What question did this study set out to answer?

To determine the incidence, clinical characteristics, and early progression of liver disease among newborns with alpha1-antitrypsin deficiency.

June 10, 1976New England Journal of Medicine

Liver Disease in Alpha₁-Antitrypsin Deficiency Detected by Screening of 200,000 Infants

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Authors

TSTomas SvegerLund University

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Implication

Prospective screening study reveals high rates of early hepatic dysfunction in infants with alpha-1-antitrypsin deficiency, indicating widespread covert liver involvement.

Key Points

To determine the incidence, clinical characteristics, and early progression of liver disease among newborns with alpha1-antitrypsin deficiency.
Prospectively screened 200,000 newborns to detect alpha1-antitrypsin deficiency phenotypes.
Identified and monitored 120 Pi Z, 48 Pi SZ, two Pi Z-, and one Pi S- infants through clinical evaluations and liver-function tests up to six months of age.
Prolonged obstructive jaundice developed in 14 of 120 (11.7%) Pi Z infants, while 8 others had minimal enzyme abnormalities and hepatosplenomegaly; all 22 appeared clinically healthy by six months.
Asymptomatic hepatic dysfunction was frequent, presenting with abnormal liver-function tests in 44 of 84 (52.4%) Pi Z infants at three months and 36 of 60 (60.0%) at six months, as well as 10 of 42 (23.8%) Pi SZ infants at three months.
Infants with clinical liver disease were significantly more likely to be small for gestational age than those without overt disease (P < 0.001).

Cite This Study

Tomas Sveger (1976) studied this question.

synapsesocial.com/papers/69fa16844b0a0fc8eb6401be https://doi.org/10.1056/nejm197606102942404

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Implication

Prospective screening study reveals high rates of early hepatic dysfunction in infants with alpha-1-antitrypsin deficiency, indicating widespread covert liver involvement.

Liver Disease in Alpha₁-Antitrypsin Deficiency Detected by Screening of 200,000 Infants

Discussion

Member takes

Implication

Key Points

Cite This Study

Liver Disease in Alpha₁-Antitrypsin Deficiency Detected by Screening of 200,000 Infants

Discussion

Member takes

Implication

Key Points

Cite This Study

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