Clostridial necrotizing enteritis
Encyclopedia
Clostridial necrotizing enteritis (CNE) -- also called enteritis necroticans and pigbel -- is an often fatal type of food poisoning caused by a β-toxin of Clostridium perfringens
Clostridium perfringens
Clostridium perfringens is a Gram-positive, rod-shaped, anaerobic, spore-forming bacterium of the genus Clostridium. C. perfringens is ever present in nature and can be found as a normal component of decaying vegetation, marine sediment, the intestinal tract of humans and other vertebrates,...

, Type C. It occurs in some developing countries, but was also documented in Germany
Germany
Germany , officially the Federal Republic of Germany , is a federal parliamentary republic in Europe. The country consists of 16 states while the capital and largest city is Berlin. Germany covers an area of 357,021 km2 and has a largely temperate seasonal climate...

 following World War II
World War II
World War II, or the Second World War , was a global conflict lasting from 1939 to 1945, involving most of the world's nations—including all of the great powers—eventually forming two opposing military alliances: the Allies and the Axis...

. The toxin is normally inactivated by certain proteolytic enzymes and by normal cooking, but when these protections are impeded, the disease emerges.

Etiology

All the factors collectively causing CNE are generally only present in the hinterlands of New Guinea
New Guinea
New Guinea is the world's second largest island, after Greenland, covering a land area of 786,000 km2. Located in the southwest Pacific Ocean, it lies geographically to the east of the Malay Archipelago, with which it is sometimes included as part of a greater Indo-Australian Archipelago...

 and parts of Africa
Africa
Africa is the world's second largest and second most populous continent, after Asia. At about 30.2 million km² including adjacent islands, it covers 6% of the Earth's total surface area and 20.4% of the total land area...

, Latin America
Latin America
Latin America is a region of the Americas where Romance languages  – particularly Spanish and Portuguese, and variably French – are primarily spoken. Latin America has an area of approximately 21,069,500 km² , almost 3.9% of the Earth's surface or 14.1% of its land surface area...

, and Asia
Asia
Asia is the world's largest and most populous continent, located primarily in the eastern and northern hemispheres. It covers 8.7% of the Earth's total surface area and with approximately 3.879 billion people, it hosts 60% of the world's current human population...

. These factors include protein deprivation
Malnutrition
Malnutrition is the condition that results from taking an unbalanced diet in which certain nutrients are lacking, in excess , or in the wrong proportions....

 (causing inadequate synthesis of trypsin protease (an enzyme), to which the toxin is very sensitive), poor food hygiene, episodic meat feasting, staple diets containing trypsin
Trypsin
Trypsin is a serine protease found in the digestive system of many vertebrates, where it hydrolyses proteins. Trypsin is produced in the pancreas as the inactive proenzyme trypsinogen. Trypsin cleaves peptide chains mainly at the carboxyl side of the amino acids lysine or arginine, except when...

 inhibitors (sweet potatoes), and infection by Ascaris
Ascaris
Ascaris is a genus of parasitic nematode worms known as the "giant intestinal roundworms". One species, A. suum, typically infects pigs, while another, A. lumbricoides, affects human populations, typically in sub-tropical and tropical areas with poor sanitation. A...

parasites which secrete a trypsin inhibitor. In New Guinea (origin of the term “pigbel”), the disease is usually spread through contaminated meat (especially pork) and perhaps by peanuts. (CNE was also diagnosed in post WWII Germany, where it was known as Darmbrand or "fire bowels").

Clinical aspects

CNE is a necrotizing inflammation of the small bowel (especially the jejunum but also the ileum). Clinical results may vary from mild diarrhea to a life-threatening sequence of severe abdominal pain, vomiting, bloody stool, ulceration of the small intestine with leakage (perforation) into the peritoneal cavity
Peritoneal cavity
The peritoneal cavity is a potential space between the parietal peritoneum and visceral peritoneum, that is, the two membranes that separate the organs in the abdominal cavity from the abdominal wall...

 and possible death within a single day due to peritonitis
Peritonitis
Peritonitis is an inflammation of the peritoneum, the serous membrane that lines part of the abdominal cavity and viscera. Peritonitis may be localised or generalised, and may result from infection or from a non-infectious process.-Abdominal pain and tenderness:The main manifestations of...

. Many patients exhibit meteorism. Treatment involves suppressing the toxin-producing organisms with antibiotics such as penicillin G or metronidazole
Metronidazole
Metronidazole is a nitroimidazole antibiotic medication used particularly for anaerobic bacteria and protozoa. Metronidazole is an antibiotic, amebicide, and antiprotozoal....

. About half of seriously ill patients require surgery for perforation, persistent intestinal obstruction, or failure to respond to the antibiotics. An investigational toxoid vaccine has been used successfully in some developing countries but is not available outside of research.

Other Clostridial toxemias

  • Leukemia
    Leukemia
    Leukemia or leukaemia is a type of cancer of the blood or bone marrow characterized by an abnormal increase of immature white blood cells called "blasts". Leukemia is a broad term covering a spectrum of diseases...

     patients, cancer chemotherapy
    Chemotherapy
    Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....

     recipients and others suffering from suppressed white blood cells (neutropenia
    Neutropenia
    Neutropenia, from Latin prefix neutro- and Greek suffix -πενία , is a granulocyte disorder characterized by an abnormally low number of neutrophils, the most important type of white blood cell...

    ) can be afflicted by a similar syndrome, neutropenic enterocolitis, in which the cecum
    Cecum
    The cecum or caecum is a pouch, connecting the ileum with the ascending colon of the large intestine. It is separated from the ileum by the ileocecal valve or Bauhin's valve, and is considered to be the beginning of the large intestine. It is also separated from the colon by the cecocolic...

     is targeted by Clostridium septicum
    Clostridium septicum
    Clostridium septicum is a gram positive, spore forming, obligate anaerobic bacterium.Clostridium septicum can cause gas gangrene, but unlike other Clostridium species like Clostridium perfringens, no trauma is necessary at the site of the infection. It is thought that the infection is established...

    in much the same way.

  • In neonatal intensive-care units, the syndrome of neonatal necrotizing enterocolitis may be caused in a similar way by C. perfringens
    Clostridium perfringens
    Clostridium perfringens is a Gram-positive, rod-shaped, anaerobic, spore-forming bacterium of the genus Clostridium. C. perfringens is ever present in nature and can be found as a normal component of decaying vegetation, marine sediment, the intestinal tract of humans and other vertebrates,...

    , C. butyricum
    Clostridium butyricum
    Clostridium butyricum is an anaerobic prokaryote that requires the absolute absence of oxygen to grow. It produces butyric acid which is an indicator of the presence of saccharolytic clostridia. It is an endospore-former and can live under rough conditions...

    , and C. difficile
    Clostridium difficile
    Clostridium difficile , also known as "CDF/cdf", or "C...

    , but this has not been proved.
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