Autoimmune complications associated with hepatitis A virus infection in children.

Hepatitis A virus infection is usually a self-limited disease during childhood. Autoimmune manifestations are rarely reported among patients. We describe two children with acute hepatitis A infection who developed immune thrombocytopenia and hepatic venous thrombosis during the course of acute infection. Antiphospholipid antibodies were increased in both of them during the thrombocytopenic and thrombotic complications and decreased during the resolution of these events.

Blue rubber bleb nevus syndrome.

Blue rubber bleb nevus syndrome is a rare disorder characterized by distinctive cutaneous and gastrointestinal venous malformations that usually cause massive or occult gastrointestinal hemorrhage and iron deficiency anemia secondary to the bleeding episodes. It is even a rare cause of gastrointestinal hemorrhage during childhood. We describe a 6-year-old boy who had multiple venous malformations all over his body. He also suffered from several episodes of melena, chronic anemia, and growth retardation. The endoscopic examination of the gastrointestinal tract revealed multiple bluish-black sessile and polypoid venous malformations in various sizes. It was possible to remove the largest venous malformations causing massive bleeding during colonoscopy.

Congenital microvillus atrophy in a 4-month-old girl.

Congenital microvillus atrophy is a severe generalized enteropathy with ultrastructural abnormalities of the intestinal brush border. It is a rather new clinicopathological entity which needs to be differentiated from other enteropathies within the spectrum of intractable diarrhea of infancy. The presented case was a four-month-old girl with a chronic, intractable diarrhea, beginning at birth. The diagnosis was established only after the electron microscopic examination of small intestinal mucosa which revealed the characteristic features of the disease. Congenital microvillus atrophy is a rare autosomal recessively inherited disorder and bowel transplantation becomes a realistic option of treatment. Therefore, it should be specifically considered in the differential diagnosis of chronic intractable diarrhea of infancy.

Diagnosis of liver cirrhosis in children based on colour Doppler ultrasonography with histopathological correlation.

BACKGROUND: Changes in hepatic architecture in cirrhosis and chronic active hepatitis affect liver vascular haemodynamics. OBJECTIVE: To determine the criteria for the diagnosis of liver cirrhosis using Doppler US. MATERIALS AND METHODS: Twenty-two children with liver disease of unknown histology were prospectively examined and compared with eight normal children. Doppler US of portal vein velocity, arterio-portal velocity ratio, loss of reverse flow component in the hepatic vein and hepatic artery visualisation were examined prior to liver biopsy. Doppler results were compared with histological activity indices. Twelve patients had cirrhosis and ten had chronic active hepatitis. RESULTS: The most sensitive method (83%) for the assessment of cirrhosis was portal vein velocity less than 20 cm/sec. Arterio-portal velocity ratio (greater than 3) and hepatic artery visualisation were less sensitive (75% and 33% respectively) but specificity was 100% for all three methods. When these three methods were evaluated together, sensitivity increased to 91% and accuracy to 96%. Loss of reverse flow component was less specific (77%) but was sensitive (75%). CONCLUSIONS: Portal vein velocity, arterio-portal vein ratio and hepatic artery visualisation together were reliable in diagnosis of cirrhosis in the paediatric age group.

Superior mesenteric artery blood flow in children with celiac disease.

BACKGROUND: Knowledge of splanchinic hemodynamics in celiac disease is scarce. The hemodynamic parameters of the superior mesenteric artery were evaluated by duplex Doppler ultrasonography in children with celiac disease to show whether histomorphologic changes in small bowel mucosa led to any alteration in splanchinic blood flow. METHODS: The hemodynamic parameters of the superior mesenteric artery were evaluated by Doppler ultrasonography in 23 children with celiac disease. Ten patients were studied at the time of diagnosis. The remaining 13 children were studied after complete clinical and histologic recovery induced by gluten-free diet. Additionally, 9 patients out of 13 who were on a gluten-free diet for about 2 years were given gluten challenge, and superior mesenteric artery blood flow was measured after the challenge. The results were compared with those of healthy children. RESULTS: Peak systolic velocity of the superior mesenteric artery was higher in untreated celiac patients than in healthy controls and treated celiac patients. Peak systolic velocity of the superior mesenteric artery in the treated group of children was close to that of control subjects, implying that successful treatment with gluten-free diet improves hemodynamic changes. The comparison of Doppler ultrasonographic measurements of the challenge group before and after the gluten challenge revealed that the peak systolic velocity, resistive index, and blood flow of the superior mesenteric artery were changed significantly. CONCLUSIONS: The pathophysiologic events in small bowel mucosa during the active phase of celiac disease induce some hemodynamic changes that can be detected noninvasively by duplex Doppler ultrasonography.

Severe cytomegalovirus infection and immunosuppressive therapy in pediatric liver transplantation.

Twenty-two post-orthotropic liver transplant (OLT) recipients were studied to investigate the clinical, laboratory and histopathological differences between rejection and CMV infection. The mean age at the time of transplantation was five years. Nine of 22 (41%) patients developed positive CMV, CF-IgG and IgM antibody titers and cultures for CMV following surgery, and three (group 1a) developed interstitial pneumonitis. CMV specific inclusion bodies were found in lung and liver biopsies. Two patients in group 1a were treated successfully with DHPG and decreasing immunosuppressive treatment, while the third died. Clinical presentation of rejection episodes were similar in all groups. CMV infected patients (group 1) received more transfusions of blood and blood products than the non-infected patients (group 2). Rejection episodes occurred sooner and more frequently in group 1a than in group 1b (CMV infected-asymptomatic) and group 2 (non-infected). Group 2 received fewer steroid boluses as well as azathioprine and OKT 3. A percutaneous liver biopsy with routine stains helped detect CMV when inclusion bodies were seen. We conclude that culture proven CMV infection is common post-OLT. Severe CMV infection occurred more frequently in those who had received greater doses of immunosuppressive therapy for possible graft rejection. Monitoring CMV infection following OLT is absolutely necessary. After OLT, decreasing the immunosuppressives and using antiviral agents are important in the management of CMV infection.