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Case Reports Indian Pediatrics 2001; 38: 1416-1418 |
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Portal Hypertension Due to Schistosomiasis |
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Joao Guilherme Bezerra Alves Fernanda Maria Ulisses Montenegro
Schistosomiasis is responsible for approximately 200 million human infections and 200,000 deaths every year(1). This infection is endemic in tropical and subtropical regions of Africa, Asia, the Caribean, and South America(2). Hepatosplenic schistoso-miasis is one of the most common cause of liver disease worlwide(3). Schistosoma mansoni affects more than 7.1 million people in Brazil and causes significant morbidity and mortality as a result of granulomata formation in the liver(4). The resulting hepatic fibrosis can lead to portal hypertension that eventually can be complicated by splenomegaly, esophageal varices, hematemesis, and death(5). Hepatic fibrosis resulting from chronic infections has been described usually in adolescent to late 20s, having harbored the parasites for 5 to 15 years. In the literature review (MEDLINE, LILACS) we did not find any report of periportal fibrosis with portal hypertension due to schistosomiasis in children under 7 years. We report a case of S. mansoni hepatic fibrosis with portal hypertension in a child aged 2 years and 10 months. Case Report A 2 years and 10 months old girl, living in Jundiá-Alagoas, northeast of Brazil, was admitted with complaints of increased distention of abdomen for about 6 months. She had received blood transfusions four months ago for severe anemia. There was no history of hematemesis, umbilical infection or catheter insertion in the umbilical vein. There was no neonatal dehydration or systemic infection. There was history of river bath since 3 months of age, one to three times per week. Examination revealed an afebrile patient with moderate pallor. Anthropometric assessment confirmed that she was stunted and malnourished. There was no ascites, jaundice and no stigmata of cirrhosis such as palmar erythema and vascular telangiectasias. She had an enlarged, hard liver and splenomegaly. Investigations revealed a hemoglobin of 6.5 g/dl, WBC count of 15,000/mm3, eosinophil counts of 29%, platelet counts of 199,500/mm3. Liver enzymes, alkaline phosphatase and bilirubin were normal. Prothrombin time was 15 seconds with 62% of enzymatic activity. Albumin was 1.66 g/dl and gammaglobulin 3.7 g/dl. HbsAg was negative. Stoool analysis showed S. mansoni and Ascaris eggs ans strongyloides larvae. Abdominal ultrasound showed multiple echogenic areas, each one with central sonolucency in the liver, which represents periportal fibrosis around portal vein tributaries (Fig 1). Endoscopy detected esophageal varices. Percutaneous liver biopsy specimens revealed "Symmers pipe stem fibrosis". She was treated with oxaminiquine and was discharged with an improved nutritional status. Discussion In our literature review we did not find any report of hepatic fibrosis due to schistosomiasis in this age. The youngest child to be reported previously as having portal hypertension due to Symmers’ schistosomiasis fibrosis was a 7 year old child(6). It is believed that hepatosplenic schistosomiasis results from accumulated injury over time, thus, Symmers’ fibrosis requires prolonged, moderately intense infection with schistosomes. Patients with hepatosplenic schistosomiasis are usually adolescents to late 20s, having harbored the parasites for 5 to 15 years(3).
Fig. 1. Hepatic sonogram showing increased echogenicity of periportal spaces. It is defficult, based on actual understanding of the physiopathology to explain the findings in our case. The intensity of exposure, differences in parasite strains, nutritional status (including micronutrient deficiencies), and the presence of other infections, all may contribute to variations in the severity of infection and disease morbidity. Our patient had severe malnutrition and intesti-nal infection by Ascaris and Strongyloides. Host genetic differences, not studied in our case, such as polymorphisms in the interferon gamma (INF-gamma) receptor gene, may play a major role in determining the risk of severe hepatic fibrosis(7,8). Hematemesis due to portal hypertension is the main cause of death in schistosomiasis. Even though the morbidity and mortality of this parasite in the first decade of life is low, in areas where schistosmiasis is endemic, children may become infected in its first months of life and present with hepatic fibrosis at a younger age. It seems that portal hypertension due to schistosomiasis in children may present earlier than 7 years of age. Contributors: JGBA carried out the clinical workup and also drafted the manuscript. He will act as the guarantor for the paper. FMUM was responsible for the clinical workup, and co-drafting. Funding: None. Competing interests: None declared.
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