Schistosomiasis
From Wikipedia, the free encyclopedia
Schistosomiasis |
Classification and external resources |
Skin vesicles on the forearm, created by the penetration of Schistosoma. Source: CDC |
ICD-10 |
B65 |
ICD-9 |
120 |
MeSH |
D012552 |
Schistosomiasis (also known as
bilharzia,
bilharziosis or
snail fever) is a
parasitic disease caused by several species of
trematodes (
platyhelminth infection, or "flukes"), a
parasitic worm of the genus
Schistosoma.
Snails serve as the intermediary agent between mammalian hosts.
Individuals within developing countries who cannot afford proper
sanitation facilities are often exposed to contaminated water
containing the infected snails.
[1]
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.
[2]
This disease is most commonly found in
Asia,
Africa, and
South America, especially in areas where the water contains numerous
freshwater snails, which may carry the parasite.
The disease affects many people in developing countries,
particularly children who may acquire the disease by swimming or
playing in infected water.
[2]
When children come into contact with a contaminated water source, the
parasitic larvae 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.
[1]
[edit] Classification
Species of
Schistosoma that can infect humans:
Avian schistosomiasis species cause
swimmer's itch and
clam digger itch
Species of
Schistosoma that can infect other animals:
S. bovis — normally infects cattle, sheep and goats in Africa, parts of Southern Europe and the Middle East
S. mattheei — normally infects cattle, sheep and goats in Central and Southern Africa
S. margrebowiei — normally infects antelope, buffalo and waterbuck in Southern and Central Africa
S. curassoni — normally infects domestic ruminants in West Africa
S. rodhaini — normally infects rodents and carnivores in parts of Central Africa
[edit] Signs and symptoms
Above all, schistosomiasis is a
chronic disease.
Many infections are subclinically symptomatic, with mild anemia and
malnutrition being common in endemic areas. Acute schistosomiasis
(Katayama's fever) may occur weeks after the initial infection,
especially by
S. mansoni and
S. japonicum. Manifestations include:
Occasionally
central nervous system lesions occur:
cerebral granulomatous disease may be caused by ectopic
S. japonicum eggs in the
brain, and granulomatous lesions around ectopic eggs in the
spinal cord from
S. mansoni and
S. haematobium infections may result in a
transverse myelitis with flaccid
paraplegia.
Calcification of the bladder wall on a plain x-ray image of the pelvis,
in a sub-Saharan man of 44 years old. This is due to urinary
schistosomiasis.
Continuing infection may cause granulomatous reactions and
fibrosis in the affected organs, which may result in manifestations that include:
Bladder cancer diagnosis and mortality are generally elevated in affected areas.
[edit] Pathophysiology
[edit] Life cycle
Schistosoma life cycle. Source: CDC
Schistosomes have a typical
trematode vertebrate-invertebrate lifecycle, with humans being the definitive host.
[edit] Snails
The life cycles of all five human schistosomes are broadly similar:
parasite eggs are released into the environment from infected
individuals, hatching on contact with fresh water to release the
free-swimming
miracidium. Miracidia infect
fresh-water snails
by penetrating the snail's foot. After infection, close to the site of
penetration, the miracidium transforms into a primary (mother)
sporocyst. Germ cells within the primary sporocyst will then begin
dividing to produce secondary (daughter) sporocysts, which migrate to
the snail's
hepatopancreas.
Once at the hepatopancreas, germ cells within the secondary sporocyst
begin to divide again, this time producing thousands of new parasites,
known as
cercariae, which are the larvae capable of infecting mammals.
Cercariae emerge daily from the snail host in a
circadian
rhythm, dependent on ambient temperature and light. Young cercariae are
highly mobile, alternating between vigorous upward movement and sinking
to maintain their position in the water. Cercarial activity is
particularly stimulated by water turbulence, by shadows and by
chemicals found on human skin.
[edit] Humans
Penetration of the human skin occurs after the cercaria have
attached to and explored the skin. The parasite secretes enzymes that
break down the skin's protein to enable penetration of the cercarial
head through the skin. As the cercaria penetrates the skin it
transforms into a migrating
schistosomulum stage.
Photomicrography of bladder in
S. hematobium infection, showing clusters of the parasite eggs with intense eosinophilia, Source: CDC
The newly transformed schistosomulum may remain in the skin for 2 days before locating a post-capillary
venule;
from here the schistosomulum travels to the lungs where it undergoes
further developmental changes necessary for subsequent migration to the
liver. Eight to ten days after penetration of the skin, the parasite
migrates to the
liver sinusoids.
S. japonicum migrates more quickly than
S. mansoni, and usually reaches the liver within 8 days of penetration. Juvenile
S. mansoni and
S. japonicum
worms develop an oral sucker after arriving at the liver, and it is
during this period that the parasite begins to feed on red blood cells.
The nearly-mature worms pair, with the longer female worm residing in
the
gynaecophoric channel of the shorter male. Adult worms are about 10 mm long. Worm pairs of
S. mansoni and
S. japonicum relocate to the
mesenteric or rectal veins.
S. haematobium
schistosomula ultimately migrate from the liver to the perivesical
venous plexus of the bladder, ureters, and kidneys through the
hemorrhoidal plexus.
Parasites reach maturity in six to eight weeks, at which time they begin to produce eggs. Adult
S. mansoni pairs residing in the mesenteric vessels may produce up to 300 eggs per day during their reproductive lives.
S. japonicum
may produce up to 3000 eggs per day. Many of the eggs pass through the
walls of the blood vessels, and through the intestinal wall, to be
passed out of the body in feces.
S. haematobium eggs pass
through the ureteral or bladder wall and into the urine. Only mature
eggs are capable of crossing into the digestive tract, possibly through
the release of
proteolytic
enzymes, but also as a function of host immune response, which fosters
local tissue ulceration. Up to half the eggs released by the worm pairs
become trapped in the mesenteric veins, or will be washed back into the
liver, where they will become lodged. Worm pairs can live in the body
for an average of four and a half years, but may persist up to 20 years.
Trapped eggs mature normally, secreting
antigens that elicit a vigorous
immune
response. The eggs themselves do not damage the body. Rather it is the
cellular infiltration resultant from the immune response that causes
the pathology classically associated with schistosomiasis.
[edit] Diagnosis
High powered detailed micrograph of
Schistosoma parasite eggs in human bladder tissue.
S. japonicum eggs in hepatic portal tract.
Microscopic identification of eggs in
stool or
urine
is the most practical method for diagnosis. The stool exam is the more
common of the two. For the measurement of eggs in the feces of
presenting patients the scientific unit used is
eggs per gram (epg). Stool examination should be performed when infection with
S. mansoni or
S. japonicum is suspected, and urine examination should be performed if
S. haematobium is suspected.
Eggs can be present in the stool in infections with all
Schistosoma
species. The examination can be performed on a simple smear (1 to 2 mg
of fecal material). Since eggs may be passed intermittently or in small
amounts, their detection will be enhanced by repeated examinations
and/or concentration procedures (such as the formalin-ethyl acetate
technique). In addition, for field surveys and investigational
purposes, the egg output can be quantified by using the
Kato-Katz technique (20 to 50 mg of fecal material) or the Ritchie technique.
Eggs can be found in the urine in infections with
S. japonicum and with
S. intercalatum (recommended time for collection: between noon and 3 PM). Detection will be enhanced by
centrifugation and examination of the sediment. Quantification is possible by using filtration through a
nucleopore membrane of a standard volume of urine followed by egg counts on the membrane. Investigation of
S. haematobium should also include a pelvic x-ray as bladder wall calcificaition is highly characteristic of chronic infection.
Recently a field evaluation of a novel handheld microscope was
undertaken in Uganda for the diagnosis of intestinal schistosomiasis by
a team led by Dr. Russell Stothard from the Natural History Museum of
London, working with the Schistosomiasis Control Initiative, London.
[7]
Tissue
biopsy (rectal biopsy for all species and biopsy of the bladder for
S. haematobium) may demonstrate eggs when stool or urine examinations are negative.
The eggs of
S. haematobium are ellipsoidal with a terminal spine,
S. mansoni eggs are also ellipsoidal but with a lateral spine,
S. japonicum eggs are spheroidal with a small knob.
Antibody detection can be useful in both clinical management and for
epidemiologic surveys.
[edit] Prevention
[edit] Eliminating or avoiding the snails
Prevention is best accomplished by eliminating the water-dwelling snails that are the
natural reservoir of the disease.
Acrolein,
copper sulfate, and
niclosamide
can be used for this purpose. Recent studies have suggested that snail
populations can be controlled by the introduction of, or augmentation
of existing,
crayfish populations; as with all ecological interventions, however, this technique must be approached with caution.
In 1989,
Aklilu Lemma and
Legesse Wolde-Yohannes received the
Right Livelihood Award for their research on the
sarcoca
plant, as a preventative measure for the disease by controlling the
snail. Concurrently, Dr Chidzere of Zimbabwe researched the similar
gopo berry
during the 1980s and found that it could be used in the control of
infected freshwater snails. In 1989 he drew attention to his concerns
that big chemical companies denigrated the gopo berry alternative for
snail control.
[8]
Gopo berries from hotter Ethiopia climates reputedly yield the best
results. Later studies were conducted between 1993 and 1995 by the
Danish Research Network for international health.
[9][10]
For many years from the 1950s onwards, civil engineers built vast dam
and irrigation schemes, oblivious to the fact that they would cause a
massive rise in water-borne infections from schistosomiasis. The
detailed specifications laid out in various UN documents since the
1950s could have minimized this problem. Irrigation schemes can be
designed to make it hard for the snails to colonize the water, and to
reduce the contact with the local population.
[11]
This has been cited as a classic case of the
relevance paradox
because guidelines on how to design these schemes to minimise the
spread of the disease had been published years before, but the
designers were unaware of them.
[12]
[edit] Treatment
Schistosomiasis is readily treated using a single oral dose of the drug
praziquantel annually.
[13] As with other major parasitic diseases, there is ongoing and extensive research into developing a
schistosomiasis vaccine that will prevent the parasite from completing its life cycle in humans. In 2009,
Eurogentec Biologics developed a vaccine against bilharziosis in partnership with
INSERM and researchers from the
Pasteur Institute.
[14][15][16]
The World Health Organization has developed guidelines for community
treatment of schistosomiasis based on the impact the disease has on
children in endemic villages:
[13]
- When a village reports more than 50 percent of children have blood in their urine, everyone in the village receives treatment.[13]
- When 20 to 50 percent of children have bloody urine, only school-age children are treated.[13]
- When less than 20 percent of children have symptoms, mass treatment is not implemented.[13]
The
Bill & Melinda Gates Foundation has recently funded an operational research program---the
Schistosomiasis Consortium for Operational Research and Evaluation (SCORE)
to answer strategic questions about how to move forward with
schistosomiasis control and elimination. The focus of SCORE is on
development of tools and evaluation of strategies for use in mass drug
administration campaigns.
Antimony has been used in the past to treat the disease. In low doses, this
toxic metalloid bonds to
sulfur atoms in
enzymes used by the parasite and kills it without harming the host. This treatment is not referred to in present-day
peer-review scholarship; praziquantel is universally used. Outside of the U.S., there is a drug available exclusively for treating
Schistosoma mansoni (
oxamniquine) and one exclusively for treating
S.hematobium (
metrifonate). While metrifonate has been discontinued for use by the British
National Health Service, a Cochrane review found it equally effective in treating urinary schistosomiasis as the leading drug, praziquantel.
[17]
Mirazid, an
Egyptian drug made from
myrrh, was under investigation for oral treatment of the disease up until 2005.
[18]
The efficacy of praziquantel was proven to be about 8 times than that
of Mirazid and therefore Mirazid was not recommended as a suitable
agent to control schistosomiasis.
[19]
[edit] Epidemiology
Disability-adjusted life year for schistosomiasis per 100,000 inhabitants.
no data
less than 50
50-75
75-100
100-150
150-200
200-250
250-300
300-350
350-400
400-450
450-500
more than 500
The disease is found in
tropical countries in
Africa, the
Caribbean, eastern
South America,
Southeast Asia and in the
Middle East.
Schistosoma mansoni is found in parts of South America and the Caribbean, Africa, and the Middle East;
S. haematobium in Africa and the Middle East; and
S. japonicum in the
Far East.
S. mekongi and
S. intercalatum are found locally in
Southeast Asia and central
West Africa, respectively.
Among human parasitic diseases, schistosomiasis (sometimes called bilharziasis) ranks second behind
malaria in terms of socio-economic and public health importance in tropical and subtropical areas. The disease is endemic in 74-76
[verification needed]
developing countries, infecting more than 207 million people, 85% of
whom live in Africa. They live in rural agricultural and peri-urban
areas, and placing more than 700 million people at risk.
[20]
Of the infected patients, 20 million suffer severe consequences from the disease.
[21] Some estimate that there are approximately 20,000 deaths related to schistosomiasis yearly.
[citation needed]
In many areas, schistosomiasis infects a large proportion of children
under 14 years of age. An estimated 600 million people worldwide are at
risk from the disease.
A few countries have eradicated the disease, and many more are working toward it.
[citation needed] The
World Health Organization
is promoting these efforts. In some cases, urbanization, pollution,
and/or consequent destruction of snail habitat has reduced exposure,
with a subsequent decrease in new infections. The most common way of
getting schistosomiasis in developing countries is by wading or
swimming in lakes, ponds and other bodies of water that are infested
with the
snails (usually of the genera
Biomphalaria,
Bulinus, or
Oncomelania) that are the
natural reservoirs of the
Schistosoma pathogen.
[edit] History
Schistosomiasis is known as bilharzia or bilharziosis in many countries, after
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.