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Biomedical subjects

W E Berquist

Publications and source records attributed to W E Berquist.

At least 37 records · Page 2Linked to original sources
PubMed1989

Surgical management of chronic intestinal pseudo-obstruction in infancy and childhood.

Chronic intestinal pseudo-obstruction is an uncommon cause of repeated obstruction in children of undetermined etiology, often leading to repeated laparotomies and early death. TPN combined with venting gastrostomy provides sufficient calories for growth and minimizes the need for operations and/or hospitalizations. Seventeen children managed with these techniques experienced a sixfold decrease in the number of hospitalizations, and more than a tenfold decrease in the number of laparotomies for obstruction compared to the period before routine use of TPN and gastrostomies. Oral feedings are encouraged whenever possible.

Age Factors↗
PubMed1989

Absence of hyperparathyroidism in severe liver disease.

Elevated serum levels of intact parathyroid hormone (PTH) have been reported in severe versus mild biliary cirrhosis. The aim of this study was to determine whether hyperparathyroidism was present in severe liver disease on the basis of the inability of the liver to catabolize the hormone. Because biologic activity resides in the amino terminal, and amino terminal PTH determinations have not been routinely made in liver disease, it is possible that hyperparathyroidism was previously missed in these patients. Accordingly, we obtained fasting blood from 11 patients with severe liver disease and 8 age-matched controls. We measured intact, amino terminal, and mid-region PTH, vitamin D metabolites, bone gamma carboxyglutamic acid protein (BGP), ionized calcium, phosphorus, magnesium, and liver function tests. Serum levels of PTH were normal with all assays and 1,25(OH)2D levels were not elevated. These findings argue against the possibility that hyperparathyroidism plays a role in the pathogenesis of hepatic osteodystrophy.

Adult↗
PubMed1989

Sclerotherapy for extrahepatic portal hypertension in childhood.

Ten children with extrahepatic portal hypertension who had major bleeding from esophageal varices were treated with sclerotherapy of esophageal varices by means of flexible fiberoptic endoscopy and intravenous sedation. Four had had no previous therapy, five had had previous surgery for variceal bleeding, and five had received propranolol orally. During therapy and follow-up monitoring of 1.4 to 7.1 years (mean 4.7 years), only two patients bled again from esophageal varices, one before complete obliteration of varices and one who temporarily defaulted on follow-up. The few complications were easily managed, and only three required any specific therapy. No child bled from gastric varices. Frequency of sclerotherapy sessions and quantity of sclerosant could be decreased with time, usually after 3 years of sclerotherapy, suggesting that the natural history of decreased bleeding with time in extrahepatic portal hypertension may be accelerated by sclerotherapy. Esophageal varices in children with extrahepatic portal hypertension may be treated safely with sclerotherapy, which is effective in preventing chronic and recurrent gastrointestinal bleeding.

Adolescent↗
PubMed1988

Fiberoptic upper intestinal endoscopy in infants and children.

Fiberoptic upper intestinal endoscopy has been shown in the last decade to be the most sensitive technique to diagnose upper gastrointestinal disease. Diagnostic endoscopy has been shown to be safe, with a low complication rate of less than 2 per cent, and most of those reported have been minor. Furthermore, therapeutic endoscopy has been used to treat bleeding varices by sclerotherapy, avoiding the need for shunting procedures, which often fall in young children. Dilation of strictures with wire-guided dilators, endoscopic incision of antral mucosal diaphragms, and percutaneous placement of gastrostomy tubes are other ways endoscopy is being used as a means of treatment. Electrocoagulation and photocoagulation of bleeding upper gastrointestinal lesions have not been used.

Child↗
PubMed1988

Metabolic bone disease of total parenteral nutrition: course after changing from casein to amino acids in parenteral solutions with reduced aluminum content.

Bone disease with total parenteral nutrition (TPN) has been attributed to aluminum loading or vitamin D therapy. We studied 17 patients who first received TPN containing casein hydrolysate with high Al and ergocalciferol (25 micrograms/d) for 6-72 mo followed by TPN containing amino acids with reduced Al and ergocalciferol (5 micrograms/d) for 9-58 mo. We also did a cross-sectional study of 22 patients receiving casein and ergocalciferol (25 micrograms/d) compared with 46 patients receiving amino acids and ergocalciferol (5 micrograms/d) for 6-58 mo. Bone formation was higher and osteoid area, bone-surface stainable Al and total bone Al were lower with amino acid TPN than with casein TPN. Bone formation varied inversely with both plasma Al and bone-surface Al, suggesting that plasma or bone-surface Al, acquired during TPN, can reduce bone formation and lead to patchy osteomalacia. Serum levels of iPTH and 1,25-dihydroxyvitamin D were higher with amino acid TPN.

Aluminum↗
PubMed1987

SZ phenotype alpha-1-antitrypsin deficiency with paucity of the interlobular bile ducts.

A child is reported whose alpha-1-antitrypsin phenotype is SZ and who has chronic cholestatic liver disease that began in the neonatal period. Liver biopsy demonstrated paucity of the interlobular bile ducts, marked hepatocellular deposition in periportal areas of PAS-positive, diastase-resistant granules, and bridging portal fibrosis. The association of paucity of the interlobular bile ducts with SZ phenotype alpha-1-antitrypsin deficiency has not been reported previously.

Bile Ducts, Intrahepatic↗
PubMed1986

Home parenteral nutrition in the infant.

Home parenteral nutrition has become an important technique for the provision of nutritional support to infants in their home environment. It allows infants to exhibit long-term growth and development while the slow process of intestinal adaptation occurs. An important insight into the role of specific nutrients and their effect on body metabolism has been gained with the clinical use of total parenteral nutrition.

Ambulatory Care↗
PubMed1985

Chronic intestinal pseudo-obstruction. Management with total parenteral nutrition and a venting enterostomy.

A variety of remedies have been suggested for patients with chronic intestinal pseudo-obstruction. However, none of these treatments have been particularly effective, and many of these patients have died of malnutrition. Therefore, in an effort to maintain nutrition and avoid repetitive nasogastric intubation, 22 patients (12 adults and ten children) with chronic intestinal pseudo-obstruction were managed with long-term total parenteral nutrition (TPN), and 20 also received a "venting" enterostomy. Only two patients had to undergo revision of their gastrostomies. Prior to referral, these 22 patients required 56 admissions for obstruction (1.2 admissions per patient-year). Since the initiation of TPN and placement of a venting enterostomy, these 22 patients required only 17 hospitalizations for obstruction in a total of 80 patient years (0.2 admissions per patient-year). We conclude that patients with chronic intestinal pseudo-obstruction who receive TPN at home and have a venting enterostomy can be safely managed for prolonged periods and require fewer hospitalizations for obstruction.

Adolescent↗
PubMed1985

Improved survival in very short small bowel of infancy with use of long-term parenteral nutrition.

Thirteen children with very short small bowel (less than or equal to 38 cm jejunoileum) beginning in the first month of life were enrolled in a home parenteral nutrition program between 1977 and 1984. Their survival is compared with the collective reported experience with short bowel syndrome before 1972: nine (69%) of 13 have survived, compared with seven (23%) of 30 previously. Five discontinued parenteral nutrition after periods of 4 to 32 months of therapy, and have normal growth and development. Two still receive partial (50% and 60%) parenteral nutrition after 9 and 55 months, respectively, and two still receive total parenteral nutrition after 66 and 68 months of therapy, respectively; all four infants have grown normally, and three are developmentally normal. In the combined categories of 15 to 38 cm jejunoileum without the ileocecal valve and less than 15 cm jejunoileum with and without the ileocecal valve, seven (70%) of 10 have survived, compared with none (0%) of 16 before 1972; three of these discontinued parenteral nutrition. Ultimate survival with normal growth without parenteral nutrition is now possible with as little as 11 cm jejunoileum with an intact ileocecal valve and as little as 25 cm jejunoileum without an ileocecal valve.

Age Factors↗
PubMed1985

Upper GI function in sleeping infants.

In using the intraesophageal pH probe, considerable information has been gathered surrounding upper GI function during sleep in infants. However, there is considerable variability in the position of probe placement and the establishment of control values. Data as to whether the feedings are acidified, as well as position, has varied from study to study. Despite these shortcomings, one can conclude the following: asymptomatic episodes of gastroesophageal reflux occur throughout the 24-h period, but with considerably less frequency and duration during the sleep period. This is true for adults as well as for infant control subjects and the sleep reflux scores appear to be slightly higher in infants than in adults; intraesophageal pH monitoring is helpful in establishing a temporal relationship between pulmonary symptoms and episodes of gastroesophageal reflux, during sleep as well as awake periods. This relationship has been established in a very limited number of patients who have subsequently responded to both medical and surgical management; the mechanisms for gastroesophageal reflux in infants appear to be predominantly not related to a weak lower esophageal sphincter pressure but may occur as a complex result of "inappropriate" relaxation of the lower esophageal sphincter and/or increased intra-abdominal pressure overcoming the lower esophageal sphincter pressure barrier. Further investigation as to mechanisms responsible for physiologic as well as pathologic reflux need to be pursued; standardization of the intraesophageal pH monitoring test has to be achieved and efforts must be made to obtain the best control values possible while following ethical guidelines for clinical trials for pediatrics. This would include the standardization of the probe position, meals, scoring system, patient position, and intervals to be used in monitoring.

Adult↗
PubMed1984

Quantitative gastroesophageal reflux and pulmonary function in asthmatic children and normal adults receiving placebo, theophylline, and metaproterenol sulfate therapy.

There is evidence that theophylline therapy inhibits lower esophageal sphincter pressure and enhances gastroesophageal reflux in normal adults and adult asthmatics. No studies have used intraesophageal pH monitoring to assess the effect of bronchodilators over prolonged periods. With the use of the latter procedure, we studied 10 randomly selected chronic asthmatic children (mean age 10.6 yr) and 10 normal adult subjects; all subjects were without clinical symptoms of GER. In this single-blind study, all subjects were evaluated for GER during three separate 24-hr periods while they were receiving oral doses of placebo, theophylline, and metaproterenol sulfate therapy. No significant increase of GER was found in asthmatic subjects or normal subjects on either drug. No adverse correlation of GER and pulmonary function was noted in either group. Asthmatic subjects and normal subjects had asymptomatic or "silent" physiologic GER during sleep and in the 2-hr postprandial period. This study confirms that in chronic asthmatic children standard oral bronchodilator therapy does not adversely affect "silent" GER or pulmonary functions.

Adolescent↗
PubMed1984

Hepatic aluminum accumulation in children on total parenteral nutrition.

Five children receiving long-term total parenteral nutrition (TPN) containing casein hydrolysate as the protein source underwent percutaneous liver biopsies because of the development of cholestasis and abnormal liver function tests. All five demonstrated moderate to severe histopathologic changes. In addition, hepatic aluminum content was determined to be markedly elevated in all cases. Although the hepatotoxicity of aluminum is as yet undetermined, deposition of other metals has been associated with liver damage, and aluminum has been associated with pathology in other tissues. Thus, the possibility that aluminum deposition may play a role in the pathogenesis or exacerbate the course of liver dysfunction associated with TPN should be considered.

Aluminum↗
PubMed1984

Splenic cyst presenting as a gastric mass.

A 14-year-old girl with bulimia was found to have an apparent submucosal mass of the gastric cardia by upper gastrointestinal series and endoscopy. At laparotomy the mass was found to be a 5 cm in diameter subcapsular serous splenic cyst compressing the stomach but not attached to it. The clinical and diagnostic features of this unusual lesion are reviewed, with particular emphasis on the endoscopic findings.

Adolescent↗
PubMed1983

Increased risk of cholelithiasis with prolonged total parenteral nutrition.

Patients who receive hyperalimentation undergo prolonged periods of fasting which may alter bile composition and lead to gallbladder stasis, both important factors in gallstone formation. Therefore, we tested the hypothesis that patients who receive long-term TPN are at increased risk for cholelithiasis by performing cholecystosonography on adult patients who had received a minimum of 3 months of intravenous hyperalimentation during 1981. Seventy-one patients whose mean age was 41.9 years, 41 percent of whom were men, met these criteria. Gallstones had been diagnosed in 11 of the 71 patients (15 percent) before the initiation of parenteral nutrition. Twenty-one of the remaining 60 at risk patients (35 percent) were discovered to have cholelithiasis after hyperalimentation was started. The 45 percent prevalence of gallstones in our 71 patients was significantly higher (p less than 0.001) than predicted from autopsy data. In addition, the 49 percent prevalence of cholelithiasis in our 53 patients with ileal disorders was significantly greater (p less than 0.02) than predicted from a study of patients with similarly defined ileal disorders. This analysis strongly suggests that patients who receive long-term TPN are at increased risk for the development of cholelithiasis.

Adolescent↗