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Hemolytic Uremic Syndrome (HUS)

Alternative names:  Haemolytic-uraemic syndrome, HUS

Definition:
Hemolytic uremic syndrome, or HUS, is a kidney condition that happens when red blood cells are destroyed and block the kidneys‘ filtering system. Red blood cells contain hemoglobin—an iron-rich protein that gives blood its red color and carries oxygen from the lungs to all parts of the body.

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When the kidneys and glomeruli—the tiny units within the kidneys where blood is filtered—become clogged with the damaged red blood cells, they are unable to do their jobs. If the kidneys stop functioning, a child can develop acute kidney injury—the sudden and temporary loss of kidney function. Hemolytic uremic syndrome is the most common cause of acute kidney injury in children.

It is a disease characterized by hemolytic anemia (anemia caused by destruction of red blood cells), acute kidney failure (uremia), and a low platelet count (thrombocytopenia). It predominantly, but not exclusively, affects children. Most cases are preceded by an episode of infectious, sometimes bloody, diarrhea acquired as a foodborne illness or from a contaminated water supply and caused by E. coli O157:H7, although Shigella, Campylobacter and a variety of viruses have also been implicated. It is now the most common cause of acquired acute renal failure in childhood. It is a medical emergency and carries a 5–10% mortality; of the remainder, the majority recover without major consequences but a small proportion develop chronic kidney disease and become reliant on renal replacement therapy.

The kidneys are two bean-shaped organs, each about the size of a fist. They are located just below the rib cage, one on each side of the spine. Every day, the two kidneys filter about 120 to 150 quarts of blood to produce about 1 to 2 quarts of urine, composed of wastes and extra fluid. Children produce less urine than adults and the amount produced depends on their age. The urine flows from the kidneys to the bladder through tubes called ureters. The bladder stores urine. When the bladder empties, urine flows out of the body through a tube called the urethra, located at the bottom of the bladder.

Symptoms:
STEC-HUS occurs after ingestion of a strain of bacteria, usually types of E. coli, that expresses verotoxin (also called Shiga-like toxin). Bloody diarrhea typically follows. HUS develops about 5–10 days after onset of diarrhea, with decreased urine output (oliguria), blood in the urine (hematuria), kidney failure, thrombocytopenia (low levels of platelets) and destruction of red blood cells (microangiopathic hemolytic anemia). Hypertension is common. In some cases, there are prominent neurologic changes.

A child with hemolytic uremic syndrome may develop signs and symptoms similar to those seen with gastroenteritis—an inflammation of the lining of the stomach, small intestine, and large intestine—such as

*vomiting
*bloody diarrhea
*abdominal pain
*fever and chills
*headache

As the infection progresses, the toxins released in the intestine begin to destroy red blood cells. When the red blood cells are destroyed, the child may experience the signs and symptoms of anemia—a condition in which red blood cells are fewer or smaller than normal, which prevents the body’s cells from getting enough oxygen.

Signs and symptoms of anemia may include:-

*fatigue, or feeling tired
*weakness
*fainting
*paleness

As the damaged red blood cells clog the glomeruli, the kidneys may become damaged and make less urine. When damaged, the kidneys work harder to remove wastes and extra fluid from the blood, sometimes leading to acute kidney injury.

Other signs and symptoms of hemolytic uremic syndrome may include bruising and seizures.

When hemolytic uremic syndrome causes acute kidney injury, a child may have the following signs and symptoms:

*edema—swelling, most often in the legs, feet, or ankles and less often in the hands or face
*albuminuria—when a child’s urine has high levels of albumin, the main protein in the blood
*decreased urine output
*hypoalbuminemia—when a child’s blood has low levels of albumin
*blood in the urine

Causes:
A number of things can cause hemolytic uremic syndrome, but the most common cause — particularly in children — is an infection with a specific strain of E. coli, usually the strain known as O157:H7. However, other strains of E. coli have been linked to hemolytic uremic syndrome, too.

Normally, harmless strains, or types, of E. coli are found in the intestines and are an important part of digestion. However, if a child becomes infected with the O157:H7 strain of E. coli, the bacteria will lodge in the digestive tract and produce toxins that can enter the bloodstream. The toxins travel through the bloodstream and can destroy the red blood cells. E. coli O157:H7 can be found in:

*Contaminated meat or produce
*Swimming pools or lakes contaminated with feces
*undercooked meat, most often ground beef
*unpasteurized, or raw, milk
*unwashed, contaminated raw fruits and vegetables
*contaminated juice

Less common causes, sometimes called atypical hemolytic uremic syndrome, can include:-

*taking certain medications, such as chemotherapy
*having other viral or bacterial infections
*inheriting a certain type of hemolytic uremicsyndrome that runs in families

Children who are more likely to develop hemolytic uremic syndrome include those who
are younger than age 5 and have been diagnosedwith an E. coli O157:H7 infection

*have a weakened immune system
*have a family history of inherited hemolyticuremic syndrome
*Hemolytic uremic syndrome occurs in about two out of every 100,000 children.

Most people who are infected with E. coli, even the more dangerous strains, won’t develop hemolytic uremic syndrome. It’s also possible for hemolytic uremic syndrome to follow infection with other types of bacteria.

In adults, hemolytic uremic syndrome is more commonly caused by other factors, including:

*The use of certain medications, such as quinine (an over-the-counter muscle cramp remedy), some chemotherapy drugs, the immunosuppressant medication cyclosporine (Neoral, Sandimmune) and anti-platelet medications

*Pregnancy

*Certain infections, such as HIV/AIDS or an infection with the pneumococcal bacteria

*Genes, which can be a factor because a certain type of HUS — atypical hemolytic uremic syndrome — may be passed down from your parents

The cause of hemolytic uremic syndrome in adults is often unknown

Diagnosis:
The Doctor diagnoses hemolytic uremic syndrome with

*a medical and family history
*a physical exam
*urine tests
*a blood test
*a stool test
*kidney biopsy

The similarities between HUS, aHUS, and TTP make differential diagnosis essential. All three of these systemic TMA-causing diseases are characterized by thrombocytopenia and microangiopathic hemolysis, plus one or more of the following: neurological symptoms (e.g., confusion, cerebral convulsions, seizures); renal impairment (e.g., elevated creatinine, decreased estimated glomerular filtration rate [eGFR], abnormal urinalysis ); and gastrointestinal (GI) symptoms (e.g., diarrhea, nausea/vomiting, abdominal pain, gastroenteritis).The presence of diarrhea does not exclude aHUS as the etiology of TMA, as 28% of patients with aHUS present with diarrhea and/or gastroenteritis. First diagnosis of aHUS is often made in the context of an initial, complement-triggering infection, and Shiga-toxin has also been implicated as a trigger that identifies patients with aHUS. Additionally, in one study, mutations of genes encoding several complement regulatory proteins were detected in 8 of 36 (22%) patients diagnosed with STEC-HUS. However, the absence of an identified complement regulatory gene mutation does not preclude aHUS as the etiology of the TMA, as approximately 50% of patients with aHUS lack an identifiable mutation in complement regulatory genes.

Diagnostic work-up supports the differential diagnosis of TMA-causing diseases. A positive Shiga-toxin/EHEC test confirms an etiological cause for STEC-HUS, and severe ADAMTS13 deficiency (i.e., ?5% of normal ADAMTS13 levels) confirms a diagnosis of TTP

Complications:
Most children who develop hemolytic uremic syndrome and its complications recover without permanent damage to their health.1
However, children with hemolytic uremic syndrome may have serious and sometimes life-threatening complications, including

*acute kidney injury
*high blood pressure
*blood-clotting problems that can lead to bleeding
*seizures
*heart problems
*chronic, or long lasting, kidney disease
*stroke
*coma

Treatment:
The Doctor will treat a child’s urgent symptoms and try to prevent complications by

*observing the child closely in the hospital
*replacing minerals, such as potassium and salt, and fluids through an intravenous (IV) tube
*giving the child red blood cells and platelets—cells in the blood that help with clotting—through an IV
*giving the child IV nutrition
*treating high blood pressure with medications

Treating Acute Kidney Injury:
If necessary,the Doctor will treat acute kidney injury with dialysis—the process of filtering wastes and extra fluid from the body with an artificial kidney. The two forms of dialysis are hemodialysis and peritoneal dialysis. Most children with acute kidney injury need dialysis for a short time only.

Treating Chronic Kidney Disease:
Some children may sustain significant kidney damage that slowly develops into CKD. Children who develop CKD must receive treatment to replace the work the kidneys do. The two types of treatment are dialysis and transplantation.

In most cases, The Doctor treat CKD with a kidney transplant. A kidney transplant is surgery to place a healthy kidney from someone who has just died or a living donor, most often a family member, into a person’s body to take over the job of the failing kidney. Though some children receive a kidney transplant before their kidneys fail completely, many children begin with dialysis to stay healthy until they can have a transplant. click to know more

Prevention:

Hemolytic uremic syndrome, or HUS, is a kidney condition that happens when red blood cells are destroyed and block the kidneys’ filtering system.
The most common cause of hemolytic uremic syndrome in children is an Escherichia coli (E. coli) infection of the digestive system.
Normally, harmless strains, or types, of E. coli are found in the intestines and are an important part of digestion. However, if a child becomes infected with the O157:H7 strain of E. coli, the bacteria will lodge in the digestive tract and produce toxins that can enter the bloodstream.
A child with hemolytic uremic syndrome may develop signs and symptoms similar to those seen with gastroenteritis, an inflammation of the lining of the stomach, small intestine, and large intestine.

Most children who develop hemolytic uremic syndrome and its complications recover without permanent damage to their health.
Some children may sustain significant kidney damage that slowly develops into chronic kidney disease (CKD).

Parents and caregivers can help prevent childhood hemolytic uremic syndrome due to E. coli O157:H7 by

*avoiding unclean swimming areas
*avoiding unpasteurized milk, juice, and cider
*cleaning utensils and food surfaces often
*cooking meat to an internal temperature of at least 160° F
*defrosting meat in the microwave or refrigerator
*keeping children out of pools if they have had diarrhea
*keeping raw foods separate
*washing hands before eating
*washing hands well after using the restroom and after changing diapers

When a child is taking medications that may cause hemolytic uremic syndrome, it is important that the parent or caretaker watch for symptoms and report any changes in the child’s condition to the Doctor as soon as possible.

Prognosis:
Acute renal failure occurs in 55-70% of patients with STEC-HUS, although up to 70-85% recover renal function. Patients with aHUS generally have poor outcomes, with up to 50% progressing to ESRD or irreversible brain damage; as many as 25% die during the acute phase. However, with aggressive treatment, more than 90% of patients survive the acute phase of HUS, and only about 9% may develop ESRD. Roughly one-third of persons with HUS have abnormal kidney function many years later, and a few require long-term dialysis. Another 8% of persons with HUS have other lifelong complications, such as high blood pressure, seizures, blindness, paralysis, and the effects of having part of their colon removed. The overall mortality rate from HUS is 5-15%. Children and the elderly have a worse prognosis.

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://kidney.niddk.nih.gov/KUDiseases/pubs/childkidneydiseases/hemolytic_uremic_syndrome/
http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome
http://www.mayoclinic.org/diseases-conditions/hemolytic-uremic-syndrome/basics/causes/con-20029487

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Multiple Myeloma

Alternative Name :Plasma cell myeloma or Kahler’s disease

Definition:
Multiple myeloma is a type of cancer. Cancer is a group of many related diseases. Myeloma is a cancer that starts in plasma cells, a type of white blood cell. It’s the most common type of plasma cell cancer.

click to see the picture

Multiple myeloma (from Greek myelo-, bone marrow), a type of white blood cell normally responsible for the production of antibodies. Collections of abnormal cells accumulate in bones, where they cause bone lesions, and in the bone marrow where they interfere with the production of normal blood cells. Most cases of myeloma also feature the production of a paraprotein, an abnormal antibody that can cause kidney problems and interferes with the production of normal antibodies leading to immunodeficiency. Hypercalcemia (high calcium levels) is often encountered

Health problems caused by multiple myeloma can affect your bones, immune system, kidneys and red blood cell count.

If some one has multiple myeloma but don’t have symptoms,the doctors may just monitor his or her condition. If experiencing symptoms, a number of treatments are available to help control multiple myeloma.

The disease develops in 1–4 per 100,000 people per year. It is more common in men, and is twice as common in blacks as it is in whites. With conventional treatment, the prognosis is 3–4 years, which may be extended to 5–7 years or longer with advanced treatments. Multiple myeloma is the second most common hematological malignancy (13%) and constitutes 1% of all cancers.

Signs and symptoms:
Because many organs can be affected by myeloma, the symptoms and signs vary greatly. A mnemonic sometimes used to remember the common tetrad of multiple myeloma is CRAB: C = Calcium (elevated), R = Renal failure, A = Anemia, B = Bone lesions. Myeloma has many possible symptoms, and all symptoms may be due to other causes. They are presented here in decreasing order of incidence.

Bone pain
Myeloma bone pain usually involves the spine and ribs, and worsens with activity. Persistent localized pain may indicate a pathological bone fracture. Involvement of the vertebrae may lead to spinal cord compression. Myeloma bone disease is due to the overexpression of Receptor Activator for Nuclear Factor ? B Ligand (RANKL) by bone marrow stroma. RANKL activates osteoclasts, which resorb bone. The resultant bone lesions are lytic in nature and are best seen in plain radiographs, which may show “punched-out” resorptive lesions (including the “pepper pot” appearance of the skull on radiography). The breakdown of bone also leads to release of calcium into the blood, leading to hypercalcemia and its associated symptoms.

Infection
The most common infections are pneumonias and pyelonephritis. Common pneumonia pathogens include S. pneumoniae, S. aureus, and K. pneumoniae, while common pathogens causing pyelonephritis include E. coli and other gram-negative organisms. The greatest risk period for the occurrence of infection is in the initial few months after the start of chemotherapy. The increased risk of infection is due to immune deficiency resulting from diffuse hypogammaglobulinemia, which is due to decreased production and increased destruction of normal antibodies. A selected group of patients may benefit from replacement immunoglobulin therapy to reduce the risk of infection.

Renal failure
Renal failure may develop both acutely and chronically. It is commonly due to hypercalcemia (see above). It may also be due to tubular damage from excretion of light chains, also called Bence Jones proteins, which can manifest as the Fanconi syndrome (type II renal tubular acidosis). Other causes include glomerular deposition of amyloid, hyperuricemia, recurrent infections (pyelonephritis), and local infiltration of tumor cells.

Anemia
The anemia found in myeloma is usually normocytic and normochromic. It results from the replacement of normal bone marrow by infiltrating tumor cells and inhibition of normal red blood cell production (hematopoiesis) by cytokines.

Neurological symptoms
Common problems are weakness, confusion and fatigue due to hypercalcemia. Headache, visual changes and retinopathy may be the result of hyperviscosity of the blood depending on the properties of the paraprotein. Finally, there may be radicular pain, loss of bowel or bladder control (due to involvement of spinal cord leading to cord compression) or carpal tunnel syndrome and other neuropathies (due to infiltration of peripheral nerves by amyloid). It may give rise to paraplegia in late presenting cases.

 

Causes:
Although the exact cause isn’t known, doctors do know that multiple myeloma begins with one abnormal plasma cell in our bone marrow — the soft, blood-producing tissue that fills in the center of most of our bones.

Normal blood cells
Most blood cells develop from cells in the bone marrow called stem cells. Bone marrow is the soft material in the center of most bones.

Stem cells mature into different types of blood cells. Each type has a special job:

*White blood cells help fight infection. There are several types of white blood cells.

*Red blood cells carry oxygen to tissues throughout the body.

*Platelets help form blood clots that control bleeding.

Plasma cells are white blood cells that make antibodies. Antibodies are part of the immune system. They work with other parts of the immune system to help protect the body from germs and other harmful substances. Each type of plasma cell makes a different antibody.

Myeloma cells
Myeloma, like other cancers, begins in cells. In cancer, new cells form when the body doesn’t need them, and old or damaged cells don’t die when they should. These extra cells can form a mass of tissue called a growth or tumor.

Myeloma begins when a plasma cell becomes abnormal. The abnormal cell divides to make copies of itself. The new cells divide again and again, making more and more abnormal cells. These abnormal plasma cells are called myeloma cells.

In time, myeloma cells collect in the bone marrow. They may damage the solid part of the bone. When myeloma cells collect in several of your bones, the disease is called “multiple myeloma.” This disease may also harm other tissues and organs, such as the kidneys.

Myeloma cells make antibodies called M proteins and other proteins. These proteins can collect in the blood, urine, and organs.
click to see the picture.

..Normal plasma cells help protect the body from germs and other harmful substances.

click to see picture.

…Myeloma cell (abnormal plasma cell) making M proteins.

 

Risk Factors:
Multiple myeloma isn’t contagious. Most people who develop multiple myeloma have no clearly identifiable risk factors for the disease.

Some factors that may increase your risk of multiple myeloma include:

*Age. Multiple myeloma is most common in people over 65, affecting men more than women. It rarely occurs in young people.

*Sex. Men are more likely to develop the disease than are women.

*Race. Blacks are about twice as likely to develop multiple myeloma as are whites.History of a monoclonal gammopathy of undetermined significance. Every year 1 percent of the people with MGUS in the United States develop multiple myeloma.

*Obesity. Your risk of multiple myeloma is increased if you’re overweight or obese.

Other factors that may increase your risk of developing multiple myeloma include exposure to radiation and working in leather or rubber manufacturing or the petrol industry, obesity and radiation exposure may increase the risk of multiple myeloma.

Diagnosis:
Doctors sometimes find multiple myeloma after a routine blood test. More often, doctors suspect multiple myeloma after an x-ray for a broken bone. Usually though, patients go to the doctor because they are having other symptoms.

To find out whether such problems are from multiple myeloma or some other condition, your doctor may ask about your personal and family medical history and do a physical exam. Your doctor also may order some of the following tests:

*Blood tests: The lab does several blood tests:

…#Multiple myeloma causes high levels of proteins in the blood. The lab checks the levels of many different proteins, including M protein and other immunoglobulins (antibodies), albumin, and beta-2-microglobulin.

…#Myeloma may also cause anemia and low levels of white blood cells and platelets. The lab does a complete blood count to check the number of white blood cells, red blood cells, and platelets.

…#The lab also checks for high levels of calcium.

…#To see how well the kidneys are working, the lab tests for creatinine.

*Urine tests: The lab checks for Bence Jones protein, a type of M protein, in urine. The lab measures the amount of Bence Jones protein in urine collected over a 24-hour period. If the lab finds a high level of Bence Jones protein in your urine sample, doctors will monitor your kidneys. Bence Jones protein can clog the kidneys and damage them.

*X-rays: You may have x-rays to check for broken or thinning bones. An x-ray of your whole body can be done to see how many bones could be damaged by the myeloma.

*Biopsy: Your doctor removes tissue to look for cancer cells. A biopsy is the only sure way to know whether myeloma cells are in your bone marrow. Before the sample is taken, local anesthesia is used to numb the area. This helps reduce the pain. Your doctor removes some bone marrow from your hip bone or another large bone. A pathologist uses a microscope to check the tissue for myeloma cells.

There are two ways your doctor can obtain bone marrow. Some people will have both procedures during the same visit:

...#Bone marrow aspiration: The doctor uses a thick, hollow needle to remove samples of bone marrow.
...#Bone marrow biopsy: The doctor uses a very thick, hollow needle to remove a small piece of bone and bone marrow.

Staging and classification:
These tests can help confirm whether you have multiple myeloma or another condition. If tests indicate you have multiple myeloma, the results from these tests allow your doctor to classify your disease as stage 1, stage 2 or stage 3. People with stage 3 myeloma are more likely to have one or more signs of advanced disease, including greater numbers of myeloma cells and kidney failure.

Treatment:
Treatment for multiple myeloma is focused on disease containment and suppression. If the disease is completely asymptomatic (i.e. there is a paraprotein and an abnormal bone marrow population but no end-organ damage), treatment may be deferred.

In addition to direct treatment of the plasma cell proliferation, bisphosphonates (e.g. pamidronate or zoledronic acid) are routinely administered to prevent fractures and erythropoietin to treat anemia.

Initial therapy
Initial treatment of multiple myeloma depends on the patient’s age and comorbidities. In recent years, high-dose chemotherapy with hematopoietic stem-cell transplantation has become the preferred treatment for patients under the age of 65. Prior to stem-cell transplantation, these patients receive an initial course of induction chemotherapy. The most common induction regimens used today are thalidomide–dexamethasone, bortezomib based regimens, and lenalidomide–dexamethasone.  Autologous stem cell transplantation (ASCT), the transplantation of a patient’s own stem cells after chemotherapy, is the most common type of stem cell transplantation for multiple myeloma. It is not curative, but does prolong overall survival. Allogeneic stem cell transplantation, the transplantation of a healthy person’s stem cells into the affected patient, has the potential for a cure, but is only available to a small percentage of patients. Furthermore, there is a 5-10% treatment-associated mortality rate.

Patients over age 65 and patients with significant concurrent illness often cannot tolerate stem cell transplantation. For these patients, the standard of care has been chemotherapy with melphalan and prednisone. Recent studies among this population   suggest improved outcomes with new chemotherapy regimens. Treatment with bortezomib, melphalan and prednisone had an estimated overall survival of 83% at 30 months, lenalidomide plus low-dose dexamethasone an 82% survival at 2 years and melphalan, prednisone and lenalidomide had a 90% survival at 2 years. Head-to-head studies comparing these regimens have not been performed.

A 2009 review noted “Deep venous thrombosis and pulmonary embolism are the major side effects of thalidomide and lenalidomide. Lenalidomide causes more myelosuppression, and thalidomide causes more sedation. Peripheral neuropathy and thrombocytopenia are major side effects of bortezomib.”

Treatment of related hyperviscosity syndrome may be required to prevent renal failure

Maintenance therapy
Sometimes after the initial treatment an ongoing maintenance therapy is offered. A 2009 review of maintenance therapy concluded “In younger patients, post-ASCT maintenance therapy with thalidomide appears to increase tumor burden reduction further, which translates in[to] prolonged PFS (progression free survival).”

Another 2009 review stated “the role of maintenance therapy with thalidomide, lenalidomide, or bortezomib for patients with multiple myeloma is not definitively established; such therapy should be performed only in the context of a clinical trial.”

Relapse
The natural history of myeloma is of relapse following treatment. Depending on the patient’s condition, the prior treatment modalities used and the duration of remission, options for relapsed disease include re-treatment with the original agent, use of other agents (such as melphalan, cyclophosphamide, thalidomide or dexamethasone, alone or in combination), and a second autologous stem cell transplant.

Later in the course of the disease, “treatment resistance” occurs. This may be a reversible effect,  and some new treatment modalities may re-sensitize the tumor to standard therapy. For patients with relapsed disease, bortezomib (or Velcade) is a recent addition to the therapeutic arsenal, especially as second line therapy, since 2005. Bortezomib is a proteasome inhibitor. Finally, lenalidomide (or Revlimid), a less toxic thalidomide analog, is showing promise for treating myeloma.

Renal failure in multiple myeloma can be acute (reversible) or chronic (irreversible). Acute renal failure typically resolves when the calcium and paraprotein levels are brought under control. Treatment of chronic renal failure is dependent on the type of renal failure and may involve dialysis.

Prognosis:
The International Staging System can help to predict survival, with a median survival of 62 months for stage 1 disease, 45 months for stage 2 disease, and 29 months for stage 3 disease.

Cytogenetic analysis of myeloma cells may be of prognostic value, with deletion of chromosome 13, non-hyperdiploidy and the balanced translocations t(4;14) and t(14;16) conferring a poorer prognosis. The 11q13 and 6p21 cytogenetic abnormalities are associated with a better prognosis.

Prognostic markers such as these are always generated by retrospective analyses, and it is likely that new treatment developments will improve the outlook for those with traditionally “poor-risk” disease.

SNP array karyotyping can detect copy number alterations of prognostic significance that may be missed by a targeted FISH panel. In MM, lack of a proliferative clone makes conventional cytogenetics informative in only ~30% of cases.

1.Virtual karyotyping identified chromosomal abnormalities in 98% of MM cases

2.del(12p13.31)is an independent adverse marker

3.amp(5q31.1) is a favorable marker

4.The prognostic impact of amp(5q31.1) over-rides that of hyperdiploidy and also identifies patients who greatly benefit from high-dose therapy.

Array-based karyotyping cannot detect balanced translocations, such as t(4;14) seen in ~15% of MM. Therefore, FISH for this translocation should also be performed if using SNP arrays to detect genome-wide copy number alterations of prognostic significance in MM.

The prognoses for patients with multiple myeloma, as those with other diseases, are not the same for everyone. The average age of onset is 70 years. Older patients often are experiencing other serious diseases, which affect survival. Younger patients might have much longer survival rates.

Some myeloma centers now employ genetic testing, which they call a “gene array.” By examining DNA oncologists can determine if patients are high risk or low risk. Myeloma patients identified as high risk are at high risk of having the cancer return more quickly following treatment. Low risk patients are at low risk of having the cancer return quickly following treatment.

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/Multiple_myeloma
http://www.bbc.co.uk/health/physical_health/conditions/in_depth/cancer/myeloma1.shtml
http://www.mayoclinic.com/health/multiple-myeloma/DS00415
http://www.medicinenet.com/multiple_myeloma/article.htm
http://www.nature.com/nrc/journal/v2/n12/fig_tab/nrc952_F5.html

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Infant jaundice

Definition:
Infant jaundice is a yellow discoloration in a newborn baby’s skin and eyes. Infant jaundice occurs because the baby’s blood contains an excess of bilirubin (bil-ih-ROO-bin), a yellow-colored pigment of red blood cells. Jaundice isn’t a disease itself but the name given to the yellow appearance of skin and the conjunctiva (whites) of the eyes.

click to see

Infant jaundice is a common condition, particularly in babies born before 38 weeks gestation (preterm babies) and breast-fed babies. Infant jaundice usually occurs because a baby’s liver isn’t mature enough to get rid of bilirubin in the bloodstream. In some cases, an underlying disease may cause jaundice.

Infant jaundice can be concerning as although the majority of causes are easily treated, some rarer causes are very serious. Also, high levels of unconjugated bilirubin can cause brain damage. This is virtually never seen now due to treatment with UVB light, but it means that it is very important that the baby receives proper treatment.

Types of Infant jaundice:
The most common types of jaundice are:

Physiological (normal) jaundice: occurring in most newborns, this mild jaundice is due to the immaturity of the baby’s liver, which leads to a slow processing of bilirubin. It generally appears at 2 to 4 days of age and disappears by 1 to 2 weeks of age.

Jaundice of prematurity: occurs frequently in premature babies since they are even less ready to excrete bilirubin effectively. Jaundice in premature babies needs to be treated at a lower bilirubin level than in full term babies in order to avoid complications.

Breastfeeding jaundice: jaundice can occur when a breastfeeding baby is not getting enough breast milk because of difficulty with breastfeeding or because the mother’s milk isn’t in yet. This is not caused by a problem with the breast milk itself, but by the baby not getting enough to drink.

Breast milk jaundice: in 1% to 2% of breastfed babies, jaundice may be caused by substances produced in their mother’s breast milk that can cause the bilirubin level to rise. These can prevent the excretion of bilirubin through the intestines. It starts after the first 3 to 5 days and slowly improves over 3 to 12 weeks.

Symptoms:
The main symptom of jaundice is yellow colouring of the skin and conjunctiva of the eyes. Jaundice can also make babies sleepy which can lead to poor feeding. Poor feeding can make jaundice worse as the baby can become dehydrated.

If a baby has conjugated jaundice, it may have white chalky stool (poo) and urine that is darker than normal. (The bilirubin that normally colours the stool is excreted in the urine.)

Medical advise should be sought urgently if:
•Jaundice is present in the first 24 hours of life
•Jaundice is present when the baby is 10 days old
•The baby has problems feeding or is very sleepy
•The stools are pale or the urine is very dark

Causes:
The main cause of jaundice is:
Excess bilirubin (hyperbilirubinemia). Bilirubin is the substance that causes the yellow color of jaundice. It’s a normal part of the waste produced when “used” red blood cells are broken down. Normally, the liver filters bilirubin from the bloodstream and releases it into the intestinal tract. Before birth, a mother’s liver removes bilirubin from the baby’s blood. The liver of a newborn is immature and often can’t remove bilirubin quickly enough, causing an excess of bilirubin. Jaundice due to these normal newborn conditions is called physiologic jaundice, and it typically appears on the second or third day of life.Other causes

A baby may have an underlying disorder that is causing jaundice. In these cases, jaundice often appears much earlier or much later than physiologic jaundice.

Diseases or conditions that can cause jaundice include:
*Internal bleeding (hemorrhage)
*An infection in your baby’s blood (sepsis)
*Other viral or bacterial infections
*An incompatibility between the mother’s blood and the baby’s blood
*A liver malfunction
*An enzyme deficiency
*An abnormality of your baby’s red blood cells

Risk Factors:
Problems with the blood may lead to a rapid breakdown of cells (haemolysis) – if the mother’s blood type isn’t compatible with her baby’s. For example, she may make antibodies that attack and destroy her baby’s red blood cells.

Hormone deficiencies such as low levels of thyroid hormone (hypothyroidism) or pituitary gland hormones (hypopituitarism) can trigger jaundice.

There may be inherited genetic problems with the enzymes that convert or break down bilirubin – these include rare conditions such as Crigler-Najjar syndrome, Gilbert’s syndrome, galactosaemia and tyrosinaemia.

There may be problems with the liver, such as biliary atresia, in which the tubes that drain bile from the liver are blocked. If spotted early, an operation can prevent long-term damage (which is why it is important to investigate jaundice that is still there at 10 days).

Diagnosis:
Doctors, nurses, and family members will watch for signs of jaundice at the hospital, and after the newborn goes home.

Any infant who appears jaundiced should have bilirubin levels measured right away. This can be done with a blood test.

Many hospitals check total bilirubin levels on all babies at about 24 hours of age. Hospitals use probes that can estimate the bilirubin level just by touching the skin. High readings need to be confirmed with blood tests.

Tests that will likely be done include:
•Complete blood count
•Coomb’s test
•Reticulocyte count
Further testing may be needed for babies who need treatment or whose total bilirubin levels are rising more quickly than expected.

Treatment:
Treatment is usually not needed.

When determining treatment, the doctor must consider:

•The baby’s bilirubin level
•How fast the level has been rising
•Whether the baby was born early (babies born early are more likely to be treated at lower bilirubin levels)
•How old the baby is now
Your child will need treatment if the bilirubin level is too high or is rising too quickly.

Keep the baby well hydrated with breast milk or formula. Frequent feedings (up to 12 times a day) encourage frequent bowel movements, which help remove bilirubin through the stools. Ask your doctor before giving your newborn extra formula.

Some newborns need to be treated before they leave the hospital. Others may need to go back to the hospital when they are a few days old. Treatment in the hospital usually lasts 1 to 2 days.

Sometimes special blue lights are used on infants whose levels are very high. This is called phototherapy. These lights work by helping to break down bilirubin in the skin.

The infant is placed under artificial light in a warm, enclosed bed to maintain constant temperature. The baby will wear only a diaper and special eye shades to protect the eyes. The American Academy of Pediatrics recommends that breastfeeding be continued through phototherapy, if possible. Rarely, the baby may have an intravenous (IV) line to deliver fluids.

If the bilirubin level is not too high or is not rising quickly, you can do phototherapy at home with a fiberoptic blanket, which has tiny bright lights in it. You may also use a bed that shines light up from the mattress.

•You must keep the light therapy on your child’s skin and feed your child every 2 to 3 hours (10 to 12 times a day).
•A nurse will come to your home to teach you how to use the blanket or bed, and to check on your child.
•The nurse will return daily to check your child’s weight, feedings, skin, and bilirubin levels.
•You will be asked to count the number of wet and dirty diapers.
In the most severe cases of jaundice, an exchange transfusion is required. In this procedure, the baby’s blood is replaced with fresh blood. Treating severely jaundiced babies with intravenous immunoglobulin may also be very effective at reducing bilirubin levels.

Prognosis:
Usually newborn jaundice is not harmful. For most babies, jaundice usually gets better without treatment within 1 to 2 weeks.

Very high levels of bilirubin can damage the brain. This is called kernicterus. However, the condition is almost always diagnosed before levels become high enough to cause this damage.

For babies who need treatment, the treatment is usually effective

Possible Complications:
Rare, but serious, complications from high bilirubin levels include:

•Cerebral palsy
•Deafness
•Kernicterus — brain damage from very high bilirubin levels

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/001559.htm
http://www.mayoclinic.com/health/infant-jaundice/DS00107
http://www.bbc.co.uk/health/physical_health/conditions/jaundice2.shtml

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Haemochromatosis

Definition:
Haemochromatosis is a disease caused by excess iron in the body.

Iron is needed in the diet to maintain good health, particularly for making red blood cells that carry oxygen around the body. These red blood cells contain large amounts of iron.

Lack of iron can cause anaemia, but excessive iron is toxic. The body has few ways of disposing of unwanted iron, so it builds up in tissues causing damage and disease.

Haemochromatosis – or genetic haemochromatosis (GH) – is a disorder that causes the body to absorb an excessive amount of iron from the diet.

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We can only use a limited amount of iron and any excess is deposited around the body. This accumulates mainly in the liver, but can also affect the heart, pancreas and pituitary gland, damaging these vital body organs and resulting in a deterioration of their functional capacity.

Haemochromatosis is more common in Caucasian or white populations, with about 1 in 300 to 1 in 400 affected. About half that number are affected in black populations.

Men are more likely to have hereditary haemochromatosis and suffer from it at an earlier age, as women regularly lose iron in menstruation or use stores in pregnancy.

Symptoms:
Although haemochromatosis and the potential for the condition to cause problems is present from birth, symptoms don’t usually become apparent until middle age.

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Common symptoms that might be noticed then include:

•weakness, tiredness and lack of energy
•joint pain and stiffness – particularly in the hands and fingers
•a tanned or bronzed appearance of the skin
•impotence in men
•shrinking of testicles
•weight loss
•abdominal pain
.
Later, more serious symptoms may develop including:

•diabetes
•arthritis
•heart problems
•enlargement or damage to the liver

Clinical presentation:
Organs commonly affected by haemochromatosis are the liver, heart, and endocrine glands.

Haemochromatosis may present with the following clinical syndromes:

*Cirrhosis of the liver
*Diabetes due to pancreatic islet cell failure
*Cardiomyopathy
*Arthritis (iron deposition in joints)
*Testicular failure
*Tanning of the skin

Causes:
The causes can be distinguished between primary cases (hereditary or genetically determined) and less frequent secondary cases (acquired during life). People of Celtic (Irish, Scottish, Welsh) origin have a particularly high incidence of whom about 10% are carriers of the gene and 1% sufferers from the condition.

 

Primary haemochromatosis:
The fact that most cases of haemochromatosis were inherited was well known for most of the 20th century, though they were incorrectly assumed to depend on a single gene. The overwhelming majority actually depend on mutations of the HFE gene discovered in 1996, but since then others have been discovered and sometimes are grouped together as “non-classical hereditary haemochromatosis”, “non-HFE related hereditary haemochromatosis”, or “non-HFE haemochromatosis

It is thought to be mainly caused by a mutation of a gene called HFE, which probably allows excess iron to be absorbed from the diet. This mutation is known as C282Y and to develop haemochromatosis you usually need two genes (one from each parent) to be C282Y.

However, not everyone with the mutation may develop the disease, and it may occur if only one C282Y gene is present.

Confusingly, another mutation labelled H63D elsewhere on the HFE gene may occur alone or with C282Y and also influence iron levels.

Other rare mutations may give rise to haemochromatosis, especially in children.

Secondary haemochromatosis:
*Severe chronic haemolysis of any cause, including intravascular haemolysis and ineffective erythropoiesis (haemolysis within the bone marrow).
*Multiple frequent blood transfusions (either whole blood or just red blood cells), which are usually needed either by individuals with hereditary anaemias (such as beta-thalassaemia major, sickle cell anaemia, and Diamond–Blackfan anaemia) or by older patients with severe acquired anaemias such as in myelodysplastic syndromes.
*Excess parenteral iron supplements, such as can acutely happen in iron poisoning
*Excess dietary iron
*Some disorders do not normally cause haemochromatosis on their own, but may do so in the presence of other predisposing factors. These include cirrhosis (especially related to alcohol abuse), steatohepatitis of any cause, porphyria cutanea tarda, prolonged haemodialysis, post-portacaval shunting.

Risk Factors:
The onset of hereditary haemochromatosis usually occurs between the ages of 30 and 60 as the build up of iron takes years.

However, a rapid form of the disease does affect children. If left untreated excess iron builds up in the organs especially the liver, heart and pancreas. This may cause heart or liver failure, which can be fatal.

Diagnosis:
There are several methods available for diagnosing and monitoring iron loading including:

*Serum ferritin
*Liver biopsy
*HFE
*MRI

Serum ferritin is a low-cost, readily available, and minimally invasive method for assessing body iron stores. However, the major problem with using it as an indicator of iron overload is that it can be elevated in a range of other medical conditions unrelated to iron levels including infection, inflammation, fever, liver disease, renal disease, and cancer. Also, total iron binding capacity may be low, but can also be normal.

The standard of practice in diagnosis of hemochromatosis was recently reviewed by Pietrangelo. Positive HFE analysis confirms the clinical diagnosis of hemochromatosis in asymptomatic individuals with blood tests showing increased iron stores, or for predictive testing of individuals with a family history of hemochromatosis. The alleles evaluated by HFE gene analysis are evident in ~80% of patients with hemochromatosis; a negative report for HFE gene does not rule out hemochromatosis. In a patient with negative HFE gene testing, elevated iron status for no other obvious reason, and family history of liver disease, additional evaluation of liver iron concentration is indicated. In this case, diagnosis of hemochromatosis is based on biochemical analysis and histologic examination of a liver biopsy. Assessment of the hepatic iron index (HII) is considered the “gold standard” for diagnosis of hemochromatosis.

MRI is emerging as an alternative to liver biopsy for measuring liver iron loading. For measuring liver iron concentrations, R2-MRI (also known as FerriScan)  has been validated and is coming into use in medical centers. It is not recommended in practice guidelines at this time

Prognosis:
A third of those untreated develop hepatocellular carcinoma.

Treatment:
Routine treatment in an otherwise-healthy person consists of regularly scheduled phlebotomies (bloodletting). When first diagnosed, the phlebotomies may be fairly frequent, perhaps as often as once a week, until iron levels can be brought to within normal range. Once iron and other markers are within the normal range, phlebotomies may be scheduled every other month or every three months depending upon the patient’s rate of iron loading.

For those unable to tolerate routine blood draws, there is a chelating agent available for use. The drug Deferoxamine binds with iron in the bloodstream and enhances its elimination via urine and faeces. Typical treatment for chronic iron overload requires subcutaneous injection over a period of 8–12 hours daily. Two newer iron chelating drugs that are licensed for use in patients receiving regular blood transfusions to treat thalassemia (and, thus, who develop iron overload as a result) are deferasirox and deferiprone.

Haemochromatosis is treated by:

•Reducing the amount of iron absorbed by the body – patients are advised to avoid iron-rich foods and alcohol.
•Removing excess iron from the body by removing blood from the body (venesection therapy or phlebotomy). Initially this may involve removing a unit of blood a week (sometimes for many months) until iron levels in the blood are normal. Then most people can be kept stable by removing a unit of blood every 2-3 months.

If phlebotomy is started before liver damage occurs the outlook is good, and the affected person can expect to live an otherwise normal life.

Acquired haemochromatosis is normally treated by a drug that binds iron and allows it to be excreted from the body.

Associated problems such as heart failure and diabetes are treated as appropriate.

Good advice:-
*Limit the amount of iron in your diet.
*Eating red or organ meats (such as liver) is not recommended.
*Iron supplements should also be avoided, including iron combined with other multivitamins.
*Vitamin C increases iron absorption from the gut and intake should also be limited.
*Avoid excess alcohol as this may make liver disease worse

Future prospects:
Your prospects largely depend on the stage at which the disease was diagnosed. Symptoms of tiredness and general weakness often improve, but joint problems may not.

Abdominal pain and liver enlargement can also lessen or disappear, and heart function may also improve with treatment.

However, liver cirrhosis is irreversible and a liver transplant may be required.

Patients with liver disease are also usually monitored for liver cancer, which can be a long-term complication.

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/haemochromatosis1.shtml
http://en.wikipedia.org/wiki/Iron_overload
http://www.netdoctor.co.uk/diseases/facts/haemochromatosis.htm

https://runkle-science.wikispaces.com/Haemochromatosis

http://www.ironxs.com.au/the-symptoms-of-haemochromatosis.html

http://www.goldbamboo.com/topic-t1404-a1-6Haemochromatosis.html

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Indian Almond (Terminalia catappa)

Botanical Name :Terminalia catappa
Family: Combretaceae
Genus: Terminalia
Species: T. catappa
Kingdom: Plantae
Division: Magnoliophyta
Class: Magnoliopsida
Order: Myrtales

Common Names:Desi Badam, Bengal almond, Singapore almond , Ebelebo, Malabar almond, Indian almond, Tropical almond, Sea almond, Beach Almond, Talisay tree, Umbrella tree, Abrofo Nkatie (Akan),

Habitat :The tree has been spread widely by humans and the native range is uncertain. It has long been naturalised in a broad belt extending from Africa to Northern Australia and New Guinea through Southeast Asia and Micronesia into the Indian Subcontinent.

Description:
Terminalia catappa is a large tropical tree in the Leadwood tree family, Combretaceae.It grows to 35 metres (115 ft) tall, with an upright, symmetrical crown and horizontal branches. The Terminalia catappa has corky, light fruit that is dispersed by water. The nut within the fruit is edible when fully ripe,tasting almost like almond. As the tree gets older, its crown becomes more flattened to form a spreading, vase shape. Its branches are distinctively arranged in tiers. The leaves are large, 15–25 centimetres (5.9–9.8 in) long and 10–14 centimetres (3.9–5.5 in) broad, ovoid, glossy dark green and leathery. They are dry-season deciduous; before falling, they turn pinkish-reddish or yellow-brown, due to pigments such as violaxanthin, lutein, and zeaxanthin.

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The flowers are monoecious, with distinct male and female flowers on the same tree. Both are 1 centimetre (0.39 in) in diameter, white to greenish, inconspicuous with no petals; they are produced on axillary or terminal spikes. The fruit is a drupe 5–7 centimetres (2.0–2.8 in) long and 3–5.5 centimetres (1.2–2.2 in) broad, green at first, then yellow and finally red when ripe, containing a single seed

Cultivation:Terminalia catappa  is grown in tropical countries all over the world.

Edible Uses:
The fruit is edible, tasting slightly acidic.

Chemical Constituents:
The leaves contain several flavonoids (like kaempferol or quercetin), several tannins (such as punicalin, punicalagin or tercatin), saponines and phytosterols. Due to this chemical richness, the leaves (and also the bark) are used in different traditional medicines for various purposes. For instances, in Taiwan fallen leaves are used as a herb to treat liver diseases. In Suriname, a tea made from the leaves is prescribed against dysentery and diarrhea. It is also thought that the leaves contain agents for prevention of cancers (although they have no demonstrated anticarcinogenic properties) and antioxidant as well as anticlastogenic characteristics.

Medicinal Uses;
Extracts from the leaves and bark of the plant have proven anticarcinogenic, anti-HIV and hepatoprotective properties (liver regenerating effects), including anti-diabetic effects.  The leaves and bark have been used traditionally in the South Pacific, for fungal related conditions.  It may be potentially beneficial for overall immune support, liver detoxification and antioxidant support.  The leaves contain agents for chemo-prevention of cancer and probably have anticarciogenic potential.  They also have a anticlastogenic effect (a process which causes breaks in chromosomes) due to their antioxidant properties. The kernel of Indian almond has shown aphrodisiac activity; it can probably be used in treatment of some forms of sexual inadequacies (premature ejaculation). Ethanol extract of the leaves shown potential in the treatment of sickle cell disorders. It appears as an anti-sickling agent for those that suffer from sickle cell.  It has been shown to be of benefit for microbial balancing.; as an aid to lowering high blood pressure and stress; as a treatment for some forms of liver disorders; as an aid in reducing the effect of several heart conditions .  In Asia it has long been known that the leaves of contain a toxic, secondary metabolite, which has antibacterial properties.
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From other countries: the leaves, bark and fruits are used for dysentery in Southeast Asia; dressing for rheumatic joints in Indonesia and India; the fruits and bark are a remedy for coughs in Samoa) and  asthma in Mexico; the fruits treat leprosy and  headaches in India and motion sickness in Mexico; the leaves eliminate intestinal parasites in the Philippines and treat eye problems, rheumatism and wounds in Samoa while they’re used to  stop bleeding during teeth extraction in Mexico; fallen leaves are used to treat liver diseases in Taiwan, and young leaves for colic in South America; the juice of the leaves is used for scabies, skin diseases and leprosy in India and Pakistan; the bark is a remedy for throat and mouth problems, stomach upsets and diarrhea in Samoa and for fever and dysentery in Brazil.

Other Uses:
The wood is red, solid and has high water resistance; it has been utilized in Polynesia for making canoes. In Tamil, almond is known “Nattuvadumai”.
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Keeping the leaves in an aquarium is said to lower the pH and heavy metal content of the water. It has been utilized in this way by Betta breeders in Thailand for many years. It’s also believed that it helps prevent fungus forming on the eggs of the fish.. Local hobbyists also use it for conditioning the betta’s water for breeding and hardening of the scales.
Terminalia catappa is widely grown in tropical regions of the world as an ornamental tree, grown for the deep shade its large leaves provide.

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.herbnet.com/Herb%20Uses_AB.htm
http://en.wikipedia.org/wiki/Terminalia_catappa

http://www.backyardnature.net/yucatan/almond-t.htm

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