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Biomedical subjects

H Emery

Publications and source records attributed to H Emery.

11 recordsLinked to original sources

Pediatric scleroderma.

Scleroderma is a diverse group of conditions which have in common fibrosis of skin and other tissues. Although less common in children than in adults, these conditions are an important cause of morbidity and occasional mortality when they occur in the pediatric population. Children are more likely than adults to develop localized forms of scleroderma, and because of the impact on growth, these can result in major facial or limb asymmetry, flexion contractures, and disability. Management approaches must take into consideration the effect of medications on the child (for example, growth failure and osteoporosis from corticosteroids) as well as the psychosocial impact of chronic illness and physical deformity on the child and family. This article describes the types of scleroderma identified in children, reviews epidemiologic and etiologic factors, and discusses management options. Because this is a rare group of diseases managed by both dermatologists and rheumatologists, large series of patients are rare, and controlled studies of management are not available.

Adolescent↗

Patterns of expression of tumor necrosis factor alpha, tumor necrosis factor beta, and their receptors in synovia of patients with juvenile rheumatoid arthritis and juvenile spondylarthropathy.

OBJECTIVE: To assess the expression of tumor necrosis factor alpha (TNF alpha), TNF beta, and their receptors in synovia of patients with juvenile rheumatoid arthritis (JRA) and juvenile spondylarthropathy (JSpA), and to determine similarities with and differences from adult RA. METHODS: Twenty-eight synovial tissue samples from patients with JRA, 6 from patients with JSpA, and 6 from patients with RA, selected for the presence of inflammatory infiltrates, were analyzed for the expression of TNF alpha, TNF beta, and their receptors (p55 and p75 TNFR), utilizing the dual approach of reverse transcriptase-polymerase chain reaction and immunohistochemistry analysis. RESULTS: The presence of both TNF alpha and TNF beta expression was demonstrated in most JRA and JSpA tissues, although samples from patients with pauciarticular JRA had somewhat lesser amounts of these cytokines. TNF beta expression correlated significantly with the occurrence of lymphocytic aggregates in tissues. Staining with monoclonal antibodies specific for the p55 and p75 receptors revealed that a diverse range of cell types expressed the receptors, with the most intense p55 staining on vascular endothelial cells. In the vast majority of synovial tissues, far greater numbers of cells expressed the p55 form of the receptor than the p75 form. CONCLUSION: JRA and JSpA synovia are characterized by the presence of TNF alpha, TNF beta, and cells expressing TNFR. These findings provide further evidence that TNF, through autocrine/paracrine mechanisms, may amplify local inflammation, leading to joint destruction. The prominence of TNF beta in the synovium in particular subgroups of JRA patients and in JSpA patients may be a distinguishing feature of these diseases.

Adult↗

Immunohistological characteristics of T cell infiltrates in different forms of childhood onset chronic arthritis.

OBJECTIVE: To characterize synovial T cell infiltrate, in terms of CD4/CD8 ratio and level of activation of T cells, in juvenile rheumatoid arthritis (JRA) and juvenile spondyloarthropathy(JSpA), to correlate these findings with clinical outcomes of the different forms of disease, and to compare them with findings in adult RA synovium. METHODS: We studied synovial tissue specimens from 22 individuals with childhood onset of chronic arthritis (12 polyarticular JRA, 5 pauciarticular JRA, 5 JSpA) and 4 with adult RA. Specimens were selected from an initial bank from 40 patients on the basis of significant inflammation on hematoxylin and eosin and CD3 and CD68 monoclonal antibody staining (T cells and macrophages, respectively). Indirect immunohistochemistry was used with monoclonal antibodies to CD3, CD4, CD8, and interleukin 2 receptor alpha to determine CD4/CD8 ratios and the levels of activation within the T cell subsets. The distribution of gamma delta T cells was also studied. RESULTS: Two patterns of T cell infiltration were seen. The majority of patients had lymphocytic aggregates associated with diffuse infiltrates; a few tissue specimens had diffuse infiltrates without aggregates. The CD4/CD8 ratio was significantly lower in pauciarticular course JRA than polyarticular JRA (p < 0.01) and RA (p < 0.05). Similarly patients with JSpA had a significantly lower CD4/CD8 ratio than patients with polyarticular JRA (P < 0.05). The level of T cell activation (CD3+IL-2R+) was significantly higher in pauciarticular compared with both polyarticular JRA (P < 0.01) and RA (p < 0.05). In general, higher levels of activation of CD8 cells than CD4 cells were seen, particularly in the pauciarticular JRA and JSpA groups. gamma delta T cells were prominent in 2 patients. CONCLUSION: Demonstrated differences in T cell subset distribution between types of childhood chronic arthritis at a histopathological level may reflect different pathogenic mechanisms.

Adolescent↗

Neonatal onset multisystem inflammatory disease.

Neonatal onset multisystem inflammatory disease (NOMID) is a rare disorder involving a triad of arthropathy, rash, and central nervous system (CNS) involvement. We describe a girl with NOMID who presented with typical neonatal rash, arthropathy, fever, and failure to thrive, but has not developed evidence of ocular or CNS involvement. This case illustrates the spectrum of involvement seen in NOMID. Histopathology of the skin demonstrated neutrophilic eccrine hidradenitis, a unique finding, which may serve as a diagnostic clue in patients with this rare disorder.

Age of Onset↗

Clinical aspects of systemic lupus erythematosus in childhood.

Systemic lupus erythematosus (SLE) is an uncommon childhood illness that is characterized by the formation of autoantibodies and immune complexes, which mediate inflammatory responses in multiple organ systems. Children who develop SLE are frequently very ill at the time of presentation and need careful evaluation to determine which organ systems are involved and how severely, meticulous control of medications to suppress active disease, and close monitoring to avoid complications from both the disease and its treatment.

Bone Diseases↗

Platelet ascorbic acid levels in normal subjects and in disease.

The platelet ascorbic acid concentration was measured in 26 normal subjects and found to be 20 times as high as in plasma. This is in agreement with previous reports in the literature. The platelets of patients with uraemia, leukaemia, and megaloblastic anaemia had a lower than normal platelet ascorbic acid content. In uraemia and megaloblastic anaemia the plasma ascorbic acid concentration was normal suggesting that a platelet defect may be responsible for the low platelet ascorbic acid content. In leukaemia the low platelet ascorbic acid content is probably secondary to a low plasma level.

Acute Disease↗

Factors relating to hip joint arthritis following three childhood diseases--juvenile rheumatoid arthritis, Perthes disease, and postreduction avascular necrosis in congenital hip dislocation.

This article discusses factors that relate to the prognosis for development of hip joint arthritis following juvenile rheumatoid arthritis (JRA), Perthes disease, and postreduction avascular necrosis in congenital hip dislocation (CDH). In JRA, the integrity of the articular cartilage determines prognosis. In Perthes disease, prognosis is strictly related to the shape of the hip. In postreduction avascular necrosis in CDH, half the patients have a prognosis primarily related to hip joint shape, and half have a prognosis apparently more related to the integrity of the articular cartilage.

Adult↗