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Biomedical subjects

J R Poley

Publications and source records attributed to J R Poley.

At least 37 records · Page 2Linked to original sources

Quantitative changes of serum lipoprotein-X after cholestyramine administration in infants with cholestatic biliary tract and liver disease.

Lipoprotein-X (LP-X) was determined before and after the administration of cholestyramine in fifty-five infants with persistent cholestatic jaundice to differentiate between intra- and extrahepatic disease. In twenty-seven infants with biliary atresia, serum LP-X prior to cholestyramine ranged from 0.87 to 11.42 g/l (mean: 3.43 g/l; the average concentration was significantly lower (P less than 0.001) in males. After cholestyramine, LP-X rose in twenty-three, remained the same in two, and decreased slightly in two infants. Serum LP-X was present in twenty of the twenty-eight infants with intrahepatic cholestasis prior to cholestyramine in concentrations from 0.84 to 14.19 g/l (mean: 3.13 g/l). After cholestyramine, LP-X decreased in all by an average of 78% (P less than 0.005). The other eight infants did not have LP-X before or after cholestyramine. This study shows that LP-X in the serum of infants with cholestatic jaundice indicates severe cholestasis, but is not itself diagnostic of biliary atresia. The differentiation of biliary atresia from other diseases is readily achieved, as the administration of cholestyramine for 2-3 weeks causes a marked decrease of serum LP-X in patients with patent extrahepatic bile ducts. The absence of serum LP-X excludes biliary atresia.

Bile Ducts, Intrahepatic↗

Disaccharidase deficiency in infants with cow's milk protein intolerance. Response to treatment.

7 infants, aged 5 weeks to 11 months, with clinically documented intolerance to cow's milk protein, chronic diarrhea, and failure to thrive, underwent small intestinal (peroal, suction) biopsy before and after withdrawal of milk proteins. Mucosal specimens were examined by light microscopy and assayed for disaccharidase activities. In all patients, moderate to severe mucosal changes were presented, associated with marked inflammation of lamina propria and damages to the brushborder. Disaccharidase activities (lactase, sucrase, maltase and palatinase) were markedly depressed in all. Follow-up biopsies were obtained in 6 infants, after 3-5 months on a milk-protein-free diet. At the time of the second biopsy, the disaccharidase activities had risen significantly and histologic improvement had occurred in each instance. In infancy, intestinal mucosal lesions due to intolerance to cow's milk protein are histologically indistinguishable from those seen in gluten-sensitive enteropathy and are associated with marked secondary disaccharidase deficiencies. Following therapy, the activity of the disaccharidases become normal or near normal prior to the complete morphologic recovery of the small intestinal mucosa.

Animals↗

Intestinal disaccharidase activities in relation to age, race, and mucosal damage.

Studies were undertaken to determine the relationship of intestinal disaccharidase activity to age and race, and the relationship of mucosal damage to a primary low lactase activity. The first study consisted of data on 399 persons (339 whites, 53 blacks, and 7 American Indians) ages 1 month to 93 years, with normal intestinal histology. Among whites, all 117 children 5 years old or under had high lactase levels, whereas low levels were found only in subjects over 5 years of age. No low lactase levels were identified among the 11 black children 3 years old or under, but in comparison to coetaneous white children, their mean lactase activity was signficantly less. The majority of older blacks had low lactases. In whites and blacks alpha-disaccharidases did not participate in the age-related changes demonstrated with lactase. Of the 7 American Indians, none under 26 months old had low lactase levels, whereas the 4 over 10 years old had low activities. Heterozygotes for sucrase-isomaltase deficiency were identified only among whites. Low lactase levels developed during childhood in all races studied, however, many for unknown reasons maintained their lactose tolerance until adulthood. In the second study of 13 additional children with secondary disaccharidase deficiencies, emergence of a primary low lactase was related to age and race, rather than to mucosal damage. It appears that primary low intestinal lactase levels are absent or rare in whites under 5 and blacks under 3 years of age, and the deficiency is not related to mucosal damage.

Adolescent↗

Abdominal thermography in infantile and childhood liver disease.

Thermography provides an indication of the infrared energy emitted from various skin surfaces and, usually, reflects heat conveyed there from underlying organs. We have used abdominal thermography to study 62 infants and children with acute and chronic liver disease. Thirty-four healthy infants and children were studied as controls. Thermograms were abnormal in 96% of all patients with liver disease. The most common pattern was diffuse heat emission over the liver area, either alone or in combination with vascular and/or mottled patterns. A mottled pattern was more common in chronic liver disease. Although follow-up thermograms were obtained in 28 patients, the data obtained for different types of liver disease are as yet insufficient to warrant conclusions. However, it appeared that reversal of an abnormal thermogram to normal was associated with improvement of liver function and histologic findings in two patients with acute viral hepatitis. On the other hand, persistence of abnormal thermographic records was always associated with persistent hepatic disease as determined by histology and tests of liver function.

Abdomen↗

Role of fat maldigestion in pathogenesis of steatorrhea in ileal resection. Fat digestion after two sequential test meals with and without cholestyramine.

To clarify the role of fat maldigestion in the pathogenesis of steatorrhea in patients with ileal resection the total and aqueous phase concentrations of bile acid and fatty acid were characterized in 8 such patients (5 patients with small ileal resection, bile acid diarrhea, and steatorrhea less than 20 g per day; 3 patients with large ileal resection, fatty acid diarrhea, and steatorrhea greater than 20 g per day) as well as 4 healthy control subjects after a morning and an afternoon liquid test meal. The study was then repeated with cholestyramine, 4 g being administered before each meal to induce fat maldigestion. After a conventional test meal, patients with large resections and severe steatorrhea had significantly lower aqueous phase concentrations of bile acids (and fatty acids) than patients with smaller resections or control subjects, explained in part by intraluminal precipitation of about one-half of the bile acids during digestion. When cholestyramine was administered before the meal, aqueous phase bile acid concentrations decreased in all patients, including the normal control subjects; the degree of fat maldigestion induced in the patients with small resections (and the control subjects) became similar to that present after the conventional test meal in the patients with large resections. Because steatorrhea increased little in the patients with small resections when cholestyramine was administered continuously, the data suggest that fat maldigestion per se does not induce severe fat malabsorption in patients with sufficient anatomical reserve, because such patients can absorb fat efficiently by utilizing the distal small intestine. In patients with large ileal resections, severe steatorrhea is explained in part by the combination of fat maldigestion and decreased surface area. It is also speculated that the steatorrhea occurring in patients with small resections and relatively normal fat digestion during two test meals may be explained by impaired fat digestion which occurs during the final meal of the day, which is often the largest meal.

Adult↗

Zinc therapy in acrodermatitis enteropathica.

An infant is described with acrodermatitis enteropathica, who initially presented with severe and intractable watery diarrhea. Diagnosis was established at the age of eleven weeks. Serum-zinc concentrations were extremely low and urinary zinc excretion was diminished. Eleven days after oral zinc supplement (100 mg elemental zinc per day), the skin lesions had healed. The high therapeutic doses of zinc required for healing are suggestive that zinc malabsorption is an important pathogenetical factor of this disease.

Acrodermatitis↗

Hepatic portoenterostomy: an assessment of its value in the treatment of biliary atresia.

A series of 12 infants undergoing hepatic portoenterostomy for incorrectable-type biliary atresia is reviewed. There has been no evidence of a sustained postoperative increase in bile excretion or improvement in biliary cirrhosis in any patient. Survival statistics for the group as a whole are poor with a mean postop survival time of 11 months and a mean total survival time of 15.3 months. These survival times are worse than that previously reported for infants with untreated biliary atresia. Our disappointing results with hepatic portoenterostomy raise doubts concerning its value in the treatment of biliary atresia.

Biliary Tract↗