Tag Archives: Liver

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

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Artemisia capillaries

 

Botanical Name : Artemisia capillaries
Family: Asteraceae
Genus: Artemisia
Species: A. scoparia
Order: Asterales

Common Names : Yin Chen Hao
English Name:Capillary Wormwood Herb
Pin Yin Name:Yin Chen

Other Pin Yin Name:Mian Yin Chen,Bai Hao,Rong Hao,Song Mao Ai,Ma Xian,Po Po Hao,Ye Lan Hao

Habitat :Artemisia capillaries  is native to E. Asia – China, Japan, Korea, Manchuria. It  grows on  the grassy thickets, and along rivers and seashores, C. and S. Japan. Humid slopes, hills, terraces, roadsides and river banks at elevations of 100 – 2700 metres in China.

Description:
Artemisia capillaris is a deciduous perennial herb or subshrub.Stem erect height 0.5 to 1 m,root woody,surface color yellow brown,vertical stripin,branches;seedling covered with brown silk hair,hairless when grow up.Bottom Leaf split wide and short,covered with short silky foliage;middle leaf split long and slim as hair,1mm width;top leaf split into 3 parts or no split,no hair.capitulum small and numerous,flower color yellow,pipe like,outer layer 3 to 5 bud,female,fertible,inner layer bisexual 5 to 7,infertility.Fruit long round shape width 0.8mm,hairless.Flowering during September to October.The flowers are hermaphrodite (have both male and female organs) and are pollinated by Wind.and the seeds ripen from Sep to October.

Click to see the pictures.

The plant prefers light (sandy) and medium (loamy) soils and requires well-drained soil.The plant prefers neutral and basic (alkaline) soils..It can grow in semi-shade (light woodland) or no shade.It requires dry or moist soil and can tolerate drought.The plant can tolerates strong winds but not maritime exposure.

Cultivation:
An easily grown plant, succeeding in a well-drained circumneutral or slightly alkaline loamy soil, preferring a sunny position. Established plants are drought tolerant. Plants are longer lived, more hardy and more aromatic when they are grown in a poor dry soil. This species is probably not hardy in all parts of Britain, it tolerates temperatures down to at least -5°c. Plants in this genus are notably resistant to honey fungus. Members of this genus are rarely if ever troubled by browsing deer.

Propagation :
Seed – surface sow from late winter to early summer in a greenhouse. When large enough to handle, prick the seedlings out into individual pots and plant them out in the summer. Cuttings of half-ripe wood, July/August in a frame. Division in spring or autumn.

Edible Uses :
Edible Parts: Leaves.

Leaves and stems – soaked and boiled

Medicinal Uses:
Antibacterial;  Anticholesterolemic;  Antiviral;  CholagogueDiureticFebrifugeHepatic;  Vasodilator.

Yin Chen Hao has been used in Chinese herbal medicine for over 2,000 years. It is considered to be a bitter and cooling herb, clearing “damp heat” from the liver and gall ducts and relieving fevers. It is an effective remedy for liver problems, being specifically helpful in treating hepatitis with jaundice. Modern research has confirmed that the plant has a tonic and strengthening effect upon the liver, gallbladder and digestive system. The leaves and young shoots are antibacterial, anticholesterolemic, antiviral, cholagogue, diuretic, febrifuge and vasodilator. An infusion is used internally in the treatment of jaundice, hepatitis, gall bladder complaints and feverish illnesses. Externally it has been applied in the form of a plaster for treating headaches. The plant is harvested in late spring and can be dried for later use. Yin Chen Hao is contraindicated for pregnant women

Yin chen hao is an effective remedy for liver problems, being specifically helpful for treating hepatitis with jaundice.  Traditional Chinese medicine holds that it is bitter and cooling, clearing “damp heat” from the liver and gall ducts and relieving fevers.  Yin chen hao is also anti-inflammatory and diuretic.  It was formerly used in a plaster for headaches.  Research indicates that yin chen hao has a tonic and strengthening effect on the liver and gallbladder and digestive system.  It is an effective remedy for liver problems, being specifically helpful in treating hepatitis with jaundice.    An infusion of the young shoots is used internally in the treatment of jaundice, hepatitis, gall bladder complaints and feverish illnesses. Externally it has been applied in the form of a plaster for treating headaches.

Known Hazards : Although no reports of toxicity have been seen for this species, skin contact with some members of this genus can cause dermatitis or other allergic reactions in some people.

Disclaimer:The information presented herein is intended for educational purposes only. Individual results may vary, and before using any supplements, it is always advisable to consult with your own health care provider

Resources:
http://www.mdidea.com/products/proper/proper05203.html
http://www.pfaf.org/user/Plant.aspx?LatinName=Artemisia+capillaris
http://www.herbnet.com/Herb%20Uses_UZ.htm

http://library.thinkquest.org/25983/4.%20Capillaris.htm

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Aristolochia contorta

 

Botanical Name : Aristolochia contorta
Family: Aristolochiaceae
Subfamily: Aristolochioideae
Genus: Aristolochia
Species : Aristolochia contorta
Order: Piperales

Synonyms : A. nipponica.

Common Name: Ma Dou Ling

Habitat : E. Asia – China, Japan, Korea, Manchuria. .-Aug. Grows in edges of mountain woods.

Description:
Aristolochia contorta is a  perennial  herb, growing to 1.5 m (5ft). It is in flower from May to July, and the seeds ripen from Sep to October. The flowers are hermaphrodite (have both male and female organs) and are pollinated by Flies.

…...CLICK & SEE THE PICTURES

The shrub  has  stout elongated rhizomes. Stem slender, glabrous. Leaves alternate, cordate or broadly ovate-cordate, 4-10 cm long, 3.5-8 cm wide, acute or obtuse at tip, cordate at base, entire, petioles 1-7 cm long.(CLICK & SEE) Peduncles axillary, 1-4 cm long, with prominent bracts at base. Flowers few in axils, fascicled, the pedicels 1-4 cm long; the calyx tubular, inflated and globose at base, loosely pilose inside; the limb dilated, obliquely truncate, narrowly deltoid, long-acuminate to a filiform point; stamens 6, ovary inferior. Fruit a capsule,globose, 3 cm in diameter, 6 valved. Jul.-Aug……...CLICK & SEE

The plant prefers light (sandy), medium (loamy) and heavy (clay) soils and requires well-drained soil.The plant prefers acid, neutral and basic (alkaline) soils..It can grow in semi-shade (light woodland) or no shade.It requires moist soil.

Cultivation:
We have very little information on this species and do not know if it will be hardy in Britain, though judging by its native range it should succeed outdoors in many parts of this country. The following notes are based on the general needs of the genus. Prefers a well-drained loamy soil, rich in organic matter, in sun or semi-shade. Succeeds in ordinary garden soil. Most species in this genus have malodorous flowers that are pollinated by flies.

Propagation
Seed – best sown in a greenhouse as soon as it is ripe in the autumn. Pre-soak stored seed for 48 hours in hand-hot water and surface sow in a greenhouse. Germination usually takes place within 1 – 3 months at 20°c. Stored seed germinates better if it is given 3 months cold stratification at 5°c. When large enough to handle, prick the seedlings out into individual pots and grow them on in the greenhouse for their first winter. Plant out in late spring or early summer after the last expected frosts. Division in autumn. Root cuttings in winter.

Edible Uses :
Edible Parts: Leaves.

Medicinal Uses;
Antiasthmatic;  Antiseptic;  Antitussive;  Cancer;  Expectorant;  Sedative.

The fruit and its capsule are antiasthmatic, antiseptic, antitussive and expectorant. A decoction of the fruit is used in the treatment of cancer, coughs, inflammation of the respiratory organs, haemorrhoids and hypertension. It is also used to resolve phlegm and lower blood pressure. It has an antibacterial action, effective against Staphylococcus aureus, Pneumococci, bacillus dysentericae etc. The root contains aristolochic acid. This has anti-cancer properties and can be used in conjunction with chemotherapy and radiotherapy. Aristolochic acid can also be used in the treatment of acute and serious infections such as TB, hepatitis, liver cirrhosis and infantile pneumonia. It also increases the cellular immunity and phagocytosis function of the phagocytic cells. Aristolochic acid is said to be too toxic for clinical use. The root is used as a purgative in the treatment of rabies and also has sedative properties.

A decoction of the fruit is used in the treatment of cancer, coughs, inflammation of the respiratory organs, hemorrhoids and hypertension. It is also used to resolve phlegm and lower blood pressure. It has an antibacterial action, effective against Staphylococcus aureus, Pneumococci, bacillus dysentericae etc. The root contains aristolochic acid. This has anti-cancer properties and can be used in conjunction with chemotherapy and radiotherapy. Aristolochic acid can also be used in the treatment of acute and serious infections such as TB, hepatitis, liver cirrhosis and infantile pneumonia. It also increases the cellular immunity and phagocytosis function of the phagocytic cells. Aristolochic acid is said to be too toxic for clinical use. The root is used as a purgative in the treatment of rabies and also has sedative properties.

Known Hazards:  No specific details for this species is found but most members of this genus have poisonous roots and stems. The plant contains aristolochic acid, this has received rather mixed reports on its toxicity. According to one report aristolochic acid stimulates white blood cell activity and speeds the healing of wounds, but is also carcinogenic and damaging to the kidneys. Another report says that it is an active antitumour agent but is too toxic for clinical use. Another report says that aristolochic acid has anti-cancer properties and can be used in conjunction with chemotherapy and radiotherapy and that it also increases the cellular immunity and phagocytosis function of the phagocytic cells.

Disclaimer:
The information presented herein is intended for educational purposes only. Individual results may vary, and before using any supplements, it is always advisable to consult with your own health care provider.

Resources:
http://www.pfaf.org/user/Plant.aspx?LatinName=Aristolochia+contorta
http://species.wikimedia.org/wiki/Aristolochia_contorta
http://www.herbnet.com/Herb%20Uses_LMN.htm

http://www.wpro.who.int/internet/files/pub/97/33.pdf

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Ruptured spleen

Definition:
The spleen is a soft plum-coloured organ, packed with blood-filled tissues and covered by a smooth membrane. It’s located in the abdomen just beneath the left side of the diaphragm, under the ribs, and is shaped like a loosely clenched fist.
CLICK & SEE THE PICTURES
The spleen’s vulnerable location and softness means it’s at risk of injury. A severe blow to the stomach area can squash the spleen, splitting or tearing its covering membrane and the tissue inside and allowing blood to rapidly leak out – rather like a squashed tomato.

A ruptured spleen is a serious condition that can occur when your spleen experiences a trauma. With enough force, a blow to your abdomen — during a sporting mishap, a fistfight or a car crash, for example — might lead to a ruptured spleen. Without emergency treatment, a ruptured spleen can cause life-threatening internal bleeding.

Though some ruptured spleens require emergency surgery, others with ruptured spleens can be treated with several days of hospital care.

You may click to seedifferent pictures of  Ruptured spleen

Symptoms
The abdomen usually feels tender and painful when the spleen ruptures. Blood leaks into the abdomen, causing irritation with subsequent tenderness and pain. Classically, a patient with a ruptured spleen describes feeling left shoulder-tip pain (this pain comes from irritation of the diaphragm by the spilt blood).

If the leak of blood is gradual, symptoms may not occur until the blood supply to the body is diminished. This will result in low blood pressure and light-headedness, blurred vision, confusion and loss of consciousness, as the oxygen supply to the heart and brain is affected. If blood loss is rapid, the person may suddenly collapse.

Causes:
A ruptured spleen is the most common serious complication of an abdominal injury and may occur as a consequence of road traffic accidents, sports injuries and violent, physical attacks.

*Injury to the left side of the body. A ruptured spleen is typically caused by a blow to the left upper abdomen or the left lower chest, such as might happen during sporting mishaps, fistfights and car crashes.

*An injured spleen may rupture soon after the abdominal trauma or, in some cases, days or even weeks after the injury.An enlarged spleen. Your spleen can become enlarged when blood cells accumulate in the spleen. An enlarged spleen can be caused by various underlying problems, such as mononucleosis and other infections, liver disease and blood cancers.

Complications: A ruptured spleen can cause life-threatening bleeding into your abdominal cavity.

Diagnosis:
Normal procedures  to diagnose a ruptured spleen include:

*A physical exam. During a physical exam  doctor will use his or her hands to place pressure on your abdomen to determine the size of your spleen and whether you’re experiencing any abdominal tenderness.

*Drawing fluid from  abdomen.  The Doctor may use a needle to draw a sample of fluid from the abdomen. If the sample reveals blood in the abdomen,  the patient may be referred for emergency treatment.

*Imaging tests of  the abdomen. If your diagnosis isn’t clear, the doctor may recommend an abdominal computerized tomography (CT) scan or another imaging test to identify or rule out other possible causes as per symptoms.

Treatment :
Untreated, a ruptured spleen can be rapidly fatal, so it requires urgent medical and surgical treatment.

Fluids must quickly be given through an intravenous drip in order to maintain the circulation to the organs of the body (including a blood transfusion) and emergency surgery is performed to stop the leak of blood.

Sometimes, if the rupture is only small, it’s possible for the surgeon to repair the spleen. However, usually the entire spleen needs to be removed in an operation called a splenectomy.

The spleen plays an important part in protecting the body against infection. In particular it clears a type of bacteria known as pneumococcus from the body. So it’s important that those who’ve had their spleen removed take extra precautions to protect themselves against infection. In particular, they should be vaccinated against pneumococcal infection.

Prognosis:
Splenic rupture permits large amounts of blood to leak into the abdominal cavity, possibly resulting in shock and death. Patients typically require emergency surgery, although it is becoming more common to simply monitor the patient to make sure the bleeding stops by itself and to allow the spleen to heal itself. Rupture of a normal spleen can be caused by trauma, for example, in an accident. If an individual’s spleen is enlarged, as is frequent in mononucleosis, most physicians will not allow activities (such as contact sports) where injury to the abdomen could be catastrophic.

Prevention
The spleen is a useful organ and it is essential to life. It is sometimes removed (splenectomy) in those who have blood disorders, such as thalassemia or hemolytic anemia. If the spleen is removed, a person must receive certain immunizations to help prevent infections that the spleen normally fights.

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/rupturedspleen1.shtml
http://en.wikipedia.org/wiki/Ruptured_spleen
http://www.mayoclinic.com/health/ruptured-spleen/DS00872

how-to-treat-torn-ruptured-spleen-photo

Wilson’s Disease

Definition :
Wilson’s disease is an inherited disorder that causes too much copper to accumulate in your liver, brain and other vital organs. Another term for Wilson’s disease is hepatolenticular degeneration.

Copper plays a key role in the development of healthy nerves, bones, collagen and the skin pigment melanin. Normally, copper is absorbed from your food, and any excess is excreted through bile — a substance produced in your liver.

CLICK  & SEE THE PICTURES

Normal absorption and distribution of copper. Cu = copper, CP = ceruloplasmin, green = ATP7B carrying copper.

But in people with Wilson’s disease, copper isn’t eliminated properly and instead accumulates, possibly to a life-threatening level. Left untreated, Wilson’s disease is fatal. When diagnosed early, Wilson’s disease is treatable, and many people with the disorder live normal lives.

The excess copper can build up in the liver and/or brain causing liver damage and/or neurological problems. It can also collect in other parts of the body including the eyes and the kidneys.
Copper begins to accumulate immediately after birth but the symptoms usually appear in the 2nd to 3rd decade. The first signs are hepatic (liver) in about 40% of cases, neurological (brain) in about 35% of cases and psychiatric, renal (kidney), haematological (blood), or endocrine (glands) in the remainder.

The condition is due to mutations in the Wilson disease protein (ATP7B) gene. A single abnormal copy of the gene is present in 1 in 100 people, who do not develop any symptoms (they are carriers). If a child inherits the gene from both parents, they may develop Wilson’s disease. Symptoms usually appear between the ages of 6 and 20 years, but cases in much older people have been described. Wilson’s disease occurs in 1 to 4 per 100,000 people.  Wilson’s disease is named after Samuel Alexander Kinnier Wilson (1878–1937), the British neurologist who first described the condition in 1912


Symptoms:
The most pathognomonic sign of Wilson’s disease results from a buildup of copper in the eyes. These rings are
called Kayser – Fleischer rings. Rings are brownish, visible aroound the corneo – scleral junction (limbus).
95% of Wilson’ s disease patients presenting with neurological signs will have Kayser – Fleischer rings and 65% of Wilson’s disease patients presenting with hepatic signs will present a ring.

CLICK & SEE

Wilson’s disease causes a wide variety of signs and symptoms that are often mistaken for other diseases and conditions. Signs and symptoms vary depending on what parts of your body are affected by Wilson’s disease.
Signs and symptoms of Wilson’s disease include:

*Clumsiness
*Depression
*Difficulty speaking
*Difficulty swallowing
*Difficulty walking
*Drooling
*Easy bruising
*Fatigue
*Involuntary shaking
*Joint pain
*Loss of appetite
*Nausea
*Skin rash
*Swelling of arms and legs
*Yellowing of the skin and eyes (jaundice)

The main sites of copper accumulation are the liver and the brain, and consequently liver disease and neuropsychiatric symptoms are the main features that lead to diagnosis.  People with liver problems tend to come to medical attention earlier, generally as children or teenagers, than those with neurological and psychiatric symptoms, who tend to be in their twenties or older. Some are identified only because relatives have been diagnosed with Wilson’s disease; many of these, when tested, turn out to have been experiencing symptoms of the condition but haven’t received a diagnosis.:

Causes:
Wilson’s disease occurs when a genetic mutation leads to an accumulation of copper in  one’s body.

How the genetic mutation occurs
The genetic mutation that causes Wilson’s disease is most commonly passed from one generation to the next. Wilson’s disease is inherited as an autosomal recessive trait, which means that to develop the disease you must inherit two copies of the defective gene, one from each parent. If you receive only one abnormal gene, you won’t become ill yourself, but you’re considered a carrier and can pass the gene to your children.

How the genetic mutation causes Wilson’s disease
The mutation that causes Wilson’s disease occurs in a gene called ATP7B. When a mutation occurs on this gene, it leads to problems with a protein that’s responsible for moving excess copper out of your liver.

Your body collects copper from the food you eat during the digestive process. The copper is transported to your liver where liver cells use it for everyday tasks. Most people eat more copper than they need. In these cases, the liver takes what it needs and excretes the rest in bile, a digestive juice made by the liver.

But in people with Wilson’s disease, the extra copper doesn’t leave your body. Instead, copper builds up in the liver, where it can cause serious and sometimes irreversible damage. In time, excess copper leaves the liver and begins accumulating in and harming other organs, especially the brain, eyes and kidneys.

 

Complications:
Wilson’s disease can cause serious complications such as:

*Scarring of the liver (cirrhosis). As liver cells try to make repairs to damage done by excess copper, scar tissue forms in the liver. The scar tissue makes it more difficult for the liver to function.

*Liver failure. Liver failure can occur suddenly (acute liver failure), or it can develop slowly over many years. If liver function progresses, a liver transplant may be a treatment option.

*Liver cancer. Damage to the liver caused by Wilson’s disease may increase the risk of liver cancer.

*Persistent neurological problems. Neurological problems usually improve with treatment for Wilson’s disease. However, some people may experience persistent neurological difficulty, despite treatment.
*Kidney problems. Wilson’s disease can damage the kidneys, leading to kidney problems, such as kidney stones and an abnormal number of amino acids excreted in the urine (aminoaciduria).

Diagnosis:
Wilson’s disease may be suspected on the basis of any of the symptoms mentioned above, or when a close relative has been found to have Wilson’s. Most have slightly abnormal liver function tests such as a raised aspartate transaminase, alanine transaminase and bilirubin level. If the liver damage is significant, albumin may be decreased due to an inability of damaged liver cells to produce this protein; likewise, the prothrombin time (a test of coagulation) may be prolonged as the liver is unable to produce proteins known as clotting factors. Alkaline phosphatase levels are relatively low in those with Wilson’s-related acute liver failure. If there are neurological symptoms, magnetic resonance imaging (MRI) of the brain is usually performed; this shows hyperintensities in the part of the brain called the basal ganglia in the T2 setting.  MRI may also demonstrate the characteristic “face of the giant panda” pattern.

There is no totally reliable test for Wilson’s disease, but levels of ceruloplasmin and copper in the blood, as well of the amount of copper excreted in urine during a 24-hour period, are together used to form an impression of the amount of copper in the body. The gold standard or most ideal test is a liver biopsy

Ceruloplasmin
Levels of ceruloplasmin are abnormally low (<0.2 g/L) in 80–95% of cases. It can, however, be present at normal levels in people with ongoing inflammation as it is an acute phase protein. Low ceruloplasmin is also found in Menkes disease and aceruloplasminemia, which are related to, but much rarer than Wilson’s disease.

The combination of neurological symptoms, Kayser–Fleisher rings and a low ceruloplasmin level is considered sufficient for the diagnosis of Wilson’s disease. In many cases, however, further tests are needed.
Serum and urine copper
Serum copper is paradoxically low but urine copper is elevated in Wilson’s disease. Urine is collected for 24 hours in a bottle with a copper-free liner. Levels above 100 ?g/24h (1.6 ?mol/24h) confirm Wilson’s disease, and levels above 40 ?g/24h (0.6 ?mol/24h) are strongly indicative.[1] High urine copper levels are not unique to Wilson’s disease; they are sometimes observed in autoimmune hepatitis and in cholestasis (any disease obstructing the flow of bile from the liver to the small bowel).

In children, the penicillamine test may be used. A 500 mg oral dose of penicillamine is administered, and urine collected for 24 hours. If this contains more than 1600 ?g (25 ?mol), it is a reliable indicator of Wilson’s disease. This test has not been validated in adults.

Liver biopsy
Once other investigations have indicated Wilson’s disease, the ideal test is the removal of a small amount of liver tissue through a liver biopsy. This is assessed microscopically for the degree of steatosis and cirrhosis, and histochemistry and quantification of copper are used to measure the severity of the copper accumulation. A level of 250 ?g of copper per gram of dried liver tissue confirms Wilson’s disease. Occasionally, lower levels of copper are found; in that case, the combination of the biopsy findings with all other tests could still lead to a formal diagnosis of Wilson’s.

In the earlier stages of the disease, the biopsy typically shows steatosis (deposition of fatty material), increased glycogen in the nucleus, and areas of necrosis (cell death). In more advanced disease, the changes observed are quite similar to those seen in autoimmune hepatitis, such as infiltration by inflammatory cells, piecemeal necrosis and fibrosis (scar tissue). In advanced disease, finally, cirrhosis is the main finding. In acute liver failure, degeneration of the liver cells and collapse of the liver tissue architecture is seen, typically on a background of cirrhotic changes. Histochemical methods for detecting copper are inconsistent and unreliable, and taken alone are regarded as insufficient to establish a diagnosis.

Genetic testing
Mutation analysis of the ATP7B gene, as well as other genes linked to copper accumulation in the liver, may be performed. Once a mutation is confirmed, it is possible to screen family members for the disease as part of clinical genetics family counselling

Treatment:
DietaryIn general, a diet low in copper-containing foods is recommended, with the avoidance of mushrooms, nuts, chocolate, dried fruit, liver, and shellfish.

Medication
Various treatments are available for Wilson’s disease. Some increase the removal of copper from the body, while others prevent the absorption of copper from the diet.

Generally, penicillamine is the first treatment used. This binds copper (chelation) and leads to excretion of copper in the urine. Hence, monitoring of the amount of copper in the urine can be done to ensure a sufficiently high dose is taken. Penicillamine is not without problems: about 20% experience a side effect or complication of penicillamine treatment, such as drug-induced lupus (causing joint pains and a skin rash) or myasthenia (a nerve condition leading to muscle weakness). In those who presented with neurological symptoms, almost half experience a paradoxical worsening in their symptoms. While this phenomenon is also observed in other treatments for Wilson’s, it is usually taken as an indication for discontinuing penicillamine and commencing second-line treatment.  Intolerant to penicillamine may instead be commenced on trientine hydrochloride, which also has chelating properties. Some recommend trientine as first-line treatment, but experience with penicillamine is more extensive.  A further agent with known activity in Wilson’s disease is tetrathiomolybdate. This is still regarded as experimental,  although some studies have shown a beneficial effect.

Once all results have returned to normal, zinc (usually in the form of a zinc acetate prescription called Galzin) may be used instead of chelators to maintain stable copper levels in the body. Zinc stimulates metallothionein, a protein in gut cells that binds copper and prevents their absorption and transport to the liver. Zinc therapy is continued unless symptoms recur, or if the urinary excretion of copper increases.

In rare cases where none of the oral treatments are effective, especially in severe neurological disease, dimercaprol (British anti-Lewisite) is still occasionally necessary. This treatment is injected intramuscularly (into a muscle) every few weeks, and has a number of unpleasant side effects such as pain.

People who are asymptomatic (for instance those diagnosed through family screening or only as a result of abnormal test results) are generally treated, as the copper accumulation may cause long-term damage in the future. It is unclear whether these people are best treated with penicillamine or zinc acetate.

Physical therapy
Physiotherapy is beneficial for those patients with the neurologic form of the disease. The copper chelating treatment may take up to six months to start working, and physical therapy can assist in coping with ataxia, dystonia, and tremors, as well as preventing the development of contractures that can result from dystonia.

Transplantation
Liver transplantation is an effective cure for Wilson’s disease, but is used only in particular scenarios because of the numerous risks and complications associated with the procedure. It is used mainly in people with fulminant liver failure who fail to respond to medical treatment, or in those with advanced chronic liver disease. Liver transplantation is avoided in severe neuropsychiatric illness, in which its benefit has not been demonstrated
Lifestyle and home remedies:

Doctors sometimes recommend limiting the amount of copper you consume in your diet during the first year of your treatment for Wilson’s disease. As your signs and symptoms recede and the copper levels in your body drop, you may be able to include copper-containing foods in your diet.

Copper-containing foods
Foods that contain high levels of copper include:

*Copper-containing vitamin and mineral supplements
*Liver
*Shellfish
*Mushrooms
*Nuts
*Chocolate
*Dried fruit
*Dried peas, beans and lentils
*Avocados
*Bran products

Copper in tap water
Have your tap water’s copper levels tested if you have copper pipes in your home or if your water comes from a well. Most municipal water systems don’t contain high levels of copper.

If you have copper pipes, run the tap for several seconds before collecting water for drinking or cooking. Water that sits in the copper pipes can pick up copper particles. Running the water flushes that contaminated water out of the pipes.

Copper pots and pans
Don’t use copper pots, pans or storage containers for your food or drinks.

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://en.wikipedia.org/wiki/Wilson’s_disease
http://www.eurowilson.org/en/living/guide/what/index.phtml
http://www.mayoclinic.com/health/wilsons-disease/DS00411

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