Mark Glassman

Diagnostic utility of hepatobiliary scintigraphy with 99mTc-DISIDA in neonatal cholestasis

We retrospectively evaluated the utility of hepatobiliary scintigraphy and various clinical factors in differentiating intrahepatic cholestasis from biliary atresia in 28 consecutive infants with neonatal cholestasis. One millicurie of technetium-labeled diisopropyliminodiacetic acid (DISIDA) was administered intravenously, and images were obtained for up to 24 hours or until gastrointestinal excretion was noted. Nine separate studies in seven infants with biliary atresia were correctly interpreted as showing no gastrointestinal excretion of radionuclide. Of the 21 patients with intrahepatic cholestasis, only nine had gastrointestinal excretion on the first study; in eight without excretion, a second study was done, and five of these showed gut excretion. All infants with either neonatal hepatitis (six) or inspissated bile syndrome (three) had demonstrable gastrointestinal excretion either on the first or second DISIDA study. However, five of six infants with paucity of intrahepatic bile ducts, two of six infants with cholestasis secondary to total parenteral nutrition, and one infant with cholangiolitis did not show evidence of gastrointestinal excretion. The mean birth weight, mean gestational age, and mean weight at study were significantly greater (P less than 0.005) for infants with biliary atresia without excretion than for infants with intrahepatic cholestasis without excretion. The mean direct bilirubin concentration was 6.0 mg/dL for both infants with biliary atresia and infants with intrahepatic cholestasis without excretion; however, infants with excretion had a significantly lower (P less than 0.02) mean direct bilirubin value of 3.4 mg/dL. Excretion was noted in four infants with total bilirubin values greater than 10.0 mg/dL. The absence of gut excretion on the first DISIDA study was 100% sensitive but only 43% specific for biliary atresia. In infants without gut excretion of DISIDA, birth weight greater than 2200 g was 100% sensitive and 92% specific for biliary atresia. We conclude that DISIDA scanning, together with clinical data, is useful in differentiating extrahepatic from intrahepatic cholestasis. The absence of gut excretion on the first DISIDA study does not necessarily indicate extrahepatic obstruction; the study should be repeated if the diagnosis is not clear.

Patterns of gastroesophageal reflux (GER) in patients with apparent life-threatening events.

The incidence and temporal patterns of gastroesophageal reflux (GER) in infants presenting with an apparent life-threatening event (ALTE) was compared with GER characteristics of infants evaluated for persistent emesis, utilizing continuous 24 h intraesophageal pH monitoring. These data indicate that the incidence of significant GER was similar in both groups, despite the absence of a clinical vomiting history in 46% of ALTE patients. Furthermore, infants with ALTE demonstrate a significantly higher incidence of steep reflux when compared with control infants presenting with vomiting alone (27 vs. 0%, p < 0.001). Awake GER beyond the first two postprandial hours was not observed in either study group. Monitoring results, therefore, indicate that significant GER is common in infants with ALTE; and these infants manifest an apparently unique pattern of GER distinct from that observed in age-matched controls with GER alone. Possible relationships between GER in ALTE patients and the development/onset of apneic episodes are discussed.

Immune-complex glomerulonephritis in Crohn's disease.

A 13-year-old girl presented with a history of fever, arthritis, conjunctivitis, abdominal pain, and diarrhea. Colonoscopy and barium enema were consistent with Crohns disease. A renal biopsy, performed because of persistent proteinuria and hematuria in the absence of obstruction to the urinary tract, revealed diffuse proliferative necrotizing glomerulonephritis: IgG, IgM, and C3 were deposited on the involved glomeruli. Circulating immune complexes were present in her serum. This immune-complex glomerulonephritis represents a previously unreported extraintestinal manifestation of inflammatory bowel disease.

Endoscopic removal of inflammatory esophagogastric polyps in children

Two adolescent patients with inflammatory esophagogastric polyps (IEPs) are presented. In each case, the patients complained of chest pain and dysphagia. In one patient, there was no histological evidence of esophagitis in association with the IEPs. Their clinical course suggests that the presentation of IEPs in adolescents is indistinguishable from and may result in secondary gastroesophageal reflux and esophagitis. In each case, endoscopic polypectomy was utilized effectively as the mode of therapy.

Esophageal chest pain in children with asthma.

Sixteen patients with asthma and chest pain of >2 months duration underwent gastroenterological evaluation utilizing fiber-optic esophagogastroduodenoscopy (EGD), esophageal manometry, and Bernstein testing. Eleven of 16 patients (75%) had endoscopic and histologic evidence of esophagitis. One patient with esophagitis exhibited high-amplitude peristaltic contractions during motility testing. Four of these 11 patients (36%) had a positive Bernstein test. Extended intraesophageal pH monitoring of seven patients with esophagitis revealed significant gastroesophageal reflux (GER) in all of these patients. Chest pain was associated with an episode of GER in three patients (43%). Nine of 11 patients (82%) with esophagitis responded to medical therapy, resulting in resolution of esophageal inflammation and chest pain. One patient required Nissen fundoplication surgery after failure of medical therapy, and one patient who refused surgery progressively developed more severe esophagitis during 9 months of medical therapy. Children with asthma may have chest pain due to gastroesophageal reflux-associated esophagitis that usually responds to medical therapy.

Coincidence of congenital toxoplasmosis and biliary atresia in an infant.

A 4-week-old infant presenting with neonatal cholestasis was found to have congenital toxoplasmosis and biliary atresia. This is the first patient in which their coincidence is reported. Because biliary atresia can coexist with either congenital infection or inborn errors of metabolism, evaluation for an obstructive etiology of jaundice in infants with a recognized cause of intrahepatic cholestasis is necessary.

Acute obstructive pancreatitis secondary to a duodenal hematoma

An 18-year-old male with hemophilia presented with symptoms and signs of upper intestinal obstruction. Evaluation was consistent with an intramural duodenal hematoma and obstructive pancreatitis. As it is not possible to distinguish between these two disorders on a clinical basis, it is important to realize that pancreatitis may occur in such patients more often than is recognized.

Coombs-positive hemolytic anemia preceding ulcerative proctitis

A 12-year-old girl is described who developed rectal bleeding 5 months after being diagnosed as having a Coombs-positive hemolytic anemia. Colonoscopy showed that the rectal bleeding was due to ulcerative proctitis. This is the first case report of Coombs-positive hemolytic anemia preceding the onset of ulcerative proctitis in a child.