A Case Report of Ablepharon-Macrostomia Syndrome with Amniotic Membrane Grafting

We describe a rare case of an infant who was born with multiple congenital anomalies, including the absence of eyelids. This patient had many dysmorphic features consistent with a severe phenotype of ablepharon-macrostomia syndrome (AMS) including a fish-like appearance of the mouth, rudimentary ears, absence of body hair, thin skin, absent nipples, abdominal distension, and genital abnormalities. Upon presentation, there was severe exposure keratopathy causing large bilateral sterile ulcers culminating in corneal melting of both eyes. An amniotic membrane graft was used to attempt to maintain the corneal surface integrity. However, because of the late presentation, the corneas could not be salvaged. Extensive surgical reconstruction of both eyelids and bilateral penetrating keratoplasty was ultimately performed successfully to protect the ocular surfaces while trying to maximize the visual potential. Early amniotic membrane grafting may be done at the bedside and may help preserve the ocular in patients with severe eyelid deformities until more definitive treatment is performed.Key Words: Ablepharon, Macrostomia, Congenital eyelid anomalies, Absent hair, Absent eyelids     

Optimizing insulin pump therapy: the potential advantages of using a structured diabetes management program

Use of continuous subcutaneous insulin infusion (CSII) therapy improves glycemic control, reduces hypoglycemia and increases treatment satisfaction in individuals with diabetes. As a number of patient- and clinician-related factors can hinder the effectiveness and optimal usage of CSII therapy, new approaches are needed to address these obstacles.

Ceriello and colleagues recently proposed a model of care that incorporates the collaborative use of structured SMBG into a formal approach to personalized diabetes management within all diabetes populations. We adapted this model for use in CSII-treated patients in order to enable the implementation of a workflow structure that enhances patient–physician communication and supports patients’ diabetes self-management skills.

We recognize that time constraints and current reimbursement policies pose significant challenges to healthcare providers integrating the Personalised Diabetes Management (PDM) process into clinical practice. We believe, however, that the time invested in modifying practice workflow and learning to apply the various steps of the PDM process will be offset by improved workflow and more effective patient consultations. This article describes how to implement PDM into clinical practice as a systematic, standardized process that can optimize CSII therapy.     

Total fluid intake of children and adolescents: cross-sectional surveys in 13 countries worldwide

Purpose

To describe total fluid intake (TFI) according to socio-demographic characteristics in children and adolescents worldwide.

Methods

Data of 3611 children (4–9 years) and 8109 adolescents (10–18 years) were retrieved from 13 cross-sectional surveys (47 % males). In three countries, school classes were randomly recruited with stratified cluster sampling design. In the other countries, participants were randomly recruited based on a quota method. TFI (drinking water and beverages of all kinds) was obtained with a fluid-specific record over 7 consecutive days. Adequacy was assessed by comparing TFI to 80 % of adequate intake (AI) for total water intake set by European Food Safety Authority. Data on height, weight and socio-economic level were collected in most countries.

Results

The mean (SD) TFI ranged from [1.32 (0.68)] to [1.35 (0.71)] L/day. Non-adherence to AIs for fluids ranged from 10 % (Uruguay) to >90 % (Belgium). Females were more likely to meet the AIs for fluids than males (4–9 years: 28 %, OR 0.72, p = 0.002; 10–18 years: 20 %, OR 0.80, p = 0.001), while adolescents were less likely to meet the AI than children (OR 1.645, p < 0.001 in males and OR 1.625, p < 0.001 in females).

Conclusions

A high proportion of children and adolescents are at risk of an inadequate fluid intake. This risk is especially high in males and adolescents when compared with females or children categories. This highlights water intake among young populations as an issue of global concern.

     

Echocardiographic evaluation of pulmonary hypertension,right ventricular function,and right ventricular-pulmonary arterial coupling in patients with rheumatoid arthritis

Clinical Rheumatology - Rheumatoid arthritis (RA) patients are at increased risk for developing cardiovascular disease, including right heart failure. The evaluation of right ventricle (RV) using...     

Determinants of ST-segment elevation myocardial infarction as clinical presentation of acute coronary syndrome

Journal of Thrombosis and Thrombolysis - Antiplatelet agents and statin therapies are widely used in patients with known cardiovascular disease. Plaque rupture (PR) and plaque erosion (PE) are the...     

Low Quality of Life,Falls, and Pre-Frailty are Associated with Depressive Symptoms in Virologically Suppressed PLWHIV in Salvador,Brazil

AIDS and Behavior - Depression is the leading cause of years lived with disability worldwide and PLWHIV present a higher risk of developing depressive symptoms. We aimed to evaluate depressive...     

A 5-year follow-up of ambulatory blood pressure in healthy older adults

BACKGROUND: This study assessed 5-year changes in ambulatory blood pressure (ABP) in healthy, older individuals and determined the extent to which it could be predicted from earlier BP measures and other cardiovascular risk factors. METHODS: A total of 162 men and women, aged 55 to 79 years, with no prior medical disorders, completed a medical examination and two 24-h ABP sessions. The procedures were repeated 5 years later in 80% (130) of these subjects. A modified hierarchical regression analysis was used to determine whether initial ABP and casual blood pressure (CBP) measures and demographic and physical examination data could predict ABP in 5 years. RESULTS: The CBP and most ABP levels during waking and sleep increased after 5 years. However, CBP remained in the normotensive range for 73% of the subjects. The ABP variability tended to decrease over time. The ABP and CBP measures accounted for at least 50% of the variance in the prediction of ABP level after 5 years. In comparison, the predictability of ABP variability was quite low, particularly during sleep (<30% of the variance accounted for). CONCLUSIONS: The ABP and CBP were good predictors of future ABP level in healthy older subjects, but ABP variability was more difficult to predict. Except for age, none of the standard cardiovascular risk factors contributed significantly to the prediction of ABP level or variability.