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  • 1
    ISSN: 1432-1084
    Keywords: Key words: Child ; gastrointestinal tract ; Gallbladder ; wall thickening ; Liver ; US ; Histiocytosis ; Hepatitis ; Hemophagocytic lymphohistiocytosis ; Hemophagocytic syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Hemophagocytic lymphohistiocytosis, a variant of histiocytosis, is characterized by an uncontrolled activation of the cellular immune system, including hepatic mononuclear phagocytic cells. Abdominal ultrasound findings in children are evaluated in this disease. We present six pediatric cases, two with familial and four with sporadic hemophagocytic lymphohistiocytosis, examined by abdominal sonography. Three signs were frequently observed: thickening of the gallbladder wall (all cases), increased periportal echogenicity (four cases), and enlarged lymph nodes in the porta hepatis (four cases). Hepatomegaly, splenomegaly, and ascitic fluid may also be found. These imaging findings are not specific and may be seen in viral hepatitis. However, once hepatitis is excluded, they may suggest the diagnosis of hemophagocytic lymphohistiocytosis in a critically ill child. A bone smear must be done to establish the diagnosis.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Keywords Thrombosis ; Portal vein ; Stomatocytosis ; Splenectomy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Portal vein thrombosis is a rare but potentially lethal complication in children requiring splenectomy. We report on a 15-year-old boy with a dehydrated hereditary stomatocytosis, who underwent splenectomy and presented a postoperative partial portal vein thrombosis. With prompt heparin therapy, neither propagation of the thrombus nor further cavernous transformation in the following occurred 6 years. Conclusion Recent data suggest that hereditary stomatocytosis carries a high risk of thrombotic complications, especially after splenectomy. This procedure, the benefit of which is limited in this condition, should therefore be strongly avoided.
    Type of Medium: Electronic Resource
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