Chest infections in young neuromuscular patients: the critical importance of preserving Vital Capacity.
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Child↗
Biomedical subjects
Andrea Vianello
Publications and source records attributed to Andrea Vianello.
Does helmet CPAP reduce cerebral blood flow and volume by comparison with Infant Flow driver CPAP in preterm neonates?
OBJECTIVE: We compared neonatal helmet continuous positive airway pressure (CPAP) and the conventional nasal Infant Flow driver (IFD) CPAP in the noninvasive assessment of absolute cerebral blood flow (CBF) and relative cerebral blood volume changes (DeltaCBV) by near-infrared spectroscopy. DESIGN AND SETTING: A randomized crossover study in a tertiary referral NICU. PATIENTS AND INTERVENTIONS: Assessment of CBF and DeltaCBV in 17 very low birth weight infants with respiratory distress (median age 5 days) treated with two CPAP devices at a continuous distending pressure of 4 mbar. MEASUREMENTS AND RESULTS: Neonates were studied for two consecutive 60-min periods with helmet CPAP and with IFD CPAP. Basal chromophore traces enabled DeltaCBV changes to be calculated. CBF was calculated in milliliters per 100 grams per minute from the saturation rise integral and rate of rise O(2)Hb-HHb. Median (range) CBF with helmet CPAP was 27.37 (9.47-48.20) vs. IFD CBF 34.74 (13.59-60.10)(p=0.049) and DeltaCBV 0.15 (0.09-0.28) with IFD and 0.13 (0.07-0.27) with helmet CPAP (NS). Using helmet and IFD CPAP, the neonates showed no difference in mean physiological parameters (transcutaneous carbon dioxide and oxygen tension, pulse oximetry saturation, heart rate, breathing rate, mean arterial blood pressure, desaturation rate, axillary temperature). CONCLUSION: Assessing CBF and DeltaCBV measured by near-infrared spectroscopy with two CPAP devices revealed no differences in relative blood volume, but CBF was lower with helmet CPAP. Greater active vasoconstriction and/or passive capillary and/or venous vessel compression seem the most likely reason, due to a positive pressure around the head, neck, and shoulders by comparison with the airway pressure.
Analysis of Variance↗
Body composition and resting energy expenditure in elderly male patients with chronic obstructive pulmonary disease.
STUDY OBJECTIVE: Our study investigates nutritional status, resting energy expenditure (REE) and physical performance in elderly patients with stable COPD to identify any early conditions of hypermetabolism, malnutrition and sarcopenia. METHODS: Eighty-six males (40 stable COPD and 46 healthy subjects) over 65 years old were studied. All subjects underwent spirometry, blood gas analysis and a 6-min walking test (6MWT). Fat-free mass (FFM) and appendicular skeletal muscle mass (ASMM) were measured by dual energy X-ray absorptiometry (DEXA). REE was measured by indirect calorimetry. RESULTS: COPD patients had a lower FFM both expressed in kilograms and after correction for height squared. The prevalence of sarcopenia was higher for COPD subjects (38% vs 31%). REE, both in absolute values and adjusted for FFM was significantly higher in COPD patients. Hypermetabolism was found in 60% of COPD cases and 13.7% (P<0.01) of healthy subjects. No relationship was found in COPD patients between the measured/predicted REE ratio (REE(m)/REE(p)) and FEV1. In the hypermetabolic COPD subgroup, the REE(m)/REE(p) ratio correlated with 6MWT. CONCLUSIONS: Elderly patients with stable COPD develop an increased REE. This hypermetabolism seems to be independent of the severity of the pulmonary obstruction and to influence the patient's physical performance.
Adipose Tissue↗
Clinical and molecular characterization of patients with limb-girdle muscular dystrophy type 2I.
BACKGROUND: Limb-girdle muscular dystrophy type 2I is caused by mutations in the fukutin-related protein gene (FKRP). FKRP encodes a putative glycosyltransferase protein that is involved in alpha-dystroglycan glycosylation. OBJECTIVES: To identify patients with limb-girdle muscular dystrophy type 2I and to derive genotype-phenotype correlations. DESIGN: Two hundred fourteen patients who showed muscle histopathologic features consistent with muscular dystrophy or myopathy of unknown etiology were studied. The entire 1.5-kilobase FKRP coding sequence from patient DNA was analyzed using denaturing high-performance liquid chromatography of overlapping polymerase chain reaction products, followed by direct sequencing of heteroduplexes. RESULTS: Thirteen patients with limb-girdle muscular dystrophy type 2I (6% of all patients tested) were identified by FKRP mutation analysis, and 7 additional patients were identified by family screening. Six missense mutations (1 novel) were identified. The 826C>A nucleotide change was a common mutation, present in 35% of the mutated chromosomes. Clinical presentations included asymptomatic hyperCKemia, severe early-onset muscular dystrophy, and mild late-onset muscular dystrophy. Dilated cardiomyopathy and ventilatory impairment were frequent features. Significant intrafamilial and interfamilial clinical variability was observed. CONCLUSIONS: FKRP mutations are a frequent cause of limb-girdle muscular dystrophies. The degree of respiratory and cardiac insufficiency in patients did not correlate with the severity of muscle involvement. The finding of 2 asymptomatic patients with FKRP mutations suggests that modulating factors may ameliorate the clinical phenotype.
Adolescent↗
Tracheoinnominate fistula in a Duchenne muscular dystrophy patient: successful management with an endovascular stent.
Tracheoinnominate fistula is a rare but often fatal complication occurring in Duchenne Muscular Dystrophy (DMD) patients with long-term tracheostomy. We report a 16-year-old boy with DMD who developed a fistula causing massive haemorrhage 26 months after tracheostomy. Due to the high risk of perioperative complications, a minimally invasive technique with placement of an endovascular stent grafting the innominate artery was performed. The patient was successfully managed and recovered uneventfully. We believe that endovascular repair of tracheoinnominate fistula by stent grafting may be the treatment of choice in severely compromised patients and that clinicians who treat tracheostomised DMD patients should be familiar with this management strategy.
Adolescent↗
Foot pulse oximeter perfusion index correlates with calf muscle perfusion measured by near-infrared spectroscopy in healthy neonates.
OBJECTIVE: In critically ill neonates, peripheral perfusion and oxygenation assessment may provide indirect information on the circulatory failure of vital organs during circulatory shock. The development of pulse oximetry has recently made it possible to calculate the perfusion index (PI), obtained from the ratio between the pulsatile and nonpulsatile signals of absorbed light. The main goals of this study were: (1) to study foot PI; and (2) to evaluate the relationship between foot PI, obtained continuously by pulse oximetry, and a number of variables, i.e. blood flow (BF), oxygen delivery (DO(2)), oxygen consumption (VO(2)), and fractional oxygen extraction (FOE), measured indirectly by near-infrared spectroscopy (NIRS) on the calf in 43 healthy term neonates (weight 3474.6 +/- 466.9 g; gestational age 39.1 +/- 1.4 weeks). STUDY DESIGN: Calf BF, DO(2) and VO(2) were assessed by NIRS on short-lived venous and arterial occlusion maneuvers. PI was measured on the contralateral foot. RESULTS: Foot PI was 1.26 +/- 0.39. There was a positive correlation between foot PI and both calf BF (r = 0.32, p = 0.03) and DO(2) (r = 0.32, p = 0.03), but no correlation was found between foot PI and calf FOE and between foot PI and VO(2). CONCLUSIONS: In the neonatal intensive care unit, continuously measuring foot PI by pulse oximetry seems clinically more feasible for peripheral perfusion monitoring than spot measurements of the calf BF and/or VO(2) by indirect NIRS.
Foot↗
Mechanical insufflation-exsufflation improves outcomes for neuromuscular disease patients with respiratory tract infections.
OBJECTIVES: The efficacy of mechanical insufflation-exsufflation, in addition to standard chest physical treatments, was investigated as a first-line intervention for patients with neuromuscular diseases with respiratory tract infections and airway mucous encumbrance. DESIGN: The short-term outcomes of 11 consecutive neuromuscular disease patients with respiratory tract infections and tracheobronchial mucous encumbrance who were administered mechanical insufflation-exsufflation and conventional chest physical treatments in an intensive care unit were compared with the outcomes of 16 historical matched controls who had received chest physical treatments alone. Treatment failure was defined as the need for cricothyroid "minitracheostomy" or endotracheal intubation, despite treatment. The number of subjects administered bronchoscopy-assisted aspiration during the hospital stay was also compared. RESULTS: Treatment failure was significantly lower (P < 0.05) in the mechanical insufflation-exsufflation group than in the conventional chest physical treatments group (2/11 vs. 10/16 cases). The use of bronchoscopy-assisted aspiration was similar in the two groups (5/11 vs. 6/16 cases). Mechanical insufflation-exsufflation did not produce serious side effects and was well tolerated by all subjects. CONCLUSIONS: Provision of mechanical insufflation-exsufflation in combination with standard chest physical treatments may improve the management of airway mucous encumbrance in neuromyopathic patients; its use should be included in the noninvasive approach to treatment of respiratory tract infections with impaired mucous clearance.
Adolescent↗
Obstructive sleep apnea syndrome in morbid obesity: effects of intragastric balloon.
STUDY OBJECTIVES: In obese patients, obstructive sleep apnea syndrome (OSAS) is attributed to a reduction in pharyngeal cross-sectional area due to peripharyngeal fat deposition. The effect of weight loss on the size of the upper airways of obese subjects is still unknown. We analyzed the pharyngeal cross-sectional area before and after weight loss in morbidly obese patients with OSAS. DESIGN, SETTING, AND SUBJECTS: A group of 17 middle-aged, morbidly obese men was evaluated by anthropometry and cardiorespiratory sleep studies before and after weight loss obtained by insertion of an intragastric balloon. The pharyngeal cross-sectional area was measured by acoustic pharyngometry. RESULTS: The mean (+/- SD) body mass index was 55.8 +/- 9.9 kg/m2 at baseline and 48.6 +/- 11.2 kg/m2 at the time of balloon removal (6 months after insertion) [p < 0.001]. At baseline, patients had visceral obesity, large necks, and severe OSAS. Weight loss was associated with a significant mean reduction of waist circumference (156.4 +/- 17.6 vs 136.7 +/- 18.4 cm, respectively; p < 0.001), sagittal abdominal diameter (37.8 +/- 3.0 vs 32.3 +/- 4.0 cm, respectively; p < 0.001), and neck circumference (51.1 +/- 3.7 vs 47.9 +/- 4.3 cm, respectively; p < 0.001). Moreover, weight loss induced a nearly complete resolution of OSAS (apnea-hypopnea index, 52.1 +/- 14.9 vs 14.0 +/- 12.4 events/h, respectively; p < 0.001). At baseline, obese patients had significantly lower pharyngeal cross-sectional areas compared to a group of 20 nonobese male control subjects, both in the upright and supine position, at different levels of the pharynx. In obese patients, the weight loss induced by the positioning of the intragastric balloon was associated with an increase in the size of the upper airway passage. After weight loss, both the mean pharyngeal cross-sectional area and the area at glottis level were still lower in obese subjects than in nonobese subjects; however, the pharyngeal cross-sectional area at the oropharyngeal junction was similar in the two groups. CONCLUSIONS: Morbidly obese men with OSAS have a reduced pharyngeal cross-sectional area. A weight reduction of about 15% of baseline body weight may substantially increase the pharyngeal cross-sectional area and substantially improve the severity of OSAS in morbidly obese subjects with sleep apnea.
Adult↗
Pneumothorax associated with long-term non-invasive positive pressure ventilation in Duchenne muscular dystrophy.
Long-term non-invasive positive pressure ventilation (NPPV) is effective for improving the quality of life and survival of neuromuscular patients. This treatment is usually easy to administer and severe complications have rarely been reported. We describe two cases in which the development of pneumothorax was associated with long-term NPPV administered to Duchenne muscular dystrophy patients. Given the wide scale utilization of chronic NPPV, we suggest that care-givers and patients be made aware of this serious complication.
Adult↗
Assessment of physiologic variables and subjective comfort under different levels of pressure support ventilation.
STUDY OBJECTIVES: To evaluate the effects of 12 ventilator settings (pressure support ventilation [PSV] plus positive end-expiratory pressure [PEEP], 30 + 0 cm H(2)O; 25 + 5 cm H(2)O; 25 + 0 cm H(2)O; 20 + 5 cm H(2)O; 20 + 0 cm H(2)O; 15 + 5 cm H(2)O; 15 + 0 cm H(2)O; 10 + 5 cm H(2)O; 10 + 0 cm H(2)O; 5 + 5 cm H(2)O; 5 + 0 cm H(2)O; and 0 + 5 cm H(2)O) on physiologic variables; the percentage of ineffective efforts; patient comfort; and whether the diagnosis of COPD may influence results. DESIGN: Prospective, randomized, physiologic study. SETTING: Three weaning centers. PATIENTS: Thirty-six consecutive patients (20 patients with COPD). INTERVENTION: Patients were randomly submitted to the 12 settings. MEASUREMENTS AND RESULTS: Breathing pattern, respiratory drive (p0.1), arterial oxygen saturation (Sato(2)), heart rate, percentage of ineffective efforts per minute, patient comfort measured by means of a visual analogue scale (VAS), and BORG scale were recorded under each setting. Under different levels of assistance, breathing pattern, Sato(2), and p0.1 significantly and linearly changed (p < 0.0001) while VAS and BORG scale presented a significant (p = 0.027) U-shaped trend; high or low assistance caused the most discomfort. Under high levels of assistance, a higher (analysis of variance, p = 0.023) frequency of ineffective effort percentage was observed in the subgroup of 26 patients who presented this phenomenon. Breathing pattern significantly (p = 0.013) changed when compared to PSV alone (PSV plus zero end-expiratory pressure [ZEEP]) at the same total inspiratory pressure assistance (PSV plus PEEP). A huge variability among patients in breathing pattern and comfort was found under the setting rated as the most comfortable by patients. The diagnosis of COPD did not influence the overall results. CONCLUSIONS: The following conclusions are made: (1) physiologic variables followed a linear trend, while comfort followed a U-shaped trend under different levels of PSV (irrespective of COPD diagnosis); (2) high assistance caused an increase in ineffective efforts; (3) only the breathing pattern significantly changed when total assistance was given as PSV plus PEEP when compared to PSV alone (PSV plus ZEEP); and (4) the extreme levels of PSV are not associated with the best comfort.
Aged↗
Safety and efficacy of ibuprofen versus indomethacin in preterm infants treated for patent ductus arteriosus: a randomised controlled trial.
UNLABELLED: Indomethacin (INDO) and, more recently, ibuprofen (IBU) have been used to treat haemodynamically significant patent ductus arteriosus (PDA) in preterm infants. Both are cyclo-oxygenase blockers, but seem to have a different influence on regional circulation. In a prospective, randomised, controlled study, we compared INDO and IBU with regard to efficacy and safety for the early non-invasive treatment of PDA. Doppler echocardiography was used to study 232 preterm infants (gestational age 23-34 weeks) with respiratory distress syndrome of whom 175 had persistent, haemodynamically significant PDA at 48-72 h of life. They were randomised to receive three intravenous doses of either INDO (0.2 mg/kg, at 12 h intervals) or IBU (a first 10 mg/kg dose followed by two doses of 5 mg/kg at 24 h intervals), recording rate of ductal closure, need for additional treatment, side-effects and clinical course. The efficacy of the pharmacological treatment was similar in the two groups (56/81, 69% INDO; 69/94, 73% IBU). Patients treated with INDO showed a significant increase in serum creatinine (89 +/- 24 versus 82 +/- 20 mmol/l, P = 0.03) and a near-significant tendency for a lower fractional excretion of sodium (3 +/- 3 versus 4 +/- 2%, P = 0.08); moreover, 12/81 (15%) INDO patients versus 1/94 (1%) IBU patients became oliguric (< 1 ml/kg per h) during treatment (P = 0.017). CONCLUSION: Our findings confirm that, by comparison with indomethacin, ibuprofen has fewer effects on renal function in terms of urine output and fluid retention, with much the same efficacy and safety in closing patent ductus arteriosus in preterm infants with respiratory distress syndrome. In particular, no increased incidence of intracranial haemorrhage was observed after ibuprofen treatment.
Analysis of Variance↗
Where to perform long-term ventilation.
Given that most VAI prefer to be cared for at home and that their return home is the desire of families, health care professionals, and others concerned with LTMV, the effort to transform prolonged mechanical ventilation from a hospital-centered to a home-centered treatment needs to be continued and further developed. Nevertheless, the future of high-technology home care will undoubtedly be influenced by improvements in quality and containment of costs, as in its current status treatment of VAI at home too often leads to family disruption and presents a dramatically increasing cost burden. Careful selection of patients, closer attention to education and training, and collection of outcome data are all factors that presumably will facilitate the development of better-quality and cost-saving home care.
Chronic Disease↗