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Abstract Objective: The aim of this study was to describe the disease and treatment and to alert health professionals for the identification of signs and symptoms and the need for an early diagnosis in patients with xeroderma pigmentosum (XP). Case description: An 8-year-old male patient was referred to the Joana de Gusmão Hospital (HIJG) in 2021 for evaluation and specialized care. Previously, the child was followed in his place of origin by oncologic and palliative care, where he was submitted to surgeries and chemotherapy. He was admitted to the HIJG using vismodegib, acitrein, tramadol, and solar protective measures. On physical examination, there were tumors and disseminated macular verrucous and ulcerated lesions. The imaging examination showed solid and expansive lesions on the face, and atelectasis and fibroscarring changes in the lung. The histopathological report proved the existence of melanocanthoma, carcinoma, and pyogenic granuloma. After the evaluation of the case, no surgery, chemotherapy, or radiotherapy was performed. It was decided to maintain the palliative treatment and to continue the use of tramadol for pain, and vismodegib and acitretin were used to control carcinomas and prophylactic measures. Comments: The XP is a rare disease of autosomal recessive inheritance whose mechanism comes from failure in the DNA repair by exposure to ultraviolet rays, resulting in lesions on the skin and mucous membranes. They start as sunburns and can progress to melanosis, areas with altered pigmentation, premature aging, poikiloderma, and areas of high risk for neoplasms.
RESUMO Objetivo: Descrever a patologia e o tratamento realizado e alertar profissionais de saúde sobre os sinais e sintomas e sobre a necessidade de diagnóstico precoce em pacientes com xeroderma pigmentoso (XP). Descrição do caso: Paciente do sexo masculino, oito anos, foi encaminhado ao Hospital Joana de Gusmão (HIJG) em 2021, com dianóstico de XP, para avaliação e atendimento especializado. Anteriomente, encontrava-se em serviço de oncologia e de cuidados paliativos em sua cidade de origem, mas já realizara cirurgias e quimioterapias previamente. Foi internado no HIJG em uso de vismodegibe, acitretina, tramadol e medidas de proteção solar. Ao exame físico, apresentou lesões maculares, verrucosas, ulceradas e tumores pelo corpo. Os exames de imagem revelaram lesões sólidas e expansivas na face e atelectasias e alterações fibrocicroscópicas no pulmão. O laudo histopatológico comprovou a existência de melanocantoma, carcinoma e granuloma piogênico. Após a avaliação do caso, optou-se por não realizar cirurgias, quimioterapia nem radioterapia. Decidiu-se manter o tratamento paliativo, continuando o uso de tramadol para dor, vismodegibe e acitretina para o controle de carcinomas e profilaxia à exposição ao sol. Comentários: O XP é uma doença rara de herança autossômica recessiva, cujo mecanismo provém de falha no reparo do DNA pela exposição à luz ultravioleta, resultando em lesões de pele e mucosas. Inicia-se como queimaduras solares e pode avançar para melanoses, áreas com pigmentação alterada, envelhecimento precoce, poiquilodermia e áreas de alto risco neoplásico.
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Objective:To learn about the awareness, education status and willingness of iodine deficiency disorders (IDD) among elementary school students in Guizhou Province, and to provide a scientific basis for exploring intervention strategies for health education of iodine deficiency in the future.Methods:From June 2021 to May 2022, each IDD monitoring county was selected from the east, south, west, north and middle directions of Guizhou Province, and one elementary school was selected from each county. All students in two classes of Grade 5 and Grade 6 were selected in whole groups to conduct on-site questionnaire surveys in the form of anonymous examinations. The survey mainly included general demographic information and IDD awareness, education status and willingness, and binary logistic regression was used to analyze the relevant influencing factors.Results:A total of 1 259 elementary school students in Guizhou Province were investigated, the rates of awareness of IDD, acceptance of IDD publicity and education, and willingness to accept IDD publicity and education among elementary school students were 37.7% (1 900/5 036), 25.1% (316/1 259) and 69.6% (876/1 259), respectively. By binary logistic regression analysis, gender, residence, grade and father's education level were the influencing factors of pupils' awareness of iodine deficiency ( P < 0.05); residence, age and father's education level were the influential factors of elementary school students receiving iodine deficiency education ( P < 0.05); gender, residence, ethnicity and whether the child was the only child or not were the influential factors of elementary school students' willingness to accept IDD education ( P < 0.05). Conclusions:The elementary school students in Guizhou Province have insufficient knowledge about IDD. The publicity and education for iodine deficiency prevention is limited, and the students' willingness to learn is not high. The publicity, education and intervention for iodine deficiency prevention among elementary school students should be comprehensively strengthened.
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Globally, 1.9 billion individuals have inadequate iodine nutrition, of whom 285 million are school?aged children. Universal salt iodization is the favored preventive strategy for iodine deficiency disorders (IDD), the single largest cause of preventable mental retardation. Two thousand and twenty estimates show nearly 1 billion people do not consume iodized salt. When at least 90% of households consume adequately iodized salt (?15 ppm), a country is said to have attained USI. Only 23 countries had household coverage of 90% or higher. India has a household coverage of 76.3%. One 67 million people are at risk of IDD, 54 million suffer from goiter, and 2 million from cretinism. Yearly, 9 million pregnant women and 8 million newborns are at risk. Nagaland, with the second highest coverage of iodized salt in India, the study aims to assess the state?specific approach, track various mitigating measures that effectively sustained the gains of the program.
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Background: Iodine insufficiency is prevalent throughout the world, and is rated as one of the most significant public health concerns in more than 125 countries. Secondary to the insufficient dietary consumption of iodine, almost two billion individuals are prone to suffer from iodine deficiency disorders worldwide. The present survey was conducted in district Chamba of Himachal Pradesh in order to assess the extent of iodine deficiency disorders, 55 years after the initiation of salt iodisation programme.Methods: A cross sectional survey was conducted in district Chamba of Himachal Pradesh. A close ended pretested questionnaire was used to collect the socio-demographic information of the participants. Iodine content of salt and water was assessed with the use of I-Check and AQUA test kits in the households.Results: A total of 230 households were surveyed for the consumption of iodised salt which showed that 100% of the study population was using packet iodised salt for consumption. Majority of the population (92.6%) were placing their salt container near chulah in their kitchen and 73.5% were adding the salt in the beginning of the cooking followed by 16.1% in the middle of the cooking. Iodine content of salt sample collected revealed that 95.7% contain only 7 ppm iodine and 99.6% of the water sample contains less than 0.1 level iodine in water testing.Conclusions: The results of study indicated that population of district Chamba is using iodised salt but there is still need of strengthening of monitoring system as well as health education regarding the cooking practices in the households.
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Objective To comprehensively evaluate the health education effects of iodine deficiency disorders in different areas of Tianjin, and to provide a reference for improving the quality of iodine deficiency disorders health education. Methods The rank sum ratio method (RSR), TOPSIS and fuzzy combination were used to comprehensively evaluate the effect of health education intervention on iodine deficiency disorders in 16 areas of Tianjin. Results The evaluation results of RSR, TOPSIS and fuzzy combination method ranked N, D, and E areas in the last three places, while G, F, and P areas ranked first, second, and third. Combined with the RSR method, it was shown that in the evaluation of the effects of iodine deficiency health education interventions in 16 areas, the health education effect in G, F and P areas was classified as superior, the health education effect in D ,E and N areas was classified as inferior, and other areas were moderate. Conclusion The fuzzy combined RSR-TOPSIS method avoided the limitation of using either RSR or TOPSIS alone and made the conclusion more objective and accurate, and provided comprehensive quantitative results for the health education of iodine deficiency diseases in different areas of Tianjin. This fuzzy combination method objectively evaluated the intervention effect of health education in different areas, which provides a reference for improving the quality of health education for iodine deficiency disorders.
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Objective To investigate the awareness status of iodine deficiency disorders (IDD) prevention and treatment knowledge of target population in Hubei Province, and to evaluate the intervention effect of IDD health education. Methods From 2013 to 2016, 13 counties (cities and districts) were selected as project counties in Hubei Province each year and 7 counties (cities and districts) were selected each year in 2017-2018. Three townships (towns) were chosen in each project county, and the health education activities were carried out among the grade 4-6 students in the central primary school of each township (town). Three administrative villages were selected in each township (town) to carry out community health education activities. Before and after the implementation of health education, 30 students in a fifth grade class from each central elementary school, and 15 housewives around the school were selected to conduct a questionnaire survey on IDD prevention and control knowledge. Results Totally 5 940 elementary school students and 2,970 housewives were investigated from 2013-2018. After carrying out IDD health education activities, the awareness rates of IDD prevention knowledge among primary school students and housewives were 94.28% (16 801/17 820) and 93.20% (8 304/8 910), respectively, which increased significantly compared with those before the health education intervention [71.60% (12 759/17 820) and 70.93% (6 320/8 910), respectively]. The differences were statistically significant (χ2=3,239.83, 1,500.78，P<0.01). Among the primary school students and housewives, the knowledge awareness rates of IDD prevention and treatment were increased by 22.68% and 22.27%, respectively. Conclusion Health education intervention could significantly increase the awareness of IDD among primary school students and housewives, and it is an important measure to maintain IDD elimination status.
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Background: Iodine deficiency disorder is one of the preventable public health diseases in India. Prevalence of goiter is also still prevalence at sub-Himalayan region of West Bengal. Study was conducted to assess prevalence of goiter among school students between 6 and 12 years age, to find out urinary excretion of iodine and iodine content in salt sample and to recommend for consuming iodized salt.Methods: We conducted the study among the school students aged 6 to 12 years during the month of June 2018 in Purba Medinipur district, West Bengal, India. Multistage cluster sampling method was conducted for selecting the study population. Clinical examination was done by medical officer to detect goiters. Salt and urine samples were taken from the selected students and were sent to State Iodine Monitoring Laboratory, Kolkata for examination of iodine content in household salt samples and urine samples.Results: Total 2821 students were examined for survey. Over all prevalence of goiter was 3.75% (109/2821). Girls had more prevalence 4.63% than boys 3.18%. Average urinary iodine excretion (UIE) level is 184.74% in 30 clusters of 339 urine samples. Only 5.60% showed UIE level ≤100 microgram/liter. Total 541 household salt samples were tested for iodine and average iodine content was 22.85%. Among them 91.5% (495) had iodine ≥15 ppm indicating adequate iodine contents in salt samples.Conclusions:Purba Medinipur may be considered a non-endemic district. We recommended continuing use of iodized salt.
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Objective To explore the methods and effects of health education on brucellosis, iodine deficiency disorders and leprosy, and consolidate the achievements of endemic diseases and leprosy control. Methods Three groups of local residents, students and medical staff were selected for health education intervention in Tongguan County of Weinan City, Shaanxi Province in 2017. Among the three groups, the choice of different propaganda modes (brucellosis case warning film, iodine deficiency disorders public service advertisement, leprosy knowledge preaching, etc.) and propaganda materials (promotional foldout, brochure, leaflet, shopping bag, etc.) were compared. At the same time, questionnaire survey was used to compare the awareness rate of health knowledge before and after intervention. Results A total of 375 people were surveyed. On the propaganda modes , 296 people chose to watch the brucellosis case warning film, accounting for 78.93％ (296/375); 184 people chose to watch the iodine deficiency disorders public service advertisement, accounting for 49.07％ (184/375); 286 people chose leprosy knowledge preaching, accounting for 76.27％ (286/375); and 117 people chose to read information , accounting for 31.20％ (117/375). On the propaganda materials, 185 people chose promotional foldout, accounting for 49.33％ (185/375); 232 people chose brochure, accounting for 61.87％ (232/375); 76 people chose leaflet, accounting for 20.27％ (76/375); 277 people chose shopping bag, accounting for 73.87％ (277/375); and 286 people chose exercise book, accounting for 76.27％ (286/375). Before intervention, the total awareness of health knowledge of brucellosis, iodine deficiency disorders and leprosy was 62.18％ (4197/6750), 61.49％ (1153/1875) and 30.02％ (788/2625), respectively; after intervention, the total awareness of health knowledge of brucellosis, iodine deficiency disorders and leprosy was 91.13％(6151/6750), 89.49％(1678/1875) and 89.22％(2342/2625), respectively . The differences were statistically significant before and after intervention (χ2= 1580.30, 397.28, 1910.65, P < 0.01). Conclusions Warning film, knowledge preaching and the issuance of shopping bag and exercise book methods are more popular and more easily accepted by all types of people. Short term health education has a certain effect. In order to acquire and form good health knowledge behaviors really, it is necessary to carry out long-term, extensive and sustained, and diversified work of healthy education.
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ABSTRACT Trichothiodystrophy belongs to a group of rare genetic diseases characterized by DNA repair anomalies. Ocular manifestations can occur in 50% of cases, including cataract, refractive errors, strabismus, microcornea, microphthalmia, dry eye, and pigmentary macular changes. We report a case of childhood glaucoma in a patient with trichothiodystrophy who underwent trabeculectomy in the left eye. To our knowledge, this is the first clinical report of childhood glaucoma associated with trichothiodystrophy.
RESUMO A tricotiodistrofia pertence a um grupo de doenças genéticas raras caracterizadas por anomalias da reparação do DNA. Manifestações oculares podem ocorrer em 50% dos casos, incluindo catarata, erros refrativos, estrabismo, microcórnea, microftalmia, olho seco e alterações maculares pigmentares. Relatamos um caso de glaucoma infantil em um paciente com tricotiodistrofia submetido à trabeculectomia no olho esquerdo. No nosso conhecimento, este é o primeiro caso descrito de glaucoma infantil associado à tricotiodistrofia.
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Humans , Male , Child , Eye Abnormalities/diagnosis , Glaucoma/diagnosis , Trichothiodystrophy Syndromes/diagnosis , Telangiectasis/diagnosis , Trabeculectomy , Eye Abnormalities/surgery , Glaucoma/surgery , Erythema/diagnosis , Intraocular PressureABSTRACT
Micronutrient malnutrition (MNM) can affect all age groups, but young children and women of reproductive age tend to be among those most at risk of developing micronutrient deficiencies. In India, Iodine Deficiency Disorders and Iron Deficiency Anaemia are important Public Health problems.No state in India is free from iodine deficiency. Out of 343 districts surveyed, 286 are reported have Iodine Deficiency Disorders. Iron Deficiency Anaemia is reported in about 60% population across all age groups. The magnitude of vitamin B12 Deficiency is reported to be about 47% in urban population of Delhi while Zinc Deficiency is reported in about 44% amongst Indian children. In addition, there are other micronutrients and minerals like folic acid which are of public health concern.
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As a new model organism,Zebra.fish has been widely used in many aspects of life science research for its unique advantages in growth and development and the convenience of transgenic manipulation.Endemic disease is serious in China.Currently,on one hand,the pathogenesis of endemic fluorosis,endemic arsenism and iodine deficiency disorders is unclear,on the other hand,the cause of Keshan disease and KashinBeck disease is still unknown.This poses great difficulties in the prevention and control of endemic diseases.At present,there are some shortcomings and deficiencies in the animal models used to study the etiology and mechanism of endemic diseases.However,the advantages of Zebrafish as a new model organism just can make up for these deficiencies.Therefore,this paper reviews the application prospects of a new model organism,Zebrafish,in the field of endemic diseases.
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Objective To find out the cognition and participation of people in the disease areas of Shandong Province on prevention and treatment of iodine deficiency disorders (IDD), and to evaluate the effectiveness of a health education project on prevention and control of IDD. Methods From 2011 to 2015, a prospective research was used in study, 46 iodine-deficient counties (cities, districts) were selected as project counties in Shandong Province each year, and 3 townships (towns) were chosen in each project county. Health educational activities on IDD prevention and control knowledge were carried out in the central primary school, 3 villages were chosen in each selected township (town) where the health educational activities in the community were carried out. Before and after the healthy education activities, questionnaire surveys on IDD prevention and control knowledge were conducted among 30 students of grade 5 in the central primary school and 15 housewives lived near the schools in each selected township ( town ) . Results After the health education activities , the knowledge awareness rates of students and housewives on IDD prevention and control were 96.06％ ( 61232/63471 ) and 95.44％(32576/34131), respectively, which increased significantly compared with those before the intervention [ 79 . 00％ ( 50038/63342 ) and 81 . 25％( 27281/33576 ) ] , and the differences were statistically significant (χ2 = 8435.19, 3326.19, P < 0.05). Among the primary school students and housewives, the knowledge awareness rates of IDD prevention were increased by 17.06％ and 14.19％, respectively. Conclusion After implementing the intervention measures of IDD health education, the awareness rate of IDD prevention and control knowledge in iodine deficiency areas in Shandong Province is significantly increased, which has achieved the effectiveness of IDD health education behavioral intervention.
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Objective To explore the iodine nutritional status of people after adjusting iodine content in iodized salt in Anhui Province. Methods In 2014, 30 counties (cities, districts) were selected in Anhui Province according to the probability ratio sampling method (PPS), and one school was selected in each county (city, district), and 50 children aged 8 - 10 years were selected in each school. Urine and household salt samples were collected, urine and salt iodine levels were tested, and thyroid was examined using B-ultrasound. In the township where the school was located, 20 pregnant women's urine samples and household salt samples were collected for urine and salt iodine detection. The salt iodine level was measured by direct titration, Chuan salt and other fortified salt iodine levels were determined by arbitration method (GB/T 13025.7-2012). Urinary iodine was determined by arsenic-cerium catalytic spectrophotometry (WS/T 107-2006). Results A total of 1575 children's household salt samples were collected, the median salt iodine was 23.77 mg/kg, the qualified iodized salt consumption rate was 93.65％(1475/1575); 600 household salt samples were collected from pregnant women, the salt iodine median was 23.50 mg/kg, the consumption rate of qualified iodine salt was 96.33％( 578/600 ) . A total of 1575 urine samples were collected from children, the median urinary iodine was 242.20 μg/L; six hundred urine samples were collected from pregnant women, and the median urinary iodine was 158.15μg/L, 43.33％(13/30) of the counties (cities, districts) pregnant wowen median urinary iodine < 150 μg/L. A total of 1575 thyroid glands were examined in children aged 8 to 10 years, and the thyroid enlargement rate was 3.75％ (59/1575). Conclusion The iodine nutrition of children aged 8 - 10 years in Anhui Province is higher than the appropriate level, but the iodine is not in the excessive state, and the iodine nutrition of pregnant women in some counties (cities, districts) is insufficient.
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@#Iodine deficiency disorders (IDD) during pregnancy may impair the neurological development of the fetus. The aim of this study is to determine the iodine status among pregnant women (PW) in Sarawak after introduction of mandatory universal salt iodisation (USI) for seven years.
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Objective To evaluate the testing ability of iodine in the disease control and prevention institutes at all levels in Shandong,and to raise their testing ability.Methods The testing ability of salt iodine,water iodine and urinary iodine of iodine deficiency disorders laboratories at provincial,prefectural and county levels in Shandong in 2016 was evaluated.The testing results of salt iodine were evaluated by using reference value ± uncertainty.The testing results of water iodine and urinary iodine of all the participatory laboratories were evaluated by using standard Z score generated from laboratories participated in the examination.Results One provincial and 17 prefectural salt iodine,water iodine and urinary iodine laboratories and 30 county level salt iodine laboratories took part in the national examination.Both the feedback rate and qualified rate of the testing results were 100％.The 65 urinary iodine laboratories at the county level took part in the provincial examination.The feedback rate was 92.3％ (60/65),and the qualified rate was 75.4％ (49/65).Conclusions The results of the national examination have showed that the testing ability at all levels of the laboratory is maintained at a higher level;the results of the provincial examination have showed that the testing ability of most of the county level urinary iodine laboratories is relatively stable at a higher level.The testing ability of some county level urinary iodine laboratories is low.We should focus on strengthening the county level urinary iodine laboratory construction.
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Objective To evaluate the testing ability of iodine in the disease control and prevention institutes at all levels in Shandong,and to raise their testing ability.Methods The testing ability of salt iodine,water iodine and urinary iodine of iodine deficiency disorders laboratories at provincial,prefectural and county levels in Shandong in 2016 was evaluated.The testing results of salt iodine were evaluated by using reference value ± uncertainty.The testing results of water iodine and urinary iodine of all the participatory laboratories were evaluated by using standard Z score generated from laboratories participated in the examination.Results One provincial and 17 prefectural salt iodine,water iodine and urinary iodine laboratories and 30 county level salt iodine laboratories took part in the national examination.Both the feedback rate and qualified rate of the testing results were 100％.The 65 urinary iodine laboratories at the county level took part in the provincial examination.The feedback rate was 92.3％ (60/65),and the qualified rate was 75.4％ (49/65).Conclusions The results of the national examination have showed that the testing ability at all levels of the laboratory is maintained at a higher level;the results of the provincial examination have showed that the testing ability of most of the county level urinary iodine laboratories is relatively stable at a higher level.The testing ability of some county level urinary iodine laboratories is low.We should focus on strengthening the county level urinary iodine laboratory construction.
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We carried out this study to assess iodine deficiency disorders among school children of 6-12 years age group in Aligarh district of India. The prevalence of goiter was 5.2%. Median Urinary Iodine Excretion level was 150 µg/L; 22.5% of students had biochemical iodine deficiency. 50.4% households were consuming adequately iodized salt.
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<p><b>OBJECTIVE</b>To assess the effect of different levels of salt iodine content on thyroid volume (ThV) distribution using data from the 1999, 2011, and 2014 Chinese national iodine deficiency disorder (IDD) surveys.</p><p><b>METHODS</b>Probability proportion to size (PPS) sampling method was used to obtain a representative national sample of 34,547, 38,932, and 47,188 Chinese children aged 8-10 years in 1999, 2011, and 2014 Chinese national IDD surveys, respectively. The iodine content in household iodized salt and urinary iodine concentration were measured and thyroid ultrasound examination was performed. The data were analyzed by SAS software using histograms and box plots. The skewness and kurtosis were calculated for testing the normality of ThV.</p><p><b>RESULTS</b>The median iodine content in household iodized salt dropped from 42.30 mg/kg in 1999 to 25.00 mg/kg in 2014. The median urinary iodine concentration of children aged 8-10 years decreased from 306.0 μg/L in 1999 to 197.9 μg/L in 2014. The median and interquartile range (IQR) of ThV in 1999, 2011, and 2014 surveys were 3.44 mL and 1.50 mL, 2.60 mL and 1.37 mL, 2.63 mL and 1.25 mL, respectively. The skewness and kurtosis of ThV distribution in 1999, 2011, and 2014 surveys were 1.34 and 5.84, 0.98 and 3.54, 1.27 and 5.49, respectively.</p><p><b>CONCLUSION</b>With reduced salt iodization levels, the median urinary iodine concentration and median ThV of children decreased significantly, and the symmetry of the ThV distribution improved.</p>
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Child , Female , Humans , Male , China , Iodine , Nutritional Status , Sodium Chloride, Dietary , Thyroid Gland , Diagnostic Imaging , UltrasonographyABSTRACT
The iodine deficiency disorders surveillances were performed in different historical periods according to different surveillance plans in China as various monitoring purposes.Compared with other editions of surveillance plan,the biggest changes were to observe the urinary iodine level,the salt iodine level and the goiter rate in children and pregnant women in the county level in this National Iodine Deficiency Disorders Surveillance Plan (the 2016 edition).In this paper,the implementation details on this surveillance plan were further interrelated,which would play an instrumental role for the staff in iodine deficiency disorders prevention and control.
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Objective To understand the dynamic conditions after reaching the stage goal of elimination of iodine deficiency disorders (IDD) in Wenzhou, and to provide a scientific basis for prevention and treatment of IDD. Methods Three counties that the annual consumption rate of qualified iodized salt < 80% in 2014 and had the prevalence of endemic cretinism in history, Cangnan, Taishun and Yongjia, were selected as high risk monitoring areas. Three townships were selected in each area, and two primary schools were selected from each township, and 40 children urine samples were collected in each school (half male and half female) and the age of children were 8-10 years old. And near the location of these primary schools, we randomly selected 10 pregnant women in each village, and estimated the urinary iodine level and salt iodine concentration, respectively. The examination of thyroid by B ultrasound was performed in children by provincial professionals. Urinary iodine was determined using the arsenic cerium catalytic spectrophotometric method (GB/T 13025.7-2012). Salt iodine was determined by direct titration. Results Endemic cretinism case was not found in this survey, total goiter rate of 8-10 years old children was 2.04%(16/783). The median of urinary iodine was 116.1 and 108.2 μg/L, respectively, in 8 - 10 years old children and pregnant women. Iodized salt coverage rate was 90.48%(171/189), the intaking rate of qualified iodized salt was 84.66% (160/189). The concentration of pregnant women urinary iodine and salt iodine was positively correlated (r=0.54, P< 0.05). Conclusions Children's iodine nutrition is in the appropriate level, but pregnant women are in iodine definciency in Wenzhou City.