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Biomedical subjects

J R Lilly

Publications and source records attributed to J R Lilly.

At least 55 records · Page 3Linked to original sources
PubMed1987

Treatment of vitamin E deficiency during chronic childhood cholestasis with oral d-alpha-tocopheryl polyethylene glycol-1000 succinate.

Treatment of vitamin E deficiency during chronic childhood cholestasis is hampered by the poor intestinal absorption of available oral preparations of vitamin E when bile flow is severely impaired; thus parenteral vitamin E has been the only effective therapy for many children with this problem. We studied the intestinal absorption, efficacy, and safety of a water-soluble oral form of vitamin E, d-alpha-tocopheryl polyethylene glycol 1000 succinate (TPGS), in 22 children (7 mo to 19 yr old) with severe cholestasis and vitamin E deficiency who were unresponsive to massive oral doses (100-200 IU/kg.day) of dl-alpha-tocopherol. The results of oral vitamin E tolerance tests showed that TPGS was well absorbed in virtually all study subjects, that TPGS intestinal absorption was superior to that of dl-alpha-tocopherol, and that TPGS absorption in teenage children with chronic cholestasis was similar to that of normal adults. In addition, 1.7% +/- 1.6% (mean +/- SD) of the administered polyethylene glycol 1000 contained in the TPGS was absorbed and excreted in the urine of the 13 subjects analyzed, compared with 3.0% +/- 1.3% in 4 normal adults. A chronic oral dose of 15-25 IU/kg.day of TPGS corrected the biochemical vitamin E deficiency state over 1-19 mo (mean, 10.6 mo) of TPGS therapy. No clinical or biochemical evidence of gastrointestinal, renal, hepatic, or hematologic toxicity was demonstrated. This study suggests that TPGS administered orally in a dose of 15-25 IU/kg.day may be a safe and effective form of vitamin E for prevention and correction of vitamin E deficiency during severe childhood cholestasis.

Administration, Oral↗
PubMed1987

Portal vein dynamics in biliary atresia.

A prospective sonographic investigation of portal vein caliber was done in 40 children with biliary atresia (n = 37) and biliary hypoplasia (n = 3) from 1 to 135 months old. The patients were divided into two groups according to hepatobiliary function (HBF). HBF was determined by serum bilirubin (greater than less than 1.5 mg/dL), serum albumin (greater than less than 3.5 g/dL), and bile bilirubin excretion (greater than less than 6 mg/d). There were 21 patients with "good" and 19 patients with "poor" HBF. The portal vein caliber, measured just proximal to its bifurcation, was 3 to 16 mm (median 7 mm), in the good HBF patients and 2 to 6 mm (median 4 mm) in the poor HBF group, the difference being statistically significant (P less than .01). Sequential studies of portal vein caliber were done in 30 patients. In the 15 patients with good HBF there was a progressive increase in portal vein caliber in nine, no change in six, and no patient had a decrease in caliber. Of the 15 patients with poor HBF, eight patients had a decrease in portal vein caliber, seven had no change, and no patient had an increase in caliber. The results indicate that patients with biliary atresia and poor HBF have an absolute and/or relative decrease in portal vein caliber and that the decrease may be progressive. Since portal vein anastomosis is essential to successful liver transplantation, referral must be done before portal vein shrinkage eliminates this option.

Age Factors↗
PubMed1987

The surgery of "correctable" biliary atresia.

Seven of 114 (6%) patients treated in Denver for biliary atresia had a variant of the disease in which only microscopic bile ducts or ductules were identified at the porta hepatis, hepatic lesions were present, even in the neonatal example, and intrahepatic biliary hypoplasia was uniformly present. The excised bile duct cysts consisted of fibrosis or scar tissue and with little or no epithelial lining. Six patients had corrective surgery. The first patient was treated by choledochoenterostomy and became totally obstructed. Definitive operation in this patient and all subsequent patients consisted of excision of all extrahepatic duct structures and Roux-en-Y portoenterostomy. Two patients died, the one who did not have surgical correction and a second who had correction at age 35 weeks. The other five patients have been followed for 18 to 158 months and are anicteric but have some degree of residual liver damage. We conclude that so-called correctable biliary atresia occurs in less than 10% of cases, that because of coincident biliary hypoplasia, "cure" is not a possible outcome, and that the treatment of choice is complete surgical excision of the extrahepatic bile ducts and Roux-en-Y portoenterostomy.

Bile Ducts↗
PubMed1987

Surgical implications of bronchopulmonary dysplasia.

Twenty of 30 patients with bronchopulmonary dysplasia (BPD) had major tracheobronchial abnormalities, which in 18 could be incriminated as contributing to their symptoms. There were 15 examples of tracheobronchial stenosis, 13 of tracheomalacia, nine of bronchomalacia, and one of tracheal web. Sixteen patients had operations. Tracheostomy was successful as a temporizing measure in ten patients although there was significant morbidity. Balloon dilatation of tracheobronchial stenosis produced temporary (2) or long-term (2) improvement in four patients. Electroresection of tracheobronchial stenosis was successful in the three instances it was employed. Lobectomy for lobar emphysema was curative in both patients. Aortopexy for tracheomalacia improved all three patients. We conclude that (1) symptomatic major airway lesions are not uncommon manifestations of BPD, (2) many of the lesions are amenable to surgical therapy, and (3) bronchoscopic evaluation should be considered early in the course of infants with BPD.

Bronchopulmonary Dysplasia↗
PubMed1986

Biliary atresia in the newborn.

A prenatal sonographic diagnosis of extrahepatic biliary atresia was made and, 76 hours after birth, operatively confirmed. A standard Kasai operation was performed, with the exception of the use of an ancillary appendiceal conduit to provide biliary drainage of an independent bile duct draining the right anterior hepatic segment. Hepatic and ductal histology were identical to those usually found in biliary atresia in a 6- to 8-week-old infant. The child is well at 16 months.

Biliary Atresia↗
PubMed1986

Surgery for total congenital tracheal stenosis.

A 4 1/2-month-old boy with complete tracheal rings throughout the entire trachea and agenesis of the right lung was successfully treated by operative insertion of a cartilagenous graft extending the length of his trachea. The commonly held viewpoint that this lesion is not surgically correctable and these infants will all die, can no longer be supported. Surgical repair should be strongly considered in all these children in the future.

Bronchoscopy↗
PubMed1986

Contemporary surgery of tracheomalacia.

During the past 7 years, 41 infants were treated for tracheomalacia. The etiology was primary/congenital in 28 patients and secondary/acquired in 16 patients, of which three patients were originally in the primary group. The primary group consisted of patients with pulsatile tracheomalacia but normal vascular anatomy, idiopathic disease, or tracheomalacia associated with tracheoesophageal fistula. The secondary group consisted of patients with tracheal compression due to great vessel abnormalities, or tracheostomy-tracheomalacia. Diagnosis was made by bronchoscopic demonstration of major (greater than 40%) collapse of the trachea. Treatment consisted of conservative therapy, tracheostomy, aortopexy, or tracheal reconstruction. The 15 patients with mild primary tracheomalacia treated conservatively had gradual resolution of symptoms by the age 2 years. All five patients with primary tracheomalacia treated by tracheostomy developed secondary tracheomalacia and/or cicatrix at the tracheostomy site. In the 9 patients with primary tracheomalacia treated by aortopexy, 5 are symptom free, 1 is improved, 1 had recurrent apnea, and 2 died, 1 from unrelated complications. Of the 10 patients in the acquired group treated by aortopexy, 6 were cured, 2 were improved, and 2 failed. Of 6 patients with tracheostomy-tracheomalacia, 3 were eventually extubated, 1 had major reconstruction, and 2 had tracheostomies when lost to follow-up at 1 and 5 years. Our conclusions are that, when feasible, conservative therapy in milder cases is preferred, and no perfect operation currently exists for severe tracheomalacia although aortopexy may have less long-term morbidity than tracheostomy.

Aorta, Thoracic↗
PubMed1985

Corticosteroid therapy in biliary atresia.

Sixteen patients with biliary atresia had 44 steroid courses for treatment of cholangitis or diminution of bile flow following Kasai hepatic portoenterostomy operations. A "blast" type (high dose/short duration) steroid administration was employed to potentiate the choleretic and anti-inflammatory effect. There was a significant augmentation of bile flow and a reduction in maximum temperature, serum bilirubin, and alkaline phosphatase.

Adrenal Cortex Hormones↗