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Biomedical subjects

B S Kirschner

Publications and source records attributed to B S Kirschner.

51 records · Page 3Linked to original sources

Nutritional consequences of inflammatory bowel disease on growth.

This review summarizes much of the current information concerning the problem of growth failure in children and adolescents with inflammatory bowel disease. Growth impairment is discussed as a dynamic process and ways of recognizing and monitoring subtle changes in growth velocity are presented. Factors which contribute to growth delay are described, with special attention to prolonged insufficient nutrient intake. Studies using different methods of nutritional intervention to reverse growth failure are presented as a guide to the management of this challenging group of patients.

Adolescent↗

Somatomedin-C levels in growth-impaired children and adolescents with chronic inflammatory bowel disease.

The relationship between caloric insufficiency and impaired growth in children with chronic inflammatory bowel disease has been increasingly recognized in recent years. The mechanism by which nutritional insufficiency leads to decreased growth in these children is unclear. Our study suggests that chronic undernutrition lowers circulating somatomedin-C, which is known to exert anabolic effects on peripheral tissues. Therapeutic intervention that increases caloric intake results in improved somatomedin-C levels and growth velocity. Monitoring somatomedin-C levels in growth-impaired children with inflammatory bowel disease provides an important marker of nutritional sufficiency and reversibility of growth retardation.

Adolescent↗

Diagnosis and differentiation of fat malabsorption in children using 13C-labeled lipids: trioctanoin, triolein, and palmitic acid breath tests.

Three substrates labeled with nonradioactive 13C have been employed to establish a trilogy of noninvasive breath tests to detect fat malabsorption in children and then to differentiate the etiology of the steatorrhea. Administration of 17 mg/kg of (13C)triolein Lipomul (The Upjohn Co., Kalamazoo, Mich.) resulted in a peak excretion rate of 13CO2 greater than 2.7% dose/h in 10 normal subjects (mean value 4.96 +/- 2.2% dose/h) whereas all 17 subjects with fat malabsorption were below this value (mean value, 0.75% +/- 0.63% dose/h); p less than 0.001). For the detection of fat malabsorption, the discriminative value of (13C)triolein was superior, 100% sensitive, and 89% specific, while the use of (13C)palmitic acid (17 mg/kg) or (13C)trioctanoin (7.5 mg/kg) alone yielded both false-positive and false-negative results. In 6 out of 6 cases, pancreatic insufficiency could be differentiated from mucosal disease (7 patients) or bile salt deficiency (4 patients) by the presence of abnormal triolein or trioctanoin breath tests, or both but normal palmitic acid breath tests. However, further differentiation of mucosal disorders from bile salt disorders could not be achieved using either a single- or a multiple-substrate breath test. The use of the single triolein breath test in children offers an attractive, sensitive alternative to conventional fecal fat measurements to establish the presence of steatorrhea, and when using 12C-lipid with multiple substrates, the tests are capable of providing additional insight into the mechanism of fat malabsorption.

Adolescent↗

Reversal of growth retardation in Crohn's disease with therapy emphasizing oral nutritional restitution.

Seven children with Crohn's disease and growth retardation responded to therapy which focused upon oral nutritional restitution and maintainence. The study population included children whose disease activity varied from mild to severe, but patients who were receiving daily suppressive doses of corticosteroids were not included. Diet counselling and supplementation were designed to reverse the prominent deficit in caloric intake which was characteristic of each patient before therapy. An increase in caloric intake from 1535 +/- 148 kcal/day to 2493 +/- 108 kcal/day (a change from 56% to 91% of that recommended for height age and sex) was achieved. Five patients increased growth velocity from a mean of 1.8 cm/yr to a mean of 6.2 cm/yr after drug therapy and oral nutritional supplementation. In 2 other patients, oral nutritional therapy was supplemented for a period of 4 wk by peripheral intravenous nutrition; growth velocity in these patients increased from < 2.0 cm/24 mo to 4.2-6.5 cm in the subsequent 12 mo. All patients showed an increase in height percentile with 5 of 7 patients reaching within 5% of their preillness height percentile. These results emphasize the importance of nutritional restitution in restoring growth in children with Crohn's disease and provide a promising approach to the treatment and prevention of growth retardation in many of these children.

Adolescent↗

Lactose malabsorption in children and adolescents with inflammatory bowel disease.

Lactose breath hydrogen tests were given to 70 children and adolescents with chronic ulcerative colitis and Crohn's disease in order to determine the prevalence of lactose malabsorption in childhood inflammatory bowel disease. Twenty-nine percent of these patients demonstrated lactose malabsorption; the majority of these children (70%) experienced gastro-intestinal symptoms during the test. The prevalence was not significantly different whether the diagnosis was ulcerative colitis or Crohn's disease. With the exception of those with diffuse small bowel disease, the location of intestinal involvement with Crohn's disease and the severity of clinical symptoms did not affect lactose malabsorption. Lactose malabsorption was not more frequent in patients with inflammatory bowel disease than in a group of children with recurrent abdominal pain and normal gastrointestinal x-rays, although significant differences in the prevalence of lactose malabsorption were observed in relation to ethnic background. Milk incubated with commercially available yeast lactase (lactAid, Surgarlo Co., Atlantic City, N.J.) for greater than 24 h prevented an increase in breath hydrogen when administered to 6 patients previously shown to have lactose malabsorption.

Adolescent↗

Chronic bullous dermatosis of childhood.

The clinical features, laboratory studies, and therapeutic responses of two boys with chronic bullous dermatosis of childhood are described. Direct immunofluorescent preparations of sections from a lesion, skin adjacent to a lesion, and uninvolved skin demonstrated linear deposition of IgA at the dermoepidermal junction in all three biopsy specimens from one patient. Similar preparations from the second child were negative for staining. No circulating antibodies to skin components were detected in either child by means of multiple substrates. Neither child had clinical manifestations nor laboratory findings suggestive of an associated gastrointestinal lesion; therefore, small bowel biopsies were not performed. Immunologic studies failed to demonstrate any of the abnormalities frequently described in dermatitis herpetiformis. Both boys responded dramatically to sulfapyridine therapy.

Age Factors↗

Peptic ulcer disease in children.

Physician recognition of peptic ulcers in children is a challenging problem because of the multiple, often nonspecific, symptoms. A compatible history and upper gastrointestinal contrast study were formerly all that was available to the practitioner trying to confirm his clinical impressions. With the advent of new developments in gastroduodenal endoscopy, lesions not detectable radiologically can be localized, and the diagnosis firmly established. As experience with this procedure in children accumulates, the incidence of other conditions previously attributed to ulcer disease may be apparent, thus enlarging our concepts of causes of upper gastrointestinal bleeding in children.

Age Factors↗

Defect in fatty acid oxidation: laboratory and pathologic findings in a patient.

The clinical, laboratory, and pathologic findings in a patient with a previously undescribed deficiency in fatty acid oxidation are summarized. The patient had a fatal defect in fatty acid metabolism profoundly affecting heart, skeletal muscle, liver, and kidney. Oxidation of palmitate was 38-51% of controls. Complementation assays demonstrated that the patient's fibroblasts complemented fibroblast lines from all known defects in fatty acid oxidation except long-chain acyl-CoA dehydrogenase deficiency. Urine and serum carnitine profiles also were indicative of a defect in the oxidation of long-chain substrate; however, the palmitoyl-CoA dehydrogenase activity was actually increased. This finding indicates that the patient had a defect that was distinct from, but possibly related to, long-chain acyl-CoA dehydrogenase deficiency. This patient demonstrates the laboratory and pathologic findings in defects in fatty acid oxidation and how they differ from those in Reye syndrome.

Acyl-CoA Dehydrogenase, Long-Chain↗

Secondary amyloidosis in Crohn's disease of childhood.

Secondary amyloidosis is a rare complication of Crohn's disease, especially in pediatric patients. This report describes an 11-year-old child with Crohn's disease who developed intermittent proteinuria and thyroid enlargement at 14 years of age as the initial manifestations of secondary amyloidosis. Deterioration in renal function resulted in her death at 20 years of age. Published reports that discuss the occurrence of systemic amyloidosis in Crohn's disease and the pediatric age group are reviewed.

Adolescent↗