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Respiratory function in children with neuromuscular disease.

Respiratory failure is the most common cause of death in children with neuromuscular disease (NMD). This article provides a conceptual framework for understanding failure of the respiratory pump in these children. Pump failure can be attributed to faulty control of respiration, to mechanical defects, and to respiratory muscle fatigue. In most patients with NMD, respiratory drive is intact. Mechanical defects can be due to either an abnormal pump or an excessive respiratory load on which the pump must operate. In patients with NMD, the weak respiratory muscles cannot generate normal respiratory pressures. Furthermore, inefficient chest wall motion leads to increased work for the respiratory system. Abnormalities of lung and chest wall compliance increase respiratory load. The combination of a weak, inefficient respiratory pump and an abnormally high elastic load can predispose these patients to respiratory muscle fatigue. Respiratory muscle rest with nocturnal assisted ventilation, therefore, provides a rational approach to the treatment of chronic respiratory failure in patients with NMD. Areas of future research that may prove useful in the care of children with respiratory failure due to NMD include: the development of better ways of assessing respiratory muscle fatigue; studies to increase understanding of the role of mechanical ventilation in improving chest wall and respiratory pump function; and studies delineating the role of respiratory muscle training in these patients.

Child

[Are there new aspects in thoracic surgery in children?].

A comparison was made between the operations of the respiratory tract in childhood and their associated bronchological examinations were analyzed and also there were other kinds of indication for examination and operation. The surgical therapy of abnormalities has the priority.

Child

Acute respiratory failure following pharmacologically induced hyperventilation: an experimental animal study.

The pulmonary effects of hyperventilation following infusion of sodium salicylate into the cisterna magna was studied in 16 spontaneously breathing adult sheep. We found a fall in PaO2, a decrease in the static compliance of the respiratory system, abnormal chest roentgenographic films, and grossly abnormal lungs following 3.5 to 13 h of hyperventilation. A control group of 15 sheep (10 sheep similarly injected with sodium salicylate, but then sedated and paralyzed and ventilated at normal tidal volume and respiratory rate on a mechanical ventilator, and 5 sheep infused with saline alone and breathing spontaneously) showed no pulmonary or arterial blood gas abnormalities. We conclude that prolonged hyperventilation under the conditions of this experiment precipitated events that resulted in acute lung injury.

Animals

[Stridor in children].

Stridor is the presenting symptom of abnormalities of larynx and trachea in children. The anatomical differences in size and consistency of the larynx play a crucial role in stridor in children as compared with adults. From 1983 to 1988, 81 children (65 males, 16 females) with severe or prolonged stridor were admitted to our hospital. Thorough examination, including endoscopy of larynx and trachea and radiology revealed various congenital abnormalities and acquired conditions as the cause of stridor. Congenital laryngeal anomalies accounted for stridor in 26 children, there were 12 children with congenital tracheal or vascular anomalies, 28 with infectious diseases and 15 with other diseases. Intubation and (or) tracheotomy were performed in one-third of the patients: 5 of them died of severe complications. For proper treatment of children with stridor it is essential to investigate its aetiology carefully.

Child

Respiratory system stability and abnormal carbon dioxide homeostasis.

We have tested the hypothesis that interactions among eight parameters of the respiratory and cardiovascular systems that determine the loop gain (LG) of the respiratory CO2 feedback control system might account for the degree of stability or instability of breathing patterns in healthy sleeping volunteers as well as in familial dysautonomia (FD) and congenital central hypoventilation syndrome (CCHS) patients. The predictability of cycle duration was tested as well. We measured the values of CO2 sensitivity, CO2 delivery capacity in the circulation, circulation delay, mean lung volume for CO2, and mixed venous PCO2 in 8 FD patients, 2 CCHS patients, and 19 healthy controls. The values of these parameters were used in a mathematical model to compute the LG of the respiratory control system during sleep for each epoch of respiration analyzed. The strength of the ventilatory oscillations (R) was quantified using power density spectra of the ventilation time series. All subjects were studied at inspiratory O2 concentrations (FIO2) of 0.21 and 0.15; CCHS patients and controls were also studied at 0.12 FIO2 to examine the effect of steady-state hypoxia on respiratory system stability. In 2 FD patients, LG was elevated at both levels of FIO2 and periodic breathing was observed; the values of R were elevated. Elevated mixed venous PCO2 and reduced CO2 delivery capacity were chiefly responsible for the abnormally high LG observed. In three healthy volunteers, high LG and unstable patterns were associated with high chemosensitivity. The CCHS patients, however, remained stable even at 0.12 FIO2 because LG remained equivalent to zero due to a lack of chemosensitivity.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Is there a place for rigid bronchoscopy in the management of pediatric lung disease?

A review of 364 rigid bronchoscopies in children is presented of whom 55% were younger than 3 years old. The commonest diagnoses in the under 1 year old group were related to congenital anomalies, in the 1-3 year group to inhaled foreign bodies, and in the over 3 year group to bronchiectasis. It is noted that some 30-40% of the tests could have been undertaken with a flexible bronchoscope, but it is suggested that the advantages of the flexible instrument in children with lung disease, as distinct from upper airway disease, are less than in adult practice. Clear benefits for the management of the patient should be obvious before undertaking bronchoscopy in children, and careful consideration should be given to the type of instrument most likely to provide the results desired.

Adolescent

The epidemiology of neonatal respiratory disease.

The epidemiology of many conditions affecting the newborn infant is influenced by the health of the mother, prematurity and the effects of medical and obstetric management. In this review we have considered the role of each of these factors in seven respiratory conditions presenting in the newborn infant.

Apnea