Multicore myopathy with restrictive cardiomyopathy

A 10-y-old girl is presented who suffered mild muscular weakness and exercise intolerance from the age of 1 y onwards, with progression appearing from the age of about 8 y. Multicore myopathy and restrictive cardiomyopathy were diagnosed. Literature concerning the coexistence of multicore myopathy and cardiomyopathy is reviewed.     

The effect of changes in dietary fat on the food group and nutrient intake of 4- to 10-year-old children

OBJECTIVE: To determine how young children changed their overall diet when they changed their fat intake after 3 months of participating in a nutrition education demonstration study designed to lower low-density lipoprotein cholesterol and cardiovascular risk. METHODS: Three 24-hour dietary recalls were collected from 303 4- to 10-year-old children at baseline and 3 months later. At both times, mean number of servings from food groups, grams of fat contributed from food groups, and intake of calories and nutrients were calculated and compared among quartiles of children formed according to change in their percent of calories from total fat after 3 months. RESULTS: Children who reduced their percent of calories from total fat most (ie, by an average of 8.5%) after 3 months consumed fewer servings from meats, eggs, dairy, fats/oils, and breads but tended to increase their number of servings from lower-fat foods within those food groups, particularly from dairy foods. These children also increased their mean intake of fruits, vegetables, and desserts, and maintained average intakes of all nutrients (except vitamin D) in excess of two thirds of the respective recommended dietary allowance. CONCLUSIONS: Young children who reduced their percent of calories from total fat in accordance with the current National Cholesterol Education Program recommendations accomplished this by reducing their overall intake of higher-fat foods, replacing higher-fat foods with lower-fat foods within several food groups, particularly within the dairy group (eg, drinking skim milk instead of whole milk) and by consuming more servings of fruits, vegetables, and very-low-fat desserts. These behaviors did not compromise their mean calorie or nutrient intakes, showing that it is possible for young children to lower their fat intake safely to reduce their risk of future heart disease.     

Cyclic food restriction, insulin and mammary cell proliferation in the rat

We reported recently that weight cycling significantly increased the incidence of mammary cancer in virgin female rats that were pretreated with N-methyl-N-nitrosourea. The present study investigated the effect of weight cycling on mammary epithelial cell proliferation and its relationship to changes in plasma insulin, estrogen, progesterone and urinary corticosterone in 30 female virgin Sprague-Dawley rats. Animals were fed a modified AIN-76A diet containing 24.6% corn oil by weight. Weight-cycled (WC) rats were food restricted daily by either 33% or 50% of non-restricted controls for 1 week followed by 3 weeks compensatory refeeding and weight recovery over 18 weeks or 4.5 weight cycles. WC rats consumed 6-10% less food than controls (P = 0.01) but showed a 71- 89% greater efficiency of food utilization for growth (P < 0.0001) than controls. There were no differences in total weight gain during treatment. Mammary lobuloalveolar and ductal cell proliferation of WC rats, measured by 5-bromo-2'deoxyuridine labelling, increased in a dose- response fashion, P = 0.03, P = 0.06 respectively in comparison to controls. Energy and substrate utilization measured by indirect calorimetry indicated WC animals expended less energy (P = 0.005) and utilized less glucose (P = 0.0001) and protein (P = 0.006) during restriction, and less lipid during recovery (P = 0.05) than controls. There were no significant differences in hormone levels between groups. Multiple regression analysis with plasma insulin, estrogen, progesterone and urinary corticosterone as independent variables (r = 0.947, r2 = 0.897, P = 0.003) showed that plasma insulin was the only significant predictor (P < 0.01) of mammary cell proliferation. In accord with this observation, tyrosine-phosphorylated activation of insulin receptor substrate-1, detected by immunoprecipitation and Western immunoblot analysis in mammary tumors of WC rats from our previous study, was 3-5 times greater than in non-restricted controls (P < 0.01). Present findings suggest that weight cycling in rats increases risk of breast cancer development via insulin stimulated mammary cell proliferation.      

Oral anticoagulation in paediatric patients: dose requirements and complications

The lack of oral anticoagulant guidelines specific to paediatric practice has led to the adoption of adult regimens, often without scientific evidence of efficacy or safety. A two year prospective study of anticoagulant control was carried out in 45 children aged 9 months to 18 years, the majority of whom were receiving primary prophylactic anticoagulation. The main indication was congenital heart disease, either with (n = 8) or without (n = 34) mechanical valve prosthesis. During a follow up period of 602 patient months the average interval between visits was three weeks. Target international normalised ratios (INRs) were achieved on 62% and 39% of visits for children with low target INR (2.0-3.0) and high target INR (3.0-4.0) respectively. However warfarin dose was altered on only 22% of visits. Warfarin doses required to achieve a stable INR of 2.0-3.0 in 33 children were strongly correlated with weight [dose (mg/d) = 0.07 x weight (kg) + 0.54] but independently influenced by age. No thrombotic complications were recorded, and haemorrhagic events were infrequent (2.1% of visits) and, with one exception, minor. Safe outpatient oral anticoagulation is feasible in children, whose warfarin requirements appear moderately predictable and whose control is no more erratic than that of adults.     

Immune responses to liver membrane antigens in patients with cystic fibrosis and liver disease

Biliary obstruction by viscid mucus, although important, may not be the only factor for the development of liver disease in some patients with cystic fibrosis. In the present study the relationship between immune responses to liver antigens and the presence of liver damage was investigated using the leucocyte migration test and lymphocyte cytotoxicity to isolated rabbit hepatocytes. Inhibition of leucocyte migration by purified liver-specific lipoprotein, derived from hepatocyte plasma membrane, was found in 9 of 11 children with liver disease, but in only 5 of 14 with cystic fibrosis and no overt liver disease (P < 0.025). Lymphocyte toxicity to isolated rabbit hepatocytes was significantly increased in 10 of 13 children with liver disease, but in only 6 of 29 children without liver disease (P < 0.001). Experiments using lymphocyte subpopulations showed that the cytotoxicity was mediated by a non-T-cell population and could be blocked with liver-specific lipoprotein in 7 out of 10 cases, suggesting that the reaction in these patients was specifically directed against liver-specific lipoprotein. The study suggests that sensitisation against liver membrane antigens, whether arising primarily or secondarily in some way to other hepatic lesions, may contribute to the progression of liver damage in cystic fibrosis.     

Calcium, phosphorus, and magnesium concentrations in plasma during first week of life and their relation to type of milk feed

Serial changes in plasma levels of calcium, phosphorus, magnesium, alkaline phosphatase, and total proteins have been investigated in 138 healthy, term Caucasian infants. Blood samples were obtained for each infant from cord blood and on day 1 and day 6. The infants were studied in three groups according to whether they were breast fed, received 'Ostermilk No. 1' or 'Cow and Gate V' formulas. Levels of calcium, phosphorus, magnesium, alkaline phosphatase, and total proteins did not differ between the groups at birth or on day 1. By day 6 calcium levels were higher and phosphorus levels lower in the breast-fed infants compared with either of the artificially-fed groups. Phosphorus levels were lower in the V Formula group compared with the Ostermilk group but the mean calcium levels of these two groups did not differ significantly. However, only 2-8% of the V Formula group developed hypocalcaemia compared with 18-2% of the Ostermilk group. The only infant developing clinical tetany belonged to the group fed Ostermilk. Evidence is also given which suggests that those infants with low calcium levels on day 1 who were fed the high-solute milk tended to show a fall in calcium by day 6. This did not apply to the two other groups. It is concluded that the use of adapted cows' milk preparations for infant feeding should lead to a reduction in the incidence of neonatal tetany.     

Infections of the musculoskeletal system: high-field-strength MR imaging

Twenty-two patients with clinical findings consistent with osteomyelitis, soft-tissue infection, or both were studied with magnetic resonance (MR) imaging at 1.5 T. Another 15 patients with joint effusion but no clinical or laboratory signs of infection served as controls. Soft-tissue abscesses, osteomyelitis, joint and tendon sheath effusion, and cellulitis were well depicted on MR imaging, allowing the correct diagnosis of presence and extent of infection in all but two cases. MR imaging was as sensitive as technetium-99m methylene diphosphonate bone scintigraphy in demonstrating osteomyelitis and was more specific and more sensitive than other scintigraphic techniques in demonstrating soft-tissue infections, primarily because of its superior spatial resolution. Computed tomography, performed in seven cases, was as accurate as MR imaging in demonstrating bone and soft-tissue infections. Infected and noninfected synovial effusions had the same signal intensity, but associated findings such as soft-tissue fluid collections or osteomyelitis made the distinction possible.     

Gastrointestinal tract: dynamic MR studies with echo-planar imaging

Gastrointestinal (GI) tract motility was depicted in four human volunteers with the high-speed echo-planar imaging technique: modulus blipped echo-planar single-pulse technique (MBEST). Data acquisition times of 64 and 128 msec obviate image degradation due to motion without the need for gut paralysis and allow imaging of the GI tract in real time. Peristaltic patterns of the gastric antrum and proximal small intestine were depicted for fasting and fed subjects and subjects in whom peristalsis had been pharmacologically stimulated. The potential for quantitative measurements of GI tract motion with this new technique was demonstrated.