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Biomedical subjects

E R Howard

Publications and source records attributed to E R Howard.

15 recordsLinked to original sources

The relative importance of familial, reproductive and environmental factors in biliary atresia: etiological implications and effect on patient survival.

1. The clinical records of 237 patients with extrahepatic biliary atresia (EHBA) attending King's College Hospital, London, between March 1973 and October 1985 were analyzed in terms of familial and reproductive factors, as well as of their possible effect on patient survival. 2. The male:female ratio was 0.93, and the ages of the patients' mothers and fathers were significantly higher than would have been expected from sibship data. Similarly, the number of first-born children having EHBA was less than expected. 3. In a subsample of 189 mothers, the obstetrical histories of women who had children with associated EHBA (20% of the total) showed: 1) a higher frequency of illness before and during pregnancy; 2) a higher level of pharmaceutical drug consumption during pregnancy, and 3) more fetal losses, especially in the gestation immediately preceding the patient's birth. 4. Age at death (average 15.1 +/- 13.2 months) and survival rates depend critically on surgical intervention and were not related to the presence or absence of extrahepatic malformations or to the type of atresia. 5. The present observations, taken together with those of others, indicate that problems in the reproductive process or exposure to noxious environmental agents may be etiological factors in associated EHBA.

Adolescent

Benign inflammatory tumour of the common bile duct.

A rare cause of obstructive jaundice in childhood is presented. Ultrasound and subsequent percutaneous cholangiography demonstrated the site of obstruction. Histological examination of the resected tumour showed features consistent with a diagnosis of benign inflammatory tumour of the common bile duct.

Adolescent

Surgery in children with homozygous sickle cell anaemia.

During the 25 years 1952--77 31 surgical procedures were performed on children aged up to 16 years with sickle cell anaemia (SCA). Six emergency operations were carried out, all for complications of SCA indistinguishable from the acute surgical conditions they mimicked. Seventeen minor operations were well tolerated and major surgery was undertaken 8 times, including 5 splenectomies for hypersplenism and increased transfusion requirements. The preparation for surgery by planned multiple transfusions and the indications for splenectomy are discussed. Recommendations are made for the preparation of patients for acute and routine surgery.

Adolescent

Malignant synovioma: electron microscopical findings in three patients and review of the literature.

Ultrastructural findings in three malignant synoviomas are described. Two typical "biphasic" tumours contained "epithelial" cells possessing filopodia or microvilli, specialised cell attachment and a basal lamina, and smaller "stromal" cells showing transitions to fibroblasts. In one case, the microvilli included fibrils resembling those in epithelial cells of the intestine and renal tubules. The third tumour was mainly spindle-celled with little epithelial differentiation and no clear division into cell types, but intracytoplasmic microfibrils were conspicuous, forming ovoid masses. The tumour cells differ a good deal from normal human synovial cells but some of the features of the neoplasm are found in inflamed human synovium and in normal synovial membranes of other species. The cytoplasmic fibrils in the third case are similar to those reported by others in epithelioid sarcoma, a tumour that may be of related origin to synovioma; however, the phenomenon may be merely degenerative.

Adolescent

Hepatobiliary imaging: a comparison of 99Tcm-dihydrothioctic acid and 99Tcm-pyridoxylidene glutamate in the non-jaundiced patient.

A comparison of two hepatobiliary imaging agents, 99Tcm-dihydrothioctic acid (99Tcm-DHT) and (99Tcm-pyridoxylidene glutamate (99Tcm-PG) has been carried out in 44 non-jaundiced patients. Thirty-one patients were admitted for investigation of upper abdominal pain and 13 patients were volunteers who were undergoing treatment for unrelated conditions. Satisfactory liver images were obtained with both agents in patients without liver disease, but they were inferior to those seen after 99Tcm-sulphur colloid. 99Tcm-PG produced clearer images of the gall-bladder and bile ducts than 99Tcm-DHT. Non-visualization of the gall-bladder was interpreted as gall-bladder disease; in patients with inflammatory gall-bladder disease no gall-bladder image was seen (nine 99Tcm-DHT, nine 99Tcm-PG). The gall-bladder was also not demonstrated in two of the volunteers' group (one 99Tcm-DHT, one 99Tcm-PG), nor was a gall-bladder seen in five patients whose abdominal pain was not due to acute cholecystitis. Despite this, there was agreement between the results of imaging and oral cholecystography in 21 out of 22 subjects. 99Tcm-PG is non-toxic, cheap and rapidly excreted by the liver into the bile. A 99Tcm-PG scan would be useful when rapid diagnosis is required in suspected acute cholecystitis when conventional contrast radiology is unlikely to be of value.

Adult

Hepatobiliary imaging: the use of 99Tcm-pyridoxylidene glutamate scanning in jaundiced adults and infants.

99Tcm-pyridoxylidene glutamate (99Tcm-PG) scans have been carried out in 24 jaundiced adults (mean total bilirubin 255 mumol/l and 11 infants with prolonged obstructive jaundice (mean total bilirubin 165 mumol/l). Absence of radioactivity in the gut was interpreted as complete biliary obstruction which was the cause of jaundice in ten adults and six infants. Using this criterion occlusion or patency of the bile ducts was correctly determined in 21 adults and eight infants. False-negative gut images were found in one adult and two infants, and three scans were inconclusive (two adults, one infant). The scan was unable to show details of the site of obstruction or pathology but the technique was simple and atraumatic and was safely performed in patients with serious complications, e.g. renal failure, coagulation defects, septicaemia. In infants the 99Tcm-PG scan compared well with the 131I rose bengal faecal excretion test and with liver biopsy in the investigation of prolonged obstructive jaundice. Repeat scans in infants with biliary atresia were used to assess postoperative bile drainage. It is suggested that 99Tcm-PG scanning is useful screening test in difficult cases of jaundice. It is especially useful in frail patients, and patients with complications.

Adult

Spontaneous perforation of common bile duct in infants.

Two infants with spontaneous perforation of the common bile duct are described. One presented with mild jaundice, dark urine, acholic stools, and hydroceles, the other with bilateral inguinal hernia. In both the diagnosis was unsuspected until bile-stained ascites was discovered. Both eventually developed bile-staining of the scrotum. Neither was acutely ill. The 131I-Rose Bengal faecal excretion test showed reduced faecal excretion at 8% and 12% of the injected dose with 16-5 and 17%/dl of the dose being recovered in the ascitic fluid 48 hours after intravenous injection. The ascitic:plasma ratio of isotope at that time was 32:1 and 28:1. Operative cholangiography in both showed a perforation at the junction of the cystic duct and common bile duct with no contrast entering the duodenum. Cholecystenterostomy using a Roux-en-Y loop of jejunum produced a rapid sustained recovery and is suggested as the treatment of choice. This condition should be considered in the differential diagnosis of obstructive jaundice in infancy since early surgical correction is necessary.

Ascitic Fluid

Liver and gall-bladder imaging with technetium-99m dihydorthioctic acid and technetium-99m pyridoxylidene glutamate-a study in the young pig.

Seventeen scans on ten young pigs were performed after injection of Technetium-99m labelled dihydrothioctic acid (99Tcm-DHT) and Technetium-99m labelled pyridoxylidene glutamate (99Tcm-PG) using a gamma-camera. Sequential images of the liver, gall-bladder and intestine were observed in all healthy animals. 99Tcm-DHT produced the clearer liver images, whereas 99Tcm-PG produced more rapid and intense gall-bladder images. Analysis of hepatic bile in three animals also suggested a more rapid and higher concentration of 99Tcm-PG than 99Tcm-DHT (36% vs. 4% of the injected dose over two hours). Bowel images were not seen in an animal that had undergone exicision of the extrahepatic biliary tract, but scans performed after hepaticojejunostomy demonstrated intestinal transit of isotope. This study shows that the hepatobiliary system can be easily imaged using Technetium-99m labelled compounds which are excreted into bile. These agents may be useful in the diagnosis of obstructive lesions of the extrahepatic biliary tract of adults and infants.

Animals

Unsaturated monohydroxy bile acids as a cause of idiopathic obstructive cholangiopathy.

It is proposed that some cases of biliary atresia and neonatal hepatitis may be initiated by an adverse effect of unsaturated monohydroxy bile acids on the fetal and infantile hepatobiliary system. The hypothesis is founded on morphological observations in patients with biliary atresia and on the toxic effects of monohydroxy bile acids on the hepatobiliary system.

Bile Acids and Salts