Echinococcosis

From the Center for Disease Control and Prevention (CDC)

Echinococcosis is a parasitic disease caused by infection with tiny tapeworms of the genus Echinocococcus. Echinococcosis is classified as either cystic echinococcosis or alveolar echinococcosis.

Cystic echinocccosis (CE), also known as hydatid disease, is caused by infection with the larval stage of Echinococcus granulosus, a ~2-7 millimeter long tapeworm found in dogs (definitive host) and sheep, cattle, goats, and pigs (intermediate hosts). Although most infections in humans are asymptomatic, CE causes harmful, slowly enlarging cysts in the liver, lungs, and other organs that often grow unnoticed and neglected for years.

Alveolar echinococcosis (AE) disease is caused by infection with the larval stage of Echinococcus multilocularis, a ~1-4 millimeter long tapeworm found in foxes, coyotes, and dogs (definitive hosts). Small rodents are intermediate hosts for E. multilocularis. Although cases of AE in animals in endemic areas are relatively common, human cases are rare. AE poses a much greater health threat to people than CE, causing parasitic tumors that can form in the liver, lungs, brain, and other organs. If left untreated, AE can be fatal.

Frequently Asked Questions (FAQs)

Cystic echinococcosis (CE) disease results from being infected with the larval stage of Echinococcus granulosus, a tiny tapeworm (~2-7 millimeters in length) found in dogs (definitive host), sheep, cattle, goats, foxes, and pigs, amongst others (intermediate hosts). Most infections in humans are asymptomatic, but CE, also known as hydatid disease, causes slowly enlarging masses, most commonly in the liver and the lungs. Treatment can involve both medication and surgery.

More on: Cystic Echinococcosis (CE) FAQs

Alveolar echinococcosis (AE) disease results from being infected with the larval stage of Echinococcus multilocularis, a tiny tapeworm (~1-4 millimeters in length) found in foxes, coyotes, dogs, and cats (definitive hosts). Although human cases are rare, infection in humans causes parasitic tumors to form in the liver, and, less commonly, the lungs, brain, and other organs. If left untreated, infection with AE can be fatal.

More on: Alveolar Echinococcosis (AE) FAQs

Symptoms

Persons with cystic echinococcosis often remain asymptomatic until hydatid cysts containing the larval parasites grow large enough to cause discomfort, pain, nausea, and vomiting. The cysts grow over the course of several years before reaching maturity and the rate at which symptoms appear typically depends on the location of the cyst. The cysts are mainly found in the liver and lungs but can also appear in the spleen, kidneys, heart, bone, and central nervous system, including the brain and eyes. Cyst rupture is most frequently caused by trauma and may cause mild to severe anaphylactic reactions, even death, as a result of the release of cystic fluid.

Alveolar echinococcosis (AE) is characterized by parasitic tumors in the liver and may spread to other organs including the lungs and brain. In humans, the larval forms of E. multilocularis do not fully mature into cysts but cause vesicles that invade and destroy surrounding tissues and cause discomfort or pain, weight loss, and malaise. AE can cause liver failure and death because of the spread into nearby tissues and, rarely, the brain. AE is a dangerous disease resulting in a mortality rate between 50% and 75%, especially because most affected people live in remote locations and have poor health care.

Treatment

In the past, surgery was the only treatment for cystic echinococcal cysts. Chemotherapy, cyst puncture, and PAIR (percutaneous aspiration, injection of chemicals and reaspiration) have been used to replace surgery as effective treatments for cystic echinococcosis. However, surgery remains the most effective treatment to remove the cyst and can lead to a complete cure. Some cysts are not causing any symptoms and are inactive; those cysts often go away without any treatment.

The treatment of alveolar echinococcosis is more difficult than cystic echinococcosis and usually requires radical surgery, long-term chemotherapy, or both.

More on: Resources for Health Professionals: Treatment

Causes

Human echinococcosis (hydatidosis, or hydatid disease) is caused by the larval stages of cestodes (tapeworms) of the genus Echinococcus. Echinococcus granulosus causes cystic echinococcosis (CE), the form most frequently encountered; E. multilocularis causes alveolar echinococcosis (AE); E. vogeli causes polycystic echinococcosis; and E. oligarthrus is an extremely rare cause of human echinococcosis.

echinococcus_lifecycle

The adult Echinococcus granulosus (3 to 6 mm long) The number 1 resides in the small bowel of the definitive hosts, dogs or other canids. Gravid proglottids release eggs The number 2 that are passed in the feces. After ingestion by a suitable intermediate host (under natural conditions: sheep, goat, swine, cattle, horses, camel), the egg hatches in the small bowel and releases an oncosphere The number 3 that penetrates the intestinal wall and migrates through the circulatory system into various organs, especially the liver and lungs. In these organs, the oncosphere develops into a cyst The number 4 that enlarges gradually, producing protoscolices and daughter cysts that fill the cyst interior. The definitive host becomes infected by ingesting the cyst-containing organs of the infected intermediate host. After ingestion, the protoscolices The number 5 evaginate, attach to the intestinal mucosa The number 6, and develop into adult stages The number 1 in 32 to 80 days.

The same life cycle occurs with E. multilocularis (1.2 to 3.7 mm), with the following differences: the definitive hosts are foxes, and to a lesser extent dogs, cats, coyotes and wolves; the intermediate host are small rodents; and larval growth (in the liver) remains indefinitely in the proliferative stage, resulting in invasion of the surrounding tissues. With E. vogeli (up to 5.6 mm long), the definitive hosts are bush dogs and dogs; the intermediate hosts are rodents; and the larval stage (in the liver, lungs and other organs) develops both externally and internally, resulting in multiple vesicles. E. oligarthrus (up to 2.9 mm long) has a life cycle that involves wild felids as definitive hosts and rodents as intermediate hosts. Humans become infected by ingesting eggs The number 2, with resulting release of oncospheres The number 3 in the intestine and the development of cysts The number 4, The number 4, The number 4, The number 4, The number 4, The number 4in various organs.

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