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
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Whooping cough
vaccination
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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
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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”.
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1986
The British Paediatric Surveillance Unit
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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
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Searching for a needle in a haystack
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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
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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
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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
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vCJD has not gone away
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1976
National influenza immunisation programme suspended following reports of Guillain-Barre
syndrome following swine ’flu vaccination
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December 2009
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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
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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
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Support the BPSU – cut down bureacracy
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BPSU surveillance as seen from space