Cystic fibrosis important dates Flashcards
About how many patients in the UK currently have CF
About 9,000 patients in the UK
What happened when it was first characterised
The mortality rate was extremely high with very few making it past infancy to childhood due to pneumonia and malnutrition
What was the median predicted survival and how has it changed from 2009 to 2013
Median survival was 34.4 in 2009 and in 2013 36. The study of cystic fibrosis (CF) provides a fascinating insight into advances in medicine in the 20th century.
What were the three findings by Dorothy Anderson in 1938
She described in great detail the clinical and autopsy findings of 49 children,
Respiratory Conditions
Intestinal obstruction
Pancreatic changes
RIP Kids
The epithelial metaplasia seen in vitamin A deficiency was also present in half of the infants and this was thought to be what predisposed patients to bronchiectasis
She discussed the relationship with meconium ileus and described approaches to treatment, including administration of pancreatic enzymes.
In 1936 it had been earlier identified in Europe by Franconi
What were 1940 treatments
Sulphonamides in the early stages of infection and penicillin in the later stages other antibiotics were used. Most children became infected with staph aureus, which responded to small frequent doses of aerosolised penicillin or IM penicillin.
The nutritional treatment involved a high protein diet, relatively crude pancreatic extract with meals, and IM vitamin A which Anderson A believed was the cause of the respiratory problems. These decreased the mortality of patients with CF however, many children failed to reach 7 years
Who was Di Sant ‘Agnese
In 1953 there was a heatwave in New York. Perceptive physicians noted the relative excess of children with CF who presented with heat prostration. Di Sant’Agnese realised the significance and discovered the excess of sodium and chloride in the sweat which became the
cornerstone of the diagnostic test and pointed the way to the study of exocrine glands and secretory cells.
Di Sant’Agnese stimulated Gibson and Cooke to publish a standardised way of sweat collection by pilocarpine sweat test which became the gold standard for CF and it is still used today.
1950 who discovered the exercise?
Winifred young a physician in London in recommended postural draining for children with CF
What was set up in 1950?
The Cleveland centre also set up a committee to provide a treatment programme for CF which included early diagnosis by sweat test, regular physiotherapy, frequent follow-up, antibiotics, and using phenylephedrine for decongestant. This programme was so successful and mortality rate went from 10% to 2%. This was influential in 1960 development
What are the main findings from 1950?
Heat-wave Paul di Sant Agnese- heat intolerance Sweat electrolytes test Gibson and Cooke CERDRIC CARTER 1953 ESTABLISHED IT IS A RECESSIVE GENE (25%) Postural Draining Winifred young Cleveland centre
1960s
Mist tent- as mucolytics, so believe the iodine was good to aerate
Organisations - promote and facilitate research
Introduction of pancrease which caused a revolution in the enzyme and diet management -acid resistant microspheres
HIGH FAT TO REVERSE MALNOURISHMENT - reverse from high protein
STEROIDS
Pseudomonas was identified as a pathogen
1970
Reversal of mist tent
Spread of pseudomonas and B cepacia
Introduction of CF camps in the UK to improve psychosocial opportunities for children
1980
In 1981 Margaret Hodson - gentamicin and nebulised carbenicliiln
In 1983 Quinton described it is due to chloride channel defect
James Littlewood in 1985 - Early infection with pseudomonas was eradicated with nebulised colistitin (a polymyxin effective gram negatives)
HOME IV TREATMENT AND HEART LUNG TRANSPLANT AS FUNCTION DECLINES
In 1989 JOHN RIORDAN CLONED CFTR
In 2018
Novochem therapeutics will introduce NO releasing polymer effective against 9 micro-organisms in CF
1960 what bug was identified
Pseudomonas
When did James little wood discover colistitin a polymixin effective against gram negative
1985
Published important benefit of nebulised carbenecilin and gentamicin
1980
When did Gibson and Cook discover the pilocarpine sweat test?
Development of the Gibson and Cooke sweat test in 1959 was a milestone in the diagnosis of CF.
Since then the measurement of sweat electrolytes by pilocarpine iontophoresis has served as the main diagnostic test for CF. The sweat test is a reliable test for the diagnosis of CF in approximately 98% of patients with CF.
Pilocarpine iontophoresis has served as the main diagnostic test for CF
All thanks to Gibson and Cooke
How reliable is the pilocarpine iontophoresis test?
The sweat test is a reliable test for the diagnosis of CF in approximately 98% of patients with CF
