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W F Balistreri

Publications and source records attributed to W F Balistreri.

At least 19 recordsLinked to original sources
PubMed1992

Scintigraphic documentation of an improvement in hepatobiliary excretory function after treatment with ursodeoxycholic acid in patients with cystic fibrosis and associated liver disease.

We have previously documented that ursodeoxycholic acid exerts a beneficial effect on liver function and bile acid metabolism in patients with cystic fibrosis. We hypothesized that the mechanism of action may be related in part to the choleretic properties of the administered bile acid. We therefore compared hepatobiliary scintigraphic images obtained before and 1 yr after initiation of ursodeoxycholic acid therapy to document an improvement in bile flow in 13 patients with cystic fibrosis and hepatobiliary involvement. Before therapy, hepatobiliary scintigraphy documented biliary stasis with retention of the isotope in intrahepatic and extrahepatic bile ducts in nine patients; during therapy, duct dilatation decreased substantially in eight patients, with decreased intrahepatic retention and more rapid biliary outflow of the tracer. The time of appearance of isotope in the intestine decreased (from a mean of 36.9 +/- 17.8 min to 18.8 +/- 9.0 min; p less than 0.01) in all patients in whom it had been abnormal, and the half-time of hepatic washout decreased from a mean of 35 +/- 20.7 min to 26 +/- 15.6 min (p less than 0.05). During ursodeoxycholic acid administration enrichment of bile was achieved, with the mean ursodeoxycholic acid percent composition increasing from 5.8% +/- 2.9% to 35.7% +/- 8.5%. Ursodeoxycholic acid became the predominant bile acid in serum. Liver function improved in all 10 of the patients with abnormal values at baseline. We conclude that hepatobiliary scintigraphy is of value in monitoring the therapeutic responses of cystic fibrosis patients with liver disease to ursodeoxycholic acid therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

Hepatobiliary scintigraphy in pediatric liver transplant recipients.

The clinical usefulness of hepatobiliary scintigraphy was evaluated in pediatric liver transplant recipients. One hundred fifteen hepatobiliary scintigraphic studies were performed in 30 patients who received 22 whole liver and 16 segmental grafts. Parameters that were useful in predicting an adverse outcome were failure to visualize excreted radiopharmaceutical at or beyond the biliary anastomosis on a study performed within 24 hours after transplant, and persistent or increasing delay in the time of visualization of excreted radiopharmaceutical. Abnormalities of liver uptake and excretion were seen in rejection, but they were also seen in patients who remained well without rejection or parenchymal disease. Significant biliary leaks were identified in the three cases in which they were known to be present. In liver transplant recipients, hepatobiliary imaging is useful in predicting graft survival and identifying biliary leaks.

Adolescent↗
PubMed1992

Monoethylglycinexylide formation in assessing pediatric donor liver function.

Lidocaine metabolism to monoethylglycinexylide (MEGX) has been described as a novel method to assess liver function in adult transplant donors and recipients. While this assay appears to offer a number of advantages over existing liver function tests, limited work has been done to evaluate its potential in the pediatric population. This study evaluated MEGX formation in potential pediatric liver donors (n = 35) and a control group of children (n = 16). The mean MEGX formation was significantly higher in pediatric donors than in the control group (156 +/- 62 vs 106 +/- 33 ng/ml, p < 0.05). No correlation with age, total bilirubin, liver transaminases, or alkaline phosphatase could be made within each group. Significant differences in MEGX levels were noted when each group was compared to its adult counterpart. Both pediatric donors and controls had greater mean MEGX formation than has been reported for adult donors and controls (156 +/- 62 vs 127 +/- 61 ng/ml, p < 0.05 and 106 +/- 33 vs 72 +/- 36 ng/ml, p < 0.05, respectively). Drugs that alter lidocaine pharmacokinetics and their potential influence on MEGX formation were evaluated in the pediatric donor group. Donors exposed to hepatic enzyme-inducing drugs had a higher mean MEGX formation (187 +/- 60 vs 146 +/- 63 ng/ml). No significant differences were noted between donors receiving and not receiving vasopressors. In conclusion, the significant differences between pediatric and adult MEGX formation should be noted when establishing reference or normal ranges for this diagnostic test. Furthermore, concomitant drug therapy may significantly alter MEGX formation.

Adult↗
PubMed1992

Liver transplantation in children.

Children with end-stage liver disease now have a greater chance of survival through treatment with hepatic transplantation. This article reviews the pediatric liver transplantation process, including selection and evaluation of candidates, operative procedures, postoperative complications, and long-term survival.

Biliary Atresia↗
PubMed1992

Rapid improvement in the renal tubular dysfunction associated with tyrosinemia following hepatic replacement.

The postoperative management of patients with hereditary tyrosinemia type I (McKusick 27670) following liver transplantation is often complicated by the renal tubular dysfunction associated with this disease. To characterize better the temporal course of the improvement in renal excretory activity following hepatic replacement, renal tubular function and metabolite excretion were studied in a 4-year-old girl with hereditary tyrosinemia during the immediate post-transplantation course. Tubular reabsorption of bicarbonate and phosphate were normal 5 days following transplantation, in contrast to glucosuria, hyperaminoaciduria, and tyrosyluria, which persisted for approximately 3 weeks. After hepatic replacement, serum amino acid concentrations returned to normal and succinylacetone was no longer detected in the urine. This is the third tyrosinemia patient reported to achieve complete resolution of urinary abnormalities following transplantation, and the only patient in whom renal tubular function was formally assessed within the first postoperative week.

Amino Acid Metabolism, Inborn Errors↗
PubMed1991

Selection criteria for liver transplant donors.

As the number of successful liver transplants has increased, the demand for donors has outpaced the supply. Approximately 25% of patients die awaiting an appropriate donor. Current criteria for assessing potential donors need to be closely examined. Fifty-six potential donors were evaluated by our transplant team by utilizing standard liver function tests (LFT's)-SGOT, SGPT, bilirubin. Additionally, a lidocaine metabolism test was performed by giving a 1 mg/kg IV dose of lidocaine over 1 minute and measuring the accumulation of the major metabolite monoethylglycinxylidide (MEGX) at 15 minutes by fluorescent polarization immunoassay (Abbott Diagnostics, Abbott Park, IL). Previous work has suggested that a MEGX less than 50 ng/mL is associated with initial non-function. Thirty-four donors were transplanted (group I) and all had initial function (all MEGX values were greater than 50). Twenty-two donors (39%) were judged unacceptable (group II) by our transplant team and by outside centers based upon one of the following criteria: II A) elevated LFT's--8, 11 B) donor age--5, II C) donor instability--4, II D) no available recipient--3, II E) miscellaneous--2. Standard LFT's were not statistically different in the donors used and in those not used when excluding category II A. Six of seven donors excluded in group I had acceptable MEGX values indicating they may have been transplantable. Ten of 12 patients excluded in groups II B-D had normal LFT's and nine of 12 had acceptable MEGX values indicating they may have been transplantable also. In this era of organ shortage, a reevaluation of donor selection criteria utilizing new tests like MEGX may be necessary to meet the increased need.

Adolescent↗
PubMed1991

Fetal and neonatal bile acid synthesis and metabolism--clinical implications.

It has become apparent that with sophisticated technology we are now able to recognize defective bile acid metabolism in a wide variety of disease states. Recognition of specific aberrations, such as inborn errors in bile acid metabolism manifesting as neonatal cholestasis, offers new opportunities for therapeutic intervention. Future studies should determine the incidence of inborn errors in patients with enigmatic and unexplained liver diseases such as idiopathic neonatal hepatitis.

Animals↗
PubMed1991

Segmental orthotopic hepatic transplantation as a means to improve patient survival and diminish waiting-list mortality.

Pediatric liver transplantation continues to be limited by the availability of suitable liver donors, a factor that restricts programmatic development and ultimately contributes to death on the recipient waiting list. We report the application of segmental liver transplantation as a technique to address both these problems as well as improving the outcome of the child undergoing the transplant procedure. Since 1986, 37 children have undergone orthotopic liver transplantation. Twenty-three children have received whole-organ grafts; 81% survived. Of those receiving whole-organ grafts, 15% had arterial thrombotic complications and 23% required retransplantation. More importantly, 29% of those children listed for transplantation died while waiting for a donor organ to become available, with a mean interval of 1.7 months (range, 2 days to 4.5 months). Since July 1988, segmental liver transplantation has been a component of our therapeutic armamentarium, and of the past 20 liver recipients, 16 have received a left lobe segmental graft. The results of the segmental transplant series have shown striking improvements. First, no child has died while awaiting donor organ availability. Second, segmental liver recipient survival is equivalent to that of whole-organ graft recipients (81%). Third, hepatic arterial thrombosis, especially a problem in high-risk infant transplants, was reduced by this technique (5%). Retransplantation due to graft complications has not increased (21%). These data suggest a vital role for segmental liver transplantation not only as a remedial salvage procedure for the critically ill child, but also as a primary transplant option.

Adolescent↗
PubMed1990

Oral rehydration in acute infantile diarrhea.

The significance of acute diarrheal illness, a common disorder in infants and children, is presented. Although in developed countries this disease is seldom fatal, it is the second most common reason that children are hospitalized. Methods for managing acute diarrhea and its subsequent dehydration, including intravenous rehydration and refeeding, are discussed, with emphasis placed on the use of oral rehydration solutions.

Acute Disease↗
PubMed1990

Assessment of lidocaine metabolite formation as a quantitative liver function test in children.

Lidocaine, an aminoethylamide, undergoes deethylation in the liver after intravenous injection, resulting in the formation of monoethylglycinexylidide. Serum monoethylglycinexylidide concentration can be measured by a simple, rapid fluorescent polarization immunoassay. We sought to determine whether lidocaine metabolism, as indicated by monoethylglycinexylidide formation, could be used as a quantitative index of hepatic function. Therefore we measured monoethylglycinexylidide formation in 10 healthy children and in 28 children with chronic liver disease. Patients with chronic liver disease were divided by disease severity as being at low, moderate or high risk for deterioration based on a clinical scoring system for liver transplant candidates. In healthy controls, the peak monoethylglycinexylidide concentration 15 min after injection of lidocaine (1 mg/kg) was 97 +/- 12 micrograms/L (mean +/- S.E.). However, monoethylglycinexylidide concentration was decreased in 28 children with chronic liver disease (32 +/- 5 micrograms/L, p less than 0.001). Furthermore, this value was inversely proportional to the severity of liver disease. The mean difference in monoethylglycinexylidide concentration between the low-risk and high-risk groups was statistically significant (42 +/- 6 vs. 11 +/- 4 micrograms/L, respectively, p less than 0.01). We conclude that the rate of formation of the lidocaine metabolite monoethylglycinexylidide is decreased in patients with chronic liver disease and is inversely related to disease severity. Therefore measurement of monoethylglycinexylidide concentration after lidocaine injection may be used as a quantitative assessment of liver function in children. We speculate that monoethylglycinexylidide formation may be a useful prognostic index in children with liver disease.

Adolescent↗
PubMed1990

Efficacy of cornstarch therapy in type III glycogen-storage disease.

Type III glycogen-storage disease (GSD-III), due to decreased activity of the glycogen debranching enzyme amylo-1,6 glucosidase, may cause hepatic dysfunction, growth failure, and myopathy. The prevention of hypoglycemia by nocturnal intragastric formula infusion has been shown to enhance growth and improve the metabolic abnormalities associated with GSD-III. Cornstarch therapy was effective in preventing hypoglycemia in a few patients with GSD-III who were previously treated with nocturnal enteral formula infusion, but oral cornstarch had not been evaluated as an initial treatment. We studied three patients with GSD-III who exhibited growth failure, elevated serum aminotransferase concentrations, and asymptomatic hypoglycemia. Cornstarch therapy was associated with maintenance of normoglycemia, increased growth velocity, and decreased serum aminotransferase concentrations in all patients. Our experience suggests that cornstarch therapy can be effective as an initial treatment for patients with GSD-III.

Blood Glucose↗
PubMed1990

Evaluation of children for liver transplantation: value of MR imaging and sonography.

Diagnostic imaging plays a major role in the diagnosis and treatment of chronic liver disease in children. Decisions regarding the need for sclerotherapy, palliative shunts, and anatomic suitability for hepatic transplantation are based on the upper abdominal anatomy. Thirty-six children with various forms of chronic liver disease were referred for diagnostic imaging, including MR and sonography. Each study was evaluated independently without knowledge of the other examination. The first six patients were evaluated retrospectively and the last 30 patients prospectively. The size of the portal vein and inferior vena cava varied in this population, with excellent agreement between sonography and MR. A 2-mm portal vein was shown only on MR imaging in two patients, but MR missed a 3-mm portal vein. Portosystemic collateral vessels also were evaluated and noted to be detected more readily with MR imaging (64%) than with sonography (22%). In particular, paraumbilical veins were detected more often with MR (28% vs 6% by sonography). Associated or coexisting anomalies were relatively common in children with chronic liver disease (14%). These included polysplenia (two cases), preduodenal portal vein (one), unilateral dysplastic kidneys (two), ureteropelvic junction obstruction (one), and splenic (one) and renal (one) cysts. MR imaging was more sensitive than sonography for detection of abnormal anatomy in this group of patients and should be considered the pretransplantation imaging technique of choice in children with end-stage liver disease.

Adolescent↗
PubMed1989

Vitamin E deficiency in primary biliary cirrhosis: gastrointestinal malabsorption, frequency and relationship to other lipid-soluble vitamins.

In contrast to deficiencies of vitamins A, D and K, little is known of the prevalence, clinical manifestations and mechanisms of vitamin E deficiency in adult patients with cholestasis. We measured serum vitamin E levels in 45 patients with primary biliary cirrhosis, 20 with primary sclerosing cholangitis, 9 with cryptogenic cirrhosis and 12 with alcoholic cirrhosis. To correct for the hyperlipidemia often found in patients with primary biliary cirrhosis and primary sclerosing cholangitis, total serum lipids were measured and vitamin E levels were expressed as the vitamin E/total serum lipid ratio. Serum vitamin A and D levels and prothrombin time were also determined. Six of 45 patients with primary biliary cirrhosis (13%) but none of the patients with sclerosing cholangitis, cryptogenic cirrhosis or alcoholic cirrhosis and subnormal vitamin E/total serum lipids ratios. Vitamin E deficiency was found in two of eight patients with asymptomatic primary biliary cirrhosis. There was no correlation between standard liver biochemical tests, fasting serum cholylglycine and vitamin E levels. Patients with primary biliary cirrhosis and primary sclerosing cholangitis had significantly lower vitamin E/total serum lipids ratios than patients with either cryptogenic or alcoholic cirrhosis. Twenty-three percent of patients with primary biliary cirrhosis were vitamin D deficient and 14% had low vitamin A levels. Two of the six patients with vitamin E deficiency were also deficient in vitamin D, only one was vitamin A deficient and none had prolonged prothrombin time. We also investigated the gastrointestinal absorption of vitamin E in nine patients with primary biliary cirrhosis and normal vitamin E levels as well as in six normal controls.(ABSTRACT TRUNCATED AT 250 WORDS)

Cholesterol↗
PubMed1989

Intestinal malabsorption of vitamin E in primary biliary cirrhosis.

We studied intestinal absorption of vitamin E in 26 adults with primary biliary cirrhosis (PBC) and 6 control subjects. Seven (27%) PBC patients were vitamin E-deficient based on the ratio of serum vitamin E to serum total lipid concentrations. An oral vitamin E tolerance test was performed in all patients and control subjects using a loading dose of 2000 IU alpha-tocopheryl acetate with measurement of serial serum vitamin E concentrations over 24 h. Vitamin E absorption was expressed as the maximal rise in serum vitamin E above baseline, the area under the oral tolerance test curve, and these two values divided by the fasting total serum lipid concentration. Absorption of vitamin E was significantly impaired in all PBC patients vs. control subjects (p less than 0.01), in vitamin E-deficient vs. vitamin E-sufficient PBC patients (p less than 0.05 to p less than 0.01), and in PBC patients with serum vitamin E levels below 10 micrograms/ml vs. those with serum vitamin E levels above 10 micrograms/ml (p less than 0.01). Vitamin E absorption was inversely related to stage of PBC, serum cholylglycine, total bilirubin, cholesterol, alkaline phosphatase, aspartate aminotransferase, and prothrombin time. Patients with serum vitamin E below 10 micrograms/ml, serum total bilirubin above 3 mg/dl, serum cholylglycine above 600 micrograms/dl, or serum alkaline phosphatase above 1000 IU/L had severe malabsorption of vitamin E and would be at high risk for the development of vitamin E deficiency. Therefore, vitamin E supplementation should be considered not only in patients in whom overt vitamin E deficiency is present, but also in PBC patients meeting these criteria.

Cholestasis↗