One Case-report of Hand-Schuller-christian Syndrome in Children
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Abstract:
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- Schuller-Christian syndrome is the most Chronic and Variable form of the disease. This can be diagnosed by the following third: a: One or more destructive foci in the bones. b: Uni or bilateral exophthalmus. c: Diabetes insipidus with or without other symptoms of Pituitary deficiency. This form of disease is seen more in children but can occur in any age group. The reported Case is a 3- Year old boy who was Presented with following Clinical Signs and symptoms: Polyuria, polydipsia and low weight. A Skin biopsy revealed histiocytosis X (Hand - SchullerChristian) and drugs used are Vinblastine,6 MP, Prednisolone and D.D.A.V.P
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Hand--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 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 textLong-term Remission following Methotrexate Therapy in a Case of Hand-schuller-christian Disease.
The fatal outcome in the case described was unfortunate but was undoubtedly largely attributable to the patient's respiratory condition. The great difficulty which may be encountered in closing the duodenal stump in the presence of massive infiltration must, however, be stressed. We are indebted to Dr. A. A. Miller for valuable assistance with the pathological studies and for helpful advice. RE...
full textمعرفی یک بیمار مبتلا به هیستیوسیتوزیس ایکس از نوع Hand Schuller Christian disease
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 ...
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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volume 2 issue 6
pages 35- 39
publication date 1993-04
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