Epidemiological situation of leprosy in a province in China: a long time to diagnosis and a high rate of deformity.

BACKGROUND: This epidemiological study aimed to analyse both the distribution and characteristics of leprosy in an endemic province in Northwest China. METHODS: The medical records of leprosy patients in the province of Shaanxi, China, from 1998 to 2018 were collected from the Chinese Leprosy Management Information System (LEPMIS). Epidemiological variables were analysed in this study. RESULTS: A total of 477 new cases were diagnosed between 1998 and 2018 in this region. The average annual detection rate was 0.070/100,000 population, and the average annual prevalence was 0.305/100,000 population. The mean age of the newly diagnosed patients was 46.7 years, and the ratio of males to females was 2.5:1. There were 399 cases (83.6%) of multibacillary (MB) leprosy. One hundred forty-eight patients (31.0%) had grade 2 disability. The mean diagnosis time for new cases was 62.0 months. CONCLUSION: This epidemiological study showed that the characteristics of newly diagnosed leprosy cases in our province were a long time to diagnosis and a high rate of deformity, indicating the need for actions focusing on early diagnosis and treatment and strengthening the detection of leprosy in low-prevalence areas.

Quantitative evaluation of maxillary bone deformation by computed tomography in patients with leprosy.

BACKGROUND: Facial deformation as a sequela of leprosy is caused not only by a saddle nose but also by regression of the maxilla, as well documented in paleopathological observations of excavated skeletal remains of patients with leprosy. However, maxillary changes in living patients have been evaluated only by the subjective visual grading. Here, we attempted to evaluate maxillary bone deformation in patients with leprosy using three-dimensional computed tomography (3D-CT). METHODS: Three-dimensional images centered on the maxilla were reconstructed using multiplanar reconstruction methods in former patients with leprosy (n = 10) and control subjects (n = 5); the anterior-posterior length of the maxilla (MA-P) was then measured. The difference between the MA-P of the patients and those of controls was evaluated after compensating for individual skull size. These findings were also compared with those from previous paleopathological studies. FINDINGS: Three former patients with lepromatous leprosy showed marked atrophy of the maxilla at the prosthion (-8.6, -11.1 and -17.9 mm) which corresponded with the visual appearance of the maxillary deformity, and these results were consistent with paleopathological findings of excavated skeletal remains. Additionally, the precise bone defects of the maxilla could be individually calculated for accurate reconstructive surgery. INTERPRETATION: We have successfully illustrated maxillary bone deformities in living patients with leprosy. This study also confirmed the maxillary regression described in paleopathological studies.

The impact of thalidomide use in birth defects in Brazil.

Although the thalidomide tragedy occurred more than 50 years ago, the medication is still being used worldwide for different reasons, and several aspects regarding its teratogenicity remain unsolved. Despite the strict regulation implemented, new cases of thalidomide embryopathy (TE) are still being registered in Brazil. Furthermore, the molecular processes that lead to malformations when the embryo is exposed to thalidomide have not yet been fully identified. In this article, we perform a critical analysis of thalidomide's history in Brazil, highlighting aspects of the occurrence of TE over the decades. Finally, we present the main perspectives and challenges for ongoing surveillance and prevention of TE in Brazil. The effective control of dispensing thalidomide, especially in areas where leprosy is endemic, is one of the most important and challenging points. Furthermore, the emergence of thalidomide analogues is fast approaching, and their availability would pose additional concerns. The understanding of the molecular mechanisms and targets of thalidomide in both experimental and human models is essential for generating new insights into teratogenic mechanisms, so that safer thalidomide analogues can be developed.

Perfil sociodemográfico e clínico dos pacientes em atendimento fisioterapêutico no Hospital Universitário da Universidade Federal de Juiz de Fora / Sociodemographic and clinical profile of patients in physical therapy at the University Hospital of the Federal University of Juiz de Fora

A Hanseníase é uma doença endêmica no Brasil e se manifesta por sinais e sintomas dermatoneurológicos, tendo alto poder incapacitante. Assim, o fisioterapeuta desempenha importante papel na prevenção de incapacidades motoras e sensitivas. O objetivo deste trabalho foi traçar o perfil sociodemográfico e clínico de pacientes atendidos no Serviço de Fisioterapia do Hospital Universitário da Universidade Federal de Juiz de Fora e fazer um breve relato do trabalho realizado em Hanseníase. Foram coletadas informações nos prontuários eletrônicos e fichas de avaliação de 16 pacientes em atendimento entre outubro de 2013 e março de 2014. Após análise exploratória, foram calculadas frequências simples, médias, desvio padrão e percentuais das variáveis analisadas. A idade média dos pacientes foi de 56,6 anos sendo que receberam o diagnóstico com 47,7 anos. A maioria dos pacientes eram homens (56,2%), da raça branca (62,5%), aposentados (56,2%) e moradores de Juiz de Fora (75%). Houve predomínio do tipo multibacilar (81,2%) e da forma clínica virchowiana (31,3%). Quatorze finalizaram o tratamento poliquimioterápico, embora metade usasse medicação para prevenção de incapacidades e 5 tinham deformidades instaladas. Os primeiros sintomas observados foram: manchas, fraqueza, dores no corpo e perda de sensibilidade. Verificamos que este perfil é semelhante ao de outros serviços: homens, brancos, aposentados, idosos e adultos jovens, com hanseníase multibacilar da forma clínica virchowiana. A maioria não apresenta sequelas, não foi avaliada quanto ao grau de incapacidade e metade estava em reação no momento da pesquisa. Os sintomas, sequelas e principais queixas justificam sua presença em um Serviço de Reabilitação.

Chronic rhinosinusitis in ex-lepromatous leprosy patients with atrophic rhinitis.

AIM: Rhino-sinus mucosal involvement is well documented in untreated lepromatous leprosy, but less understood in ex-leprosy patients (i.e. leprosy patients who have been treated and cured) with atrophic rhinitis. MATERIALS AND METHODS: Rhino-sinus abnormalities were investigated in 13 ex-lepromatous leprosy patients with atrophic rhinitis, using interviews enquiring about sinonasal symptoms, nasal endoscopy, nasal swab culture and computed tomography. Endoscopic sinus surgery had been performed in three patients. The clinical course, computed tomography findings and nasal biopsy results of these three patients were evaluated. RESULTS: All patients had turbinate atrophy and 6 of the 13 (46.2 per cent) had septal perforation. Paranasal sinus involvement was noted in 9 of 12 examined patients (75 per cent). The most commonly affected sinus was the maxillary sinus (in 8 of 12; 66.7 per cent). All three patients treated by endoscopic sinus surgery experienced relapse and required further surgery. Maxillary sinus irrigation was effective for reduction of persistent symptoms such as postnasal discharge and crusts. CONCLUSION: Ex-lepromatous leprosy patients with atrophic rhinitis had various rhino-sinus abnormalities and persistent symptoms. These patients had chronic rhinosinusitis because of underlying atrophic rhinitis. These patients required repeated otolaryngological observations together with combined surgery and conservative treatment.

[Guidelines for the treatment of Hansen's disease in Japan (third edition)].

ad hoc committee of Japanese Leprosy Association recommends revised standard treatment protocol of leprosy in Japan, which is a modification of World Health Organization's multidrug therapy (WHO/MDT, 2010). For paucibacillary (PB) leprosy, 6 months treatment by rifampicin and dapsone (MDT/PB) is enough. However, for high bacterial load multibacillary (MB) leprosy, 12 months treatment seems insufficient. Thus, (A) For MB with bacterial index (BI) > 3 before treatment, 2 years treatment by rifampicin, dapsone and clofazimine (MDT/MB) is necessary. When BI becomes negative and active lesion is lost within 2 years, no maintenance therapy is necessary. When BI is still positive, one year of MDT/MB is added (3 years in total), followed by maintenance therapy by dapsone and clofazimine until BI negativity and loss of active lesions. (B) For MB with BI < 3 or fresh MB (less than 6 months after the onset of the disease) with BI > 3, 1 year treatment by MDT/MB is necessary. When BI becomes negative and active lesion is lost within one year, no maintenance therapy is necessary. When BI is still positive or active lesion is remaining, additional therapy with MDT/MB for one more year is recommended. Brief summary of diagnosis, purpose of therapy, character of drugs, and prevention of deformity is also described.

Giant congenital melanocytic nevus (bathing trunk nevus) associated with lipoma and neurofibroma: report of two cases.

Giant congenital melanocytic nevi are rare and occur in about one out of every 2,00,000 to 5,00,000 births. There is a significant association between bathing trunk nevus and neurofibromatosis and lipomatosis. Apart from this, association of bathing trunk nevus with abnormalities like spina bifida occulta, meningocele, club foot and hypertrophy or atrophy of deeper structures of a limb, have been described. We are herewith reporting two cases of bathing trunk nevi. In our first case, an eight-year-old girl presented with a bathing trunk nevus studded with multiple, large nodules. Histopathological examination of the biopsy taken from one nodule revealed features of both neurofibroma and lipoma. To the best of our knowledge, features of both these hamartomas in one nodule of a single patient are probably not reported in the literature. In our second case, a 12-year-old girl presented with bathing trunk nevus and she had spina bifida occulta. She also had lipoma in the lesion of bathing trunk nevus. Both of our patients had satellite melanocytic nevi over the face, forearm, upper back and legs. Our second patient, in addition, had small melanocytic nevi over the medial canthus and sclerocorneal junction of the right eye. By the time this girl presented to us, the melanocytic nevus started fading in color and it had become brownish. We are reporting these cases for their peculiarities and for their rare features.

[Guideline for the treatment of Hansen's disease in Japan (Second edition)].

ad hoc committee of Japanese Leprosy Association recommends revised standard treatment protocol of leprosy in Japan, which is a modification of World Health Organization's multidrug therapy (WHO/MDT, 1997). For paucibacillary (PB) leprosy, 6 months treatment by rifampicin and dapsone (MDT/PB) is enough. However, for high bacterial load multibacillary (MB) leprosy, 12 months treatment seems insufficient. Thus, (A) For MB with bacterial index (BI) > or = 3 before treatment, 2 years treatment by rifampicin, dapsone and clofazimine (MDT/MB) is necessary. When BI become negative and active lesion is lost within 2 years, no maintenance therapy is necessary. When BI is still positive, one year of MDT/MB is added (3 years in total), followed by maintenance therapy by dapsone and clofazimine until BI negativity and loss of active lesions. (B) For MB with BI < 3 or fresh MB (less than 6 months after the onset of the disease) with BI > or = 3, 1 year treatment by MDT/MB is necessary. When BI become negative and active lesion is lost within one year, no maintenance therapy is necessary. When BI is still positive or active lesion is remaining, additional therapy with MDT/MB for one more year is recommended. This is a simplification of first version in 2000. Brief summary of diagnosis, purpose of therapy, character of drugs, and prevention of deformity is also described.

Disability prevention and medical rehabilitation (DPMR)--prevention of disability and timely referral in leprosy.

The stigma and discrimination due to disabilities in leprosy has a profound effect on a leprosy individual and the disability hampers his/her earning capacity and therefore socio-economic status. The Government of India has recently come out with an approved plan for disability prevention and medical rehabilitation. There are some objectives to provide appropriate services to the disabled or to prevent the disability. In order to prevent disability early treatment is necessary but there are a lot of studies on delayed presentation. It is in fact suggested that not only early correct treatment of leprosy with MDT but also early correct treatment of reactions and nuritis can prevent disability. Some cases as specified in the text require referral to prevent the menace. Many deformities can be corrected by reconstructive surgery. The management protocol to prevent primary impairment is depicted in Figs within the article.

[Congenital malformations in children born after IVF].

BACKGROUND: Major congenital malformations are a leading cause of perinatal morbidity and mortality. Congenital malformations are caused by three factors: genetic, environmental or multifactorial, all of which are present in the context of artificial reproductive techniques. FINDINGS: In 1999 Bergh et al. conducted a retrospective study, which included all the children born following IVF treatment in Sweden. The relative risk found was RR = 1.39 [95% CI 1.25-1.54] and there was no stratification for maternal age and parity. In 2002 Hansen et al. conducted a well-established retrospective study in Western Australia. When only term singletons were included in the study, the OR found was OR = 2.1 [1.4-3.2] in the IVF group and OR = 2.2 [1.2-4] in the ICSI group. Results were stratified for maternal age, parity and offspring sex. A meta-analysis of 19 studies found a relative risk of 1.29 for major malformations among IVF pregnancies. DISCUSSION: Explanations for the increased risk of fetal malformations could be divided into three categories: first, the characteristics of the infertile population which include many risk factors: older age, lower parity, chronic diseases and infertility itself. Second, the techniques used to treat infertility are not physiologic. Third, the characteristics of the pregnancy achieved: the incidence of high-order pregnancies is much greater and this fact exposes the offspring to other risk factors such as preterm birth and low birth weight. CONCLUSIONS: Major advances in reproductive techniques offered hope for many couples, but they were also the reason for much concern regarding the outcome of the awaited offspring. The recent studies seem to justify some of those doubts.

Study on integration of the National Leprosy Eradication Programme into primary health care services: a pilot project.

The Central Leprosy Training and Research Institute, Chengalpattu, Tamil Nadu, undertook an interventional study, as a pilot project, on integration of the National Leprosy Eradication Programme (NLEP) vertical activities into the PHC system in two selected districts of Visakhapatnam and Krishna of Andhra Pradesh, between September 1997 and March 1999. The objective of the study was to assess the various operational aspects and implications of integrated implementation of the activities of the NLEP through the PHC system. 1,304,163 people (239,142 in the study group and 1,065,020 in the control group) were enrolled in the study. The methodology employed in carrying out the pilot project in the two selected districts is described. MDT was in operation for more than 5 years in these two districts through vertical approach. Prevalence has declined from >50/10000 at the start of MDT to about 3/10000 at the start of the project. During the implementation phase, 1459 new leprosy cases were detected (NCDR 5.39/10000 person-years) and 1458 cases were released from treatment (RFT rate: 93.04%) and towards the end of the project 450 cases were under treatment (PR 1.66/10000). Simple rates, ratios and proportions were used in the analysis of data and results were compared. The interventional study was designed to assess the implications and impact of implementation of NLEP activities through PHC staff. The study design, rationale, data collection, data processing, quality control methods employed in the study project and the results are discussed. The results of the study indicate that integrated implementation of the NLEP by PHC staff is feasible, if proper support is provided.

New concerns about thalidomide.

Recently, the Food and Drug Administration (FDA) approved thalidomide for the treatment of the painful symptoms of erythema nodosum leprosum. This most recent FDA decision is a marked reversal to its previous rejection of this drug in the 1960s. The initial rejection by the FDA in the 1960s spared countless American children as thalidomide was shown to cause birth defects and miscarriages worldwide. The FDA's reputation as one of the finest consumer safety authorities was strengthened because of this decision. The recent approval of thalidomide by the FDA, with accompanying strict guidelines and monitoring procedures, has not only brought forth potential benefits, but also created new potential problems.

Surgical correction of deformities and disabilities in leprosy patients.

Despite some positive experiences, surgical rehabilitation, presently, is minimally available in endemic countries and the contingent of patients in need of this modality of treatment is enlarging constantly. With the reduction of prevalence of leprosy and the progressive integration of care of leprosy-affected persons into the general health services, surgical rehabilitation should be made available in these centres. Training health personnel of general health services in leprosy surgery is mandatory and urgent. There is a need for the settlement of a multi-organizational task-force (WHO-ILEP-ILA) to deal with this problem. The aim of this task-force should be the definition of strategies to organize such training and to increase awareness among medical societies and schools regarding this issue. This group should also remember that the referral centres in Africa, Asia, North and South America should act as reservoir of this knowledge and, as such, should be fully supported and enhanced, since they have an important and unique role to play in the training of general health services personnel.