Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019270 (hernia)
 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lung volume and lung mechanics were measured in 19 infants, aged 2 to 8 weeks, with congenital pulmonary hypoplasia of various causes. These included nine infants with congenital diaphragmatic hernia, five infants with Werdnig-Hoffman disease of intrauterine onset, and five infants with isolated pulmonary hypoplasia or associated anomalies such as thoracic dystrophy and spina bifida. Infants were studied within the first two months of life in order to exclude the effects of natural adaptation and adverse influences such as lower respiratory tract infection. Lung volume and airway resistance were estimated by whole body plethysmography, total pulmonary resistance, and dynamic lung compliance with esophageal balloons and pneumotachography. Reductions in dynamic lung compliance were found in seven of the nine infants with congenital diaphragmatic hernia, probably reflecting lung overdistention after operative repair. Low airflow resistance was found in 12 infants, representing subjects from each category. The low airflow resistance may be related to reductions in airway generation number found when the disturbance of lung development occurs before 16 weeks of gestational age. This finding suggests a role for measurements of airflow resistance in characterizing the type of pulmonary hypoplasia.
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PMID:Lung function in infants with congenital pulmonary hypoplasia. 714 68

The relative abundance of pulmonary neuroendocrine cells synthesizing gastrin-releasing peptide (GRP) was estimated for normal fetal lungs and hypoplastic lungs. Percentage of bronchiolar epithelial area staining positively with anti-GRP antiserum was computed for each case using a SAMBA 4000 image analyzer. The majority of hypoplastic lungs (10 of 12 cases) showed markedly diminished GRP immunoreactivity, which appeared to vary with etiology. Six cases of pulmonary hypoplasia associated with renal anomalies, three cases associated with hydrops, and one case of diaphragmatic hernia showed an average fivefold reduction in percentage of GRP immunostaining. A case of hypoplasia associated with Werdnig-Hoffmann disease had GRP immunoreactivity similar to that of controls, and GRP expression was markedly elevated (fivefold) in a case of hypoplasia with omphalocele.
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PMID:Gastrin-releasing peptide in hypoplastic lungs. 902 90