Increased Risk of Cholesteatoma in Individuals With Alpha-1 Antitrypsin Deficiency: A Cohort Study. [PDF]
Patients with AATD had a 3.6‐fold increased risk of cholesteatoma surgery (HR: 3.62) compared to controls. These findings suggest that AATD may contribute to the development of cholesteatoma. ABSTRACT Objective To estimate the risk of cholesteatoma in patients with alpha‐1 antitrypsin deficiency (AATD) compared to the general population using time‐to ...
Ali A, Ravn HA, Dahl M, Djurhuus BD.
europepmc +2 more sources
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare, smoking related, progressive diffuse cystic lung disease that occurs primarily in smokers. The aim of this study was to determine if there was an increase in alpha-1 antitrypsin deficient alleles ...
Cormac McCarthy +6 more
doaj +1 more source
Liver Transplantation for Advanced Liver Disease with Alpha-1antitrypsin Deficiency [PDF]
ALPHA-1-antitrypsin deficiency associated with chronic obstructive airway disease was recognized in 1963 by Laurell and Ericksson.1 In 1969, Sharp2 described the first cases of alpha-1-antitrypsin-deficiency disease in children with cirrhosis. Since then,
Allan G. Redeker +26 more
core +1 more source
AASLD practice guidance on drug, herbal, and dietary supplement–induced liver injury
Hepatology, EarlyView.
Robert J. Fontana +6 more
wiley +1 more source
Alpha-1 antitrypsin deficiency
Alpha-1 antitrypsin deficiency (AATD) is a rare hereditary condition caused by decreased plasma and tissue levels of alpha-1 antitrypsin (AAT) that can lead to serious lung and liver disease in children and adults. AATD patients face challenges such as under diagnosis, clinical variability, and limited treatment options for liver disease.
+9 more sources
Case-finding for alpha1-antitrypsin deficiency in Kazakh patients with COPD
Background Alpha-1-antitrypsin deficiency (AATD) is an under-diagnosed condition in patients with chronic obstructive pulmonary disease (COPD). The aim of this study was to screen for AATD in Kazakh patients with COPD using dried blood spot specimens ...
Ardak Zhumagaliyeva +12 more
doaj +1 more source
Alpha-1 Antitrypsin Deficiency: Home Therapy
While available in only a few countries, home therapy is a possible strategy for the treatment of alpha-1 antitrypsin deficiency. We want to describe our experience in the management of human alpha-1 antitrypsin using home care intravenous augmentation ...
Anna Annunziata +5 more
doaj +1 more source
REVERSAL OF HEPATIC ALPHA-1-ANTITRYPSIN DEPOSITION AFTER PORTACAVAL SHUNT [PDF]
End-to-side portacaval shunts were carried out in three children with the liver disease of alpha-1-antitrypsin deficiency and complications of portal hypertension. Their clinical courses have been stable for 31/2 to almost 7 years.
Aagenaes +17 more
core +1 more source
Leukocyte adhesion and recruitment, and alpha-1-antitrypsin deficiency: a report from ATS 2001, May 18-23, San Francisco [PDF]
The program at this year's American Thoracic Society international conference included over 300 scientific and clinical symposia. In this report I have reviewed the data presented on two important areas of lung inflammation, namely leukocyte recruitment ...
Woolhouse Ian
core +2 more sources
Idiopathic Hemochromotosis and Alpha‐1‐Antitrypsin Deficiency: Coexistence in a Family with Progressive Liver Disease in the Proband [PDF]
A patient with coexistent hemochromatosis and alpha‐1‐antitrypsin deficiency which led to cirrhosis and death despite adequate therapy for hemochromatosis is reported. Evaluation of the family revealed first degree relatives with iron overload and others
Addison +21 more
core +1 more source

