bowang0911/MedQuAD huggingface dataset:
The health care provider prescribes antibiotics to destroy the T. whipplei bacteria and treat Whipple disease. Health care providers choose antibiotics that treat the infection in the small intestine and cross the blood-brain barriera layer of tissue around the brain. Using antibiotics that cross the blood-brain barrier ensures destruction of any bacteria that may have entered the patients brain and central nervous system.
                
The health care provider usually prescribes IV antibiotics for the first 2 weeks of treatment. Most patients feel relief from symptoms within the first week or two. A nurse or technician places an IV in the patients arm to give the antibiotics. IV antibiotics used to treat Whipple disease may include
                
- ceftriaxone (Rocephin)  - meropenem (Merrem I.V.)  - penicillin G (Pfizerpen)  - streptomycin (Streptomycin)
                
After a patient completes the IV antibiotics, the health care provider will prescribe long-term oral antibiotics. Patients receive long-term treatmentat least 1 to 2 yearsto cure the infection anywhere in the body. Oral antibiotics may include
                
- trimethoprim/sulfamethoxazole (Septra, Bactrim)a combination antibiotic  - doxycycline (Vibramycin)
                
Patients should finish the prescribed course of antibiotics to ensure the medication destroyed all T. whipplei bacteria in the body. Patients who feel better may still have the bacteria in the small intestine or other areas of the body for 1 to 2 years. A health care provider will monitor the patient closely, repeat the blood tests, and repeat the upper GI endoscopy with biopsy during and after treatment to determine whether T. whipplei is still present.
                
People may relapse during or after treatment. A health care provider will prescribe additional or new antibiotics if a relapse occurs. Some people will relapse years after treatment, so it is important for patients to schedule routine follow-ups with the health care provider. Most patients have good outcomes with an early diagnosis and complete treatment.
                
Health care providers treat patients with neurologic symptoms at diagnosis or during relapse more aggressively. Treatment may include
                
- a combination of antibiotics  - hydroxychloroquine (Plaquenil)an antimalarial medication  - weekly injections of interferon gammaa substance made by the body that activates the immune system  - corticosteroidsmedications that decrease inflammation
- Whipple disease is a rare bacterial infection that primarily affects the small intestine. Left untreated, Whipple disease gets worse and is usually life threatening.  - Bacteria called Tropheryma whipplei (T. whipplei) cause Whipple disease. T. whipplei infection can cause internal sores, also called lesions, and thickening of tissues in the small intestine.  - Anyone can get Whipple disease. However, it is more common in Caucasian men between 40 and 60 years old.  - Signs and symptoms of Whipple disease can vary widely from person to person. The most common symptoms of Whipple disease are      - diarrhea    - weight loss caused by malabsorption    - People with Whipple disease may have complications caused by malnutrition, which is due to damaged villi in the small intestine.  - The health care provider prescribes antibiotics to destroy the T. whipplei bacteria and treat Whipple disease.  - The health care provider usually prescribes intravenous (IV) antibiotics for the first 2 weeks of treatment. Most patients feel relief from symptoms within the first week or two.  - After a patient completes the IV antibiotics, the health care provider will prescribe long-term oral antibiotics.  - Most patients have good outcomes with an early diagnosis and complete treatment.
People with diabetes can lower their risk of sexual and urologic problems by keeping their blood glucose, blood pressure, and cholesterol levels close to the target numbers their health care provider recommends. Being physically active and maintaining a healthy weight can also help prevent the long-term complications of diabetes. For those who smoke, quitting will lower the risk of developing sexual and urologic problems due to nerve damage and also lower the risk for other health problems related to diabetes, including heart attack, stroke, and kidney disease.
                
More information about preventing diabetes complications is provided in the NIDDK health topic, Prevent diabetes problems: Keep your diabetes under control, available from the National Diabetes Information Clearinghouse at 1-800-860-8747.
The nerve damage of diabetes may cause sexual or urologic problems.
                
- Sexual problems in men with diabetes include      - erectile dysfunction    - retrograde ejaculation    - Sexual problems in women with diabetes include      - decreased vaginal lubrication and uncomfortable or painful intercourse    - decreased or no sexual desire    - decreased or absent sexual response    - Urologic problems in men and women with diabetes include      - bladder problems related to nerve damage, such as overactive bladder, poor control of sphincter muscles, and urine retention    - urinary tract infections    - Controlling diabetes through diet, physical activity, and medications as needed can help prevent sexual and urologic problems.  - Treatment is available for sexual and urologic problems.
Nerves that work poorly can lead to three different kinds of bladder control problems.
                
Overactive bladder. Damaged nerves may send signals to the bladder at the wrong time, causing its muscles to squeeze without warning. The symptoms of overactive bladder include
                
- urinary frequencydefined as urination eight or more times a day or two or more times at night  - urinary urgencythe sudden, strong need to urinate immediately  - urge incontinenceleakage of urine that follows a sudden, strong urge to urinate
                
Poor control of sphincter muscles. Sphincter muscles surround the urethra and keep it closed to hold urine in the bladder. If the nerves to the sphincter muscles are damaged, the muscles may become loose and allow leakage or stay tight when you are trying to release urine.
                
Urine retention. For some people, nerve damage means their bladder muscles do not get the message that it is time to release urine or are too weak to completely empty the bladder. If the bladder becomes too full, urine may back up and the increasing pressure may damage the kidneys. Or urine that stays too long may lead to an infection in the kidneys or bladder. Urine retention may also lead to overflow incontinence.
Many events or conditions can damage nerves and nerve pathways. Some of the most common causes are
                
- vaginal childbirth  - infections of the brain or spinal cord  - diabetes  - stroke  - accidents that injure the brain or spinal cord  - multiple sclerosis  - heavy metal poisoning
                
In addition, some children are born with nerve problems that can keep the bladder from releasing urine, leading to urinary infections or kidney damage.
The job of the sphincter muscles is to hold urine in the bladder by squeezing the urethra shut. If the urethral sphincter fails to stay closed, urine may leak out of the bladder. When nerve signals are coordinated properly, the sphincter muscles relax to allow urine to pass through the urethra as the bladder contracts to push out urine. If the signals are not coordinated, the bladder and the sphincter may contract at the same time, so urine cannot pass easily.
                
Drug therapy for an uncoordinated bladder and urethra. Scientists have not yet found a drug that works selectively on the urethral sphincter muscles, but drugs used to reduce muscle spasms or tremors are sometimes used to help the sphincter relax. Baclofen (Lioresal) is prescribed for muscle spasms or cramping in patients with multiple sclerosis and spinal injuries. Diazepam (Valium) can be taken as a muscle relaxant or to reduce anxiety. Drugs called alpha-adrenergic blockers can also be used to relax the sphincter. Examples of these drugs are alfuzosin (UroXatral), tamsulosin (Flomax), terazosin (Hytrin), and doxazosin (Cardura). The main side effects are low blood pressure, dizziness, fainting, and nasal congestion. All of these drugs have been used to relax the urethral sphincter in people whose sphincter does not relax well on its own.
                
Botox injection. Botulinum toxin type A (Botox) is best known as a cosmetic treatment for facial wrinkles. Doctors have also found that botulinum toxin is useful in blocking spasms like eye ticks or relaxing muscles in patients with multiple sclerosis. Urologists have found that injecting botulinum toxin into the tissue surrounding the sphincter can help it to relax. Although the FDA has approved botulinum toxin only for facial cosmetic purposes, researchers are studying the safety and effectiveness of botulinum toxin injection into the sphincter for possible FDA approval in the future.
Urine retention may occur either because the bladder wall muscles cannot contract or because the sphincter muscles cannot relax.
                
Catheter. A catheter is a thin tube that can be inserted through the urethra into the bladder to allow urine to flow into a collection bag. If you are able to place the catheter yourself, you can learn to carry out the procedure at regular intervals, a practice called clean intermittent catheterization. Some patients cannot place their own catheters because nerve damage affects their hand coordination as well as their voiding function. These patients need to have a caregiver place the catheter for them at regular intervals. If regular catheter placement is not feasible, the patients may need to have an indwelling catheter that can be changed less often. Indwelling catheters have several risks, including infection, bladder stones, and bladder tumors. However, if the bladder cannot be emptied any other way, then the catheter is the only way to stop the buildup of urine in the bladder that can damage the kidneys.
                
Urethral stent. Stents are small tube-like devices inserted into the urethra and allowed to expand, like a spring, widening the opening for urine to flow out. Stents can help prevent urine backup when the bladder wall and sphincter contract at the same time because of improper nerve signals. However, stents can cause problems if they move or lead to infection.
                
Surgery. Men may consider a surgery that removes the external sphincter-a sphincterotomy-or a piece of it-a sphincter resection-to prevent urinary retention. The surgeon will pass a thin instrument through the urethra to deliver electrical or laser energy that burns away sphincter tissue. Possible complications include bleeding that requires a transfusion and, rarely, problems with erections. This procedure causes loss of urine control and requires the patient to collect urine by wearing an external catheter that fits over the penis like a condom. No external collection device is available for women.
                
Urinary diversion. If other treatments fail and urine regularly backs up and damages the kidneys, the doctor may recommend a urinary diversion, a procedure that may require an outside collection bag attached to a stoma, a surgically created opening where urine passes out of the body. Another form of urinary diversion replaces the bladder with a continent urinary reservoir, an internal pouch made from sections of the bowel or other tissue. This method allows the person to store urine inside the body until a catheter is used to empty it through a stoma.
For people who are on dialysis or approaching total kidney failure, adequate nutrition is important for maintaining energy, strength, healthy sleep patterns, bone health, heart health, and good mental health. A persons treatment will dictate the type of diet that should be followed:
                
- People on hemodialysis must watch how much fluid they drink and avoid eating foods with too much sodium, potassium, and phosphorus.  - In contrast, people on peritoneal dialysisa type of dialysis that uses the lining of the abdomen, or belly, to filter the blood inside the bodymay be able to eat more potassium-rich foods because peritoneal dialysis removes potassium from the body more efficiently than hemodialysis.  - Both hemodialysis and peritoneal dialysis can remove proteins from the body, so anyone on either form of dialysis should eat protein-rich foods such as meat, fish, and eggs.
                
All dialysis centers and transplant clinics have a renal dietitian who specializes in helping people with kidney failure. People who are on dialysis or have a kidney transplant should talk with their clinics renal dietitian to develop a meal plan that will enhance the effectiveness of their treatment.
                
For more information about nutrition for people with advanced CKD or who are on dialysis, see NIDDK health topics, Nutrition for Advanced Chronic Kidney Disease in Adults or Kidney Failure: Eat Right to Feel Right on Hemodialysis.
- Kidney failure can affect a persons health in several ways.  - When the kidneys stop working, waste products build up in the blood, a condition known as uremia.  - People with kidney failure can avoid most of the problems of uremia by having regular dialysis treatments and limiting foods that contain sodium, potassium, and phosphorus.  - Anemia is common in people with chronic kidney disease (CKD), as well as those on dialysis, because the damaged kidneys slow the produc-tion of the hormone erythropoietin (EPO), which helps the bone marrow make red blood cells.  - People with kidney failure, particularly dialysis patients, have far higher rates of heart and blood vessel problems than people without kidney problems.  - People who have uremia often lose their appetite.  - Many people treated with hemodialysis complain of itchy skin.  - Kidney failure weakens the bones due to a condition called chronic kidney disease-mineral and bone disorder.  - Kidney failure can cause pain, stiffness, and fluid in the joints. These symptoms result from amyloidosis, a condition in which an abnormal protein in the blood called amyloid is deposited in tissues and organs, including the joints and tendons.  - People on dialysis often have insomnia, sleep apnea syndrome, and restless legs syndrome.  - People who have kidney failure and depression should tell their health care provider because depression can often be treated with adjustments to the diet and dialysis dose, medications, counseling, and cognitive behavioral therapy.  - For people who are on dialysis or approaching total kidney failure, adequate nutrition is important for maintaining energy, strength, healthy sleep patterns, bone health, heart health, and good mental health.  - All dialysis centers and transplant clinics have a renal dietitian who specializes in helping people with kidney failure. People who are on dialysis or have a kidney transplant should talk with their clinics renal dietitian to develop a meal plan that will enhance the effectiveness of their treatment.
Wilson disease is a genetic disease that prevents the body from removing extra copper. The body needs a small amount of copper from food to stay healthy; however, too much copper is poisonous. Normally, the liver filters extra copper and releases it into bile. Bile is a fluid made by the liver that carries toxins and wastes out of the body through the gastrointestinal tract. In Wilson disease, the liver does not filter copper correctly and copper builds up in the liver, brain, eyes, and other organs. Over time, high copper levels can cause life-threatening organ damage.
The liver is the bodys largest internal organ. The liver is called the bodys metabolic factory because of the important role it plays in metabolismthe way cells change food into energy after food is digested and absorbed into the blood. The liver has many important functions, including
                
- taking up, storing, and processing nutrients from foodincluding fat, sugar, and proteinand delivering them to the rest of the body when needed.  - making new proteins, such as clotting factors and immune factors.  - producing bile. In addition to carrying toxins and waste products out of the body, bile helps the body digest fats and the fat-soluble vitamins A, D, E, and K.  - removing waste products the kidneys cannot remove, such as fats, cholesterol, toxins, and medications.
                
A healthy liver is necessary for survival. The liver can regenerate most of its own cells when they become damaged. However, if injury to the liver is too severe or long lasting, regeneration is incomplete and the liver creates scar tissue.
Wilson disease is caused by an inherited autosomal recessive mutation, or change, in the ATP7B gene. In an autosomal recessive disease, the child has to inherit the gene mutation from both parents to have an increased likelihood for the disease. The chance of a child inheriting autosomal recessive mutations from both parents with a gene mutation is 25 percent, or one in four. If only one parent carries the mutated gene, the child will not get the disease, although the child may inherit one copy of the gene mutation. The child is called a carrier of the disease and can pass the gene mutation to the next generation. Genetic testing is a procedure that identifies changes in a patients genes and can show whether a parent or child is a carrier of a mutated gene. Autosomal recessive diseases are typically not seen in every generation of an affected family.
                
The following chart shows the chance of inheriting an autosomal recessive mutation from parents who both carry the mutated gene.
                

                
Genetic Diseases   Each cell contains thousands of genes that provide the instructions for making proteins for growth and repair of the body. If a gene has a mutation, the protein made by that gene may not function properly. Not all gene mutations cause a disease.   People have two copies of most genes; they inherit one copy from each parent. A genetic disease occurs when one or both parents pass a mutated gene to a child at conception. A genetic disease can also occur through a spontaneous gene mutation, meaning neither parent carries a copy of the mutated gene. Once a spontaneous gene mutation has occurred in a person, that person can pass the gene mutation on to a child.   Read more about genes and genetic conditions in the U.S. National Library of Medicines Genetics Home Reference at www.ghr.nlm.nih.gov.
People who have Wilson disease that is not treated or diagnosed early can have serious complications, such as
                
- cirrhosisscarring of the liver  - kidney damageas liver function decreases, the kidneys may be damaged  - persistent nervous system problems when nervous system symptoms do not resolve  - liver cancerhepatocellular carcinoma is a type of liver cancer that can occur in people with cirrhosis  - liver failurea condition in which the liver stops working properly  - death, if left untreated
A health care provider will treat Wilson disease with a lifelong effort to reduce and control the amount of copper in the body. Treatment may include
                
- medications  - changes in eating, diet, and nutrition  - a liver transplant
                
Medications
                
A health care provider will prescribe medications to treat Wilson disease. The medications have different actions that health care providers use during different phases of the treatment.
                
Chelating agents. Chelating agents are medications that remove extra copper from the body by releasing it from organs into the bloodstream. Once the cooper is in the bloodstream, the kidneys then filter the copper and pass it into the urine. A health care provider usually recommends chelating agents at the beginning of treatment. A potential side effect of chelating agents is that nervous system symptoms may become worse during treatment. The two medications available for this type of treatment include
                
- trientine (Syprine)the risk for side effects and worsening nervous system symptoms appears to be lower with trientine than d-penicillamine. Researchers are still studying the side effects; however, some health care providers prefer to prescribe trientine as the first treatment of choice because it appears to be safer.  - d-penicillaminepeople taking d-penicillamine may have other reactions or side effects, such as      - fever    - a rash    - kidney problems    - bone marrow problems
                
A health care provider will prescribe a lower dose of a chelating agent to women who are pregnant to reduce the risk of birth defects. A health care provider should consider future screening on any newborn whose parent has Wilson disease.
                
Zinc. A health care provider will prescribe zinc for patients who do not have symptoms, or after a person has completed successful treatment using a chelating agent and symptoms begin to improve. Zinc, taken by mouth as zinc salts such as zinc acetate (Galzin), blocks the digestive tracts absorption of copper from food. Although most people taking zinc usually do not experience side effects, some people may experience stomach upset. A health care provider may prescribe zinc for children with Wilson disease who show no symptoms. Women may take the full dosage of zinc safely during pregnancy.
                
Maintenance, or long term, treatment begins when symptoms improve and tests show that copper is at a safe level. Maintenance treatment typically includes taking zinc or a lower dose of a chelating agent. A health care provider closely monitors the person and reviews regular blood and urine tests to ensure maintenance treatment controls the copper level in the body.
                
Treatment for people with Wilson disease who have no symptoms may include a chelating agent or zinc in order to prevent symptoms from developing and stop or slow disease progression.
                
People with Wilson disease will take medications for the rest of their lives. Follow-up and adherence to the health care providers treatment plan is necessary to manage symptoms and prevent organ damage.