bile system may be needed to prevent the infection from returning.
Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.
Resources:
http://www.nlm.nih.gov/medlineplus/ency/article/000290.htm
http://en.wikipedia.org/wiki/Ascending_cholangitis

Amyloidosis

Alternative Names: Amyloid – primary

Definition:
In medicine, amyloidosis refers to a variety of conditions in which amyloid proteins are abnormally deposited in organs and/or tissues. A protein is described as being amyloid if, due to an alteration in its secondary structure, it takes on a particular aggregated insoluble form similar to the beta-pleated sheet.  Symptoms vary widely depending upon the site of amyloid deposition. Amyloidosis may be inherited or acquired.

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The collection of these abnormal proteins interferes with the normal functioning of the organ affected.

Since there are more than 20 different proteins that may form amyloid, there are also many different types of amyloidosis.

Classification of amyloid:
The modern classification of amyloid disease tends to use an abbreviation of the protein that makes the majority of deposits, prefixed with the letter A. For example amyloidosis caused by transthyretin is termed “ATTR.” Deposition patterns vary between patients but are almost always composed of just one amyloidogenic protein. Deposition can be systemic (affecting many different organ systems) or organ-specific. Many amyloidoses are inherited, due to mutations in the precursor protein. Other forms are due to different diseases causing overabundant or abnormal protein production – such as with over production of immunoglobulin light chains in multiple myeloma (termed AL amyloid), or with continuous overproduction of acute phase proteins in chronic inflammation (which can lead to AA amyloid).

Out of the approximately 60 amyloid proteins that have been identified so far,  at least 36 have been associated in some way with a human disease.

Amyloidosis is rare, being diagnosed in between one and five in every 100,000 people every year. It’s more common in older people and is also slightly more common in men than in women.

Causes:
The cause of primary amyloidosis is unknown, but the condition is related to abnormal production of antibodies by a type of immune cell called plasma cells.

The symptoms depend on the organs affected by the deposits. These organs can include the tongue, intestines, skeletal and smooth muscles, nerves, skin, ligaments, heart, liver, spleen, and kidneys.

Primary amyloidosis can result in conditions that include:

•Carpal tunnel syndrome
•Gastrointestinal reflux (GERD)
•Heart muscle damage (cardiomyopathy)
•Kidney failure
•Malabsorption
The deposits build up in the affected organs, causing them to become stiff, which decreases their ability to function.

Risk factors have not been identified. Primary amyloidosis is rare. It is similar to multiple myeloma, and is treated the same way.

Symptoms:

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•Enlarged tongue
•Fatigue
•Irregular heart rhythm
•Numbness of hands and feet
•Shortness of breath
•Skin changes
•Swallowing difficulties
•Swelling in the arms and legs
•Weak hand grip
•Weight loss

Additional symptoms that may be associated with this disease:
•Clay-colored stools
•Decreased urine output
•Diarrhea
•Hoarseness or changing voice
•Joint pain
•Other tongue problems
•Weakness

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Diagnosis:
Exams and Tests
Your doctor may discover that you have an enlarged liver or spleen.

If specific organ damage is suspected, your doctor may order tests to confirm amyloidosis of that organ. For example:

•Abdominal ultrasound may reveal a swollen liver or spleen.
•An abdominal fat pad biopsy, rectal mucosa biopsy, or a bone marrow biopsy can help confirm the diagnosis.
•A heart evaluation, including an ECG,may reveal arrhythmias, abnormal heart sounds, or signs of congestive heart failure. An echocardiogram shows poor motion of the heart wall, due to a stiff heart muscle.
•A carpal tunnel syndrome evaluation may show that hand grips are weak.Nerve conduction velocity shows abnormalities.
•Kidney function tests may show signs of kidney failure or too much protein in the urine ( nephrotic syndrome).
?BUN level is increased.
?Serum creatinine is increased.
?Urinalysis shows protein, casts, or fat bodies.

This disease may also alter the results of the following tests:
•Bence-Jones protein (quantitative)
•Carpal tunnel biopsy
•Gum biopsy
•Immunoelectrophoresis – serum
•Myocardial biopsy
•Nerve biopsy
•Quantitative immunoglobulins
•Tongue biopsy
•Urine protein

Treatment:
It isn’t always easy to treat amyloidosis, and there is no treatment yet that specifically targets the amyloid depositing in the tissues. In cases where it’s secondary to another problem (AA amyloidosis), such as rheumatoid arthritis, treating that original problem may stop the progress of amyloidosis or may even reverse it.

In cases of primary amyloidosis (AL amyloidosis), chemotherapy drugs may be given to suppress production of new amyloid and cause regression of existing amyloid deposits.

In secondary amyloidosis, aggressive treatment of the underlying disease can improve symptoms and/or slow progression of disease. Complications such as heart failure, kidney failure, and other problems can sometimes be treated as necessary.

Occasionally, transplantation of a damaged organ is necessary. However, even after this has been carried out the new organ may become affected by amyloidosis.

Treatment may also be aimed at supporting the function of damaged tissues and treating complications such as heart or kidney failure.

Overall, many types of amyloidosis follow a steadily progressive course and may prove fatal within a year or two.

Prognosis :
The severity of the disease depends upon the organs affected. Heart and kidney involvement may lead to organ failure and death. Systemic involvement is associated with death within 1 to 3 years.

Possible Complications:
•Congestive heart failure
•Death
•Endocrine failure (hormonal disorder)
•Kidney failure
•Respiratory failure

Prevention : There is no known prevention.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.

Resources:
http://www.bbc.co.uk/health/physical_health/conditions/amyloidosis1.shtml
http://www.nlm.nih.gov/medlineplus/ency/article/000533.htm
http://en.wikipedia.org/wiki/Amyloidosis

http://health.allrefer.com/pictures-images/amyloidosis-on-the-face.html

http://health.allrefer.com/health/cardiac-amyloidosis-dilated-cardiomyopathy.html

http://morningreporttgh.blogspot.com/2010/04/amyloidosis.html

http://gsm.utmck.edu/research/HICP/overview.cfm

http://www.pathologyatlas.ro/amyloidosis-kidney-pathology.php

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Scotoma

Definition:A scotoma (Greek: darkness; plural: “scotomas” or “scotomata”) is an area or island of loss or impairment of visual acuity surrounded by a field of normal or relatively well-preserved vision.
Every normal mammalian eye has a scotoma in its field of vision, usually termed its blind spot. This is a location with no photoreceptors, where the retinal ganglion cell axons that comprise the optic nerve exit the retina. This location is called the optic disc. The blindspot does not intrude into consciousness because the corresponding visual field locations of the optic discs in the two eyes differ: The visual signals that are absent in one eye are sent to the cortex by signals from the other eye.

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The presence of the scotoma can be demonstrated subjectively by covering one eye, carefully holding fixation with the open eye, and placing an object (such as your thumb) in the lateral and horizontal visual field, about 15 degrees from fixation (see the blind spot article). The size of the monocular scotoma is surprisingly large – 5×7 deg of visual angle.

It is a common type of vision loss post stroke or traumatic brain injury, a scotoma is an island of visual field loss (blindness) or impaired vision surrounded by relatively normal vision. The eyes of mammals naturally have a small scotoma (blind spot) that we normally don’t detect. However, a wide range of diseases and injuries can cause a pathological scotoma. For example, a scotoma can be a sign of optic nerve damage sustained during a stroke or brain injury. Previously considered untreatable, new research has led to exciting developments in treating scotoma.

Types of Scotoma: After a stroke or brain injury, a scotoma may occur in any shape or size, and it may affect any portion of the visual field. In some cases, a scotoma will include and enlarge the blind spot occurring naturally in a person’s eye. The main types of scotomas include:

* Central scotoma: an area of decreased or lost vision that interferes with central vision (likely to affect daily life)...CLICK & SEE
* Hemianopic scotoma: an area of decreased or lost vision that affects half of the central visual field….CLICK & SEE
* Peripheral scotoma: an area of decreased or lost vision toward the edge of the visual field (less likely to affect daily life)...CLICK & SEE

Symptoms: The main symptom of scotoma is one or more dark, light, or blurred areas in the field of vision. Those affected by visual field loss may also experience a need for greater illumination and contrast when reading, and may have difficulty perceiving certain colors.

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Symptom-producing or pathological scotomata may be due to a wide range of disease processes, affecting either the retina (in particular its most sensitive portion, the macula) or the optic nerve itself. A pathological scotoma may involve any part of the visual field and may be of any shape or size. A scotoma may include and enlarge the normal blind spot. Even a small scotoma that happens to affect central or macular vision will produce a severe visual handicap, whereas a large scotoma in the more peripheral part of a visual field may go unnoticed by the bearer due to the normal reduced visual resolution in the peripheral visual field.

Causes:Common causes of scotomata include demyelinating disease such as multiple sclerosis (retrobulbar neuritis), toxic substances such as methyl alcohol, ethambutol and quinine, nutritional deficiencies, and vascular blockages either in the retina or in the optic nerve. Scintillating scotoma is a common visual aura in migraine.   Less common, but important because sometimes reversible or curable by surgery, are scotomata due to tumors such as those arising from the pituitary gland, which may compress the optic nerve or interfere with its blood supply.

Rarely, scotomata are bilateral. One important variety of bilateral scotoma may occur when a pituitary tumour begins to compress the optic chiasm (as distinct from a single optic nerve) and produces a bi-temporal hemicentral scotomatous hemianopia. This type of visual field defect tends to be very eloquent symptom-wise but often evades early objective diagnosis, as it is more difficult to detect by cursory clinical examination than the classical or text-book bi-temporal peripheral hemianopia and may even elude sophisticated electronic modes of visual field assessment.

In a pregnant woman, scotomata can present as a symptom of severe preeclampsia, a form of pregnancy-induced hypertension.

Click To learn about Detection:->

* Amsler grid…..CLICK & SEE
* Perimetry……..CLICK & SEE
* Visual field test….CLICK & SEE

Treatment: There is no treatment for scotomas.

When they are in the peripheral areas and are not large, they usually do not cause severe problems in general visual functioning. If the scotomas are large or numerous, mobility may be affected.

Central scotomas are another situation entirely. Functional acuity is severely affected and educational adjustments are indicated. Magnification or large print may be indicated. Higher levels of illumination and good contrast in reading materials may also be useful. Color perception may be affected.

Vision loss post stroke or brain injury, which may include scotoma, hemianopia / quadrantanopia, and diffuse field defect / low vision, can drastically impact a person’s quality of life. In the past, these vision defects were considered untreatable. However, cutting-edge research into neuroplasticity, the brain’s ability to grow and heal throughout adulthood, has led to effective methods of vision rehabilitation.

Developed by NovaVision, one such method of vision rehab, called Vision Restoration Therapy, works by stimulating the brain in precise, consistent ways. Studies show that 70 percent of patients who complete Vision Restoration Therapy experience significant improvements in their vision, which improves their quality of life. Today, the therapy is available at premier institutions and medical centers across the United States.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://www.helpforvisionloss.com/vision-loss/scotoma/#types
http://en.wikipedia.org/wiki/Scotoma
http://www.spedex.com/resource/documents/veb/scotoma.html