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Biomedical subjects

W E Berquist

Publications and source records attributed to W E Berquist.

At least 19 recordsLinked to original sources

An increased incidence of Epstein-Barr virus infection and lymphoproliferative disorder in young children on FK506 after liver transplantation.

The incidence of Epstein-Barr virus (EBV) infection and lymphoproliferative disorder (LPD) was determined in a pediatric liver transplant population consisting of 51 children treated with FK506 and 91 treated with cyclosporine. The incidence of symptomatic EBV infection was 21.9% (23 of 105 cases) in children < 5 yr old and 10.8% (4 of 37 cases) in children 5 to 17 yr old as compared with 2.7% (9 of 323 cases) in adults (P < 0.0001). In the under 5 yr old group on cyclosporine, the incidences of EBV infection and LPD were 9 of 68 (13.2%) and 2 of 68 children, (2.9%), respectively. In contrast, in children under 5 yr old group on FK506, the incidences of EBV infection and LPD in the FK506 group were 14 of 37 (37.8%) and 7 of 37 children (18.9%), respectively. The difference between these two groups was statistically significant (P < 0.02). There were no cases of LPD in the 5-17 yr-old children on either cyclosporine (n = 23) or FK506 (n = 14). The incidence of EBV infections in the 5 to 17 yr age group, 17.4% on cyclosporine and 0% on FK506, was less than for the younger children on FK506 (37.8%). A total of 39% (9 of 23) of children under 5 yr old who had symptomatic EBV infections developed LPD, and 44% (4 of 9) with LPD died. The higher incidence of EBV infections and LPD in the younger children treated with FK506 was probably related to a greater intensity of immunosuppression for patients on FK506 than those on cyclosporine.

Adolescent

Biofeedback therapy for anorectal disorders in children.

The physiological rationale, methodology, and results of biofeedback therapy in the management of anorectal disorders in children are presented. Balloon manometry and surface electromyography (EMG) are current biofeedback methods used to teach improved recognition of rectal distension and coordination of muscle function to either maintain fecal continence or to allow proper defecation. These techniques have permitted a success rate of over 70% in the management of children with functional or organic anorectal disease refractory to conventional medical and behavioral therapy. Biofeedback therapy is recommended to assist in the successful rehabilitation of these patients.

Anus Diseases

Graft involvement by Legionella in a liver transplant recipient.

Legionella pneumophila, serogroup 1, was identified by direct immunofluorescence in the lung and liver graft from a 2 1/2-month-old infant who underwent orthotopic liver transplantation because of fulminant hepatic failure secondary to neonatal hepatitis. The patient died of respiratory failure owing to this infection 22 days after transplantation despite treatment with erythromycin lactobionate. To our knowledge, this represents the first reported case of hepatic infection with Legionella in liver transplant recipients.

Fluorescent Antibody Technique

The impairment of true glomerular filtration rate in long-term cyclosporine-treated pediatric allograft recipients.

We performed indium-111-DTPA plasma clearance studies in 61 pediatric kidney and liver recipients treated with cyclosporine to compare true glomerular filtration rate with calculated GFR (cGFR). The mean true GFR of 61.9 +/- 36.6 ml/min/1.73 m2 indicated renal impairment. The mean cGFR of 85.2 +/- 22.4 ml/min/1.73 m2 was significantly higher (P less than 0.001), and overestimated GFR by 38%. cGFR alone did not accurately reflect the degree of renal dysfunction. A group of 48 pediatric orthotopic liver transplant recipients was studied in more detail: 73% of these patients had a true GFR less than 70 ml/min/1.73 m2, while 85% had a true GFR below 90 ml/min/1.73 m2, the lower limit for normal GFR in children. The mean true GFR for patients treated more than 24 months with CsA was lower (P = 0.02) than patients treated with CsA for 12 to 24 months. OLT patients with normal true GFR (greater than 90 ml/min/1.73 m2) had significantly lower plasma CsA levels, and 50% of patients with a true GFR less than or equal to 50 ml/min/1.73 m2 had hypertension. There was no effect on true GFR of age, liver function, azathioprine use, or peritransplant treatment with other nephrotoxic drugs. We conclude that true GFR is significantly impaired in long-term CsA-treated allograft pediatric recipients. Calculations of GFR underestimate the degree of renal dysfunction. As patients treated greater than 24 months had the lowest true GFRs, the fall in GFR may be progressive.

Adolescent

Home central venous hyperalimentation in fifteen patients with severe scleroderma bowel disease.

Fifteen patients with severe scleroderma bowel disease began receiving home central venous hyperalimentation (HCVH) between 1979 and 1987. The major reasons for instituting HCVH were intestinal pseudo-obstruction, malabsorption, and malnutrition. Eleven patients had an improved quality of life. Serious complications encountered over these 15,700 catheter-use days were 2 episodes of septicemia and 2 episodes of superior vena cava obstruction. Seven patients died, but none directly from their gastrointestinal disease or from the HCVH.

Adult

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

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

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

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

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

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