Orthodontic Treatment and Long-Term Management of a Patient with Marfan Syndrome

2017 ◽  
Vol 54 (3) ◽  
pp. 358-367 ◽  
Author(s):  
Momotoshi Shiga ◽  
Takuya Ogawa ◽  
Issareeya Ekprachayakoon ◽  
Keiji Moriyama
Keyword(s):  
Marfan Syndrome ◽  
Healthy Subjects ◽  
Orthodontic Treatment ◽  
Tooth Movement ◽  
Clinical Manifestations ◽  
Facial Growth ◽  
Increased Risk ◽  
Term Management ◽  
Over Time

Marfan syndrome (MFS) is caused by abnormal systemic connective tissue. The main clinical manifestations include long limbs, long slender fingers, lens subluxation, abnormal cardiac valves, and aortic aneurysm. We report the case of an 11-year-old patient with MFS who underwent orthodontic treatment and was followed up until the age of 25 years. We found no significant differences in tooth movement between the patient with MFS and healthy subjects. However, because patients with MFS show characteristic facial growth and an increased risk of developing systemic comorbidities, their dental status requires careful observation over time.

Protected Areas: offering security to whom, when and where?

2016 ◽  
Vol 43 (2) ◽  
pp. 172-180 ◽  
Author(s):  
ALICE B. KELLY ◽  
A. CLARE GUPTA
Keyword(s):  
Protected Areas ◽  
Environmental Conditions ◽  
Protected Area ◽  
Spatial Scales ◽  
Local Population ◽  
Well Being ◽  
Economic Context ◽  
Term Management ◽  
Over Time

SUMMARYThis study considers the issue of security in the context of protected areas in Cameroon and Botswana. Though the literature on issues of security and well-being in relation to protected areas is extensive, there has been less discussion of how and in what ways these impacts and relationships can change over time, vary with space and differ across spatial scales. Looking at two very different historical trajectories, this study considers the heterogeneity of the security landscapes created by Waza and Chobe protected areas over time and space. This study finds that conservation measures that various subsets of the local population once considered to be ‘bad’ (e.g. violent, exclusionary protected area creation) may be construed as ‘good’ at different historical moments and geographical areas. Similarly, complacency or resignation to the presence of a park can be reversed by changing environmental conditions. Changes in the ways security (material and otherwise) has fluctuated within these two protected areas has implications for the long-term management and funding strategies of newly created and already existing protected areas today. This study suggests that parks must be adaptively managed not only for changing ecological conditions, but also for shifts in a protected area's social, political and economic context.

Abstract 10756: Chronic Kidney Disease in Long-term Survivors After Fontan Palliation

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Sheena Sharma ◽  
Rebecca L Ruebner ◽  
Susan L Furth ◽  
Kathryn M Dodds ◽  
Jack Rychik ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Kidney Function ◽  
Healthy Subjects ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Long Term Survivors ◽  
Fontan Palliation ◽  
Over Time

Background: The Fontan operation is a palliative procedure for children with congenital single ventricle heart disease. With advances in prenatal diagnosis and surgical techniques, more children are surviving into adulthood with unique cardiovascular physiology. Little is currently known about long-term kidney function in these patients. Hypothesis: We hypothesize that long-term survivors after Fontan palliation will have a higher prevalence of chronic kidney disease (CKD) compared to healthy controls. Methods: We performed a retrospective cohort study of subjects evaluated through the Single Ventricle Survivorship Program (SVSP) at the Children’s Hospital of Philadelphia between July 1, 2010 and December 5, 2014 and healthy children similar in sex and age. The primary outcome was CKD, defined as eGFR <90 ml/min/1.73m2 calculated with age-appropriate estimating equations using creatinine and cystatin C. Secondary outcomes included proteinuria and hyperparathyroidism. Results: The Fontan cohort included 68 subjects with mean age of 13.9 years (SD 5.8) at SVSP visit who were 11.2 years (SD 5.7) from Fontan operation. The healthy cohort included 70 patients with mean age of 15.9 years (SD 3.9). Mean eGFR was 102.6 versus 101.9ml/min/1.73m2 (p=0.89) in pediatric Fontan versus healthy subjects using the complete CKiD equation, and 128.5 versus 129.7ml/min/1.73m2 (p=0.56) in adult Fontan versus healthy subjects using the CKD-EPI creatinine and cystatin formula. 10% of Fontan subjects had an eGFR<90 ml/min/1.73m2. Mean intact parathyroid hormone level was higher at 68.0pg/mL (SD 35.4) in the Fontan group compared to 26.0pg/mL (SD 13.6) in the healthy group. Proteinuria was found within 34% of the Fontan group compared to 4.6% within the control group. Conclusion: We found that 10% of subjects have eGFR <90ml/min/1.73m2 after Fontan palliation which would indicate CKD if this remained persistent over time. Although the majority of the cohort had normal kidney function by eGFR, we found a higher proportion with proteinuria and increased parathyroid hormone levels which may indicate early kidney disease. Future studies will focus on evaluating changes in kidney function over time in long-term survivors after Fontan palliation.

scholarly journals Expert consensus and evidence-based recommendations for the assessment of flow-mediated dilation in humans

2019 ◽  
Vol 40 (30) ◽  
pp. 2534-2547 ◽  
Author(s):  
Dick H J Thijssen ◽  
Rosa Maria Bruno ◽  
Anke C C M van Mil ◽  
Sophie M Holder ◽  
Francesco Faita ◽  
...  
Keyword(s):  
Endothelial Function ◽  
Clinical Symptoms ◽  
Clinical Manifestations ◽  
Arterial Disease ◽  
Expert Consensus ◽  
Flow Mediated Dilation ◽  
Pharmacological Interventions ◽  
Increased Risk ◽  
Long Term Impact

Abstract Endothelial dysfunction is involved in the development of atherosclerosis, which precedes asymptomatic structural vascular alterations as well as clinical manifestations of cardiovascular disease (CVD). Endothelial function can be assessed non-invasively using the flow-mediated dilation (FMD) technique. Flow-mediated dilation represents an endothelium-dependent, largely nitric oxide (NO)-mediated dilatation of conduit arteries in response to an imposed increase in blood flow and shear stress. Flow-mediated dilation is affected by cardiovascular (CV) risk factors, relates to coronary artery endothelial function, and independently predicts CVD outcome. Accordingly, FMD is a tool for examining the pathophysiology of CVD and possibly identifying subjects at increased risk for future CV events. Moreover, it has merit in examining the acute and long-term impact of physiological and pharmacological interventions in humans. Despite concerns about its reproducibility, the available evidence shows that highly reliable FMD measurements can be achieved when specialized laboratories follow standardized protocols. For this purpose, updated expert consensus guidelines for the performance of FMD are presented, which are based on critical appraisal of novel technical approaches, development of analysis software, and studies exploring the physiological principles underlying the technique. Uniformity in FMD performance will (i) improve comparability between studies, (ii) contribute to construction of reference values, and (iii) offer an easy accessible and early marker of atherosclerosis that could complement clinical symptoms of structural arterial disease and facilitate early diagnosis and prediction of CVD outcomes.

scholarly journals Risk of Venous Thromboembolism after New Onset Heart Failure

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Nathaniel R. Smilowitz ◽  
Qi Zhao ◽  
Li Wang ◽  
Sulena Shrestha ◽  
Onur Baser ◽  
...  
Keyword(s):  
Heart Failure ◽  
Venous Thromboembolism ◽  
Cardiovascular Morbidity And Mortality ◽  
Increased Risk ◽  
New Diagnosis ◽  
Over Time ◽  
Long Term Mortality ◽  
New Onset

AbstractNew-onset heart failure (HF) is associated with cardiovascular morbidity and mortality. It is uncertain to what extent HF confers an increased risk of venous thromboembolism (VTE). Adults ≥65 years old hospitalized with a new diagnosis of HF were identified from Medicare claims from 2007–2013. We identified the incidence, predictors and outcomes of VTE in HF. We compared VTE incidence during follow-up after HF hospitalization with a corresponding period 1-year prior to the HF diagnosis. Among 207,535 patients with a new HF diagnosis, the cumulative incidence of VTE was 1.4%, 2.5%, and 10.5% at 30 days, 1 year, and 5 years, respectively. The odds of VTE were greatest immediately after new-onset HF and steadily declined over time (OR 2.2 [95% CI 2.0–2.3], OR 1.5 [1.4–1.7], and OR 1.2 [1.2–1.3] at 0–30 days, 4–6 months, and 7–9 months, respectively). Over 26-month follow-up, patients with HF were at two-fold higher risk of VTE than patients without HF (adjusted HR 2.31 [2.18–2.45]). VTE during follow-up was associated with long-term mortality (adjusted HR 1.60, 95% CI 1.56–1.64). In conclusion, patients with HF are at increased risk of VTE early after a new HF diagnosis. VTE in patients with HF is associated with long-term mortality.

Peak Impact Accelerations during Track and Treadmill Running

2013 ◽  
Vol 29 (5) ◽  
pp. 639-644 ◽  
Author(s):  
Erin M.R. Bigelow ◽  
Niell G. Elvin ◽  
Alex A. Elvin ◽  
Steven P. Arnoczky
Keyword(s):  
Healthy Subjects ◽  
Treadmill Running ◽  
Acrylic Plate ◽  
Custom Made ◽  
The Individual ◽  
Long Term Impact ◽  
Wireless Accelerometer ◽  
Vertical Impact ◽  
Over Time

To determine whether peak vertical and horizontal impact accelerations were different while running on a track or on a treadmill, 12 healthy subjects (average age 32.8 ± 9.8 y), were fitted with a novel, wireless accelerometer capable of recording triaxial acceleration over time. The accelerometer was attached to a custom-made acrylic plate and secured at the level of the L5 vertebra via a tight fitting triathlon belt. Each subject ran 4 miles on a synthetic, indoor track at a self-selected pace and accelerations were recorded on three perpendicular axes. Seven days later, the subjects ran 4 miles on a treadmill set at the individual runner’s average pace on the track and the peak vertical and horizontal impact magnitudes between the track and treadmill were compared. There was no difference (P= .52) in the average peak vertical impact accelerations between the track and treadmill over the 4 mile run. However, peak horizontal impact accelerations were greater (P= .0012) on the track when compared with the treadmill. This study demonstrated the feasibility for long-term impact accelerations monitoring using a novel wireless accelerometer.

scholarly journals Gastrointestinal Manifestations of Marfan Syndrome

2017 ◽  
Author(s):  
Nashiz Inayet ◽  
Jamal Hayat ◽  
Andrew Poullis ◽  
Maite Tome ◽  
Ann Child
Keyword(s):  
Connective Tissue ◽  
Marfan Syndrome ◽  
Medical Literature ◽  
Review Article ◽  
Connective Tissue Disorders ◽  
Gastrointestinal Complications ◽  
Gastrointestinal Manifestations ◽  
Gastrointestinal Pathology ◽  
Term Management

Symptoms attributed to the gastrointestinal manifestations of multi-system disorders play an important role in the long-term management of these conditions. Gastrointestinal complications of a variety of connective tissue disorders have been studied and there is an increased interest in the incidence and prevalence of these symptoms. Descriptions of the occurrence of gastrointestinal complications in Marfan syndrome have appeared infrequently in the medical literature. In this review article we focus on both structural and functional gastrointestinal pathology that may occur in patients with Marfan syndrome.

scholarly journals Informal caregiving patterns and trajectories of psychological distress in the UK Household Longitudinal Study

2018 ◽  
Vol 49 (10) ◽  
pp. 1652-1660 ◽  
Author(s):  
Rebecca E. Lacey ◽  
Anne McMunn ◽  
Elizabeth Webb
Keyword(s):  
Psychological Distress ◽  
Longitudinal Study ◽  
Regression Coefficient ◽  
Informal Caregivers ◽  
Informal Caregiving ◽  
Representative Study ◽  
Increased Risk ◽  
The Uk ◽  
Over Time

AbstractBackgroundApproximately seven million people in the UK are engaged in informal caregiving. Informal caregivers are at risk of poorer mental and physical health. However, less is known about how the relationship between the informal caregiving and psychological distress changes over time. The aim of this study was to investigate longitudinal associations between the informal caregiving and psychological distress amongst UK men and women aged 16+.MethodsData were analysed from the UK Household Longitudinal Study (UKHLS, n = 9368), a nationally representative study of UK households. Longitudinal linear mixed modelling was used to estimate associations between the longitudinal patterns of informal caregiving (non-caregiver/one episode of 1–2 years/intermittent caregiving/3+ years caregiving) and trajectories of psychological distress across seven waves of UKHLS data.ResultsInformal caregiving was not associated with psychological distress for men. Women engaged in long-term (⩾3 years) or intermittent caregiving had higher levels of psychological distress at the point of initiation, compared with women who were not caregivers throughout the study period (3+ years caregiver: regression coefficient 0.48, 95% confidence interval (CI) 0.07–0.89; intermittent caregiver: regression coefficient 0.47, 95% CI 0.02–0.92). Trajectories of psychological distress changed little over time, suggesting a plateau effect for these caregiving women.ConclusionsWomen engaged in long-term or repeated shorter episodes of informal caregiving reported more symptoms of psychological distress than non-caregiving women. Given the increased risk of reporting psychological distress and the increasing importance of the informal care sector, the risk of poorer mental health of informal caregivers should be a priority for public health.

scholarly journals Anterior hypopituitarism secondary to biopsy-proven IgG4-related hypophysitis in a young man

2019 ◽  
Vol 2019 ◽  
Author(s):  
H Joshi ◽  
M Hikmat ◽  
A P Devadass ◽  
S O Oyibo ◽  
S V Sagi
Keyword(s):  
Clinical Manifestations ◽  
Pituitary Hormone ◽  
List Type ◽  
Igg4 Related Disease ◽  
Anterior Pituitary Hormone ◽  
Immune Mediated ◽  
Learning Points ◽  
Term Management

Summary IgG4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory condition which can affect various organs including the pituitary gland. The true annual incidence of this condition remains widely unknown. In addition, it is unclear whether IgG4 antibodies are causative or the end result of a trigger. With no specific biomarkers available, the diagnosis of IgG4-related hypophysitis remains a challenge. Additionally, there is a wide differential diagnosis. We report a case of biopsy-proven IgG4-related hypophysitis in a young man with type 2 diabetes mellitus. Learning points: IgG4-related hypophysitis is part of a spectrum of IgG4-related diseases. Clinical manifestations result from anterior pituitary hormone deficiencies with or without diabetes insipidus, which can be temporary or permanent. A combination of clinical, radiological, serological and histological evidence with careful interpretation is required to make the diagnosis. Tissue biopsy remains the gold standard investigation. Disease monitoring and long-term management of this condition is a challenge as relapses occur frequently.

Persistent triple antiphospholipid antibody positivity as a strong risk factor of first thrombosis, in a long-term follow-up study of patients without history of thrombosis or obstetrical morbidity

Lupus ◽  
2016 ◽  
Vol 26 (2) ◽  
pp. 163-169 ◽  
Author(s):  
C M Yelnik ◽  
G Urbanski ◽  
E Drumez ◽  
V Sobanski ◽  
H Maillard ◽  
...  
Keyword(s):  
Antiphospholipid Antibody ◽  
Increased Risk ◽  
Long Term Follow Up ◽  
Antibody Positivity ◽  
History Of ◽  
Thrombosis Rate ◽  
Over Time ◽  
Aspirin Prophylaxis

Introduction The long-term risk of first thrombosis and benefit of prophylaxis in antiphospholipid antibody (aPL) carriers without history of thrombosis or obstetrical morbidity is poorly known. This study aimed to evaluate the long-term rate and risk factors associated with a first thrombosis in those patients. Patients and methods After a prior study ended in December 2005 and was already published, we extended the follow-up period of our cohort of aPL carriers. Results Ninety-eight of the 103 patients of the previous study were included. The annual first thrombosis rate was 2.3% per patient-year during a median of 13 years (6–17). None of the baseline characteristics was predictive of risk of first thrombosis, but persistent aPL over time were associated with an increased risk. The stronger association was found in triple aPL-positive carriers: OR 3.38 (95% CI: 1.24–9.22). Of note, conversely to our previous findings, no benefit of aspirin prophylaxis was observed. Conclusion The risk of first thrombosis in aPL carriers without history of thrombosis or obstetrical morbidity was significant, persisted linearly over time and was associated with persistent aPL. This risk was especially increased in triple aPL-positive carriers, in whom a close follow-up seems to be necessary. Nevertheless, the benefit of aspirin prophylaxis remained unclear.

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