undertaking rare disease research and surveillance dr chris verity, consultant paediatric...
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Undertaking rare disease research and surveillance
Dr Chris Verity, Consultant Paediatric Neurologist
#raredisease
Whooping cough
vaccination
Pertussis notifications and vaccine uptake 1940 - 2005
0
50,000
100,000
150,000
200,00019
40
1945
1950
1955
1960
1965
1970
1975
1980
1985
1990
1995
2000
2005
years
noti
fica
tion
s of
pe
rtus
sis
0
20
40
60
80
100
vacc
ine
cove
rage
notifications coverage
Pertussis immunisation and serious acute neurologicalillness in children
D L Miller, E M Ross, R Alderslade, M H Bellman, N S B Rawson BMJ 1981, 282, 1595-9
• 1000 cases notified to the National Childhood Encephalopathy Study between 1976 and 1979. Diagnoses included encephalitis/encephalopathy, prolonged convulsions, infantile spasms, and Reye syndrome.
• Conclusion: pertussis vaccine not the cause of “brain damage”.
1986
The British Paediatric Surveillance Unit
The epidemiology of progressive intellectual and neurological deterioration in childhood
Verity CM, Winstone AM, Stellitano L, Will R, Nicoll A
Arch Dis Child 2010;95:361-4
The PIND StudyA spectrum of
neurodegenerative diseases
Searching for a needle in a haystack
Progressive Intellectual and Neurological Deterioration (PIND)
Case definition
Child 16 years or less at onset with:• progressive deterioration > 3months• loss of intellectual/developmental abilities• abnormal neurological signs
Even if diagnosis already made
PIND Study – 3445 reported casesMay 1997 – July 2013
No case, 1450
Under investigat-ion, 231
No diagnosis,
168
vCJD, 6
Definite diagnosis,
1475
Out-standing,
115
Prion protein genotypes in the population
VV, 12%
MV, 51%
MM, 37%
All confirmed vCJD cases: methionine homozygotes (MM)
Methionine/valine genotypes might have longer incubation
vCJD has not gone away
1976
National influenza immunisation programme suspended following reports of Guillain-Barre
syndrome following swine ’flu vaccination
December 2009
British Paediatric Surveillance Unit Report Card
HIV & AIDS
Progressive Intellectual & Neurological Deterioration
Congenital Rubella
Severe Neonatal Hypernatraemia
Guillain-Barré syndrome / Fisher syndrome (UK Only)
CNS Inflammatory Demyelinating Disease
Gonorrhoea, Syphilis, Chlamydia, and Trichomonas infectious
Congenital Syphilis
March 2010 [1003]
CODE No [ ]Nothing to Report Specify in box the number of cases seen
ConclusionsDH England
Children: H1N1 vaccn recorded in 855,378
Our study (whole of UK): 112 GBS/3 FS cases
93/115 cases had preceding infections
2: H1N1 vaccination < 3m before GBS
3: seasonal ‘flu vaccination > 3m before
Outcome better than in studies of adults
Support the BPSU – cut down bureacracy
BPSU surveillance as seen from space