Respiratory function assessment in cooperative patients. Part I. Spiromery and bronchodilator reversibility testing

Assessment of respiratory function is the principal tool in the study of patients with lung diseases, allowing physiopathological alterations to be detected, and the severity of the process, its clinical course, and treatment response to be identified. Nowadays, assessment of respiratory function is among the investigations used by Spanish pediatricians. The Techniques Group of the Spanish Society of Pediatric Pneumology undertook the design of a protocol for the study of pulmonary function in children that would incorporate the most recent published consensus documents on basic pulmonary function assessment (spirometry and bronchodilator reversibility testing) and on airway hyperreactivity evaluation using nonspecific provocation tests. The aim of this protocol is to provide a guide to good clinical practice until new changes, based on scientific evidence, are produced.     

Follow-up in patients with aspartylglucosaminuria. Part II. Adaptive skills

Aspartylglucosaminuria is a lysosomal storage disorder inherited as an autosomal recessive trait. Progressive mental retardation is the main symptom, and the lifespan of the patient is abnormlly short. The adaptive skills and maladaptive behaviour of 110 patients aged from 7 to 56 years were analysed using the Portage scale and the AAMD Adaptive Behaviour Scale Part two. The sample was divided into four groups; school-aged, young adults, adults and middle-aged. All patients needed help in the household and transactions outside the home. The school-aged turned out to be superior and middle-aged inferior to other groups concerning adaptive skills. The school-aged and young adults were quite independent in dressing, toileting, bathing and walking alone near the home. Out-of-home replacement became necessary in the majority in adulthood. Twenty-eight patients (25%) were regarded as having behavioural disturbances and the amount of personal disturbances increased significantly with age.     

Respiratory function in the prune-belly syndrome.

Respiratory function was evaluated in 11 patients with prune-belly syndrome. Nine had evidence of gas trapping and six of restrictive lung disease. These abnormalities of lung function appear to be secondary to the musculoskeletal disorder associated with prune-belly syndrome rather than parenchymal lung disease.     

Respiratory function in patients with thalassaemia major: relation with iron overload.

AIMS: (1) To determine the pattern of respiratory impairment in children with thalassaemia major (TM); (2) to assess the relation between the degree of respiratory impairment and total body iron content. METHODS: Twenty nine TM patients were recruited. All underwent physical examination, standardised pulmonary function tests (spirometry, lung volume, and single breath diffusion capacity for carbon monoxide), and magnetic resonance imaging measurements of the liver. Serum ferritin was measured. The signal intensity ratio of liver to that of paraspinal muscle (T1 weighted sequence) and serum ferritin were used as surrogate index of body iron content. RESULTS: Sixteen boys and 13 girls (median age 14.2 years) were studied. None had clinical evidence of congestive heart failure. Sixteen had normal lung function. Impairment of diffusion capacity (median DL(co) 83.5% predicted) was the most common abnormality, being observed in 34% of patients. Pure restrictive and obstructive ventilatory impairment was found in one and two patients respectively. Five patients had a combination of ventilation and diffusion defects. There was no correlation between the degree of impairment of each respiratory abnormality and body iron content. CONCLUSION: Diffusion impairment was the commonest abnormality found in our cohort of paediatric TM patients. Our data did not support the notion that respiratory function impairment was correlated with body iron content.     

Physical function assessment in patients with advanced cancer

Accepted physical therapy assessment techniques were compared with the Karnofsky Performance Status scores in a group of patients with advanced cancer. Assessment of patients' physical functioning by physical therapy evaluation techniques was highly correlated with the Karnofsky Performance Status score. The KPS has been demonstrated to be a reliable assessment tool and is a good indicator of those patients most likely to maintain independent physical function. Extensive physical therapy measures in this patient population appear to offer no advantage over the KPS as an evaluation tool for studies.     

Respiratory therapy in childhood asthma. Part 1 (author's transl)]

The therapeutic approach in childhood asthma can be substantially supported by various forms of respiratory therapy. Objective improvements and increased well-being have been demonstrated by different authors. The physiology of correct and defective breathing are outlined briefly. The major aim of respiratory therapy is the return to normal breathing patterns including normalized clearing functions of the lung. If successful even moderately severe attacks of bronchial asthma may be averted. Personality changes in the form of increased self-confidence and loss of fear of dyspnoea are important. Part 1 outlines physicial treatment forms such as postural drainage and percussions of the chest.     

Neonatal renal function assessment.

Urine samples from neonates admitted to a special care baby unit were analysed to establish ranges for urinary retinol binding protein, albumin, and total protein concentrations in healthy term and preterm infants and to investigate changes seen in disease states. Urinary excretion of retinol binding protein was greater in preterm infants and was increased in sick infants. This was greater than would be predicted from changes in creatinine excretion with gestational or postconceptional age. Urinary retinol binding protein appeared more sensitive to illness than did urinary albumin or total protein.     

Respiratory function in patients with thalassaemia major: relation with iron overload

Aims: (1) To determine the pattern of respiratory impairment in children with thalassaemia major (TM); (2) to assess the relation between the degree of respiratory impairment and total body iron content. Methods: Twenty nine TM patients were recruited. All underwent physical examination, standardised pulmonary function tests (spirometry, lung volume, and single breath diffusion capacity for carbon monoxide), and magnetic resonance imaging measurements of the liver. Serum ferritin was measured. The signal intensity ratio of liver to that of paraspinal muscle (T1 weighted sequence) and serum ferritin were used as surrogate index of body iron content. Results: Sixteen boys and 13 girls (median age 14.2 years) were studied. None had clinical evidence of congestive heart failure. Sixteen had normal lung function. Impairment of diffusion capacity (median DL_co 83.5% predicted) was the most common abnormality, being observed in 34% of patients. Pure restrictive and obstructive ventilatory impairment was found in one and two patients respectively. Five patients had a combination of ventilation and diffusion defects. There was no correlation between the degree of impairment of each respiratory abnormality and body iron content. Conclusion: Diffusion impairment was the commonest abnormality found in our cohort of paediatric TM patients. Our data did not support the notion that respiratory function impairment was correlated with body iron content.     

Monitoring in non-traumatic coma. Part II: Electroencephalography.

Forty eight comatose children had electroencephalograms (EEG) recorded during the acute phase of their illnesses. These were classified according to a simple grading system and the findings correlated with the presence of seizures, deep coma, minimum cerebral perfusion pressure, and eventual neurological outcome. Serial EEGs proved important, particularly when slow activity was seen initially. None of the 20 patients who showed low amplitude EEG activity or electrocerebral silence at any stage of the acute illness did well. Discharges were seen in only 13 of the 29 patients with seizures and their presence did not correlate with outcome except in five patients with a distinctive pattern of discharges, none of whom had a good outcome. EEG findings associated with poor outcome did not always correlate with the clinical assessment of deep coma, emphasising the difficulties of neurological evaluation in these patients. Five of the patients with cerebral perfusion pressures greater than 42 mm Hg had a poor outcome that was predicted by serial EEGs. In nine patients with a minimum cerebral perfusion pressure in the borderline range 38-42 mm Hg the EEG was useful as an indication of the outcome. The EEG reflects changes in cerebral function which may be due to multifactorial or repeated insults. An EEG is therefore important in both the initial assessment and as an indicator of the neurological outcome, particularly in those patients in whom the cerebral perfusion pressure has apparently been adequate or within the borderline range.     

Respiratory function in the prune-belly syndrome

Respiratory function was evaluated in 11 patients with prune-belly syndrome. Nine had evidence of gas trapping and six of restrictive lung disease. These abnormalities of lung function appear to be secondary to the musculoskeletal disorder associated with prune-belly syndrome rather than parenchymal lung disease.     

Respiratory function after repair of congenital diaphragmatic hernia.

Respiratory function studies were carried out in 22 infants who had successful repair of diaphragmatic herniae of the Bochdalek type. Thoracic gas volume was initially reduced in only 3 of these, but subsequent studies showed that improvement occurred. There were no consistent abnormalities in either dynamic compliance or mean pulmonary conductance. This is evidence that there is rapid adaptation which compensates for any alteration in the parenchymatous tissue in the lungs or abnormalities in the bronchial tree in infants soon after the repair of congenital diaphragmetic herniae. Further studies are necessary to determine the changes in these lungs with growth.     

Respiratory sequelae and lung function after whooping cough in infancy.

The lung function of 31 children, aged 6-13 years, who had whooping cough as infants and 32 control children matched for age, sex, and residence area were compared in a community based cohort study. Family history of obstructive airway disease, smoking habits in the family, atopy, and other background factors examined were similar in the two groups. The ratios of recalled repeated acute respiratory infections did not differ among the groups. Children in the control group were slightly more involved in physical activities. History of obstructive airway disease, findings on chest radiography, and distribution of immunoglobulin concentrations, including IgE, did not differ significantly. Lung function before and after exercise and after inhalation of salbutamol were not different. No impairment of small airways was detected. Our data do not support the hypothesis that whooping cough in itself is a causal factor for later obstructive respiratory disease.