Huntington Disease - principles and practice of nutritional management.

Huntington disease (HD) is a degenerative brain disease clinically manifested by the characteristic triad: physical symptoms including involuntary movements and poor coordination, cognitive changes with less ability to organize routine tasks, and some emotional and behavioral disturbances. For patients with HD, feeding is one of the problems they have to face. People with HD often have lower than average body weight and struggle with malnutrition. As a part of therapy, good nutrition is an intervention maintaining health and functional ability for maximally prolonged time. In the early stages of HD, small amounts of blenderized foods given orally are recommended. In more advanced stages, enteral nutrition is essential using gastric, or jejunal tubes for short term. Most severe cases require gastrostomy or gastrojejunostomy. Although enteral feeding is well tolerated by most of the patients, a number of complications may occur, including damage to the nose, pharynx, or esophagus, aspiration pneumonia, sinusitis, metabolic imbalances due to improper nutrient and fluid supply, adverse effects affecting gastrointestinal system, and refeeding syndrome.

Measurement of bronchial and alveolar nitric oxide production in normal children and children with asthma.

RATIONALE: Airway inflammation is characteristic of asthma. Distal inflammation may be particularly important. OBJECTIVE: To calculate alveolar nitric oxide (NO) concentration (C(alv)) and bronchial flux NO (J(NO)) in children. METHODS: We measured C(alv) and J(NO) from the fractional exhaled NO (FeNO(50)) measured at multiple exhalation flow rates in 132 children (aged 4-18 yr) with known atopic status, medication, and asthma control. MEASUREMENTS AND MAIN RESULTS: Of participants, 85% (112/132) completed all measurements. In 20 of 112, the result did not fit the linear model. Thus, J(NO) and C(alv) were assessed in 92 (70%) subjects. The median (range) values of asthmatic (n = 52), normal (n = 20), and nonasthmatic atopic (n = 20) children were as follows: FeNO(50): 28.1 (4.3-190), 10.35 (3.3-29), 21.8 (8.7-69) ppb, respectively; J(NO): 1,230 (204-9,236), 480 (196-1,913), 1,225 (486-4,119) pl/s, respectively; C(alv): 2.22 (0.44-6.63), 1.63 (0.44-3), 1.21 (0.03-2.85) ppb, respectively. A reproducibility study in 18 other children gave intraclass correlation coefficients (single measures) of 0.99 (J(NO)) and 0.81 (C(alv)). J(NO) and C(alv) were higher in children with asthma than normal children (p = 0.0004 and p = 0.0002, respectively). Children with poorly controlled asthma (n = 27) had higher FeNO(50) measurements than children with good symptom control (n = 25): C(alv): mean (+/- SD), 3.17 +/- 1.62 versus 2.26 +/- 1.30 ppb, p = 0.03; J(NO): mean (+/- SD), 2,634 +/- 2,255 versus 1,193 +/- 1,294 pl/s, p = 0.007, respectively. CONCLUSIONS: Measurement of J(NO) and C(alv) is feasible in 70% of school-age children. FeNO(50) and J(NO) give the same information (r = 0.97, p < 0.0001), C(alv) is higher in asthmatic children than in normal children and is affected by asthma control, but not by atopy. C(alv) may possibly reflect alveolar inflammation in asthma.