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Biomedical subjects

R C Stern

Publications and source records attributed to R C Stern.

144 records · Page 8Linked to original sources
PubMed1976

Course of cystic fibrosis in 95 patients.

The course of 95 patients with cystic fibrosis is presented. Survivors have a mean follow-up period of over 14 years (minimum: 13 years). Of 45 patients diagnosed prior to extensive irreversible pulmonary involvement, only one has died and none is disabled. In contrast, of the other 50 patients diagnosed after substantial irreversible pulmonary disease was present, 26 have died. Mortality and morbidity has been greater in females. Possible factors contributing to the improving prognosis include early diagnosis, aggressive management with comprehensive care, easy access to specialized care, and improved antimicrobial therapy.

Adolescent↗
PubMed1976

Course of cystic fibrosis in black patients.

Seventeen black patients showed typical sweat gland, gastrointestinal, and pulmonary manifestations of cystic fibrosis. There was an unusually high incidence of meconium ileus equivalent (35%). Three infant siblings of patients in this study had died of cystic fibrosis prior to referral of a family member to this center. One patient died at age four years after emotional problems interfered with the treatment program; status asthmaticus played a major role in his terminal respiratory failure. The remainder of the patients have had substantially less morbidity from pulmonary disease than a comparable, much larger group of white patients. Only one patient, age 28, who is married and employed full-time has severe pulmonary involvement as assessed by pulmonary function studies and chest roentgenogram. The 16 surviving patients have a mean age of 13 1/2 years after a mean follow-up period of 10 years. If black patients with cystic fibrosis survive infancy, they then, as a group, may have a relatively good prognosis.

Black People↗
PubMed1976

Purine nucleoside metabolism in the erythrocytes of patients with adenosine deaminase deficiency and severe combined immunodeficiency.

Deficiency of erythrocytic and lymphocytic adenosine deaminase (ADA) occurs in some patients with severe combined immunodeficiency disease (SCID). SCID with ADA deficiency is inherited as an autosomal recessive trait. ADA is markedly reduced or undetectable in affected patients (homozygotes), and approximately one-half normal levels are found in individuals heterozygous for ADA deficiency. The metabolism of purine nucleosides was studied in erythrocytes from normal individuals, four ADA-deficiency patients, and two heterozygous individuals. ADA deficiency in intake erythrocytes was confirmed by a very sensitive ammonia-liberation technique. Erythrocytic ADA activity in three heterozygous individuals (0.07,0.08, and 0.14 mumolar units/ml of packed cells) was between that of the four normal controls (0.20-0.37 mumol/ml) and the ADA-deficient patients (no activity). In vitro, adenosine was incorporated principally into IMP in the heterozygous and normal individuals but into the adenosine nucleotides in the ADa-deficient patients. Coformycin (3-beta-D-ribofuranosyl-6,7,8-trihydroimidazo[4,5-4] [1,3] diazepin-8 (R)-ol), a potent inhibitor of ADA, made possible incorporation of adenosine nucleotides in the ADA-deficient patients...

Adenosine Deaminase↗
PubMed1976

Symptomatic hepatic disease in cystic fibrosis: incidence, course, and outcome of portal systemic hunting.

Fifteen (2.2%) of 693 patients with cystic fibrosis seen over an 18-year period developed clinical hepatic disease. In 13 patients all symptoms were secondary to portal hypertension. Ten had hypersplenism and 6 had variceal bleeding, including 3 who developed both conditions. All 5 patients who survived the initial episode of gastrointestinal bleeding underwent portal systemic shunting. A shunting procedure also was performed on 1 patients with hypersplenism but no variceal bleeding. No subsequent deterioration of intellectual function occurred in either the shunted or unshunted patients. Only 1 of the shunted patients showed progression of hepatic disease after surgery. These results suggest that portal systemic shunting is useful in the treatment of bleeding esophageal varices in cystic fibrosis. A sweat test to rule out cystic fibrosis should be included in the evaluation of any teenage or young adult patient with unexplained portal hypertension.

Adolescent↗
PubMed1975

Restoration of in-vitro lymphocyte responses with exogenous adenosine deaminase in a patient with severe combined immunodeficiency.

Deficiency of adenosine deaminase (A.D.A.) occurs in an autosomal recessive form of severe combined immunodeficiency (S.C.I.D.). The role of this enzyme deficiency in the pathogenesis of the immune defects is not clear. A patient with A.D.A. S.C.I.D., studied during the first six weeks of life, was found to have B and T lymphocytes as well as 25% of normal lymphocyte responses to mitogens. This patient subsequently became severely lymphopenic with loss of mitogen responsiveness. Addition of calf-intestinal A.D.A. or human-erythrocyte A.D.A. to cultures of this patient's lymphocytes restored their ability to proliferate when stimulated with mitogens. These data indicate that A.D.A. deficiency is causally related to the cellular immune defects observed in A.D.A. S.C.I.D. and suggests a possible role for enzyme replacement in the therapy of this disorder.

Adenosine Deaminase↗
PubMed1975

Hyperreactivity to cow milk in young children with pulmonary hemosiderosis and cor pulmonale secondary to nasopharyngeal obstruction.

Six black infants and young children with high titers of milk precipitins were identified by screening the sera of 160 children with idiopathic chronic lung disease. None of the six had immunoglobulin deficiency, elevation of sweat chlorides, SS hemoglobin, or recurrent aspiration. All six children had typical manifestations of milk-induced pulmonary hemosiderosis: recurrent pulmonary infiltrates (6/6), hemosiderin-laden pulmonary macrophages (5/6), intermittent wheezing (5/6), eosinophilia (4/6), anemia (4/6), iron deficiency (4/4), failure to thrive (4/6), and elevated levels of serum IgE (4/4). Three children also had chronic rhinitis and eventually developed large adenoids, hypercapnia and acidosis during sleep, and right heart failure. Elimination of cow milk from the diet, symptomatic therapy, and adenoidectomy when indicated resulted in improvement of all six patients. Pulmonary hemosiderosis and some cases of upper airway obstruction with pulmonary hypertension appear to be two stages, early and delayed, of the same immunophysiologic process. Early dietary intervention may prevent the cardiovascular complications of this process.

Black or African American↗
PubMed1975

Open thoracotomy for pneumothorax in cystic fibrosis.

The results of open thoractomy and pleurectomy or pleural abrasion for 17 episodes of pneumothorax in patients with cystic fibrosis were compared with the results of observation, closed thoracostomy, and closed thoracostomy with sclerosing agents. Open thoracotomy had the advantage of insuring prompt resolution of the pneumothorax. A small incision minimized postoperative morbidity. The average hospital stay was 15 days after open thoracotomy and 19 days after closed thoracostomy, with or without sclerosing agents. Complications of all treatments were infrequent. A limited symptomatic recurrence was observed in 2 of 17 pneumothoraces successfully treated with open thoracotomy. Recurrence also occurred in 1 of 8 treated with observation, 3 of 9 treated with closed thoracostomy, and 1 of 14 treated with closed thoracostomy and sclerosing agents. A loss of vital capacity was usually noted 4 to 18 months after resolution, regardless of treatment. Forty per cent of patients in both operative and nonoperative groups survived 3 years. In our experience, open thoracotomy can be used safely for treatment of unresolved or recurrent pneumothorax and warrants further evaluation as a primary therapy for pneumothorax in patients with cystic fibrosis.

Cystic Fibrosis↗
PubMed1975

Studies on cor pulmonale in cystic fibrosis: I. Effects of diuresis.

The effects of acute diuresis in patients with cor pulmonale secondary to cystic fibrosis were studied. Both hemodynamic parameters and arterial blood gas changes were investigated. The major effect noted was a significant reduction of systemic venous pressure. Pulmonary artery and pulmonary artery wedge pressure either remained constant or declined slightly. No consistent changes were noted in cardiac output or arterial blood gases. Acute diuresis of moderate degree appears to be a safe and effective manner in which to treat the systemic venous congestion of cor pulmonale in this situation.

Acid-Base Equilibrium↗
PubMedDate not supplied

Single-dose pharmacokinetics of aztreonam in children with cystic fibrosis.

The single-dose pharmacokinetics of aztreonam was evaluated in 10 clinically stable subjects with cystic fibrosis. Each child received 30 mg aztreonam/kg intravenously over 2 to 3 minutes. Multiple timed blood samples were obtained over 8 hours for determination of aztreonam elimination kinetics; all urine excreted for 24 hours was collected in timed aliquots for the determination of aztreonam and its microbiologically inactive metabolite, SQ 26,992. Aztreonam pharmacokinetic parameters were determined by model-independent methods. Mean t1/2, steady-state volume distribution, and body clearance were 1.3 hr, 0.25 L/kg, and 127.2 ml/min/1.73m2, respectively. In 9 of the 10 subjects, two-compartment pharmacokinetic analysis was possible and compared favorably with model-independent parameter estimates. Twenty-four-hour urinary recovery of aztreonam was 76.3% of the administered dose; 2.6% was recovered as the metabolite SQ 26,992. The renal clearance of aztreonam averaged 92.5 ml/min/1.73m2. When these data are combined with in vitro susceptibility data for aztreonam against Pseudomonas aeruginosa isolated from the sputum of patients with cystic fibrosis, a dose of 200 mg aztreonam/kg/day divided six hourly would be predicted to maintain serum concentrations above the minimum inhibitory concentration (MIC) for these organisms for the majority of the dosing interval.

Adolescent↗
PubMedDate not supplied

Therapeutic evaluation of piperacillin for acute pulmonary exacerbations in cystic fibrosis.

The efficacy and pharmacokinetics of piperacillin monotherapy were studied in 46 patients with cystic fibrosis. Two patients were dropped from the study within 24 hr of enrollment because of drug-associated nausea and vomiting. Initially fourteen older patients (greater than 12 years) receiving piperacillin 450 mg/kg/day underwent a preliminary evaluation. Based on the results, 30 younger patients (less than or equal to 12 years) randomized in a double-blind fashion received either 600 or 900 mg/kg/day of piperacillin in six divided doses. Pharmacokinetic parameter estimates for t1/2 Vdss, and Cl were similar for first dose and steady-state evaluations. In 27 patients, approximately 43% of the administered dose was recovered in the urine after 4 hr. Piperacillin CiR averaged 49% of the total Cl. No difference in overall clinical efficacy could be identified between 600 and 900 mg/kg/day of piperacillin using two different objective scoring systems. Although a reduction in sputum Pseudomonas colony counts was greater following the 900 mg/kg/day regimen, this appeared to be independent of clinical effect. In 14 patients (32%), a distinct adverse serum-sicknesslike reaction was observed. The incidence of this reaction appeared to increase as the dose of piperacillin increased. All signs and symptoms of this reaction resolved within 36 hr of discontinuing piperacillin administration but recurred immediately on rechallenge in four patients. All patients with the adverse reaction were subsequently treated with beta-lactam antibodies without ill effect. Overall, clinical improvement appeared to be independent of the piperacillin dose. Our data support the use of total daily piperacillin dosages not exceeding 600 mg/kg.

Adolescent↗
PubMedDate not supplied

Imipenem/cilastatin in acute pulmonary exacerbations of cystic fibrosis.

Nineteen patients with pulmonary exacerbations of cystic fibrosis due to Pseudomonas aeruginosa were given imipenem/cilastatin for six to 10 days at dosages of 30-90 mg/kg per day. Mean Shwachman scores rose from 46.6 to 50.3 (P less than .001), clinical efficacy scores from 34.3 to 43.3 (P less than .001), vital capacity from 53.7% to 58.5% of the predicted value (P less than .01), forced expiratory volume in 1 sec from 39.5% to 42.6%, and partial pressure of oxygen in arterial blood from 68.2 mm Hg to 72.6 mm Hg. Treatment failed in only two instances. The concentration of P. aeruginosa in the sputum decreased to a modest extent (8.5 log10 cfu/ml on day 1, 8.1 log10 cfu/ml on day 10; P greater than .1). Four patients had imipenem-resistant strains of P. aeruginosa at the start of therapy, and 11 additional patients developed resistant strains during treatment; in eight patients greater than 90% of all Pseudomonas organisms in the sputum were resistant at the end of therapy. Six patients acquired Candida in their sputum. There was no correlation between bacteriologic improvement or the development of resistance to imipenem and either clinical outcome or improvement in pulmonary function. In summary, imipenem/cilastatin therapy is associated with a good clinical outcome in patients with cystic fibrosis, but resistance emerges rapidly.

Adolescent↗
PubMedDate not supplied

Cytomorphologic features of sebaceous carcinoma on fine needle aspiration.

OBJECTIVE: To describe three cases of sebaceous carcinoma metastatic to regional lymph nodes diagnosed by fine needle aspiration (FNA). STUDY DESIGN: FNA was performed using standard techniques. A portion of each specimen was stained with Diff-Quick (Dade, Miami, Florida, U.S.A.); another portion was fixed in 95% ethanol and stained with a modified Papanicolaou stain or fixed in formalin and stained with hematoxylin and eosin. RESULTS: All carcinomas were moderately cellular, with primarily irregular cell clusters. The cytoplasm was finely reticular and contained variable numbers of small vaculoes. Nuclei were centrally located and pleomorphic and contained coarse chromatin. Variably sized but often large nucleoli were seen. Mitotic figures were easily identified. CONCLUSION: Sebaceous carcinoma is a rare but cytologically distinct neoplasm. It frequently metastasizes to regional lymph nodes and may then appear as a mass amenable to FNA. Aspiration cytologists, particularly those who aspirate head and neck lesions, should be familiar with the distinct features of this neoplasm.

Aged↗
PubMedDate not supplied

Long-term parenteral nutrition in cystic fibrosis.

The effects of parenteral nutrition (PN) with high lipid content were studied in 18 cystic fibrosis patients in this pilot investigation. The patients were randomly assigned to one of two groups. During the first 4-mo period, group 1 received PN and group 2 received routine therapy. During the second 4-mo period, PN was discontinued in group 1 and instituted in group 2. When the effect of PN was considered for both treatment groups, its general effect was to increase body fat content with little or no impact on respiratory function, exercise tolerance, or recurrent infections. However, subsequent analysis and clinical observation suggested that patients receiving PN responded in two seemingly distinct patterns: some demonstrated apparent clinical improvement and benefit, and others did not. A positive response in pulmonary and exercise function was closely correlated to a rise in serum dihomo-gamma-linolenic acid (DHLA) concentrations during PN. Pulmonary function improved in patients who normalized their DHLA levels (vital capacity increased from 2.2 +/- 0.3 to 2.6 +/- 0.3 area %, p < 0.05), whereas those who continued to have undetectable levels of DHLA deteriorated (forced expiratory volume in 1 s decreased from 0.7 +/- to 0.6 +/- 0.1, p < 0.001). PN applied to malnourished patients with cystic fibrosis results in beneficial effects in a subgroup characterized by the presence of DHLA in serum; for the group as a whole, the positive effects are minimal.

Adolescent↗