Understanding noma: WHO's recognition and the path forward in global health.

The World Health Organization's (WHO) designation of noma as a neglected tropical disease (NTD) on 15 December 2023 marks a crucial advancement in global health efforts. This move sheds light on a condition predominantly affecting undernourished children in isolated regions of sub-Saharan Africa. Recognized as the 21st NTD, noma, or cancrum oris, is a serious condition leading to orofacial gangrene. The disease largely impacts young children and those with compromised immune systems, including individuals with human immunodeficiency virus or leukaemia. Determining the exact prevalence of noma is complex, hindered by rapid disease progression, societal stigma and a lack of reporting, especially in impoverished areas. The WHO's acknowledgment is a significant step, emphasizing the need for more in-depth research and resources to address this overlooked disease. It highlights the critical role of multifaceted prevention strategies, including economic empowerment, improved nutrition and enhanced vaccination efforts. This recognition is pivotal in guiding international health initiatives towards better outcomes for some of the most at-risk populations globally.

Retrospective Characterization of Noma Cases Found Incidentally across Nigeria during Outreach Programs for Cleft Lip from 2011-2020.

Reports of cases of noma in Nigeria remain scarce despite its known and devastating effects on victims. This report presents a retrospective cross-sectional study based on data regarding on patients with noma encountered incidentally during Oral Health Advocacy Initiative outreach on orofacial diseases across 34 states and the Federal Capital Territory in Nigeria over 10 years (2011-2020), which was aimed at contributing to an understanding of the epidemiology of noma in Nigeria. The data were collated and analyzed, and are presented in frequency distribution tables and charts. A total of 7,195 patients with noma were encountered. The northeastern region had the greatest number of patients (n = 1,785, 24.8%) whereas the southwestern region had the least (n = 196, 2.7%). When aggregated by state, Ondo State had the least number of patients (n = 31, 0.4%) whereas Kano State had the greatest (n = 623, 8.7%). Patient age ranged from 3 to 70 years, with a slight male preponderance (56.9%). This report highlights the fact that noma is prevalent in Nigeria but remains neglected, with extensive but preventable physical, emotional, and social debilitation and devastation of the victims across all age groups. There is a need for a more robust survey to determine the true burden of the disease. There is also an urgent need for collaboration between governments and nongovernmental organizations to institute appropriate interventions by way of public education and enlightenment, as well as case detection and early treatment to mitigate the devastating consequences of delayed or poorly managed cases.

A review of noma cases in a tertiary hospital located in a conflict endemic region in Nigeria.

Noma, a neglected tropical disease (NTD), is a preventable oro-facial gangrenous infection causing destruction of oro-facial tissues if untreated. This descriptive study was conducted in North-Eastern Nigeria which has witnessed armed insurgency increasing risk of noma. Data was obtained from patients' records at a tertiary hospital after a dental educational outreach using radio and visits to the hospital's ante/postnatal clinics. Data analysis was conducted at 95% confidence interval with p≤ 0.05 considered significant. Records of 49 patients were retrieved but 31 (63.3%) were utilised due to poor documentation. Age ranged from 2-63 years. Children (0-16 years) and Adults (17-59 years) were 67.7% and 25.8% respectively. Males were 13 (41.9%) and females, 18 (58.1%). Poor oral hygiene and malnutrition were common findings. Stage (WHO Staging System) 2 (51.6%) and 4 (22.6%) were the most common presentation. Successfully managed and referred cases were 51.6% and 48.4% respectively. Successful management was associated with early stages of noma (p = <0.001). Children were most vulnerable. Outreaches using radio and education to pregnant women and nursing mothers can boost early presentation and better outcomes. Attention to case documentation and inclusion of noma into the WHO list of NTDs are recommended.

Noma, a neglected disease: prevention is better than cure.

PURPOSE OF REVIEW: There is a need for concerted effort to increase Global awareness about noma (cancrum oris). This paper aims to summarize the recent literature on noma and provide suggestions that could be implemented to raise awareness about this neglected disease. RECENT FINDINGS: Noma has been recognized, diagnosed and reported for centuries. Despite significant progress in scientific methods over time, the published literature on noma has predominantly been of low level clinical and scientific evidence. Recent studies have reported on noma's global distribution and its predisposing risk factors, its treatment, its knowledge and beliefs and has included a number of literature reviews. Noma cases are being reported from an increasingly diverse set of geographical locations. SUMMARY: Noma has largely been neglected in the research sphere. Noma is a preventable disease and its progression can be halted if patients are recognized and treated in the early stages of disease. Treatment for late stage noma survivors remains complex and time consuming, requiring substantial human and financial resources most commonly not achieving functional and cosmetic anatomy. The ultimate aim is therefore prevention, initiatives should be integrated into existing health programs.

Noma: A Neglected Area for Research.

Noma, a debilitating and destructive orofacial gangrene, remains endemic in the poor countries of sub-Saharan Africa and other noma hotbeds across the globe, mainly in countries characterized as underdeveloped economies with significant impoverished populations. Noma mostly affects children and infants. This is in spite of the universally held notion that noma is a preventable disease. Indeed, the current noma status quo has been cast as a human rights shortfall, since this devasting disease overwhelmingly affects children from poor countries. At the recently held Noma Research Day, a renewed call for the World Health Organization (WHO) to recognize and include noma as one of the neglected tropical diseases was accompanied by a recognition that research into all aspects of noma has waned or remained completely lacking-particularly that which addresses the basic science questions of the etiology, pathophysiology/pathobiology, and underlying mechanisms of the disease. Yet, a lack of incremental knowledge on the various aspects of noma continues to hamper our composite understanding of its biology. Without a fundamental understanding of the biology of noma, current preventive measures and treatment modalities will continue to fall short of the goals of prevention and eradication. This opinion piece draws renewed attention to the urgency of listing noma as a neglected tropical disease by the WHO. It also calls for major international research funding agencies, including the WHO and the National Institutes of Health, to renew their resolve to robustly fund structured, collaborative, and coordinated proposals that address questions on the epidemiology, etiology, pathophysiology/pathobiology, and molecular mechanisms of the disease. This is with a view to achieving more effective public health approaches toward prevention and to designing potential therapeutic regimens for early lesions. These steps are key to the ultimate eradication of noma.

From trench mouth to noma: Experiences from Nazi extermination camps.

Trench mouth, as it occurred especially in World War I in soldiers, is a necrotizing ulcerative gingivitis (NUG) in people who have poor oral hygiene, malnutrition, vitamin deficiency, a smoking history, and psychic stress. When not treated properly, this condition can lead to noma. NUG and noma are mainly seen today in severely malnourished, poorly cared for, and immunocompromised children in extremely poor living conditions, mainly in sub-Saharan Africa. In 20th century history, the occurrence of noma in Turkey was described by Albert Eckstein (1891-1959), but noma is particularly linked to the atrocious living conditions in the Nazi exterminations camps, such as Auschwitz, where large numbers of patients with noma were treated in Berthold Epstein's (1890-1962) Noma Department in the Zigeunerlager (Gypsy Camp) under the supervision of SS physician Josef Mengele (1911-1979). Although these patients were treated successfully, all of them, mostly children, were ultimately killed. The protocols of the noma research are lost, and descriptions from Auschwitz are scarce. Fortuitously, there are some testimonies, especially from postwar trials, that give insight on this ambiguous and repressed topic.

Noma (cancrum oris): A scoping literature review of a neglected disease (1843 to 2021).

BACKGROUND: Noma (cancrum oris) is an ancient but neglected and poorly understood preventable disease, afflicting the most disenfranchised populations in the world. It is a devastating and often fatal condition that requires urgent and intensive clinical and surgical care, often difficult to access as most cases of noma occur in resource-limited settings. We conducted a scoping review of the literature published on noma to understand the size and scope of available research on the disease and identify research gaps that need to be addressed to evolve our understanding of how to address this disease. METHODS: We searched 11 databases and collected primary peer reviewed articles on noma in all languages, the final search was conducted on 24th August 2021. The oldest manuscript identified was from 28th March 1843 and the most recently published manuscript was from 3rd June 2021. Search terms included cancrum oris and noma. Data was extracted using a standardised data extraction tool and key areas of interest were identified. The Preferred Reporting Items for Systemic review and Meta-Analyses requirements were followed. RESULTS: The review included 147 articles, the majority of the studies (n = 94, 64%) were case reports. Most manuscripts (n = 81, 55%) were published in the 2000s, 49 (33%) were from the 1900s and 17 (12%) from the 1800s. The main areas of interest identified were the history and epidemiology of the disease, noma's clinical progression and aetiology, treatment regimens, mortality rates and the risk factors for the development of noma. CONCLUSIONS: Noma has been reported in the literature for hundreds of years; however important gaps in our understanding of the disease remain. Future research should focus on determining the burden and distribution of disease; the true mortality rate, pathogenic cause(s) and the factors that influence prognosis and outcomes after treatment.

Model of care, Noma Children's Hospital, northwest Nigeria.

The Nigerian Ministry of Health has been offering care for noma patients for many years at the Noma Children's Hospital (NCH) in Sokoto, northwest Nigeria, and Médecins Sans Frontières has supported these initiatives since 2014. The comprehensive model of care consists of four main components: acute care, care for noma sequelae, integrated hospital-based services and community-based services. The model of care is based on the limited evidence available for prevention and treatment of noma and follows WHO's protocols for acute patients and best practice guidelines for the surgical treatment of noma survivors. The model is updated continually as new evidence becomes available, including evidence generated through the operational research studies performed at NCH. By describing the model of care, we wish to share the lessons learned with other actors working in the noma and neglected tropical disease sphere in the hope of guiding programme development.

'I treat it but I don't know what this disease is': a qualitative study on noma (cancrum oris) and traditional healing in northwest Nigeria.

BACKGROUND: Noma, a neglected disease mostly affecting children, with a 90% mortality rate if untreated, is an orofacial gangrene that disintegrates the tissues of the face in <1 wk. Noma can become inactive with early stage antibiotic treatment. Traditional healers, known as mai maganin gargajiya in Hausa, play an important role in the health system and provide care to noma patients. METHODS: We conducted 12 in-depth interviews with caretakers who were looking after noma patients admitted at the Noma Children's Hospital and 15 traditional healers in their home villages in Sokoto state, northwest Nigeria. We explored perceptions of noma, relationship dynamics, healthcare practices and intervention opportunities. Interviews were audiorecorded, transcribed and translated. Manual coding and thematic analysis were utilised. RESULTS: Traditional healers offered specialised forms of care for specific conditions and referral guidance. They viewed the stages of noma as different conditions with individualised remedies and were willing to refer noma patients. Caretakers trusted traditional healers. CONCLUSIONS: Traditional healers could play a crucial role in the early detection of noma and the health-seeking decision-making process of patients. Intervention programmes should include traditional healers through training and referral partnerships. This collaboration could save lives and reduce the severity of noma complications.

Noma-knowledge and practice competence among primary healthcare workers: a cross-sectional study in Burkina Faso.

BACKGROUND: Noma is a quickly progressing, neglected opportunistic infection. It starts in the mouth as an oral lesion but can relatively quickly develop into extensive facial destruction and lead to death if not treated in time. This study aims to shed light on primary healthcare workers' practice competences in working with and knowledge of noma. METHODS: A structured questionnaire using questions and case scenarios was filled out by 76 healthcare workers in Burkina Faso. Half of the nurses included in this study participated in a 2-day noma training. Data were analysed descriptively and Fisher's exact test was used to study differences between occupational groups using Stata. RESULTS: Most healthcare workers reported having examined the mouth of children with diseases predisposing to noma. The total practice competence was poor, with almost 70% having suboptimal or very low competences. However, competences varied between different stages of noma disease. Knowledge scores varied between occupational groups. The majority of nurses and odontostomatology specialist nurses had optimal or good knowledge of noma. Significant differences in knowledge and practice competence were found between nurses who attended a 2-day training course on noma and those who did not. CONCLUSIONS: Health care workers in this study had quite poor practice competences in managing noma. The knowledge scores of these health workers were moderate. It is important for healthcare workers to be able to identify noma patients at an early stage, as at this point the disease can still be completely reversed.

Management of noma: practice competence and knowledge among healthcare workers in a rural district of Zambia.

BACKGROUND: Noma is an infectious but opportunistic disease that often results in severe facial disfigurements and mortality if untreated. As noma progresses quickly, early detection and treatment are important to prevent its development. OBJECTIVES: The objective of this study was to investigate primary healthcare workers' knowledge and management of noma in a rural part of Zambia. METHODS: A cross-sectional self-completed survey was conducted among 35 healthcare workers from two district hospitals and 15 rural health centres in Serenje District, Zambia. Participants' practice competences and knowledge were grouped into 'optimal', 'medium', 'suboptimal' and 'very low'. RESULTS: Most of the healthcare workers stated that they perform mouth examination of a child below five years of age who is suffering from measles, malnutrition or HIV. A majority diagnosed gingivitis correctly and 40% had a medium level of practice competence of the same noma stage. All participants had a suboptimal or very low level on overall practice competence regarding management of noma and two-thirds had a very low level of reported knowledge. CONCLUSION: General knowledge on noma and competences of diagnosing and treating noma patients was low among healthcare workers. Lack of knowledge could present a barrier for correctly managing noma at an early stage. Improving knowledge among healthcare workers is one way to prevent the development of the disease. In order to prevent noma from the start, actions need to be focussed on improving (oral) hygiene and health education as well. Telemedicine could also be considered as it can help healthcare workers in handling noma patients through enabling communication and exchange of information with specialist.

Noma as a complication of false teeth (Ebiino) extraction: a case report.

BACKGROUND: Ebiino, also known as false tooth extraction, is a traditional practice done mainly in the remote areas of African countries, including Uganda. It involves the extraction of tooth buds in babies with common childhood illnesses such as fever, cough, and diarrhea. It is thought that the tooth buds are responsible for the ailments seen in these infants. The practice is performed by traditional healers using unsterile instruments. The complications associated with this dangerous practice have been mentioned in the literature and include anemia and septicemia, among others. This case report describes a baby with noma, an orofacial gangrenous infection. CASE PRESENTATION: A 16-month-old girl from western Uganda belonging to the Banyankole ethnic group was admitted to Mbarara University Teaching Hospital with a 5-day history of a dark lesion on the left cheek. The lesion had started from the left upper gum at the site where a tooth bud had been extracted 1 week prior to admission. The child had experienced occasional cough and fever and also had erupting tooth buds. These tooth buds had been seen as the cause of the cough and fever by the traditional herbalist; hence, they were extracted. An unsterile instrument had been used for the procedure. At the hospital, a local examination showed necrotic tissue involving the left cheek and extending into the left upper gingival area of the girl's mouth. A clinical diagnosis of orofacial gangrene (noma) was then made. CONCLUSIONS: Ebiino, or false tooth extraction, is still practiced in some remote areas of Uganda. Noma has been mentioned as a possible complication of this traditional practice; however, case reports in the literature are scant. Public awareness of the dangers of this practice is therefore still required to prevent this dangerous complication.

Noma: Overview of a Neglected Disease and Human Rights Violation.

Noma is an orofacial gangrene affecting malnourished children and mainly observed in tropical countries, particularly sub-Saharan Africa. Epidemiological data on noma are scarce, but a current estimate of the global incidence is 30,000-40,000 cases per year, with a mortality rate of approximately 85% and a burden of disease calculated to be a loss of 1-10 million disability-adjusted life years. The etiology of noma is multifactorial with malnutrition as an ever present factor, often in combination with concomitant diseases, such as measles, malaria, and human immunodeficiency virus (HIV), and poor oral hygiene. The pathogenesis is a fast-spreading, noncontagious gangrenous infection occurring in the face, often preceded by acute necrotizing gingivitis, and stomatitis. Rare microbiological studies suggest an opportunistic infection caused by an imbalance in normal intraoral microorganisms. Prevention lies in food security, measles vaccination, prevention of malaria and HIV, including the early detection and treatment of necrotizing gingivitis and stomatitis. Early treatment with antibiotics may prevent gangrene or reduce its extent. Late treatment consists of surgical rehabilitation, which is often complex. However, access to medical care is very limited for noma patients due to the extremely poor conditions in which they live that are frequently located in remote rural areas. The authors support the United Nations Human Rights Council Resolution 19/7 adopted on March 22, 2012 "The right to food," and advocate for the inclusion of noma on the list of neglected tropical diseases to encourage more medical and institutional attention for this often lethal or very mutilating infectious gangrene.

A Review on Noma: A Recent Update.

Noma is a gangrenous infection primarily affecting under developed countries. The aim of this paper was to review all recent articles on noma from January 2003 to August 2014 and briefly update the latest information related to the topic. A literature search was done on PUBMED using the keywords "noma/cancrum oris". Noma is commonly seen in malnourished children. There has been an increased incidence of noma in HIV patients. Apart from these, noma has also been reported in association with cyclic neutropenia, herpetic stomatitis, leukemia, Down's syndrome and Burkett's disease. Treatment of acute noma includes transfusion of blood and intravenous fluids, administration of antibiotics, putting the patient on a high protein diet and debridement of necrotic areas. Surgical phase is usually initiated 6 to 18 months after a period of quiescence. Although, the mortality rate associated with noma has reduced significantly with the advent of modern generation antibiotics, the functional, cosmetic and psychological challenges associated with the destruction of soft or hard tissues still remains a huge challenge. Adequate steps must be implemented by the government or medical professionals to prevent the disease and provide an early intervention.

[Noma/Cancrum oris: a neglected disease]. / La enfermedad de Noma/cancrum oris: una enfermedad olvidada.

Noma is an aggressive orofacial gangrenous pathology that damages hard and soft tissues of the mouth and the face. Throughout the centuries it has been present around the globe, but nowadays it has practically disappeared from developed countries and mainly affects children from the most disadvantaged places, especially in Africa. Noma disease is a multifactorial process; malnutrition, debilitating diseases (bacterial or viral systemic diseases, HIV-associated immunosuppression, etc.) and intraoral infections are some of the factors implied. The characteristic tissue necrosis is produced by a polymicrobial infection. Fusobacterium necrophorum, Prevotella intermedia, Prevotella melaninogenica, Fusobacterium nucleatum, Bacteroides fragilis, Bacillus cereus, Trueperella pyogenes, spyrochetes, etc, are some of the species that have been isolated from the affected areas. Without treatment, noma is lethal in a short period of time, and the patients that survive show severe sequelae that hinder their life and interpersonal relationships. The aim of this paper is to unify the existing information and to promote wider knowledge and awareness among the population.

[Noma]. / Le Noma.

Noma is a necrotizing ulcerative stomatitis known since Antiquity. It occurs mostly in poor countries, the Sahel countries being the most affected. Each year, several hundred thousand cases are reported. Noma affects especially malnourished children who are less than 6 years old and rarely adults with acquired immunodeficiency (HIV, cancer). Ulcerative lesion is occurring rapidly due to the production of endotoxins by bacteria from oral commensal, telluric and animal origin. Necrotic debridement leads to huge defects: loss of soft tissue (skin, nerves, vessels, eye), bone (maxilla, mandible) and teeth. Death occurs rapidly in a few weeks in 80 % of the cases. In case of survival, the consequences are functional, aesthetic, psychological and social. The goal of the treatment in the acute phase is the patient's survival and the fight against limited mouth opening. The management of the phase of sequela is an anaesthetic, surgical and physiotherapy challenge. Its purpose is the social reintegration of the patient.

[Evoluting form of cancrum oris, about 55 cases collected at the Academic Hospital Yalgado Ouedraogo of Ouagadougou]. / Le noma évolutif, à propos de 55 observations vues au centre hospitalier universitaire Yalgado Ouedraogo de Ouagadougou.

The cancrum oris is still an up to date disease in our environment. The death rate and the after effects of this disease make all together the main interest of this survey. In a retrospective survey carried out from January 2003 to December 2012, we colligated 55 cases of progressive cancrum oris followed at the stomatological and maxillofacial surgery at the Academic Hospital Yalgado OUEDRAOGO. On the epidemiological level, we noticed an impact of 5.5 cases per year. The average age of our patients was about 7.64 with a sex ratio of 1.03. Most of the patients were from an underprivileged family (96.4%). On the clinical level, we noticed that most of the patients consulted only after the gangrene had fallen (89.1%) and were seriously affected (67.3%) with a bad oral and dental hygiene (38.1%). The attacks were mainly jugal (25%) and labial (24.1%). The cancrum oris was in most of the cases associated to broncho pneumonitis, malaria and to HIV infection (31.37%). For the medical treatment, we focused on resuscitation, re nutrition, hydro electrolytic rebalancing and antibiotherapy. The surgical treatment was essentially made on the affected areas, controlled skinning and most often followed by sequestrectomy. 81.8% of the patients recovered completely from the infection, 60% had after effect injuries. We recorded a death rate of 14.5%. In order to overcome this disease we need both national and international support.