PubMed Health⌕ Search

Biomedical subjects

H Shwachman

Publications and source records attributed to H Shwachman.

At least 19 recordsLinked to original sources
PubMed1979

Bronchial artery embolization in cystic fibrosis; technique and long-term results.

Severe bronchial hemorrhage in 13 patients with cystic fibrosis was treated by catheter embolization of bronchial arteries. Indications were either excessive bleeding persisting for several days, or bleeding serious enough to interfere with pulmonary drainage and recurring over weeks or months. In follow-up ranging from one to 30 months, cessation of major bleeding was achieved in 12 of 13 patients (93%), although 5 of 13 patients (40%) did have recurrence of minor hemoptysis. No neurologic or other major complications were encountered. However, there are potential risks and this approach at present should be limited to patients with life-threatening bleeding and carried out only by experienced angiographers.

Adolescent↗
PubMed1979

Brain abscess and cystic fibrosis.

Brain abscess has only recently been considered a complication of cystic fibrosis. Three patients are reported here and a fourth cited from the literature. All of our patients were young adults with advanced pulmonary disease. The bacteria involved were mouth organisms and were found in the sputum culture in only one of the patients. Resistance was present to previously given antibiotics. As patients with cystic fibrosis survive into adulthood, the risk of developing a brain abscess appears to increase.

Adolescent↗
PubMed1979

Vocational development and adjustment of adolescents with cystic fibrosis.

Adolescents with cystic fibrosis were compared with normal adolescents of the same age and educational grade on several measures of vocational development and adjustment. The cystic fibrosis group in ages 12--16 and in grades 7, 8, and 9 scored lower than their normal counterparts in measures of vocational and educational plans, and were less realistic than normals in considering their limitations and financial constraints. In contrast, the cystic fibrosis group at nearly all age and grade levels scored significantly higher on strength of commitment to vocational choice, work values, and awareness of occupational information. Compared to 4 additional handicapped groups already studied, adolescents with cystic fibrosis scored favorably. They not only plan to work but also hold strong work values compared to normals. Rehabilitation programs should emphasize preparation for a career and the development of educational and vocational plans while the adolescent is still in high school.

Adolescent↗
PubMed1979

Cystic fibrosis in adults: an autopsy study.

An annotated catalog of clinical and pathologic observations in an adult population with CF has been presented. The spectrum of disease is broad, and the diagnosis usually requires the demonstration of abnormalities in multiple systems. Tissues of each germ layer have been found involved in CF. Correlation of clinical and pathologic observations suggests that manifestations are essentially episodic and focal. In this population, the most characteristic abnormalities recognized by the pathologist are focal biliary cirrhosis, distinctive obstructive lesions of the male genital tract, prolonged staphylococcal or pseudomonas colonization of respiratory secretions, and obstructive bronchopulmonary disease. Some form of pancreatic atrophy is usually present.

Adult↗
PubMed1979

Quality of sweat test performance in the diagnosis of cystic fibrosis.

The sweat test, correctly performed, appears to be reliable, but results were found to be unreliable about half the time in 84 small community hospitals assessed. The reasons are inexperienced and untrained technicians, the infrequency of test performance, the use of unstandardized equipment, and the lack of appropriate standards. It behooves all clinical laboratory directors to re-examine and standardize their procedures and use the recommendations of the Cystic Fibrosis Foundation. Physicians should be made aware of the current situation and send their patients only to laboratories that perform the test reliably.

Adolescent↗
PubMed1978

Sweat tests.

Explore the source record for details and available documents.

Clinical Laboratory Techniques↗
PubMed1978

Studies in meconium. An approach to screening tests to detect cystic fibrosis.

Screening for cystic fibrosis (CF) in newborns is desirable, and efforts should continue in establishing a system of easily available, reliable, simple, and inexpensive tests. In addition to the Boehringer-Mannheim (BM) test for the detection of albumin, we propose the assay for lactase and beta-D-fucosidase in meconium. These latter two enzymes are present in the meconium of babies with CF and absent in meconium of most healthy babies. In a mass screening program for CF involving 20,182 specimens of meconium using only the BM strip, we found 46 positive results. Twenty-nine specimens came from infants with CF, six of whom had meconium ileus. Seventeen specimens yielded false-positive results by the BM test. Eleven of these would have been excluded by the addition of the lactase and beta-D-fucoside assay, thus reducing the false-positive test results by nearly 61%. In a comparative study of the three methods, the lactase and beta-D-fucosidase yielded 1.2% false-positives when examined independently. Performance of these two assays may allow greater specificity in diagnosis when used in addition to the BM test.

Albumins↗
PubMed1978

Disaccharidase activities in small intestinal mucosa in patients with cystic fibrosis.

The disaccharidase activities in small intestinal biopsies were related to the morphology of the mucosa and the ages of 63 patients with cystic fibrosis and 177 healthy control subjects of Caucasian origin. In patients with CF and in the healthy control subjects under 5 years of age with normal intestinal mucosa, no low lactase activity was found. In those patients with CF who were over 5 years of age, one group had high and one group had low lactase activity, as occurs in healthy Caucasian control subjects of the same age. This finding supports the view that in patients with CF, lactase deficiency is not related to the disease entity. In patients with or without CF who had the same degree of mucosal atrophy, the decrease of disaccharidase activities followed the same pattern, indicating that enzyme activities are affected to the same extent by the damage of the mucosa. In patients with CF with pancreatic insufficiency, the disaccharidase activities were significantly (P less than 0.001) higher when compared to those in control subjects of the same age and ethnic group, although the increase was not uniform in all patients with cystic fibrosis.

Atrophy↗
PubMed1978

Studies in meconium in cystic fibrosis: the activities of alpha-D-mannosidase, beta-glucuronidase, beta-D-fucosidase, acid and alkaline phosphatase.

The specific activities of alpha-D-mannosidase, beta-glucuronidase, beta-D-fucosidase, acid and alkaline phosphatase were studied in meconium from infants with cystic fibrosis (CF) and control subjects. The study revealed significant variations in the specific activity of the enzymes except for acid phosphatase. The variations were not uniform. The activities of alpha-D-mannosidase, beta-glucuronidase and alkaline phosphatase were markedly decreased (p less than 0.001, p less than 0.002, p less than 0.001, respectively), while the activity of beta-D-fucosidase was significantly increased (p less than 0.001) in meconium from the infants with CF. It is suggested that the decreased activity of alpha-D-mannosidase and beta-glucuronidase might contribute to the accumulation of the abnormal substances in CF meconium. The highly increased activity of beta-D-fucosidase raises the possibility of an additional or alternative method for screening newborns for CF using meconium as the test material.

Acid Phosphatase↗
PubMed1978

Gluten-sensitive enteropathy: genetic analysis and organ culture study in 35 families.

The genetic marker histocompatibility antigen HLA-B8 is present in 80% of patients with gluten-sensitive enteropathy (GSE). We studied 35 families with at least one affected member to determine whether an HLA-region gene alone could determine susceptibility to GSE. The incidence of HLA-B8 in the patients was 69% vs 22% for normals (P less than 0.001). The incidence of GSE in HLA-genotype-identical siblings of patients was only 8%, and in HLA-B8-haplotype-identical siblings and parents of patients was only 14% and 5%, respectively. In addition, intestinal biopsies of HLA-identical or partially identical relatives of patients were studied in an in vitro organ culture system capable of detecting gluten sensitivity in subjects ingesting a normal diet. The results confirmed the low incidence of gluten sensitivity in these individuals. The organ culture system could not differentiate mucosa obtained from unaffected parents or siblings of patients with GSE (who presumably carry the HLA-associated genetic information) from mucosa obtained from normals. We conclude that the genetic material inherited with HLA-B8 alone is not sufficient to produce clinical or subclinical disease. Other genetic and environmental factors appear to be important for disease pathogenesis.

Adolescent↗
PubMed1977

The management of portal hypertension in cystic fibrosis.

We have found that with proper selection and preoperative preparation, a major portosystemic shunt can be done with considerable safety in a majority of cystic fibrosis patients and thus provide them with significant palliation and improved quality of life.

Child↗
PubMed1977

Disaccharidase and lysosomal enzyme activities in amniotic fluid, intestinal mucosa and meconium. Correlation between morphology and disaccharidase activities in human fetal small intestine.

Brush border membrane bound disaccharidases (sucrase and maltase) and lysosomal enzyme (alpha-glucosidase, beta-D-fucosidase and N-acetyl-beta-glucosaminidase) activities awere studied in amniotic fluid (AF). The above enzymes except N-acetyl-beta-glucosaminidase showed a decrease in activity with gestational age beginning at about the 19th week. The activities of sucrase and maltase correlate with the morphological maturation of fetal intestinal mucosa. The distribution of disaccharidases and lysosomal alpha-glucosidase in AF and intestinal mucosa showed different patterns suggesting that these enzymes originate in diverse fetal tissues.

Acetylglucosaminidase↗
PubMed1977

Degeneration of fasciculus gracilis in cystic fibrosis.

The spinal cords of 19 percent of patients dying with cystic fibrosis after 5 years of age showed posterior column degeneration. The risk did not appear to increase with advancing age. None of these patients had findings of pernicious anemia or spinocerebellar degeneration. The lesions had been undetected clinically. It is possible that nutritional, toxic, or hereditary factors may play a role in producing this lesion.

Adolescent↗
PubMed1977

Cystic fibrosis: a new outlook. 70 patients above 25 years of age.

We have presented a group of 70 patients over 25 years of age as of January 1, 1975, who were diagnosed as having cystic fibrosis (CF) at various ages from birth to 28 years. Some patients were diagnosed late in life in spite of classical symptoms and a characteristic history of the disease. Patients with CF present with a broad spectrum of symptoms which vary considerably in severity. This older age group represents approximately 10% of our current roster of patients with CF. This disease is no longer confined to early life and will continue to be seen with increasing frequency in older patients. Our experience with this disease indicates that a number of affected individuals can attain adulthood and find a useful place in society. The quality of life provided to these adults is illustrated in their intellectual, socioeconomic achievements and marital life. Our aim has been to recognize the disease as early in life as possible and to provide a comprehensive medical program which includes all aspects of health care.

Adult↗
PubMed1977

Hemoglobin-oxygen equilibrium in cystic fibrosis.

A study of 35 patients with cystic fibrosis demonstrated that increasing severity of pulmonary involvement was associated with a mild but definite increase in erythrocyte 2,3-diphosphoglycerate (2,3-DPG) and a decrease in hemoglobin affinity for oxygen. The predominant regulators of 2,3-DPG were blood pH, cardiac output, and systemic oxygen transport. No significant relationship was observed between erythrocyte 2,3-DPG content and arterial oxygen tension. Hypophosphatemia may have prevented a greater increase in erythrocyte 2,3-DPG content. The inadequate increase in 2,3-DPG and consequent insufficient change in hemoglobin-oxygen affinity, coupled with an insufficient compensatory erythrocytic response, may adversely affect tissue oxygenation in patients with severe cystic fibrosis.

Adolescent↗