معرفی یک بیمار مبتلا به هیستیوسیتوزیس ایکس از نوع Hand Schuller Christian disease
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Abstract:
The term histiocytosis refers to a group of idiopathic clinics entities characterized by diffuse proliferation of mature histiocytes associated with tumorlike masses of foamy reticuloendothelial cells containing lipoid droplets with variable number of eosinophiles and connective tissue. This cells causes tumorlike masses in the bones and other vicera in the body thus it may have very different clinical signs and symptoms. Also it may have acute and fulminant form or chronic and slowly progressive. We have an interesting case with multiple organ involvement and chronic otitis media with granulation in the external audiotory canal and perforation of tympanic membrane. The patient was 3 years old with diabetes insipidus and visceral involvement such as hepato-splenomegaly. Histologic diagnosis was Hand Schuller Christian disease.
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معرفی یک بیمار مبتلا به هیستیوسیتوزیس ایکس از نوع hand schuller christian disease
هیستیوسیتوزیس ایکس به گروهی از حالت های بالینی اطلاق می شود که با پرولیفراسیون منتشر سلول های رسیده هیستیوسیت همراه با سلول های رتیکولواندوتلیال foamy مانند که حاوی قطرات چربی هستند و تعداد متغیری از ائوزینوفیل و بافت همبند مشخص می گردد. این سلول ها باعث توده های تور مانند در استخوان ها و ارگان های مختلف بدن می شوند و نتیجتا علایم کلینیکی مختلفی ایجاد می کنند. ممکن است بصورت حاد و fulminant یا ...
full textHand--schuller--christian Disease.
ENDOCRINOLOGICAL, METABOLIC DISORDERS hypophysis dysfunction diabetes insipidus GENITAL DISORDERS genital dysfunctions puberty, delayed HEMOPOIETIC-LYMPHORETICULAR SYSTEM DISORDERS lymphonodus disorders lymphonodus enlarged, lymphadenopathy spleen, anomalies splenomegaly, hepatosplenomegaly LABORATORY DATA lymphoreticular system, changes histiocytosis, including histiocytoma myelo-erythropoieti...
full textOne Case-report of Hand-Schuller-christian Syndrome in Children
SUMMARY Histiocytosis X is associated with idiopathic Proliferation of histiocytes and infiltration of eosinophils in bone marrow Spleen, Liver and other Soft tissues. Clinically there are three distinct forms: 1 - Eosinophilic granuloma is the most benign and Localized form of the disease. 2- Letterer- Siwe is the most malignant, generalized and fatal form of the disease. 3- Hand- Schul...
full textHand schuller christian disease: a rare case report with oral manifestation.
Langerhan's Cell Histiocytosis (LCH) is disorders which include abnormalities that result from abnormal proliferation of langerhan's cells or their precursors. LCH is clinically classified into three types-eosinophilic granuloma, Hand Schuller Christian disease and Abt-Letterer- Siwe disease. It is usually manifested in childhood as well as in adulthood. The clinical manifestations are the resu...
full textDexmedetomidine: A Saviour in a Child with Hand Schuller Christian Disease for MRI
Hand Schuller Christian disease (HSC) primarily affects infants and children. Its classical presentation includes triad of exophthalmos, diabetes insipid us, and calvariallytic lesions. Systemic involvement may include hepatomegaly, lymphadenopathy, dermatological, gastrointestinal tract, renal, pulmonary and CNS involvement. CNS involvement includes convulsions, increased intracranial pressure...
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Journal title
volume 58 issue None
pages 1- 4
publication date 2000-07
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