Lung MRI and impairment of diaphragmatic function in Pompe disease.

BACKGROUND: Pompe disease is a progressive metabolic myopathy. Involvement of respiratory muscles leads to progressive pulmonary dysfunction, particularly in supine position. Diaphragmatic weakness is considered to be the most important component. Standard spirometry is to some extent indicative but provides too little insight into diaphragmatic dynamics. We used lung MRI to study diaphragmatic and chest-wall movements in Pompe disease. METHODS: In ten adult Pompe patients and six volunteers, we acquired two static spirometer-controlled MRI scans during maximum inspiration and expiration. Images were manually segmented. After normalization for lung size, changes in lung dimensions between inspiration and expiration were used for analysis; normalization was based on the cranial-caudal length ratio (representing vertical diaphragmatic displacement), and the anterior-posterior and left-right length ratios (representing chest-wall movements due to thoracic muscles). RESULTS: We observed striking dysfunction of the diaphragm in Pompe patients; in some patients the diaphragm did not show any displacement. Patients had smaller cranial-caudal length ratios than volunteers (p < 0.001), indicating diaphragmatic weakness. This variable strongly correlated with forced vital capacity in supine position (r = 0.88) and postural drop (r = 0.89). While anterior-posterior length ratios also differed between patients and volunteers (p = 0.04), left-right length ratios did not (p = 0.1). CONCLUSIONS: MRI is an innovative tool to visualize diaphragmatic dynamics in Pompe patients and to study chest-walland diaphragmatic movements in more detail. Our data indicate that diaphragmatic displacement may be severely disturbed in patients with Pompe disease.

Food choice decision-making by women with gestational diabetes.

OBJECTIVE: To enhance the dietary education presented to women with gestational diabetes (GDM) by exploring the reasons and experiences that women with GDM reported in making their food-choice decisions after receipt of dietary education from a healthcare professional. METHODS: Food Choice Map (FCM) semi-structured in-depth interviews were conducted with 30 women with GDM living in the Winnipeg area during their pregnancies. Verbatim transcripts were generated from the interviews. A constant comparative method was used to generate common themes to answer research inquiries. RESULTS: Personal food preferences, hunger and cravings were the main factors affecting food choice decision-making in women with GDM. Although the information from healthcare professionals was 1 factor that affected food choice decision-making for most of the participants, more than half of the women, including all the women who were on insulin, reported difficulties in quick adaptation to dietary management in a limited time period. Information from other sources such as family members, friends, and internet were used to cope with the adaptation. These difficulties led to a sense of decreased control of GDM and were accompanied by frustration, especially for women taking insulin. CONCLUSIONS: Food choice decision-making varied for this group of women with GDM. Knowledge and information aided in making healthy food choices and in portion control. However, balancing individual needs and blood glucose control in a short time period was felt to be difficult and created frustration. The findings suggested that dietary consultation needs to be personalized and to be time sensitive to promote confidence in self-control.

Normal adrenal function in an infant following a pregnancy complicated by maternal adrenal cortical carcinoma and mitotane exposure.

Maternal adrenal cortical carcinoma in pregnancy is rare. We report a case of an infant born to a mother with a history of adrenal cortical carcinoma. The pregnancy was complicated by fetal exposure to mitotane and dexamethasone. Despite the potential teratogenic exposures, there was no evidence of adrenal dysfunction in the infant. Growth and development at 12 months of age are normal and prognosis appears favorable. The long-term impact of fetal exposure to mitotane and glucocorticoid requires further investigation.

Spirometer guided chest imaging in children: It is worth the effort!

PURPOSE: Computed tomography (CT) and magnetic resonance imaging (MRI) scans are used to assess and monitor several pediatric lung diseases. It is well recognized that lung volume at the moment of acquisition has a major impact on the appearance of lung parenchyma and airways. Importantly, the sensitivity of chest CT and MRI to detect bronchiectasis and gas trapping is highly dependent on adequate volume control during the image acquisition. This paper describes a feasible method to obtain accurate control of lung volume during chest imaging in pediatric patients with lung disease. PROCEDURE: A procedure to obtain maximal respiratory manoeuvres with spirometry guidance during image acquisition for CT and MRI is described. This procedure requires training of the subject, an MRI compatible spirometer and close collaboration between a lung function scientist and the radiographer. A good to excellent target volume level for the inspiratory or expiratory scan can be achieved in around 90% of children. An important condition for this success rate is the training of the subject, executed prior to each chest CT or MRI, and instructions by the lung function scientist during the chest CT. CONCLUSION: Implementing lung volume guidance with a spirometer is an important and feasible step to standardize chest imaging and to optimize the diagnostic yield of chest CT and MRI in children with lung disease. Training and the collaborative effort by a lung function scientist and radiographer is the key factor for success of this procedure. Pediatr Pulmonol. 2017;52:48-56. © 2016 Wiley Periodicals, Inc.

Demographic risk factors for fracture in First Nations people.

BACKGROUND: Recently, First Nations people were shown to be at high fracture risk compared with the general population. However, factors contributing to this risk have not been examined. This analysis focusses on geographic area of residence, income level, and diabetes mellitus as possible explanatory variables since they have been implicated in the fracture rates observed in other populations. METHODS: A retrospective, population-based matched cohort study of fracture rates was performed using the Manitoba administrative health data (1987-1999). The First Nations cohort included all Registered First Nations adults (20 years or older) as indicated in either federal and/or provincial files (n = 32,692). Controls (up to three for each First Nations subject) were matched by year of birth, sex and geographic area of residence. After exclusion of unmatched subjects, analysis was based upon 31,557 First Nations subjects and 79,720 controls. RESULTS: Overall and site-specific fracture rates were significantly higher in the First Nations cohort. Income quintile, geographic area of residence, and diabetes were fracture determinants but the excess fracture risk of First Nations ethnicity persisted even after adjustment for these factors. CONCLUSION: First Nations people are at high risk for fracture but the causal factors contributing to this are unclear. Further research is needed to evaluate the importance of other potential explanatory variables.

A randomized comparison of radioiodine doses in Graves' hyperthyroidism.

The optimal method for determining iodine-131 treatment doses for Graves' hyperthyroidism is unknown, and techniques have varied from a fixed dose to more elaborate calculations based upon gland size, iodine uptake, and iodine turnover. Patients with Graves' hyperthyroidism (n = 88) who had not been previously treated with radioactive iodine were randomized to one of four dose calculation methods: low-fixed, 235 MBq; high-fixed, 350 MBq; low-adjusted, 2.96 MBq (80 micro Ci)/g thyroid adjusted for 24 h radioiodine uptake; and high-adjusted, 4.44 MBq (120 micro Ci)/g thyroid adjusted for 24 h radioiodine uptake. Subjects were followed for mean of 63 months (range, 10-94 months) for the following clinical outcomes: euthyroid without medication, hyperthyroid requiring further radioiodine, and hypothyroid requiring life-long L-T(4). Mean treatment doses were similar in the different outcome groups. We could not demonstrate any advantage to using an adjusted dose method. Survival analysis did not demonstrate any difference in the time to outcome between the fixed and adjusted dose methods. The use of a fixed dose method simplifies the approach to treatment with potential cost savings.

Bone mineral density testing in healthy postmenopausal women. The role of clinical risk factor assessment in determining fracture risk.

The ease of measurement and the quantitative nature of bone mineral densitometry (BMD) is clinically appealing. Despite BMD's proven capability to stratify fracture risk, data indicate that clinical risk factors provide complementary information on fracture susceptibility that is independent of BMD. Methods to quantify fracture risk using both clinical and BMD variables would have great appeal for clinical decision-making. We describe a procedure for quantifying hip fracture risk (5-yr and remaining lifetime) based on (1) the individual's age alone (base model, assuming average clinical risk factors and bone density), (2) incorporation of multiple patient-specific clinical risk factor data in the base model, and (3) incorporation of both patient-specific clinical risk factor data and BMD results.

Stress and anxiety in women with gestational diabetes during dietary management.

PURPOSE: To explore the stress and anxiety experiences during dietary management in women with gestational diabetes (GDM). METHODS: Thirty women with GDM from the Winnipeg area participated in the mixed methods study. Each participant completed a Food Choice Map semistructured interview, a Perceived Stress Scale, a Pregnancy Anxiety Scale, a State-Trait Anxiety Inventory-Trait questionnaire, and a demographic questionnaire. Stress and anxiety experiences were identified from interview transcripts and categorized into themes based on the constant comparative method. Questionnaire scores aided in interpreting the stress and anxiety experience in the qualitative data. RESULTS: Three major themes were generated from the interviews: (1) stress related to GDM diagnosis and the perception of a high risk pregnancy; (2) stress over losing control of GDM during the process of dietary management; and (3) anxiety related to the fear of maternal and infant complications. Women on insulin experienced significantly higher levels of perceived stress (P < .01), and the dietary management stress was more prevalent in women using insulin compared to the ones on diet treatment only (Fisher exact test, P < .01). Unhealthy diet coping strategies occurred with the stress and anxiety. CONCLUSIONS: Stress and anxiety were associated with different contexts in this study sample. Women who were on insulin experienced significantly higher levels of perceived stress related to dietary management.

Barriers and coping strategies of women with gestational diabetes to follow dietary advice.

OBJECTIVE: To understand barriers and coping strategies of women with gestational diabetes (GDM) to follow dietary advice. DESIGN: Qualitative study. PARTICIPANTS: Thirty women with GDM from the Winnipeg area participated. Each participant completed a Food Choice Map (FCM) semi-structured interview and a demographic questionnaire. MAJOR OUTCOME MEASURES: Underlying beliefs of women with GDM and factors that hinder following dietary advice. ANALYSIS: Qualitative data analyzed using constant comparative method to identify emergent themes of factors and beliefs that affected following dietary advice. Themes were categorized within the Integrative Model of Behavioral Prediction. RESULTS: GDM women faced challenges and barriers when (1) personal food preference conflicted with dietary advice; (2) eating in different social environments where food choice and portions were out of control and food choice decisions were affected by social norms; (3) lack of knowledge and skills in dietary management and lack of a tailored dietary plan. CONCLUSIONS AND IMPLICATIONS: Quick adaptation to dietary management in a short time period created challenges for women with GDM. Stress and anxiety were reported when women talked about following dietary advice. Tailored educational and mental health consultation with consideration of the barriers may promote dietary compliance and overall better health.

Fracture risk among First Nations people: a retrospective matched cohort study.

BACKGROUND: Canadian First Nations people have unique cultural, socioeconomic and health-related factors that may affect fracture rates. We sought to determine the overall and site-specific fracture rates of First Nations people compared with non-First Nations people. METHODS: We studied fracture rates among First Nations people aged 20 years and older (n = 32 692) using the Manitoba administrative health database (1987-1999). We used federal and provincial sources to identify ethnicity, and we randomly matched each First Nations person with 3 people of the same sex and year of birth who did not meet this definition of First Nations ethnicity (n = 98 076). We used a provincial database of hospital separations and physician billing claims to calculate standardized incidence ratios (SIRs) and 95% confidence intervals (CIs) for each fracture type based on a 5-year age strata. RESULTS: First Nations people had significantly higher rates of any fracture (age- and sex-adjusted SIR 2.23, 95% CI 2.18-2.29). Hip fractures (SIR 1.88, 95% CI 1.61-2.14), wrist fractures (SIR 3.01, 95% CI 2.63-3.42) and spine fractures (SIR 1.93, 95% CI 1.79-2.20) occurred predominantly in older people and women. In contrast, craniofacial fractures (SIR 5.07, 95% CI 4.74-5.42) were predominant in men and younger adults. INTERPRETATION: First Nations people are a previously unidentified group at high risk for fracture.