This study aimed to examine hearing function in a group of children aged between the ages of six months and twelve years admitted with bacterial meningitis so as to determine the prevalence and degree of sensorineural hearing loss in them. This prospective study was conducted in the audiology unit and paediatric wards of Kenyatta National Hospital, KNH.The study involved 83 children  between the ages of six months and twelve years admitted with bacterial meningitis. The median age for the children examined was 14 months (range from 5 to 120 months). They were sequentially recruited and at discharge following treatment, underwent age-appropriate hearing testing to evaluate presence and degree of hearing loss which was analyzed. The study was limited by the absence of otoacoustic emission and auditory brainstem responses testing by excluding the significant numbers of children below six months of age admitted with bacterial meningitis.Thirty six of the 83 children (44.4%) were found to have at least a unilateral mild sensorineural hearing loss during initial audiologic testing. Of the children with hearing loss, 22 (26.5%) had mild or moderate sensorineural hearing loss and 14 (16.9%) had severe or profound sensorineural hearing loss.Sensorineural hearing loss was shown to be highly prevalent in children treated for bacterial meningitis. There is therefore a need for objective hearing assessment in infants and young children following bacterial meningitis and further studies involving larger population sizes. Deafness is one of the commonest serious complications of bacterial meningitis in childhood. In developed countries, approximately 10% of survivors of bacterial meningitis are left with permanent sensorineural hearing loss . Other cKenyatta National Hospital, KNH is Kenya’s national referral hospital. Estimates show that an average forty five children are admitted into its pediatric wards each month with a confirmed diagnosis of bacterial meningitis.Behavioral tests of hearing may be used when an infant reaches the developmental age  of six months. Infants not at this level of development and some of those with more than one disability will need to be tested by otoacoustic emissions (OAEs) and auditory brainstem responses (ABRs). Unfortunately the equipment for these latter two sets was unavailable forcing the study to be carried out in children above 6 months of age using behavioural of distraction testing.So far, in KNH no similar study had been undertaken to determine the prevalence and burden of hearing loss following bacterial meningitis in children admitted to KNH.The study involved 83 children  between the ages of six months and twelve years admitted with bacterial meningitis from the pediatric wards, KNH. All cases admitted within twenty four hours of diagnosis, who met the inclusion criteria were recruited every weekday evening during the 3-month study period.The lack of ABR and OAE testing limited the present study to behavioral tests of hearing which may only be used when an infant reaches the developmental age  of six months.All participants fulfilled the following criteria: age six months or older at the time of admission and confirmed diagnosis of bacterial meningitis. Bacterial meningitis was defined according to the World Health Organization (WHO) workbook recommendations based on laboratory findings, symptoms or signs . Those eA full medical history was documented and factors relating to the patient and prior treatment documented by the principal investigator in the questionnaire. The history included parental or a guardians’ assessment of hearing and any history of ear discharge or infection. Otoscopy was done using a Riester hand-held otoscope and its aural speculums and the findings recorded. The children then underwent a thorough physical examination. Hematological and CSF study results were documented. All these were entered in the patient’s proforma.A full physical examination and hearing assessment was done prior to discharge from hospital and two weeks after. This identified and excluded transient hearing loss.All participants’ parents or guardians gave informed consent for the study. Five guardians declined to have their children involved in the study. The study was approved by the institutional ethics and review committee of KNH.All patients completed age-appropriate hearing tests carried out by trained audiologists in the ENT audiology section in a sound- proofed booth. This was well- lit with minimal littering to minimize the child’s distraction. The standard ambient noise was 35 dB. It was also well ventilated and large enough to accommodate the child and parent/ guardian, tester and distractor.Children between six and twenty four months of age underwent the distraction test, those between twenty four and thirty six months of age had the performance tests done while pure tone audiometry was carried out on children above thirty six months of age. They were carried out in the following manner:1 Behavioral testingThe behavioral testing equipment included toys that were not too bright used by the distractor in the behavioral tests to distract the child being examined. Three warblers were used: low, mid and high frequency types. A Manchester rattle was also used to deliver high frequency sounds during behavioral testing. The G-chime was used for mid-frequency sounds and the C-chime for high frequency sound generation.a. Distraction TestA distraction test was performed if the infant was sitting and able to turn and locate the source of a sound. It was carried out with the infant sat upon an adult’s knee facing forwards where a distractor controlled the infant’s attention using toys. The tester introduced the sound signals at high, mid and moderate frequencies from 45 degrees and one meter behind, at the level of the ear. These were tested separately in order to detect hearing loss restricted to one part of the frequency range. The sounds were introduced at very quiet levels (35dBA). Care was taken not to give clues as to the tester’s position other than the test signal.a. Performance Tests2 Warble tones at 500 Hz, 1or 2 kHz and 4 kHz introduced at a very quiet level corresponding to normal hearing. The child was said to have “passed” the screen if there were two responses at the quietest level.The child was shown how to wait until a sound was heard before carrying out an action. Once this could be done the test was be performed at a meter distance and from behind. The test was performed using “Go” for low frequencies, “S” for high frequencies, introduced at the quietest voice levels or 2 Pure Tone AudiometryThis was carried out in all children recruited for the study above thirty six months of age. Pure tones  were introduced using headphones and testing carried out by air conduction (500 H-4000 Hz) and bone conduction (500-4000 Hz). Pure tone audiometry was performed using an Interacoustics clinical audiometer . Sound delivery and masking during pure tone audiometry was ensured using TDH-399 headphones. No sponsorships or competing interests were involved in these tools.The main outcome measure was presence or absence of sensorineural hearing loss in the children.If the child had made a complete recovery from meningitis, lived far from the hospital, and had no hearing loss, follow up was not done.If the child had sequalae that required further management beyond 2 weeks, follow up was continued. For those children exhibiting hearing loss, whether conductive or sensorineural, recommendations for management and follow-up were based on specific test findings and varied accordingly.Descriptive statistics  were calculated to describe the population.The main outcome was degree of sensorineural hearing loss as measured by the age-appropriate hearing test. This had three levels of outcomes: normal, mild/moderate and severe/profound. The population proportions and the 95% confidence interval, CI were estimated for each category of outcome, using statistical methods to give more precise estimates. All categorical variables were cross tabulated with the outcome and Pearson Chi Square computed.We analyzed the outcomes of eighty three children  admitted with bacterial meningitis during the three month study period. The median age for the children examined was 14 months (range from 5 to 120 months). This is represented along with the mean on Table A significant number of the patients enrolled developed sensorineural hearing loss as a sequalae (36/83). The overall prevalence of SNHL loss was estimated to be 43.37% . Of the children with hearing loss, 22 (26.5%) had mild or moderate sensorineural hearing loss, and 14 (16.8%) had severe or profound sensorineural hearing loss. The prevalence of specific categories of SNHL is presented in Table Streptococcus pneumoniae, Haemophilus influenzae and Neisseria meningitidis were isolated in 10, 4, and 3 children respectively. There were no distinctly different clinical presentations among children with S pneumoniae, H influenzae and N meningitidis meningitis.Only seventeen (20.5%) of CSF specimens examined cultured any bacteria. In the present study children proven by history and CSF findings to have bacterial meningitis were evaluated and found to have a sensorineural hearing loss prevalence following the initial hospitalization of 43.4% 95% CI). This was therefore greater than findings of previous studies. Walter et al reported an incidence of 14% 5% CI. Th,4,10,11.Consistent with most prior studies, this work did not reveal any relationship between occurrence and severity of hearing loss to the male gender. Forty nine 59%) of the children evaluated were male while 34 (41%) were female. There were no differences in the prevalence of hearing loss between the two groups. Walter et al. showed that being male was a significant independent risk factor for hearing loss % of the . HoweverHearing loss is highly prevalent in children treated for bacterial meningitis in Kenyatta National Hospital with a prevalence of 43.4%. Age at illness was found not to be a significant determinant of hearing loss following bacterial meningitis. There exists a need for objective hearing assessment in infants and young children following bacterial meningitis. This should be mandatory in all patients treated for bacterial meningitis.No sponsorships or competing interests have been disclosed for this article.Conceived and designed the experiments: BWK. Analysed the data: BWK. Wrote the first draft of the manuscript: BWK. Contributed to the writing of the manuscript: HOO, DW. Agree with manuscript results and conclusions: HOO, DW, PM. Jointly developed the structure and arguments for the paper: BWK, HOO, PM. Made critical revisions and approved final version: HOO, DW. All authors reviewed and approved of the final manuscript.


Hearing difficulties constitute the most common cause of disability globally. Yet, studies on people with hearing difficulties regarding socio-economic status (SES), work, long-term unemployment, sickness absence, and disability pension are scarce. The aim of the present study was to investigate the main income sources of men and women of working ages with and without self-reported hearing difficulties and associations with gender, age, SES, type of living area, and country of birth.A cross-sectional population-based study, using information on self-reported hearing difficulties and SES of 19 045 subjects aged 20–64 years participating in Statistics Sweden’s annual Living Conditions Surveys in any of the years 2004 through 2008. The information was linked to a nationwide database containing data on demographics and income sources. Odds ratios (ORs) and their 95% confidence intervals (CIs) were calculated, using binary logistic regression analysis.Hearing difficulties increased with age and were more common in men (age-adjusted OR: 1.42 (95% CI: 1.30-1.56)) with an overall prevalence of 13.1% in men and 9.8% in women. Using working men as reference, the OR of having hearing difficulties was 1.23 (0.94-1.60) in men with unemployment benefits and 1.36 (1.13-1.65) in men with sickness benefits or disability pension, when adjusting for age and SES. The corresponding figures in women were 1.59 (1.17-2.16) and 1.73 (1.46-2.06). The OR of having sickness benefits or disability pension in subjects with hearing difficulties was 1.36 (1.12-1.64) in men and 1.70 (1.43-2.01) in women, when adjusting for age and SES and using men and women with no hearing difficulties as reference.Hearing difficulties were more prevalent in men. After adjustment with age and SES as well as with type of living area and country of birth, a significant association with unemployment benefits was found only in women, and the associations with long-term sickness absence and disability pension tended to be stronger in women. Hearing difficulties are the most common cause of disability globally . Since tSeveral methods are used for hearing assessment, the most common being pure tone audiometry (PTA) and self-reported hearing measures. PTA relies on patient response to pure tone stimuli and is the most frequent method of clinical hearing assessment. It measures ear-specific hearing thresholds rather than other aspects of hearing, such as sound localization, and the auditory situation is not very similar to normal listening. Self-reported hearing measures are particularly useful for surveying hearing difficulties in a large population since they are easy to administer. The correlations between PTA and self-reported hearing measures have been investigated e.g. -24). As . As 24])People with hearing difficulties constitute a vulnerable group in society. They are at increased risk of underperforming in school -27, of bManual workers are overrepresented among those with hearing difficulties -37, an iIn general, there is a lack of knowledge on SES and type of income of people with self-reported hearing difficulties, compared to others. The aim of the present study was to investigate the main income sources of men and women of working ages with and without self-reported hearing difficulties and associations with gender, age, SES, type of living area, and country of birth.A cross-sectional population-based study was conducted, using survey and register data from Sweden. Included subjects (n=19 045) had participated in the Living Conditions Surveys ) in any of the years 2004 through 2008 and were registered as living in Sweden in 2005, according to Statistics Sweden’s database LISA . They were aged 20–64 years the year of participating in ULF/SILC, were not on early old-age pension, and had answered “yes” or “no” on the ULF/SILC question about having hearing difficulties. Only five subjects had failed to give this answer. None had participated more than once in ULF/SILC during these years. Information from ULF/SILC for these subjects was linked to the LISA database for the same year, using the unique personal identification number assigned to all residents in Sweden.The ULF survey is conducted yearly since 1975 on a random sample of people living in Sweden. Until 2006, data collection was mainly performed through face-to-face interviews, but after this telephone interviews are the main method of data collection. In 2008, the survey European Union Statistics on Income and Living Conditions (EU-SILC) was integrated with ULF, forming ULF/SILC. The response rate in 2004 through 2008 was about 75% .Factors included in the analyses, each included subject was assigned to one of several main income source categories based on their gross income source and number of days of gross income during the year of participation in ULF/SILC.LISA contains information on demographics and income from work, social security sources, and student allowance of all individuals above 15 years of age, registered as living in Sweden as of December 31 of each year . As furtAll individuals in Sweden with income from work or unemployment benefits are covered by the same public sickness insurance. The Social Insurance Agency provides sickness benefits to people who cannot work due to sickness. The employer provides sick pay the first 14 days of a sick-leave spell for employees. If the work capacity is permanently reduced, disability pension can be granted. Sickness benefits cover about 80% and disability pension at least 65% of the lost income . The retParents can be off work with parental benefits for 480 days per child. The benefits cover about 80% of the lost income for 390 days and less for the remaining 90 days .Students in Sweden can apply for government grants and loans  to cover costs of living when attending university or adult secondary education programs (there are no student fees for Swedish citizens). In 2012, full-time students can be granted student allowance of a maximum of 44,600 SEK  per semester .Self-reported hearing difficulties were assessed using the question on hearing difficulties in ULF/SILC. In 2004 through 2007, the question “Can you without difficulties hear what is said in a conversation between several people with or without a hearing aid?” was used. In 2008, the question “Do you have difficulties hearing what is said in a conversation between several people, even if using a hearing aid?” was used. Response alternatives were “yes” and “no” .Using LISA data, the subjects were assigned to one of eight different categories of main income source: A, work; B, parental benefits; C, student allowance; D, social assistance; E, unemployment benefits; F, sickness benefits; G, disability pension; H, miscellaneous. The main income source of a subject was defined as the most prevalent one the year of participation in ULF/SILC. A subject was assigned to category A (work) if his/her income from work exceeded a minimum amount, predefined by Statistics Sweden, where they had used an advanced regression model to render data consistent with other statistics . If haviAge was used as a continuous variable or categorized into five strata, 20–24, 25–34, 35–44, 45–54, and 55–64 years.Based on ULF/SILC data, the subjects were classified into the following SES categories, using the socio-economic classification system of Statistics Sweden ,47: profResident municipality data from LISA were used to classify the subjects as living in urban (including Stockholm (H1), Gothenburg, and Malmö (H2)), semi-urban , or sparsely populated (H4-H6) areas, based on Statistics Sweden’s homogenous region (H region) classification system .Information on country of birth was obtained from LISA and was dichotomized into Sweden and elsewhere.Different types of associations were calculated with either having hearing difficulties or having sickness benefits or disability pension as outcome measure.In analyses where hearing difficulties were used as outcome measure, the eight categories of main income source were reduced to four in order to obtain sufficient statistical power (work (A), unemployment benefits (E), sickness benefits or disability pension (F+G), and others (B+C+D+H)). For the same reason, the eight categories were dichotomized into having sickness benefits or disability pension (F+G) or not (A+B+C+D+E+H) when having sickness benefits or disability pension was used as the outcome measure.The associations between independent variables and outcome measure were calculated with binary logistic regression analysis using IBM SPSS Statistics version 20. The adequacy of the models was evaluated with goodness-of-fit statistics based on Hosmer-Lemeshow decile-of-risk test ,50; a moThe Regional Ethics Board of Stockholm, Sweden approved the study.Table The prevalence of hearing difficulties was 13.1% in men and 9.8% in women. Among the youngest (20-24-year-olds), about 1 in 20 subjects had hearing difficulties, regardless of gender. The prevalence was higher among men in all other age groups and increased with age to a maximum of 25.0% in men and 15.0% in women in the 55-64-year-olds.Among the SES categories, hearing difficulties were most common among members of the categories skilled and unskilled/semiskilled manual work level, with a prevalence of 16.7% and 15.5%, respectively, in men, and of 11.0% and 13.2%, respectively, in women.People living outside metropolitan areas more often reported hearing difficulties than urban dwellers, with the highest prevalence found in sparsely populated areas (15.5% in men and 11.6% in women).Most people had work as main income source (79.2% in men and 71.2% in women), and 12.0% of those men and 8.5% of those women reported hearing difficulties. In men with either sickness benefits or disability pension as main income source (8.4%), the prevalence of hearing difficulties was 19.7% and 26.6%, respectively. The corresponding figures in women (13.9%) were 14.4% and 19.7%, respectively. Having sickness benefits or disability pension was highly associated with age. Among the SES categories, it was most common among members of the category unskilled/semiskilled manual work level, with a prevalence of 12.5% in men and 22.7% in women. Moreover, it was more common outside metropolitan areas than among urban citizens; the highest prevalence was found among people living in sparsely populated areas (11.0% in men and 18.1% in women). Finally, among those born elsewhere, 12.3% of the men and 17.1% of the women had sickness benefits or disability pension as main income source, which were more frequent than among people born in Sweden.The prevalence of hearing difficulties in different ages is illustrated in Figure From Figure SES was also significantly associated with hearing difficulties  and 2.38 (2.03-2.79) in men and women with hearing difficulties, respectively (model 1; Tables  and 2.38Full factorial models including age, SES, hearing difficulties, type of living area, and country of birth as independent variables are presented in Tables This cross-sectional population-based study including 19 045 subjects suggests that people with hearing difficulties are more likely to be dependent on unemployment benefits, sickness benefits, or disability pension than their normal-hearing counterparts. Hearing difficulties were more common in men, but after adjustment with age and SES as well as with type of living area and country of birth, a significant association with long-term unemployment was found only in women, and the associations with long-term sickness absence and disability pension tended to be stronger in women.The associations between having hearing difficulties and unemployment, sickness absence, and disability pension presented here are in agreement with previous results -30,32-34Causality cannot be derived from a cross-sectional study, so one can only speculate about the reasons for the associations found in the present investigation. Hearing difficulties has previously been associated with poorer health ,31,54, wWomen with hearing difficulties are often found to be worse off than their male counterparts ,31,34,54Hearing impairment may have a negative impact on educational performance -27. On tThe prevalence of hearing difficulties in the present investigation was 13.1% in men and 9.8% in women, in well agreement with a new Swedish study on 16-64-year-olds, in which 14.1% of the men and 10.2% of the women reported hearing difficulties , possiblIn men, small regional differences in the prevalence of hearing difficulties were found when adjusting for age and SES as well as for all studied confounders; men living outside metropolitan areas were more likely to report hearing difficulties than urban dwellers. This is in line with the results of two other studies from Sweden ,37. PossBenefits of this study are the large sample size and that the population under investigation is representative of the adult population of Sweden. Another strength is the high quality of the data derived from the population-based register LISA. Data on hearing difficulties and SES came from ULF/SILC, a well-evaluated annual survey that has been running for almost 40 years. Yet other strengths are that information on type of income source was available for the same year as participating in the survey and that adjustment was performed with several potentially important variables, i.e. gender, age, SES, type of living region, and country of birth. Nevertheless, the identified associations might be due to confounders not included in the analyses.As in all surveys, the question about hearing difficulties might have been interpreted in different ways by the participants. Another issue with self-reported health is that some people’s reports may be designed to justify their absence from the labor market , which wThis cross-sectional study suggests that men and women with hearing difficulties are more likely to be dependent on unemployment benefits, sickness benefits, or disability pension than their normal-hearing counterparts, also after adjustment for age, SES, type of living area, and country of birth. Future investigations are warranted to explore the causalities of these associations.Hearing difficulties were more prevalent in men, but a significant association with unemployment was found only in women, and the associations with long-term sickness absence and disability pension tended to be stronger in women. Hitherto, most studies on hearing difficulties have been performed on men, although the increasing prevalence in young individuals has been addressed in several recent studies. The results presented here call for more studies on the situation in hearing-impaired women, a neglected area of research.The authors have no competing interests to declare.PVP, AW, and KA conceived and designed the experiments. PVP conducted the statistical analyzes and drafted the manuscript together with AF and KA, with essential input from AW. All authors read and approved the final version of the manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2458/12/874/prepub


Hearing impairment is the most common sensorineural deficit worldwide. Deafness has a major genetic component, and understanding how genetic variation impacts hearing needs to be extensively studied. The importance of this work is reflected in the review by Lufkin: “the sense of hearing is one of the most crucial senses endowed to a living organism and its loss can have many ramifications.” This special issue about the Genetics of Deafness contributes to these studies by describing new mutations in genes important in hearing, by exploring the clinical implications of treatment based on genotype as well as reviewing of the literature.Included in this issue are reviews of genetic syndromes where deafness is a significant component. Lassaletta and colleagues set out an important review of the molecular mechanism of vestibular schwannoma. Pillion and colleagues characterize hearing loss in osteogenesis imperfecta. Tomelleri's group explores MELAS. All address molecular pathology of hearing loss and explore treatment options.GJB2.Several papers evaluate the distribution or effect of sequence variation in known hearing loss genes. Leal and colleagues describe novel mutations in two previously identified nonsyndromic hearing loss genes. Mateos et al. describe the effects of noncoding sequence variation in Vilarino and colleagues detail the distribution of mutations in pediatric patients in Portugal. Reports of this type improve our understanding of the mechanisms of hearing and the relevance of sequence variation in gene function.GJB2 are the leading cause of congenital deafness in many countries. Malekpour and colleagues present a large study detailing the performance of cochlear implants in patients with and without GJB2 mutation.Mutations in The complexity of genetic deafness and the tremendous progress associated with inherited hearing loss make it clear to us that the “silence genes” will have a lot more to tell us.Edi Lúcia SartoratoEdi Lúcia SartoratoKaren FridericiKaren FridericiIgnacio Del CastilloIgnacio Del Castillo


Objective. This study was conducted to assess the effects of involuntary and persistent noise exposure on health and hearing among Lebanese adults in Beirut, Lebanon, where people are exposed to noise from construction sites, power generators, honking cars, and motorcycles. Methods. Using a descriptive and exploratory design with mixed methods, participants were surveyed, interviewed, and tested for hearing while street noise levels were measured near their residents and work places. Results. Self-reports of 83 Lebanese adult, who lived and worked in Beirut, helped identify common patterns in experiences such as irritability, anger, headaches, and sleep disturbances due to noise annoyance. Of those tested, 30% suffered from high-frequency hearing impairment. Our results showed that environmental sound dB had increased by 12% and sound intensity by 400% above the maximum standard level when compared to the WHO report of 1999. Conclusion. Environmental noise contributes to premature hearing loss and potentiates systemic diseases among Lebanese. The unrecognized effects of persistent exposure to environmental noise on health and hearing and the unexplored nonspecific responses to noise pollution in Beirut, Lebanon, were the focus of this study. Literature  reports The city of Beirut is a metropolitan and multicultural venue with high-rise buildings erected to replace traditional one-family dwelling and occupy a vast portion of the Mediterranean shores in condensed populated areas. The environmental noise associated with ongoing power generators and construction sites begins at dawn and continues through the night as honking cars and motorcycles zigzag through crowded narrow streets. There is no escape from noise since commercial and residential zones are adjacent in close proximity where people live above the shops and businesses. Incessant honking is an expression of frustration when narrow streets are blocked by parked cars on both sides and fruit venders at each intersection. In a surveyed community response to noise in Beirut, Lebanon , partici+. Younger nonsmokers (21–39) showed significant hearing impairment at low frequencies. In a study of 440 local residents of Beirut, ages 21–50, researchers examined the effects of environmental noise and smoking on hearing loss [Environmental noise and health were found  closely Numerous empirical studies have identified long-term exposure to noise as a major health concern. Noise from siren or ambulance can generate an immediate protective reaction known as fight or flight. In a substantial review  traffic n = 24) and exposed them to continuous noise at >75 dB for 1–2 years, 5–10 years, and over 15 years and matched them with corresponding healthy control groups who worked in a quiet environment. After using PolySomnoGraphy (PSG) for an all-night sleep study, subjects rated their sleep quality on a Visual Analogue Scale (VAS), and the results showed that workers who were exposed to occupational noise had poor quality sleep and over the years adapted to noise. However, the long-term adverse health effects of noise hidden in an adaptive process or subsequent systemic diseases were not investigated. Assessing the sleep quality of air travelers and their exhaustion level after sleeping on flights may better reveal brain responses to unrecognized and constant noise. Unprotected and regular exposure to occupational loud noise in the daytime interferes with nocturnal sleep patterns. Researchers  divided 2) of land strip with a population of 4.5 million residents. Over 80% of Lebanese reside in crowded urban areas in high-rise buildings. Researchers [According to the 1998 WHO report , LebanonFarmers using farming equipment are at risk for noise-induced hearing loss . Ninety Noise at a certain frequency and decibel is detrimental to hearing and according to the United States Environmental Protection Agency (USEPA) and World Health Organization (WHO) see  outdoor The purpose of this study was to examine the local perceptions on environmental noise levels and how exposure to involuntary and persistent noise affected young Lebanese health and hearing. To assess street noise level and its health and hearing effects, a cross-sectional design was adopted using mixed methods and the critical theory of participatory action according to Freire . This exAssured of anonymity and confidentiality participants willingly engaged in informal dialogues and interviews for 30–60 minutes. Interviews took place at various locations including workplace, shops, dormitories, or outdoors. Interviewers helped participants recall and explain past and present experiences with noise at different peak hours and describe how noise influenced their lives and probed for any pattern changes in sleep quality, appetite, and unrecognized stress. This study was initiated in September 2009 and ended in May 2010. Written consent in English and Arabic emphasized no risk for voluntary participation. Basic human rights to freedom were closely observed, and participants were given an opportunity to withdraw from the study at any time.A multidisciplinary research team of nursing, medicine, and engineering with cultural literacy retrieved ethnographic data according to Spradley . StandarThe initial proposal was aimed to include 100 equally divided men and women, and after randomly approaching residents and shop keepers on Hamra Streets we could only recruit 83 volunteer adult Lebanese (46 men and 37 women). Researcher and two graduate assistants from nursing administered survey questionnaire and interviewed participants for personal experiences with noise. One research assistant from engineering measured street noise levels in areas where participants worked and lived. Research team worked in rotations and frequently compared data to match participants with locations for increased data accuracy. Informal and semistructured interviews using open-ended questions helped retrieve participants' experiences and perceptions with noise to identify their ways of coping or adjusting to environmental noise. Participants were offered a small incentive to compensate for their time and cooperation. Anonymity was assured, and raw data was secured in locked office cabinets. Demographic data provided age range, the number of years working and/or living on premises, income, and education level. Interviews helped obtain information on health habits such as diet, sleep, and coping techniques. Later, participants were tested for hearing using pure tone screening to determine hearing impairment or loss. The street noise levels were measured at 4 busy intersections near participant's home and workplace using sound level meter (SLM). Our inclusion criteria consisted of Lebanese men and women between the ages 18–38 who lived and worked on Hamra Street for longer than 6 months. We chose a younger age group to eliminate age-related hearing loss or impairment and health complications associated with advanced age. Because this study was conducted in a metropolitan area and the majority of people in Beirut are trilingual  we chose to include people with basic language literacy (read and write) and unexposed to voluntary noise such as loud music.Data were analyzed through an immediate debriefing after each interview and compared with field notes and visual clues. Data was reviewed line by line and coded using the four phases of qualitative study by Polit and Hungler . Author'Researcher and two assistants randomly approached people at various shops, stores, apartment buildings, and dormitories on Hamra Street where residents and shopkeepers often gathered to visit. Consenting participants were later met at a location of their choosing to be surveyed and interviewed. On the same day an appointment was made for each participant to meet individually or as a group for hearing test at the audiology clinic. In three occasions when 4-5 participants were waiting for hearing test, focus group discussion was held ranging from 60–90 minutes to explore more experiences with noise. At other occasions interviews were held at the store, at home, in dormitory, or outdoors. Standard demographic data revealed an average age of 26.5 for men and 27.3 for women, with mixed educational, income, and employment backgrounds. At interviews participants were asked to elaborate on their daily experiences with environmental noise, and 70% (58) had difficulty sleeping at night, and according to the noise meter results they were exposed to Leq 12 hr >65 dBA. In support of our findings multiple studies , 20 haveMost studies on noise report human responses, and few have explored coping with constant noise. Our study focused on finding various forms of coping skills when participants shared experiences with increased craving for sweets 55% (45), caffeine intake 62% (51), and smoking 78% (65). More women 81% (30) in the study reported frustration, anger, and feeling helpless as an aftereffect of persistent noise. Men 93% (43) revealed habituation to noise by unrecognized and later described techniques such as chewing gums or tooth picks, snacking at work, and managing to sleep at night by using other incessant noise such as bathroom fan, radio, and television. Afternoon headaches were common among women 78% (29). The unexpected fear of knowing about hearing status 96% (80) among men and women revealed that majority of participants had suspected some hearing impairment when family members complained about having to speak louder or repeat themselves. Literature support identifies imbalance in endocrine system linked to emotional disturbance. Researchers , 22 remaStudy limitations were few including low budget funding, time line for project completion per 10 months Fulbright contract, long delays for obtaining IRB approval to proceed with data collection, and participants who were business owner and could not leave their work to be tested for hearing or feared results. Participants experienced the physical and emotional effects of persistent noise in form of irritability, anger, nausea, headache, and sleep disturbances. Men 93% (43) mainly reported nervous eating, chewing (gum or tooth picks), and both genders 78% (65) smoked to relieve agitating discomforts (described as auditory disturbance on an aircraft). Participants showed concerns for recent weight gain 51% (42), hypertension 46% (38), and diabetes 54% (45). We assessed participant's hearing, and our measurable indicators and output activities were achieved using audiometer pure tone testing. We found bilateral hearing sensitivity upon assessment by air-conduction audiometric testing at 500–4000 Hz frequencies. Of those tested 40% (33), there were 30% (10) who suffered from asymmetrical high-frequency hearing loss and impairment. Street noise levels were measured by sound level meter (SLM) near participant's home and workplace at 4 intersections on Hamra and Bliss streets. We selected similar time frames and durations to match the WHO study of 1999 for increased accuracy in comparison. Tabulated and analyzed data showed an increase in environmental noise level between 1999 and 2009. The mean average noise standard dBA by the WHO report during 7 am to 6 pm was 55–65, and in 2010 it was 65–75 with a 12% increase in sound dB and 400% increase in noise intensity , we still found them at risk for non-age-related hearing loss. Potential for temporary or permanent loss of hearing among children and young adults warrants greater focus on public education and awareness on noise hazards.


Chorioangioma is a benign angioma of placenta arising from chorionic tissue. Large chorioangioma has unfavourable effects on both mother and fetus. We describe a case with large chorioangioma that had a poor outcome on the fetus. We also reviewed the literature on prognostic factors affecting fetal outcome. Chronic placental insufficiency is the commonest cause for fetal growth restriction. Rare placental causes affecting fetal outcome are partial mole, chorioangioma, and placental teratoma. Large chorioangioma has adverse effects on both mother and fetus. We report a huge chorioangioma resulting in polyhydramnios, preterm labor, and neonatal death due to congestive cardiac failure. A 27-year-old third gravida, one infant death, one living child who were born vaginally presented to us at 32 weeks gestation with history of gradual abdominal distension and vague pain abdomen since one month. On examination, blood pressure was 130/80 mm Hg, abdomen was overdistended upto xiphisternum, fetal parts were not palpable, and fetal heart sounds could not be localized. She was neither diabetic nor anemic. Ultrasound showed a single live fetus corresponding to 32 weeks of gestation with polyhydramnios (AFI: 28 cm). Normal amniotic fluid index ranges from 8 to 20 cm. There were no gross structural abnormalities. Placenta was on the anterior wall upper segment, grade II. A well-defined echogenic mass measuring 11.5 cm × 12 cm different from the rest of the placenta was seen bulging on the fetal side . PatientPlacental chorioangioma is the most common benign tumor of the placenta. The largest retrospective study of 22000 placental examinations showed 138 chorioangioma with an incidence of 0.6% [preeclampsia, preterm labour, placental abruption, and polyhydramnios. Of the various reported clinical complications, the correlation of chorioangioma with hydramnios and preterm delivery is significant, latter being a sequelae of the hydramnios. Fetal congestive heart failure may develop because of the increased blood flow through the low resistance vascular channels in the chorioangioma acting as an arteriovenous shunt. Other associated complications are hydrops, hemolytic anemia, congenital anomalies, fetal thrombocytopenia, cardiomegaly, and growth restriction. Tumors of less than 5 cm are usually asymptomatic and unlikely to cause maternal and fetal complications. Large tumors probably act as arteriovenous shunts and cause complications. Maternal complications are This also helps in close fetal monitoring and hence timely delivery. Gray-scale findings are well-defined complex echogenic mass different from the rest of placenta and tumor protrudes into amniotic cavity near umbilical cord insertion. Use of Doppler to differentiate from placental teratoma, blood clot, and leiomyoma was first demonstrated by Bromley and Benacerraff [Clarke in 1978 described the first case of chorioangioma . Antenatacerraff . On Doppacerraff . UnfortuChorioangioma with complications before fetal viability requires interventions. Various techniques with varying success rates have been tried such as serial fetal transfusions , fetoscoLarge chorioangioma associated with polyhydramnios leads to high perinatal morbidity and mortality, like in this case. Postpartum hemorrhage is a well-known complication in mother. Chorioangioma is often confused with placental teratoma, degenerated myoma, and blood clot. Chorioangioma is differentiated from the rest by demonstration of vascular channels similar to fetal vessels. Echo pattern of blood clot differs with time, while chorioangioma remains same. Partial mole has diffuse pattern and myoma is seen in maternal surface .High fetal death in a case of large chorioangioma warrants institutional and timely delivery as seen in our case. Antenatal diagnosis is by ultrasound, and Doppler would have been the investigation of choice in accurate diagnosis of chorioangioma. Regular followup helps in timely diagnosis and intervention.


The concept of topographic mapping is central to the understanding of the visual system at many levels, from the developmental to the computational. It is important to be able to relate different coordinate systems, e.g. maps of the visual field and maps of the retina. Retinal maps are frequently based on flat-mount preparations. These use dissection and relaxing cuts to render the quasi-spherical retina into a 2D preparation. The variable nature of relaxing cuts and associated tears limits quantitative cross-animal comparisons. We present an algorithm, “Retistruct,” that reconstructs retinal flat-mounts by mapping them into a standard, spherical retinal space. This is achieved by: stitching the marked-up cuts of the flat-mount outline; dividing the stitched outline into a mesh whose vertices then are mapped onto a curtailed sphere; and finally moving the vertices so as to minimise a physically-inspired deformation energy function. Our validation studies indicate that the algorithm can estimate the position of a point on the intact adult retina to within 8° of arc . The coordinates in reconstructed retinae can be transformed to visuotopic coordinates. Retistruct is used to investigate the organisation of the adult mouse visual system. We orient the retina relative to the nictitating membrane and compare this to eye muscle insertions. To align the retinotopic and visuotopic coordinate systems in the mouse, we utilised the geometry of binocular vision. In standard retinal space, the composite decussation line for the uncrossed retinal projection is located 64° away from the retinal pole. Projecting anatomically defined uncrossed retinal projections into visual space gives binocular congruence if the optical axis of the mouse eye is oriented at 64° azimuth and 22° elevation, in concordance with previous results. Moreover, using these coordinates, the dorsoventral boundary for S-opsin expressing cones closely matches the horizontal meridian. PLOS Computational Biology Software ArticleThis is a retinotopic coordinates, examining the mapping of dorsal versus ventral (DV) retina and nasal versus temporal (NT) retina visuotopic mappings: upper versus lower and central versus peripheral visual field The retina projects directly and indirectly to a large number of areas in the central nervous system, such as the mammalian superior colliculus, lateral geniculate nucleus or visual cortex. Understanding the topographic mapping of these projections is a central feature of visual neuroscience Historically, the anatomical organisation of the retina was frequently examined using serial sections, with the emphasis on example sections rather than reconstructions We describe a method to infer where points on a flat-mount retina would lie in a standard, intact retinal space. The standard retina is approximated as a partial sphere, with positions identified using spherical coordinates. Our results show that the method can estimate the location of a point on a flat-mounted retina to within 8° of arc of its original location, or 3.6% of the NT or DV axis. This has allowed us to define a standard retinal space for the adult and for the developing mouse eye. Establishing the orientation of retinal space for the mouse, whose retina contains no intrinsic markers, requires experimental intervention. In the Results, we focus on data from adult mice. We show that a mark based on the centre of the nictitating membrane is reliable and this mark can be related to the insertion of the rectus eye muscles. Furthermore, we transform standard retinal space into visuotopic space and use the geometry of binocular vision, together with anatomical tracing, to address questions about the projection of the optical axis of the mouse eye into visual space Our Retistruct algorithm not only facilitates comparison of differential retinal distributions across animals but also allows analyses of distributions of labelled cells in spherical coordinates, obviating the distortions associated with 2D flat-mounts. Finally, transforming retinal coordinates into visual coordinates gives insights into the functional significance of retinal distributions.In this section we give an overview of the reconstruction algorithm; a more detailed description is contained in the Supplemental Materials and Methods . The staThe locations of mesh points in the flat-mount and their corresponding locations on the sphere define the relation between any point in the flat-mount and a standard spherical space. This relation is used to map the locations of data points and landmarks in the flat retina onto the standard retina. These can be visualised interactively on a 3D rendering of a sphere see , or reprIn order to analyse data points on the standard retina, we used spherical statistics To assess the amount of residual deformation at the end of the energy minimisation procedure, we plotted the length of each edge at mesh  , using tWe used our algorithm on 297 flat-mounted retinae, 288 of which could be reconstructed successfully, 7 of which failed due to, as-yet unresolved, software problems and 2 of which were rejected because of unsatisfactory reconstructions (see below).  measure . The str measure  indicate measure  there arThus the deformation measure as 0.070 , and 27 It should be noted that retinae which have lost tissue due to poor dissection can be forced onto the spherical surface by the algorithm, albeit with high To determine the rim angle of mouse eyes at varying stages of development we measured the distance e retina . We thenAn alternative approach to setting the rim angle is to infer, for each individual retina, the rim angle that minimises the deformation. This was done by repeating the minimisation for rim angles at 1° intervals within a range The deformation measure gives an indication of how easy it is to morph any particular flattened retina onto a partial sphere, but it does not indicate the error involved in the reconstruction, i.e. the difference between the inferred position on the spherical retina and the original position on the spherical retina. The ideal method for estimating the error would be to flatten a retina marked in known locations, and then compare the inferred with the known locations. However, this proved to be technically very difficult and so we tried another method of estimating the error that uses the inferred locations of the optic discs across a number of retinae. In mice, the optic disc is located “rather precisely in the geometric center of the retina” location . If recoWe now describe the first application of the reconstruction algorithm. The relationship between the projections from the mouse retina to the ipsilateral and contralateral dLGN has been studied in retina flat-mounts following injection of retrograde tracer into the dLGN one retina from an animal that had received bilateral injections into the dLGN , MacOS X 10.8 and Microsoft Windows Vista. The user guide, available from the same site, contains details of the two main data formats Retistruct can process. These are either in the form of coordinates of data points and retinal outline from an in-house camera-lucida setup, or in the form of bitmap images with an outline marked up in ImageJ ROI format The reconstruction and transformation methods described here have been implemented in R The program could be applied to flat-mount preparations of retinae from any vertebrate species at any age, provided the globe of the eye is approximately spherical, and it would be possible to add extra analysis routines. When examining retinae with mosaic labelling It should be stressed that the current version is not intended for use on partial retinae. However, it might eventually be possible to locate incomplete retinae in standard retinal space, if there enough markers to orient the partial retina correctly. Similarly, it should be possible to locate sections  of known orientation and hemiretinal origin in standard space.Dataset S1Complete source code for the Retistruct package (version 0.5.7). Includes demonstration data for (ZIP)Click here for additional data file.Text S1Supplemental information including an extended Discussion, and Materials and Methods containing details of experiments and detailed description of the reconstruction algorithm and analysis of reconstructed data.(PDF)Click here for additional data file.


Treacher Collins syndrome (TCS) is a rare autosomal dominant disorder characterized by craniofacial deformities. It is the most common type of mandibulofacial dysostosis (MFD). The objective of this study is to do cytogenetic analysis of a TCS family. Physical examination and all available medical records were reviewed. 50 GTG-banded metaphases were analysed to detect any structural or numerical chromosomal abnormality. Downward slanting of palpebral fissures, hypoplasia of zygomatic arch complex, and hypoplasia of mandible were present in all. Cytogenetic findings show interstitial deletion in chromosomes 5(q32-q33) and 3(q23–q25). We report four members of three generations of a family having TCS in a unique way that the deletion has been found in 3q and 5q which has not been reported. Mosaicism of deletion on 5q was detected in all affected members whereas 3q deletion was found only in one member (II.2). This finding may represent a more severe manifestation of the TCS. Thus the evaluation and counselling of the TCS patients should be undertaken with caution. TCS is a rare genetic disorder characterised by craniofacial deformities. It is named after Edward Treacher Collins 1862–1932), the English surgeon and ophthalmologist who described its essential traits in 1900. TCS is an autosomal dominant disorder which has an incidence of approximately 1 in 50,000 still births 862–1932,, 5. In sThe locus of TCS was initially mapped to a 9cM region on chromosome 5q31–34 –10. The Cytogenetic abnormalities have been useful in directing attention to candidate region in craniofacial anomalies in which the biochemical defect is not known. Chromosomal location in these cases has also been proven successfully through genetic linkage analysis , 16–19. We report a family in which four members of three generations are affected with TCS. The chromosomal analysis of the affected family members was done using GTG-banding which revealed a unique concordance of 3q and 5q deletions, never reported earlier. Although the clinical features of all the affected members are similar but a new deletion has been found in one member. This suggests that TCS locus might be positioned at a locus other than 5q32-33 but the chromosomal analysis of the other three members excluded this possibility.All affected members had normal psychomotor development and intelligence. Downward slanting palpebral fissures, hypoplasia of mandible, and hypoplasia of zygomatic arches are the only clinical features present in each affected member whereas three out of four show partial absence of lower eyelashes. None of the affected members had microphthalmia, epibulbar lipodermoid, upper or lower lid coloboma, hair on the cheeks, microstomia, and choanal atresia. Pedigree  showed tChromosomal analysis was done in TCS patients to identify for the presence of any numerical or structural chromosomal aberrations. For this lymphocyte cultures were setup, and chromosomes were analyzed by G-banding. Five mL of heparinized blood was drawn and kept in an upright position at 37°C for 30 minutes. This helps in the separation of plasma from red blood cells. Then, the plasma and the settled lymphocyte  in buffy coat tapped gently and mixed together. The needle was bent, and 0.5 mL of PLS was transferred into a sterile culture vial containing 5 mL of media RPMI-1640 and 0.2 mL Phytohaemagglutinin (PHA). The cultures were incubated for 72 hours at 37°C. After 70 hours of incubation, 0.1 mL (0.2%) of colcemid was added to the cultures. At 72 hours the samples were washed for removing colcemid. Then, they were centrifuged at a speed of 1000 rpm for 10 minutes, the supernatant was discarded, and freshly prepared pre-warmed hypotonic solution (0.56% KCl) was added and incubated for 20–25 minutes at 37°C. The cell suspension centrifuged again, and after discarding the supernatant, freshly prepared chilled carnoy's fixative (methanol : acetic acid/3 : 1) was added to the cell pellet slowly. At least three changes of fixative were given till the pellet became pale. Two drops of cell suspension were dropped from a height on a clean wet slide.Giemsa staining of chromosome preparation after proteolytic enzyme treatment revealed G-banding. The 3-day old matured unstained chromosome preparations were flooded with 0.25% trypsin for 10–15 seconds, then the slides were rinsed in phosphate buffer saline. The slides were stained in 2% Giemsa stain for 5–7 minutes, thereafter, they were washed in distilled water. Metaphases were analyzed using cytovision software (zeiss microscope) classified according to ISCN 1995. At least 50 metaphases in each patient were analyzed and karyotyped. Cytogenetic studies of peripheral blood lymphocytes showed mosaicism with an interstitial deletion of 3q and 5q. Using 400–450 band level GTG banding, the deleted portion was found to be at 3q23–25 and 5q32-33. Karyotype of the proband (IV.1)  is 46,XYTCS is the most common type of mandibulofacial dystosis. This study shows three generations of a family being affected by TCS. Due to variable expressivity of the gene responsible for the TCS, the phenotypic expression of the affected individuals and obligate carriers varies. Thus the diagnosis in obligate carriers is difficult. In our study the affected members can be easily diagnosed as they all have downward slanting palpebral fissures, hypoplasia of mandible, and hypoplasia of zygomatic arches. Thus the affected members have typical clinical features of TCS. The mode of inheritance in the pedigree is consistent with autosomal dominant (AD) pattern of inheritance. TCS is the most common type of AD mandibulofacial dysostosis. Various reports show a patient with TCS and a de novo deletion of region 4p15.32–p14  a girl wTo the best of our knowledge, there is no report in the literature, which shows the concordance between TCS and 3q2 deletion. Cytogenetic findings in our case show the deletion in 5q3 and 3q2 within a family which has never been reported earlier. Deletion in chromosome 5 has been detected in all the affected members whereas deletion in chromosome 3 has been detected only in one member of the family. We initially thought that our findings may possibly support the genetic heterogeneity, with the 3q2 deletion causing TCS. However, the detection of 5q3 deletion does not support this conclusion. One member of the family (II.2), despite having 3q2 deletion, had 5q3 deletion that excluded the 3q2 being the candidate region for TCS. The deleted 3q23–25 region has been mapped for BPES, primary ovarian failure (POF3), Bruck syndrome 2, autism (AUTS8), short stature (SHOX2), Asperger syndrome, and Leukemia . But review of several reported cases and some of obligatory features of BPES concluded that it has been linked to 3q23–25 region in our case. The region contains a forkhead transcription factor (FOXL2) gene, mutation in which causes BPES. However BPES also has been found in patients showing 7q deletion which shows that BPES is also of genetically heterogeneous entity and it may result from contiguous gene defect , 28. BPEIn summary we propose that we found a novel deletion at 3q23–25 in this family. The phenotype is consistent with that of TCS. Screening and detailed genetic analysis of more patients of TCS should be done to study the alternative candidate loci for TCS.


Limitations in performing activities of daily living (ADL) are important indicators of health and overall wellness, yet relatively few studies specifically identify the ADL abilities of people living with HIV/AIDS (PHAs). Given the wide range of abilities and ages of PHAs, there is an urgent need to utilize an assessment of ADL ability that can validly evaluate those who are very able, as well as those who are very disabled, without the presence of ceiling or floor effects, to provide sensitive measures to detect change.The purpose of this study was to gather preliminary evidence of the validity of using the Assessment of Motor and Process Skills (AMPS) with PHAs.By utilizing existing data records of PHAs from the international AMPS database, we explored (a) demographic factors; (b) person response validity by examining person and individual item goodness-of-fit to the AMPS measurement model; and (c) trends in ADL abilities of PHAs.There were 137 data records in the international AMPS database (0.08% of the database). Goodness-of-fit analyses revealed that >90% of the individuals in the sample fit AMPS measurement model and >99% of the individual item ratings fit the model. More than 80% of the data record had ADL motor abilities that were significantly lower than age expectations, and 67% had ADL process ability measures that were significantly lower than age expectations.The findings indicate that the AMPS is a valid measure of ADL ability for PHAs. Coupled with the lower than expected number of AMPS data records for PHAs and the significant ADL ability challenges that were encountered by this sample, this pilot study may indicate that PHAs encounter barriers to accessing rehabilitation services and/or may not receive referrals until significant ADL challenges are encountered. HIV has become characterized as an episodic, chronic health condition in which individuals experience ongoing cycles of health and function as well as illness and disability –3. WhileWhile higher rates of negative health outcomes have been well documented in the literature, very little research has been conducted to evaluate and measure the everyday functional consequences of living with HIV/AIDS. The International Classification of Functioning, Disability and Health (ICF) , developFurthermore, while limitations in performing activities of daily living (ADL)  are important determinants of health and overall wellness –16, publIn response, strategies for assessing and measuring the everyday functional abilities of PHAs have been developed. However, several challenges have emerged and new research priorities have been documented. For example, Heaton et al.  assert tIn order for researchers and clinicians to document and compare the everyday functional consequences of HIV, there is a need to identify sensitive, valid and reliable measures that can be utilized with diagnostically and culturally diverse persons of all ages and ability levels. A leading and internationally recognized assessment of everyday function is the Assessment of Motor and Process Skills (AMPS) ,21. The The AMPS was designed as a client-centred ADL assessment where clients are allowed to choose two relevant, familiar and appropriately challenging tasks to perform for the assessment. The AMPS includes over 110 standardized ADL tasks that range in challenge from easy to hard and include personal care tasks , home maintenance tasks , simple meal preparation tasks  and more complex meal preparation tasks . Given the chronic, episodic nature of HIV, the higher risk of disabling comorbidities and the wide age range among PHAs, an assessment like the AMPS has an enormous advantage because persons can choose tasks that are culturally and personally relevant, age appropriate and of a suitable challenge.While the AMPS can be utilized to assess the ADL ability of persons who are 2 years of age and older and with any diagnosis, specific studies have not been conducted to evaluate the utility, validity and reliability of using the AMPS specifically with PHAs. Based on studies that have been conducted among persons with relatively mild physical and/or cognitive impairments –24, therThe purpose of this study was to gather preliminary evidence of the validity of using the AMPS with persons who are living with HIV/AIDS. Within this, we address the following research questions: (a) Is the AMPS a valid tool for use with PHAs, as evidenced by acceptable person goodness-of-fit-statistics? (b) Do PHAs, who have been evaluated with the AMPS, demonstrate significantly lower ADL motor and/or ADL process ability measures than their healthy, age-matched peers? and (c) Where sufficient data exist for comparison across groups – are there statistically significant differences in mean ADL motor ability or ADL process ability across different diagnostic groups ?This is a retrospective, descriptive and comparative analysis of existing data records from the international AMPS database. This database is stored and maintained by the AMPS Project International. The database houses anonymized data that has been personally submitted by occupational therapists for the purpose of rater calibration and does not typically include data from evaluations that are conducted after rater calibration. The demographic data housed within the database includes geographic region, age, gender, diagnosis and global functional level. All of the demographic categories (except age) are entered into the user's AMPS software through the utilization of drop-down (preset) menu options.The sample for this study includes pre-existing data records of individuals in the international AMPS database, including men and women from any geographic region, who have been diagnosed with HIV/AIDS. The data records within the international AMPS database are from individuals who were assessed by occupational therapy practitioners within typical occupational therapy settings  from a variety of world regions, including both rural and urban areas. Data records selected for the study met the following inclusion criteria: (a) had a primary or secondary diagnosis of HIV/AIDS; (b) were not associated with rater scoring error; (c) had not been utilized for rater education/training; and (d) had known sex, age and global functional level ratings. Prior to initiating this study, ethics approval was obtained from the Dalhousie University Research Ethics Board.reach for grip and lift the coffee pot, broom, or socks [As stated previously, the AMPS is a standardized evaluation tool of the quality of occupational performance of ADL tasks . More spor socks . ADL proor socks . The AMPor socks .An AMPS evaluation consists of a series of steps, beginning with preparing for the AMPS interview . Within Clients are observed while performing ADL tasks and are scored on their performance according to standardized criteria . The cliMnSq ≤1.4 and associated z<2 indicating acceptable goodness-of-fit to the model [z <2) and will not threaten person-response validity [t≥3) set at ≤2.5%.A descriptive analysis (e.g. frequencies) of the demographic factors of the data records was conducted to determine (a) the frequency and type of HIV co-morbidities; (b) geographic region; (c) age; (d) sex; and (f) date of evaluation. Next, to evaluate the validity of using the AMPS with PHAs, the data were subjected to Rasch analysis using the FACETS computer software , and thehe model ,27. In gvalidity , we suppp<0.05) with the above-mentioned normative rankings, a Kruskal-Wallis test of significance was conducted.While the ADL abilities of healthy individuals improve from childhood to adulthood and then begin to naturally decline after the age of 50 ,31, we dFinally, to further evaluate the validity of the generated AMPS ADL motor and process ability measures, we used the established cut-off measures that demarcate the need for assistance to live in the community ,32,33 toAt the time of data extraction, there were a total of 168,877 data records in the international AMPS database that were not used during rater training or indicative of rater scoring error. From this potential sample, 137 data records (0.08%) met the inclusion criteria, in that every person in the database who had a primary or secondary diagnosis of HIV/AIDS met the inclusionary criteria. The data records that met our inclusionary criteria were evaluated between the years 1995 and 2011, and ranged in age from 4 to 79 years . There were 88 men (64%) and 49 women from varying world regions (i.e. North America (38%), United Kingdom (30%), Europe (25%), New Zealand/Australia (4%), Asia (1.5%) and Africa (1.5%)). The sample included persons who were reported to be independent in the community (13%), in need of minimal assistance to live in the community (36%) and in need of moderate to maximal assistance to live in the community (51%)  and 3.The data included 28 persons (20%) who had a single diagnosis of HIV/AIDS; the remaining persons in the sample had at least one comorbid health condition . The co-n=126) demonstrated acceptable goodness-of-fit to the ADL motor scale, and 92% demonstrated acceptable goodness-of-fit to the ADL process scale. The data were evaluated further, and no specific trends in the data could explain the reason for the slightly higher than expected proportion of misfitting subjects . When evaluating the proportion of misfitting ratings, we expect no more than 2.5% of all of the ratings to misfit, on the motor scale there were 36 misfitting ratings (out of a possible 5033 ratings) and on the process scale there were 42 misfitting ratings (out of a possible 6312 ratings). Thus, <1% of the ratings misfit either the motor or process scales.Of all the persons in the data set, 92% (n=18) was correctly classified as being independent (sensitivity=0.78), and 74% of the sample in need of assistance (n=119) was correctly classified as needing assistance (specificity=0.74). When using the ADL process cut-off measure of 1.0 logit, sensitivity and specificity estimates were 0.79 and 0.75, respectively. When the ADL motor and process cut-off decision points matched (n=88), 82% of the sample (n=16) that was originally rated as independent in the community was correctly classified as being independent (sensitivity=0.82), and 90% of the sample in need of assistance (n=72) was correctly classified as needing assistance (specificity=0.90).When using the ADL motor cut-off measure of 1.5 logits to predict the need for assistance, 78% of the sample that was originally rated as independent in the community  had ADL motor ability measures that were at least 3 SD below age expectations, with only 20% of the sample obtaining motor ability measures that were within age expectations. With regard to age-expected ADL process ability measures, the distribution across the three different categories was more evenly distributed, 33% of the sample had ADL process ability measures that were within age expectations, 29% were 2 SD below age expectations and 38% of the sample obtained ADL process ability measures that were at least 3 SD below age expectations.The majority of the persons in the sample  =13.25, p<0.05). Persons under the age of 18 years had a higher probability of meeting ADL motor ability age expectations and those who were older than 41 years of age had a higher probability of being 3 SD below the mean ADL motor age-expected ability measures.When comparing the age-expected ADL motor and process abilities between men and women, no significant differences were found. When comparing the expected ADL motor and process abilities across age groups, there were no significant differences with regard to ADL process ability. However, there were significant differences with regard to ADL motor ability =22.01, p<0.05 and X2 =19.9, p<0.05, respectively). Further evaluation of the data revealed that PHAs with the comorbid conditions of stroke, other neurologic condition and musculoskeletal impairment were more likely to have ADL motor ability measures that were 3 SD below age expectations. PHAs with the comorbid conditions of stroke, other neurologic condition and memory impairment were more likely to have ADL process ability measures that were 3 SD below age expectations. Finally, PHAs who also had a psychiatric diagnosis were more evenly distributed across the three age expectation categories  than the other diagnostic groups.Finally, when comparing the expected ADL abilities across diagnostic groups, there were significant differences with regard to both ADL motor and ADL process ability (n=1058) had knowingly ever served an HIV-positive client and 66% reported that they did not believe that they had adequate skills to work with clients who are living with HIV.As we consider the representation of men and women within this dataset, in the United Kingdom and North America between 66% and 74% of the HIV cases are men ,35, and significant problem is encountered when completing everyday tasks and participating in desired daily life activities. In this case, there is a possibility that many PHAs with mild to moderate ADL challenges may not receive referrals to occupational therapy and/or other health and wellness-focused rehabilitation services. Finally, it is possible that there is a small subgroup of PHAs who demonstrate ADL limitations, and these individuals are sufficiently targeted by occupational therapy services. While our findings raise many questions that deserve further exploration and critical analysis, it is our belief that there are societal and organizational barriers that prevent PHAs from accessing and receiving the rehabilitation services that are needed to promote safe and effective ADL performance, decrease the need for assistance, prevent decline in ADL abilities and/or prevent the emergence of comorbid health conditions.Possible explanations for this may be that PHAs do not experience ADL limitations until they are quite unwell and/or hospitalized with an acute condition and therefore do not receive referrals to occupational therapy. Alternatively, healthcare professionals may not be evaluating or screening PHAs for ADL or participation challenges, and as a result the presence of ADL challenges may be overlooked and appropriate referrals to rehabilitation specialists may not occur. The most likely explanation may be that health professionals only refer PHAs to occupational therapy once a Despite such concerns, the data have illuminated several important implications. First, the person goodness-of-fit statistics and proportion of misfitting ratings supports the claim that the AMPS generated valid measures for more than 90% of the HIV data records. When evaluating the proportion of misfitting ratings, we found that<1% of the ratings misfit. This finding met our criteria of<2.5% misfit, and thus we suggest that the generated AMPS motor and process ability measures were valid to use for further statistical analysis.Given the diagnostic heterogeneity of the sample, the results correspond well with earlier studies with people who have complex diagnostic profiles . Future The ADL ability measures of this sample of PHAs appear to accurately reflect the therapists’ professional judgment of the day-to-day abilities that promote or hinder independent living in the community. More specifically, we found that the predicted need for assistance (based on ADL motor and process abilities) accurately reflected the evaluating therapists’ evaluation of the need for assistance in the community. Our estimates of sensitivity and specificity were overall consistent with Merritt's earlier work . More spWhen investigating the slightly lower estimates of sensitivity, additional analyses revealed that three of the data records demonstrated inconsistent scoring between the therapists’ rating of overall functional level and problems demonstrated during the AMPS evaluations; these results may be reflective of rater scoring error (i.e. too strict) or reflective of incomplete information provided by the PHA to the therapist regarding the amount of assistance needed for community living or the challenges encountered during daily task performances . Regardless, our overall findings provide one line of evidence to support that the AMPS is a valid measure of ADL ability among PHAs and may also provide evidence to support a PHA's likely need for assistance to live in the community. Through utilizing the AMPS, healthcare providers may be able to effectively identify daily life challenges and subsequently develop proactive intervention strategies to promote health, daily life function and to prevent future ADL decline and/or other adverse health outcomes.In contrast to other studies , it is iThe PHAs within this study demonstrated ADL limitations to an extent where assistance is likely needed to manage living in the community. Alarmingly, 80% of the sample had ADL motor ability measures that were more than 2 SD below their healthy, age matched peers, and 67% of the sample had ADL process ability measures that were more than 2 SD below their healthy, age matched peers. While these figures cannot equate to the ADL abilities of all PHAs, it may be a fair representation of those who have been referred to occupational therapy services. This may indicate that PHAs do not receive referrals to occupational therapy until significant challenges are encountered when performing everyday tasks. Therefore, there exists a need to critically explore not only the ADL abilities of a representative sample of PHAs but also the potential systemic and social barriers that may limit access to rehabilitation services.When comparing the ADL ability measures of PHAs to normative (expected) ability measures, men and women demonstrated similar trends. However, we found that ADL motor limitations were more pronounced with increased age; those who were greater than 40 years of age had a higher likelihood of obtaining ADL motor ability measures that were 3 SD below their healthy peers. Similarly, PHAs with comorbid conditions of stroke and other neurologic conditions were more likely to have ADL motor and ADL process ability measures that were at least 3 SD below their healthy, age-matched peers. PHAs with musculoskeletal and memory impairments were more likely to obtain ADL motor or ADL process ability measures that were at least 3 SD below their healthy, age-matched peers, respectively. Others have found that PHAs who have HIV-associated neurocognitive disorders often have accompanying motor limitations . ThroughThe above findings were not entirely surprising given that others have documented accelerated aging among PHAs , which cOne limitation of this study is that it is unknown if the sample evaluated are representative of all PHAs. For example, due to the episodic nature of HIV and the lack of diagnostic specific information available from the database, there is no way of knowing the point along the continuum of disability that is represented by this sample and what impact this could have on our estimations of the validity of the AMPS for use with PHAs. In addition, due to the relatively small sample size, we were not able to directly compare the validity (i.e. person goodness-of-fit) between men and women. While we did not identify any consistent trends across the misfitting ratings related to sex, additional studies may be warranted. Another limitation is the sample size and the limited demographic information . Further empirical research using the AMPS with PHAs should be performed using a longitudinal design to study the consequences over time onto ADL ability. Regardless of these limitations, this study offers a preliminary exploration of the ADL abilities of PHAs; our findings contribute to the discussion of the potential functional consequences of living with HIV/AIDS and indicate the need for further studies focusing on evaluating ADL ability with sensitive assessments that demonstrate evidence of validity for the specific target group.This study provides preliminary evidence that the AMPS can be used as a valid assessment of ADL abilities among PHAs, as it demonstrates (a) evidence of person response validity in a sample with PHAs; (b) sensitivity in the measures generated, demonstrated in the absence of ceiling and floor effects; and (c) validity of the ADL motor and process ability measures in relation to overall professional judgment of the need for support to function in community. Furthermore, the empirical findings of the study highlight that PHAs may demonstrate ADL limitations that fall well below age expectations in both ADL motor and ADL process abilities, with the risk of functional decline increasing with age. As one of the goals for health-focused interventions aimed at PHAs is promoting health and overall wellness across the lifespan, our findings indicate that future HIV research should focus not only on ADL functioning across the lifespan but also equitable access to rehabilitation services .


Renal mucormycosis is a rare and potentially lethal complication of kidney transplantation. We describe two cases of renal mucormycosis following deceased donor kidney transplantation. This is the second report of renal mucormycosis following kidney transplantation in the United States, and the first case of renal mucormycosis infection presumed to be of recipient origin. Case A had an early presentation of mucormycosis isolated to the kidney allograft. He had an unexpected rise in serum creatinine and leukocytosis necessitating allograft biopsy which showed mucormycosis. He underwent transplant nephrectomy on posttransplant day 11, was treated with amphotericin B, and discharged home on posttransplant day 22. Case B had a late presentation of renal mucormycosis, preceded by a cutaneous manifestation. One year after kidney transplantation he had a nonhealing knee ulcer which on biopsy showed cutaneous mucormycosis. Treatment included aggressive debridement and amphotericin B. Allograft biopsy showed mucormycosis, necessitating transplant nephrectomy. He was discharged to a rehabilitation facility and died from noninfectious causes. Review of the published literature of renal mucormycosis cases following kidney transplantation reveals a mortality rate of more than 50%. The key to successful outcome is early recognition, prompt institution of surgical debridement of all infected tissue, and appropriate antifungal therapy. Mucorales, which are characterized by the production of aseptate hyphae and asexual spores [Mucorales is Rhizopus oryzae (44%), followed by Rhizopus microsporus (22%), Mucor circinelloides (9.5%), Mycocladus corymbifer (5.3%), Rhizomucor pusillus (3.7%), Cunninghamella bertholletiae (3.2%), Mucor indicus (2.6%), Cunninghamella echinulata (1%), and Apophysomyces elegans (0.5%) [Mucormycosis, previously known as zygomycosis, refers to rare infections caused by fungi of the order of l spores . The mosrosporus 2%, Mucormbifer 5.%, Rhizoms oryzae 4%, folloAspergillus molds (8%), endemic fungi (5%), and, least commonly, mucormycosis (2%). The majorities of these mucormycosis cases were pulmonary (56%), with the remainder being sinus (13%), cutaneous (13%), and disseminated (9%). The incidence of invasive fungal infections was the highest among the small bowel transplant recipients (11.6%) and the lowest among kidney transplant recipients (1.3%). In contrast to the presentation of infection among nontransplant recipients, the majority of infections in solid-organ transplant recipients present as pulmonary (37%), followed by rhinocerebral 16%) and cutaneous (16%) infections. The Transplant-Associated Infection Surveillance Network (Roden) performed prospective surveillance for invasive fungal infections by routine review of monthly logs from a registry of solid organ transplant patients  at 15 US transplant centers from 2001 to 2006 [% and cutThe first reported case of mucormycosis following kidney transplantation was a lethal rhinocerebral form in 1970 , 8. HoweWe describe the second report of renal mucormycosis following kidney transplantation in the United States, and the first case of renal mucormycosis infection presumed to be of recipient origin. The remainder of the world literature describes 16 cases of renal mucormycosis following kidney transplantation, nearly all late occurrences of this complication. Overall, mucormycosis is uncommon, mucormycosis of the kidney is even more uncommon, and mucormycosis of the renal allograft specifically is both uncommon and usually a late occurring infection.Case A, a 36-year-old man whose initial cause of end stage renal disease was Hemolytic uremic syndrome. His history is significant for receiving a deceased donor kidney transplant in 1991. In 1992 he underwent bilateral native nephrectomy and in 2007 a transplant nephrectomy was performed for chronic allograft nephropathy. Significant comorbidities include infection with hepatitis C virus . He received zero mismatch, cross match negative, a standard criteria deceased donor kidney transplant after 21 hours of cold preservation. Induction immunosuppression included antithymocyte globulin  and glucocorticoids. The kidney was implanted into a left lower quadrant retroperitoneal pocket. Due to a short donor ureter an ureteroureterostomy with internal stent provided drainage. After reperfusion the transplanted kidney was pink with firm turgor. Rhizomucor was isolated from the specimen (speciation not identified). On posttransplant (PT) day 1 there was scant urine output and the patient required hemodialysis. Urine output increased until postoperative day 4 when a decrease in urine output was associated with pain over the left lower quadrant and left groin. On PT 8, ultrasound was obtained due to a rising creatinine, which showed a migrated ureteral stent with hydronephrosis and he underwent cystoscopy with ureteral stent removal. Ultrasound on PT 9 showed persistent hydronephrosis and a nephrostomy tube was placed on PT 10. Nephrostogram on PT 11 showed the nephrostomy tube in proper position, but obstruction of the kidney. He had a persistent leukocytosis and fevers, but blood and urine cultures were negative for growth of aerobes and anaerobes. Due to concerns for rejection, he underwent allograft biopsy which showed mucormycosis and necrosis . He undeCystoscopy done 4 days after nephrectomy showed normal appearing bladder with no evidence of necrosis. Due to the known predilection for pulmonary and rhinocerebral manifestations of mucormycosis, CT imaging of the lungs, sinuses, and brain were done which showed no evidence of mucormycosis. The patient was discharged home on PT 21.Return to clinic on postnephrectomy day 25 revealed drainage from the incision. The patient underwent surgical debridement and wound exploration. Pathology showed no evidence of cutaneous mucormycosis in the wound. The organ procurement organization was promptly notified of this case of renal mucormycosis at the time of initial discovery. Neither the recipient of the partner kidney graft nor liver graft has demonstrated any evidence of mucormycosis.  Case B is a 54-year-old man with end-stage renal disease due to diabetes mellitus and hypertension. The patient underwent an expanded criteria deceased-donor, cross match negative kidney transplantation. Induction immunosuppression included antithymocyte globulin  and glucocorticoids. The initial postoperative course was complicated by delayed graft function, an upper extremity DVT, and a cardiac ischemic episode. One month after transplant the patient was seen for followup in clinic and noted to have a left knee ulcer, which was treated with local wound care. Five months after transplant, the patient underwent work-up for chronic elevated serum creatinine, including an allograft biopsy which showed no evidence of rejection. However, lab studies revealed cytomegalovirus viremia, for which he was appropriately treated. Nine months after transplant he was readmitted with chills, fevers, and an elevated creatinine. The differential included allograft rejection, infection, and/or anatomic obstruction. He underwent a transplant kidney biopsy which showed only acute tubular injury consistent with tacrolimus toxicity. Blood cultures were positive for coagulase-positive staphylococcus, and he was treated for his bacteremia with a two-week course of appropriate antibiotics. Eleven months after transplant he was readmitted with fever, muscle weakness, joint pain, and myalgias. He was found to have a urinary tract infection and bacteremia with coagulase-positive staphylococcus and treated with a course of antibiotics. Rheumatology performed a diagnostic aspiration of his wrist due to concern for crystalline disease, but the aspirate was negative for crystals and the joint pain and myalgias were attributed to his bacteremia and lymphedema. He was discharged to a rehab facility. Thirteen months after transplant he was seen as an outpatient, the left knee ulcer was noted to not be healing adequately and was biopsied in clinic. The biopsy specimen revealed cutaneous mucormycosis and he was admitted for treatment. He was started on amphotericin B and taken to the operating room for surgical debridement of his left knee lesion. He had an acute rise in his creatinine, which prompted a transplant kidney biopsy three days later, showing renal mucormycosis .  Due to the concern for disseminated mucormycosis, CT imaging of his head, neck, chest, abdomen, and pelvis were done which did not show any evidence mucormycosis in these sites. Due to his significant cardiac history, he first underwent a cardiac catheterization then subsequently underwent transplant nephrectomy. He was also found to have another cytomegalovirus infection which was treated with a course of valganciclovir. He was discharged to a long term care facility on postnephrectomy day 9 on a one month course of amphotericin B and then transitioned to oral posaconazole. He was seen for followup in transplant clinic one month later and was noted to have a healing left knee wound, being treated with a wound vac. He died at the rehabilitation facility from cardiac causes. Renal mucormycosis is a rarely described and potentially lethal complication of kidney transplantation. Case A developed isolated renal mucormycosis and was presented early in the posttransplantation course, undergoing transplant nephrectomy eleven days after transplantation. Histopathology showed an invasion of mucormycosis in the transplanted kidney parenchyma and both donor and recipient ureter, but none in the graft artery or vein. This finding, combined with the fact that neither of the other two recipients of grafts from the same donor developed mucormycosis, suggests that this case of renal mucormycosis originated in the recipient rather than donor derived. However, another potential source of the mucormycosis could be from contaminated preservation fluid. Case B, in contrast, was presented late (more than a year after transplantation), initially manifested as cutaneous mucormycosis treated with debridement and amphotericin B, and found to have simultaneous renal mucormycosis, treated with transplant nephrectomy. In both cases, renal mucormycosis was an unanticipated finding, but one that was promptly acted upon with aggressive surgical debridement, including graft removal and appropriate antifungal agents. Prompt surgical intervention combined with appropriate antifungal therapy contributed to successful outcome in Case A. Case B, in contrast, had significant exposure to immunosuppression therapy as indicated by his persistent CMV infection and cutaneous mucormycosis. In addition, he had significant comorbidities known to increase the risk of Mucor infection including poorly managed insulin dependent diabetes mellitus and ischemic cardiomyopathy. The Literature. The first reported case of renal mucormycosis following kidney transplantation in the United States was in 2010. Alexander et al. described two patients who developed renal mucormycosis following kidney transplant from the same deceased donor [Apophysomyces elegans were recovered from both recipients but not established in the donor, suggesting either contamination of the organs during recovery or undiagnosed donor infection. ed donor . Both paIn the world literature there are 14 additional international case reports of renal mucormycosis following renal transplantation, summarized in  While it is necessary to review the world literature on renal mucormycosis following kidney transplantation, it is important to take into account differences between US and worldwide practices in immunosuppression, organ source, recipient surveillance, and graft management. Based on this sum of 18 reported cases worldwide (including our two cases), renal mucormycosis following kidney transplantation carries a staggeringly high 50% mortality rate. Overall, the majority of renal transplant patients who develop renal mucormycosis are male are diagnosed within the first couple months with the exception of our reported cases, and despite undergoing graft nephrectomy and systemic therapy with Amphotericin B they have a mortality of 50%. The risk factors include the use of immunosuppression drugs, diabetes, environmental factors, and the use of broad spectrum antimicrobial agents. Diagnosis of mucormycosis in transplant patients is extremely difficult and challenging because of rarity, lack of serologic tests, difficulty in isolation, and growth of mucormycetes from infected tissue, blood and body fluid, and often poor staining of mucormycetes with Grocott's methenamine silver stain (GMS) and Periodic Acid Schiff (PAS) due to possible mucoid features and sometimes mimicking acute cellular rejection in clinical presentation. Although extremely rare, renal mucormycosis is a severe and potentially lethal complication of renal transplantation. The keys to successful outcome are having a high index of suspicion for mucormycosis in the differential to enable early recognition, recognizing over immunosuppression as a risk factor for mucormycosis, and prompt, aggressive institution of surgical resection of infected tissue and appropriate pharmacological therapy in order to salvage patients with renal mucormycosis. A three-point strategy in the treatment of mucormycosis is essential and includes surgery, antifungal therapy, and management of risk factors.