Determinants of vitamin D supplementation prescription in nursing homes: a survey among general practitioners.

SUMMARY: A total of 119 GPs participated to a survey aimed to assess the profile and determinants of vitamin D supplementation prescription in nursing homes. Among the respondent GPs, 65 (54.6%) systematically prescribe vitamin D to their institutionalized patients and the 54 (45.4%) others prescribe only sometimes. INTRODUCTION: The aim of this study is to assess the profile and determinants of vitamin D supplementation prescription in nursing homes. METHODS: General practitioners (GPs) having at least one patient in a nursing home in Liège, Belgium, were asked to complete the survey. RESULTS: A total of 119 GPs participated in the survey. Among the respondent GPs, 65 (54.6 %) systematically prescribe vitamin D to their institutionalized patients and the 54 (45.4%) others prescribe only sometimes. The main reasons for prescribing vitamin D cited by GPs who do so systematically are as follows: because they believe nursing home residents are mostly deficient in vitamin D status (92.1%), because they believe that vitamin D supplementation prevents osteoporotic fractures (77.8%), and because vitamin D supplementation is recommended by various scientific societies (38.1%). GPs who only prescribe vitamin D supplementation in some patients mainly do so following a diagnosis of osteoporosis (82.4%), on the basis the 25(OH)D level (78.4%), in the case of history of fracture (54.9%) or after a recent fracture (43.4%). Surprisingly, 16 physicians (31.4%) only prescribe vitamin D when they think of it. Interestingly, while 40.7% of GPs always prescribe the same dose of vitamin D, the remaining 59.3% prescribe a dose that will mainly depend on the results of the 25(OH)D level (94.0%), the patient's bone health (49.3%), or history of fracture (43.3%). CONCLUSIONS: More than half of GPs systematically prescribe vitamin D to their patients living in nursing homes. The other GPs usually prescribe vitamin D following the result of the vitamin D status or after a diagnosis of osteoporosis.

Gait speed or gait variability, which one to use as a marker of risk to develop Alzheimer disease? A pilot study.

BACKGROUND: Previous literature demonstrates the interest of gait analysis to predict cognitive decline in old people. AIMS: This pilot study aims to determine if gait speed or gait variability is a marker able to early identify, among mild cognitive impairment (MCI) subjects, those at risk to develop Alzheimer's disease (AD) in the future. METHODS: 13 MCI subjects were included in 2007. Their gait parameters (walking speed, stride length and gait frequency, regularity and symmetry) were measured in 2007 and 2008 in simple task (ST) and in dual task (DT) using a triaxial accelerometer (Locometrix(®)). Among the 13 MCI subjects included in 2007, 10 were assessed in 2008. So, 23 (13 in 2007 + 10 in 2008) gait tests were collected. In 2011, MCI people were considered as "MCI+" when they developed AD (between baseline and 2011) and as "MCI-" if they did not. Among the 23 gait tests, 15 were from MCI+ (9 gait tests in 2007 and 6 in 2008) and 8 from MCI- (4 gait tests in 2007 and 4 gait tests in 2008). Mann-Whitney non-parametric U test was used to compare gait parameters of MCI+ and MCI-. RESULTS: Gait speed, symmetry and regularity were lower in MCI+ than in MCI-. DISCUSSION: Despite the small sample size, the results presented in this original pilot study are in line as the infrequent previous literature related to this topic. The authors discuss lacks and strengths of this work. CONCLUSIONS: These results suggest that both gait speed and gait variability could be markers to early identify MCI at risk to develop AD.

[Oxidative stress, antioxydants and the ageing process]. / Quelle place pour le stress oxydant et les antioxydants dans le processus du vieillissement?

Antioxidant supplementation in the form of pills is thought to slow down the aging process through the "free radical" scavenger activity of these compounds. The idea arose from the "Free Radical Theory of Ageing" (FRTA), initially developed by Harman in 1956. In the present paper, we present some arguments against this theory. One of the most pertinent is that "free radicals", more properly renamed as reactive oxygen species (ROS), play important biological roles in defense mechanisms of the organism as illustrated, in particular, by the hormesis phenomenon. Surprisingly, a moderate production of ROS has been shown to extend the life span in animals.

[Screening for frailty: a benefit for both patients and physicians]. / Dépister la fragilité: un bénéfice pour le patient et pour le soignant.

Preventing the increasing number of depending persons is a novel priority in European Union health policy. One of the means to succeed relies on identifying, among elderly persons, those at risk of dependency, also named "the frail elderly". Screening for frailty is also useful to better assess the physiological reserves of the elderly before any therapeutic decision, as early as the first consultation. Researchers currently work on developing a new simple tool allowing a distinction between frail and robust persons. Since frailty is partly reversible, the global geriatric evaluation, in a one-day clinic, will lead to a personalized program to prevent or reverse frailty by a multidisciplinary approach.

Germline mutations of the RET ligand GDNF are not sufficient to cause Hirschsprung disease.

Hirschsprung disease (HSCR, aganglionic megacolon) is a common congenital malformation leading to bowel obstruction, with an incidence of 1/5,000 live births. It is characterized by the absence of intrinsic ganglion cells in the myenteric and submucosal plexuses along variable lengths of the gastrointestinal tract. As enteric neurons are derived from the vagal neural crest, HSCR is regarded as a neurocristopathy. On the basis of a skewed sex-ratio (M/F = 4/1) and a risk to relatives much higher than the incidence in the general population, HSCR has long been regarded as a sex-modified multifactorial disorder. Accordingly, segregation analysis suggested an incompletely penetrant dominant inheritance in HSCR families with aganglionosis extending beyond the sigmoid colon. We and others have mapped a dominant gene for HSCR to chromosome 10q11.2 and have ascribed the disease to mutations in the RET proto-oncogene. However, the lack of genotype-phenotype correlation, the low penetrance and the sex-dependent effect of RET mutations supported the existence of one or more modifier gene(s) in familial HSCR. In addition, thus far, RET mutations only accounted for 50% and 15-20% of familial and sporadic HSCR patients, respectively. RET encodes a tyrosine kinase receptor whose ligand was unknown. Recently, the Glial cell line-derived neurotrophic factor (GDNF) has been identified to be a ligand for RET. Moreover, Gdnf-/- knockout mutant mice display congenital intestinal aganglionosis and renal agenesis, a phenotype very similar to the Ret-/- mouse. These data prompted us to hypothesize that mutations of the gene encoding GDNF could either cause or modulate the HSCR phenotype in some cases.

The use of body mass index for measurement of fat mass in children is highly dependant on abdominal fat.

We examined the relationship between body fat and body mass index (BMI) in a multiethnic population of obese children. BMI z-scores were compared to DEXA measures of whole body composition and regional fat distribution. Fat mass index (FMI) was best predicted by the equation: 1/[(0.159- 0.013 x percentile of total abdominal fat)- (0.01 x BMI z-score)], where percentile of abdominal fat ranges from 1 to 5. Predicted FMI had high agreement with FMI measured by DEXA. There were no detectable differences in this relation between different ethnic groups. Both BMI and abdominal fat should be used as a proxy to determine adiposity.

Stunting is a major risk factor for overweight: results from national surveys in 5 Arab countries.

We analysed data on overweight and stunting from large national surveys performed between 2001 and 2004 in 5 Arab countries (Djibouti, Libyan Arab Jamahiriya, Morocco, Syrian Arab Republic and Yemen). Overweight and stunting were defined according to new WHO growth standards. Overweight ranged from 8.9% in Yemen to 20.2% in Syrian Arab Republic. The risk ratio (RR) for overweight in stunted children ranged from 2.14 in Djibouti to 3.85 in Libyan Arab Jamahiriya. RR ranged from 0.76 in mildly stunted children of Yemen to 7.15 in severely stunted children in Libyan Arab Jamahiriya. Etiological fraction in the population ranged from 7.49% to 69.76%.

Long-term nocturnal calcium infusions can cure rickets and promote normal mineralization in hereditary resistance to 1,25-dihydroxyvitamin D.

We report the beneficial effects of calcium infusions in a child with hereditary resistance to 1,25(OH)2D and alopecia. This patient after transient responsiveness to vitamin D derivatives became unresponsive to all therapy despite serum 1,25(OH)2D concentrations maintained at levels approximately 100-fold normal. A 7-mo trial with calcium infusions led to correction of biochemical abnormalities and healing of rickets. Bone biopsies (n = 3) showed a normal mineralization and the disappearance of the osteomalacia. Cultures of bone-derived cells demonstrated a lack of activation of 25-hydroxyvitamin D 24-hydroxylase and osteocalcin synthesis by 1,25(OH)2D3 (10(-9) and 10(-6) M). These results demonstrate that even in the absence of a normal 1,25(OH)2D3 receptor-effector system in bone cells, normal mineralization can be achieved in humans if adequate serum calcium and phosphorus concentrations are maintained; and calcium infusions may be an efficient alternative for the management of patients with this condition who are unresponsive to large doses of vitamin D derivatives.

[Anatomic study of the submental artery relationships for submental flap raising]. / Étude anatomique des rapports de l'artère sous-mentonnière pour le prélèvement du lambeau sous-mentonnier.

INTRODUCTION: Submental flap is useful for intra-oral reconstructions and reconstructions of the lower two thirds of the face. Dissection is delicate because of a difficult exposure under the lower rim of the mandible, numerous collateral arterial branches and the proximity of the marginal branch of the facial nerve. The aim of our work was to propose anatomical landmarks in order to facilitate the submental flap raising. MATERIAL AND METHOD: Ten bodies preserved in Biomet liquid were dissected bilaterally. The anatomic relationships between the marginal branch of the facial nerve and the mandible, the relationships of the submental artery, the amount and the location of its collateral branches were measured by means of a caliper. RESULTS: The highest marginal branch observed was located 0.5 cm above the mandibular lower rim, while the lower one was located 0.6cm below this rim. The mean length measured between the facial artery at its crossing over the mandibular rim at the level of the pre-angular notch and the origin of the submental artery was 1.5cm. The average number of collateral branches was 3.6. DISCUSSION: A skin incision made directly under the mandibular lower rim, as mentioned by some authors, may endanger the mandibular marginal branch of the facial nerve. Three positions of the submental artery in relation to the sub-maxillary gland are reported. The collateral branches are intended for gland, muscle, skin and bone. It is necessary to pay particular attention to the sub-lingual artery, an artery of big diameter that arises at 2.8cm on average from its origin and plunges towards the mouth's floor. It must not be followed at risk of clamping the thin pedicle destined to the digastric muscle. It is important to preserve the fat tissue around the submental pedicle in order to avoid venous congestion of the flap.

Outcome and long-term growth after extensive small bowel resection in the neonatal period: a survey of 87 children.

UNLABELLED: This retrospective study aims to analyze the outcome, the prognosis factors and the long-term growth of children after extensive small bowel (SB) resection in the neonatal period. PATIENTS AND METHODS: 87 children, born between 1975 and 1991 who had undergone extensive neonatal small bowel resection, were followed up over a mean period of 15 years. Anatomical data influencing PN dependency and duration were analyzed. Data on height and weight were collected and compared using growth standards. Final heights were studied for patients who achieved their puberty and compared to predicted height based on Tanner's formula. Patients were analyzed according to PN weaning and growth: children still receiving PN (group A), patients weaned from initial PN but requiring PN once again or enteral feeding (group B), and children with permanent intestinal autonomy (group C). RESULTS: The overall survival is 89.7 %, depending on the date of birth. The duration of PN-dependency varies according to the intestinal length and the presence of the ileocaecal valve (ICV). All patients who remain PN dependent had less than 40 cm of small bowel and/or the absence of ICV. Patients in group B had a mean small bowel length of 35 +/- 19 cm, resection of the ICV in 50 % of cases, and a PN duration of 47.4 +/- 23.8 months. There was a significant decrease in height and weight gain within the 4 years after cessation of PN, requiring enteral or parenteral feeding. Patients in group C had a mean small bowel length of 57 +/- 19 cm, presence of ICV in 81 % of cases and a PN duration of 16.1 +/- 11.4 months. After PN weaning, they grow up normally with normal puberty and final height as predicted from genetic target height. CONCLUSION: PN duration is influenced by the length of residual SB and the absence of ICV. With good anatomic prognosis factors and short duration of initial PN, normal long-term growth may be predicted. Conversely, poor anatomical factors and protracted initial PN require careful monitoring of growth and may sometimes require nutritional support to be restarted. The last group, permanently dependent on PN, might be candidates for intestinal transplantation.

Protein-metabolism kinetics and energy-substrate utilization in infants fed parenteral solutions with different glucose-fat ratios.

The relative effect of glucose and lipids on whole-body protein-metabolism kinetics was assessed in seven infants undergoing parenteral feeding. Protein intake was kept constant and nonprotein energy was either provided as glucose alone or as an isoenergetic glucose-lipid mixture according to a randomized crossover trial. Protein metabolism and energy-substrate utilization were assessed by a primed, constant L-[13C]leucine infusion, combined with indirect calorimetry. There was a significant difference in the pattern of energy-substrate utilization according to regime. Protein turnover (11.3 +/- 0.7 vs 9.8 +/- 0.4 g.kg-1.d-1; P less than 0.05), protein breakdown (8.4 +/- 0.6 vs 7.1 +/- 0.4 g.kg-1.d-1; P less than 0.05), and amino acid oxidation rates (2.7 +/- 0.4 vs 1.4 +/- 0.5 g.kg-1.d-1; P less than 0.05) were higher for the glucose than the glucose-lipid treatment, whereas protein-synthesis rates did not significantly differ. These results suggest that the nature of energy substrates delivered to parenterally fed infants may affect protein metabolism.

Intestinal calcium-binding protein 3 months after massive small bowel resection in the piglet.

Changes in intestinal calcium-binding protein and calcium binding activity were studied at resection and 3 months after 90% small bowel resection in piglets and one adult pig. A calcium-binding protein (MW congruent to 11.000) with calcium-dependent eletrophoretic mobility was partially purified from mucosal extract of proximal jejunum, mid-gut, and ileum. The concentration of calcium-binding protein and the calcium-binding activity of the intact animals were found highest in the proximal jejunal segment, lowest in the ileal segment. After resection in the four surviving animals out of nine, a significant increase in calcium-binding activity was observed in the proximal jejunum and in the distal ileal segment. The change in calcium-binding activity was much more marked in the ileum than the jejunum. These data demonstrate that pig intestinal mucosa possesses an adaptive capacity to increase the synthesis of calcium-binding protein after massive small bowel resection.

Estimating resting energy expenditure by simple lean-body-mass indicators in children on total parenteral nutrition.

The aim of this study was to determine simple predictive factors of the resting energy expenditure (REE) in children. Two groups, A (n = 14) and B (n = 23), were defined by their weight-for-height index, less than 90% and greater than 90%, respectively. Anthropometrically assessed lean body mass (LBM), 24-h urinary creatinine, and REE were measured. From multiple-regression analysis, the best-fitting equation for calculating REE (REE = 54.4 LBM (kg) + 0.095 creatinine (mmol/kg) + 4.7) was highly significant (r = 0.987, p less than 0.0001). Although the regressions of REE on weight were significantly different between the two groups, the equations using LBM or 24-h urinary creatinine did not discriminate between them. These findings suggest that an equation based on LBM or 24-h urinary creatinine excretion could be a more accurate estimate of REE than are conventional methods based on weight or height, and it may be applicable to diverse nutritional states.

Growth retardation in children receiving long-term total parenteral nutrition: effects of ornithine alpha-ketoglutarate.

We evaluated the effect of ornithine ketoglutarate (OKG) in reversing abnormal growth in six prepubertal children receiving total parenteral nutrition (TPN) for 5-10 y. They were 1-4 SDs below their expected 50th percentile for height. The energy and nitrogen intakes were unchanged from 8 mo before the beginning of the study until its completion. Two consecutive periods of 5 mo each were studied. OKG (15 g) was added to the parenteral solution during the first period (OKG+) but not during the second period (OKG-). Height velocity (HV) increased (P < 0.05) from a median of 3.8 cm/y to 6.45 cm/y (range 1.8-6.7) during the OKG+ period, and decreased (P < 0.05) to a median of 3.65 cm/y in the OKG- period. Plasma concentrations of insulin-like growth factor 1 (IGF1), glutamine, and glutamate increased (P < 0.05) during the OKG+ period. Variations of IGF1 concentrations correlated with HV variations (r = 0.82, P < 0.005) during both periods. This study demonstrates that OKG is associated with statural growth acceleration and increased IGF1 concentrations.

Simple pediatric nutritional risk score to identify children at risk of malnutrition.

BACKGROUND: Although hospitalized children are at risk of malnutrition, routine screening of nutritional status has been hindered by lack of a validated nutritional assessment tool. OBJECTIVE: Our aim was to develop a simple pediatric nutritional risk score that could be used at hospital admission to identify patients at risk of acute malnutrition during hospitalization. DESIGN: Nutritional risk was assessed prospectively in 296 children. Anthropometric measurements, food intake, ability to eat and retain food, medical condition, and symptoms interfering with feeding (pain, dyspnea, and depression) were evaluated within 48 h of admission. Pathology was classified as mild (grade 1), moderate (grade 2), or severe (grade 3). The risk of weight loss was investigated with stepwise logistic regression. RESULTS: Weight loss during hospitalization occurred in 65% of the children and was >2% of admission weight in 45% of patients. Multivariate analysis indicated that food intake <50%, pain, and grade 2 and 3 pathologic conditions (P = 0.0001 for all) were associated with weight losses of >2%. The nutritional risk score ranged from 0 to 5 and was calculated by adding the values for the significant risk factors as follows: 1 for food intake <50%, 1 for pain, 1 for grade 2 pathologic condition, and 3 for grade 3 pathologic condition. A score of 1 or 2 indicated moderate risk and a score >/=3 indicated high risk of malnutrition. CONCLUSIONS: This simple score is suitable for routine use to identify patients at risk of malnutrition during hospitalization. Implementation may prevent hospital-acquired malnutrition.

Long-term efficacy and safety of a new olive oil-based intravenous fat emulsion in pediatric patients: a double-blind randomized study.

BACKGROUND: A new intravenous lipid emulsion (ILE) prepared from a mixture of soybean and olive oils contains only long-chain triacylglycerols, with a low proportion (20%) of polyunsaturated fatty acids and 60% monounsaturated fatty acids. OBJECTIVE: The goal of this randomized, double-blind clinical trial was to assess in children the efficacy and safety of this new ILE compared with a control group receiving a soybean-oil emulsion. DESIGN: Eighteen children received for 2 mo 24% of nonprotein energy (1.80 g kg (-)(1) d(-)(1)) either as the new ILE or a soybean oil-based emulsion. Assessments were performed on days -30, 0, 30, and 60 and the changes (day 60 - day 0) assessed by analysis of variance. RESULTS: There were no significant differences in triacylglycerol, apolipoproteins A-I and B, or HDL cholesterol between the 2 groups, whereas total and LDL cholesterol were higher in the soybean oil group on day 60. The pattern of 20:4n-6 in erythrocyte membranes did not change significantly, nor did the ratio of 20:3n-9 to 20:4n-6. On day 60, 18:1n-9 was significantly higher in the olive oil group, the ratio of Sigma(n)-6 > C(18) + 18:3n-6 to 18:2n-6 was 2.20 +/- 0.09 in the olive oil group and 1.33 +/- 0.16 in the soybean-oil group, and Sigma(n)-3 > C(18) was 3.83 +/- 0.30 in the olive oil group and 4. 03 +/- 0.33 in the soybean-oil group. The peroxidation index was lower after the olive oil treatment. CONCLUSIONS: The olive oil-based emulsion was well tolerated, maintained a normal EFA status, and may be more suitable for prevention of lipid peroxidation than the soybean-oil-based emulsion.

Decrease of slow-wave sleep in children with prolonged absence of essential lipids intake.

Sleep measures of eight children fed by total parenteral nutrition (TPN) without essential lipids were compared to those of seven children fed by TPN receiving a daily supplementation of essential lipids. Slow wave sleep (SWS) is significantly decreased in the former group, particularly in the second half of the night, thus suggesting that lipids could be involved in sleep regulation.

Craniosynostosis and kidney malformation in a case of Hennekam syndrome.

Hennekam syndrome is a rare autosomal recessive syndrome which was described for the first time in 1989. Here, we present a girl with intestinal lymphangiectasia, severe lymphedema of limbs, seizures, mild mental retardation, and facial anomalies consistent with the diagnosis of Hennekam syndrome. In addition, she had an ectopic kidney and craniosynostosis of the coronal suture, 2 manifestations not previously reported in this syndrome. While the molecular basis of Hennekam syndrome remains, as yet, unknown, this report illustrates its variable clinical expression.

Craniofacial anomalies and malformations in respiratory chain deficiency.

We report on facial anomalies including round face, high forehead, flat philtrum, apparently low-set ears, and short neck in 4 unrelated patients with mitochondrial respiratory enzyme deficiency. Pre- and postnatal growth retardation with microcephaly, brachydactyly, and hypoplasia of distal and middle phalanges was present in all 4 cases. The diagnosis of respiratory chain deficiency was confirmed by enzymatic and molecular studies. The combination of facial anomalies, prenatal growth failure, and malformations is suggestive of antenatal expression of the disease, and raises the question of the part that respiratory chain deficiencies play in human malformations.

Severe combined immunodeficiency syndrome associated with autosomal recessive familial multiple gastrointestinal atresias: study of a family.

Hereditary multiple atresias involving the gastrointestinal tract from pylorus to rectum are the most unusual form of intestinal atresia; the type of inheritance was suggested to be autosomal recessive. The inheritance of the severe combined immunodeficiency syndrome can be autosomal recessive or X-linked. We report on 3 sibs with multiple-level intestinal atresias. One sib had severe combined immunodeficiency syndrome and clinical histories of the other 2 sibs strongly suggested a congenital immunodeficiency syndrome. The parents of those children were healthy and nonconsanguineous. To our knowledge, this is the first report of the association of multiple gastrointestinal atresias and immunodeficiency which appears to have an autosomal recessive pattern of transmission. Our family report suggests that, in the presence of multiple gastrointestinal atresias, attention should be given to possible associated immunological disorders.