Juvenile Xanthogranuloma

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52 relevant articles (1 outcomes, 0 trials/studies) found for this Disease

Description: Benign disorder of infants and children caused by proliferation of HISTIOCYTES, macrophages found in tissues. These histiocytes, usually lipid-laden non-Langerhans cells, form multiple yellow-red nodules most often in the skin, the eye, and sometimes in the viscera. Patients appear to have normal lipid metabolism and are classified as a normolipemic non-Langerhans cell histiocytosis.

Also Known As:

Xanthogranuloma, Juvenile; Juvenile Xanthogranulomas; Juvenile Xanthoma; Juvenile Xanthomas; Nevoxanthoendotheliomas; Xanthogranulomas, Juvenile; Xanthomas, Juvenile; Nevoxanthoendothelioma; Xanthoma, Juvenile

Relationship Network

Disease Context: Research Results

Hemic and Lymphatic Diseases

Lymphatic Diseases: 1961

Histiocytosis: 228

Non-Langerhans-Cell Histiocytosis: 20

Juvenile Xanthogranuloma: 52

Skin and Connective Tissue Diseases

Skin Diseases: 6187

Juvenile Xanthogranuloma: 52

Related Diseases

1.	Langerhans-Cell Histiocytosis (Histiocytosis, Langerhans Cell)
2.	Neoplasms (Cancer)
3.	Cataract (Cataracts)
4.	Xanthomatosis (Xanthoma)
5.	Granuloma

Experts

1.	Manquez, Maria E: 1 article (03/2008)
2.	Shields, Jerry A: 1 article (03/2008)
3.	Danzig, Carol J: 1 article (03/2008)
4.	Shields, Carol L: 1 article (03/2008)
5.	Ehya, Hormoz: 1 article (03/2008)
6.	Mashayekhi, Arman: 1 article (03/2008)
7.	Goldstein, Nira A: 1 article (02/2007)
8.	Alexis, Richard: 1 article (02/2007)
9.	Somorai, Marta: 1 article (02/2007)
10.	Giusti, Robert J: 1 article (02/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Juvenile Xanthogranuloma:

1.	SteroidsIBA 01/01/1993 - "Early treatment of juvenile xanthogranuloma of the iris with subconjunctival steroids." 05/01/1990 - "Most reported cases of successfully treated uveal juvenile xanthogranuloma have received systemic steroids, irradiation or excision. " 11/01/1966 - "Juvenile xanthogranuloma treated with local steroids." 07/01/1994 - "Since even a massive juvenile xanthogranuloma of the iris responds well to steroids, operative and radiation treatment should be limited to cases refractory to antiinflammatory drug therapy." 10/01/2009 - "Juvenile xanthogranuloma (JXG) of the uvea is a rare disease that usually responds to systemic steroids or low-dose radiotherapy. " Order ALL the reference details at left...
2.	S100 Proteins (S 100 Protein)IBA 07/01/2008 - "The diagnosis of systemic juvenile xanthogranuloma was confirmed by histology and immunohistochemical stains of the skin with liver biopsies revealing dense infiltration of lymphohistiocytes with typical Touton giant cells staining positive for CD68 and negative for CD1a and S-100 protein. " 01/01/2001 - "Expression of S-100 protein in histiocytes is unusual finding, but has been reported recently in juvenile xanthogranulomas (JXG). " 11/01/1985 - "Immunohistochemically, most lesions of juvenile xanthogranuloma displayed a positive reaction for lysozyme and alpha-1-antichymotrypsin and were negative for S-100 protein, thereby suggesting that the essential constituents of this lesion would derive from the mononuclear phagocyte system." 11/01/1990 - "Histiocytes of juvenile xanthogranuloma, necrobiotic xanthogranuloma, and traumatic granuloma were strongly positive for Mac-387 but were negative for S-100 protein and peanut agglutinin, except for the peanut agglutinin-reactive Touton giant cells. " 07/01/1983 - "The cellular localization of glial S-100 protein was investigated in paraffin-embedded sections of cutaneous histiocytosis X and in a variety of cutaneous infiltrative disorders, including juvenile xanthogranuloma, necrobiotic xanthogranuloma, papular xanthoma, eruptive histiocytoma, and reticulohistiocytosis. " Order ALL the reference details at left...
3.	Factor XIIIaIBA 11/01/2004 - "The diagnosis of juvenile xanthogranuloma was made by pathologic findings of marrow and portal tract infiltration by S-100 negative, CD1a negative, CD68 positive, and Factor XIIIa positive large pale to foamy histiocytes with Touton giant cells, and lack of Langerhans cell granule by electron microscopic examination. " 01/01/1994 - "Factor XIIIa expression in juvenile xanthogranuloma." 03/01/1995 - "The antibodies against factor XIIIa (shown on xanthoma disseminatum) and the monoclonal antibody Ki-M1P (shown on juvenile xanthogranuloma) seem to be valuable in discrimination between LCH and non-LCH. " 06/01/2000 - "To explore the use of immunohistochemistry for this diagnostic problem, we examined the expression of S-100 protein, gp100 (the antigen recognized by HMB-45), tyrosinase (T311), Melan-A (A103), Factor XIIIa (FXIIIa), and CD68 in 10 juvenile xanthogranulomas (JXGs), five epithelioid histiocytomas (EHs), and 15 melanocytic nevi composed of large epithelioid cells. " Order ALL the reference details at left...
4.	AntigensIBA 03/01/2001 - "Juvenile xanthogranuloma: case report with immunohistochemical identification of early and late cytomegalovirus antigens." 12/01/1992 - "For example, the M phi s in cat-scratch fever are nearly unique in the expression of the "early inflammation" antigen identified by antibody 27E10, and the M phi s in juvenile xanthogranulomas, unlike those in most of the other conditions, lacked the antigen detected by antibody 25F9. " 12/01/1992 - "Using a large panel of monoclonal antibodies to monocyte/M phi-related antigens and a frozen-section immunoperoxidase technique, the following conditions were studied: granulomatous inflammation of unknown etiology, sarcoidosis, cat-scratch fever, toxoplasmosis, Gaucher's disease, and juvenile xanthogranulomas. " 06/01/2000 - "To explore the use of immunohistochemistry for this diagnostic problem, we examined the expression of S-100 protein, gp100 (the antigen recognized by HMB-45), tyrosinase (T311), Melan-A (A103), Factor XIIIa (FXIIIa), and CD68 in 10 juvenile xanthogranulomas (JXGs), five epithelioid histiocytomas (EHs), and 15 melanocytic nevi composed of large epithelioid cells. " 12/01/1987 - "Immunohistochemical examinations were performed using five kinds of histiocytic markers [S100 protein, lysozyme, non-specific cross reacting antigen with carcinoembryonic antigen (NCA), alpha 1-antichymotrypsin (alpha 1-ACT) and alpha 1-antitrypsin (alpha 1-AT)] in biopsied tissues from histiocytosis X, juvenile xanthogranuloma, xanthoma tuberosum, xanthoma disseminatum, reticulohistiocytic granuloma and multicentric reticulohistiocytoma, all of which have been classified as histiocytic proliferative disorders. " Order ALL the reference details at left...
5.	Muramidase (Lysozyme)IBA 02/01/1981 - "The cells of juvenile xanthogranuloma were rich in lysozyme. " 11/01/1985 - "Immunohistochemically, most lesions of juvenile xanthogranuloma displayed a positive reaction for lysozyme and alpha-1-antichymotrypsin and were negative for S-100 protein, thereby suggesting that the essential constituents of this lesion would derive from the mononuclear phagocyte system." 12/01/1987 - "Immunohistochemical examinations were performed using five kinds of histiocytic markers [S100 protein, lysozyme, non-specific cross reacting antigen with carcinoembryonic antigen (NCA), alpha 1-antichymotrypsin (alpha 1-ACT) and alpha 1-antitrypsin (alpha 1-AT)] in biopsied tissues from histiocytosis X, juvenile xanthogranuloma, xanthoma tuberosum, xanthoma disseminatum, reticulohistiocytic granuloma and multicentric reticulohistiocytoma, all of which have been classified as histiocytic proliferative disorders. " Order ALL the reference details at left...
6.	Cholestanol (Coprostanol)IBA 02/01/2001 - "Clinically, CTX patients present with tendon xanthomas, juvenile cataracts, and progressive neurological dysfunction and can be diagnosed by the detection of elevated plasma cholestanol levels. " 12/01/2006 - "It's characterized by accumulation of cholestanol and cholesterol in many tissues, in particular tendons and brain, with tendon xanthomas, juvenile cataracts, and neurological abnormalities. " 09/01/2006 - "Cerebrotendinous xanthomatosis is a rare autosomal recessive lipid storage disease characterized by widespread tissue deposition of two neutral sterols, cholestanol and cholesterol, resulting in tendinous xanthomas, juvenile cataracts, progressive neurological defects and premature death from arteriosclerosis. " Order ALL the reference details at left...
7.	CholesterolIBA 03/01/1988 - "Patients with the disorder present with tendon xanthomas, juvenile cataracts, dementia, and pyramidal and cerebellar abnormalities but have normal plasma cholesterol. " 12/01/2006 - "It's characterized by accumulation of cholestanol and cholesterol in many tissues, in particular tendons and brain, with tendon xanthomas, juvenile cataracts, and neurological abnormalities. " 09/01/2006 - "Cerebrotendinous xanthomatosis is a rare autosomal recessive lipid storage disease characterized by widespread tissue deposition of two neutral sterols, cholestanol and cholesterol, resulting in tendinous xanthomas, juvenile cataracts, progressive neurological defects and premature death from arteriosclerosis. " Order ALL the reference details at left...
8.	ParaffinIBA 07/01/1983 - "The cellular localization of glial S-100 protein was investigated in paraffin-embedded sections of cutaneous histiocytosis X and in a variety of cutaneous infiltrative disorders, including juvenile xanthogranuloma, necrobiotic xanthogranuloma, papular xanthoma, eruptive histiocytoma, and reticulohistiocytosis. " 01/01/1988 - "Archival paraffin blocks of 9 malignant fibrous histiocytomas (MFH), 3 dermatofibrosarcoma protuberans, 9 dermatofibromas, 1 juvenile xanthogranuloma, and 1 nodular fasciitis were processed, stained with propidium iodide, and analyzed by flow cytometry. " Order ALL the reference details at left...
9.	LipidsIBA 10/01/1986 - "Histopathological findings of the excised lesion were indistinguishable from the xanthogranulomas in infants and children (juvenile xanthogranuloma), and like those in the young, the present case was not associated with abnormalities of serum lipids and was not associated with other lesions. " 01/01/1976 - "These lesions were histologically indistinguishable from the xanthogranulomas in infants and children (juvenile xanthogranuloma), and, like those in the young, they were not associated with abnormalities of serum lipids. " Order ALL the reference details at left...
10.	alpha-Mannosidase (LAMAN)IBA 05/01/1991 - "A diffuse cellular positivity of alpha-mannosidase in juvenile xanthogranuloma and congenital self-healing histiocytosis differed from a typical perinuclear globular positivity of this enzyme in Langerhans cell histiocytosis. " Order ALL the reference details at left...

Therapies and Procedures

1.	X-Ray Therapy 07/04/1964 - "NEVOXANTHO-ENDOTHELIOMA (JUVENILE XANTHOGRANULOMA) OF THE EYE CURED WITH X-RAY THERAPY." Order ALL the reference details at left...
2.	Drug Therapy (Chemotherapy) 03/01/2002 - "Surgical treatment of the cerebellar lesion was associated with vinblastin chemotherapy, that led to total regression of the cutaneous and pulmonary lesions, neurological stabilization, without recurrence on the control IRM, after a period of 7 years.DISCUSSION: This is the second reported case of histologically documented cerebellar juvenile xanthogranuloma. " 10/01/2008 - "The development of juvenile xanthogranuloma (JXG) as a sequel to langerhans cell histiocytosis (LCH) treated with chemotherapy is rare and the hypothesis is intriguing. " 06/01/2004 - "Successful treatment of congenital systemic juvenile xanthogranuloma with Langerhans cell histiocytosis-based chemotherapy." 01/01/2004 - "OBSERVATIONS: We report 2 patients affected by Langerhans cell histiocytosis who, during chemotherapy, presented cutaneous lesions with clinical and histological features of juvenile xanthogranuloma. " 07/01/1994 - "Since even a massive juvenile xanthogranuloma of the iris responds well to steroids, operative and radiation treatment should be limited to cases refractory to antiinflammatory drug therapy." Order ALL the reference details at left...
3.	Curettage 01/01/2007 - "We report a case of juvenile xanthogranuloma, which formed a relatively large nodule and rapidly progressed after curettage. " 01/01/2007 - "Juvenile xanthogranuloma presenting rapid progression after curettage: a case report with clinicopathological findings." 10/01/1984 - "Curettage was performed at seven weeks after birth and histological examination established a diagnosis of juvenile xanthogranuloma. " Order ALL the reference details at left...
4.	Liver Transplantation 02/01/2008 - "We describe a newborn girl initially described as a "blueberry muffin baby" with multiple juvenile xanthogranulomas of the skin and liver that were complicated by progressive cholestasis and portal hypertension necessitating a liver transplantation." Order ALL the reference details at left...
5.	Tracheostomy 02/01/2007 - "Managing isolated subglottic juvenile xanthogranuloma without tracheostomy: case report and review of literature." Order ALL the reference details at left...

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