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Biomedical subjects

E Lebenthal

Publications and source records attributed to E Lebenthal.

At least 181 records · Page 10Linked to original sources
PubMed1980

Gardner's syndrome. Recent developments in research and management.

In recent years, a number of comprehensive reviews have been written on inherited intestinal polyposis syndromes (1-7), but none has dealt specifically with Gardner's syndrome and none has focused on basic research being carried out in an attempt to understand this syndrome and to improve the medical management of affected patients. A better understanding of this rare genetic disorder is essential for surgeons, gastroenterologists, cancer researchers, and geneticists alike. To the clinician, it poses difficult challenges in management; to the cancer researcher, it presents a rare opportunity to study very early premalignant transformations; and to the geneticist, it poses exciting questions at the cellular, chromosomal, and molecular levels.

Adult↗
PubMed1980

Glucoamylase and disaccharidase activities in normal subjects and in patients with mucosal injury of the small intestine.

The development of glucoamylase activity was compared to that of disaccharidase in the small intestinal mucosa of infants and children. By the age of one month, infants have glucoamylase and disaccharidase levels comparable to those of young adults, indicating that young infants may be able to digest and absorb starches. In infants and children with varying degrees of mucosal injury of the small intestine, the activities of glucoamylase decreased progressively with increasing severity of the villus atrophy. However, the reduction of lactase, palatinase, and sucrase activities was more severe than the loss of activities of glucoamylase and maltase. Thus, children and infants may tolerate polymers of glucose better than disaccharides when they have mucosal injury associated with prolonged diarrhea.

Adolescent↗
PubMed1980

Intrahepatic cholestasis for 15 years without cirrhosis.

Whether prolonged cholestasis is followed by hepatic cirrhosis is still controversial. We have studied two unrelated children who have had cholestasis for 15 years, but neither of whom have developed cirrhosis. Both have severe growth retardation, peculiar facies, pulmonic stenosis, transitory renal tubular acidosis, and vitamin D-resistant rickets. The patients presented in infancy with hepatomegaly and direct hyperbilirubinemia; liver biopsy at that time revealed cholestasis and paucity of bile ducts. Subsequent serial liver biopsies have continued to demonstrate cholestasis, but there has been no evidence of cirrhosis. Electron microscopy has revealed swollen and blunted microvilli of the canalicular membrane of the hepatocyte. The patients have had elevated bile acids in the serum as well as a reversed ration of tri- to dihydroxy bile acids. Treatment with cholestyramine and phenobarbital has brought about symptomatic relief from severe pruritus and excoriation and has lowered the level of serum bile acids, although they are still above the normal range. These findings suggest that cholestasis accompanied by an elevated and reversed bile acid ratio does not universally cause hepatic cirrhosis.

Adolescent↗
PubMed1980

Reovirus type 3 infection in a suckling mouse: the effects on pancreatic structure and enzyme content.

Alterations in pancreatic function and structure were examined in suckling mice infected intraperitoneally with reovirus type 3. The results were compared to pancreatic zymogen enzyme activities and histology in adult mice infected with the same virus. No effect of the rovirus type 3 on the adult mice could be elicited. In contrast, the suckling mice infected by the reovirus type 3 revealed a definite change in pancreatic zymogen enzymes. However, the zymogen enzymes were affected in a nonparallel fashion and three groups of enzymes with different responses were noted. Amylase and lipase activities were significantly diminished (P less than 0.001) at 6 days of viral infection. The endopeptidases, trypsin (P less than 0.025) and cymotrypsin (P less than 0.001) activities were increased significantly in the infected group. The exopeptidases, carboxypeptidase A and B in the infected animals were not changed significantly compared to the control. It seems reasonable that the reovirus type 3 infection in the suckling mouse causes diminished lipase and amylase activities that might contribute to the pathogenesis of viral enteritis.

Amylases↗
PubMed1980

Small intestinal epithelial brush border enzymatic changes in suckling mice infected with reovirus type 3.

Suckling mice infected with reovirus type 3 were examined for changes in the epithelial brush border of the small intestine. After 3 days of infection with reovirus type 3, no significant changes were found in intestinal morphology or activity of any enzymes tested. After 6 days, villi were shortened and blunted with lymphangiectatic lesions and mild mononuclear infiltration in the lamina propria. In addition, there was a significant decrease in lactase (P < 0.001) and enterokinase activity (P < 0.05). However, there were no significant changes in the activities of alkaline phosphatase. In contrast, maltase (P < 0.001) and leucine aminopeptidase (P < 0.05) activities in the infected mice were significantly increased. These data suggest that brush border enzymes are affected differently by reovirus infection.

Alkaline Phosphatase↗
PubMed1980

The effect of reduced maternofetal blood flow on the development of fetal pancreatic acinar cells and enzymes.

It has been noted that from days 18 to 22 (birth) during the second intrauterine period of morphogenesis of the rat pancreas the accumulation of enzymes increases dramatically. We studied the effect of altered maternofetal blood flow on the development of the rat pancreas during the critical second period. Our studies indicate that during pancreatic cytodifferentiation, reduction in maternofetal blood flow not only reduces the weight of the pancreas (68% of control) and diminishes the total activities of enzymes but that the changes in specific activities of the enzymes do not appear to be coordinate. The specific activities of amylase decreased to 59,000 units from the control value of 103,000 units (P less than 0.01) and lipase decreased to 4000 units from a control value of 7350 units (P less than 0.001). In contrast, the specific activities of trypsin (ogen), chymotrypsin (ogen) and (pro)-carboxypeptidase A and B are not changed. These results suggest that reduction in maternofetal blood flow caused a selective decrease of fetal rat amylase and lipase during the third trimester of gestation.

Amylases↗
PubMed1980

Development of functional responses in human exocrine pancreas.

The ability of newborns to digest proteins, fats, and carbohydrates depends, to a large extent, on their level of exocrine pancreatic function. Building on the limited published data, we studied pancreatic enzyme activities in the duodenal fluid and the response of the exocrine pancreas to secretogogues in 15 premature and full-term infants at birth and at 30 days of age. We compared these findings to those obtained from identical studies of 17 children age 2 years and above. In addition, we measured the pancreatic exopeptidase, carboxypeptidase B, in relation to other pancreatic enzymes. The duodenal fluid of newborns and infants contained no amylase and negligible lipase. Carboxypeptidase B levels were also low compared to those in the older children. In contrast, chymotrypsin activity in infants was about 50% to 60% of level found in the older children. Trypsin activity, the highest of all the enzymes measured, was about the same in both newborns and older children, with a transient increase at 30 days. Administration of pancreozymin had no effect on pancreatic enzymes in the duodenal fluid of newborns and a slight effect on 1-month-old infants. But by age 2 years, a full response of the pancreas to pancreozymin was evident. In infants and newborns, responses to secretin were poor. Thus, the secretory response of the human pancreas to secretogogues, absent or minimal at birth, is acquired during the postnatal period.

Amylases↗
PubMed1980

Extent and duration of small intestinal mucosal injury in intractable diarrhea of infancy.

Thirty infants with intractable diarrhea of infancy (IDI) underwent small bowel biopsies in order to determine the extent and duration of small intestinal mucosal injury. The onset of the persistent diarrhea occurred prior to 3 months of age and continued for an average of 48 days prior to investigation. In 18 cases, no associated entities were found. Mucosal injury was invariably found in all 30 infants: grade IV injury in 11, grade III in eight, grade II in nine, and grade I atrophy in one. Disaccharidase activities were diminished and corresponded to the degree of atrophy. Lactase activity was diminished to a greater extent than sucrase and maltase. Significant, persistent mucosal injury existed for an average of six months in 16 of the 23 (70%) repeat biopsies. All infants were given an elemental diet (ED). Twelve of the 30 infants required parenteral nutrition (PN). These infants were gradually advanced to an oral elemental diet and maintained on this diet until histologic findings and disaccharidase levels were normal. Eighteen infants were fed and maintained on an elemental diet by mouth from time of admission until normal histologic findings and disaccharidases were found. No mortality occurred during management and follow-up. Twenty-two of the 28 infants in whom follow-up growth data were available excelled in weight and height velocity. The data suggest that prolonged injury to the small intestinal mucosa is a common finding in many cases of intractable diarrhea of infancy. Elemental diets should be started early in the course of protracted diarrhea in young infants, and may need to be continued for several months since histologic and enzymatic changes of the small intestine may persist for extended periods.

Body Height↗
PubMed1980

Nodular colonic mucosa of childhood: normal or pathologic?

The small colonic mucosal nodules that have been referred to in the past as lymphatic nodular hyperplasia are felt to represent a normal response of the prominent colonic lymphatic tissue in children. To support this hypothesis, a prospective study of all air-contrast barium enemas, colonoscopies, and intestinal biopsies performed during 1 yr was carried out. During that year 48 patients had air-contrast barium enemas, and 21 of these 48 patients also had colonoscopy and mucosal biopsies. Greater than half of the children studied by air-contrast barium enema (26 out of 48) and colonoscopy (12 out of 21) had nodular colonic mucosa. These nodules were described at colonoscopy as being smooth surfaced, yellowish-white, 1-2 mm in diameter. There was no correlation between the presence or absence of these nodules with any symptom or symptom complex. The younger patients, the more frequently these nodules were oberved. No immunologic disorder was associated with the nodular colonic mucosa in the children that were tested. Nodular colonic mucosa in childhood should be considered a frequent finding indicative of normal lymphatic response to nonspecific stimuli, which may often be infectious in nature.

Barium Sulfate↗
PubMed1979

Effect of postnatal malnutrition on pancreatic zymogen enzymes in the rat.

In order to characterize the response of the pancreas to undernutrition during the critical neonatal growth phase, acquired postnatal malnutrition was induced in the rat, using the expanded litter. An experimental nursing litter of 16 rats and control litters of 7 to 8 rats were formed. At 19 days of age, the pups were killed. Mean pancreatic wet weight was decreased in the malnourished rat to a greater extent than the decrease in total body weight (49 versus 60%). Decreased organ weight was predominantly the result of a decrease in DNA content and cell number. Enzyme activities expressed per total organ were all diminished; lipase to the greatest extent; trypsin and amylase to an intermediate extent; followed by chymotrypsin and the carboxypeptidases. The specific activities of lipase and trypsin were decreased with lipase, the most severely effected. The low trypsin levels can be attributed to trypsin inhibitor. It is possible therefore, that only the specific activity of lipase is significantly decreased. The decrease in enzyme activities, expressed both as specific activities and as total organ activities were decreased in a nonparallel fashion.

Amylases↗
PubMed1979

The effect of early postnatal acquired malnutrition on intestinal growth, disaccharidases and enterokinase.

The effect of undernutrition on rat small intestine during the critical newborn period was studied. A severe state of protein-energy malnutrition was induced by litter expansion which caused the mean total body weight of experimentally malnourished rats to diminish significantly as compared to control animals. Intestinal weight and total DNA were similarly diminished in the malnourished rats. DNA and protein expressed per gram wet tissue showed no significant differences between groups. Retarded intestinal growth in the malnourished animals was the result of reduced cell number. The mean specific activities of sucrase and maltase were diminished in the experimental group, with mean activities being 20 to 50% of controls, respectively. These differences were larger when expressed as total organ activities. On the other hand, specific lactase activity was significantly higher in undernourished rats but total lactase activity per organ was similar in both groups. Enterokinase specific activity or total organ activity was significantly higher in the undernourished rats.

Alkaline Phosphatase↗
PubMed1979

Small intestinal changes in enterocolitis complicating Hirschsprung's disease.

Hirschsprung's disease is one of the more common causes of childhood bowel obstruction. The disease can cause enterocolitis which, untreated, may result in considerable mortality. We describe our evaluation of two infants who suffered from intractable diarrhea of infancy secondary to Hirschsprung's disease. We found that mucosal damage to the small bowel and disaccharidase deficiency are among the most important mechanisms producing this complications. Therefore, management of enterocolitis including parenteral alimentation should proceed as soon as the complication is observed.

Biopsy↗
PubMed1979

Early detection of adenomatous polyposis coli in Gardner's syndrome.

Gardner's syndrome, an autosomal dominant disorder, consists of multiple polyposis of the colon associated with various soft- and hard-tissue tumors. The appearance of adenomatous hyperplasia and polyposis in at-risk patients before adolescence has not been full appreciated. Four preadolescent children from a kindred with Gardner's syndrome were examined by use of colonoscopy and mucosal biopsy. In three children (18 months, 6 years, and 9 years old) adenomatous hyperplasia or polyposis was found. The colon of the fourth child (3 years old) was normal. The three affected children were asymptomatic. The youngest had a barium enema and results were normal. The oldest child had polyps. Biopsies revealed focal atypical hyperplasia of the glands with pseudostratification of the epithelial cells, frequent mitotic figures, and the absence of goblet cells. More severe manifestations were noted in the splenic flexure than in the sigmoid flexure or rectum. The youngest patient showed early adenomatous hyperplasia characterized by a marked reduction of the goblet cells, especially in the surface epithelium. Colonoscopy and mucosal biopsies are mandatory in at-risk children. By deferring colonic examination until adolescence, a patient is placed at risk for malignant degeneration of the adenomatous tumor.

Adenoma↗