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Biomedical subjects

H Shwachman

Publications and source records attributed to H Shwachman.

At least 55 records · Page 3Linked to original sources
PubMed1976

Finger Clubbing: A quantitative survey by analysis of the shadowgraph.

Finger-clubbing has been measured from the magnified silhouette of the right index finger (shadowgram) in 125 subjects, comprising children and young adults. The profile and hyponychial angles were both found to be good discriminators of clubbing and to be independent of age and sex. The normal profile angle was 168.3 degrees +/- 3.6 and the mean normal hyponychial angle was 180.1 degrees +/- 4.2. Patients with cystic fibrosis and cyanotic congenital heart-disease were found to have grossly elevated values of these angles. Some asthmatics were found to have a moderate degree of clubbing with significantly elevated values of these angles. The shadowgram proved a convenient objective method for recording the degree and progress of fingerclubbing in clinical practice.

Adolescent↗
PubMed1976

Gluten-sensitive enteropathy. Inhibition by cortisol of the effect of gluten protein in vitro.

Cortisol partially prevents the harmful effect of gluten on the jejunal mucosa of patients with gluten-sensitive enteropathy. To investigate further the pathogenesis of this disorder, we analyzed the effect of cortisol in cultures of jejunal specimens obtained by biopsy. Cultures were done with and without gluten or cortisol. Morphology and alkaline phosphatase activity were assessed before and after 24 hours. Biopsies from untreated patients cultured with gluten showed low enzyme values and cuboidal epithelial cells before and after culture. Biopsies cultured in a gluten-free medium showed a threefold increase in enzyme values (P less than 0.01) and morphologic improvement with change to columnar epithelial cells. Cultures with gluten plus cortisol showed rises in alkaline phosphatase and morphologic improvement indistinguishable from cultures without gluten. Cortisol and gluten had no effect on cultures from appropriate controls. Cortisol thus prevents the harmful effects of gluten on biopsies from patients with gluten-sensitive enteropathy in vitro.

Adolescent↗
PubMed1976

Echocardiographic assessment of cor pulmonale in cystic fibrosis.

The early clinical recognition and assessment of cor pulmonale are frequently difficult. To evaluate the usefulness of echocardiographic measurement of right ventricular dimension and septal motion in assessing cor pulmonale, we studied 94 patients with cystic fibrosis of varying severity. There was a significant relationship between increasing right ventricular dimension index (RVDI) and (1) decreasing clinical score, vital capacity, forced expiratory volume in one second (FEV1), and maximal midexpiratory flow rate (MMFR); and (2) increasing residual volume and thoracic gas volume. There was no correlation between right ventricular dimension index and PaO2. These findings were supported by electrocardiogram and pathologic evidence of right ventricular involvement. Abnormal septal motion was observed more frequently in patients with severe obstructive airway disease as measured by decreased vital capacity, FEV1, and MMFR. The data show that there is echocardiographic evidence of mild right ventricular enlargement and/or hypertrophy much earlier in the course of cystic fibrosis than previously suspected and that the echocardiogram is a useful tool in the evaluation of cor pulmonale in patients with cystic fibrosis.

Adolescent↗
PubMed1976

Evaluation of the safety of amantadine-HC1 and the role of respiratory viral infections in children with cystic fibrosis.

Amantadine-HC1, an antiviral drug clinically effective against most strains of influenza A virus, was evaluated in a double-blind trial in 153 children with cystic fibrosis during the initial appearance of influenza A/England/42 virus in the New England area. Infection with this variant strain of influenza virus did not reach epidemic proportions during the study, so that the effectiveness of amantadine in this study population could not be fully assessed. However, the potential symptomatic and biochemical toxicity of amantadine was carefully monitored in a pediatric population. Serologic screening by complement fixation tests indicated that respiratory viruses may be important pathogens in exacerbations of respiratory disease in patients with cystic fibrosis.

Adolescent↗
PubMed1976

The pancreas in cystic fibrosis: chemical composition and comparative morphology.

Sections of pancreas from 16 individuals who died with cystic fibrosis (CF) were classified by morphometric criteria into four categories in increasing order of pancreatic involvement. The concentration of acini, islets, main ducts, lobular ducts, connective tissue, and fat was compared with control levels. The results show that in the least involved pancreases, from neonates who died under 5 months of age, acini were reduced to 33% of control levels and the following were increased: islets, to 410%, lobular ducts, to 250%; and main ducts, to 1700% of controls. With increasing severity of the pancreatic disease the acini were further reduced to 5% and lobular ducts to 37% of control levels, respectively. Main ducts increased by 19-fold, and fatty infiltration accounted for more than 25% of the fresh weight of the pancreas in 9 of the 16 specimens. Comparative biochemical studies of 35 fibrocystic pancreases were quantitatively related to the severity of the pancreatic involvement as follows. Water and volatile matter, normally accounting for 80 +/-% of the weight of the fresh pancreas, was reduced to less than 30% in the most affected organs. The concentration of zinc diminished from near normal mean levels of 193 mugZn/g dry pancreas to 10% of this amount in the severely involved pancreas. Elevated concentrations of calcium, amounting to over 10 times control level, were found in obstructed ductal structures. Calcium was depleted from pancreatic sections adjacent to the obstructions. The following biochemical indicators were significantly different in their mean levels in the 35 fibrocystic pancreases when compared with the 17 controls: (P less than or equal to 0.001) fat, water, zinc, calcium, copper, magnesium, potassium, and sodium (P less than or equal to 0.01).

Adolescent↗
PubMed1976

The coexistence of cystic fibrosis and celiac disease.

Two patients with cystic fibrosis (CF) who subsequently developed celiac disease (CD) are described. Since organ culture of intestinal mucosa has been used to establish an in vitro model for the study of CD, we utilized this opportunity to determine whether duodenal mucosa obtained from each of these two patients and their immediate families differed in its organ culture behavior from mucosa obtained from patients with CD alone. Additionally, as specific HL-A types are associated with CD, we used HL-A typing to determine whether the two patients with CF-CD differed genetically from patients with CD alone. One of our patients was HL-A8, the most common type associated with CD; the other was HL-A12, as are many of the non-HL-A8 celiac patients. The response in organ culture of the mucosa of these two patients was the same as the response in organ culture of the mucosa from patients with CD alone. These and other data suggest that CD occurring in patients with CF is no different than CD occurring alone.

Adolescent↗
PubMed1976

Enterokinase and trypsin activities in pancreatic insufficiency and diseases of the small intestine.

The interrelationship of enterokinase and trypsin activities were investigated in 133 infants and children with a variety of gastrointestinal and pancreatic disorders. Fourteen patients with diarrhea and grade II mucosal injury revealed a significant (P less than 0.01) reduction of enterokinase, trypsin, and disaccharidase activites as compared to 59 children with normal mucosa. Nine patients with cystic fibrosis and pancreatic insufficiency had normal mucosal enterokinase activity and elevated intraluminal enterokinase activity with very low or no trypsin activity. Patients with hypoproteinemia and gastrointestinal protein loss, associated with intestinal lymphangiectasia (4 patients) and intestinal lymphoid nodular hyperplasia (3 patients), had normal or insignificant decrease of enterokinase and trypsin activities. In patients with steatorrhea, a normal sweat test, normal intestinal mucosa, and absent trypsin activity, two entities were defined. One group (3 patients) was diagnosed as Schwachman-Diamond syndrome with pancreatic insufficiency and normal mucosal and intraluminal enterokinase activity. The second group (2 patients) with absent mucosal and intraluminal enterokinase activity and normal lipase and amylase activities was diagnosed as congenital enterokinase deficiency.

Child↗
PubMed1975

Duodenal microflora: a prospective study in pediatric gastrointestinal disorders.

Culture of the duodenal microflora was performed on 96 infants and children with a variety of gastrointestinal disorders. The resident microflora consists predominantly of oral-type microorganisms such as alpha-Streptococci and Neisseria. A fecal-type microflora was found in all 6 infants with post-surgical diarrhea and temporary monosachcaride intolerance; 4 of 22 patients with protracted diarrhea of infancy, 3 of 22 patients with celiac disease, and none of 32 patients with nonspecific diarrhea or 14 others with miscellaneous disorders. Anaerobic microorganisms were seldom isolated. Only 9% of the duodenal cultures were completely sterile, although a further 17 had a quantitative culture less than 5 times 10(4) microorganisms/ml. Small-bowel contamination with fecal-type microorganisms is associated with stasis and disturbance of the normal peristaltic-clearing mechanism. Duodenal culture is essential in any infant with temporary monosaccharide intolerance. It is occasionally useful in other situations where motility may be disturbed.

Adolescent↗
PubMed1975

Correlation of lactase activity, lactose tolerance and milk consumption in different age groups.

Small intestinal lactase activity in the health adult is either the same as in early infancy or may drop to very low levels. The behavior of the enzymatic state varies with the ethnic group studied. In those adults with low lactase activity little information is availalbe as to the age at which the lactase decreases. We attempted to determine a) the frequency of low intestinal lactase activity and b) the age at which the change occurs. For this purpose we reviewed in a large number of intestinal biopsies both histologically as well as for disaccharidase activities. The biopsies were obtained from a heterogeneous group of Caucasians, including patients, their siblings and parents. The patients were those with failure to thrive in whom no organic cause could be elicited, and those with the irritable colon syndrome. Patients ranged in age from 6 weeks to 50 years and out of a total of 1, 077 jejunal biopsies, 172 morphologically normal biopsies were selected. The milk drinking habits of 118 subjects and their families were elicited and 31 oral lactose tolerance tests performed. The mucosal lactase activity and sucrase-to-lactase ratio in those 172 individuals were plotted against age. In the first 3 years the mean lactase activity was 32.1 plus or minus 10.1 mumoles/g protein per min and the sucrase-to-lactase ratio was 1.7 plus or minus 0.5 with no change from year to year. However, after age 5 two separate groups emerge. A small group (24.6% of the population) with low lactase activity, and a second group possessing the same mean value for lactase activity as noted in the first 3 years. The low lactase activity group included children and adults with clinical lactose intolerance. These individuals consumed relatively small amounts of milk and when 12 of them were tested with an oral lactose tolerance test the result was a "flat" curve with a maximum rise in blood glucose of 9 plus or minus 3.2 mg/100 ml. The second group consumed more milk averaging 1 quart/day with no discomfort and when 19 were tested with oral lactose tolerance tests the values were normal. This study indicates that low lactase activity in the Caucasian population may make its appearance at the age of 5 years.

Adolescent↗
PubMed1975

Lead in human scalp hair: some factors affecting its variability.

The accumulation of lead in human scalp hair was compared in male and female children and adults from various locations in the United Sates, Japan, Yugoslavia, Iran, and South Africa. The most significant variables which influenced the concentration of lead in hair were ingestion of lead-containing substances, exposure to lead of environmental origin, place of residence, site from which the hair specimen was sampled relative to its distance from the scalp, and age. The least significant variables were sex and nutritional deficiencies.

Adolescent↗
PubMed1975

Selective bronchial arteriography in patients with cystic fibrosis and massive hemoptysis.

Massive hemoptysis is a potentially fatal complication of long-standing cystic fibrosis. Lobectomy may prevent a hemorrhagic death if the hemorrhage source can be identified and if involvement of the remaining lung tissue is mild. Selective bronchial arteriography was performed in six patients with cystic fibrosis to localize a lobar source of bleeding. Arteriographic findings correlated with bronchoscopic observations. Bronchial arteriography may be helpful when bronchoscopy cannot be done because of continuous hemorrhage or because the severity of the lung disease precludes general anesthesia, but it is not an adequate substitute for bronchoscopy in most patients.

Adolescent↗
PubMed1975

A new assay for cystic fibrosis factor: effects of sera from patients with cystic fibrosis in the in vitro electrical properaties of rat jejunum.

The in vitro electrical properties of rat jejunum were utilized to assay a factor or factors in serum from patients with cystic fibrosis (CF). Sera from patients with CF were found to decrease short circuit current (SCC) and the SCC response to glucose, and to increase electrical resistance. These effects were present in all 24 patients with CF and 10 of 14 parents of CF patients and absent from the serum of 21 control subjects. This quantitative technique provides an alternative to the ciliostatic assays for the detection of the CF factor or factors.

Animals↗