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Biomedical subjects

J R Lilly

Publications and source records attributed to J R Lilly.

At least 37 records · Page 2Linked to original sources
PubMed1990

Portal venous velocity in biliary atresia.

From December 1986 to April 1989, 38 patients with biliary atresia (eight newly diagnosed) were evaluated with doppler ultrasound of the portal venous system. Peak and mean velocities were computer derived from the spectral waveform. Good velocity was greater than 15 cm/s, intermediate velocity was 8 to 14 cm/s, abnormal velocity was less than 7 cm/s or hepatofugal. Patients were grouped according to clinical status: group 1 (n = 14), normal liver function; group 2 (n = 15), recurrent cholangitis; group 3 (n = 2), established bile drainage but complicated cirrhosis; group 4 (n = 7), failed portoenterostomy. All patients with normal liver function (group 1) had good or intermediate velocities. Thirteen of 15 patients with recurrent cholangitis (group 2) had good or intermediate velocities. Both patients in this group with abnormal velocities required transplantation. In group 3 the patient with abnormal velocity is on the transplant waiting list. In group 4, abnormal velocities preceded or coincided with deterioration of liver function in five of seven patients. Doppler ultrasound provides useful anatomic information, determines direction of flow, quantitates velocity of flow, and, when performed serially, provides adjunctive information on liver status in children with biliary atresia. These preliminary results suggest that patients with abnormal or significantly decreasing velocity are destined for transplantation. Patients with good portal venous velocity warrant ongoing, aggressive surgical management.

Adolescent↗
PubMed1990

Congenital biliary tract disease.

Jaundice in the pediatric patient requires prompt and directed evaluation. This dictum is highlighted in infants with biliary atresia, in whom the progressive sclerosing process results in complete obliteration of patent but microscopic hilar biliary structures by 4 months of age. Kasai's operation, if done before that time, will re-establish bile drainage in 90% of infants. One fourth to one third of patients achieve long-term jaundice-free survival. Complications of cholangitis, portal hypertension, and fat malabsorption are experienced by many patients. In children with early or late operative failure, liver replacement now offers legitimate hope for extended survival. Choledochal cyst is a conglomerate of pancreaticobiliary anomalies consisting of a choledochal cyst, a common-channel-type pancreaticobiliary junction, intrahepatic cystic disease, and partial obstruction of the distal common bile duct. Many patients have one or more of these malformations. It is now widely accepted that the preferred treatment of choledochal cyst is total excision of the diseased biliary duct with reconstruction by Roux-en-Y choledochojejunostomy. "Internal" excision avoids injury to other structures in the hepatoduodenal ligament, particularly if pericystic inflammation is present. Congenital perforation of the common bile duct responds in most cases to simple peritoneal drainage of the perforation. Retention of the tube cholecystostomy is useful for subsequent cholangiographic follow-up. Tube cholecystostomy may also be useful for irrigation of the biliary tract in infants with inspissated bile syndrome.

Biliary Atresia↗
PubMed1989

Biliary atresia: early determination of prognosis.

Analyses of bile bilirubin during the first month after Kasai operations, and of the liver biopsies obtained at the time of initial surgery, were done in 67 patients with biliary atresia. Bilirubin excretion (milligrams per day) was determined as the product of the bile volume and its concentration. Operative liver biopsies were evaluated for fibrosis, bile duct proliferation, bile stasis, giant cell transformation, and parenchymal degeneration; the severity of each abnormality was graded on a scale of 0 to 4. A forward stepwise regression procedure using the Cox proportional hazards model identified the relationship between survival and covariants. Thirty-nine of 67 patients died. Of these, 38 excreted less than 6 mg of bilirubin per day during the first postoperative month. The other patient died of a coexisting anomaly. Nine other patients who excreted less than 6 mg of bilirubin per day are alive but are either jaundiced or awaiting transplantation. Nineteen patients who excreted greater than or equal to 6 mg of bilirubin per day are alive (mean follow-up, 61 months) with normal or near-normal liver function. The severity of liver fibrosis, bile duct proliferation, and bile stasis did not correlate with survival, whereas giant cell transformation and parenchymal degeneration were highly significant (P less than or equal to .000 and P less than or equal to .0003, respectively). Twenty-six infants with grade 1-4 giant cell transformation or grade 2-4 parenchymal degeneration had a mean survival of 11 months.(ABSTRACT TRUNCATED AT 250 WORDS)

Bile↗
PubMed1989

Cholangitis after the Kasai operation for biliary atresia.

One hundred seventy-nine episodes of cholangitis in 28 consecutive patients having a Kasai operation for biliary atresia during the past 3 1/2 years were analyzed. The diagnosis was made primarily on the basis of unexplained fever (greater than 38.0 degrees C). An increase in serum bilirubin or a decrease in bile volume and in bile bilirubin concentration were often confirmatory, but other laboratory data including serum hepatic enzymes and blood and bile culture data were of little or inconsistent value. All patients were treated with systemic antibiotics. The best results were obtained with third-generation cephalosporins or imipenemcilastatin with the addition of aminoglycosides in recalcitrant cases. Antibiotic therapy was modified if defervescence did not occur within the first 24 hours. Cholangitis refractory to antibiotics was aggressively treated with pulse steroid therapy, and in some cases, operative intervention, both with good clinical success (60% and 73%, respectively).

Anti-Bacterial Agents↗
PubMed1989

Splenic embolization in children: long-term efficacy.

Eighteen partial splenic embolization procedures (PSEs) were performed in 17 children for hypersplenism (13) and/or esophageal variceal hemorrhage (12). The underlying disease was biliary atresia (BA) in nine children, portal vein thrombosis (PVT) in four, and biliary cirrhosis (BC) in four. From 20% to 90% of the spleen was embolized. Immediate morbidity was high, albeit minor, and the initial hospitalization was protracted for an average of 16 days. The children were followed from 4 to 81 months (average, 34.2). Four patients with BA patients subsequently had liver transplantation at an average of 20 months after PSE. In ten of 13 patients with hypersplenism, hematologic indexes returned to and remained normal throughout follow-up. The three exceptional patients (who had only 20%, 60% and 60% splenic embolization) developed recurrent mild hypersplenism, one of whom was reembolized and is free from hypersplenism 22 months later. Variceal hemorrhage was ameliorated in all 12 patients (average, 2.4 episodes of hemorrhage per year before PSE, 0.5 per year afterwards). Overwhelming postsplenectomy sepsis did not occur in an aggregate follow-up of 48.5 years. PSE is a legitimate treatment alternative for hypersplenism and for esophageal varices in children.

Adolescent↗
PubMed1989

The surgery of biliary atresia.

One hundred thirty-one consecutive infants with biliary atresia were operated on during the 15-year period between 1973 and 1988. Six patients did not have biliary reconstruction because of advanced cirrhosis or transplant preference. The other 125 infants had excision of all nonpatent extrahepatic bile ducts; biliary drainage was provided by a gallbladder-common bile duct conduit in 14 patients and by a Roux-en-Y portoenterostomy in 111 infants (including the seven patients with correctable biliary atresia). The bilioenteric conduit was temporarily exteriorized and, for the past 2 years, a conduit intussusception valve was incorporated. Immediate postsurgical bile drainage was achieved in 103 infants (82%). Reoperation during the first 6 postoperative weeks restored bile flow in 14 of 18 infants who had shut down. Seventy-two patients (57%) had sustained (more than 1 year) relief of biliary obstruction. Postoperative morbidity was substantial. The six children not having corrective surgery died within 19 months. Three patients were lost to follow-up. Sixty-eight patients having Kasai's operation died, 55 from complications of liver disease, 1 from a coexisting malformation, and 12 after liver transplantation. Fifty-seven patients are alive, 13 by virtue of liver replacement, 9 with mild-to-moderate hepatic sequelae, and 35 (28%) with normal to near-normal liver function. Although none is considered "cured," the 35 children are anicteric, have normal growth and development, and participate in full school activities (including contact sports). Average follow-up is 85.8 months (range 1 to 15 years).

Anastomosis, Roux-en-Y↗
PubMed1989

Biliary atresia.

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Biliary Atresia↗
PubMed1988

Endoscopic esophageal varix ligation: technique and preliminary results in children.

A technique for treating esophageal variceal hemorrhage in children using endoscopically placed rubber ligatures was evaluated in six children. The method offers a major advantage over chemical obliteration of varices by sclerosants in the absence of systemic, local, or distant organ reactions and, perhaps, in the avoidance of esophageal motor dysfunction.

Adolescent↗
PubMed1988

Manometric and pH consequences of esophageal endosclerosis in children.

Twenty-seven manometric and 22 18-hour pH monitoring studies were done in 17 consecutive patients undergoing esophageal endosclerosis. Prior to endosclerosis, esophageal manometry was normal in eight of nine patients. Peristaltic dysfunction was observed in all 13 postendosclerosis patients and consisted of (1) decreased mean peristaltic amplitude pressures. Pressures were 61.4 and 74.7 mmHg at 0 to 2 cm and 3 to 4 cm above the lower esophageal sphincter (LES), respectively, prior to treatment. After endosclerosis, values fell to 30.2 and 43.3 mmHg; (2) a drop in mean resting LES pressure from 22.3 mmHg before endosclerosis to 17.1 mmHg afterward and (3) an increase in the rate of peristaltic propagation failure from 12% to 26% after endosclerosis. Esophageal pH monitoring demonstrated gastroesophageal reflux (GER) in three of seven patients before endosclerosis and in five of 11 patients afterward. Because of the major incidence of GER before endosclerosis, the procedure could not be causally incriminated. However, GER was roughly correlated with the severity of the manometric dysfunction. There was no correlation of GER or manometric abnormality with the number of endosclerosis treatment nor the interval between endosclerosis and pH and manometric studies. The remote sequelae of the abnormalities are conjectural.

Child↗
PubMed1988

Liver transplantation and the Kasai operation in biliary atresia.

To assess the respective morbidity of the Kasai operation and liver transplantation in treating biliary atresia from the perspective of the referring institution, we analyzed the clinical course of 43 consecutive patients hospitalized at our institution from October 1980 to December 1985. Morbidity was arbitrarily defined as complications that required hospitalization, cholangitis, rejection, and was calculated by dividing the actual days hospitalized by the days "at risk." Morbidity assigned to transplantation also included pretransplant hospitalizations necessitated for life-sustaining treatment after referral. The morbidity in 40 patients who were treated by the Kasai procedure was 16.1%, which could be broken down to 50.6% in those who died after operation, 17.4% in those patients subsequently referred for transplantation, and 10.2% in the 16 patients successfully treated. Thirteen of the latter have perfect or near perfect liver function and are in excellent health. The morbidity of the 17 patients who were referred for liver transplantation, including two referred immediately after initial exploration, was 16.0%, which could be broken down to 10.4% before transplantation and 49.8% afterwards. Seven patients died awaiting transplantation, all but one of whom were referred during the first year of life. The average time spent on the waiting list was 203 days. Biliary atresia remains a disease of high morbidity regardless of treatment. Continued hospitalizations are necessary for ongoing Kasai management and life-sustaining treatment following transplant referral. The time spent on transplant waiting lists is protracted, and many patients die before obtaining a new liver.(ABSTRACT TRUNCATED AT 250 WORDS)

Biliary Atresia↗
PubMed1988

Lack of correlation between infection with reovirus 3 and extrahepatic biliary atresia or neonatal hepatitis.

Infection with reovirus 3 (Reo-3) has been suggested as the cause of extrahepatic biliary atresia and idiopathic neonatal hepatitis, but confirmation has been lacking. Therefore we have searched for a specific anti-Reo-3 antibody response in the sera of patients with biliary atresia or neonatal hepatitis and for Reo-3 antigens in their hepatobiliary tissues. Sera from 23 infants with extrahepatic biliary atresia, 12 with neonatal hepatitis, 30 age-matched control patients with other liver diseases, and 55 control patients without liver disease were tested by an enzyme-linked immunosorbent assay for total (IgA, IgG, and IgM) anti-Reo-3 antibodies; sera of infants younger than 6 months of age were tested also for IgM anti-Reo-3 antibodies alone. There was no difference between either total or IgM anti-Reo-3 antibody levels in infants with extrahepatic biliary atresia or neonatal hepatitis and levels in control infants. Reo-3 antigens were not detected in the hepatobiliary tissues of 19 infants (18 with biliary atresia, one with neonatal hepatitis) by an immunoperoxidase method that readily demonstrated Reo-3 in control infected HEp-G2 cells. Our data do not support a relationship between neonatal liver diseases and infection with Reo-3.

Antigens, Viral↗