technetium-tc-99m-disofenin and Syndrome

Biliary atresia (BA) and neonatal hepatitis syndrome (NHS) are major causes of cholestatic jaundice in infancy. Technetium-99m diisopropyl iminodiacetic acid hepatobiliary scintigraphy (99mTc-DISIDA scan) is widely used in the differentiation of these two entities. The objective of this study was to evaluate the effect of phenobarbital premedication on the accuracy of 99mTc-DISIDA scan. Ninety-five cholestatic infants (38 females and 57 males) with an age range of 2 weeks to 4 months (mean 2.1 mo) who underwent 99mTc-DISIDA scan testing were retrospectively reviewed. The patients were divided into 3 groups according to the history of phenobarbital administration prior to 99mTc-DISIDA scan examination. Group 1 (n = 48), group 2 (n = 29), and group 3 (n = 18) received phenobarbital at the dosage of 5 mg/kg/day for at least 5 days, less than 5 mg/kg/day or less than 5 days, and no premedication, respectively. The accuracy of 99mTc-DISIDA scan in differentiating BA from NHS in group 1, 2, and 3 was 72.92 per cent, 89.66 per cent, and 100 per cent, respectively. No significant difference was seen between the patients who received and did not receive phenobarbital in terms of age at presentation, age at onset of jaundice, and liver function tests. In conclusion, phenobarbital therapy may not be necessary prior to 99mTc-DISIDA scan examination in the evaluation of cholestatic infants and thus a delay in diagnosis and surgical therapy of BA can be avoided.

Topics: Biliary Atresia; Excitatory Amino Acid Antagonists; Female; Hepatitis; Humans; Infant; Infant, Newborn; Male; Phenobarbital; Radionuclide Imaging; Radiopharmaceuticals; Reproducibility of Results; Syndrome; Technetium Tc 99m Disofenin

Byler's syndrome is a rare form of autosomal recessive intrahepatic cholestasis that is fatal in children. A 10-year-old girl diagnosed with Byler's syndrome underwent reduced-size liver transplantation using lateral segments of her living mother's liver. The donor's and the recipient's liver functions after transplantation were evaluated using Tc-99m disofenin and Tc-99m SC to investigate morphology, liver perfusion, and hepatobiliary function.

Topics: Child; Cholestasis, Intrahepatic; Female; Genes, Recessive; Hepatectomy; Humans; Imino Acids; Liver Transplantation; Organotechnetium Compounds; Radionuclide Imaging; Syndrome; Technetium Tc 99m Disofenin; Technetium Tc 99m Sulfur Colloid; Tissue Donors

Topics: Abnormalities, Multiple; Aged; Colon; Diaphragm; Humans; Imino Acids; Male; Organotechnetium Compounds; Radionuclide Imaging; Syndrome; Technetium Tc 99m Disofenin

Topics: Acute Disease; Adult; Cholecystitis; Cholestasis; Humans; Imino Acids; Male; Organotechnetium Compounds; Radionuclide Imaging; Syndrome; Technetium Tc 99m Disofenin

There are several methods for detection of bile in the stomach, but none has proven satisfactory. It appears that the scintigraphic study with quantitation of duodenal-gastric reflux after corrections for the overlap of the stomach by the liver and bowel is more reliable, noninvasive, and physiologic. Fifty-four patients were divided into groups according to their clinical presentation; seven asymptomatic volunteers, 20 patients with duodenal-gastric reflux gastropathy (DRG), 16 patients with recurrent ulcers of the duodenal bulb (RUD), and 11 patients with Moynihan's disease. Each of the 47 dyspeptic patients underwent an endoscopic examination and a scintigraphic study with [99mTc]disofenin for detection and quantitation of duodenal-gastric reflux. Endoscopy revealed the presence of bile in the stomach of 16 out of 20 DRG and four out of 16 RUD, while ten out of 11 patients with Moynihan's disease had clear gastric juice. Most of the DRG cases (15 out of 20) and half of the RUD (eight out of 16) presented reflux greater than 1.5%, while of the 11 Moynhihan, ten presented reflux less than 1.5% and all the asymptomatic volunteer subjects less than 1%. This quantitation method allowed us to perceive clearly the low % of reflux in the "normal asymptomatic" subjects compared with the DRG-type of dyspeptic patients. Among the dyspeptic, the distinction seems more evident between the DRG type and the Moynihan type. Occasionally, the scintigraphic method permits identification of patients with slower gallbladder evacuation (eight out of 47 dyspeptic in our study), adding valuable information for the diagnostic approach to dyspeptic patients.

Topics: Adult; Aged; Duodenal Ulcer; Duodenogastric Reflux; Dyspepsia; Female; Gastroscopy; Humans; Imino Acids; Male; Middle Aged; Organometallic Compounds; Radionuclide Imaging; Recurrence; Syndrome; Technetium Tc 99m Disofenin

Hepatobiliary scintigraphy has proven to be of great utility in distinguishing biliary atresia from other causes of neonatal cholestasis. Arteriohepatic dysplasia (Alagille's syndrome) is an uncommon entity characterized by typical facial features, pulmonary artery stenosis, and a liver disorder which presents during the neonatal period as progressive jaundice. Two neonates, who were later shown to have Alagille's syndrome, underwent hepatobiliary scintigraphy to rule out biliary atresia. Findings on the hepatobiliary scans from the two patients were similar to those usually associated with biliary atresia and both finally required surgical exploration to rule out biliary atresia. The findings on hepatobiliary scans in these patients with Alagille's syndrome are discussed and compared with those associated with other forms of neonatal cholestasis.

Topics: Bile Ducts, Intrahepatic; Biliary Atresia; Biliary Tract; Child; Cholestasis, Intrahepatic; Constriction, Pathologic; Diagnosis, Differential; Female; Humans; Imino Acids; Infant; Male; Organometallic Compounds; Organotechnetium Compounds; Pulmonary Artery; Radionuclide Imaging; Syndrome; Technetium Tc 99m Disofenin

Three patients in whom Caroli's disease was diagnosed by cholangiographic methods were studied by scintigraphy with Tc-99m DISIDA. Cholescintigrams showed an intense concentration of the radionuclide in the form of round spots near the hepatic hilus in late images. In two patients with cholestasis, a delayed hepatic clearance of the radionuclide was observed, which improved when the exploration was performed after a choledocoduodenostomy. Tc-99m DISIDA is not only a good noninvasive method to diagnose Caroli's disease, but also a useful technique to evaluate the patency of the biliary tree during the follow-up of such patients.

Topics: Adult; Bile Duct Diseases; Bile Ducts, Intrahepatic; Dilatation, Pathologic; Female; Humans; Imino Acids; Male; Middle Aged; Radionuclide Imaging; Syndrome; Technetium; Technetium Tc 99m Disofenin

Topics: Adult; Cholecystectomy; Cystic Duct; Humans; Imino Acids; Liver; Male; Postoperative Complications; Radionuclide Imaging; Syndrome; Technetium; Technetium Tc 99m Disofenin