Noma (also known as necrotizing ulcerative stomatitis, gangrenous stomatitis, or cancrum oris) is a rapidly progressive and often fatal infection of the mouth and face. This disease predominantly affects children between the ages of two and six years old in the least developed countries around the world.

Signs and symptoms

The mucous membranes of the mouth develop ulcers, followed by rapid, painful tissue degeneration and

necrosis Necrosis () is a form of cell injury which results in the premature death of cells in living tissue by autolysis. Necrosis is caused by factors external to the cell or tissue, such as infection, or trauma which result in the unregulated dige ...

of the tissues of the bones in the face.

Causes

The underlying causes for this disease are primarily poor sanitation and malnutrition. Although the causative organisms are common in many environments, this disease nearly exclusively affects extremely impoverished and malnourished children in tropical regions. Noma is often reported as a

sequela A sequela (, ; usually used in the plural, sequelae ) is a pathological condition resulting from a disease, injury, therapy, or other trauma. Derived from the Latin word, meaning “sequel”, it is used in the medical field to mean a complication ...

acute necrotizing ulcerative gingivitis Acute necrotizing ulcerative gingivitis (ANUG) is a common, non-contagious infection of the gums with sudden onset. The main features are painful, bleeding gums, and ulceration of inter-dental papillae (the sections of gum between adjacent teeth ...

. '' Fusobacterium necrophorum'' and '' Prevotella intermedia'' are important bacterial pathogens in this disease process, interacting with one or more other bacterial organisms (such as Treponema denticola, ''

Treponema vincentii ''Treponema vincentii'' is a species of ''Treponema''. It is implicated as a pathogen in chronic periodontitis which can induce bone loss. This motile bacillus is a spirochaete A spirochaete () or spirochete is a member of the phylum Spir ...

, Porphyromonas gingivalis,

Tannerella forsythia ''Tannerella forsythia'' is an anaerobic, Gram-negative bacterial species of the Bacteroidota phylum. It has been implicated in periodontal diseases and is a member of the red complex of periodontal pathogens. ''T. forsythia'' was previously n ...

Staphylococcus aureus ''Staphylococcus aureus'' is a Gram-positive spherically shaped bacterium, a member of the Bacillota, and is a usual member of the microbiota of the body, frequently found in the upper respiratory tract and on the skin. It is often positive ...

'', and certain species of nonhemolytic '' Streptococcus''). Treatment of these organisms can help arrest the infection, but does not restore already-missing or disfigured tissue. Predisposing factors include: * malnutrition * vitamin deficiency (particularly deficiencies of Vitamin A and Vitamin B) * contaminated drinking water *

immunodeficiency Immunodeficiency, also known as immunocompromisation, is a state in which the immune system's ability to fight infectious diseases and cancer is compromised or entirely absent. Most cases are acquired ("secondary") due to extrinsic factors that a ...

* poor hygiene, particularly oral * recent illness (especially

, measles, malaria, and severe diarrhea) * living in proximity to livestock

Treatment

The progression of the disease can be halted with the use of antibiotics and improved nutrition; however, its physical effects are permanent and may require oral and maxillofacial surgery or reconstructive plastic surgery to repair. Reconstruction is usually very challenging and should be delayed until full recovery (usually about one year following initial intervention).

Prognosis

Noma is associated with a very high morbidity, and a mortality rate of approximately 90 percent.

Epidemiology

The disease affects mainly children in the poorest countries of Africa, Asia and South America. Most people who acquire this disease are between the ages of two and six years old. The World Health Organization estimates that 500,000 people are affected, and that 140,000 new cases are reported each year.

History

Known in antiquity to such physicians as Hippocrates and Galen, noma was once reported around the world, including Europe and the United States. With improvements in hygiene and nutrition, noma has disappeared from industrialized countries since the 20th century, except during World War II when it was endemic to

Auschwitz Auschwitz concentration camp ( (); also or ) was a complex of over 40 concentration and extermination camps operated by Nazi Germany in occupied Poland (in a portion annexed into Germany in 1939) during World War II and the Holocaust. It con ...

and Belsen concentration camps. The disease and treatments were studied by

Berthold Epstein Berthold Epstein (1 April 1890 – 9 June 1962) was a pediatrician, professor, and scientist who was conscripted as a doctor in the Auschwitz concentration camp during World War II. Life and career Berthold Epstein was born into a Jewish family ...

, a Czech physician and forced-labor prisoner who had recommended the study under Josef Mengele's direction.

Society and culture

Children and other noma survivors in Africa are helped by a few international charitable organizations, such as

Facing Africa Facing Africa is a British registered charity providing funds for the visits of teams of voluntary surgeons from the UK, Germany, France, Spain and Netherlands to Ethiopia to carry out facial reconstructive surgery on the victims of the disease ...

, a UK registered charity that helps affected Ethiopian, and Swiss charity

Winds of Hope Wind is the natural movement of air or other gases relative to a planet's surface. Winds occur on a range of scales, from thunderstorm flows lasting tens of minutes, to local breezes generated by heating of land surfaces and lasting a few ...

. There is one dedicated noma hospital in Nigeria, the Noma Children Hospital Sokoto, staffed by resident and visiting medical teams supported by Médecins Sans Frontières. In other countries, such as Ethiopia, international charities work in collaboration with the local

health care system Health, according to the World Health Organization, is "a state of complete physical, Mental health, mental and social well-being and not merely the absence of disease and infirmity".World Health Organization. (2006)''Constitution of the World H ...

to provide complex reconstructive surgery which can give back facial functions such as eating, speaking and smiling. Teams of volunteer medics coming from abroad are often needed to support the local capacity to address the most severe cases, which can be extremely challenging even for senior maxillofacial surgeons. On 10 June 2010 the work of such volunteer surgeons was featured in a UK

BBC Two BBC Two is a British free-to-air public broadcast television network owned and operated by the BBC. It covers a wide range of subject matter, with a remit "to broadcast programmes of depth and substance" in contrast to the more mainstream an ...

documentary presented by Ben Fogle, ''Make Me a New Face: Hope for Africa's Hidden Children''.

References

External links

{{DEFAULTSORT:Noma (Disease) Gangrene Bacterial diseases Health in Africa