Albinism, cyclic neutropenia, and ceroid pigment in the liver
نویسندگان
چکیده
منابع مشابه
Cyclic neutropenia.
Cyclic neutropenia is a rare hematologic disorder, characterized by repetitive episodes of fever, mouth ulcers, and infections attributable to recurrent severe neutropenia. Fluctuations in blood cells are due to oscillatory production of cells by the bone marrow. Recent genetic, molecular, and cellular studies have shown that autosomal-dominant cyclic neutropenia and sporadic cases of this dise...
متن کاملCyclic neutropenia in mammals.
Cyclic neutropenia (CN) has been well documented in humans and the gray collie. A recent model of the architecture and dynamics of hematopoiesis has been used to provide insights into the mechanism of cycling of this disorder. It provides a link between the cycling period and the cells where the mutated ELA2 is expressed. Assuming that the biologic defect in CN is the same in dogs, and the obse...
متن کاملclinical and laboratory findings in iranian children with cyclic neutropenia
cyclic neutropenia is a rare immunodeficiency syndrome, characterized by regular periodic oscillations in the circulating neutrophil count from normal to neutropenic levels through 3 weeks period, and lasting for 3-6 days. in order to determine the clinical features of cyclic neutropenia, this study was performed. seven patients with cyclic neutropenia (3 males and 4 females), who experienced n...
متن کاملA comparison of the defective granulopoiesis in childhood cyclic neutropenia and in severe congenital neutropenia.
BACKGROUND AND OBJECTIVES Cyclic neutropenia (CyN) in childhood and severe congenital neutropenia (SCN) are congenital disorders that cause chronic neutropenia. Mutations in the neutrophil elastase gene, ELA2, have been reported in patients with CyN and in those with SCN. We examined granulopoietic defects in CyN patients with those in SCN patients. DESIGN AND METHODS Three patients with CyN ...
متن کاملCyclic neutropenia in common variable immunodeficiency.
Detailed studies of patients with so called cyclic neutropenia has revealed involvement of monocytes, lymphocytes, reticulocytes and platelets as well(2). Hence the term cyclic hematopoiesis is more appropriate. These parameters have not been mentioned in the. reported case. There is also no mention of whether the child had organomegaly. This is relevant because patients with cyclic neutropenia...
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ژورنال
عنوان ژورنال: Hepatology
سال: 2009
ISSN: 0270-9139
DOI: 10.1002/hep.23080