Noma is a facial disfiguring childhood disease: Insights from cases of Noma in Ethiopia.

OBJECTIVES: Noma is a neglected infectious disease that causes severe destruction of facial tissues and poses a significant public health threat. The disease reportedly affects individuals with a debilitating health condition. This study aimed to identify the most vulnerable age group affected by Noma disease and assess the severity of tissue damage among Noma cases in Ethiopia. METHODS: A cross-sectional study was conducted to determine the age group most susceptible to noma and assess the disease-associated severity of tissue damage. The research objectives were met by scrutinizing electronic medical records of noma cases treated in Addis Ababa, Ethiopia, from 2007 to 2019, obtained from the Facing Africa database. Data collection was carried out using a standardized survey form, while the severity of facial tissue damage was assessed using the NOIPTUS score. RESULTS: Among 164 Noma cases with documented data on disease onset, 92.7 % occurred during childhood, with 92.1 % of those cases being children aged ten years and younger. Approximately 11.6 % of all noma cases had mild tissue damage, classified as NOITULP grade 1, while 26.2 % had an intermediate level of tissue damage, classified as NOITULP grade 2. Severe tissue damage (NOITULP grade 3) was present in 37.2 % of cases, while 25 % had very severe tissue damage (NOITULP grade 4). CONCLUSIONS: The findings underscore the vulnerability of children, particularly those aged ten years and younger, to Noma disease, supporting the argument that Noma is a facial-disfiguring childhood disease. Targeted interventions and preventive measures during childhood can be vital in controlling Noma incidence.

Proportion of paediatric admissions with any stage of noma at the Anka General Hospital, northwest Nigeria.

INTRODUCTION: Noma is a rapidly spreading infection of the oral cavity which mainly affects young children. Without early treatment, it can have a high mortality rate. Simple gingivitis is a warning sign for noma, and acute necrotizing gingivitis is the first stage of noma. The epidemiology of noma is not well understood. We aimed to understand the prevalence of all stages of noma in hospitalised children. METHODS: We conducted a prospective observational study from 1st June to 24th October 2021, enrolling patients aged 0 to 12 years who were admitted to the Anka General Hospital, Zamfara, northwest Nigeria. Consenting parents/ guardians of participants were interviewed at admission. Participants had anthropometric and oral examinations at admission and discharge. FINDINGS: Of the 2346 patients, 58 (2.5%) were diagnosed with simple gingivitis and six (n = 0.3%) with acute necrotizing gingivitis upon admission. Of those admitted to the Inpatient Therapeutic Feeding Centre (ITFC), 3.4% (n = 37, CI 2.5-4.7%) were diagnosed with simple gingivitis upon admission compared to 1.7% of those not admitted to the ITFC (n = 21, CI 1.1-2.6%) (p = 0.008). Risk factors identified for having simple gingivitis included being aged over two years (2 to 6 yrs old, odds ratio (OR) 3.4, CI 1.77-6.5; 7 to 12 yrs OR 5.0, CI 1.7-14.6; p = <0.001), being admitted to the ITFC (OR 2.1; CI 1.22-3.62) and having oral health issues in the three months prior to the assessment (OR 18.75; CI 10.65, 33.01). All (n = 4/4) those aged six months to five years acute necrotizing gingivitis had chronic malnutrition. CONCLUSION: Our study showed a small proportion of children admitted to the Anka General Hospital had simple or acute necrotizing gingivitis. Hospital admission with malnutrition was a risk factor for both simple and acute necrotizing gingivitis. The lack of access to and uptake of oral health care indicates a strong need for oral examinations to be included in routine health services. This provision could improve the oral status of the population and decrease the chance of patients developing noma.

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.

Is noma a neglected/overlooked tropical disease?

Noma is a debilitating orofacial necrotizing bacterial disease that disproportionately affects impoverished malnourished persons, particularly young children, the vast majority of whom live in tropical and subtropical areas in sub-Saharan Africa. It has a very high mortality rate; causes significant physical and psychological morbidity, stigmatization and social discrimination; could be prevented, controlled and indeed eliminated by common public health interventions; and is overlooked with regard to public health awareness, in-depth scientific research activities and allocation of funding for prevention, treatment and research. According to the WHO, noma comprises five sequential 'stages': (1) necrotizing gingivitis, (2) edema, (3) gangrene, (4) scarring and (5) sequelae. This WHO staging of noma is contentious, leading to diagnostic confusion with misestimation of the number of noma cases reported in epidemiological studies. We therefore suggest a simpler, more practical and scientifically valid two-stage classification comprising only (1) acute noma and (2) arrested noma. Noma meets all the WHO criteria for classification as a neglected tropical disease (NTD). Most survivors of noma live with gross physical disfigurement and disability, and with impaired psychosocial functioning, so they are very often stigmatized and unjustifiably discriminated against. Owing to the paucity of evidence-based epidemiological data on noma, the relatively low number of people affected worldwide, and its apparently limited geographic distribution, noma does not yet feature on the WHO's list of NTDs, or on any global health agenda, and thus has not become a health priority for global action. We strongly support the inclusion of noma within the WHO list of NTDs. Without doubt this will increase the awareness of noma among healthcare providers and promote the systematic international accumulation and recording of data about noma.

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.

Pattern of noma (cancrum oris) and its risk factors in Northwestern Nigeria: A hospital-based retrospective study.

Background: Noma (cancrum oris) remains the scourge of children and the "face of poverty" in Sub-Saharan Africa. Recent data on the burden of noma and its risk factors are needed for evaluating and redesigning interventions for its prevention and control. Objectives: This study aimed to determine the pattern of noma and its risk factors in Northwestern Nigeria. Materials and Methods: It was a retrospective study that looked into cases of noma (cancrum oris) admitted into the Noma Children Hospital, Sokoto, Nigeria, between January 1999 and December 2011. Information on patients' bio-data, the site and severity of lesions, and presence of trismus and its severity were extracted from the patients' case files and analyzed using descriptive statistics. Results: One hundred and fifty-nine (8.3%) of the 1923 patients admitted to the hospital from January 1999 to December 2011 were diagnosed with fresh noma. The mean age of the patients was 3.0 ± 1.4 years, and majority of them, 139 (87.4%) were aged 1-5 years. The soft-tissue lesions essentially involved multiple sites but most commonly the outer and inner cheeks (84.3%). The most common risk factors identified were measles (47.2%) and protein-energy malnutrition (42.1%). There were rises and falls in the prevalence of noma in the period studied. Conclusion: This study showed a high burden of noma in Northwestern Nigeria, mostly among children aged 1-5 years, and with soft-tissue lesions involving multiple sites. Measles and malnutrition were the major risk factors identified, and the disease trend showed a wave-like pattern. There is an urgent need to eliminate the disease in Nigeria through prevention and control of infectious diseases and malnutrition.

RésuméContexte: Le noma (cancrum oris) demeure le fléau des enfants et le "visage de la pauvreté" en Afrique subsaharienne. Des données récentes sur le fardeau du noma et de ses facteurs de risque sont nécessaires pour évaluer et redéfinir les interventions en vue de sa prévention et de son contrôle. Objectifs: Cette étude visait à déterminer la structure du noma et de ses facteurs de risque dans le nord-ouest du Nigeria. Matériels et Méthodes: Il s'agissait d'une étude rétrospective qui a examiné les cas de noma (cancrum oris) admis à l'Hôpital Noma Children, Sokoto, au Nigeria, entre janvier 1999 et décembre 2011. Informations sur les bio-données des patients, le site et la sévérité des les lésions, et la présence de trismus et sa gravité ont été extraites des dossiers des patients et analysées à l'aide de statistiques descriptives. Résultats: Cent cinquante-neuf (8,3%) des 1923 patients admis à l'hôpital entre janvier 1999 et décembre 2011 ont reçu un diagnostic de noma frais. L'âge moyen des patients était de 3.0 ± 1.4 ans et la majorité d'entre eux 139 (87.4%) étaient âgés de 1 à 5 ans. La lésion des tissus mous concernait essentiellement plusieurs sites, mais le plus souvent les joues externe et interne (84.3%). Les facteurs de risque les plus fréquemment identifiés étaient la rougeole (47.2%) et la malnutrition protéino-énergétique (42.1%). Il y avait des augmentations et des chutes de la prévalence du noma dans la période étudiée. Conclusion: Cette étude a montré une forte charge de noma dans le nord-ouest du Nigeria, principalement chez les enfants âgés de 1 à 5 ans, et avec des lésions des tissus mous impliquant plusieurs sites. La rougeole et la malnutrition ont été les principaux facteurs de risque identifiés, et la tendance de la maladie a montré un profil ondulatoire. Il est urgent d'éliminer la maladie au Nigéria grâce à la prévention et au contrôle des maladies infectieuses et de la malnutrition.

Risk factors for diagnosed noma in northwest Nigeria: A case-control study, 2017.

BACKGROUND: Noma (cancrum oris), a neglected tropical disease, rapidly disintegrates the hard and soft tissue of the face and leads to severe disfiguration and high mortality. The disease is poorly understood. We aimed to estimate risk factors for diagnosed noma to better guide existing prevention and treatment strategies using a case-control study design. METHODS: Cases were patients admitted between May 2015 and June 2016, who were under 15 years of age at reported onset of the disease. Controls were individuals matched to cases by village, age and sex. Caretakers answered the questionnaires. Risk factors for diagnosed noma were estimated by calculating unadjusted and adjusted odds ratios (ORs) and respective 95% confidence intervals (CI) using conditional logistic regression. FINDINGS: We included 74 cases and 222 controls (both median age 5 (IQR 3, 15)). Five cases (6.5%) and 36 (16.2%) controls had a vaccination card (p = 0.03). Vaccination coverage for polio and measles was below 7% in both groups. The two main reported water sources were a bore hole in the village (cases n = 27, 35.1%; controls n = 63, 28.4%; p = 0.08), and a well in the compound (cases n = 24, 31.2%; controls n = 102, 45.9%; p = 0.08). The adjusted analysis identified potential risk and protective factors for diagnosed noma which need further exploration. These include the potential risk factor of the child being fed pap every day (OR 9.8; CI 1.5, 62.7); and potential protective factors including the mother being the primary caretaker (OR 0.08; CI 0.01, 0.5); the caretaker being married (OR 0.006; CI 0.0006, 0.5) and colostrum being given to the baby (OR 0.4; CI 0.09, 2.09). INTERPRETATION: This study suggests that social conditions and infant feeding practices are potentially associated with being a diagnosed noma case in northwest Nigeria; these findings warrant further investigation into these factors.

Noma in a child with acute leukaemia: when the 'face of poverty' finds an ally.

A 2-year-6-month old, appropriately immunised, well-thriving boy, symptomatic for the past 6 months, presented with recurrent fever, progressive pallor, lymphadenopathy and a raw area on the right cheek, with discharging sinus. The necrotising infection of the face developed after one and half months of febrile illness. This febrile illness with bicytopaenia was diagnosed as enteric fever and treated with antibiotics. Skin grafting was performed for the full-thickness defect of the face. The patient continued to have a non-healing oral ulcer with progressive pallor and was finally diagnosed as having acute lymphoblastic leukaemia. Immunodeficiency was ruled out by appropriate investigations. Noma is an indirect measure of extreme poverty, but malignancy is known to predispose to this debilitating condition. The worldwide incidence of Noma is reported to be 30,000-140,000, with a preponderance in sub-Saharan Africa. This case emphasises the need for a thorough search for the underlying illness predisposing to a rare opportunistic infection such as Noma in a well-thriving child.

[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: neglected, forgotten and a human rights issue.

Noma, an orofacial gangrene and opportunistic infection, affects primarily malnourished children living in extreme poverty. Neglected, forgotten, unknown by most health workers, noma results in death, disfigurement and disability of some of the world's most vulnerable children. Noma is a biological indicator of multiple human rights violations, including the right to food. International support and national attention in countries with noma are lacking. The end of neglect of noma can lead to the elimination of this horrific childhood disease.

Noma (cancrum oris) in the South African context.

Noma (cancrum oris) is a destructive necrotising disease affecting orofacial tissues predominantly of malnourished young children. It is characterised by a rapid acute onset which usually starts in the mouth, spreads intra-orally destroying soft tissue and bone and progresses to perforate the facial skin, causing disfigurement. Polybacterial anaerobic infection is critical too, but is not alone sufficient for the initiation of noma. Cofactors, first and foremost malnutrition, but also systemic viral and bacterial infections are crucial to the development of noma. A patient with necrotising stomatitis or noma must be admitted to hospital for antibiotic treatment, fluid and electrolytes as well as nutritional supplementation and general supportive treatment. The epidemiology of noma in the South African population is unknown, and the clinicopathological features are poorly characterised. Although worldwide there is no evidence that HIV infection is a strong risk factor for noma, HIV infection may play a substantial role in the pathogenesis of noma in South Africa.

[Causes and pathogenesis of noma in Zinder (Niger): a socio-anthropological study]. / Étiopathogénie du noma à Zinder (Niger) : étude socioanthropologique.

OBJECTIVES: Noma is a gangrenous affliction that destroys the soft and hard tissue of the face. It affects exclusively young children from developing countries. Although the risk factors (malnutrition and debilitating diseases) are known, the cause remains a topic of debate. Etiological studies have tried to identify bacterial or viral agents responsible for the illness. We examine for the first time the possibility of a link between this illness and the social practices surrounding childbirth. METHODS: This study took place in Niger, where we conducted 18 ethnographic interviews with mothers from four villages. Noma was known in two villages, but unknown in the other two. RESULTS: Our results show that inadequate practices related to breastfeeding, weaning, and nutritional supplementation practices are by themselves insufficient to initiate the disease, insofar as most of these practices, paradoxically, were found in the villages not affected by the disease. CONCLUSION: The results indicate that these practices are not sufficient to explain noma. They suggest, however, that poor maternal health and nutritional status before and during pregnancy may have an irreversible negative impact on the child's overall health.

Noma (cancrum oris): a report of a case in a young AIDS patient with a review of the pathogenesis.

Noma (cancrum oris) is a mutilating necrotising disease of the orofacial tissues. It affects predominantly debilitated malnourished children, in whom the necrotic process may cause severe damage to mid-facial structures. Its aetiopathogenesis is uncertain, but its course is fulminating, and without timely intervention the disease may be fatal. Antibiotic treatment during any stage of necrotising stomatitis and of its sequel noma can stop progression of the disease; therefore detection and treatment of early intraoral necrotising lesions whether necrotising gingivitis, necrotising periodontitis or necrotising stomatitis are critical in preventing noma. We present an extreme case of noma in a malnourished HIV-seropositive child. There was an acute necrotic process affecting both the maxilla and the mandible with denudation of bone, spontaneous exfoliation of teeth, necrotising fasciitis and myonecrosis which destroyed the lips and cheeks and extended to the infra-orbital margins. There was severe disfigurement and severe impairment of function. Noma is primarily an anaerobic bacterial infection with secondary ischaemia leading to osteonecrosis and mid-facial destruction.

[Anaesthesia in noma-patients - facts and management of a forgotten disease]. / Anästhesie bei Noma-Patienten Hintergründe und Management einer Vergessenen Erkrankung

Noma (cancrum oris) is an unspecific bacterial infection in the oro-facial area. In the industrial countries noma is generally unknown, but in the 3rd world - especially in West Africa - it is widespread and one of the main causes of mortalitiy and reason for disability in children. Pathogenesis, clinical course, management and treatment of noma are described with the consequences for anaesthesia.