Noma (disease)
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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 membrane A mucous membrane or mucosa is a membrane that lines various cavities in the body of an organism and covers the surface of internal organs. It consists of one or more layers of epithelial cells overlying a layer of loose connective tissue. It i ...
s of the mouth develop
ulcers An ulcer is a discontinuity or break in a bodily membrane that impedes normal function of the affected organ. According to Robbins's pathology, "ulcer is the breach of the continuity of skin, epithelium or mucous membrane caused by sloughing o ...
, 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 diges ...
of the tissues of the bones in the face.


Causes

The underlying causes for this disease are primarily poor
sanitation Sanitation refers to public health conditions related to clean drinking water and treatment and disposal of human excreta and sewage. Preventing human contact with feces is part of sanitation, as is hand washing with soap. Sanitation syste ...
and
malnutrition Malnutrition occurs when an organism gets too few or too many nutrients, resulting in health problems. Specifically, it is "a deficiency, excess, or imbalance of energy, protein and other nutrients" which adversely affects the body's tissues ...
. Although the causative organisms are common in many environments, this disease nearly exclusively affects extremely impoverished and malnourished children in
tropical The tropics are the regions of Earth surrounding the Equator. They are defined in latitude by the Tropic of Cancer in the Northern Hemisphere at N and the Tropic of Capricorn in the Southern Hemisphere at S. The tropics are also referred to ...
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 ...
to
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 ''Fusobacterium necrophorum'' is a species of bacteria responsible for Lemierre's syndrome and other medical problems. Biology ''F. necrophorum'' is a rod-shaped species of Gram-negative bacteria. It is an obligate anaerobe and is a common inh ...
'' and ''
Prevotella intermedia ''Prevotella intermedia'' (formerly ''Bacteroides intermedius'') is a gram-negative, obligate anaerobic pathogenic bacterium involved in periodontal infections, including gingivitis and periodontitis, and often found in acute necrotizing ulcerat ...
'' 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 Chronic periodontitis is one of the seven categories of periodontitis as defined by the American Academy of Periodontology 1999 class ...
,
Porphyromonas gingivalis ''Porphyromonas gingivalis'' belongs to the phylum ''Bacteroidota'' and is a nonmotile, Gram-negative, rod-shaped, anaerobic, pathogenic bacterium. It forms black colonies on blood agar. It is found in the oral cavity, where it is implicate ...
, Tannerella forsythia,
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 posit ...
'', and certain species of nonhemolytic ''
Streptococcus ''Streptococcus'' is a genus of gram-positive ' (plural ) or spherical bacteria that belongs to the family Streptococcaceae, within the order Lactobacillales (lactic acid bacteria), in the phylum Bacillota. Cell division in streptococci occurs ...
''). 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 Vitamin A is a fat-soluble vitamin and an essential nutrient for humans. It is a group of organic compounds that includes retinol, retinal (also known as retinaldehyde), retinoic acid, and several provitamin A carotenoids (most notably ...
and
Vitamin B B vitamins are a class of water-soluble vitamins that play important roles in cell metabolism and synthesis of red blood cells. Though these vitamins share similar names (B1, B2, B3, etc.), they are chemically distinct compounds that often coex ...
) * 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
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 ...
, 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 Plastic surgery is a surgical specialty involving the restoration, reconstruction or alteration of the human body. It can be divided into two main categories: reconstructive surgery and cosmetic surgery. Reconstructive surgery includes cranio ...
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 A disease is a particular abnormal condition that negatively affects the structure or function of all or part of an organism, and that is not immediately due to any external injury. Diseases are often known to be medical conditions that a ...
, and a
mortality rate Mortality rate, or death rate, is a measure of the number of deaths (in general, or due to a specific cause) in a particular population, scaled to the size of that population, per unit of time. Mortality rate is typically expressed in units of d ...
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 The World Health Organization (WHO) is a specialized agency of the United Nations responsible for international public health. The WHO Constitution states its main objective as "the attainment by all peoples of the highest possible level o ...
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 Hippocrates of Kos (; grc-gre, Ἱπποκράτης ὁ Κῷος, Hippokrátēs ho Kôios; ), also known as Hippocrates II, was a Greek physician of the classical period who is considered one of the most outstanding figures in the history o ...
and
Galen Aelius Galenus or Claudius Galenus ( el, Κλαύδιος Γαληνός; September 129 – c. AD 216), often Anglicized as Galen () or Galen of Pergamon, was a Greek physician, surgeon and philosopher in the Roman Empire. Considered to be o ...
, 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 World War II or the Second World War, often abbreviated as WWII or WW2, was a world war that lasted from 1939 to 1945. It involved the World War II by country, vast majority of the world's countries—including all of the great power ...
when it was
endemic Endemism is the state of a species being found in a single defined geographic location, such as an island, state, nation, country or other defined zone; organisms that are indigenous to a place are not endemic to it if they are also found else ...
to
Auschwitz Auschwitz concentration camp ( (); also or ) was a complex of over 40 Nazi concentration camps, concentration and extermination camps operated by Nazi Germany in Polish areas annexed by Nazi Germany, occupied Poland (in a portion annexed int ...
and
Belsen Bergen-Belsen , or Belsen, was a Nazi concentration camp in what is today Lower Saxony in northern Germany, southwest of the town of Bergen near Celle. Originally established as a prisoner of war camp, in 1943, parts of it became a concentr ...
concentration camps. The disease and treatments were studied by Berthold Epstein, a Czech physician and forced-labor prisoner who had recommended the study under
Josef Mengele , allegiance = , branch = Schutzstaffel , serviceyears = 1938–1945 , rank = '' SS''-'' Hauptsturmführer'' (Captain) , servicenumber = , battles = , unit = , awards = , commands = , ...
's direction.


Society and culture

Children and other noma survivors in Africa are helped by a few international
charitable organizations A charitable organization or charity is an organization whose primary objectives are philanthropy and social well-being (e.g. educational, religious or other activities serving the public interest or common good). The legal definition of a ...
, 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 ho ...
. 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 (MSF; pronounced ), also known as Doctors Without Borders, is a humanitarian medical non-governmental organisation (NGO) or charity of French origin known for its projects in conflict zones and in countries affected by endemic diseases. ...
. 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 Reconstructive surgery is surgery performed to restore normal appearance and function to body parts malformed by a disease or medical condition. Description Reconstructive surgery is a term with training, clinical, and reimbursement implicat ...
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 a ...
documentary presented by
Ben Fogle Benjamin Myer Fogle, (born 3 November 1973) is an English broadcaster, writer and adventurer, best known for his presenting roles with British television channels Channel 5, BBC and ITV. Early life Fogle is the son of English actress Juli ...
, ''Make Me a New Face: Hope for Africa's Hidden Children''.


See also

*
Necrotizing fasciitis Necrotizing fasciitis (NF), also known as flesh-eating disease, is a bacterial infection that results in the death of parts of the body's soft tissue. It is a severe disease of sudden onset that spreads rapidly. Symptoms usually include red or p ...
*
Noma neonatorum Noma neonatorum is a cutaneous condition, a manifestation of infection, usually ''Pseudomonas aeruginosa'' sepsis, and has been reported almost exclusively in developing countries. Despite the similarity of facial lesions, ''noma neonatorum'' is no ...


References


Further reading

* *


External links

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