Sleep-related breathing disorder in Duchenne muscular dystrophy: Disease spectrum in the paediatric population

Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disease with death usually occurring because of respiratory failure. Signs of early respiratory insufficiency are usually first detectable in sleep. Objective: To study the presentation of sleep-related breathing disorder (SRBD) in patients with DMD. Method:> A retrospective review of patients with DMD attending a tertiary paediatric sleep disorder clinic over a 5-year period. Symptoms, lung function and polysomnographic indices were reviewed. Results: A total of 34 patients with DMD were referred for respiratory assessment (1-15 years). Twenty-two (64%) reported sleep-related symptomatology. Forced vital capacity (FVC) was between 12 and 107% predicted (n = 29). Thirty-two progressed to have polysomnography of which 15 were normal studies (median age: 10 years) and 10 (31%) were diagnostic of obstructive sleep apnoea (OSA) (median age: 8 years). A total of 11 patients (32%) showed hypoventilation (median age: 13 years) during the 5-year period and non-invasive ventilation (NIV) was offered to them. The median FVC of this group was 27% predicted. There was a significant improvement in the apnoea/hypopnoea index (AHI) (mean difference = 11.31, 95% CI = 5.91-16.70, P = 0.001) following the institution of NIV. Conclusions: The prevalence of SRBD in DMD is significant. There is a bimodal presentation of SRBD, with OSA found in the first decade and hypoventilation more commonly seen at the beginning of the second decade. Polysomnography is recommended in children with symptoms of OSA, or at the stage of becoming wheelchair-bound. In patients with the early stages of respiratory failure, assessment with polysomnography-identified sleep hypoventilation and assisted in initiating NIV.

Heat transfer and entropy generation optimization of forced convection in a porous-saturated duct of rectangular cross-section

We investigate analytically the first and the second law characteristics of fully developed forced convection inside a porous-saturated duct of rectangular cross-section. The Darcy-Brinkman flow model is employed. Three different types of thermal boundary conditions are examined. Expressions for the Nusselt number, the Bejan number, and the dimensionless entropy generation rate are presented in terms of the system parameters. The conclusions of this analytical study will make it possible to compare, evaluate, and optimize alternative rectangular duct design options in terms of heat transfer, pressure drop, and entropy generation. (c) 2006 Elsevier Ltd. All rights reserved.

Analytical solution of forced convection in a duct of rectangular cross-section saturated by a porous medium

A theoretical analysis is presented to investigate fully developed (both thermally and hydrodynamically) forced convection in a duct of rectangular cross-section filled with a hyper-porous medium. The Darcy-Brinkman model for flow through porous media was adopted in the present analysis. A Fourier series type solution is applied to obtain the exact velocity and temperature distribution within the duct. The case of uniform heat flux on the walls, i.e. the H boundary condition in the terminology of Kays and Crawford [1], is treated. Values of the Nusselt number and the friction factor as a function of the aspect ratio, the Darcy number, and the viscosity ratio are reported.

Entropy generation for forced convection in a porous saturated circular tube with uniform wall temperature

A numerical study is reported to investigate both the First and the Second Law of Thermodynamics for thermally developing forced convection in a circular tube filled by a saturated porous medium, with uniform wall temperature, and with the effects of viscous dissipation included. A theoretical analysis is also presented to study the problem for the asymptotic region applying the perturbation solution of the Brinkman momentum equation reported by Hooman and Kani [1]. Expressions are reported for the temperature profile, the Nusselt number, the Bejan number, and the dimensionless entropy generation rate in the asymptotic region. Numerical results are found to be in good agreement with theoretical counterparts.

Thermally developing Brinkman–Brinkman forced convection in rectangular ducts with isothermal walls

The Extended Weighted Residuals Method (EWRM) is applied to investigate the effects of viscous dissipation on the thermal development of forced convection in a porous-saturated duct of rectangular cross-section with isothermal boundary condition. The Brinkman flow model is employed for determination of the velocity field. The temperature in the flow field was computed by utilizing the Green’s function solution based on the EWRM. Following the computation of the temperature field, expressions are presented for the local Nusselt number and the bulk temperature as a function of the dimensionless longitudinal coordinate. In addition to the aspect ratio, the other parameters included in this computation are the Darcy number, viscosity ratio, and the Brinkman number.

Effects of viscous dissipation and boundary conditions on forced convection in a channel occupied by a saturated porous medium

Forced convection with viscous dissipation in a parallel plate channel filled by a saturated porous medium is investigated numerically. Three different viscous dissipation models are examined. Two different sets of wall conditions are considered: isothermal and isoflux. Analytical expressions are also presented for the asymptotic temperature profile and the asymptotic Nusselt number. With isothermal walls, the Brinkman number significantly influences the developing Nusselt number but not the asymptotic one. At constant wall heat flux, both the developing and the asymptotic Nusselt numbers are affected by the value of the Brinkman number. The Nusselt number is sensitive to the porous medium shape factor under all conditions considered.

Effects of viscous dissipation and boundary conditions on forced convection in a channel occupied by a saturated porous medium

Forced convection with viscous dissipation in a parallel plate channel filled by a saturated porous medium is investigated numerically. Three different viscous dissipation models are examined. Two different sets of wall conditions are considered: isothermal and isoflux. Analytical expressions are also presented for the asymptotic temperature profile and the asymptotic Nusselt number. With isothermal walls, the Brinkman number significantly influences the developing Nusselt number but not the asymptotic one. At constant wall heat flux, both the developing and the asymptotic Nusselt numbers are affected by the value of the Brinkman number. The Nusselt number is sensitive to the porous medium shape factor under all conditions considered.

Analysis of heat transfer and entropy generation for a thermally developing Brinkman–Brinkman forced convection problem in a rectangular duct with isoflux walls

Heat transfer and entropy generation analysis of the thermally developing forced convection in a porous-saturated duct of rectangular cross-section, with walls maintained at a constant and uniform heat flux, is investigated based on the Brinkman flow model. The classical Galerkin method is used to obtain the fully developed velocity distribution. To solve the thermal energy equation, with the effects of viscous dissipation being included, the Extended Weighted Residuals Method (EWRM) is applied. The local (three dimensional) temperature field is solved by utilizing the Green’s function solution based on the EWRM where symbolic algebra is being used for convenience in presentation. Following the computation of the temperature field, expressions are presented for the local Nusselt number and the bulk temperature as a function of the dimensionless longitudinal coordinate, the aspect ratio, the Darcy number, the viscosity ratio, and the Brinkman number. With the velocity and temperature field being determined, the Second Law (of Thermodynamics) aspect of the problem is also investigated. Approximate closed form solutions are also presented for two limiting cases of MDa values. It is observed that decreasing the aspect ratio and MDa values increases the entropy generation rate.

Oxygen transport capacity in the air-breathing fish, Megalops cyprinoides: compensations for strenuous exercise

Tarpon have high resting or routine hematocrits (Hct) (37.6+/-3.4%) and hemoglobin concentrations (120.6+/-7.3 g 1(-1)) that increased significantly following bouts of angling-induced exercise (51.9+/-3.7% and 142.8+/-13.5 g 1(-1), respectively). Strenuous exercise was accompanied by an approximately tenfold increase in blood lactate and a muscle metabolite profile indicative of a high energy demand teleost. Routine blood values were quickly restored only when this facultative air-breathing fish was given access to atmospheric air. In vitro studies of oxygen transport capacity, a function of carrying capacity and viscosity, revealed that the optimal Hct range corresponded to that observed in fish under routine behaviour. During strenuous exercise however, further increase in viscosity was largely offset by a pronounced reduction in the shear-dependence of blood which conformed closely to an ideal Newtonian fluid. The mechanism for this behaviour of the erythrocytes appears to involve the activation of surface adrenergic receptors because pre-treatment with propranolol abolished the response. High levels of activity in tarpon living in hypoxic habitats are therefore supported by an elevated Hct with adrenergically mediated viscosity reduction, and air-breathing behaviour that enables rapid metabolic recovery. (C) 2002 Elsevier Science Inc. All rights reserved.

Homomeric ring assemblies of eukaryotic Sm proteins have affinity for both RNA and DNA - Crystal structure of an oligomeric complex of yeast SmF

Sm and Sm-like proteins are key components of small ribonucleoproteins involved in many RNA and DNA processing pathways. In eukaryotes, these complexes contain seven unique Sm or Sm-like (Lsm) proteins assembled as hetero-heptameric rings, whereas in Archaea and bacteria six or seven-membered rings are made from only a single polypeptide chain. Here we show that single Sm and Lsm proteins from yeast also have the capacity to assemble into homo-oligomeric rings. Formation of homo-oligomers by the spliceosomal small nuclear ribonucleoprotein components SmE and SmF preclude hetero-interactions vital to formation of functional small nuclear RNP complexes in vivo. To better understand these unusual complexes, we have determined the crystal structure of the homomeric assembly of the spliceosomal protein SmF. Like its archaeal/bacterial homologs, the SmF complex forms a homomeric ring but in an entirely novel arrangement whereby two heptameric rings form a co-axially stacked dimer via interactions mediated by the variable loops of the individual SmF protein chains. Furthermore, we demonstrate that the homomeric assemblies of yeast Sm and Lsm proteins are capable of binding not only to oligo(U) RNA but, in the case of SmF, also to oligo(dT) single-stranded DNA.

Contractions of specific abdominal muscles in postural tasks are affected by respiratory maneuvers

The influence of respiratory activity of the abdominal muscles on their reaction time in a postural task was evaluated. The electromyographic (EMG) onsets of the abdominal muscles and deltoid were evaluated in response to shoulder flexion initiated by a visual stimulus occurring at random throughout the respiratory cycle. Increased activity of the abdominal muscles was produced by inspiratory loading, forced expiration below functional residual capacity, and a static glottis-closed expulsive maneuver. During quiet breathing, the latency between activation of the abdominal muscles and deltoid was not influenced by the respiratory cycle. When respiratory activity of the abdominal muscles increased, the EMG onset of transversus abdominis and internal oblique, relative to deltoid, was significantly earlier for movements beginning in expiration, compared with inspiration [by 97-107 ms (P < 0.01) and 64-90 ms (P < 0.01), respectively]. However, the onset of transversus abdominis EMG was delayed by 31-54 ms (P < 0.01) when movement was performed during a static expulsive effort, compared with quiet respiration. Thus changes occur in early anticipatory contraction of transversus abdominis during respiratory tasks but they cannot be explained simply by existing activation of the motoneuron pool.