Challenges and advances in serological and molecular tests to aid leprosy diagnosis.

Leprosy is a neglected chronic infectious disease caused by obligate intracellular bacilli, Mycobacterium leprae and Mycobacterium lepromatosis. Despite multidrug therapy (MDT) success, leprosy accounts for more than 200,000 new cases yearly. Leprosy diagnosis remains based on the dermato-neurologic examination, but histopathology of skin biopsy and bacilloscopy of intradermal scraping are subsidiary diagnostic tests that require expertise and laboratory infrastructure. This minireview summarizes the state of the art of serologic tests to aid leprosy diagnosis, highlighting enzyme-linked immunosorbent assay (ELISA) and point-of-care tests (POCT) biotechnologies. Also, the impact of the postgenomic era on the description of new recombinantly expressed M. leprae-specific protein antigens, such as leprosy Infectious Disease Research Institute (IDRI) diagnostic (LID)-1 is summarized. Highly specific and sensitive molecular techniques to detect M. leprae DNA as the quantitative polymerase chain reaction (qPCR) and the loop-mediated isothermal amplification (LAMP) are briefly reviewed. Serology studies using phenolic glycolipid-I (PGL-I) semi-synthetic antigens, LID-1 fusion antigen, and the single fusion complex natural disaccharide-octyl (NDO)-LID show high sensitivity in multibacillary (MB) patients. However, serology is not applicable to paucibacillary patients, as they have weak humoral response and robust cell-mediated response, requiring tests for cellular biomarkers. Unlike ELISA-based tests, leprosy-specific POCT based on semi-synthetic PGL-I antigens and NDO-LID 1 antigen is easy to perform, cheaper, equipment-free, and can contribute to early diagnosis avoiding permanent incapacities and helping to interrupt M. leprae transmission. Besides its use to help diagnosis of household contacts or at-risk populations in endemic areas, potential applications of leprosy serology include monitoring MDT efficacy, identification of recent infection, especially in young children, as surrogate markers of disease progression to orient adult chemoprophylaxis and as a predictor of type 2 leprosy reactions. Advances in molecular biology techniques have reduced the complexity and execution time of qPCR confirming its utility to help diagnosis while leprosy-specific LAMP holds promise as an adjunct test to detect M. leprae DNA.

A qualitative study of groin hernia management in adolescents.

INTRODUCTION: Groin hernias in adolescents are rare and their management is associated with challenges for surgeons as some adolescents are fully grown, whereas others are not. Current groin hernia guidelines only differentiate between young children and adults; hence, no guidelines exist that may aid surgeons in handling adolescents. The aim of this study was to explore surgeons' considerations on the management of groin hernias in adolescents. METHODS: We conducted a qualitative study using pilot-tested individual semi-structured interviews. The participants were surgical specialists with experience in groin hernia repair in adolescents aged 10-17 years. Data were analysed using content analysis where essential quotes were extracted from transcripts and coded, categorised and interpreted into themes. RESULTS: Sixteen surgeons were included. Their considerations were reflected in four themes: 1) mesh-related concerns, 2) watchful waiting, 3) growth and 4) lack of evidence and guidelines. Surgeons performed sutured repairs on adolescents who are still growing due to concerns about mesh-related complications. A watchful waiting strategy was used by some to postpone surgery until adolescents were fully grown, thereby enabling mesh repair. Methods for evaluating growth varied and were not standardised. Finally, surgeons highlighted the need for evidence and guidelines to support their decision-making. CONCLUSIONS: This study found a lack of consensus and uniformity on the management of groin hernias in adolescents. Increased research efforts producing clinical guidelines are needed. FUNDING: This study was funded by the Michaelsen Foundation, the Aage and Johanne Louis-Hansens Foundation, Direktør Emil C. Hertz and Hustru Inger Hertz' Foundation, and the Torben and Alice Frimodts Foundation. The funders had no role in the design, conduct or reporting of the study. TRIAL REGISTRATION: not relevant.

Contact hypersensitivity to Indian standard patch test series correlates with disease severity among children with atopic dermatitis.

Background There is emerging evidence of a relationship between atopic dermatitis (AD) and allergic contact dermatitis (ACD), though the data available are scarce with conflicting viewpoints. We explored the occurrence of contact hypersensitivity among children with atopic dermatitis by patch testing them with the Indian standard series and tried to correlate the presence of contact hypersensitivity with the clinical severity of AD in these children. Methods In this single-centre, cross-sectional study, children between 6 months and 12 years diagnosed with atopic dermatitis were included and patch tested with the Indian standard series. Outcome parameters were the proportion of patients having positive patch-test reactions, the proportion of positive patch-test reactions for each allergen and factors associated with patch test positivity in atopic dermatitis. Results Of the 136 patients, 80 were boys. The mean age of the study population was 5.6 ± 3.2 years. Twenty-eight (20.6%) patients had patch test positivity at 96 h. Fragrance mix was the commonest allergen, followed by potassium dichromate, cobalt chloride hexahydrate and nickel. SCORing atopic dermatitis (SCORAD) was significantly higher in patients with positive patch tests as compared to patients with negative patch tests (P = 0.009). Conclusion Greater disease severity in atopic dermatitis was found to be associated with patch test positivity. Limitations Inability to establish relevance in about 50% of the patients was a limitation of our study. Follow-up data regarding the impact of allergen avoidance is not available.

Educação e trabalho na saúde sob participação social organizada e disseminada: a acumulação das conferências livres nacionais 2022-2023 / Education and work in health under organized and disseminated social participation: the accumulation of national free conferences 2022-2023

A coletânea "Educação e trabalho na saúde sob participação social organizada e disseminada: a acumulação das conferências livres nacionais 2022 ­ 2023", reunida por Maria Luiza Jaeger, Ricardo Burg Ceccim e Priscila Viégas Barreto de Oliveira, é uma obra decorrente do cotejamento de seis conferências livres nacionais, realizadas entre final de 2022 e início de 2023, como parte da construção da 17ª Conferência Nacional de Saúde, das quais participou a Associação Científica Rede Unida […]. O foco da entidade é a Educação e Trabalho na Saúde, fazendo parte da comunidade de entidades/sociedades científicas com forte presença junto ao Conselho Nacional de Saúde, inclusive integrando várias de suas Comissões, em especial a Comissão Intersetorial de Recursos Humanos e Relações de Trabalho. A entidade também integra o Fórum de Entidades Nacionais de Trabalhadores da Área da Saúde, instância que congrega Conselhos, Federações e Associações presentes no campo da saúde. Considerada essa personalidade institucional, a Rede Unida integrou conferências nacionais livres onde o tema da Educação e Trabalho na Saúde formaram tópicos estratégicos ao debate dos rumos no Sistema Único de Saúde. […] A obra representa um esforço de memória e apoio ao exercício do controle social em saúde nos termos da Constituição Federal relativamente ao setor da saúde e especialmente no tocante à Lei Federal 8.142, de 28 de dezembro de 1990. A anotação "participação social organizada e disseminada" ao título da coletânea diz respeito à congregação de participantes país afora de modo orquestrado pelo Conselho Nacional de Saúde em um dispositivo inovador à democracia: a chancela às estratégias autogeridas, capazes de acumular engajamento, reflexão e contribuição cidadã às políticas públicas de saúde. Essa nova realidade certamente se tornará mais um patrimônio brasileiro aos sistemas de saúde. A coletânea inicia a acaba no escopo da Educação e Trabalho na Saúde, este é seu propósito: recolher da participação social organizada e disseminada as contribuições de um campo particular pouco reconhecido como campo nas áreas de Ensino da Saúde e da Saúde Coletiva. Contudo, um campo germinativo, potente e aglutinador à ciência, à docência, à intervenção e à inovação.

A Pandemia e o Trabalho em Saúde: vozes do cotidiano / The Pandemic and Health Work: everyday voices

Com esta obra buscamos apresentar ao leitor reflexões sobre a COVID -19 e seus impactos no mercado de trabalho, especialmente num contexto de desigualdade social, onde muitos trabalhadores de ocupações essenciais ou não precisaram se expor ao vírus para manter a sua sobrevivência e a de sua família. Ao final das análises, pontuamos a necessidade proteção dos trabalhadores em tempos de Covid, orientações para os serviços e gestores de saúde, com a finalidade de desencadear a discussão sobre a invisibilidade de muitos trabalhadores que ficaram desprotegidos pelas políticas sociais brasileiras durante a pandemia de COVID-19. Com esse conjunto de textos, esperamos ampliar a compreensão sobre os efeitos da pandemia nos trabalhadores e trabalhadoras e, ao mesmo tempo, ativar o pensamento para iniciativas que são visibilizadas pelas autoras e autores. Ou seja, mais do que buscar aprendizagem com a experiência trágica da pandemia, pretendemos também construir novas manhãs para o trabalho na saúde. Objetivo oportuno, já que estamos diante da preparação da 17ª Conferência Nacional de Saúde, que terá o tema "Garantir Direitos e Defender o SUS, a Vida e a Democracia ­ Amanhã Vai Ser Outro Dia". Acreditamos que a pandemia será memória, aprendizagem e mobilização para retomarmos a agenda da proteção do trabalho, ampliarmos a compreensão de que o trabalho é direito humano e não dependência do capital, expandirmos iniciativas de políticas de proteção da vida e avançarmos no processo civilizatório que é necessário, como bem nos lembrou Sérgio Arouca na abertura da 8ª Conferência Nacional de Saúde, para que o SUS seja possível. Por fim, nossa homenagem às trabalhadoras e trabalhadores que, asfixiados pela pandemia e órfãos da ação governamental responsável, tiveram suas vidas tomadas nesses anos. Especialmente àqueles que foram vitimados desde sua inserção no trabalho em saúde. Aliás, não somente aos que partiram, também a todas as pessoas que se ocupam do trabalho em saúde nos sistemas, redes e serviços de saúde, mas também nos pontos de atenção nos territórios, com trabalhos informais e, muitas vezes, invisíveis. A vocês, nossas palmas, nosso reconhecimento e nossa luta para novas manhãs!

Vozes, olhares e sentidos da Participação Social no SUS / Voices, looks and senses of Social Participation in the SUS

Olhando para a trajetória histórica compreende-se melhor a relação entre "Democracia e Saúde" estabelecida para 16ª Conferência Nacional de Saúde realizada em 2019, pois a luta pelo direito à saúde e a implementação do SUS está vinculada a busca pela redemocratização e a ampliação dos direitos sociais no Brasil. As instâncias do controle social se consolidaram no decorrer das três décadas de sua existência, os movimentos sociais, bem como, as mais diversas organizações da sociedade civil, ocuparam estes espaços e buscaram transformá-los numa arena democrática de defesa da sua concepção de saúde, de política pública, de Estado, de desenvolvimento e de direitos humanos.

1ª Conferência Nacional de Vigilância em Saúde: processo ascendente e participativo / 1st National Health Surveillance Conference: bottom-up and participatory process

Este livro foi organizado e produzido para documentar e expandir o acesso às deliberações da 1ª Conferência Nacional de Vigilância em Saúde, com a expectativa de que se tornem vivas no cotidiano de todas as pessoas em todo o território brasileiro. Afinal, sistematizar o trabalho que se realiza pelo controle social no SUS é fundamental, inclusive para ocupar a condição de relevância pública que a Constituição Brasileira de 1988 atribuiu às ações e serviços de saúde. Tornar esse trabalho visível e reconhecê-lo nessa condição tem a dupla finalidade de fortalecer o SUS como política de saúde do estado brasileiro, conquistada pela população em esforço e luta, e de consolidar a relação entre a saúde e a democracia, aqui expressa pela designação das necessidades nos diferentes territórios e de forma ascendente, materializando a diretriz que embasa a institucionalidade do aparelho estatal na definição constitucional de que a saúde é bem de todos e que os governos devem agir para preservá-la e fortalecê-la.

Prevalência de pacientes notificados com hanseníase no município de São Luís, Maranhão, durante 2010-2020 / Prevalencia de pacientes notificados con lepra en el municipio de São Luís, Maranhão, durante 2010-2020 / Prevalence of leprosy patients notified in the municipality of São Luís, Maranhão, from 2010 to 2020

Objetivo: identificar a prevalência de pacientes notificados com hanseníase em São Luís, Maranhão, durante 2010-2020. Método: estudo descritivo, exploratório, retrospectivo, transversal, quantitativo, realizado no Banco de Dados público do DATASUS cuja coleta ocorreu em agosto de 2021. A amostra foi composta por 9.387 pacientes notificados com Hanseníase. Resultados: observa-se prevalência amostral em maio de 2012, sexo masculino, faixa etária entre 30-39 anos, Ensino Médio completo, pardos, casos multibacilares, avaliação de incapacidade física e de cura no estágio Grau 0, apresentaram mais que cinco lesões, baciloscopia negativa, episódios sem reação,sendo abordados como casos novos cuja forma prevalente foi a dimorfa, tendo como esquema terapêutico mais prescrito a poliquimioterapia durante 12 meses e prevalência de cura. Conclusão: houve uma prevalência dos casos de hanseníase em homens jovens com bom grau de instrução escolar. Apesar da alta prevalência de casos novos, o tratamento foi efetivo de modo a levá-los à cura.(AU)

Objective: to identify the prevalence of patients notified with leprosy in São Luís, Maranhão, during 2010-2020. Method: descriptive, exploratory, retrospective, cross-sectional, quantitative study, conducted in the public database of the DATASUS whose collection occurred in August 2021. The sample was composed of 9,387 patients notified with Leprosy. Results: sample prevalence was observed in May 2012, male gender, age range 30-39 years, complete High School, brown, multibacillary cases, evaluation of physical disability and cure in Grade 0 stage, presented more than five lesions, negative bacilloscopy, episodes without reaction, being addressed as new cases whose prevalent form was the dimorphic, having as the most prescribed therapeutic scheme the polychemotherapy for 12 months and prevalence of cure. Conclusion: there was a prevalence of leprosy cases in young men with good schooling. Despite the high prevalence of new cases, the treatment was effective in order to lead them to cure(AU)

Objetivo: identificar la prevalencia de pacientes con lepra notificados en São Luís, Maranhão, durante 2010-2020. Método: estudio descriptivo, exploratorio, retrospectivo, transversal, cuantitativo, realizado en la base de datos pública DATASUS, la cual fue recolectada en agosto de 2021. La muestra estuvo conformada por 9.387 pacientes reportados con lepra. Resultados: se tiene una muestra de prevalencia en mayo de 2012, sexo masculino, grupo etario entre 30-39 años, bachillerato completo, pardos, casos multibacilares, valoración de incapacidad física y curación en la etapa Grado 0, presentaba más de cinco lesiones, baciloscopía negativa , episodios sin reacción, siendo abordados como casos nuevos cuya forma prevalente fue borderline, siendo el régimen terapéutico más prescrito la polimedicación durante 12 meses y prevalencia de cura.Conclusión: había una prevalencia de casos de lepra en hombres jóvenes con buena escolaridad. A pesar de la alta prevalencia de casos nuevos, el trataminto fue eficaz de manera que los curará(AU)

Development and validation of a multiplex real-time qPCR assay using GMP-grade reagents for leprosy diagnosis.

Leprosy is a chronic dermato-neurological disease caused by Mycobacterium leprae, an obligate intracellular bacterium. Timely detection is a challenge in leprosy diagnosis, relying on clinical examination and trained health professionals. Furthermore, adequate care and transmission control depend on early and reliable pathogen detection. Here, we describe a qPCR test for routine diagnosis of leprosy-suspected patients. The reaction simultaneously amplifies two specific Mycobacterium leprae targets (16S rRNA and RLEP), and the human 18S rRNA gene as internal control. The limit of detection was estimated to be 2.29 copies of the M. leprae genome. Analytical specificity was evaluated using a panel of 20 other skin pathogenic microorganisms and Mycobacteria, showing no cross-reactivity. Intra- and inter-operator Cp variation was evaluated using dilution curves of M. leprae DNA or a synthetic gene, and no significant difference was observed between three operators in two different laboratories. The multiplex assay was evaluated using 97 patient samples with clinical and histopathological leprosy confirmation, displaying high diagnostic sensitivity (91%) and specificity (100%). Validation tests in an independent panel of 50 samples confirmed sensitivity and specificity of 97% and 98%, respectively. Importantly, assay performance remained stable for at least five months. Our results show that the newly developed multiplex qPCR effectively and specifically detects M. leprae DNA in skin samples, contributing to an efficient diagnosis that expedites the appropriate treatment.

Disease Surveillance Among U.S.-Bound Immigrants and Refugees - Electronic Disease Notification System, United States, 2014-2019.

PROBLEM/CONDITION: Each year, approximately 500,000 immigrants and tens of thousands of refugees (range: 12,000-85,000 during 2001-2020) move to the United States. While still abroad, immigrants, refugees, and others who apply for admission to live permanently in the United States must undergo a medical examination. This examination identifies persons with class A or B conditions. Applicants with class A conditions are inadmissible. Infectious conditions that cause an applicant to be inadmissible include infectious tuberculosis (TB) disease (class A TB), infectious syphilis, gonorrhea, and infectious Hansen's disease. Applicants with class B conditions are admissible but might require treatment or follow-up. Class B TB includes persons who completed successful treatment overseas for TB disease (class B0), those with signs or symptoms suggestive of TB but whose overseas laboratory tests and clinical examinations ruled out current infectious TB disease (class B1), those with a diagnosis of latent TB infection (LTBI) (class B2), and the close contacts of persons known to have TB disease (class B3). Voluntary public health interventions might also be offered during the overseas examination. After arriving in the United States, a follow-up TB examination is recommended for persons with class B TB. PERIOD COVERED: This report summarizes health information that was reported to CDC's Electronic Disease Notification (EDN) system for refugees, immigrants, and eligible others who arrived in the United States during 2014-2019. Eligible others are persons who although not classified as refugees (e.g., certain parolees, special immigrant visa holders, and follow-to-join asylees) are eligible for the same services and benefits as refugees. DESCRIPTION OF SYSTEM: The EDN system has both surveillance and programmatic components. The surveillance component is a centralized database that collects 1) health-related data from the overseas medical examination for immigrants with class A or B conditions and for all refugees and eligible others and 2) TB-related data from the postarrival TB examination. The programmatic component is a reporting system that sends arrival notifications to state and local health agencies in the jurisdiction where newly arriving persons have reported intending to live and provides state and local health agencies and other authorized users with medical data from overseas examinations. RESULTS: During 2014-2019, approximately 3.5 million persons moved to the United States from abroad, including 3.2 million immigrants, 313,890 refugees, and 95,993 eligible others. Among these, the overseas examination identified 139,683 persons (3,903 per 100,000 persons examined) with class B TB, 54 with primary or secondary syphilis (30 per 100,000 persons tested), 761 with latent syphilis (415 per 100,000 persons tested), and, after laboratory testing for gonorrhea was added in 2016, a total of 131 with gonorrhea (374 per 100,000 persons tested). Refugees were offered additional, voluntary interventions, including vaccinations and presumptive treatment for parasites. By 2019, first- and second-dose coverage with measles-containing vaccine were 96% and 80%, respectively. In refugee populations for whom presumptive treatment is recommended, up to 96% of refugees, depending on the specific regimen, were offered and accepted treatment. For the 139,683 persons identified overseas with class B TB, EDN sent arrival notifications and overseas medical data to the appropriate state or local health agency to facilitate postarrival TB examinations. Among 101,119 persons identified overseas as having class B0 TB (6,586) or class B1 TB (94,533), a total of 67,432 (67%) had a complete postarrival examination reported to EDN. Among 35,814 children aged 2-14 years identified overseas with class B2 TB, 20,758 (58%) had a complete postarrival examination reported to EDN. (Adults are not routinely tested for immune reactivity to Mycobacterium tuberculosis during the overseas medical examination.) Among those with a complete postarrival examination reported to EDN, the number with a diagnosis of culture-positive TB disease within the first year of arrival was 464 (688 cases per 100,000 persons examined) for those with class B0 or B1 TB and was 11 (53 cases per 100,000 persons examined) for children with class B2 TB. INTERPRETATION: During 2014-2019, the overseas medical examination system prevented importation of 6,586 cases of infectious TB, 815 cases of syphilis, and 131 cases of gonorrhea. When the examination is used to offer public health interventions, most refugees (up to 96%) accept the intervention. Postarrival follow-up examinations, which were completed for 88,190 persons and identified 475 cases of culture-positive TB, represent an important opportunity to further limit spread of TB disease in the United States by identifying and providing, if needed, preventive care for those with LTBI or treatment for those with disease. PUBLIC HEALTH ACTION: Federal, state, and local health departments and agencies should continue to use EDN data to monitor, evaluate, and improve health-related programs and policies aimed at U.S.-bound or recently arrived immigrants, refugees, and eligible others. Additional public health interventions that could be offered during the overseas medical examination should be considered (e.g., treatment for LTBI). Finally, for persons with class B TB, measures should be taken to identify and remove barriers to completing postarrival examinations to reduce risk for TB disease and community transmission, along with measures to encourage reporting of completed examinations for better data-driven decision-making.

Efficacy of hemoporfin photodynamic therapy for pulsed dye laser-resistant facial port-wine stains in 107 children: A retrospective study.

BACKGROUND: Port-wine stains occur in 0.3-0.5% newborns, mainly on the face and neck. Pulsed dye laser is recognized as the gold standard treatment; nevertheless, it is associated with a low cure rate and a high recurrence rate. AIMS: This study aims to evaluate the efficacy of hemoporfin photodynamic therapy for pulsed dye laser-resistant port-wine stains in children. METHODS: We studied 107 children who received hemoporfin photodynamic therapy for port-wine stains on the face and neck that were resistant to pulsed dye laser. After intravenous injection of 5 mg/kg hemoporfin, the local lesion was irradiated with 532 nm LED green light for 20 min with a power density of 80-100 mW/cm2. A total of 65 patients were given a second treatment after eight weeks. The efficacy and therapeutic responses were recorded at four days and eight weeks after each treatment. RESULTS: The efficacy was positively correlated with the number of treatments received; two treatment sessions yielded significantly better results compared to a single treatment with a response rate of 96.9%, a significant response rate of 50.8% and a cure rate of 21.5%, respectively (P < 0.001). After two treatment sessions, the efficacy was negatively correlated with age (P = 0.04). The efficacy for port-wine stains located on the lateral part was better than that of the central face (P = 0.04). The efficacy for the pink type was better than that for the red and purple types (P = 0.03). No allergic or systematic adverse reactions were reported. LIMITATIONS: No objective measurement data were available. CONCLUSION: Hemoporfin photodynamic therapy is effective and safe for pulsed dye laser-resistant facial port-wine stains in children.

Hindi translation and validation of quality of life score in Indian patients with epidermolysis bullosa; and its correlation with the clinical severity assessment scores: A cross-sectional study.

BACKGROUND: Quality of life (QoL) has not been evaluated in Indian patients having epidermolysis bullosa (EB). AIMS: The aims of the study were to measure health-related QoL in Indian patients having EB using the quality of life in epidermolysis bullosa (QoLEB) questionnaire, and to find its correlation with clinically measured disease severity. METHODS: In this observational cross-sectional study, the QoLEB questionnaire was translated from English to Hindi (QoLEB-Hin) and culturally adapted without a change in concept following standard guidelines. QoLEB-Hin and three clinical scores that have been independently validated in EB, that is, Birmingham Epidermolysis Bullosa severity score (BEBs), Instrument for Scoring Clinical Outcomes of Research for Epidermolysis Bullosa (iscorEB) and Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI), were administered to EB patients/their parents in the presence of an expert. This was followed by validity and correlation studies. RESULTS: Fifty-four patients were recruited (19-females, 35-males; median age 5 years, range 0.025-36 years and 12 patients with an age >13 years). The parents answered the questions for 42 patients (age <13 years). Dystrophic epidermolysis bullosa was diagnosed in 32 (59.2%) patients (dominant dystrophic epidermolysis bullosa [DDEB]-19 [35.2%] and recessive dystrophic epidermolysis bullosa [RDEB]-13 [24.1%]). Junctional epidermolysis bullosa (JEB) and epidermolysis bullosa simplex (EBS) were each diagnosed in 11 (20.4%) patients. The mean ± standard deviation (SD) of QoLEB-Hin score of all epidermolysis bullosa patients was 11.3 ± 7.6 (range 0-28; median and interquartile range [IQR], 10, 10) and reflected an overall moderate degree of affliction on QoL of patients. Mean ± SD of QoLEB-Hin scores for EBS, JEB, DDEB and RDEB were 5.4 ± 3.7 (range, 1-13; median and IQR, 6, 6), 11 ± 6.2 (range, 1-22; median and IQR, 10, 6), 9 ± 5.7 (range, 0-19; median and IQR, 10, 10) and 20.1 ± 6.4 (range, 12-28; median and IQR, 19, 12.5), respectively (P < 0.001, Kruskal-Wallis analysis of variance). Cronbach's alpha coefficient of 0.946 was obtained for all items indicating excellent internal consistency and reliability. Mean sample adequacy was 0.91; absolute fit based off diagonal values was 0.99; indices root mean square error of approximation and root mean square residual were 0.04 and 0.05, respectively, and Tucker Lewis index was >1 indicating overfit. The mean time taken to complete the questionnaire was 6.1 min (range, 6-8 min). QoLEB-Hin correlated significantly (P < 0.001) with BEBs (ρ = 0.79), iscorEB (ρ= 0.63) and EBDASI (ρ = 0.77). Three multiple linear regression models were used to ascertain the strength of relationship between QoL-Hin, and BEBs, iSCOREB and EBDASI, respectively, after adjusting for age, gender and disease subtype. The EBDASI clinical score accounted for approximately 74% (R2 = 0.736, P < 0.001) of the variability in QOL-Hin, as compared to 73% and 55% by BEBs (R2 = 0.731, P < 0.001) and iscorEB (R2 = 0.545, P < 0.001), respectively. LIMITATIONS: Parents filled out the questionnaires for many patients and probably led to an overall moderate degree of affliction of QoL. Comparison with Dermatology Life Quality Index and other QoL scores were not done in this study. Furthermore, the scoring was done at one point in time, and test-retest measurements could not be performed. CONCLUSION: This study validated QoLEB-Hin in an Indian population finding an overall moderate reduction in QoL due to EB. Maximally affected QoL was seen in patients with RDEB. Furthermore, QoLEB-Hin had a variable positive correlation and association with all clinical severity assessment scores.

Juvenile dermatomyositis in Thai children: Retrospective review of 30 cases from a tertiary care center.

BACKGROUND: Juvenile dermatomyositis is a rare condition, but it is the most common idiopathic inflammatory myopathy in pediatric patients. AIM: To study the clinical manifestations, investigations, treatment, clinical course, and outcomes of juvenile dermatomyositis in Thai children. METHOD: This retrospective study included juvenile dermatomyositis patients treated at Siriraj Hospital, a 2,300-bed national tertiary referral center in Bangkok, Thailand, from 1994 to 2019. RESULTS: Thirty patients (22 females and 8 males) were included with a female to male ratio of 2.7:1. Median age at diagnosis was 5.1 years (range, 2.6-14.8 years). Median duration of illness before diagnosis was 6.5 months (range, 0.3-84.0 months). Acute and subacute onset occurred in the majority of patients. Presenting symptoms included muscle weakness in 27/30 (90%), skin rash in 26/30 (86.7%), muscle pain in 17/26 (65.4%), and arthralgia in 4/18 (22.2%) of patients. Dermatologic examination revealed Gottron's rash, heliotrope rash, and periungual telangiectasia in 25/30 (83.3%), 21/30 (70.0%), and 15/24 (62.5%) of patients, respectively. Interestingly, scalp dermatitis was found in 8/21 (38.1%) of patients. The most commonly used treatment regimen in this series was a combination of prednisolone and methotrexate. During the median follow-up of 3.1 years (range, 0.0-18.5 years), only one-third of patients were seen to have monocyclic disease. Extraskeletal osteosarcoma at a previous lesion of calcinosis cutis was observed in one patient at 12 years after juvenile dermatomyositis onset. LIMITATIONS: This was a retrospective single-center study, and our results may not be generalizable to other healthcare settings. Prospective multicenter studies are needed to confirm the findings of this study. CONCLUSION: juvenile dermatomyositis usually poses a diagnostic and therapeutic challenge, which can be compounded by the ethnic variations in the clinical presentation, as observed in this study. Asian patients tend to present with acute or subacute onset of disease, and arthralgia and/or arthritis are less common than in Caucasian patients. Scalp dermatitis is not uncommon in pediatric juvenile dermatomyositis patients. An association between juvenile dermatomyositis and malignancy, though rare, can occur.

Characteristics of children with leprosy: Factors associated with delay in disease diagnosis.

OBJECTIVES: The objectives of the study were to characterize the clinical profile of childhood leprosy presenting at tertiary leprosy care hospitals in the states of Bihar, West Bengal and Uttar Pradesh in India, and to determine the possible risk factors associated with disabilities at presentation. METHODS: Subjects were children with newly diagnosed leprosy registered for treatment at tertiary Leprosy Mission Hospitals in Muzaffarpur (Bihar), Purulia (West Bengal) and Faizabad (Uttar Pradesh), India, between June and December 2019. Demographic and leprosy characteristics were collected at the time of diagnosis. Parents/guardians were interviewed on reasons for delay in presenting at the hospital. Associations between various factors and delay in diagnosis were assessed. RESULTS: Among the 84 children, the mean (SD) age was 10 (3) years with a range of 4-14 years. There were more boys (58%) and most children were currently in school (93%), resident in rural areas (90%) and belonged to a lower socioeconomic status (68%). More children were diagnosed with multibacillary leprosy (69%), one-third of them being skin smear positive for Mycobacterium leprae. On presentation, 17% had deformity (5% grade 1 deformity and 12% grade 2), 29% had nerve involvement and skin lesions were spread across the body in half of the children. Mean (SD) duration of delay was 10.5 (9.8) months. Delayed presentation was more in boys (43% vs. 17%; P = 0.01), those without a history of migration for work compared to those who had a history of migration (40% vs. 9%; P = 0.008) and in those children who were from a poor economic status compared with those that came from a better economic status (44% vs. 7%; P = 0.001) Limitations: Because our study was conducted at tertiary care hospitals, the findings are not representative of the situation in the field. Furthermore, a comparison group of newly diagnosed adult leprosy patients with disability could have been included in the study. CONCLUSION: Childhood leprosy continues to occur in endemic pockets in India and a substantial number present with skin smear positivity and deformity. Guardians of these children cite many reasons for the delay in presentation.

Hanseníase em gêmeos de 3 anos em Mato Grosso, Brasil: a importância da baciloscopia no diagnóstico / Leprosy in 3-year-old twin children in Mato Grosso, Brazil: the importance of bacilloscopy in the diagnosis

Introdução: o diagnóstico clínico da hanseníase em crianças é particularmente difícil. Relato de Caso: crianças gêmeas bivitelinas, com três anos de idade, eram contactantes de pai com hanseníase Virchowiana. Os dois pacientes têm lesões cutâneas bem definidas e irregulares, anteriormente tratadas como micoses e uma cicatriz de BCG. Foram confirmados positivos para Mycobacterium por análise histopatológica da pele. Discussão: especialmente, com menos de cinco anos, os diagnósticos de hanseníase são raros e difíceis porque simulam outras doenças. Esses diagnósticos são alarmes epidemiológicos para áreas endêmicas e mostram a importância dos sintomas em crianças e o rastreamento nos contactantes dos pacientes.

Introduction: the clinical diagnosis of leprosy in children is particularly difficult. Case Report: fraternal twins, three years old, were in contact with a father with Virchowian leprosy. Both patients have well-defined and irregular skin lesions previously treated as mycoses and a BCG scar. They were confirmed positive for Mycobacterium by histopathological analysis of the skin. Discussion:especially, with less than five years, leprosy diagnoses are rare and difficult because they simulate other diseases. These diagnoses are epidemiological alarms for endemic areas and show the importance of symptoms in children and tracking of patients' contacts.

Leprosy and cutaneous leishmaniasis affecting the same individuals: A retrospective cohort analysis in a hyperendemic area in Brazil.

BACKGROUND: Leprosy and cutaneous leishmaniasis (CL) are neglected tropical diseases (NTDs) affecting the skin. Their control is challenging but the integration of skin NTDs control programs is recommended to improve timely detection and treatment. However, little is known about the occurrence of leprosy and CL in the same individuals, and what are the characteristics of such patients. This study aimed to identify and characterize patients diagnosed with both leprosy and CL (i.e., outcome) in the hyperendemic state of Mato Grosso, Brazil. Also, we investigated the demographic risk factors associated with the period between the diagnosis of both diseases. METHODOLOGY/PRINCIPAL FINDINGS: A retrospective cohort study was conducted with patients diagnosed between 2008 and 2017. From the leprosy (n = 28,204) and CL (n = 24,771) databases of the national reporting system, 414 (0.8%; 414/52,561) patients presenting both diseases were identified through a probabilistic linkage procedure. This observed number was much higher than the number of patients that would be expected by chance alone (n = 22). The spatial distribution of patients presenting the outcome was concentrated in the North and Northeast mesoregions of the state. Through survival analysis, we detected that the probability of a patient developing both diseases increased over time from 0.2% in the first year to 1.0% within seven years. Further, using a Cox model we identified male sex (HR: 2.3; 95% CI: 1.7-2.9) and low schooling level (HR: 1.5; 95% CI: 1.2-1.9) as positively associated with the outcome. Furthermore, the hazard of developing the outcome was higher among individuals aged 40-55 years. CONCLUSIONS/SIGNIFICANCE: Leprosy and CL are affecting the same individuals in the area. Integration of control policies for both diseases will help to efficiently cover such patients. Measures should be focused on timely diagnosis by following-up patients diagnosed with CL, active case detection, and training of health professionals.

Risk factors for physical disability in patients with leprosy disease in Yunnan, China: Evidence from a retrospective observational study.

BACKGROUND: Leprosy is potentially debilitating. The risk factors related to physical disabilities associated with leprosy disease in Yunnan, China was not clear. METHODOLOGY/PRINCIPAL FINDINGS: We studied 10644 newly detected leprosy patients from Yunnan, China, from 1990 to 2019. Factors associated with Grade 1 (G1D) and Grade 2 (G2D) physical disabilities or overall physical disabilities (combined G1D and G2D) associated with leprosy were analyzed using multinomial and ordinal logistic regression analyses. The following factors were associated with the development of physical disability in these patients with leprosy: delayed diagnosis [odds ratio (OR): 5.652, 4.399, and 2.275; 95% confidence intervals (CIs): 4.516-7.073, 3.714-5.212, and 2.063-2.509; for ≥ 10, 5-10 y, and 2-5 years, respectively], nerve damage (OR: 3.474 and 2.428; 95% CI: 2.843-4.244, and 1.959-3.008; for 2 and 1 damaged nerves, respectively), WHO classification of PB (OR: 1.759; 95% CI: 1.341-2.307), Ridley-Jopling classification (OR: 1.479, 1.438, 1.522 and 1.239; 95% CI: 1.052-2.079, 1.075-1.923, 1.261-1.838, and 1.072-1.431; for TT, BT, BB, and BL when compared with LL, respectively), advanced age (OR: 1.472 and 2.053; 95% CI: 1.106-1.960 and 1.498-2.814; for 15-59 and over 60 years old, respectively), zero skin lesions (OR: 1.916; 95% CI: 1.522-2.413), leprosy reaction (OR: 1.528; 95% CI: 1.195-1.952), rural occupation (OR: 1.364; 95% CI: 1.128-1.650), Han ethnicity (OR: 1.268; 95% CI: 1.159-1.386), and male sex (OR: 1.128; 95% CI: 1.024-1.243). CONCLUSIONS: Delayed diagnosis, nerve damage, no skin lesions, WHO and Ridley-Jopling classifications, leprosy reactions, advanced age, rural occupation, Han ethnicity, and male sex were associated with disability in leprosy patients. Identifying risk factors could help to prevent physical disability.