Family and carer smoking control programmes for reducing children's exposure to environmental tobacco smoke

Background: Exposure to other people’s cigarette smoke (environmental tobacco smoke, or ETS) is an important child health issue.
Objectives: To determine the effectiveness of interventions aiming to reduce exposure of children to ETS.
Search strategy: The Tobacco Addiction Group register of studies was searched.MEDLINE, EMBASE and four other health and psychology databases were searched electronically, bibliographies of retrieved primary studies were checked and specialists in the area consulted.
Selection criteria: Controlled trials with or without random allocation were included in this review if they addressed participants (parents and other family members, child care workers and teachers) involved with the care and education of infants and young children (aged 0-12 years). All mechanisms for reduction of children’s environmental tobacco smoke exposure, and smoking prevention, cessation, and control programmes targeting these participants are included. These include smoke free policies and legislation, health promotion, social behavioural therapies, technology, education and clinical interventions.
Data collection and analysis: Two reviewers independently assessed studies and extracted data. Due to heterogeneity of methodologies and outcomes, no summary measures were possible and results were synthesised using narrative summaries.
Main results: Nineteen studies met the inclusion criteria, one of which was subsequently excluded. Three interventions were targeted at populations or community settings, seven studies were conducted in the well child health care setting and eight in the ill child health care setting. Twelve of these studies are from North America. In 12 of the 18 studies there was reduction of ETS exposure for children in both intervention and comparison groups. In only four of the 18 studies was there a statistically significant intervention effect. Three of these successful studies employed intensive counselling interventions targeted to smoking parents. There is little difference between the well infant, child respiratory illness and other child illness settings as contexts for parental smoking cessation interventions. The fourth successful intervention was in the school setting targeting the ETS exposure of children from smoking fathers.
Authors’ conclusions: Brief counselling interventions, successful in the adult health setting when coming from physicians, cannot be extrapolated to adults in the setting of child health. There is limited support for more intensive counselling interventions. There is no clear evidence for differences between the respiratory, non-respiratory ill child, well child and peripartum settings as contexts for reduction of children’s ETS exposure.

Interventions for educating children who have attended the emergency room for asthma (Review)

Background: Asthma is one of the most common reasons for paediatric admissions to hospital, with substantial cost to the community. There is some evidence to suggest that many hospital admissions could be prevented with effective education about asthma and its management.
Objectives: To conduct a systematic review of the literature in order to identify whether asthma education leads to improved health outcomes in children who have attended the emergency department for asthma.
Search strategy: We searched the Cochrane Airways Group trials register, including MEDLINE, EMBASE, and CINAHL databases, and reference lists of trials and review articles.
Selection criteria: Randomised controlled trials or controlled clinical trials of asthma education for children who had attended the emergency department for asthma, with or without hospitalisation, within the previous 12 months. Data collection and analysis:Two reviewers independently assessed trial quality and extracted data. Study authors were contacted for additional information.
Main results: Eight trials involving 1407 patients were included, in all the education was provided by nurses or researchers. Compared to control (usual care or lower intensity education) education did not reduce subsequent emergency department (ED) visits [4 trials; relative risk (RR)= 0.87, 95% confidence interval (CI) 0.37 to 2.08], hospital admissions [5 trials; RR=0.74, 95% CI 0.38 to 1.46] and unscheduled doctor visits [5 trials; RR= 0.74, 95% CI 0.49 to 1.12). Each analysis showed evidence of heterogeneity among the studies (P<0.01). Subgroup analyses by the overall difference in scale of intervention between treatment and control groups (comprehensive programme versus information only) or the timing of the intervention/recruitment (early versus delayed) gave similar results to the main analysis and still revealed significant heterogeneity between trials. Authors' conclusions: On the basis of the published trials, there is no firm evidence to support the use of asthma education for children who have attended the emergency department for asthma as a means of reducing subsequent ED visits, hospital admissions or unscheduled doctor visits. Some trials appeared to show clear evidence of benefit, but reasons for differences between these and the negative studies is not clear. More research is required.

Nursing administration of medication via enteral tubes in adults : a systematic review

Background Enteral tubes are frequently inserted as part of medical treatment in a wide range of patient situations. Patients with an enteral tube are cared for by nurses in a variety of settings, including general and specialised acute care areas, aged care facilities and at home. Regardless of the setting, nurses have the primary responsibility for administering medication through enteral tubes. Medication administration via an enteral tube is a reasonably common nursing intervention that entails a number of skills, including preparing the medication, verifying the tube position, flushing the tube and assessing for potential complications. If medications are not given effectively through an enteral tube, harmful consequences may result leading to increased morbidity, for example, tube occlusion, diarrhoea and aspiration pneumonia. There are resultant costs for the health-care system related to possible increased length of stay and increased use of equipment. Presently what is considered to be best practice to give medications through enteral tubes is unknown.

Objectives The objective of this systematic review was to determine the best available evidence on which nursing interventions are effective in minimising the complications associated with the administration of medications via enteral tubes in adults. Nursing interventions and considerations related to medication administration included form of medication, verifying tube placement before administration, methods used to give medication, methods used to flush tubes, maintenance of tube patency and specific practices to prevent possible complications related to the administration of enteral medications.

Search strategy The following databases were searched for literature reported in English only: CINAHL, MEDLINE, The Cochrane Library, Current Contents/All Editions, EMBASE, Australasian Medical Index and PsychINFO. There was no date restriction applied. In addition, the reference lists of all included studies were scrutinised for other potentially relevant studies.

Selection criteria Systematic reviews of randomised controlled trials (RCTs) and RCTs that compared the effectiveness of nursing interventions and considerations used in the administration of medications via enteral tubes. Other research methods, such as non-randomised controlled trials, longitudinal studies, cohort and case control studies, were also included. Exclusion criteria included studies investigating drug–nutrient interactions or the bioavailability of specific medications.

Data collection and analysis Initial consideration of potential relevance to the review was carried out by the primary author (NP). Two reviewers independently assessed study eligibility for inclusion. A meta-analysis could not be undertaken, as there were no comparable RCTs identified. All data were presented in a narrative summary.

Results There is very limited evidence regarding the effectiveness of nursing interventions in minimising the complications associated with enteral tube medication administration in adults. The review highlights a lack of high quality research on many important nursing issues relating to enteral medication administration. There is huge scope for further research. Some of the evidence that was identified included that nurses should consider the use of liquid form medications as there may be fewer tube occlusions than with solid forms in nasoenteral tubes and silicone percutaneous endoscopic gastronomy tubes. Nurses may need to consider the sorbitol content of some liquid medications, for example, elixirs, as diarrhoea has been attributed to the sorbitol content of the elixir, not the drug itself. In addition, the use of 30 mL of water for irrigation when administering medications or flushing small-diameter nasoenteral tubes may reduce the number of tube occlusions.

A systematic review of nursing administration of medication via enteral tubes in adults

Aim. This systematic review aimed to determine the best available evidence regarding the effectiveness of nursing interventions in minimising the complications associated with administering medication via enteral tubes in adults.
Background. Giving enteral medication is a fairly common nursing intervention entailing several skills: verifying tube position, preparing medication, flushing the tube and assessing for potential complications. If not carried out effectively harmful consequences may result leading to increased morbidity and even mortality. Until now, what was considered to be best practice in this area was unknown.
Design. Systematic review.
Methods. CINAHL, MEDLINE, The Cochrane Library, Current Contents/All Editions, EMBASE, Australasian Medical Index and PsychINFO databases were searched up to September 2005. Reference lists of included studies were appraised. Two reviewers independently assessed study eligibility for inclusion. There were no comparable randomised-controlled trials; data
were presented in a narrative summary.
Results. Identified evidence included using 30 ml of water for irrigation when giving medication or flushing small-diameter nasoenteral tubes may reduce tube occlusion. Using liquid medication should be considered as there may be less tube occlusions than with solid forms in nasoenteral tubes and silicone percutaneous endoscopic gastrostomy tubes. In addition, nurses may need to consider the sorbitol content of some liquid medications, for example elixirs, as diarrhoea has been attributed to the sorbitol content of the elixir, not the drug itself.
Conclusion. The evidence was limited. There was a lack of high-quality research on many important issues relating to giving enteral medication.
Relevance to clinical practice. Nurses have the primary responsibility for giving medication through enteral tubes and need knowledge of the best available evidence. Some of the nursing considerations and interventions relating to this skill have been researched in the clinical area and have implications for practice. There is a need for further studies to strengthen these findings.

Nutrition and mental health research in Australia and New Zealand: A review of progress and directions for the future

This review summarises nutrition and mental health research in Australia and New Zealand between 1986 and 2006. The method used to identify papers for inclusion was a search of computerised databases: Medline, Cinahl and Meditext 1986–2006, with subsequent bibliographical review. Key search words were nutrition, diet, mental disorder, mental illness, weight, physical health, Australia and New Zealand. Inclusion criteria included: English language, original data in peer reviewed journals, and examination of some component of nutrition in people with a mental illness. The review of thirteen papers found that the evidence base for dietetic practice in mental health has developed through small assessment and interventional research, often with multidisciplinary collaboration. Future research should include quality and outcome measures with intersectoral partnerships. Dietitians are well positioned to lead and participate in mental health research and to implement research findings to improve the nutritional status of this vulnerable group.

A critical review of daily sedation interruption in the intensive care unit

Background. Daily sedation interruption (DSI) has been proposed as a method of improving sedation management of critically ill patients by reducing the adverse effects of continuous sedation infusions.

Aim. To critique the research regarding daily sedation interruption, to inform education, research and practice in this area of intensive care practice.

Design. Literature review.

Method. Medline, CINAHL and Web of Science were searched for relevant key terms. Eight research-based studies, published in the English language between 1995–December 2006 and three conference abstracts were retrieved.

Results. Of the eight articles and three conference abstracts reviewed, five originated from one intensive care unit (ICU) in the USA. The research indicates that DSI reduces ventilation time, length of stay in ICU, complications of critical illness, incidence of post-traumatic stress disorder and is reportedly used by 15–62% of ICU clinicians in Australia, Europe, USA and Canada.

Conclusions. DSI improves patients' physiological and psychological outcomes when compared with routine sedation management. However, research relating to these findings has methodological limitations, such as the use of homogenous samples, single-centre trials and retrospective design, thus limiting their generalisability.

Relevance to clinical practice. DSI may provide clinicians with a simple, cost-effective method of reducing some adverse effects of sedation on ICU patients. However, the evidence supporting DSI is limited and cannot be generalised to heterogeneous ICU populations internationally. More robust research is required to assess the potential impact of DSI on the physical and mental health of ICU survivors.

The effectiveness of nutrition interventions on dietary outcomes by relative social disadvantage : a systematic review

Objective: To determine whether nutrition interventions widen dietary inequalities across socioeconomic status groups.

Design: Systematic review of interventions that aim to promote healthy eating.

Data sources: CINAHL and MEDLINE were searched between 1990 and 2007.

Review methods: Studies were included if they were randomised controlled trials or concurrent controlled trials of interventions to promote healthy eating delivered at a group level to low socioeconomic status groups or studies where it was possible to disaggregate data by socioeconomic status.

Results: Six studies met the inclusion criteria. Four were set in educational setting (three elementary schools, one vocational training). The first found greater increases in fruit and vegetable consumption in children from high-income families after 1 year (mean difference 2.4 portions per day, p<0.0001) than in children in low-income families (mean difference 1.3 portions per day, p<0.0003). The second did not report effect sizes but reported the nutrition intervention to be less effective in disadvantaged areas (p<0.01). The third found that 24-h fruit juice and vegetable consumption increased more in children born outside the Netherlands ("non-native") after a nutrition intervention (beta coefficient = 1.30, p<0.01) than in "native" children (beta coefficient = 0.24, p<0.05). The vocational training study found that the group with better educated participants achieved 34% of dietary goals compared with the group who had more non-US born and non-English speakers, which achieved 60% of dietary goals. Two studies were conducted in primary care settings. The first found that, as a result of the intervention, the difference in consumption of added fat between the intervention and the control group was –8.9 g/day for blacks and –12.0 g/day for whites (p<0.05). In the second study, there was greater attrition among the ethnic minority participants than among the white participants (p<0.04).

Conclusions: Nutrition interventions have differential effects by socioeconomic status, although in this review we found only limited evidence that nutrition interventions widen dietary inequalities. Due to small numbers of included studies, the possibility that nutrition interventions widen inequalities cannot be excluded. This needs to be considered when formulating public health policy.

Effectiveness of prevention programmes for obesity and chronic diseases amongst immigrants to developed countries - a systematic review

Objective: To determine whether interventions tailored specifically to  particular immigrant groups from developing to developed countries  decrease the risk of obesity and obesity-related diseases.

Design: Databases searched were MEDLINE (1966–September 2008), CINAHL (1982–September 2008) and PsychINFO (1960–September 2008), as well as Sociological Abstracts, PsychARTICLES, Science Direct, Web of Knowledge and Google Scholar. Studies were included if they were randomised control trials, ‘quasi-randomised’ trials or controlled before-and-after studies. Due to the heterogeneity of study characteristics only a narrative synthesis was undertaken, describing the target population, type and reported impact of the intervention and the effect size.

Results: Thirteen studies met the inclusion criteria. Ten out of thirteen (77 %) studies focused on diabetes, seven (70 %) of which showed significant improvement in addressing diabetes-related behaviours and glycaemic control. The effect on diabetes was greater in culturally tailored and facilitated interventions that encompassed multiple strategies. Six out of the thirteen studies (46 %) incorporated anthropometric data, physical activity and healthy eating as ways to minimise weight gain and diabetes-related outcomes. Of the six interventions that included anthropometric data, only two (33 %) reported improvement in BMI Z-scores, total skinfold thickness or proportion of body fat. Only one in three (33 %) of the studies that included cardiovascular risk factors reported improvement in diastolic blood pressure after adjusting for baseline characteristics. All studies, except four, were of poor quality (small sample size, poor internal consistency of scale, not controlling for baseline characteristics).

Conclusions: Due to the small number of studies included in the present review, the findings that culturally tailored and facilitated interventions produce better outcomes than generalised interventions, and that intervention content is more important than the duration or venue, require further investigation.

Validity and reliability of measures of television viewing time and other non-occupational sedentary behaviour of adults : a review

Time spent in non-occupational sedentary behaviours (particularly television viewing time) is associated with excess adiposity and an increased risk of metabolic disorders among adults; however, there are no reviews of the validity and reliability of assessing these behaviours. This paper aims to document measures used to assess adults' time spent in leisure-time sedentary behaviours and to review the evidence on their reliability and validity. Medline, CINAHL and Psych INFO databases and reference lists from published papers were searched to identify studies in which leisure-time sedentary behaviours had been measured in adults. Sixty papers reporting measurement of at least one type of leisure-time sedentary behaviour were identified. Television viewing time was the most commonly measured sedentary behaviour. The main method of data collection was by questionnaire. Nine studies examined reliability and three examined validity for the questionnaire method of data collection. Test–retest reliabilities were predominantly moderate to high, but the validity studies reported large differences in correlations of self-completion questionnaire data with the various referent measures used. To strengthen future epidemiological and health behaviour studies, the development of reliable and valid self-report instruments that cover the full range of leisure-time sedentary behaviour is a priority.

Quantification of urbanization in relation to chronic diseases in developing countries : a systematic review

During and beyond the twentieth century, urbanization has represented a major demographic shift particularly in the developed world. The rapid urbanization experienced in the developing world brings increased mortality from lifestyle diseases such as cancer and cardiovascular disease. We set out to understand how urbanization has been measured in studies which examined chronic disease as an outcome. Following a pilot search of PUBMED, a full search strategy was developed to identify papers reporting the effect of urbanization in relation to chronic disease in the developing world. Full searches were conducted in MEDLINE, EMBASE, CINAHL, and GLOBAL HEALTH. Of the 868 titles identified in the initial search, nine studies met the final inclusion criteria. Five of these studies used demographic measures (such as population density) at an area level to measure urbanization. Four studies used more complicated summary measures of individual and area level data (such as distance from a city, occupation, home and land ownership) to define urbanization. The papers reviewed were limited by using simple area level summary measures (e.g., urban rural dichotomy) or having to rely on preexisting data at the individual level. Further work is needed to develop a measure of urbanization that treats urbanization as a process and which is sensitive enough to track changes in “urbanicity” and subsequent emergence of chronic disease risk factors and mortality.

The effect of tai chi on psychosocial well-being : a systematic review of randomized controlled trials

Objective : This systematic review aimed to critically appraise published clinical trials designed to assess the effect of Tai Chi on psychosocial well-being.

Data Sources : Databases searched included MEDLINE, CINAHL, EMBASE, HEALT, PsycINFO, CISCOM, the Cochrane Central Register of Controlled Trials of the Cochrane Library, and dissertations and conference proceedings from inception to August 2008.

Review Methods : Methodological quality was assessed using a modified Jadad scale. A total of 15 studies met the inclusion criteria (i.e. English publications of randomized controlled trials with Tai Chi as an intervention and psychological well-being as an outcome measure), of which eight were high quality trials. The psychosocial outcomes measured included anxiety (eight studies), depression (eight studies), mood (four studies), stress (two studies), general mental health three studies), anger, positive and negative effect, self-esteem, life satisfaction, social interaction and self-rated health (one study each).

Results : Tai Chi intervention was found to have a significant effect in 13 studies, especially in the management of depression and anxiety. Although the results seemed to suggest Tai Chi is effective, they should be interpreted cautiously as the quality of the trials varied substantially. Furthermore, significant findings were shown in only six high quality studies. Moreover, significant between group differences after Tai Chi intervention was demonstrated in only one high quality study (the other three significant results were observed in non-high quality studies). Two high quality studies in fact found no significant Tai Chi effects.

Conclusion : It is still premature to make any conclusive remarks on the effect of Tai Chi on psychosocial well-being.

Review of the quality of studies on the economic effects of smoke-free policies on the hospitality industry

Objective: To compare the quality and funding source of studies concluding a negative economic impact of smoke-free policies in the hospitality industry to studies concluding no such negative impact.

Data sources: Researchers sought all studies produced before 31 August 2002. Articles published in scientific journals were located with Medline, Science Citation Index, Social Sciences Citation Index, Current Contents, PsychInfo, Econlit, and Healthstar. Unpublished studies were located from tobacco company websites and through internet searches.

Study selection: 97 studies that made statements about economic impact were included. 93% of the studies located met the selection criteria as determined by consensus between multiple reviewers.

Data extraction: Findings and characteristics of studies (apart from funding source) were classified independently by two researchers. A third assessor blind to both the objective of the present study and to funding source also classified each study.

Data synthesis: In studies concluding a negative impact, the odds of using a subjective outcome measure was 4.0 times (95% confidence interval (CI) 1.4 to 9.6; p = 0.007) and the odds of not being peer reviewed was 20 times (95% CI 2.6 to 166.7; p = 0.004) that of studies concluding no such negative impact. All of the studies concluding a negative impact were supported by the tobacco industry. 94% of the tobacco industry supported studies concluded a negative economic impact compared to none of the non-industry supported studies.

Conclusion: All of the best designed studies report no impact or a positive impact of smoke-free restaurant and bar laws on sales or employment. Policymakers can act to protect workers and patrons from the toxins in secondhand smoke confident in rejecting industry claims that there will be an adverse economic impact.

Food security measurement in cultural pluralism : missing the point or conceptual misunderstanding?

Objective : Food Security has become a global concern, yet its measurement has varied considerably across disciplines and countries. We examined the current discrepancies in the definitions of food security and propose a framework for understanding and measuring food security.

Methods : This conceptual review draws from a range of works published in Medline and the gray literature to advance the understanding of food security concepts. We begin by examining the historical background of food security and then move on to examine its various definitions and interpret food through cultural lenses in terms of food access and utilization. We finish by examining various measurements and indicators of food security and reviewing implications for public health.

Results : We argue that the reliance on coping strategies as surrogate measurements of food insecurity without taking into account the social, cultural, and political contexts in which they occur is misleading, and viewing food insecurity solely from a food access or availability perspective, without taking into account food utilization and asset creation as pillars of food security, paints an incomplete picture. Although this review does not claim to provide solutions to the discrepancies in the conceptual definition of food security, it attempts to highlights areas of concern and provide a way forward.

Conclusion : When coping strategies are used as an indicator of food insecurity, they need to be culturally relevant and focus tested, and together with objective measurements of nutritional outcomes, would allow policy makers to make evidence-based decisions to inform social and nutrition policies.

Is adiposity an under-recognized risk factor for tendinopathy? A systematic review

Objective : Tendon injuries have been reported to occur more frequently in individuals with increased adiposity. Treatment also appears to have poorer outcomes among these individuals. Our objective was to examine the extent and consistency of associations between adiposity and tendinopathy.

Methods : A systematic review of observational studies was conducted. Eight electronic databases were searched (Allied and Complementary Medicine, Biological Abstracts, CINAHL, Current Contents, EMBase, Medline, SPORTDiscus, and Web of Science) and citation tracking was performed on included reports. Studies were included if they compared adiposity between subjects with and without tendon injury or examined adiposity as a predictor of conservative treatment success.

Results : Four longitudinal cohorts, 14 cross-sectional studies, 8 case-control studies, and 2 interventional studies (28 in total) met the inclusion criteria, providing a total of 19,949 individuals. Forty-two subpopulations were identified, 18 of which showed elevated adiposity to be associated with tendon injury (43%). Sensitivity analyses indicated a clustering of positive findings among studies that included clinical patients (81% positive) and among case-control studies (77% positive).

Conclusion : Elevated adiposity is frequently associated with tendon injury. Published reports suggest that elevated adiposity is a risk factor for tendon injury, although this association appears to vary depending on aspects of study design and measurement. Adiposity is of particular interest in tendon research because, unlike a number of other reported risk factors for tendon injury, it is somewhat preventable and modifiable. Further research is required to determine if reducing adiposity will reduce the risk of tendon injury or improve the results of treatment.

Cystic fibrosis in children of the eastern Arabian peninsula : a clinical, spatial and genetic study

Aim: The aim of this thesis is to describe the process by which the inherited disease, cystic fibrosis, (CF) was recognised as an important clinical entity in the United Arab Emirates (UAE) and the Sultanate of Oman (Oman). It examines the clinical presentation of the first patients and assesses their degree of severity. Further, it describes the first studies carried out to determine the underlying CF mutations associated with the disease in the UAE and Oman. An estimate is offered of the birth frequency of the condition. Overall, the cultural, geographical and historical aspect of the societies in which the disease occurs is stressed. Methods: An initial literature search was carried out using Medline of any literature pertaining to the Arab World and CF. this was read and classified into the relevance to Arabs in general, the Middle East and then specifically the Arab (Persian) Gulf societies. Thereafter, a clinic was established at Tawam Hospital, Al Ain, UAE, for children presenting With chronic respiratory disease that could serve as a national referral centre. It was run by the Author as a service of the Paediatric Department of the UAE University Medical School. I sent a letter to every Paediatrician working in the UAE informing them of our clinic and offering our services for the diagnosis and management of chronic respiratory disease in children. This was based on the author's experience as a respiratory paediatrician in Australia and New Zealand and as the Professor of Paediatrics in the UAE. No such service then existed in the UAE. Funding was sought to establish a research programme and develop a molecular genetics laboratory in the UAE Medical School. A series of successful research applications provided the grants to commence the investigations. Once a small number of children had been identified as having CF from those referred to the respiratory clinic, the initial project was to assess and report their clinical presentation. Following this an early start was made on the identification of the mutations responsible. Once these were established an attempt was made to estimate the frequency of the condition at birth. Additional clinical studies revolved around assessing the severity of the condition that was associated with the main mutations that were identified. A clinical comparison was made with those with the mutation AF508 and the other main mutation, despite the obvious limitation of small numbers then available. Radiological assessment was made to evaluate the progression of the disease. The final aspect of the study was to assess patients from Oman and compare their findings and mutations with the neighbouring UAE. Based on information gained hypotheses are proposed regarding the spread of the gene mutation by population drift. Thesis outline: A literature review is presented in the form of a critique on the disease and a resume of the relevant aspects of the genetics of CF. Additionally, facts about the two countries' geography and history are presented. Finally, knowledge about CF mutations and population origins from other areas is presented. The second main section deals with the clinical features of the disorder as it presents in the UAE. Molecular findings are then presented and details of the common mutation found in Bedouin Arabs. Hypotheses are then presented based on the information gathered. Results: CF is not a rare disease in the Arab children of the UAE and Oman. These findings refute previous reports of CF being a rare or non-existent disease in Arabs. The condition presents with a severe clinical picture, with early colonisation of the respiratory tract with staphylococcus, haemophilus and pseudomonas organisms, even with conventional CF management practices in place. The CF mutation S549R is prevalent in Arabs of Bedouin stock, while AF508 is found in those of Baluch origin. The former may be descendants of Arabs who left southern Arabia and travelled to the Trucial Coast at the time of the destruction of the great dam at Marib. The origins of this mutation may lie in the area that corresponds to the modern Republic of Yemen. The latter groups are descendants of those who came originally from Baluchistan. It is hypothesised also that the ancestral home of the AF508 mutation may be in the geographical area now known as Baluchistan, that spans three separate modern political territories. The evidence presented supports the concept that the S549R mutation may be associated with a severe, if not the severest, clinical pattern recognised. It equates with that seen with the homozygous AF508 genotype. The absence of an additional mutation in the promoter region accounts for the different clinical pattern seen in previously described patients. Conclusions: There needs to be a major awareness of the presence of CF as a severe clinical disease in the children of the Gulf States. The clinical presentation and findings support the concept of under recognition of the disease. Climatic conditions put the children at special risk of hyponatraemia and electrolyte imbalance. The absence of surviving adults with the disease suggests premature deaths have occurred, but the high fertility rates have maintained the gene pool for this recessive disorder.