Noma (disease)
Encyclopedia
Noma also known as cancrum oris or gangrenous stomatitis, is a gangrenous
disease
leading to tissue
destruction of the face, especially the mouth
and cheek
.
and Prevotella intermedia
are thought to be key players in the process and interact with one or more other bacterial organisms (such as Borrelia vincentii, Porphyromonas gingivalis
, Tannerella forsynthesis, Treponema denticola
, Staphylococcus aureus
, and nonhemolytic Streptococcus
spp).
The reported predisposing factors include:
Noma was observed in Nazi concentration camps
in World War II
, and was studied by Nazi physician Josef Mengele
. In many cases a recent debilitating illness (usually measles
and sometimes herpes simplex
, varicella (chicken pox), scarlet fever
, malaria
, tuberculosis
, gastroenteritis
, and bronchopneumonia
) precedes the appearance of noma as well as cancers such as leukemia
. In many instances the infection begins as necrotizing ulcerative gingivitis (NUG). Early presentation is unclear as noma is often well progressed at initial presentation.
Noma, unlike most infections, is able to spread through anatomic barriers such as muscle.
In the developed world, noma has virtually disappeared except for an occasional case related to immunosuppressive conditions such as HIV infection, severe combined immunodeficiency syndrome, or intense immunosuppressive therapy. The World Health Organization (WHO) estimates the global yearly incidence to be approximately 140,000, of which 100,000 are between 1 and 7 years old and living in sub-Saharan Africa.
s of the mouth develop ulcers, and rapid, painless tissue degeneration ensues, which can degrade tissues of the bones in the face.
In a condition sometimes called noma pudendi, noma can also cause tissue damage to the genitals.
The disease is associated with high morbidity and mortality
and mainly affects children under the age of twelve in the poorest countries of Africa
. Children in Asia
and some countries of South America
are also affected. Most children who get the disease are between the ages of two and six years old. The WHO
estimates that 500,000 people are affected, and that 140,000 new cases are reported each year. Estimates claim that 80 - 90% of noma cases die from the disease.
and Galen
, noma was once reported around the world, including Europe
and the United States
. With the improvement 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 and Belsen concentration camps. The disease and treatments were studied by Berthold Epstein
, a Czech inmate physician who had recommended the study to Josef Mengele
, the Nazi doctor.
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 reconstructive plastic surgery to repair. Reconstruction is usually very challenging and it should be delayed until full recovery (usually about one year following initial intervention).
Children and other noma survivors in Africa
are helped by a few international charitable organizations. There is one dedicated noma hospital in Nigeria
, the Noma Children Hospital Sokoto, staffed by resident and visiting medical teams. In other countries, like Ethiopia
, international charities work in collaboration with the local health care system
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 BBC2 documentary presented by Ben Fogle
titled 'Make me a new face - Hope for Africa
's hidden children'.
Gangrene
Gangrene is a serious and potentially life-threatening condition that arises when a considerable mass of body tissue dies . This may occur after an injury or infection, or in people suffering from any chronic health problem affecting blood circulation. The primary cause of gangrene is reduced blood...
disease
Disease
A disease is an abnormal condition affecting the body of an organism. It is often construed to be a medical condition associated with specific symptoms and signs. It may be caused by external factors, such as infectious disease, or it may be caused by internal dysfunctions, such as autoimmune...
leading to tissue
Tissue (biology)
Tissue is a cellular organizational level intermediate between cells and a complete organism. A tissue is an ensemble of cells, not necessarily identical, but from the same origin, that together carry out a specific function. These are called tissues because of their identical functioning...
destruction of the face, especially the mouth
Mouth
The mouth is the first portion of the alimentary canal that receives food andsaliva. The oral mucosa is the mucous membrane epithelium lining the inside of the mouth....
and cheek
Cheek
Cheeks constitute the area of the face below the eyes and between the nose and the left or right ear. They may also be referred to as jowls. "Buccal" means relating to the cheek. In humans, the region is innervated by the buccal nerve...
.
Causes
Noma is a rapidly progressive, polymicrobial, opportunistic infection that occurs during periods of compromised immune function. Fusobacterium necrophorumFusobacterium necrophorum
Fusobacterium necrophorum is the species of Fusobacterium that is responsible for Lemierre's syndrome, and appears to be responsible for 10% of all acute sore throats, 21% of all recurring sore throats, and 23% of peritonsillar abscesses with the remainder being caused by Group A streptococci or...
and Prevotella intermedia
Prevotella intermedia
Prevotella intermedia is a gram-negative obligate anaerobic pathogen involved in periodontal infections, including gingivitis and periodontitis, and often found in Acute necrotizing ulcerative gingivitis . It is commonly isolated from Dentoalveolar abscesses, where obligate anaerobes...
are thought to be key players in the process and interact with one or more other bacterial organisms (such as Borrelia vincentii, Porphyromonas gingivalis
Porphyromonas gingivalis
Porphyromonas gingivalis belongs to the phylum Bacteroidetes and is a non-motile, gram-negative, rod-shaped, anaerobic pathogenic bacterium. It forms black colonies on blood agar....
, Tannerella forsynthesis, Treponema denticola
Treponema denticola
Treponema denticola is a motile and highly proteolytic bacterium. The Gram-negative oral spirochete is associated with the incidence and severity of human periodontal disease. Treponema denticola levels in the mouth are elevated in patients with periodontal diseases and the species is considered...
, Staphylococcus aureus
Staphylococcus aureus
Staphylococcus aureus is a facultative anaerobic Gram-positive coccal bacterium. It is frequently found as part of the normal skin flora on the skin and nasal passages. It is estimated that 20% of the human population are long-term carriers of S. aureus. S. aureus is the most common species of...
, and nonhemolytic Streptococcus
Streptococcus
Streptococcus is a genus of spherical Gram-positive bacteria belonging to the phylum Firmicutes and the lactic acid bacteria group. Cellular division occurs along a single axis in these bacteria, and thus they grow in chains or pairs, hence the name — from Greek στρεπτος streptos, meaning...
spp).
The reported predisposing factors include:
- Malnutrition or dehydration
- Poor oral hygiene
- Poor sanitation
- Unsafe drinking water
- Proximity to unkempt livestock
- Recent illness
- Malignancy
- An immunodeficiency disorder, including AIDSAIDSAcquired immune deficiency syndrome or acquired immunodeficiency syndrome is a disease of the human immune system caused by the human immunodeficiency virus...
Noma was observed in Nazi concentration camps
Nazi concentration camps
Nazi Germany maintained concentration camps throughout the territories it controlled. The first Nazi concentration camps set up in Germany were greatly expanded after the Reichstag fire of 1933, and were intended to hold political prisoners and opponents of the regime...
in 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...
, and was studied by Nazi physician Josef Mengele
Josef Mengele
Josef Rudolf Mengele , also known as the Angel of Death was a German SS officer and a physician in the Nazi concentration camp Auschwitz-Birkenau. He earned doctorates in anthropology from Munich University and in medicine from Frankfurt University...
. In many cases a recent debilitating illness (usually measles
Measles
Measles, also known as rubeola or morbilli, is an infection of the respiratory system caused by a virus, specifically a paramyxovirus of the genus Morbillivirus. Morbilliviruses, like other paramyxoviruses, are enveloped, single-stranded, negative-sense RNA viruses...
and sometimes herpes simplex
Herpes simplex
Herpes simplex is a viral disease caused by both Herpes simplex virus type 1 and type 2 . Infection with the herpes virus is categorized into one of several distinct disorders based on the site of infection. Oral herpes, the visible symptoms of which are colloquially called cold sores or fever...
, varicella (chicken pox), scarlet fever
Scarlet fever
Scarlet fever is a disease caused by exotoxin released by Streptococcus pyogenes. Once a major cause of death, it is now effectively treated with antibiotics...
, malaria
Malaria
Malaria is a mosquito-borne infectious disease of humans and other animals caused by eukaryotic protists of the genus Plasmodium. The disease results from the multiplication of Plasmodium parasites within red blood cells, causing symptoms that typically include fever and headache, in severe cases...
, tuberculosis
Tuberculosis
Tuberculosis, MTB, or TB is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. Tuberculosis usually attacks the lungs but can also affect other parts of the body...
, gastroenteritis
Gastroenteritis
Gastroenteritis is marked by severe inflammation of the gastrointestinal tract involving both the stomach and small intestine resulting in acute diarrhea and vomiting. It can be transferred by contact with contaminated food and water...
, and bronchopneumonia
Bronchopneumonia
Bronchopneumonia or bronchial pneumonia or "Bronchogenic pneumonia" is the acute inflammation of the walls of the bronchioles...
) precedes the appearance of noma as well as cancers such as 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...
. In many instances the infection begins as necrotizing ulcerative gingivitis (NUG). Early presentation is unclear as noma is often well progressed at initial presentation.
Noma, unlike most infections, is able to spread through anatomic barriers such as muscle.
In the developed world, noma has virtually disappeared except for an occasional case related to immunosuppressive conditions such as HIV infection, severe combined immunodeficiency syndrome, or intense immunosuppressive therapy. The World Health Organization (WHO) estimates the global yearly incidence to be approximately 140,000, of which 100,000 are between 1 and 7 years old and living in sub-Saharan Africa.
Presentation and prognosis
The mucous membraneMucous membrane
The mucous membranes are linings of mostly endodermal origin, covered in epithelium, which are involved in absorption and secretion. They line cavities that are exposed to the external environment and internal organs...
s of the mouth develop ulcers, and rapid, painless tissue degeneration ensues, which can degrade tissues of the bones in the face.
In a condition sometimes called noma pudendi, noma can also cause tissue damage to the genitals.
The disease is associated with high morbidity and mortality
Death
Death is the permanent termination of the biological functions that sustain a living organism. Phenomena which commonly bring about death include old age, predation, malnutrition, disease, and accidents or trauma resulting in terminal injury....
and mainly affects children under the age of twelve in the poorest countries 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...
. Children in 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...
and some countries of South America
South America
South America is a continent situated in the Western Hemisphere, mostly in the Southern Hemisphere, with a relatively small portion in the Northern Hemisphere. The continent is also considered a subcontinent of the Americas. It is bordered on the west by the Pacific Ocean and on the north and east...
are also affected. Most children who get the disease are between the ages of two and six years old. The WHO
Who
Who may refer to:* Who , an English-language pronoun* who , a Unix command* Who?, one of the Five Ws in journalism- Art and entertainment :* Who? , a 1958 novel by Algis Budrys...
estimates that 500,000 people are affected, and that 140,000 new cases are reported each year. Estimates claim that 80 - 90% of noma cases die from the disease.
Treatment
Known in antiquity to such physicians as HippocratesHippocrates
Hippocrates of Cos or Hippokrates of Kos was an ancient Greek physician of the Age of Pericles , and is considered one of the most outstanding figures in the history of medicine...
and Galen
Galen
Aelius Galenus or Claudius Galenus , better known as Galen of Pergamon , was a prominent Roman physician, surgeon and philosopher...
, noma was once reported around the world, including Europe
Europe
Europe is, by convention, one of the world's seven continents. Comprising the westernmost peninsula of Eurasia, Europe is generally 'divided' from Asia to its east by the watershed divides of the Ural and Caucasus Mountains, the Ural River, the Caspian and Black Seas, and the waterways connecting...
and the United States
United States
The United States of America is a federal constitutional republic comprising fifty states and a federal district...
. With the improvement in hygiene and nutrition, noma has disappeared from industrialized countries since the 20th Century, except during 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...
when it was endemic
Endemic (epidemiology)
In epidemiology, an infection is said to be endemic in a population when that infection is maintained in the population without the need for external inputs. For example, chickenpox is endemic in the UK, but malaria is not...
to Auschwitz and Belsen concentration camps. The disease and treatments were studied by Berthold Epstein
Berthold Epstein
Berthold Epstein was a Czechoslovakian pediatrician, professor, and scientist who worked as a doctor in the Auschwitz concentration camp during World War II.-Early life:...
, a Czech inmate physician who had recommended the study to Josef Mengele
Josef Mengele
Josef Rudolf Mengele , also known as the Angel of Death was a German SS officer and a physician in the Nazi concentration camp Auschwitz-Birkenau. He earned doctorates in anthropology from Munich University and in medicine from Frankfurt University...
, the Nazi doctor.
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 reconstructive plastic surgery to repair. Reconstruction is usually very challenging and it should be delayed until full recovery (usually about one year following initial intervention).
Children and other noma survivors in 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...
are helped by a few international charitable organizations. There is one dedicated noma hospital in Nigeria
Nigeria
Nigeria , officially the Federal Republic of Nigeria, is a federal constitutional republic comprising 36 states and its Federal Capital Territory, Abuja. The country is located in West Africa and shares land borders with the Republic of Benin in the west, Chad and Cameroon in the east, and Niger in...
, the Noma Children Hospital Sokoto, staffed by resident and visiting medical teams. In other countries, like Ethiopia
Ethiopia
Ethiopia , officially known as the Federal Democratic Republic of Ethiopia, is a country located in the Horn of Africa. It is the second-most populous nation in Africa, with over 82 million inhabitants, and the tenth-largest by area, occupying 1,100,000 km2...
, international charities work in collaboration with the local health care system
Health care system
A health care system is the organization of people, institutions, and resources to deliver health care services to meet the health needs of target populations....
to provide complex reconstructive surgery
Reconstructive surgery
Reconstructive surgery is, in its broadest sense, the use of surgery to restore the form and function of the body, although Maxillo-Facial Surgeons, Plastic Surgeons and Otolaryngologists do reconstructive surgery on faces after trauma and to reconstruct the head and neck after cancer.Other...
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 BBC2 documentary presented by Ben Fogle
Ben Fogle
Ben Fogle is an English television presenter, adventurer and writer.-Early life:Fogle is the son of actress Julia Foster and broadcasting veterinary surgeon Bruce Fogle...
titled 'Make me a new face - Hope for 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...
's hidden children'.
External links
- The fight against noma
- Fondation Sentinelles
- NOMA-HILFE-SCHWEIZ
- Graphic Noma Images
- All Refer Health article on Noma
- Project Harar
- The European Noma Network
- Les Enfants du Noma
- Facing Africa
- The Geneva Study Group on Noma
- The Dutch Noma Foundation
- Bertrand Piccard Winds of Hope Foundation
- Gegen Noma e.V. - Against Noma organisation
- Noma awareness ad on YouTube "Just €10 can save a life"