A.D.A.M. Medical Encyclopedia.

Schistosomiasis
Bilharzia; Katayama fever; Swimmer's itch; Blood fluke
Schistosomiasis, also known as bilharzia, is a disease caused by parasitic worms. Although the worms that cause schistosomiasis are not found in the United States, more than 200 million people are infected worldwide. In terms of impact this disease is second only to malaria as the most devastating parasitic disease. Schistosomiasis is considered one of the Neglected Tropical Diseases (NTDs). The parasites that cause schistosomiasis live in certain types of freshwater snails. The infectious form of the parasite, known as cercariae, emerge from the snail, hence contaminating water. You can become infected when your skin comes in contact with contaminated freshwater. Most human infections are caused by Schistosoma mansoni, S. haematobium, or S. japonicum.

Epidemiology & Risk Factors

Schistosomiasis is an important cause of disease in many parts of the world, most commonly in places with poor sanitation. School-age children who live in these areas are often most at risk because they tend to spend time swimming or bathing in water containing infectious cercariae. If you live in, or travel to, areas where schistosomiasis is found and are exposed to contaminated freshwater, you are at risk. Areas where human schistosomiasis is found include: Schistosoma mansoni
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distributed throughout Africa: There is risk of infection in freshwater in southern and sub-Saharan Africaincluding the great lakes and rivers as well as smaller bodies of water. Transmission also occurs in the Nile River valley in Sudan and Egypt South America: including Brazil, Suriname, Venezuela Caribbean (risk is low): Dominican Republic, Guadeloupe, Martinique, and Saint Lucia.

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S. haematobium
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distributed throughout Africa: There is risk of infection in freshwater in southern and sub-Saharan Africaincluding the great lakes and rivers as well as smaller bodies of water. Transmission also occurs in the Nile River valley in Egypt and the Mahgreb region of North Africa.

found in areas of the Middle East

S. japonicum
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found in Indonesia and parts of China and Southeast Asia

S. mekongi
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found in Cambodia and Laos

S. intercalatum
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found in parts of Central and West Africa.

Diagnosis
Stool or urine samples can be examined microscopically for parasite eggs (stool for S. mansonior S. japonicum eggs and urine for S. haematobium eggs). The eggs tend to be passed intermittently and in small amounts and may not be detected, so it may be necessary to perform a blood (serologic) test.

Treatment
Safe and effective medication is available for treatment of both urinary and intestinal schistosomiasis. Praziquantel, a prescription medication, is taken for 1-2 days to treat infections caused by all Schistosoma species.

Resources for Health Professionals

On this Page Diagnosis Disease Treatment Diagnosis Examination of stool and/or urine for ova is the primary methods of diagnosis for suspected schistosome infections. The choice of sample to diagnose schistosomiasis depends on the species of parasite likely causing the infection. Adult stages ofS. mansoni, S. japonicum, S. mekongi, and S. intercalatum reside in the mesenteric venous plexus of infected hosts and eggs are shed in feces; S. haematobium adult

worms are found in the venous plexus of the lower urinary tract and eggs are shed in urine. Careful review of travel and residence history is critical for determining whether infection is likely and which species may be causing infection. It is important to remember that both S. mansoni and S. haematobium are endemic in some areas of sub-Saharan Africa; patients with freshwater exposures in those areas should have both stool and urine samples examined for eggs. Testing of stool or urine can be of limited sensitivity, particularly for travelers who may have lighter burden infections. To increase the sensitivity of stool and urine examination, three samples should be collected on different days. For S. haematobium, presence of hematuria can suggest infection but this test is more useful for population studies in Africa and is not sufficiently sensitive or specific for individual patient diagnosis. The eggs are shed intermittently and in low amounts in light-intensity infections. Serologic testing for antischistosomal antibody is indicated for diagnosis of travelers or immigrants from endemic areas who have not been treated appropriately for schistosomiasis in the past. Commonly used serologic tests detect antibody to the adult worm. For new infections, the serum sample tested should be collected at least 6 to 8 weeks after likely infection, to allow for full development of the parasite and antibody to the adult stage. Serologic testing may not be appropriate for determination of active infection in patients who have been repeatedly infected and treated in the past because specific antibody can persist despite cure. In these patients, serologic testing cannot distinguish resolved infection from active infection. An antigen test has been developed that can detect active infection based on the presence of schistosomal antigen, but this test is not commercially available in the United States and at this time is undergoing field evaluations for accurate diagnosis of low-intensity infections. Back to Top Disease The incubation period for patients with acute schistosomiasis is usually 14-84 days; however, many people are asymptomatic and have subclinical disease during both acute and chronic stages of infection. Persons with acute infection (also known as Katayama syndrome) may present with rash, fever, headache, myalgia, and respiratory symptoms. Often eosinophilia is present with hepato- and/or splenomegaly. DPDx Resources DPDx: Schistosomiasis Specimen collection: Stool Specimen collection: Urine

Clinical manifestations of chronic disease result from host immune responses to schistosome eggs. S. mansoni and S. japonicum eggs most commonly lodge in the blood vessels of the liver or intestine and can cause diarrhea, constipation, and blood in the stool. Chronic inflammation can lead to bowel wall ulceration, hyperplasia, and polyposis and, with heavy infections, to liver fibrosis and portal hypertension. S. haematobium eggs tend to lodge in the urinary tract causing damage, dysuria and hematuria. Chronic infections may increase the risk of bladder cancer. S. haematobium egg deposition has also been associated with damage to the female genital tract, causing female genital schistosomiasis that can affect the cervix, Fallopian tubes, and vagina and lead to increased susceptibility to other infections. Central nervous system lesions have been reported, but are rare. Disease is the result of ectopic deposition of eggs in the spinal cord (S. mansoni or S. haematobium) or brain (S. japonicum) and inflammatory reactions, typically the formation of granulomas that act as space occupying lesions. Back to Top Treatment Infections with all major Schistosoma species can be treated with praziquantel. The timing of treatment is important since praziquantel is most effective against the adult worm and requires the presence of a mature antibody response to the parasite. For travelers, treatment should be at least 6-8 weeks after last exposure to potentially contaminated freshwater. One study has suggested an effect of praziquantel on schistosome eggs lodged in tissues. Limited evidence of parasite resistance to praziquantel has been reported based on low cure rates in recently exposed or heavily infected populations; however, widespread clinical resistance has not occurred. Thus, praziquantel remains the drug of choice for treatment of schistosomiasis. Host immune response differences may impact individual response to treatment with praziquantel. Although a single course of treatment is usually curative, the immune response in lightly infected patients may be less robust, and repeat treatment may be needed after 2 to 4 weeks to increase effectiveness. If the pre-treatment stool or urine examination was positive for schistosome eggs, follow up examination at 1 to 2 months post-treatment is suggested to help confirm successful cure. Schistosoma species infection Schistosoma mansoni, S. haematobium, S. intercalatum S. japonicum, S. mekongi Praziquantel dose and Duration 40 mg/kg per day orally in two divided doses for one day 60 mg/kg per day orally in three

Schistosoma species infection

Praziquantel dose and Duration divided doses for one day

Praziquantel Oral praziquantel is available for human use in the United States. Note on Treatment in Pregnancy Note on Treatment During Lactation Note on Treatment in Pediatric Patients WHO Recommendations There is a lack of safety trial data for the use of praziquantel in children less than 4 years of age or pregnant women. However, this drug has been distributed widely in mass drug administration programs and WHO now recommends that pregnant women should be treated as part of those campaigns based on extensive experience with the drug and review of the veterinary and human evidence. Similarly, WHO reports that there is growing evidence that infected children as young as 1 year old can be effectively treated with praziquantel without serious side effects; however, the drug is commonly available in the form of large, hard-toswallow pills, which puts young children at risk for choking and other difficulties swallowing the drug.

Bavkround Schistosomiasis is a parasitic disease caused by blood flukes (trematodes) of the genus Schistosoma. After malaria and intestinal helminthiasis, schistosomiasis is the third most devastating tropical disease in the world, being a major source of morbidity and mortality for developing countries in Africa, South America, the Caribbean, the Middle East, and Asia. (See Epidemiology and Prognosis.)[1] More than 207 million people, 85% of who live in Africa, are infected withschistosomiasis,[1] and an estimated 700 million people are at risk of infection in 76 countries where the disease is considered endemic, as their agricultural work, domestic chores, and recreational activities expose them to infested water.[1, 2]Globally, 200,000 deaths are attributed to schistosomiasis annually.[3]Transmission is interrupted in some countries.[2] (See Etiology and Epidemiology.) Sometimes referred to as bilharzias, bilharziasis, or snail fever, schistosomiasis was discovered by Theodore Bilharz, a German surgeon working in Cairo, who first identified the etiological agent Schistosoma hematobium in 1851.[4] ASchistosoma egg is seen below.

Egg of Schistosoma hematobium, with its typical terminal spine.

Most human schistosomiasis is caused by S haematobium, S mansoni, and S japonicum. Less prevalent species, such as S mekongi and S intercalatum, may also cause systemic human disease. Less importantly, other schistosomes with avian or mammalian primary hosts can cause severe dermatitis in humans (eg, swimmer's itch secondary to Trichobilharzia ocellata). (See Etiology.)

Characteristics of schistosomiasis
Schistosomiasis is due to immunologic reactions to Schistosoma eggs trapped in tissues. Antigens released from the egg stimulate a granulomatous reaction involving T cells, macrophages, and eosinophils that results in clinical disease (see the image below). Symptoms and signs depend on the number and location of eggs trapped in the tissues. Initially, the inflammatory reaction is readily reversible. In the latter stages of the disease, the pathology is associated with collagen deposition and fibrosis, resulting in organ damage that may be only partially reversible. (See Pathophysiology, Etiology, and Presentation.)

Granuloma in the liver due to Schistosoma mansoni. The S mansoni egg is at the center of the granuloma.

Eggs can end up in the skin, brain, muscle, adrenal glands, and eyes. As the eggs penetrate the urinary system, they can find their way to the female genital region and form granulomas in the uterus, fallopian tube, and ovaries. Central nervous system (CNS) involvement occurs because of embolization of eggs from the portal mesenteric system to the brain and spinal cord via the paravertebral venous plexus.[5, 6, 7]

Snail hosts
The different species of Schistosoma have different types of snails serving as their intermediate hosts; these hosts are as follows[8, 9, 10] :

Biomphalaria for S mansoni Oncomelania for S japonicum Tricula (Neotricula aperta) for S mekongi Bulinus for S haematobium and S intercalatum

At-risk populations
Today, 120 million people are symptomatic with schistosomiasis, with 20 million having severe clinical disease.[1] More than 200,000 deaths per year are due to schistosomiasis in sub-Saharan Africa.[11] Women washing clothes in infested water are at risk.[12] Hygiene and playing in mud and water make children vulnerable to infection. Forty million women of childbearing age are infected.[13]Approximately 10 million women in Africa have schistosomiasis during pregnancy.[13] In endemic areas, the infection is usually acquired as a child.[2] In Brazil and Africa, refugee movements and migration to urban areas are introducing the disease to new locations. Increasing population size and corresponding needs for power and water have led to increased transmission. Infections are not uniformly distributed within communities. It has been estimated that 5-10% of an endemic community may be heavily infected, and the remainder has mild to moderate infections. The risk of infection is highest amongst those who lived near lakes or rivers. [14] In Uganda, almost no transmission was found to have occurred at altitudes greater than 1400 m or where the annual rainfall was less than 900 mm.[14] With the rise of tourism and travel, an increasing number of tourists are contracting it. Tourists often present with severe acute infection and unusual problems including paralysis.

The intensity and prevalence of infection rises with age and peaks usually between ages 15 and 20 years. In older adults, no significant change is found in the prevalence of disease, but the parasite burden or the intensity decreases.[8, 15, 16]The disease is not endemic in United States.

Complications
Complications of schistosomiasis include the following:

Gastrointestinal (GI) bleeding GI obstruction Malnutrition Schistosomal nephropathy Renal failure Pyelonephritis Hematuria Hemospermia Squamous cell bladder cancer Sepsis (Salmonella) Pulmonary hypertension Cor pulmonale Neuroschistosomiasis - Transverse myelitis, paralysis, and cerebral microinfarcts Infertility Severe anemia Low birth-weight babies Spontaneous abortion Higher risk for ectopic pregnancies End-organ disease Portal hypertension Obstructive uropathy Pregnancy complications from vulvar or fallopian granuloma Carcinoma of the liver, bladder, or gallbladder