Biomaterial Database

[Alpha 1 antitrypsin deficiency in childhood]. 1979

B Böhme

The author deals with the clinical importance of the alpha-1-antitrypsin deficiency in form of a survey with tabular representation of own cases. Alpha-1-antitrypsin as an inactivator of proteolytically effective enzymes essentially participates in the localised effect of proteinases. A genetically determined decreased alpha-1-antitrypsin serum level (= alpha-1-antitrypsin deficiency) by this causally participates in the pathogenesis of certain hepatopathies and in the pulmonary emphysema appearing already at the carly adult age. Within differential-diagnostic considerations an alpha-1-antitrypsin deficiency should be excluded in: 1. etiologically unclear cholestasis in infancy, 2. etiologically unclear hepatopathy in childhood, and in 3. early emphysema.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008099	Liver	A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.	Livers
D008107	Liver Diseases	Pathological processes of the LIVER.	Liver Dysfunction,Disease, Liver,Diseases, Liver,Dysfunction, Liver,Dysfunctions, Liver,Liver Disease,Liver Dysfunctions
D008297	Male		Males
D011656	Pulmonary Emphysema	Enlargement of air spaces distal to the TERMINAL BRONCHIOLES where gas-exchange normally takes place. This is usually due to destruction of the alveolar wall. Pulmonary emphysema can be classified by the location and distribution of the lesions.	Emphysema, Pulmonary,Centriacinar Emphysema,Centrilobular Emphysema,Emphysemas, Pulmonary,Focal Emphysema,Panacinar Emphysema,Panlobular Emphysema,Pulmonary Emphysemas,Centriacinar Emphysemas,Centrilobular Emphysemas,Emphysema, Centriacinar,Emphysema, Centrilobular,Emphysema, Focal,Emphysema, Panacinar,Emphysema, Panlobular,Emphysemas, Centriacinar,Emphysemas, Centrilobular,Emphysemas, Focal,Emphysemas, Panacinar,Emphysemas, Panlobular,Focal Emphysemas,Panacinar Emphysemas,Panlobular Emphysemas
D012127	Respiratory Distress Syndrome, Newborn	A condition of the newborn marked by DYSPNEA with CYANOSIS, heralded by such prodromal signs as dilatation of the alae nasi, expiratory grunt, and retraction of the suprasternal notch or costal margins, mostly frequently occurring in premature infants, children of diabetic mothers, and infants delivered by cesarean section, and sometimes with no apparent predisposing cause.	Infantile Respiratory Distress Syndrome,Neonatal Respiratory Distress Syndrome,Respiratory Distress Syndrome, Infant
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D004790	Enzyme Induction	An increase in the rate of synthesis of an enzyme due to the presence of an inducer which acts to derepress the gene responsible for enzyme synthesis.	Induction, Enzyme
D005260	Female		Females