نتایج جستجو برای: familial histiocytosis
تعداد نتایج: 59536 فیلتر نتایج به سال:
histiocytosis of langerhans cells (class 1 histiocytosis) consists of a range of clinical manifestations, including bone eosinophilic granuloma, hand-schüller-christian syndrome, and letterer-siwe disease. these syndromes represent a spectrum of severity and prognosis of an underlying disorder which is usually sporadic. this report describes three cases in one family, who developed the disease ...
FHTG: familial hypertriglyceridemia HDL: high-density lipoprotein LCH: Langerhan’s cell histiocytosis LDL: low-density lipoprotein VLDL: very low-density lipoprotein XD: xanthoma disseminatum INTRODUCTION Xanthoma disseminatum (XD) is a rare subtype of non-Langerhan’s cell histiocytosis (non-LCH). To date, just greater than 100 cases of XD have been reported; however, association with hyperlipi...
Histiocytosis X is a rare disorder with no particular predilection for race, age or sex. Since its discovery by Hand in 1893, the etiology has remained unknown, although viruses, bacteria and genetic factors have been implicated. Familial occurrence of this disease is very rare, and only a handful of such cases have been reported. The present study adds further evidence to support the influence...
INTRODUCTION Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Syndrome, a nonmalignant variant of histiocytosis (1) is a chronic reactive disorder. It presents as a benign, chronic, massive enlargement of cervical lymph nodes (LN) accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate (ESR) and hyperglobulinamia (2). There is no malignant prolif...
Histiocytic syndromes in children are divided into 3 classes: (i) Langerhans-cell histiocytosis (LCH) or Histiocytosis-X (HX), (ii) Hemophagocytic syndromes—Familial erythrophagocytic lymphohistiocytosis (FEL) and infection-associated hemophagocytic syndrome (IAHS) due to viruses (VAHS), and occasionally bacterial, fungal or parasitic, and (iii) Malignant histiocytic disorders(l). The lesional ...
The histiocytoses are a heterogeneous group of disorders characterised by an excessive number of histiocytes. In most cases the pathophysiology is unclear and treatment is nonspecific. Faisalabad histiocytosis (FHC) (MIM 602782) has been classed as an autosomal recessively inherited form of histiocytosis with similarities to Rosai-Dorfman disease (RDD) (also known as sinus histiocytosis with ma...
The clinical and pathological findings are recorded in two siblings who died in early infancy from familial histiocytic (;haemophagocytic') reticulosis. The nature of this condition is obscure but probably represents a primary histiocytosis. The only other family on record is that described by Farquhar and Claireaux (1952) and by Farquhar, Macgregor, and Richmond (1958).
Hemophagocytic lymphohistiocytosis is character-ized by fever, hepatosplenomegaly, cytopenia, hyper-triglyceridemia, hypofibrinogenemia, and hemophago-cytosis. Ascites is not mentioned as a symptom of hemophagocytic syndrome. We report a one month-old girl suffering from familial erythrophagocytic lympho-histiocytosis, who presented with ascites.
Histiocytosis is a heterogenous group of disorders that are characterized by proliferation and activation of mononuclear phagocyte system. Langerhans Cell Histiocytosis (LCH) or Class I histiocytosis is a rare disorder of unknown etiology with proliferation of Langerhan cells which may infiltrate a single or multiple organs. This disease is more common in infants and children. It is usually spo...
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