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Biomedical subjects

S W Bender

Publications and source records attributed to S W Bender.

At least 37 records · Page 2Linked to original sources

Biochemical quantification of crypt hyperplastic villous atrophy by aldolase activity assay.

Aldolase activity with the two substrates fructose-1-phosphate and fructose-1,6-diphosphate was measured in the homogenate of small intestinal biopsy specimens from children with different malabsorptive diseases (celiac disease, cow's milk protein intolerance, infectious diarrhea, giardiasis, and Crohn's disease) and controls. It is demonstrated that the ratio of fructose-1,6-diphosphate/fructose-1-phosphate activity, which reflects the relative amounts of the crypt enzyme aldolase A (EC 4.1.2.13) and the villous enzyme aldolase B (EC 4.1.2.7), correlates very well with both the ratio of crypt to villous height (correlation factor r = 0.92) and the mitotic index (r = 0.80).

Biopsy↗

Primary intestinal enteropeptidase deficiency.

A rare case of primary enteropeptidase deficiency is reported. Details are given of the initial clinical presentation, treatment, and response of the patient from birth to the age of 18 months. Biochemical analysis of a small intestinal biopsy and duodenal juice samples confirmed the total absence of enteropeptidase activity. Proteolytic activity was very low in native duodenal juice, but normal levels could be induced by activation in vitro with a small amount of porcine enteropeptidase.

Diet↗

Anion transport and 2,3-diphosphoglycerate in cystic fibrosis red blood cells.

The monovalent and divalent anion transport in red blood cells from patients with cystic fibrosis and controls is not significantly different. The protein pattern of the erythrocyte membrane, as seen by sodium dodecyl sulfate-polyacrylamide gel electropherograms, is similar in cystic fibrosis and control red blood cells. The 2,3-diphosphoglycerate content of the investigated cystic fibrosis red blood cells is normal.

2,3-Diphosphoglycerate↗

Early diagnosis of intestinal non-Hodgkin lymphoma by endoscopy.

The early diagnosis of intestinal non-Hodgkin lymphoma, stages I and II, lymphoblastic of Burkitt's type, was made by colonoscopy combined with selective radiologic visualization of the tumor in two children, ages 4 and 5. This technique is recommended in patients with abdominal pain, iron-deficiency anemia, intestinal blood loss, and an elevated sedimentation rate, when conventional radiologic and ultrasound techniques fail.

Abdomen↗

[Abetalipoproteinemia (author's transl)].

A 21/2-years-old Greek girl with marked failure to thrive and steatorrhea diagnoses to suffer from abetalipoproteinemia by the dissecting microscope appearance of a white intestinal mucosa. This specific finding is due to storage of lipids in the enterocytes. Another typical finding is the acanthocytosis in blood smears. Serum lipids were studied in detail in the patient and her relatives; there was a marked reduction of the low and very low density lipoproteins in the patient only (LDL/VLDL). The most characteristic finding is the lack of apolipoprotein B.

Abetalipoproteinemia↗

[Infection due to Yersinia enterocolitica in healthy and chronically ill children (author's transl)].

A septicaemic course of infection due to Yersinia enterocolitica developed in three children, two Italian girls with thalassemia (serotype 03) and a previously healthy boy (serotype 09). There were marked gastrointestinal symptoms. The boy required two transfusions for acute haemolysis. Because it may take a serious course, the possibility of this organism as the causative agent should be considered in the differential diagnosis of severe infections, especially in patients with chronic debilitating disease. The death rate is about 30%.

Blood Transfusion↗

Alpha-1-antitrypsin deficiency: fulminant course in early infancy.

A severe course of alpha-1-antitrypsin deficiency in two children with Pi ZZ genotype is demonstrated. Clinically both infants presented with signs indistinguishable from biliary atresia with acholic stools and a low I131-rose bengal excretion over 72 h. Rapid development of liver fibrosis documented by serial liver biopsies, indicated a bad prognosis. The infants died at the age of 6 and 15 months. The extent of fibrosis is estimated to be the most useful prognostic sign in prediction of the probable outcome in alpha-1-antitrypsin deficiency.

Bile Ducts↗

Evaluation of long term tobramycin therapy in patients with cystic fibrosis and advanced pulmonary disease.

To nine cystic fibrosis patients with chronic bronchopulmonary infection of severely damaged lungs invaded by Pseudomonas aeruginosa, eleven courses of prolonged tobramycin treatment (5 mg/kg/day) for four to 16 weeks were administered. Pulmonary symptoms improved and a better quality of life was achieved in all but one patient. Objective parameters (chest X-ray, pulmonary function tests) changed to a lesser extent. In only one patient was Pseudomonas eradicated from the sputum but reappeared after discontinuation of therapy. In the rest of the patients Pseudomonas was significantly suppressed or replaced by other pathogens. Four patients showed rises of antibody titres to Candida and two to Aspergillus fumigatus. No nephrotoxic side effects were observed, but vestibular function was reversibly impaired in one patient without corresponding clinical symptoms. No bacterial resistance to tobramycin was observed during therapy.

Adolescent↗

Gamma heavy chain disease simulating alpha chain disease.

A young Turkish girl presented with all the clinicopathological features of a digestive form of alpha chain disease. A gamma heavy chain disease protein, however, was found in her serum and also in the cells invading the intestinal mucosa and mesenteric lymph nodes.

Child↗