Investigating young children's physical activity through time and place.

BACKGROUND: Previous research indicates the start of primary school (4-5-year-old) as an essential period for the development of children's physical activity (PA) patterns, as from this point, the age-related decline of PA is most often observed. During this period, young children are exposed to a wider variety of environmental- and social contexts and therefore their PA is influenced by more diverse factors. However, in order to understand children's daily PA patterns and identify relevant opportunities for PA promotion, it is important to further unravel in which (social) contexts throughout the day, PA of young children takes place. METHODS: We included a cross-national sample of 21 primary schools from the Startvaardig study. In total, 248 children provided valid accelerometer and global positioning (GPS) data. Geospatial analyses were conducted to quantify PA in (social) environments based on their school and home. Transport-related PA was evaluated using GPS speed-algorithms. PA was analysed at different environments, time-periods and for week- and weekend days separately. RESULTS: Children accumulated an average of 60 min of moderate-to-vigorous PA (MVPA), both during week- and weekend days. Schools contributed to approximately half of daily MVPA during weekdays. During weekends, environments within 100 m from home were important, as well as locations outside the home-school neighbourhood. Pedestrian trips contributed to almost half of the daily MVPA. CONCLUSIONS: We identified several social contexts relevant for children's daily MVPA. Schools have the potential to significantly contribute to young children's PA patterns and are therefore encouraged to systematically evaluate and implement parts of the school-system that stimulate PA and potentially also learning processes. Pedestrian trips also have substantial contribution to daily MVPA of young children, which highlights the importance of daily active transport in school- and parental routines.

Arsenic speciation in food and estimation of the dietary intake of inorganic arsenic in a rural village of West Bengal, India.

Arsenic (As) species were quantified by HPLC-HG-AFS in water and vegetables from a rural area of West Bengal (India). Inorganic species predominated in vegetables (including rice) and drinking water; in fact, inorganic arsenic (i-As) represented more than 80% of the total arsenic (t-As) content. To evaluate i-As intake in an arsenic affected rural village, a food survey was carried out on 129 people (69 men and 60 women). The data from the survey showed that the basic diet, of this rural population, was mainly rice and vegetables, representing more than 50% of their total daily food intake. During the periods when nonvegetarian foods (fish and meat) were scarce, the importance of rice increased, and rice alone represented more than 70% of the total daily food intake. The food analysis and the food questionnaires administrated led us to establish a daily intake of i-As of about 170 microg i-As day (-1), which was above the tolerable daily intake of 150 microg i-As day (-1), generally admitted. Our results clearly demonstrated that food is a very important source of i-As and that this source should never be forgotten in populations depending heavily on vegetables (mainly rice) for their diet.

Life-long course and molecular characterization of the original Dutch family with epidermolysis bullosa simplex with muscular dystrophy due to a homozygous novel plectin point mutation.

Plectin is one of the largest and most versatile cytolinker proteins known. Cloned and sequenced in 1991, it was later shown to have nonsense mutations in recessive epidermolysis bullosa with muscular dystrophy. A dominant mutation in the gene was found to cause epidermolysis bullosa simplex Ogna without muscular dystrophy. Here we report the DNA sequencing of the plectin gene (PLEC1) in a Dutch family originally described in 1972 as having epidermolysis bullosa with muscular dystrophy. The results revealed homozygosity for a new plectin nonsense mutation at position 13187 and its specific 8q24 marker haplotype profile. Western blotting of cultured fibroblasts and immunofluorescence microscopy of skin biopsy confirm that the plectin protein expression is grossly reduced or absent. A summary of the life-long clinical course of the two affected brothers homozygous for the new E1914X mutation is given.

Effect of structure and form on the ability of plant sterols to inhibit cholesterol absorption in hamsters.

We investigated the effect of three types of plant sterols (4-desmethylsterols, 4,4'-dimethylsterols, and pentacyclic triterpene alcohols) in three forms (free, esterified with FA, or with phenolic acids) on cholesterol absorption. Plant sterol fractions derived from soybean (99% 4-desmethylsterols), rice bran (70% 4,4'-dimethylsterols), or shea nut (89% pentacyclic triterpene alcohols) were fed to male hamsters (n = 20/group) as free sterols or esterified with FA or phenolic acids (cinnamic or ferulic). Cholesterol absorption was measured after 5-8.5 (mean, 7) wk by a dual-isotope technique. Soybean sterol intake significantly reduced cholesterol absorption efficiency (23%) and plasma total cholesterol (11%). Rice bran sterols tended to lower cholesterol absorption efficiency by 7% and plasma total cholesterol by 5%, whereas shea nut sterols had no effect. In hamsters, dietary 4-desmethylsterols were more effective than 4,4'-dimethylsterols in lowering cholesterol absorption and levels of cholesterol in blood. Pentacyclic triterpene alcohols had no effect on the absorption of cholesterol or on its level in blood. Esterification with FA did not impair the ability of 4-desmethylsterols and 4,4'-dimethylsterols to inhibit cholesterol absorption, whereas esterification with phenolic acids reduced this ability. This study supports the use of 4-desmethylsterols, esterified with FA to increase solubility, as the most effective cholesterol-lowering plant sterols in the diet.

Effect of esterified 4-desmethylsterols and -stanols or 4,4'-dimethylsterols on cholesterol and bile acid metabolism in hamsters.

4-Desmethylsterols and -stanols reduce plasma total cholesterol (TC) and LDL cholesterol by inhibition of intestinal cholesterol absorption, while the cholesterol-lowering potential of 4,4'-dimethylsterols is less well defined. The present study aimed to compare the effects of 4-desmethylsterols, -stanols, and 4,4'-dimethylsterols on plasma and hepatic cholesterol, sterol excretion and bile acid metabolism. Male golden Syrian hamsters were fed diets containing 13 g/100 g fat, 008 g/100 g cholesterol and 0 (control), 0.24 or 0.48% (w/w) esterified 4-desmethylsterols (sterols) and esterified hydrogenated 4-desmethylsterols (stanols) from common vegetable oils or esterified 4,4'-dimethylsterols from rice bran oil for 5 weeks. Sterol and stanol esters at the dose of 0.24% were equally effective and significantly (P<0.05) lowered TC by 15%, while 0.24% 4,4-dimethylsterols reduced TC by 10%. Liver total and esterified cholesterol concentrations were significantly (P<0.05) lowered by 40, 22, 43 and 31% in hamsters fed 0.48% sterols, 0.24% stanols, 0.48% stanols or 0.48% dimethylsterols, respectively. Daily faecal bile acid excretion and hepatic cholesterol 7alpha-hydroxylase activity were not altered, indicating that sterols, stanols and dimethylsterols had no effect on the intestinal re-absorption of bile acids or on hepatic bile acid synthesis. Daily excretion of cholesterol was significantly higher in hamsters fed esterified sterols and stanols, but was only slightly increased in those fed dimethylsterols. The results indicate that esterified sterols and stanols were equally effective in lowering plasma TC and LDL cholesterol, while dimethylsterol esters caused a weaker cholesterol-lowering effect. Sterols and stanols achieve their cholesterol-lowering effect by stimulating faecal cholesterol excretion through inhibiting intestinal cholesterol absorption, but do not affect bile acid excretion. Other mechanisms need to be considered to explain the effect on plasma and hepatic cholesterol of dimethylsterols.

[Little difference in quality of life of dialysis patients in Utrecht and Willemstad]. / Kwaliteit van leven van dialysepatiënten in Utrecht en Willemstad weinig verschillend.

OBJECTIVE: To assess and compare the quality of life of patients treated with haemodialysis and chronic ambulatory peritoneal dialysis (CAPD) in Utrecht and Willemstad, Curaçao. DESIGN: Transverse multicentre study. METHODS: All haemodialysis and CAPD patients in Utrecht and all haemodialysis patients in Curaçao under treatment for over 6 months were studied. The objective tests applied were the 'Nottingham health profile', the 'affect balance scale', the 'index of well-being' and the 'Amsterdam complaint profile'. Possible correlations between individual patient-related and treatment-related factors and biochemical variables were also investigated. RESULTS: The objective and subjective tests revealed only slight differences in quality of life in the three groups. In a few respects, the CAPD patients rated the quality of life slightly better. In the Utrecht group a positive relationship was seen between haematocrit (higher owing to treatment with erythropoietin) and plasma bicarbonate concentration, and the quality of life.

Clinical features of an affected father and daughter with Ehlers-Danlos syndrome type VIIB.

The clinical features of a father and daughter with Ehlers-Danlos syndrome type VIIB are described. They included severe cutaneous fragility, generalized joint laxity, kyphoscoliosis and a slightly dysmorphic face in the adult, with generalized joint laxity and congenital hip dislocation, hyperextensible skin and easy bruising in the child. The dermis contained slightly distorted collagen fibrils when examined by electron microscopy. The disorder is caused by G to A point mutation in the first base of intervening sequence 6 with resultant mis-splicing.

Practical phlebology. Sclerotherapy of large veins.

A practical, step-by-step guide for the sclerotherapy of large varicose veins is offered. First, an orientation regarding the place of sclerotherapy and surgery in the total treatment of varicose veins is given, with a practical clinical classification of varicose veins. This is followed by a detailed discussion of patient education before sclerotherapy, material to be used with recommended concentrations, injection technique, compression after sclerotherapy, instructions to the patient after injection, and procedures at subsequent visits.

Treatment of varicose veins: modern concepts and methods.

Varicose veins have been a somewhat neglected entity. Treatment usually consists of routine stripping of the main trunks and excision of all distal tributaries, resulting in many unsightly scars. Modern diagnostic methods trace the origin of the reflux causing the condition. If the saphenofemoral or saphenopopliteal junction is involved, limited surgery is done, followed by sclerotherapy of the varicose tributaries later. Many cases can be treated with sclerotherapy only. A description of the new concepts is given. A diagnostic classification is presented, together with the corresponding methods of treatment.

Ito's hypomelanosis (incontinentia pigmenti achromians). A review of four cases.

In 1952 Ito described the occurrence of a bilateral systematized depigmented nevus in a 22-year-old Japanese woman. He used the term incontinentia pigmenti achromians. The condition has been described under various designations, such as for instance Ito's hypomelanosis. Till now 71 patients with this syndrome are described. We will report 4 cases, 2 boys and 2 girls, 2 Caucasian, 1 Indonesian and 1 Caribean child. The cutaneous signs in these 4 patients fit in with the syndrome of Ito's hypomelanosis. Of these 4 children 3 are mentally retarded, 2 have epilepsy. Congenital malformations are seen in 3 children. Electronmicroscopy of skin biopsies of the hypomelanotic nevus and of the normal skin were performed. In the biopsy of the normal skin of one patient interruption of the basement membrane is seen. Anomalies of the central nervous system as seen in our patients occur in about 40% of the cases. Abnormalities of skin derivatives next to other ectodermal anomalies are described. Affection of other germ layers also occur to a varying degree. In our 4 patients some of these abnormalities exist also. These 4 cases are presented to underline the fact that this syndrome seems not to be as extremely rare as is proposed.

A new method for the determination of steroid sulphatase activity in leukocytes in X-linked recessive ichthyosis.

The diagnosis of X-linked ichthyosis can now be reliably established by using a non-radioactive method to detect steroid sulphatase deficiency in leukocytes. This new method yields the same results with leukocytes as with cultured fibroblasts. The second type of microsomal arylsulphatase previously described in cultured fibroblasts is also present in leukocytes.

Pathology of the lung in Behçet's disease. Case report and review of the literature.

Histopathologic changes in the lung in a patient with Behçet's disease are described and the literature dealing with pulmonary pathologic changes in this disease is summarized. The basic lesion is a lymphocytic and necrotizing vasculitis involving all sized pulmonary arteries, veins and septal capillaries. Complications include aneurysms of elastic pulmonary arteries, arterial and venous thromboses, pulmonary infarcts, and bronchial erosion by pulmonary artery aneurysms. Striking periadventitial fibrosis develops and is believed to be related to repetitive vascular inflammatory insults. Peculiar newly formed collateral vessels, lacking elastic lamellae and derived from smooth muscle metaplasia around arterioles, are found in the periadventitial fibrous tissues around thrombosed arteries and aneurysms. Cases of pulmonary artery aneurysms of obscure origin, including those found in the Hughes-Stoven syndrome, are reviewed and both clinical and pathologic findings in these reports are found to be remarkably similar to those observed in Behçet's disease. It is suggested that such cases represent unrecognized or incomplete expressions of Behçet's disease.

Familial acrogeria (Gottron).

A mother and her oldest son suffering from acrogeria are described; in the mother the disease was complicated by elastosis perforans serpiginosa. Microscopic and ultramicroscopic details are given.

Sex-linked ichthyosis and placental sulphatase C deficiency.

Ichthyosis was diagnosed in six boys born from pregnancies in which placental steroid sulphatase deficiency was present. In four cases the diagnosis of sex-linked ichthyosis was certain, in the other two the ichthyosis was probably of the sex-linked type. No arylsulphatase C activity could be demonstrated histochemically either in the epidermis of these boys, or in the epidermis of adults with sex-linked ichthyosis, whilst it was present in normal controls. In cultured skin fibroblasts steroid-sulphatase activity was extremely low. We conclude that deficiency of steroid sulphatase C or arylsulphatase C or a third functionally linked enzyme is an important factor in the pathogenesis of sex-linked ichthyosis.