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alpha 1-antitrypsin deficiency/protease

Холбоосыг санах ойд хадгалдаг
Хуудас 1 -аас 231 үр дүн

[The protease inhibitor system in children (in diseases of the lung, liver and hereditary alpha 1-antitrypsin deficiency)].

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх
The balance of the protease inhibitor system is decisive for the pulmonary conditions of children. Our investigations showed that an imbalance of this system can be interpreted as manifestation of a severe pulmonary inflammation (e.g. deficit of inter-alpha-antitrypsin inhibitor in case of dyspnoe)

Effective treatment with alpha 1-protease inhibitor of chronic cutaneous vasculitis associated with alpha 1-antitrypsin deficiency.

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх
The primary serum proteinase inhibitor is alpha 1-antitrypsin, and deficiency of this enzyme has been associated with a variety of systemic and cutaneous disorders. We report cutaneous vasculitis in a 49-year-old man with alpha 1-antitrypsin deficiency. His condition persisted despite treatment with

Replacement therapy of alpha 1-antitrypsin deficiency. Reversal of protease-antiprotease imbalance within the alveolar structures of PiZ subjects.

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх
The emphysema associated with the inherited serum deficiency of alpha 1-antitrypsin appears to result from an imbalance between neutrophil elastase and its major inhibitor within the alveolar structures. In the present study we assessed the feasibility of reversing this biochemical defect within the

[Substitution therapy in alpha 1-protease inhibitor deficiency (alpha 1-antitrypsin deficiency)].

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх
Deficiency of alpha 1-protease inhibitor is a dominant autosomally inherited error of metabolism leading to destruction of alveolar septa by proteolytic enzymes mainly released by neutrophils often before the fifth decade. Diagnosis and determination of phenotype are achieved by serologic tests.

Assessment of alpha-1-antitrypsin deficiency heterozygosity as a risk factor in the etiology of emphysema. Physiological comparison of adult normal and heterozygous protease inhibitor phenotype subjects from a random population.

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх
For plethysmographic studies of lung mechanics and measurement of pulmonary diffusing capacity, 62 subjects were drawn from a randomly selected population sample. Data obtained from the 24 subjects of heterozygous phenotype for alpha-1-antitrypsin deficiency (PiMZ) were compared by age group with

Serum protease inhibitory capacity. (Recent knowledge on alpha 1-antitrypsin deficiency).

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх
The molecular structure and the serum levels of alpha 1-antitrypsin, the major antiprotease of human serum, are controlled by a series of codominant alleles at a single chromosomal locus, known as the Pi(protease-inhibitor) locus. The congenital deficiency of this inhibitor is known to be associated

Transport of proteases across neonatal intestine and development of liver disease in infants with alpha-1 antitrypsin deficiency.

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх
The transport of macromolecules from the intestinal lumen into the systemic circulation is considerably greater in neonatal than in adult animals. Transport of both immunoglobin and non-immunoglobulin proteins is enhanced. It is postulated that luminal enzymes are also transported into the systemic

Alpha-1 Antitrypsin Deficiency: Beyond the Protease/Antiprotease Paradigm.

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх
From the discovery that alpha-1 antitrypsin (AAT) was an effective inhibitor of neutrophil elastase originated the classic paradigm of protease/antiprotease imbalance, linking lung destruction to the unopposed effect of proteases in patients with the deficiency. Notwithstanding its importance as an

Treatment of alpha-1-antitrypsin deficiency, massive edema, and panniculitis with alpha-1 protease inhibitor.

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх

Treatment of panniculitis associated with alpha-1-antitrypsin deficiency with alpha-1-protease inhibitor.

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх

[Mucous membrane specific protease inhibitors in bronchial mucus in severe chronic obstructive bronchitis and in alpha 1-antitrypsin deficiency syndrome].

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх
In the bronchial mucus of 40 patients with chronic obstructive airway diseases we measured proteolytic activities, the total protein concentrations, alpha1-antitrypsin, alpha1-antichymotrypsin, and the free and bound proteinase inhibitors together with the total proteinase inhibition against trypsin

Does Protease-Antiprotease Imbalance Explain Chronic Obstructive Pulmonary Disease?

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх
Chronic obstructive pulmonary disease (COPD) is defined as airflow limitation that is not fully reversible. The airflow limitation is usually progressive and is associated with the inhalation of noxious gases, typically cigarette smoke. The protease-antiprotease paradigm suggests that the

Patient-Derived Induced Pluripotent Stem Cells for Alpha-1 Antitrypsin Deficiency Disease Modeling and Therapeutic Discovery.

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх
PIZZ alpha-1 antitrypsin deficiency (AATD) is an autosomal recessive disease affecting approximately 100,000 individuals in the United States and one of the most common hereditary causes of liver disease.1 The most common form of the disease results from a single base pair mutation

Intraoperative Aortic Dissection during Lung Transplantation in a Patient with Alpha-1 Antitrypsin Deficiency.

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх
Alpha-1 antitrypsin deficiency (AATD) is an inherited disorder affecting the lung, liver, and rarely skin. The most frequent features of AATD consist of chronic lung disorders related to protease-antiprotease imbalance in the respiratory system, to which lung transplantation is frequently indicated.

Alpha 1-antitrypsin deficiency in intracranial aneurysms and cervical artery dissection.

Зөвхөн бүртгэлтэй хэрэглэгчид л нийтлэл орчуулах боломжтой
Нэвтрэх / Бүртгүүлэх
The pathogenesis of ruptured intracranial aneurysms and cervical artery dissections is poorly understood but may be similar in these two disorders. We report four patients with alpha 1-antitrypsin deficiency who developed a ruptured intracranial aneurysm or spontaneous dissection of the cervical
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