Antibody testing in Indian children with celiac disease.

BACKGROUND: We prospectively evaluated the usefulness of IgA tissue transglutaminase antibodies (IgA tTG) in the initial diagnosis of celiac disease (CD) and compared its diagnostic potential with that of IgA anti-endomysial antibodies (IgA EMA) and anti-IgA and IgG gliadin antibodies (AGA and AGG, respectively). METHODS: Sera of 23 untreated children fulfilling the revised ESPGHAN criteria for diagnosis of CD (Group I; mean age 10.8 y); 19 disease controls (Group II; mean age 8.5 y) presenting with chronic diarrhea, short stature or both; and 22 healthy children (Group III; mean age 8.8 y) were studied. These were tested in a blinded manner for AGA, AGG, IgA tTG (guinea pig as antigen) and IgA EMA. RESULTS: In Group I, IgA EMA was positive in 19, IgA tTG in 17, AGA in 14 and AGG in 17 patients. In Group II, these tests were positive in 1, 0, 2 and 14 patients, respectively and in Group III, in 0, 0, 0 and 1 child, respectively. Analyzing data from Group I and II, IgA EMA, IgA tTG, AGA and AGG had sensitivity rates of 83%, 74%, 61% and 74%, respectively; the specificity rates were 95%, 100%, 89% and 26%; positive predictive values were 95%, 100%, 88% and 55% and negative predictive values were 82%, 74%, 65% and 45%, respectively. CONCLUSION: IgA tTG is useful for the diagnosis of CD, with sensitivity and specificity rates comparable to those of EMA and this test is well suited for use in tropical countries like India.

Endoscopic outcome beyond esophageal variceal eradication in children with extrahepatic portal venous obstruction.

OBJECTIVES: To find out the recurrence of esophageal varices, evolution of gastric varices, portal hypertensive gastropathy (PHG) and risk of rebleeding following esophageal variceal eradication. METHODS: Between 1992 and 2002, children with extrahepatic portal venous obstruction (EHPVO) and bleeding from esophageal varices received endoscopic injection sclerotherapy until eradication. Surveillance endoscopy was performed initially at 3 months and subsequently at intervals of 6 months to one year to detect esophageal and gastric varices, and PHG. Gastric varices were classified as gastroesophageal (GOV) or isolated gastric varices (IGV). Gastroesophageal varices included types GOV1 and GOV2 that extend along lesser and greater curvatures respectively. Patients who had recurrence of bleeding were evaluated by emergency upper gastrointestinal endoscopy. RESULTS: 163 of 183 children who achieved esophageal variceal eradication were evaluated. Esophageal varices recurred in 40% cases. Primary gastric varices (before sclerotherapy) were seen in 61% cases [GOV 98% (83% GOV1, 15% GOV2) and IGV 2%] and secondary (after sclerotherapy) in 28% [GOV 71% (47% GOV1, 24% GOV2) and IGV 29%]. Secondary gastric varices were distributed as 20% GOV1, 42% GOV2 and 87% IGV. Frequency of gastric varices before sclerotherapy and at the last follow up showed decrease in GOV1 from 82 to 56 (P = 0.02), increase in GOV2 from 15 to 23 and increase in IGV from 2 to 15 (P < 0.001). PHG increased in frequency from 12% to 41% (P < 0.001) and severity from one patient to 12 (P < 0.001). Eleven cases had rebleeding from gastric varices (5 GOV1, 4 GOV2 and 2 IGV). CONCLUSIONS: Following esophageal variceal eradication in children with EHPVO a significant decrease in GOV1, increase in IGV and increased frequency and severity of PHG takes place. Small rebleeding risk persists from gastric varices irrespective of the type.

Profile of liver involvement in dengue virus infection.

BACKGROUND: Data are scarce on liver involvement in adult patients with dengue virus infection. METHODS: During a recent outbreak of dengue fever in Uttar Pradesh, India, we looked for evidence of liver dysfunction among patients with dengue fever. RESULTS: A total of 45 patients with dengue fever (age 7-65 [median 33] years; 29 men; 39 adults) were studied, including 23 with uncomplicated dengue fever, 15 with dengue haemorrhagic fever and 7 with dengue shock syndrome. The median platelet count was 34 x 10(9)/L (9-99 x 10(9)). Seven patients (15%) had jaundice, 11 (24%) hepatomegaly and 9 clinically detectable ascites; none had splenomegaly. Twelve patients (30%) had hyperbilirubinaemia. Serum alanine and aspartate aminotransferase activities were elevated in 43 patients (96%) each; 5-fold elevated levels were more frequent in severe disease. Hypoalbuminaemia was found in 31/41 patients (76%). Seven patients died, including 2 with acute liver failure. CONCLUSION: Our data show that liver injury is common in adult patients with dengue infection. Further studies are needed to determine the mechanism of liver injury in this disease.

Superior sagittal sinus thrombosis and Budd-Chiari syndrome due to paroxysmal nocturnal hemoglobinuria managed with transjugular intrahepatic portosystemic shunt: a case report.

BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH), caused by somatic mutation of hematopoietic cells, is associated with complement-mediated hemolysis and a hypercoagulable state. Thrombotic complications in this disease are associated with reduced survival. We report a patient with PNH complicated by intracranial venous thrombosis and Budd-Chiari syndrome, who was managed with transjugular intrahepatic portosystemic shunt. CASE PRESENTATION: A 26-year-old man presented with thrombosis of the superior sagittal and right sigmoid sinuses. Initial investigations did not reveal any underlying cause. Nine months later, he developed hepatic venous thrombosis. At this time, Ham test was positive. Flow cytometry confirmed the diagnosis of PNH. The patient was treated with transjugular intrahepatic portosystemic shunt; one episode of stent blockage one month later was managed successfully with balloon dilatation and restenting. CONCLUSION: PNH should be considered in patients with unexplained venous thrombosis. Thrombosis in these patients needs to be managed with prolonged anticoagulation. For Budd-Chiari syndrome in patients with underlying PNH, transjugular intrahepatic portosystemic shunt may be a good option but caution is needed to prevent stent occlusion.

Dapsone induced cholangitis as a part of dapsone syndrome: a case report.

BACKGROUND: Dapsone can rarely cause a hypersensitivity reaction called dapsone syndrome, consisting of fever, hepatitis, exfoliative dermatitis, lymphadenopathy and hemolytic anemia. Dapsone syndrome is a manifestation of the DRESS (drug rash with eosinophilia and systemic symptoms) syndrome which is a serious condition that has been reported in association with various drugs. Cholangitis in dapsone syndrome has not been reported so far in the world literature. CASE PRESENTATION: We report a patient who presented with fever, exfoliative dermatitis, jaundice and anemia within three weeks of starting of dapsone therapy. These features are typical of dapsone syndrome, which is due to dapsone hypersensitivity and is potentially fatal. Unlike previous reports of hepatitic or cholestatic injury in dapsone syndrome we report here a case that had cholangitic liver injury. It responded to corticosteroids. CONCLUSION: We conclude that cholangitis, though unusual, can also form a part of dapsone syndrome. Physicians should be aware of this unusual picture of potentially fatal dapsone syndrome.