Survival in the Swedish cohort with alpha-1-antitrypsin deficiency, up to the age of 43–45 years

Figures & data

Table 1 Demographic data of the study population

Characteristics	Total N=184	PiZZ and PiZnull N=129	PiSZ and PiSnull N=55
Men, n (%)	106 (58)	76 (59)	30 (55)
Smoking habits
Never, n (%)	127 (69)	93 (72)	38 (69)
Former, n (%)	30 (16)	26 (20)	7 (13)
Current, n (%)	11 (6)	5 (4)	6 (11)
Unknown	16 (9)	5 (4)	4 (7)
Deaths, n (%)	11 (6)	7 (5)	4 (7)

Table 2 SMRs of a cohort of severe (PiZZ) and moderate (PiSZ) AAT-deficient newborn infants was identified by neonatal screening

	No	Observed/expected	SMR (95% CI)
PiZZ and PiZnull	129	7/3.66	1.91 (0.77–3.94)
PiSZ	55	4/1.53	2.61 (0.71–6.71)
Men	106	6/3.52	1.71 (0.63–3.72)
Women	78	5/1.67	3.00 (0.97–7.00)

Abbreviations: AAT, Alpha-1-antitrypsin; SMR, standardized mortality ratio.

Table 3 Characteristics, previous liver disease, and causes of death in eleven deceased individuals

No	Sex	Pi	Smoking habits	Age at death	Liver disease	Cause of death
						Underlying	Immediate
1	Female	ZZ	–	0.16 years (2 months)	Hepatomegaly, liver cirrhosis	Atrium septum defect, liver cirrhosis	Osteomyelitis, septicemia
2	Male	ZZ	–	3.7 years	Hepatomegaly, liver cirrhosis	Aplastic anemia	Bleeding diathesis
3	Male	ZZ	–	5.6 years	Hepatomegaly, liver fibrosis		Traffic accident
4	Female	ZZ	–	7.1 years	Hepato-splenomegaly	Ascetic liver failure	Coma hepatica
5	Male	ZZ	–	7.7 years	Not known	IgG deficiency	Septicemia
6	Female	ZZ	–	36 years	No	Mild thanatophoric dysplasia	Pneumonia, septicemia
7	Male	ZZ	Former	42 years	Liver cirrhosis	Liver cirrhosis, esophagus varices	Septicemia, liver failure
8	Male	SZ	–	0.28 years (3 months)	Icterus		Sudden infant death syndrome
9	Male	SZ	Former	36 years	Fatty liver at autopsy		Methadone overdosea
10	Female	SZ	Current	38 years	Hepatomegaly and fatty liver at autopsy		Acute myocarditis
11	Female	SZ	Current	39 years	Alcohol-induced liver cirrhosisa	Liver failure	Septicemia

Note:

a The patient had drug and alcohol abuse.