Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An enzyme-linked immunoassay to quantitate lung surfactant apoproteins (15 to 250 ng/ml) in human amniotic fluid is described. The immunoassay was used to quantify lung surfactant in 72 samples of amniotic fluid, for which lecithin/sphingomyelin (L/S) ratios, lecithin and phosphatidylglycerol concentrations, and foam stability indices were also available. The results obtained with the immunoassay were in general agreement with those of the other methods. Measurement of the apoproteins, however, may be a better predictor of fetal lung immaturity and of respiratory distress syndrome than the L/S ratio and the concentration of lecithin. This conclusion is based on the data obtained in the analyses of samples of amniotic fluid from four diabetic and five nondiabetic pregnancies, the infants of which developed respiratory distress. In all cases, the apoprotein concentration was less than 2.1 micrograms/ml, which indicated fetal lung immaturity. In six of these cases (one diabetic and five nondiabetic pregnancies), lung immaturity was also predicted on the basis of other tests. However, in three other cases of diabetic pregnancy, the L/S ratio and lecithin concentration falsely indicated lung maturity. In addition to its being an effective predictor of fetal lung maturity in diabetic, as well as nondiabetic, pregnancies, the immunoassay is better suited for clinical use because of its high specificity, sensitivity, and ease of performance.
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PMID:Deficient lung surfactant apoproteins in amniotic fluid with mature phospholipid profile from diabetic pregnancies. 654 25

Cytochrome P450 1A2 (CYP1A2) is a constitutively expressed hepatic enzyme that is highly conserved among mammals. This protein is primarily involved in oxidative metabolism of xenobiotics and is capable of metabolically activating numerous procarcinogens including aflatoxin B1, arylamines, heterocyclic amine food mutagens, and polycylic aromatic hydrocarbons. Expression of CYP1A2 is induced after exposure to certain aromatic hydrocarbons (i.e., 2,3,7,8-tetrachlorodibenzo-p-dioxin). Direct evidence for a role of CYP1A2 in any physiological or developmental pathway has not been documented. We now demonstrate that mice homozygous for a targeted mutation in the Cyp1a-2 gene are nonviable. Lethality occurs shortly after birth with symptoms of severe respiratory distress. Mutant neonates display impaired respiratory function associated with histological signs of lung immaturity, lack of air in alveoli at birth, and changes in expression of surfactant apoprotein in alveolar type II cells. The penetrance of the phenotype is not complete (19 mutants survived to adulthood out of 599 mice). Surviving animals, although lacking expression of CYP1A2, appear to be normal and are able to reproduce. These findings establish that CYP1A2 is critical for neonatal survival by influencing the physiology of respiration in neonates, thus offering etiological insights for neonatal respiratory distress syndrome.
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PMID:Neonatal lethality associated with respiratory distress in mice lacking cytochrome P450 1A2. 776 62

The high mortality for congenital diaphragmatic hernia (CDH) has been attributed to a combination of pulmonary hypoplasia and persistent pulmonary hypertension. Recent experimental studies suggest that surfactant deficiency may also contribute to CDH pathophysiology. In this report, the authors immunohistochemically and morphometrically examined whether or not the hypoplastic lungs of CDH are associated with the immaturity of the surfactant system, especially alveolar type II cell function. Nine autopsy cases with CDH were immunohistochemically examined for the expression of surfactant apoprotein, using anti-IgG against human surfactant apoprotein A (SP-A), and the findings were compared with those in a gestational and postnatal age-matched control group. The lung/body weight ratio in the CDH was less than that in the controls (0.010 +/- 0.005 versus 0.021 +/- 0.013, P < .01). The radial alveolar count (RAC) of the CDH cases were also significantly less than that of the control cases (2.10 +/- 0.52 versus 3.48 +/- 0.39, P < .01). In the CDH cases, the RAC of the lung on the affected side were also significantly less than that of the lung on the unaffected side (1.71 +/- 0.34 versus 2.50 +/- 0.26, P < .01). In the immunohistochemical distribution of SP-A, compared with the control cases, the number of SP-A-positive cells on the alveolar septa of the CDH cases decreased in number, and this immunohistochemical reaction was weak even in positive type II cells. In addition, the immunoreaction observed in the alveolar type II cells of the unaffected side lungs in the four CDH cases was stronger than that of the unaffected side lungs. These results suggest that in the lungs of the CDH cases, especially on the affected side, there is a possible delay in both the structural growth and functional maturation or development of SP-A synthesis by alveolar type II cells, and this retardation of the functional maturation in alveolar type II cells is also considered to play a role in postnatal respiratory insufficiency in CDH patients.
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PMID:Immunohistochemical distribution of surfactant apoprotein-A in congenital diaphragmatic hernia. 916 47

The alveolar adenoma of the lung is a rare benign tumor characterized by a proliferation of both the alveolar epithelial cells and the mesenchymal septal cells. Immunohistochemically, the epithelial cells stain for cytokeratin (CK) AE1AE3, CK7, thyroid transcription factor 1 (TTF1), and surfactant apoprotein confirming the derivation by the type 2 pneumocytes. The stromal cells are negative for these markers but they show focally smooth muscle and muscle-specific actin positivity. We describe two cases that showed immunohistochemically a CD34 positivity of the mesenchymal septal cells. This aspect has been previously described in a two cases report, but not emphasized by the authors as a distinctive feature of the lesion. We consider this CD34 positivity as a marker of immaturity or stemness of the lesional septal spindle cells, that could be responsible of the different phenotypic and morphological profile of the interstitial cells, that could be, therefore, considered neoplastic and not reactive.
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PMID:CD34 Expression in the Stromal Cells of Alveolar Adenoma. 2311 69