Improving asthma documentation in a paediatric emergency department.

OBJECTIVE: To improve documentation for children presenting to the Emergency Department (ED) of The Children's Hospital with acute asthma. METHODOLOGY: In phase I, the documentation process was analysed using a standard total quality management (TQM) approach to identify specific problems leading to poor documentation. Fifty-two medical records of children presenting over a 3 week period were reviewed for nursing and medical documentation. A set of minimum criteria, consistent with the Paediatric Asthma Management Plan, were established for documentation by both medical and nursing staff. Following dissemination and education, compliance with documentation was evaluated and compared to an asthma survey performed in the ED in 1991. In phase II, a specific proforma for medical assessment was developed and 80 medical records of children presenting over a 3 week period were reviewed. Fifty-two (65%) with completed proformas were evaluated. The outcome measure was the documentation rate for minimum criteria established by TQM process. RESULTS: In phase I, nursing compliance with documentation ranged from 46% for signs of respiratory distress to 83% for a past history of asthma and 100% for pulse rate. Doctors were similarly poor at documenting essential elements such as severity (31%), palpable pulsus paradoxus (29%), the child's usual doctor (46%) and follow-up arrangements (21-56%). In phase II, the documentation of the severity of acute asthma (42%) and of the child's usual doctor (42%) remained poor but there were statistically significant improvements in documentation of interval medications, palpable pulsus paradoxus, respiratory rate, pre-treatment oximetry, education, follow-up arrangements and communication letters. CONCLUSION: The process of TQM has proved valuable in improving some aspects of documentation of children presenting to ED with acute asthma. It remains to be shown whether improved documentation will result in improved outcome.

Genetic determination of exocrine pancreatic function in cystic fibrosis.

We showed elsewhere that the pancreatic function status of cystic fibrosis (CF) patients could be correlated to mutations in the CF transmembrane conductance regulator (CFTR) gene. Although the majority of CF mutations--including the most common, delta F508--strongly correlated with pancreatic insufficiency (PI), approximately 10% of the mutant alleles may confer pancreatic sufficiency (PS). To extend this observation, genomic DNA of 538 CF patients with well-documented pancreatic function status were analyzed for a series of known mutations in their CFTR genes. Only 20 of the 25 mutations tested were found in this population. They accounted for 84% of the CF chromosomes, with delta F508 being the most frequent (71%), and the other mutations accounted for less than 5% each. A total of 30 different, complete genotypes could be determined in 394 (73%) of the patients. The data showed that each genotype was associated only with PI or only with PS, but not with both. This result is thus consistent with the hypothesis that PI and PS in CF are predisposed by the genotype at the CFTR locus; the PS phenotype occurs in patients who have one or two mild CFTR mutations, such as R117H, R334W, R347P, A455E, and P574H, whereas the PI phenotype occurs in patients with two severe alleles, such as delta F508, delta I507, Q493X, G542X, R553X, W1282X, 621 + 1G----T, 1717-1G----A, 556delA, 3659delC, I148T, G480C, V520F, G551D, and R560T.

Infantile hepatic haemangioma: investigation and treatment.

Infantile hepatic haemangiomas are benign tumours which tend to regress in late infancy. We describe five infants with hepatic haemangioma, three of whom developed congestive cardiac failure. Technetium-99m labelled red blood cell scan was useful in the diagnosis and follow-up of the lesion. The three symptomatic infants were treated with prednisone (2 mg/kg per day) for 2 months in addition to digoxin and diuretics. Significant clinical improvement occurred within 3 weeks in each case. We conclude that the red blood cells scan is a good non-invasive diagnostic test and recommend corticosteroids as initial management for symptomatic infantile hepatic haemangiomas.

Chronic undernutrition/growth retardation in cystic fibrosis.

There is now sufficient evidence to prove that the maintenance of a high energy normally balanced diet prevents malnutrition/growth retardation and may well enhance prognosis in patients with CF. Most patients are able to tolerate the normal to high fat content without undue problems with steatorrhoea and with the recent advent of more effective enzyme replacement therapy, this should be even less of a problem. Conversely, there is an equally large bulk of evidence to indicate that the maintenance of a low fat diet, while controlling symptoms from steatorrhoea in some patients, is energy depriving and produces growth failure. In children or teenagers who are presently growth retarded, installation of a high energy intake may improve growth in some, but not in others. Further investigation of the latter patients is required to evaluate their persistent anorexia and advantages and disadvantages of nutritional supplementation by invasive techniques. Certainly growth and wellbeing can be vastly improved. However, such studies may well have to be multicentered to obtain sufficient patients to control for the many variables involved, in order to demonstrate the effects on pulmonary function.