Congenital cystic adenomatoid malformation. A report of three unusual cases

Most children with congenital cystic adenomatoid malformation present in the first six months of life. Three cases are described with unusually late presentation. One child was completely asymptomatic and another became symptomatic only after secondary infection of a congenital cystic adenomatoid malformation. In two cases the correct diagnosis was not made preoperatively. The value of preoperative lung scanning was demonstrated in one case in which a lung scan showed a larger perfused area than indicated on the radiograph. At operation, a lobulated cystic structure was seen attached by a thin pedicle to the interior surface of the left upper lobe.     

Control of carcinoid syndrome with hepatic artery embolization

Eighteen patients with metastatic carcinoid were treated by hepatic artery embolization with Gelfoam or polyvinyl alcohol foam for control of the carcinoid syndrome. Seventeen showed subjective or objective clinical improvement, including less skin flushing, diarrhea, and bronchospasm. Fourteen showed improvement in biochemical indices, including decreased urinary 5-hydroxyindoleacetic acid levels. The mean life span of the treated patients from the first episode of flushing to the time of this report was 5.4 years, and half of the patients are still alive. This survival time compares favorably with previous reports of survival of 3.2 years from the onset of flushing.     

The acid deoxyribonuclease of neutrophils: a possible participant in apoptosis-associated genome destruction

Human neutrophils are terminally differentiated cells that spontaneously undergo apoptosis in tissue culture. Apoptosis in these cells can be delayed by culture in the presence of granulocyte colony- stimulating factor or other inflammatory mediators. Neutrophils were found to contain an acid endonuclease that appeared to be responsible for the internucleosomal DNA cleavage that accompanies apoptosis. As measured by a plasmid nicking assay, this endonuclease had a molecular weight (M(r)) of 35,000, a pH optimum of 5.5, and a threshold for activity of pH 6.6 to 6.8. It was weakly inhibited by divalent cations (Ca2+, Mg2+, and Zn2+) and more strongly inhibited by aurintricarboxylic acid and N-bromosuccinimide. DNA from neutrophils treated with nigericin in buffers of defined pH displayed nucleosomal ladders whose prominence varied with pH in a manner that paralleled the pH dependence of the plasmid cleavage assays, consistent with internucleosomal DNA cleavage by the acid endonuclease. We have previously shown that neutrophils undergo acidification to a pH value as low as 6.0 during apoptosis; we suggest that this endonuclease may be responsible for the DNA cleavage seen in apoptotic neutrophils.     

Plasminogen activator inhibitor-1 and diabetic nephropathy

SUMMARY:   Diabetic nephropathy is characterized by excessive accumulation of extracellular matrix (ECM) in the kidney. Decreased ECM degradation as well as increased ECM synthesis plays an important role in ECM remodeling that favours tissue fibrosis. Plasminogen activator (PA)/plasmin/PA inhibitor (PAI) system is involved in ECM degradation and PAI-1 plays a critical role in ECM remodeling in the kidney. Normal human kidneys do not express PAI-1 but PAI-1 is overexpressed in pathologic conditions associated with renal fibrosis including diabetic nephropathy. Reactive oxygen species mediate PAI-1 up-regulation in renal cells cultured under high glucose, hypoxia, and TGF-β1. Recent studies utilizing PAI-1 deficient mice suggest that PAI-1 induce ECM deposition in diabetic kidney through increased ECM synthesis by TGF-β1 up-regulation as well as through decreased ECM degradation by suppression of plasmin and MMP-2 activity.