Bronchogenic cyst masquerading as asthma.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to L L Fan.
Explore the source record for details and available documents.
An inorganic pyrophosphatase (PPase) was purified from thylakoid membrane of spinach leaves to electrophoretic purity by methods including detergent solubilization, ammonium sulfate fractionation, and successive chromatographic techniques. Current protocol yielded about 10% recovery of total activity with a 30-fold purification. The specific activity of the purified enzyme was approximately 400 micromol PPi consumed/mg protein x h. This enzyme is a monomer with a molecular mass of 55 kDa. Several properties, including subunit composition, substrate specificity, ion requirements, inhibitor sensitivities, and amino acid composition, have been studied. Mg2+ is an essential cofactor for the thylakoid PPase. The preferred substrate for the hydrolytic reaction of PPase appears to be dimagnesium pyrophosphate. K+ could not stimulate the enzymatic activity of thylakoid PPase, while F- was a potent inhibitor. Group-specific modification of the thylakoid PPase demonstrates possible involvement of carboxylate residues in the enzymatic activity. Furthermore, antibodies raised against thylakoid PPase in a rabbit could inactivate the PPi hydrolysis of thylakoid and the purified enzyme, but not that of vacuolar H+-PPase, indicating both PPi hydrolases are structurally distinct.
We have investigated the diagnostic value of (BAL) in 29 immunocompetent children (ages 1 month to 18 years) with chronic diffuse pulmonary infiltrates on chest radiograph who presented for evaluation over a 3-year period. The median age at the time of the BAL was 20 months with a range of 1-210 months. Positive results (1) diagnostic of a primary disorder; (2) consistent with a diagnosis; or (3) diagnostic of a secondary disorder, were obtained in 20/29 patients (13 with a single positive BAL finding and 7 with more than one finding). BAL was diagnostic of a primary disorder in only 5 patients (17%) with aspiration detected in 3 and infection in 2. The differential diagnosis was narrowed in 15 patients by the presence of lymphocytosis, neutrophilia, or eosinophilia. A secondary disorder was uncovered in 8 patients. Negative results were obtained in 9 additional patients. We conclude that BAL provided useful information in children with chronic diffuse infiltrates, but its ability to determine the primary cause was limited.
We studied the effects of recurrent aspiration of milk on neural control of airways in young developing rabbits. Beginning at 1 week of age, rabbits received 0.5 ml/kg of whole milk or sterile physiologic saline intranasally while under light methoxyflourane anesthesia 5 days a week for a period of 3 weeks. At 4 and 8 weeks of age, in vitro studies of contractile and relaxant responses of tracheal smooth muscle (TSM) segments were evaluated. To assess the neurally mediated contractile responses, frequency response curves to electrical field stimulation (EFS) were performed with results expressed in terms of frequency of EFS causing 50% of the maximal contractile response (ES50) values. In addition, the contractile responsiveness of TSM to methacholine (MCh) as reflected by the concentration causing 50% of the maximal contractile response (EC50) values was also determined to evaluate the underlying cholinergic reactivity of this segment of airway. To assess nonadrenergic noncholinergic inhibitory (NANCi) responses, experiments were performed on TSM contracted with neurokinin A in the presence of atropine, propranolol, and indomethacin. EFS was delivered to the contracted tissue at stimulation frequencies ranging from 5 to 30 Hz with results expressed as mean percent relaxation. Recurrent aspiration of milk but not saline increased EFS-induced contractile responses, as shown by significantly lower ES50 values compared with the control group: P = 0.02 and P = 0.001 at 4 and 8 weeks of age, respectively. TSM responsiveness to MCh was no different between the two groups, suggesting that alterations in prejunctional mechanisms of neural control were most likely responsible for the increased contractile response to EFS. The NANCi responses were significantly decreased by milk aspiration at both 4 and 8 weeks of age, with the abnormalities less pronounced at the later time point. These findings demonstrate that repeated aspiration of milk leads to abnormal mechanisms of neural control within airways of developing rabbits. While aspiration of milk altered both contractile and relaxant responses to EFS, the former abnormalities became more pronounced with time while the latter appeared to be resolving. These observations suggest that injury to an airway early in development does not necessarily resolve with time but may persist, with functional abnormalities becoming more pronounced even after the airway insult has ceased.
OBJECTIVES: To evaluate the diagnostic value of transbronchial biopsy (TBB), video-assisted thoracoscopy (VAT), and open lung biopsy (OLB) in immunocompetent children with chronic, diffuse infiltrates; to identify factors that may predict diagnosis in children requiring biopsy; to determine whether age, number of biopsies, or type of procedure are associated with diagnostic yield in children undergoing transthoracic biopsy; and to compare morbidity of VAT with that of OLB. STUDY DESIGN: As part of a prospective, descriptive study to define the clinical spectrum of pediatric interstitial lung disease, 30 immunocompetent children required TBB, VAT, and/or OLB for diagnosis of diffuse infiltrates. We reviewed and analyzed the following clinical variables: age; preoperative diagnosis; type of procedure; number of lobes undergoing biopsies; durations of surgery, chest tube insertion, and hospitalization; tissue diagnosis; and complications. RESULTS: Specific diagnoses were made in 50%, 60%, and 53% of patients undergoing TBB, VAT, and OLB, respectively. A variety of rare disorders was found, and tissue diagnosis confirmed the preoperative diagnosis in 25% of all procedures. For patients who underwent transthoracic biopsy, patient age of greater than 24 months was significantly associated with increased diagnostic yield, but the number of lobes biopsied and type of procedure were not. VAT was associated with shorter operating time, chest tube placement, and hospitalization when compared with OLB. The complications of VAT and OLB were comparable. CONCLUSION: Lung biopsy is an important tool for the diagnosis of interstitial lung disease in immunocompetent children, but the diagnosis of many children, particularly those aged 2 years or younger, remains uncertain.
To investigate factors influencing survival in children with chronic interstitial lung disease (ILD), we extracted specific clinical information from a data base of 99 children with ILD who met entry criteria for our study. The effects of a weight below the fifth percentile for the patient's age, crackles, clubbing, family history of ILD, symptom duration, and severity-of-illness score on survival were examined with univariate and multivariate statistical analyses. There were 15 recorded deaths in the group, with a probability that a patient would survive to 24 mo, 48 mo, and 60 mo after onset of symptoms of 83%, 72%, and 64%, respectively. Of the clinical features, only increasing severity-of-illness score was significantly associated with a higher probability of decreased survival. Children with ILD have decreased survival probabilities that can be predicted with a severity-of-illness score.
The cardiotonic and vasodilating effects of oxyphenamone were studied in preparations of rat Langendorff's heart, isolated cardiac muscles of guinea pigs and isolated arteries of dogs. In the preparations of rat Langendorff's heart, infusion of oxyphenamone at 0.1 and 1.0 mumol.L-1 increased myocardial contractility and coronary blood flow moderately and decreased heart rate slightly. Oxyphenamone (1-100 mumol.L-1) increased the contractile force of papillary muscle (PM) and left atrium (LA) of guinea pigs dose-dependently. The positive inotropic effect of oxyphenamone (the ED50 was found to be 18.35 mumol.L-1 in PM and 9.17 mumol.L-1 in LA) was close to that of milrinone, an inhibitor of phosphodiesterase(the ED50 was shown to be 6.23 mumol.L-1 in PM and 4.59 mumol.L-1 in LA). The difference between these two agents is that oxyphenamone decreased the spontaneous pulse rate of right atrium, while milrinone increased it. The vasoconstrictions of dog coronary, basilar and mesenteric arteries induced by KCl, 5-HT and CaCl2 were antagonized non-competitively by oxyphenamone at 1 and 50 mumol.L-1, suggesting that oxyphenamone has a relaxant effect on vascular smooth muscles.
For studying the cardiotonic and vasaodilating effect of oxyphenamone in vivo, cardiac hemodynamic variables of anesthetized normal rats, cats and dogs were determined with a polygraph and electromagnetic flowmeters. Intravenous injection of oxyphenamone (0.5-10 mg.kg-1) dose-dependently decreased the mean arterial pressure and systemic vascular resistance moderately and increased cardiac output, myocardial contractile force and +/- dT/dtmax. The blood flow of coronary and femoral arteries increased markedly and their vascular resistance decreased but the blood flow and vascular resistance of cerebral artery did not change. Some species differences were observed in the effect of oxyphenamone on heart rate (HR) and left ventricular pressure (LVP). Oxyphenamone did not influence the HR and LVP in normal cats. It decreased the HR, left ventricular systolic pressure (LVSP) and +/- dP/dtmax, while did not affect the left ventricular end diastolic pressure (LVEDP) in rats. In contrast, administration of oxyphenamone to dogs increased LVSP and +/- dP/dtmax markedly and diminished LVEDP slightly. Oxyphenamone decreased HR slightly at lower dosage (1 and 3 mg.kg-1), but increased heart rate moderately at high dose (6 mg.kg-1) in dogs. These indicate that oxyphenamone has cardiotonic and vasodilating effects in vivo. Whether the effects of oxyphenamone on cardiac hemodynamics would be useful for the treatment of heart failure should be evaluated further.
Thoracoscopic techniques were used to perform lung biopsies and limited resections in 36 consecutively treated cases. Biopsies were performed for interstitial lung disease in 27 cases, presumed metastatic lesions in 5, and cavitary lesions in 4. Histological diagnosis was obtained in 35 of the 36 cases, and therapy was directly affected by the results in 30 of 36 cases. There were no postoperative complications, and the average hospital stay for patients admitted the morning of surgery was less than 2 days. Limited thoracoscopic resection provides a safe and effective means for diagnosing and treating parenchymal disease of the lung.
During the acquisition of a series of 92 children with interstitial lung disease (ILD) over a 14 year period, a significant minority (8/92 or 9%) were initially diagnosed as having ILD, but were subsequently found to have a variety of arterial, venous, and/or capillary disorders that explained their initial pulmonary findings. This subgroup of patients has had a very high morbidity and mortality, with only three of eight patients currently surviving. The presentation, evaluation, and natural history of these eight children were reviewed. We developed a strategy of cardiac and pulmonary evaluation for children presenting with clinical and radiographic features of ILD that helped us to identify rapidly those with pulmonary vascular disorders.
Explore the source record for details and available documents.
Asthma is increasingly treated as an inflammatory disease with inhaled and/or systemic corticosteroids. We report 3 cases of unusual pneumonias associated with high doses of oral steroids. Two patients contracted Legionella pneumonia and one patient contracted Pneumocystis carinii pneumonia. With increasing usage, it is important for physicians to be aware of the possible infectious complications of high dose steroids. This report highlights the risk of corticosteroid treatment in asthma in predisposing to opportunistic infections that have not heretofore been readily associated with asthma.
The interstitial pneumonias in children are a diverse group of rare conditions associated with considerable morbidity and mortality. Newer diagnostic modalities such as high-resolution computed tomography and bronchoalveolar lavage have improved the ability to diagnose these conditions. Open lung biopsy remains the most useful diagnostic study, and thoracoscopic methods are being used in children more frequently with favorable results. Treatment with corticosteroids, hydroxychloroquine, and cytotoxic agents is empirical due to a lack of controlled therapeutic trials. Lung transplantation, even in infants, is being done more often and with greater success for end-stage lung disease.
Explore the source record for details and available documents.
Fifty-two infants seronegative to or without prior infection with influenza type A viruses were enrolled in a study to evaluate reactogenicity and immunogenicity of three bivalent cold recombinant type A (CRA) and two trivalent inactivated influenza (TI) vaccines. Controls consisted of infants receiving normal saline by nose drops (Pli.n.) or intramuscularly (Pli.m.). CRA and TI vaccines were monitored for local and systemic reactions after vaccination. Serum specimens obtained prior to and 6 weeks postvaccination were analysed for neutralizing antibody to influenza H1N1 and H3N2 viruses. CRA vaccines and Pli.n. recipients had similar numbers of acute respiratory infections and comparable rates of illnesses during the trial. Significantly fewer CRA vaccinees without an intercurrent viral infection had fever (0/16 versus 4/10, p = 0.04) and cough (4/16 versus 9/10, p = 0.002) than CRA vaccinees with a confirmed intercurrent viral infection. Recipients of TI vaccine and Pli.m. did not develop reactions at the injection site. For each of the CRA vaccines tested, a dominant CRA virus was identified. The dominant CRA viruses were isolated from a greater number of infants or for a longer duration than the non-dominant CRA viruses. All 14 non-dominant CRA viruses were recovered from infants within the first week after vaccination; 24 of 77 dominant CRA viruses were recovered more than 7 days after vaccination. The immunogenicity of CRA vaccines was not affected by a confirmed intercurrent viral infection or low titres of influenza-specific antibody.(ABSTRACT TRUNCATED AT 250 WORDS)
To describe the clinical spectrum of interstitial lung disease in children, we reviewed our experience with 48 patients during a 12-year period. Most patients initially had typical findings of restrictive lung disease and hypoxemia. Growth failure or pulmonary hypertension or both were found in more than one third. Specific diagnosis, made in 35 patients (70%), most often required invasive studies, particularly open lung biopsy. Although the diagnostic yield from open lung biopsy was high, the diagnosis of many patients remained uncertain. Many different disorders were encountered. The response to corticosteroids, bronchodilators, and chloroquine was inconsistent. Six patients died, five within 1 year after the initial evaluation. The spectrum of pediatric interstitial lung disease includes a large, heterogeneous group of rare disorders associated with high morbidity and mortality rates.
The effects of recombinant human G-CSF (rhG-CSF) and retinoic acid (RA) were studied on the proliferation and differentiation of HL-60 cells and human acute myeloid leukemic cells. Synergistic effect on granulocyte differentiation was observed when HL-60 cells and primary acute promyelocytic leukemic cells were cocultured with RA plus rhG-CSF. rhG-CSF combined with RA increased more significantly the percentage of mature cells than RA alone and greatly increased NBT reduction activity (P < 0.001). These results suggested that proliferated effect of rhG-CSF on leukemic cells may be important for inducing differentiation of myeloid leukemic cells. But this effect might expose the patients to the risk of acute myeloblastic leukemia if G-CSF was used alone. However, RA could not only rule out the latter situation but retain former merit as well. The authors suggest that the combined use of G-CSF with RA is probably a new approach to the treatment of leukemia.
To ascertain the beneficial effect of puerarin on the myocardium against reperfusion injury, studies on myocardial metabolism and ultrastructure were made. Twelve dogs divided into two equal groups were placed on moderate hypothermic cardiopulmonary bypass, and their hearts were subjected to 140 minutes cold cardioplegic arrest and 60 minute reperfusion. In the control group, the hearts were perfused with crystalloid cardioplegic solution (CPS) every 20 minutes during arrest. In the treated group, the hearts received CPS containing puerarin (2 mg/kg). Myocardial oxygen consumption, lactate production, creatine phosphokinase (CPK) release, water content and ultrastructural alterations were determined before ischemia, during cardiac arrest and at reperfusion. The results showed that intermittent infusion of CPS containing puerarin significantly decreased myocardial lactate production during ischemia, as well as myocardial oxygen consumption, CPK release and water content during reperfusion. Under electronmicroscopy, the degree of ischemic damage judged by a scoring method was less pronounced in the puerarin group than in the control. The authors conclude that puerarin has protective effects on the function of hearts that have undergone long periods of arrest and reperfusion.