Respiratory insufficiency in neuronopathic and neuropathic disorders

Twenty-nine patients with a neuronopathic or neuropathic disorder were referred for assessment of respiratory insufficiency between 1978 and 1994. Diagnoses included spinal muscular atrophy (6), chronic idiopathic demyelinating neuropathy (4), Vialetto-van Laere syndrome (3), hereditary motor and sensory neuropathy (3) and a miscellaneous group (5). We also describe seven patients with Guillain-Barre syndrome (GBS) who required long-term ventilatory support for over 6 months to 7 years after the initial illness. Respiratory insufficiency occurred as a consequence of respiratory muscle weakness, impaired bulbar function and restrictive lung defects. In some groups presentation was with progressive nocturnal hypoventilation culminating in acute respiratory failure. Five patients with GBS or chronic idiopathic demyelinating neuropathy were weaned from ventilatory support up to 18 months after the initial illness. The remaining 24 patients required continuous or nocturnal ventilatory support using intermittent positive-pressure ventilation (13), negative pressure ventilation (4), nasal-mask-delivered intermittent positive-pressure ventilation (4), nasal-mask-delivered continuous positive-pressure ventilation (3), mouthpiece-assisted ventilation by day (2) and rocking bed (1). None have been weaned from support after a period of ventilation ranging from one month to 10 years. Eight patients have subsequently died.      

A fuzzy logic model for differential diagnosis of lower urinary tract dysfunctions

Lower urinary tract dysfunctions (LUTD) restrict quality of life, resulting in decreased work productivity and emotional well‐being. However, most people are not diagnosed because they do not seek medical treatment. In addition, some facilities do not adequately train health professionals in the evaluation, diagnosis and treatment of these conditions. The study's objective was to develop a decision support system modelled on fuzzy logic that defines LUTD using the terminology of the International Continence Society. This methodological study aimed to develop a model that uses the maximum–minimum composition (max–min) of fuzzy relations that can perform differential diagnoses of LUTD. The model was tested in 100 cases (50 men and 50 women), and the data were obtained from medical records containing the clinical data and results of urodynamic studies. All medical records were reviewed by a specialist in urology. The model was capable of determining a diagnosis in full (62%) or partial (36%) agreement with the medical report. Agreement between the model and the medical report was excellent (kappa = 0·98, p ? 0·001, CI = 0·88–1) or substantial (kappa = 0·53, p ? 0·001, CI = 0·45–0·60), considering overestimative accordance (where accordance is assumed when at least one diagnosis is equal) and underestimative accordance (where accordance is assumed when all diagnoses are equal), respectively. The proposed model based on the max–min composition of fuzzy relationships is very simple and performed well. However, more tests are recommended before the model is used as a decision support system.     

Chronic treatment with tempol does not significantly ameliorate renal tissue hypoxia or disease progression in a rodent model of polycystic kidney disease

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Cycling efficiency is not compromised for moderate exercise in moderately severe COPD

INTRODUCTION/PURPOSE: Hyperpnea and hyperinflation have been proposed as contributors to exaggerated energy demands in chronic obstructive pulmonary disease (COPD), yet there are incomplete data on exercise requirements. This study compared total-body energy demands of the internal (unloaded) and external work of cycling and delta mechanical efficiency in 40 patients (FEV1: 36+/-14% predicted) with COPD and 28 healthy age-matched controls while characterizing dynamic hyperinflation. METHODS: Steady-state V O2 was obtained at rest, during unloaded and 20-W cycling, and at 20, 50, and 65% peak cycling power. Delta mechanical efficiency was calculated between constant-load cycling at 65 and 20% peak power. Dynamic hyperinflation was assessed from inspiratory capacity maneuvers. RESULTS: Oxygen demands (L.min) at rest, for internal work (0.47+/-0.14 vs 0.45+/-0.11) or external work at 20 W (0.62+/-0.20 vs 0.57+/-0.13), were not different between patients and controls, although ventilation was elevated in COPD. Cycling at 65% of peak power induced dynamic hyperinflation in COPD, which indices were not related to cycling efficiency. Delta efficiency (%) was not different between patients (26.3+/-8.1) and controls (24.8+/-4.0). CONCLUSION: Findings suggest that bioenergetics of submaximal cycling is not compromised in moderately severe COPD despite tachypnea and dynamic hyperinflation.