Results 71 to 80 of about 459,657 (249)

Human neutrophils express the alpha 1-antitrypsin gene and produce alpha 1-antitrypsin [PDF]

Blood, 1991
The potent serine protease, neutrophil elastase (NE), is stored in neutrophil azurophilic granules, where it is available to degrade phagocytosed material and can be released by the cell to assist in tissue migration and help clear tissue debris.
R M, du Bois, J F, Bernaudin, P, Paakko, R, Hubbard, H, Takahashi, V, Ferrans, R G, Crystal   +6 more
openaire   +3 more sources

Gross Intrahepatic Mass Formation Predicts the Primary Site of Perihilar Cholangiocarcinoma Based on Molecular Pathologic Studies

Journal of Hepato-Biliary-Pancreatic Sciences, EarlyView.
ABSTRACT Background/Purpose Intrahepatic cholangiocarcinoma (iCCA) and extrahepatic cholangiocarcinoma (eCCA) are clinically and genetically distinct. However, the classification of perihilar cholangiocarcinoma (phCCA) with an intrahepatic tumor mass remains unclear.
Yuki Masuda, Naoto Kubota, Ryo Takemura, Yasuhito Arai, Yuta Abe, Osamu Itano, Minoru Esaki, Tatsuhiro Shibata, Yuko Kitagawa, Hidenori Ojima   +9 more
wiley   +1 more source

Diagnosis of alpha-1-antitrypsin deficiency by DNA analysis of children with liver disease

Arquivos de Gastroenterologia, 2001
Background - Alpha-1-antitrypsin deficiency is a genetic disorder which is transmitted in a co-dominant, autosomal form. Alpha-1-antitrypsin deficiency affects mainly the lungs and the liver leading, in the latter case, to neonatal cholestasis, chronic ...
De TOMMASO Adriana Maria Alves, ROSSI Cláudio Lúcio, ESCANHOELA Cecília Amélia Fazzio, SERRA Heliane Guerra, BERTUZZO Carmen Sílvia, HESSEL Gabriel   +5 more
doaj  

Alpha-1 Antitrypsin Substitution for Extrapulmonary Conditions in Alpha-1 Antitrypsin Deficient Patients [PDF]

Chronic Obstructive Pulmonary Diseases: Journal of the COPD Foundation, 2018
Alpha-1 antitrypsin deficiency (AATD) is a genetic disorder which most commonly manifests as pulmonary emphysema. Accordingly, alpha-1 antitrypsin (AAT) augmentation therapy aims to reduce the progression of emphysema, as achieved by life-long weekly slow-drip infusions of plasma-derived affinity-purified human AAT.
Boris M, Baranovski, Ronen, Schuster, Omer, Nisim, Ido, Brami, Yotam, Lior, Eli C, Lewis   +5 more
openaire   +2 more sources

Treatment of lung disease in alpha-1 antitrypsin deficiency: a systematic review

International Journal of COPD, 2017
Background Alpha-1 antitrypsin deficiency (AATD) is a rare genetic condition predisposing individuals to chronic obstructive pulmonary disease (COPD). The treatment is generally extrapolated from COPD unrelated to AATD; however, most COPD trials exclude ...
R. Edgar, Mitesh K. Patel, S. Bayliss, Diana Crossley, E. Sapey, A. Turner   +5 more
semanticscholar   +1 more source

Preliminary evidence of improved liver biomarkers in adolescents with obesity and suspected metabolic dysfunction‐associated steatotic liver disease treated with semaglutide: A case series

JPGN Reports, EarlyView.
Abstract Metabolic dysfunction‐associated steatotic liver disease (MASLD) affects 30%–40% of youth with obesity and lacks approved pharmacologic therapies. In this single‐center retrospective case series at a tertiary care safety‐net children's hospital, we evaluated five adolescents (mean age: 17.0 years; 100% Hispanic; 60% male; mean body mass index:
Rachel Schenker, Katya Fomenko, Fotini T. Mitsinikos, Alaina P. Vidmar   +3 more
wiley   +1 more source

Comparative evaluation of serum alpha-1antitrypsin levels in patients with oral squamous cell carcinoma and in subjects with tobacco habit without carcinoma

Journal of Family Medicine and Primary Care, 2019
Aim/Objectives: To evaluate serum levels of Alpha-1 antitrypsin in patients with oral squamous cell carcinoma and compare them with that of healthy subjects with and without tobacco habits. Materials and Method: The sample of 83 subjects was divided into
Upasana S Ahuja, Nidhi Puri, Anjana Bagewadi, Vaishali Keluskar, Anshuman Ahuja, Harkanwal P Singh   +5 more
doaj   +1 more source

5 Year Expression and Neutrophil Defect Repair after Gene Therapy in Alpha-1 Antitrypsin Deficiency

Molecular Therapy, 2017
Alpha-1 antitrypsin deficiency is a monogenic disorder resulting in emphysema due principally to the unopposed effects of neutrophil elastase. We previously reported achieving plasma wild-type alpha-1 antitrypsin concentrations at 2.5%–3.8% of the ...
C. Mueller, Gwladys Gernoux, A. Gruntman, F. Borel, E. Reeves, R. Calcedo, Farshid N. Rouhani, A. Yachnis, M. Humphries, M. Campbell-Thompson, L. Messina, J. Chulay, B. Trapnell, James M. Wilson, N. McElvaney, T. Flotte   +15 more
semanticscholar   +1 more source

Late Onset Telomere Biology Disorder Presenting With Pancytopenia, Immune Dysregulation, Interstitial Lung Disease and Alopecia

American Journal of Hematology, EarlyView.
Bo A. Wan, Hamish Nicolson, Heather A. Leitch, Ryan J. Stubbins, Luke Y. C. Chen, Timothy Murray, Amy Trottier, Anthony Gador   +7 more
wiley   +1 more source

Hepatopulmonary syndrome as presentation of pediatric metabolic dysfunction‐associated steatohepatitis

JPGN Reports, EarlyView.
Abstract Childhood obesity is rising and leading to serious co‐morbidities, among which is metabolic dysfunction‐associated steatotic liver disease (MASLD) predisposing individuals to cirrhosis. We describe a young 11‐year‐old Hispanic male who presented with hepatopulmonary syndrome secondary to cirrhotic portal hypertension from metabolic dysfunction‐
Shruti Sakhuja, Stacey S. Beer, Kalyani R. Patel, Alexiss M. Williams, Nhu Thao Galvan, John A. Goss, Krupa R. Mysore   +6 more
wiley   +1 more source