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==Overview==
==Overview==
'''Schistosomiasis''' (also known as '''[[Theodor Bilharz|bilharzia]]''', '''bilharziosis''' or '''snail fever''') is a [[parasitic disease]] caused by several species of [[Trematoda|trematodes]] ([[platyhelminth]] infection, or "flukes"), a [[parasitic worm]] of the genus ''[[Schistosoma]]''.  [[Snail]]s serve as the intermediary agent between mammalian [[Host (biology)|host]]s. Individuals within developing countries who cannot afford proper water and [[sanitation]] facilities are often exposed to contaminated water containing the infected snails.<ref name="CIA - The World Factbook"> "CIA - The World Factbook." Central Intelligence Agency, 4 Apr. 2007 {{cite web|url=https://www.cia.gov/library/publications/the-world-factbook/docs/notesanddefs.html#2193 | title=cia.gov}}</ref>
Schistosomiasis is an [[infection]] acquired through contact with fresh water infested with the [[infectious]] larval form of [[Schistosoma]] flat worms ([[trematodes]]). Although schistosomiasis is a [[tropical disease]], [[Traveler's diarrhea|travelers]], students, immigrants, veterans, and tourists who previously lived in or visited regions where schistosomiasis is endemic, present worldwide. Early disease is usually [[asymptomatic]], unless [[Katayama fever]], an [[acute]] [[Immune complex disease|immune complex]] [[disease]] occurs. Late disease is symptomatic and includes [[Hepatosplenic T cell lymphoma|hepatosplenic]] schistosomiasis (pre [[sinusoidal]] [[portal hypertension]]), [[urinary]] and [[urogenital]] schistosomiasis ([[urinary obstruction]], [[genital]] symptoms), schistosomal [[glomerulopathy]] (chronic [[immune complex]] deposition in the [[kidney]]), and ectopic [[disease]] in areas such as the [[lungs]] and [[central nervous system]] (CNS). Diagnostic methods include visualization of Schistosoma eggs in formed [[stool]], [[urine]], and crushed [[Biopsy|biopsy tissues]]; [[Serological testing|serologic assays]]; and urinary antigen testing. Scarring patterns characteristic of hepatosplenic and urogenital disease may be seen on [[ultrasonography]]. [[Praziquantel|Praziquante]]<nowiki/>l is first-line treatment.


Although it has a low [[mortality rate]], schistosomiasis often is a [[chronic illness]] that can damage internal organs and, in children, impair growth and [[cognitive development]]. The [[urinary]] form of schistosomiasis is associated with increased risks for [[bladder cancer]] in adults. Schistosomiasis is the second most socioeconomically devastating parasitic disease after [[malaria]].<ref name="SCP"/>
==Historical Perspective==
Schistosomiasis is known as [[bilharzia]] or [[bilharziosis]] in many countries, after German physician [[Theodor Bilharz]], who first described the cause of urinary schistosomiasis in 1851. The first doctor who described the entire [[disease]] cycle was [[Pirajá da Silva]] in 1908. It was a common cause of death for ancient Egyptians in the Greco-Roman period.
 
==Classification==
Schistosomiasis may be classified based on the organ involvement into [[intestinal]] and [[urogenital]] schistosomiasis.
 
==Pathophysiology==
The pathogenesis of [[acute]] human schistosomiasis is mainly related to egg deposition and liberation of [[antigens]] of adult worms and eggs. A strong [[inflammatory response]] characterized by high levels of pro-inflammatory [[cytokines]], such as [[IL-1|interleukins 1]], [[Interleukin 6|6]],  [[TNF-α|tumor necrosis factor-α]], and by circulating [[immune complexes]] participates in the pathogenesis of the acute phase of the disease. Schistosomes have a typical [[Trematodes|trematode]] vertebrate-invertebrate lifecycle, with humans being the definitive host. The life cycles of all five human schistosomes are broadly similar. Infection can occur by penetration of the human skin by [[cercaria]] or following the handling of contaminated soil. [[Cercaria]] gets transformed into migrating schistosomulum stage in the [[skin]]. The [[incubation period]] for acute schistosomiasis is usually 14-84 days. Both the early and late manifestations of schistosomiasis are [[Immunologically privileged site|immunologically]] mediated. The major [[pathology]] of infection occurs with chronic schistosomiasis in which retention of eggs in the host tissues is associated with [[Chronic granulomatous disease|chronic granulomatous]] injury.
==Causes==
Schistosomiasis is caused by Schistosoma. The major intestinal schistosomes include ''[[Schistosoma japonicum|S.japonicum]]'', ''[[Schistosoma mekongi|S.mekongi]]'', ''[[Schistosoma mansoni|S.mansoni]]'', ''[[Schistosoma intercalatum|S.intercalatum.]]'' The major urogenital schistosome is ''[[Schistosoma haematobium|S.haematobium.]]''


This disease is most commonly found in [[Asia]], [[Africa]], and [[South America]], especially in areas where the water contains numerous [[freshwater snail]]s, which may carry the parasite.
==Differentiating Schistosomiasis from Other Diseases==
[[Schistosomiasis]] must be differentiated from tapeworm infections that cause [[abdominal pain]], [[fever]], [[chills]], [[cough]], and [[muscle aches]] such as like [[diphyllobothriasis]], [[hymenolepiasis]], and [[taeniasis]].


The disease affects many people in developing countries, particularly children who may acquire the disease by swimming or playing in infected water.<ref name="SCP">{{Cite web | author= The Carter Center| title=Schistosomiasis Control Program|url=http://www.cartercenter.org/health/schistosomiasis/index.html|accessdate=2008-07-17 | postscript= <!--None-->}}</ref>  When children come into contact with a contaminated water source, the parasitic [[larva]]e easily enter through their skin and further mature within organ tissues. As of 2009, 74 developing countries statistically identified epidemics of Schistosomiasis within their respective populations.<ref name="CIA - The World Factbook"/>
==Epidemiology and Demographics==
More than 600 million persons are exposed to Schistosoma [[parasites]], 200 million persons are [[infected]], and 20 million symptomatic cases of schistosomiasis are reported worldwide. All age groups are vulnerable to [[Schistosoma]] [[infection]], but school-aged children and adolescents living in endemic areas tend to have the highest intensity of disease. There is no [[Race (classification of human beings)|racial predilection]] to schistosomiasis. Schistosomiasis affects men and women equally.
==Risk Factors==
The most potent [[risk factor]] in the development of schistosomiasis is [[skin]] exposure to contaminated fresh water (wading, [[swimming]], [[washing]], or [[Working area|working]] in fresh water that is infested with [[Cercaria|cercariae]]). Other risk factors include travel to endemic areas.
==Screening==
Routine [[Screening (medicine)|screening]] of travelers for schistosomiasis is not recommended. Screening is recommended only to guide mass public health treatment programs to targeted villages in endemic areas.


==Historical Perspective==
==Natural History, Complications and Prognosis==
Schistosomiasis is known as bilharzia or bilharziosis in many countries, after German physician [[Theodor Bilharz]], who first described the cause of urinary schistosomiasis in 1851. The first doctor who described the entire disease cycle was [[Pirajá da Silva]] in 1908. It was a common cause of death for [[Ancient Egypt]]ians in the Greco-Roman Period.<ref>[http://www.ucalgary.ca/uofc/Others/HOM/Proceedings-2004.pdf#page=13 "Proceedings of the 13h Annual History of Medicine Days"], a medical historical paper from University of Calgary. March 2004.</ref>
If left untreated, most of the patients with schistosomiasis may progress to develop [[ulceration]] or [[cancer of the bladder]], [[liver]] or [[kidney failure]]. Common complications of schistosomiasis include [[hematuria]], [[malnutrition]], [[Polyps|intestinal polyps]], [[hydronephrosis]], [[glomerulonephritis]], bladder polyps, [[Bladder cancer|bladder cancer,]] [[infertility]], [[ectopic pregnancy]], [[renal failure]], and [[Cor pulmonale|cor-pulmonale]]. Depending on the extent of the [[disease]] progression at the time of diagnosis, the prognosis of schistosomiasis may vary. However, the prognosis is generally regarded as good with treatment.
==Classification==
 
There are five species of flatworms that cause schistosomiasis. Each causes a different clinical presentation of the [[disease]]. Schistosomiasis may localize in different parts of the body, and its localization determines its particular clinical profile.
==Diagnosis==
 
===History and Symptoms===
The majority of patients with schistosomiasis in early phase are asymptomatic, unless [[katayama fever]], an acute [[immune complex]] disease, occurs. Late Schistosomiasis is symptomatic and includes hepatosplenic schistosomiasis ([[Portal hypertension|pre sinusoidal portal hypertension]]), urinary and urogenital schistosomiasis ([[urinary obstruction]], genital symptoms), schistosomal [[glomerulopathy]] (chronic immune complex deposition in the [[kidney]]), and ectopic disease in areas such as the [[lungs]] and [[central nervous system]] (CNS).
===Physical Examination===
Common physical examination findings of schistosomiasis include generalized [[lymphadenopathy]], [[hepatosplenomegaly]], [[rash]], [[fever]], [[right upper quadrant tenderness]], [[urticaria]], [[bloody stool]].
 
===Laboratory Findings===
Methods for diagnosing schistosomiasis include visualization of Schistosoma eggs in formed [[stool]], [[urine]], and crushed biopsy tissues; serologic assays; and urinary antigen testing. Laboratory findings consistent with the diagnosis of schistosomiasis include detection of circulating antibodies to schistosomes and schistosomal [[antigen]] in serum.
===Electrocardiogram===
There are no ECG findings associated with schistosomiasis.
===X-ray===
A chest x-ray may be helpful in the diagnosis of [[pulmonary]] schistosomiasis. Findings on a chest x-ray suggestive pulmonary schistosomiasis include patchy infiltrates, signs of increased vascular and interstitial marking and [[Hilar lymphadenopathy|mild lymphadenopathy]].
 
===CT scan===
Head, abdomen and lung CT may be helpful in the diagnosis of schistosomiasis. Findings include nodular and ring-enhancing lesions with surrounding edema in neuro-schistosomiasis, calcified capsules in hepatosplenic schistosomiasis and [[interstitial fibrosis]] in pulmonary schistosomiasis.
===MRI===
There are no MRI findings associated with schistosomiasis.
===Ultrasound===
Ultrasound may be helpful in the diagnosis of urogenital and intestinal schistosomiasis. Findings on an ultrasound suggestive of urogenital schistosomiasis include scarring patterns of bladder wall. Findings on an ultrasound suggestive of intestinal schistosomiasis (periportal fibrosis) include multiple echogenic areas, each with central echo lucency.
===Other imaging findings===
There are no other imaging findings associated with schistosomiasis.
===Other diagnostic studies===
There are no other diagnostic studies associated with schistosomiasis.
 
==Treatment==
===Medical Therapy===
The mainstay of treatment for schistosomiasis is pharmacotherapy. [[Praziquantel]] is the drug of choice in treating schistosomiasis. [[Corticosteroids]] should be administered in addition to [[praziquantel]] in patients with symptoms due to neuro-schistosomiasis and patients with severe katayama fever. The goals of treatment of schistosomiasis are to eradicate the [[helminth]] and correct any sequelae of [[infection]]. While [[praziquantel]] is safe and highly effective in curing an infected patient, it does not prevent re-infection by [[Cercaria|cercariae]] and is thus not an optimum treatment for people living in endemic areas.
===Surgery===
Surgical intervention is not usually recommended for the management of schistosomiasis but may be indicated to treat associated complications. Surgery does not treat or eradicate Schistosoma infection. Surgical options include [[Portacaval shunt|portacaval shunting]], [[Esophageal varices|ligations of esophageal varices]], and surgical removal of genitourinary [[Granuloma|granulomatous masses]].
===Primary Prevention===
Effective measures for the primary prevention of schistosomiasis include avoiding swimming or wading in freshwater that may be infested with cercariae, water used for bathing should be brought to a rolling boil for 1 minute to kill any cercariae, and then cooled before bathing to avoid scalding. Vigorous towel drying after an accidental water exposure may help to prevent the Schistosoma parasite from penetrating the skin.
===Secondary Prevention===
Secondary preventive measures of schistosomiasis are similar to primary preventive measures.


==References==
==References==
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[[Category:Hepatology]]
[[Category:Hepatology]]
[[Category:Neglected diseases]]
[[Category:Neglected diseases]]
[[Category:Infectious disease]]

Latest revision as of 23:54, 6 February 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]

Overview

Schistosomiasis is an infection acquired through contact with fresh water infested with the infectious larval form of Schistosoma flat worms (trematodes). Although schistosomiasis is a tropical disease, travelers, students, immigrants, veterans, and tourists who previously lived in or visited regions where schistosomiasis is endemic, present worldwide. Early disease is usually asymptomatic, unless Katayama fever, an acute immune complex disease occurs. Late disease is symptomatic and includes hepatosplenic schistosomiasis (pre sinusoidal portal hypertension), urinary and urogenital schistosomiasis (urinary obstruction, genital symptoms), schistosomal glomerulopathy (chronic immune complex deposition in the kidney), and ectopic disease in areas such as the lungs and central nervous system (CNS). Diagnostic methods include visualization of Schistosoma eggs in formed stool, urine, and crushed biopsy tissues; serologic assays; and urinary antigen testing. Scarring patterns characteristic of hepatosplenic and urogenital disease may be seen on ultrasonography. Praziquantel is first-line treatment.

Historical Perspective

Schistosomiasis is known as bilharzia or bilharziosis in many countries, after German physician Theodor Bilharz, who first described the cause of urinary schistosomiasis in 1851. The first doctor who described the entire disease cycle was Pirajá da Silva in 1908. It was a common cause of death for ancient Egyptians in the Greco-Roman period.

Classification

Schistosomiasis may be classified based on the organ involvement into intestinal and urogenital schistosomiasis.

Pathophysiology

The pathogenesis of acute human schistosomiasis is mainly related to egg deposition and liberation of antigens of adult worms and eggs. A strong inflammatory response characterized by high levels of pro-inflammatory cytokines, such as interleukins 1, 6, tumor necrosis factor-α, and by circulating immune complexes participates in the pathogenesis of the acute phase of the disease. Schistosomes have a typical trematode vertebrate-invertebrate lifecycle, with humans being the definitive host. The life cycles of all five human schistosomes are broadly similar. Infection can occur by penetration of the human skin by cercaria or following the handling of contaminated soil. Cercaria gets transformed into migrating schistosomulum stage in the skin. The incubation period for acute schistosomiasis is usually 14-84 days. Both the early and late manifestations of schistosomiasis are immunologically mediated. The major pathology of infection occurs with chronic schistosomiasis in which retention of eggs in the host tissues is associated with chronic granulomatous injury.

Causes

Schistosomiasis is caused by Schistosoma. The major intestinal schistosomes include S.japonicum, S.mekongi, S.mansoni, S.intercalatum. The major urogenital schistosome is S.haematobium.

Differentiating Schistosomiasis from Other Diseases

Schistosomiasis must be differentiated from tapeworm infections that cause abdominal pain, fever, chills, cough, and muscle aches such as like diphyllobothriasis, hymenolepiasis, and taeniasis.

Epidemiology and Demographics

More than 600 million persons are exposed to Schistosoma parasites, 200 million persons are infected, and 20 million symptomatic cases of schistosomiasis are reported worldwide. All age groups are vulnerable to Schistosoma infection, but school-aged children and adolescents living in endemic areas tend to have the highest intensity of disease. There is no racial predilection to schistosomiasis. Schistosomiasis affects men and women equally.

Risk Factors

The most potent risk factor in the development of schistosomiasis is skin exposure to contaminated fresh water (wading, swimming, washing, or working in fresh water that is infested with cercariae). Other risk factors include travel to endemic areas.

Screening

Routine screening of travelers for schistosomiasis is not recommended. Screening is recommended only to guide mass public health treatment programs to targeted villages in endemic areas.

Natural History, Complications and Prognosis

If left untreated, most of the patients with schistosomiasis may progress to develop ulceration or cancer of the bladder, liver or kidney failure. Common complications of schistosomiasis include hematuria, malnutrition, intestinal polyps, hydronephrosis, glomerulonephritis, bladder polyps, bladder cancer, infertility, ectopic pregnancy, renal failure, and cor-pulmonale. Depending on the extent of the disease progression at the time of diagnosis, the prognosis of schistosomiasis may vary. However, the prognosis is generally regarded as good with treatment.

Diagnosis

History and Symptoms

The majority of patients with schistosomiasis in early phase are asymptomatic, unless katayama fever, an acute immune complex disease, occurs. Late Schistosomiasis is symptomatic and includes hepatosplenic schistosomiasis (pre sinusoidal portal hypertension), urinary and urogenital schistosomiasis (urinary obstruction, genital symptoms), schistosomal glomerulopathy (chronic immune complex deposition in the kidney), and ectopic disease in areas such as the lungs and central nervous system (CNS).

Physical Examination

Common physical examination findings of schistosomiasis include generalized lymphadenopathy, hepatosplenomegaly, rash, fever, right upper quadrant tenderness, urticaria, bloody stool.

Laboratory Findings

Methods for diagnosing schistosomiasis include visualization of Schistosoma eggs in formed stool, urine, and crushed biopsy tissues; serologic assays; and urinary antigen testing. Laboratory findings consistent with the diagnosis of schistosomiasis include detection of circulating antibodies to schistosomes and schistosomal antigen in serum.

Electrocardiogram

There are no ECG findings associated with schistosomiasis.

X-ray

A chest x-ray may be helpful in the diagnosis of pulmonary schistosomiasis. Findings on a chest x-ray suggestive pulmonary schistosomiasis include patchy infiltrates, signs of increased vascular and interstitial marking and mild lymphadenopathy.

CT scan

Head, abdomen and lung CT may be helpful in the diagnosis of schistosomiasis. Findings include nodular and ring-enhancing lesions with surrounding edema in neuro-schistosomiasis, calcified capsules in hepatosplenic schistosomiasis and interstitial fibrosis in pulmonary schistosomiasis.

MRI

There are no MRI findings associated with schistosomiasis.

Ultrasound

Ultrasound may be helpful in the diagnosis of urogenital and intestinal schistosomiasis. Findings on an ultrasound suggestive of urogenital schistosomiasis include scarring patterns of bladder wall. Findings on an ultrasound suggestive of intestinal schistosomiasis (periportal fibrosis) include multiple echogenic areas, each with central echo lucency.

Other imaging findings

There are no other imaging findings associated with schistosomiasis.

Other diagnostic studies

There are no other diagnostic studies associated with schistosomiasis.

Treatment

Medical Therapy

The mainstay of treatment for schistosomiasis is pharmacotherapy. Praziquantel is the drug of choice in treating schistosomiasis. Corticosteroids should be administered in addition to praziquantel in patients with symptoms due to neuro-schistosomiasis and patients with severe katayama fever. The goals of treatment of schistosomiasis are to eradicate the helminth and correct any sequelae of infection. While praziquantel is safe and highly effective in curing an infected patient, it does not prevent re-infection by cercariae and is thus not an optimum treatment for people living in endemic areas.

Surgery

Surgical intervention is not usually recommended for the management of schistosomiasis but may be indicated to treat associated complications. Surgery does not treat or eradicate Schistosoma infection. Surgical options include portacaval shunting, ligations of esophageal varices, and surgical removal of genitourinary granulomatous masses.

Primary Prevention

Effective measures for the primary prevention of schistosomiasis include avoiding swimming or wading in freshwater that may be infested with cercariae, water used for bathing should be brought to a rolling boil for 1 minute to kill any cercariae, and then cooled before bathing to avoid scalding. Vigorous towel drying after an accidental water exposure may help to prevent the Schistosoma parasite from penetrating the skin.

Secondary Prevention

Secondary preventive measures of schistosomiasis are similar to primary preventive measures.

References