Charles Mueller

Assessment of bone mineral density in adults and children with Marfan syndrome

Recent studies indicate that decreased bone mineral density (BMD) occurs in the spine, femoral necks and greater trochanters of some adults and children with Marfan syndrome. Because there is uncertainty regarding the BMD status of patients with Marfan syndrome, we undertook an analysis of BMD in both adults and children with Marfan syndrome. Dual energy X-ray absorptiometry analysis was performed on a convenience sample of 51 patients (30 adults and 21 children) with diagnosed Marfan syndrome from 1993 to 2000. T-Scores (i.e. the number of standard deviations above or below the average normal peak bone density) were determined for comparison of adults. Mean±SD of individual BMD values were used for comparison of the data of children. Compared to standard values obtained from normal adult patients, adult males with Marfan syndrome demonstrated significantly reduced femoral neck BMD with an average T-score of -1.54 (P<0.001), diagnostic of osteopenia. Although osteopenia and osteoporosis were observed in several middle aged and pre- and postmenopausal women, the average T-score value for adult females and children were within normal limits. The etiology and full significance of decreased BMD in adult male patients with Marfan syndrome remain uncertain at the present time. Our results lead us to question the value of aggressive BMD evaluations by DXA in these patients, particularly prior to reaching mid-age. Further investigations will be required to shed insights into the natural history of BMD in adults and children with Marfan syndrome. Any application of bone mineral replacement therapy such as bisphosphonate, selective estrogen receptor modulators, hormone replacement therapy and vitamin D in these patients may be premature based on the existing evidence.

Bone Mineral Density Determinations by Dual-Energy x-ray Absorptiometry in the Management of Patients with Marfan Syndrome—Some Factors Which Affect the Measurement

Reduced bone mineral density (BMD) was sporadically reported in patients with Marfan syndrome. This may or may not place the Marfan patient at increased risk for bone fracture. In comparing the BMDs of our patients with those reported in the literature, it seemed that agreement between values, and hence the degree of osteoporosis or osteopenia reported, was dependent on the instrumentation used. The objective of this study was to statistically assess this impression. Bone mineral density measurements from our previously published study of 30 adults with Marfan syndrome performed on a Lunar DPXL machine were compared with studies published between 1993–2000 measured using either Lunar or Hologic bone densitometry instruments. The differences of our measurements compared with those made on other Lunar machines were not statistically significant, but did differ significantly with published results from Hologic machines (P < 0.001). Before progress can be made in the assessment of BMD and fracture risk in Marfan patients and in the evidence-based orthopedic management of these patients, standardization of instrumental bone density determinations will be required along with considerations of height, obesity, age, and sex.

An after-school dance and lifestyle education program reduces risk factors for heart disease and diabetes in elementary school children

Abstract Background: Forty-three percent of New York City’s (NYC) school-age children are overweight or obese, placing them at risk for heart disease and type 2 diabetes mellitus (T2DM). Objective: The objective of this study was to determine if an intensive after-school dance and lifestyle education program would reduce risk factors for heart disease, T2DM, and improve lifestyle choices. Subjects: Subject include 64 fourth- and fifth-grade students at an elementary school in NYC. Methods: Students received freestyle dance and lifestyle classes for 16 weeks and were evaluated for changes in body composition, endurance, biochemical measurements, and lifestyle choices. Results: Significant improvements in BMI percentiles were found among children in the overweight and obese categories as well as in endurance and biochemical measurements that reflect heart disease and diabetes risk. Improvement was also reported in lifestyle choices. Conclusion: An intensive after-school dance and lifestyle education program can reduce risk factors for heart disease and T2DM and improve lifestyle choices among elementary school children.

Regulation of Medical Foods: Toward a Rational Policy

Medical foods are enterally administered formulas used as complete or supplemental nutrition support in the treatment of diseases and clinical disorders of hospitalized or incapacitated adult patients. Because clinical trials have failed to demonstrate a consistent benefit of these products on morbidity and mortality rates, it has been difficult to reach consensus on their primary role. Are medical foods components of supportive care or should they be considered pharmacologic treatment? To date, the US Congress has defined medical foods, but the Food and Drug Administration has not developed regulatory statutes for them beyond those designed to ensure the sanitary manufacture of all processed foods. Before effective regulations can be developed, medical foods need to be defined further in order to distinguish them from each other and from other classes of nutritional products such as parenteral nutrients or infant formulas. Because we believe that the role of medical foods is in supportive care rather than pharmacologic therapy, we argue that these products should be subject to regulations similar to those that apply to conventional foods.

Parenteral Nutrition Support of a Patient With Chronic Mesenteric Artery Occlusive Disease

Vascular catastrophe resulting in a bowel infarction requiring massive resection is one of the most common indications for long-term total parenteral nutrition (TPN). The causes of mesenteric artery disease include embolic and thrombotic occlusions, nonocclusive mesenteric ischemia, and chronic mesenteric ischemia. This paper describes a case of a patient with chronic ischemia. The indication for TPN was intestinal angina limiting oral intake, not short-bowel syndrome as a result of bowel infarction and surgery. The patient had an extensive history of atherosclerotic disease and abdominal symptoms. Her nutritional status was maintained with TPN and oral intake as symptomatically tolerated. She eventually developed catheter sepsis. Her cardiopulmonary status deteriorated and she died. Progressive mesenteric ischemia and possible infarction may have contributed to her death. The patient had indicated she did not want surgery for a bowel infarction. She did consent to surgical correction of her disease, if feasible. Although TPN can maintain the nutritional and metabolic status of a patient with chronic mesenteric ischemia, the associated risk of catheter sepsis emphasizes the necessity for expedient treatment of the primary pathology.

Report from the Advanced-Level Clinical Practice Audit Task Force of the Commission on Dietetic Registration: Results of the 2013 Advanced-Level Clinical Practice Audit

H ISTORICALLY,THECOMMISSION on Dietetic Registration (CDR) and the Academy of Nutrition and Dietetics (Academy) have used consensus-based definitions of advanced-level practice (ALP) in nutrition and dietetics. Prior research by Bradley and colleagues, Skipper and Lewis, and Brody and colleauges defined attributes of ALP registered dietitian nutritionists (RDNs) in general, medical nutrition therapy, and clinical nutrition and dietetics practice. The Academy broadly defines the ALP RDN as one who “demonstrates a high level of skills, knowledge, and behaviors. The individual exhibits a set of characteristics that include leadership and vision and demonstrates effectiveness in planning, evaluating, and communicating targeted outcomes.” Yet, delineation of job functions representative of ALP has been difficult to elucidate, either generally or in focused areas of practice. From 2005 through 2007, CDR conducted a practice audit to identify and delineate ALP in nutrition and dietetics and to examine theneed for anadvanced practice credential. A common core of advanced practice tasks representing all practice segments was indiscernible from these data. Study conclusions suggested focused practice areas including clinical nutrition, community nutrition, management, business, or education/research were likely to have unique ALP characteristics and practice tasks, justifying the need for future studies to concentrate on only one practice area. Thus, in 2011, the CDR commissioned a second Task Force, the AdvancedLevel Clinical Practice Audit Task Force, to conduct a practice audit in clinical nutrition practice and, if feasible, developan advancedpractice credential for RDNs in clinical practice. Clinical nutrition was selected as the specific practice segment because it represents the segment in which the largest proportionofRDNspractice. In “Developing an Advanced Practice Credential for Registered Dietitian Nutritionists in Clinical Nutrition Practice,” Brody and colleagues describe the charge of the Task Force, the definition of clinical nutrition and dietetics practice, and the process used to explore, define, and design the new certification program recognizing advanced clinical nutrition practice. An important step in that process, once the basic concept and potential value of the certification program had been provisionally proven in the market, was to discover what work those currently practicing in clinical nutrition at an advanced level actually do—the work that sets them apart from entrylevel and beyond-entry-level (BEL) RDNs. Defining the credential in terms of the tasks performed by actual ALP RDNs provides the fundamental justification for certification and credentialing, namely to protect the public by warranting that credentialed practitioners are able to perform their work in a safe and effective manner. Task identification is also a requirement for developing a valid, fair, and legally defensible credentialing exam. According to the National Commission for Certifying Agencies:

Characterization of Insulin Resistance Syndrome in Children and Young Adults. When to Screen for Prediabetes

CONTEXT Insulin resistance syndrome (IRS) is associated with the development of type 2 diabetes mellitus (DM2). However, it is unclear which individuals with insulin resistance will develop DM2. AIM To study the prevalence of IRS in childhood and to identify the group with the highest risk of further progression to DM2. METHODS In a cross-sectional study, 86 obese individuals underwent an intravenous glucose tolerance test (IVGTT). Insulin resistance index (Si(IVGTT)), acute insulin response (AIR) and disposition index (DI) were calculated from IVGTT. RESULTS For analysis the participants were divided into insulin-sensitive (IS) (n = 25, 13.3 +/- 5.9 yr) and insulin-resistant (IR) groups on the basis of having an Si(IVGTT) greater or lesser than 4.5 x 10(-4) mU/ml/min, respectively. The IR group was then subdivided according to DI, with the standard cut-off value of 0.13 min(-1), into compensated IR (CIR) (n = 37, 13.0 +/- 3.5 yr) and decompensated IR (DIR) (n = 24, 21.9 +/- 12.6 yr) groups. The frequency of IRS was 43% in children, 78% in adolescents and 83.6% in adults. Decompensated insulin response first appeared during adolescence. The frequency of decompensation increased from 22% in adolescence to 67% in adulthood. The DIR group had increased triglycerides (TG) and urinary free cortisol levels. CONCLUSIONS The frequency and severity of IR increases with age. Decompensation first presents in adolescence with low AIR and elevated TG. Decompensated adolescents are the group at highest risk for further progression to DM2.

The 2010 Commission on Dietetic Registration Entry-Level Dietetics Practice Audit: Distinguishing between Educational Attributes

O d g b a w w m e a t a g d The 2010 Entry-Level Dietetics Practice Audit determined the nature of entry-level practice for egistered dietitians (RDs) and ditetic technicians, registered (DTRs). he primary goal of the audit was to rovide the profession with quantitaive insight about the level and freuency of involvement and perceived isk associated with activity stateents of entry-level RDs (EL RDs) nd DTRs (EL DTRs) in the first 3 ears of practice. The Commission on ietetic Registration uses the audit esults to establish RD and DTR xam content domains while the ommission on Accreditation on Ditetics Education assesses the audit utcomes and accreditation stanards for educators. This report sumarizes the original methods used nd distinguishes between character-

Carbohydrate- vs Fat-Controlled Diet Effect on Weight Loss and Coronary Artery Disease Risk A Pilot Feeding Study

This pilot study compared weight loss and serum indicators of coronary artery disease (CAD) risk between 2 weight loss (energy-deficit) diets, one controlled for carbohydrate as a percentage of total calories and the other controlled for fat as percentage of total calories. Participants were randomized to 1 of 2 diets and fed on an outpatient basis for 70 days, after which they followed their diets using their own resources for an additional 70 days. Energy deficit for the diets was determined by indirect calorimetry with a 500- to 750-calorie per day adjustment. Weight and CAD risk indicators and serum lipid and C-reactive protein levels were measured at baseline, day 70, and day 140. The study was completed by 16 of 20 participants who were able to comply with the feeding portion of the study as well as with follow-up appointments during the second (self-management) period of the study. Participants lost weight in both diet groups (24.4 lbs, carbohydrate controlled; 18.5 lbs, fat controlled), and serum CAD risk factors decreased in both groups. There were no significant differences in CAD risk factors between diet groups, although there was a trend toward lighter low-density lipoprotein (LDL) size in the carbohydrate-controlled group. During the self-management portion of the study, weight loss stalled or regained from loss during the previous feeding period. The results, although underpowered, are consistent with recent studies in which macronutrient ratio of total calories in diet did not affect degree of weight loss and in which carbohydrate-controlled diets produced a predominance of lighter LDLs.

Inflammation, Old Age, and Nutrition Assessment

Nutrition assessment is a comprehensive evaluation of nutritional status that uses medical, nutritional, and medication histories, physical examination, anthropometric measurements, and laboratory data. Inflammatory metabolism both acute and chronic affects a number of physical, anthropometric, and laboratory measurements used in the assessment process. Chronic diseases associated with old age and perhaps aging itself produce chronic inflammatory alterations that affect immunity, hepatic protein metabolism, lean body mass homeostasis, and fluid compartment shifts that obfuscate the ability of clinicians to evaluate nutritional status and monitor the outcomes of nutritional interventions. Additional problems unique to the elderly include sarcopenia and frailty, perhaps related to inflammatory alterations, which also cause anthropometric abnormalities and likewise are difficult to integrate with nutrition assessment and monitoring. The most useful assessment methodologies in the elderly are those that focus on recent weight changes and the functional capacity of an individual to achieve or maintain nutritional adequacy. Activities, including instrumental activities, of daily living are an excellent tool to evaluate the functional capacity of an elderly individual. Minimum Data Set accomplishes this as part of a comprehensive assessment of clinical status in long-term care facilities in the United States. In Europe, the Mini Nutrition Assessment has been validated as the optimal tool to evaluate nutritional status in the elderly and has been suggested as a useful tool to use in settings in the United States where the Minimum Data Set is not used.