ISSN:0277-2116    

出版商:OVID    

年代:1986

数据来源: OVID

ISSN:0277-2116    

出版商:OVID    

年代:1986

数据来源: OVID

ISSN:0277-2116    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

SummaryIt appears that neonates, especially those with very low birthweights, may be at especially high risk of developing cholestasis associated with total parenteral nutrition (TPN). Within 2 weeks of starting intravenous alimentation, it would appear highly desirable to be able to start at least small enteral feedings to interrupt the physiology of fasting. Such feedings may not have to be of much nutritional benefit to improve cholestasis. Calorie and amino acid intake should be limited to the requirements of the infant being treated. Whether protection of the TPN infusate from light is of benefit remains to be determined. Drug therapies for TPN-associated cholestasis of infancy have not been proven safe or effective. Additional investigations to further clarify the pathogenesis of this syndrome, and clinical studies of prophylaxis and therapy, are needed to enhance our ability to provide nutritionally effective and metabolically safe parenteral nutrition.

ISSN:0277-2116    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

SummaryTo elucidate urinary bile acid patterns in patients with biliary atresia (BA), 15 sulfated and nonsulfated bile acids in urine were separately measured by high-performance liquid chromatography. This relatively simple technique for fluorescence detection utilizes the enzyme 3α-hydroxysteroid dehydrogenase (3α-HSD) to reveal urinary bile acid patterns. By this method, recovery rates of sulfated and nonsulfated bile acids in urine were satisfactory, and this analysis was shown to be applicable to clinical situations. In 10 patients with BA, the mean level of total bile acids in urine (23.35 ± 18.51 μmol/day) was seven times higher than the mean level in eight normal infants (3.05 ± 2.05 μmol/day). In the infants with BA, the mean level of total sulfated bile acids was about half of the total bile acid level. The main components of urinary nonsulfated bile acids in BA were glycocholic acid (6.21 ± 5.55 μmol/day) and taurocholic acid (2.28 ± 1.33 μmol/day), whereas the main components of the urinary sulfated bile acids were glycochenodeoxycholic acid (4.58 ± 6.97 μmol/day) and taurochenodeoxycholic acid (3.67 ± 3.54 μmol/day). Chenodeoxycholic acid, which is relatively toxic to the liver, may more easily be conjugated with sulfate and, hence, excreted into urine at a faster rate than cholic acid. Marked individual variations in urinary bile acid patterns were observed not only in BA patients but also in normal controls.

ISSN:0277-2116    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

SummaryTwenty-eight patients with neonatal cholestasis were given appropriate tests to arrive at a definitive diagnosis. Comparison of the results showing total obstruction on scintigraphy but not on liver biopsy is presented. It is suggested that liver biopsy be performed to confirm the diagnosis prior to undertaking exploratory laparotomy when radionuclear scan suggests extrahepatic biliary atresia.

ISSN:0277-2116    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

SummaryTwenty-four (3.6%) of 670 patients with cystic fibrosis seen over a 25-year period developed symptomatic gallbladder disease. Only four patients were less than 16 years old. Four patients presented with unusual problems, including one with acute cholangitis and two with atonic gallbladder, one of whom required cholecystectomy. Another patient was found to have cholangiocarcinoma of the gallbladder when an exploratory laparotomy was performed to investigate biliary obstruction. Twenty patients had cholelithiasis, 15 of whom underwent cholecystectomy. Only one patient had substantial pulmonary difficulties postoperatively. Patients who presented with classic biliary colic had no further symptoms after cholecystectomy. One patient developed intrahepatic stones 6 years later and required a choledochoduodenostomy. As the pulmonary status of most cystic fibrosis patients will eventually deteriorate, we recommend that serious consideration be given to performing a cholecystectomy as soon as practical after the diagnosis of symptomatic cholelithiasis. Our experience indicates that surgery can be performed safely unless pulmonary status is already extremely compromised and the patient is in overt respiratory failure.

ISSN:0277-2116    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

SummaryChildren treated with large doses of corticosteroids were found to develop hepatomegaly within a few days. No relationship could be established between the condition for which steroids were given and the liver enlargement. Liver biopsy was thought to be indicated, and thus was performed in three children because of diagnostic uncertainty. The light and electron microscopic examinations revealed normal liver architecture, without edema, sinusoid engorgement, or inflammatory changes. The hepatocytes were distended by increased amounts of glycogen. In a pattern reminiscent of some glycogen storage diseases, mitochondria and other cytosol components were displaced toward the cell membrane or around the nucleus, which occasionally contained glycogen. Moderate sinusoidal compression, interhepatocytic free glycogen particles, and mild increase in lipid droplets were also found. It is concluded that the hepatomegaly noted in patients given short-term, high-dosage steroid therapy is due to excessive glycogen accumulation within parenchymal cells. This finding is in accordance with observations in animals, as well as with biochemical studies demonstrating hepatocytic glycogen deposition after steroid therapy. Being benign and reversible, early hepatomegaly following administration of high-dose corticosteroids should not influence the initial therapeutic plan required by the basic disease.

ISSN:0277-2116    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

SummaryLower esophageal sphincter (LES) pressure, as well as esophageal peristaltic amplitude, duration, and velocity were measured in 16 infants with gastroesophageal reflux before administration of subcutaneous bethanechol, and at 10, 20, and 30 min after. Seven infants received 0.1 mg/kg, and 9 received 0.2 mg/kg. Significant increases in LES pressure occurred at both doses and lasted for 20 min. The amplitude and duration of peristaltic contractions were increased only after the larger bethanechol dose, and the increases were of greater magnitude in the distal esophagus than in the middle esophagus. The velocity of peristalsis decreased significantly in both the lower and middle esophagus, but only after the larger dose of bethanechol. Bethanechol had no effect on any motor function of the upper third of the esophagus. The changes in esophageal peristalsis produced by bethanechol may improve the efficiency of distal esophageal acid clearance and thus may be responsible in part for the therapeutic effect of bethanechol in infants with gastroesophageal reflux.

ISSN:0277-2116    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

SummaryIn order to compare the standard Nissen fundoplication with the more recent Angelchik antireflux prosthesis, 47 pediatric-sized, developmentally disabled patients were prospectively randomized to receive either of these gastroesophageal antireflux procedures. The two groups were comparable at the time of surgery with regard to age, weight, surface area, emesis, number of chest radiographs, medications, lower esophageal sphincter pressure, and number and duration of reflux episodes during standard acid reflux testing. The Angelchik group required significantly less anesthesia time than the Nissen group: 107.4 ± 22.6 min (mean ± SD) versus 156.5 ± 42.4 min (p < 0.001). There was no difference in length of postsurgical hospital stay. Six months after surgery, both groups showed improvement. There was no significant difference between the Angelchik and Nissen groups in mean subjective assessment score, increased weight gain, decreases in emesis, numbers of chest radiographs, hospital days, or medications, increased lower esophageal sphincter pressure, or decreased numbers and duration of reflux during acid reflux testing. Long-term follow-up of eight Angelchik and 10 Nissen patients 12–30 months postsurgery (Angelchik 21.6 ± 6.0 months, Nissen 21.9 ± 5.0 months) demonstrated no significant difference in the percent of time the distal esophageal pH was below 4.0. Significant complications potentially related to the type of procedure developed in 1/21 patients in the Angelchik group and 1/17 patients in the Nissen group. We conclude that both procedures are usually effective for the surgical treatment of gastroesophageal reflux. Further study is indicated in order to establish the long-term superiority of one procedure.

ISSN:0277-2116    

出版商:OVID    

年代:1986

数据来源: OVID