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)

Pathological or spontaneous fractures in childhood are rare and are mostly caused by benign bone diseases; the fractures must be treated on an individual basis, as there are no constant rules. Since the new method of osteosynthesis called "elastic stable intramedullary nailing" (ESIN) or "embrochage centro-medullaire elastique stable" (ECMES) has demonstrated the best results in the treatment of normal fractures in childhood, this method is rapidly being used in the treatment of spontaneous or pathological fractures. We report our experience in the treatment of spontaneous fractures in childhood with "elastic stable intramedullary nailing", in nine patients with ten fractures. The pathological diagnosis was in 5 cases a juvenile bone cyst; in addition, there were cases of histiocytosis X, lymphangiomatosis, hemangiomatosis and osteoporosis (one each). The location was the femur (two cases) and humerus (seven cases). All fractures healed completely without pseudarthrosis; as complications we saw one incidence of osteomyelitis, one of a second fracture after "elastic stable intramedullary nailing" and one coxa vara in a child with histiocytosis X of the proximal femur. In the 5 children with juvenile bone cysts the nails are still in situ; in two cases the nails had to be changed.
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PMID:[Treatment of pathological fractures of long tubular bones in childhood using elastic stable intramedullary nailing]. 876 36

Congenital self-healing reticulohistiocytosis (CSRH), a rare benign variant of Langerhans cell histiocytosis (LCH), is characterized by (a). congenital skin lesions, (b). a healthy infant with no systemic involvement, (c). the histopathologic finding of a Langerhans cell infiltrate and (d). spontaneous involution within the first year of life without sequelae. We report a Taiwanese girl born with widespread hemangioma-like lesions. The diagnosis of LCH was confirmed by finding a diffuse dermal infiltrate of S-100-protein- and OKT6 (CD1a)-positive mononuclear cells and the presence of Birbeck granules in 10% of the mononuclear cells ultrastructurally. The diagnosis of CSRH was further established by rapid and complete involution of the lesions in 3 months. No recurrence was noted for 7 years. Our case illustrates that CSRH can mimic diffuse neonatal hemangiomatosis clinically; thus, it is important to include CSRH in the differential diagnosis of congenital or neonatal hemangiomatosis.
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PMID:Congenital self-healing reticulohistiocytosis mimicking diffuse neonatal hemangiomatosis. 1505 4