Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 15-month-old girl with Langerhans cell histiocytosis (LCH), Letterer-Siwe disease, was referred to our hospital in 1984. Whilst on treatment with cytotoxic drugs, a perirenal mass was detected and hydronephrosis became evident when she was 29 months old. Percutaneous nephrostomy tubes were placed in the pelvis, bilaterally and replaced every 6 months. The mass was not completely controlled and chronic pyelonephritis continued. Biopsy of the mass convoluted kidney hilus revealed histiocytic invasion. Although multiple organ systems are involved in LCH and abdominal malignant tumours may be accompanied by hydronephrosis, to our knowledge, this is the first case report of abdominal LCH and the ensuing hydronephrosis. Percutaneous nephrostomy tubes proved useful, but more convenient, less painful and infection-limited approaches need to be designed.
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PMID:Perirenal mass of Langerhans cell histiocytosis. 772 Jul 38

A boy with skin eruptions resembling varicella and specific for Langerhans cell histiocytosis (LCH) is reported. At his initial visit when he was four months old, vesiculopustular lesions were present over the entire body; these had first appeared on the third day post partus. Histopathological, immunohistochemical, and electron microscopical examination confirmed the Langerhans cell phenotype and Birbeck granules in the responsible cells. He also had hydronephrosis, recurrent fever, and cutaneous bacterial infections. His parents refused further medical treatment and he died of diarrhea with cachexia about two years later. LCH may present diagnostic difficulties by manifesting as a skin eruption which resembles varicella.
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PMID:Langerhans cell histiocytosis presenting as a varicelliform eruption over the entire skin. 801 75

Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis characterized by progressive histiocytic proliferation with multiorgan involvement, typically of the kidney, skin, brain, and lung, and less frequently, the heart and retro-orbital tissue. Fluorine-18 fluorodeoxyglucose positron emission tomography (F-18 FDG PET) plays an important role in the management of this disease. It has been reported that FDG PET imaging allows accurate evaluation of the extent of the disease at baseline, as well as assessment of response to any specific therapy. In this case, a 57-year-old Chinese man presented with functional decline and a urinary tract infection. He had a prior history of xanthogranulomas of bilateral canthal masses. On imaging, he was found to have left hydronephrosis, diffuse urothelial thickening, increased density of the perinephric fat, mural thickening of the descending aorta and soft tissue masses along the posterior wall of the right atrium extending into the region of the interatrial septum and involving the right atrioventricular groove. Histopathology revealed retroperitoneal fibrosis. An IV contrast-enhanced FDG PET scan showed increased activity in a previously unidentified brain stem mass and the shafts of bilateral femora. Varying levels of FDG uptake were seen in the other lesions.
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PMID:Intensely hypermetabolic extra-axial brainstem tumor in Erdheim-Chester disease. 1969 24