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Biomedical subjects

R C Beckerman

Publications and source records attributed to R C Beckerman.

At least 19 recordsLinked to original sources

Predicting outcome following pulmonary resection in cystic fibrosis patients.

A small subset of cystic fibrosis (CF) patients develop pulmonary disease primarily limited to one lobe or lung segment requiring prolonged recurrent hospitalizations with intensive medical therapy. Although surgery has been advocated for patients who do not respond to medical therapy, very little is known about criteria for selection of patients who might benefit from resection of the involved parenchyma. In an attempt to further define criteria for pulmonary resection in these patients, we retrospectively reviewed our experience at Tulane Medical Center over the past 10 years. Fourteen patients with CF, ranging from 3 to 30 years of age, underwent 17 pulmonary resections. Indications for surgery were persistent lobar or pulmonary atelectasis and bronchiectasis requiring multiple hospitalizations and unresponsive to medical therapy (n = 13), bronchopleural fistula (n = 2), or hemoptysis not responding to medical therapy or selective embolization (n = 2). Thirteen lobectomies and four pneumonectomies were performed. Only two resections were on the left side and 11 right upper lobectomies were performed. Postoperative hospitalization ranged from 5 to 21 days (mean, 8.5 days). Preoperative pulmonary function tests showed widely divergent function in these patients. Forced expiratory volume (FEV1) ranged from 11% to 88% whereas forced vital capacity (FVC) ranged from 20% to 100% of predicted values. Oxygen saturation ranged from 86% to 99%. Although there was no significant difference in preoperative and postoperative FVC or O2 saturation, there was a significant (P less than .003) decrease in the postoperative FEV1. In the 12 surviving patients followed for at least 1 year, there was also a significant reduction (P less than .001) in the number of hospitalizations required due to pulmonary exacerbations from an average 2.2 admissions per year (range, 0.44 to 3.5 admissions per year) to 1.1 admissions per year (range, 0 to 8).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

MR imaging of pancreas in cystic fibrosis.

The pancreatic regions of 18 patients with cystic fibrosis were analyzed with a 1.5 Tesla MR unit. Signal intensity of the pancreas was correlated with clinical data and ultrasound. A hyperintense pancreas on T1-weighted image was consistent with fatty replacement of pancreatic insufficiency. A pancreas of normal soft tissue intensity was found in two asymptomatic and one symptomatic patient. A very hypointense pancreas on any pulse sequence was considered to be an intermediate stage of pancreatic degeneration.

Adolescent

Influence of SK&F 95587 and BN 50730 on bronchoconstrictor responses in the cat.

The effects of SK&F 95587 (4[2-(benzenesulfonamido)-ethyl] phenoxyacetic acid), a thromboxane (TX) receptor blocking agent, on bronchoconstrictor responses were investigated in paralyzed, anesthetized, mechanically ventilated cats. Intravenous injections of the TXA2 receptor mimics, U-46619 [(15S)-hydroxy-11 alpha,9 alpha-(epoxymethano)prosta5Z,13E-dienoic acid] and U-44069 (9,11-dideoxy-11 alpha,9 alpha-epoxymethano PGF2 alpha), produced dose-related increases in transpulmonary pressure and lung resistance and decreases in dynamic compliance. After administration of SK&F 95587, 5 mg/kg i.v., bronchoconstrictor responses to U-46619 and U-44069 were reduced markedly, whereas airway responses to prostaglandin (PG)F2 alpha, serotonin, PGD2 or the PGD2 metabolite, 11 beta-PGF2 alpha, were not altered. The duration of action of SK&F 95587 was greater than 3 hr, and the blockade was overcome when 10-fold larger doses of the TXA2 mimics were administered. Bronchoconstrictor responses to platelet-activating factor (PAF) were blocked by SK&F 95587 and by the novel PAF receptor antagonist, BN 50730. BN 50730 also blocked the fall in systemic arterial pressure in response to PAF. However, BN 50730 did not influence airway responses to U-46619, PGF2 alpha, PGD2 or serotonin and had no effect on baseline bronchomotor tone or arterial pressure. The PAF receptor antagonism with BN 50730 was overcome when 10-fold larger doses of PAF were administered and the dose-response curves for changes in lung resistance and dynamic compliance were shifted to the right in a parallel manner. The present data suggest that SK&F 95587 has selective TX receptor blocking activity, and that BN 50730 has selective PAF receptor blocking properties in the airways of the cat. The present data also provide support for the hypothesis that bronchoconstrictor responses to PAF are mediated by specific receptors, which are coupled to a phospholipase and, when activated, result in the release of TXA2 and contraction of airway smooth muscle.

15-Hydroxy-11 alpha,9 alpha-(epoxymethano)prosta-5

Obstructive sleep apnea syndrome in sickle cell disease.

We present a series of 21 children with obstructive sleep apnea syndrome and sickle cell disease (SCD). Thirteen patients underwent adenotonsillectomy with resolution of symptoms and improvement in alveolar hypoventilation. Adenotonsillectomy involves significantly increased risks in patients with SCD. We present our protocol for management of these difficult surgical patients, including vigorous hydration and transfusion therapy. Polysomnography has proven valuable both in diagnosis and in identifying severely affected patients. Increased end tidal alveolar carbon dioxide during sleep has proven to be a significant predictor of disease, while oxygen saturation monitoring was shown to be unreliable. The incidence of adenotonsillar hypertrophy (ATH) in SCD appears increased and not related to infectious diseases. We suggest that ATH represents a part of the natural course of compensatory lymphoid tissue enlargement in children with SCD.

Adenoidectomy

A comparison of tracheal breath sounds, airflow, and impedance pneumography in the detection of childhood apnea.

Impedance respiratory monitoring is not capable of detecting obstructive apneas. We compared a microphone breath sound detector, coupled to the chest wall, with a standard impedance device in 10 sleeping infants and children in order to determine the ability of the breath sound detector to detect normal respirations and central and obstructive apneas. Airflow was used as a standard for all measurements. No difference was found between the breath sound detector and impedance device techniques in the detection rate of either normal respirations or central apneic intervals. There was no statistically significant difference between breath sounds and airflow in the ability of either technique to detect obstructive apnea. The use of a breath sound detector avoids unnecessary stimulation of a sleeping child, whose monitoring would otherwise require that two or three airflow sensing devices be taped on the face. Breath sound monitoring may represent an alternative to impedance and airflow techniques for evaluation of apnea in closely observed infants and children.

Adolescent

Cystic fibrosis mistaken for idiopathic biliary atresia.

Previous reports of prolonged jaundice in cystic fibrosis have not described operative and histopathological findings in the liver and biliary tree. In the two cases reported here, obstructive jaundice in the neonatal period was associated with anatomical evidence of intra- or extrahepatic biliary obstruction. Hepatoportoenterostomy, a surgical procedure that is not without complications, was performed on one of the patients for biliary atresia before the diagnosis of cystic fibrosis was suspected. Prolonged obstructive jaundice may be an early manifestation of cystic fibrosis and may resolve without operative management. A sweat test should be performed on all patients with prolonged obstructive neonatal jaundice to rule out cystic fibrosis.

Bile Ducts

Familial idiopathic pulmonary hemosiderosis.

Two brothers, aged 3 and 6 years, respectively, had their pulmonary conditions diagnosed as idiopathic pulmonary hemosiderosis (IPH). Both boys had severe iron-deficiency anemia, chronic cough, hemoptysis, and exertional dyspnea, and one had recurrent epistaxis. The results of light microscopic lung histopathologic studies in both patients showed numerous hemosiderinladen macrophages and chronic interstitial pneumonitis. No specific patterns of immunofluorescence of the alveolar capillary basement membranes were found. The results of electron microscopic examinations showed intact alveolar and capillary basement membranes and no evidence of electron-dense deposits. The lack of clinical or biochemical evidence for renal disease as well as the absence of serum antinuclear and antibasement membrane antibodies excluded associated autoimmune disorders. Evaluation for milk-protein allergy was negative and neither child demonstrated a clinical response to a milk-free diet. Sequential pulmonary function studies performed over four years showed episodes of acute obstructive airway disease that correlated with pulmonary hemorrhage and mild persistent restrictive lung disease. The results of this family study suggested that some cases of IPH may have a genetic basis.

Anemia, Hypochromic

Altered polyamine metabolism in cystic fibrosis.

Children with cystic fibrosis excreted elevated urinary levels of all three polyamines--putrescine, spermidine, and spermine. Heterozygote parents excreted intermediate concentrations of the polyamines, but not levels significantly different from levels in normal controls. Patients with cystic fibrosis who were administered a tracer amount of [14C]spermidine excreted 11--13% of the radiolabel within 72 hr whereas normal controls excreted 60--76% of the radiolabel within 72 hr. Spermine excretion was positively correlated with increased pathology as assessed by the National Institutes of Health (NIH) clinical score, whereas urinary putrescine and spermidine levels were negatively correlated with increased pathology.

Adult

Hypoelectrolytemia and metabolic alkalosis in infants with cystic fibrosis.

The records of all children in the Tucson area diagnosed as having cystic fibrosis (CF) before the age of 12 months were reviewed to ascertain the prevalence of metabolic alkalosis as a major presenting manifestation of CF. Five of eleven infants (46%) in whom CF had been diagnosed between 1 and 12 months of age initially were seen with hypokalemia, hypochloremia, and metabolic alkalosis unassociated with marked dehydration, hyperpyrexia, or major pulmonary and/or gastrointestinal symptoms. Two infants had repeated episodes of metabolic alkalosis; for one of these infants, both episodes of metabolic alkalosis occurred before the diagnosis of CF. It is postulated that chronic loss of sweat electrolytes together with mild gastrointestinal or respiratory illness may predispose the infant with cystic fibrosis to a severe electrolyte and acid-base disturbance. The lack of shock and hyperpyrexia together with the apparent chronicity of electrolyte losses differentiates metabolic alkalosis from the heat prostration syndrome, a more acute complication of cystic fibrosis. Quantitative sweat testing should be part of the evaluation of any infant with unexplained metabolic alkalosis. Serum electrolytes should be assessed regularly in infants with cystic fibrosis during hot weather months.

Alkalosis

Cystic fibrosis: decreased conjugation and excretion of [14C]spermidine.

Free and conjugated [14C]spermidine were measured in plasma samples from normal individuals and cystic fibrosis patients. Within 4 minutes, the 14C-labeled material in the plasma from normal individuals was 70 percent conjugated compared to no detectable conjugation by cystic fibrosis patients. Further, the patients excreted only 11 to 13 percent of the [14C]spermidine in their urine within 72 hours whereas normal excretion was 60 to 76 percent. In both cases, the labeled material was in a conjugated form.

Cystic Fibrosis