Genetic and clinical features of patients with cystic fibrosis diagnosed after the age of 16 years.

Background: Cystic fibrosis is usually diagnosed in childhood, but a number of patients are not diagnosed until adulthood. The aim of this study was to investigate whether patients diagnosed at an older age had a different genetic constitution, manifestations of disease, and prognosis from those diagnosed at an early age.

Methods: Clinical data and results of lung function tests and DNA analysis of 143 adult patients with cystic fibrosis were entered into a computerised database.

[A comparison of changes of cerebrovascular oxygen saturation in retrograde and selective cerebral perfusion during aortic arch surgery].

To evaluate the effect of retrograde cerebral perfusion (RCP) and selective cerebral perfusion (SCP) on brain protection, changes of cerebrovascular oxygen saturation (rSO2) were studied in 14 patients with aortic arch reconstruction during the procedure. The rSO2 was monitored with spectroscopy instrument (Invos 3100, Somanetics). The mean value of rSO2 measured just before cardiopulmonary bypass was 65.

Structure-function relationship of bacterial prolipoprotein diacylglyceryl transferase: functionally significant conserved regions.

The structure-function relationship of bacterial prolipoprotein diacylgyceryl transferase (LGT) Has been investigated by a comparison of the primary structures of this enzyme in phylogenetically distant bacterial species, analysis of the sequences of mutant enzymes, and specific chemical modification of the Escherichia coli enzyme. A clone containing the gene for LGT, lgt, of the gram-positive species Staphylococcus aureus was isolated by complementation of the temperature-sensitive lgt mutant of E. coli (strain SK634) defective in LGT activity.

One-stage primary repair of distal esophageal perforation using fundic wrap.

Esophageal leak following primary repair of esophageal perforation is a serious complication that can lead to severe mediastinitis and sepsis. Complete diversion with esophageal exclusion or resection is designed to minimize further mediastinal contamination. However, this approach is not necessarily associated with less morbidity or mortality.

A cystic fibrosis mutation associated with mild lung disease.

Background: Cystic fibrosis is the most common lethal autosomal recessive disorder among whites. Among Dutch patients with cystic fibrosis, delta F508 is the most common mutation and A455E the second most common mutation of the cystic fibrosis transmembrane conductance regulator gene on chromosome 7. A455E is associated with preserved pancreatic function and residual secretion of chloride across membranes.

[Mitral valve repair for the treatment of ischemic mitral regurgitation].

Twenty-six patients with moderate and severe ischemic mitral regurgitation due to papillary muscle dysfunction underwent mitral valve replacement (MVR) or mitral annuloplasty (MAP) using modified Kay method. Emergent operation was performed in 12 patients of whom 11 had severe congestive heart failure even under IABP, 5 had cardiogenic shock and 9 needed respiratory care with intubation preoperatively. Elective operation was performed in 14 patients of whom 6 had history of congestive heart failure and 1 had episodes of ventricular tachycardia.

Respiratory-related heart rate variability persists during central apnea in dogs: mechanisms and implications.

The aim of this study was to determine the mechanism(s) responsible for the persistence of respiratory sinus arrhythmia (RSA) during central apnea. In five awake dogs, heart rate (HR) was recorded during constant mechanical ventilation (MV) and during central apneas produced by cessation of MV. For each of 10 control ventilator cycles before MV was stopped, instantaneous HR was plotted against the time from the onset of lung inflation; the fundamental and first harmonic of a sine wave (at the ventilator frequency) was then fitted to the HR data.

Neutrophil enzymes in urine for the detection of urethral infection in men.

Objectives: To determine if assaying the neutrophil enzymes, neutrophil elastase (NE) and myeloperoxidase (MPO) in the urine of men attending a genitourinary medicine clinic could identify those with Neisseria gonorrhoeae or Chlamydia trachomatis infections, and those with urethritis (with or without an identified infection with either organism), and to compare the new assays with the performance of the leucocyte esterase test (LET).

Method: 100 men had urethral specimens taken for Gram-stained urethral smear, culture for N gonorrhoeae, and for C trachomatis testing by enzyme immunoassay. First-voided urines were tested for leucocyte esterase by commercial dipstick (positives were defined as greater than "trace") and then frozen at -20 degrees C prior to being assayed for NE and MPO.

The umpA gene of Escherichia coli encodes phosphatidylglycerol:prolipoprotein diacylglyceryl transferase (lgt) and regulates thymidylate synthase levels through translational coupling.

Using a combination of biochemical, physical, and genetic techniques, we have shown that the umpA gene of Escherichia coli is allelic with the lgt (phosphatidylglycerol:prolipoprotein diacylglyceryl transferase) of Salmonella typhimurium. These genes are essential for the viability of the respective organism and exhibit 92.8% sequence identity at the amino acid level.

Mitral valve repair using an annuloplasty ring made of artificial woven Dacron graft.

A woven Dacron ring made of artificial graft was successfully used in combination with a modified Kay's annuloplasty for mitral valve repair. In this procedure, after excision and repair of the redundant prolapsed leaflets, Kay's annuloplasty was performed at both commissures to reduce the posterior annulus and to coapt the leaflets. A woven Dacron ring was then seated and tied to the annulus to provide long-term stabilization and prevent its further dilatation.

Comparison of a high lipase pancreatic enzyme extract with a regular pancreatin preparation in adult cystic fibrosis patients.

Background: Malabsorption due to exocrine pancreatic insufficiency is the main gastrointestinal problem in cystic fibrosis. Despite high doses of pancreatic enzyme supplements it is often not possible to normalize fat absorption. We compared a new high lipase pancreatic enzyme preparation (Pancrease-HL; Cilag, Brussels, Belgium), containing enteric coated microspheres with 25,000 U of lipase, 22,500 U of amylase and 1250 U of protease per capsule, with regular Pancrease capsules, containing 5000 U of lipase, 2900 U of amylase and 330 U of protease per capsule.

Tidal ventilation at low airway pressures can augment lung injury.

Intermittent positive pressure ventilation with large tidal volumes and high peak airway pressures can result in pulmonary barotrauma. In the present study, we examined the hypothesis that ventilation at very low lung volumes can also worsen lung injury by repeated opening and closing of airway and alveolar duct units as ventilation occurs from below to above the infection point (Pinf) as determined from the inspiratory pressure-volume curve. We ventilated isolated, nonperfused, lavaged rat lungs with physiologic tidal volumes (5 to 6 ml/kg) at different end-expiratory pressures (above and below Pinf) and studied the effect on compliance and lung injury.

On-line determination of pulmonary blood flow using respiratory inert gas analysis.

An inert gas analysis method has been developed to perform on-line real time determination of pulmonary blood flow using a nonrebreathing approach. This technique is based on a mathematical model describing mass balance of two inert gases which are breathed using an open gas circuit. The measurements using this method are noninvasive, easy to peform, and do not disturb normal physiological processes.