Sickle-cell anaemia

Metadata

Name	Sickle-cell anaemia (sickle_cell)
Category	Haematological or immunological
Sex
Adult	Yes
UUID	02e97f39-3a8c-479e-a744-bbc2df44ab3e
Definition	Sickle cell anemia is an inherited red blood cell disorder in which there aren’t enough healthy red blood cells to carry oxygen throughout the body.

Ontologies

Disease Ontology (DOID)	DOID_10923
GWAS Catalogue	Orphanet_232
MeSH	D000755
SNOMED-CT	127040003
FinnGen	D3_SICKLECELLDIS

Key Summary Statistics

Cases from any source

Total	201
Female (%)	129 (64%)
Male (%)	72 (36%)

Year first recorded

Cases linked in all UK Biobank sources

Cross-source concordance

Vertical bars correspond to number of cases per source/intersection; horizontal bars to left correspond to total number of cases per source.

Incidence & prevalence

Baseline prevalence stratified by sex

Female	2.71 (1.6,3.82)
Male	1.54 (0.63,2.45)

Incidence stratified by sex

Female	0.09 (0.01,0.16)
Male	0.11 (0.01,0.2)

Incidence stratified by age

Ages 40-44	0.2 (-0.08,0.48)
Ages 45-49	0.22 (-0.03,0.47)
Ages 50-54	0.13 (-0.05,0.3)
Ages 55-59	0.16 (-0.02,0.33)
Ages 60-64	0.0 (0.0,0.0)
Ages 65-69	0.0 (0.0,0.0)

Period prevalence

Methods

Comparison with general population

Sex-standardised prevalence from CALIBER study (Kuan et al. 2019)

Population	Ages 40-49	Ages 50-59	Ages 60-69
UK Biobank	3 (2,6)	2 (1,4)	1 (1,2)
CALIBER	3 (2,3)	–	–

Stratified by age and Townsend deprivation status

Deprivation status	Ages 40-49	Ages 50-59	Ages 60-69
Least deprived	1(0,7)	0(NaN,NaN)	0(NaN,NaN)
Low	0(NaN,NaN)	2(0,7)	1(0,3)
Medium	3(1,10)	2(0,6)	0(0,3)
High	2(0,8)	3(1,8)	2(1,6)
Most deprived	9(4,16)	6(2,11)	2(1,6)

Stratified by age and country

Country	Ages 40-49	Ages 50-59	Ages 60-69
England	4(2,7)	3(2,5)	1(0,2)
Scotland	2(0,13)	0(NaN,NaN)	0(NaN,NaN)
Wales	0(NaN,NaN)	2(0,9)	3(0,10)

Definition

UK Biobank field id	Description	Code	Value
20002	Non-cancer illness code, self-reported	1339	sickle cell disease
41202	Diagnoses - main ICD10	D57.0	Sickle-cell anaemia with crisis
41202	Diagnoses - main ICD10	D57.1	Sickle-cell anaemia without crisis
41202	Diagnoses - main ICD10	D57.2	Double heterozygous sickling disorders
41202	Diagnoses - main ICD10	D57.8	Other sickle-cell disorders
41204	Diagnoses - secondary ICD10	D57.0	Sickle-cell anaemia with crisis
41204	Diagnoses - secondary ICD10	D57.1	Sickle-cell anaemia without crisis
41204	Diagnoses - secondary ICD10	D57.2	Double heterozygous sickling disorders
41204	Diagnoses - secondary ICD10	D57.8	Other sickle-cell disorders
40001	Underlying (primary) cause of death: ICD10	D57.0	Sickle-cell anaemia with crisis
40001	Underlying (primary) cause of death: ICD10	D57.1	Sickle-cell anaemia without crisis
40001	Underlying (primary) cause of death: ICD10	D57.2	Double heterozygous sickling disorders
40001	Underlying (primary) cause of death: ICD10	D57.8	Other sickle-cell disorders
40002	Contributory (secondary) causes of death: ICD10	D57.0	Sickle-cell anaemia with crisis
40002	Contributory (secondary) causes of death: ICD10	D57.1	Sickle-cell anaemia without crisis
40002	Contributory (secondary) causes of death: ICD10	D57.2	Double heterozygous sickling disorders
40002	Contributory (secondary) causes of death: ICD10	D57.8	Other sickle-cell disorders
42040	GP clinical event records	1458.00	History of sickle cell anaemia
42040	GP clinical event records	D104211	Sickle-cell thalassaemia
42040	GP clinical event records	D106000	Sickle-cell anaemia of unspecified type
42040	GP clinical event records	D106100	Sickle-cell anaemia with no crisis
42040	GP clinical event records	D106200	Sickle-cell anaemia with crisis
42040	GP clinical event records	D106300	Sickle-cell anaemia with haemoglobin C disease
42040	GP clinical event records	D106400	Sickle-cell anaemia with haemoglobin D disease
42040	GP clinical event records	D106500	Sickle-cell anaemia with haemoglobin E disease
42040	GP clinical event records	D106.00	Sickle-cell anaemia
42040	GP clinical event records	D106z00	Sickle-cell anaemia NOS
42040	GP clinical event records	Dyu1200	[X]Other sickle-cell disorders
42040	GP clinical event records	F422100	Proliferative retinopathy due to sickle cell disease
42040	GP clinical event records	K0G..00	Sickle cell nephropathy
42040	GP clinical event records	X30Kx	Sickle cell nephropathy