The use of thermography in clubbing.
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Adolescent↗
Biomedical subjects
A Shore
Publications and source records attributed to A Shore.
Pressure of lymphatic capillaries in human skin.
The network of lymphatic capillaries of the human skin was depicted at the distal part of the tibial plateau by fluorescence microlymphography (fluorescein isothiocyanate-dextran 150,000). Intralymphatic pressure was determined in 28 lymphatic capillaries of 21 healthy volunteers (mean diameter 56.0 +/- 10.0 microns) by a servo-nulling pressure system. It averaged 4.0 +/- 4.5 mmHg (range: -6.8 to +10.7 mmHg). These are the first measurements of pressure in the initial lymphatics of human skin and form a basis with which to compare measurements made in patients with different forms of edema.
Adult↗
Childhood linear scleroderma: a possible role of thermography for evaluation.
Linear scleroderma is a rare, at times debilitating, disease with an unpredictable course. Currently, there is no satisfactory objective method for assessment of disease activity upon which to base therapeutic decisions. We evaluated 11 children with 18 linear scleroderma lesions (mean age 11.7 years, mean duration of disease 5.1 years) for disease severity and the presence of immunologic abnormalities, and attempted to correlate these results with thermography. Positive thermography was defined as warmer than surrounding skin or opposite limb by 0.5 degrees C. Six patients were thermography positive. Mean age, sex, disease duration and the presence of hypergammaglobulinemia and autoantibodies were similar in thermography positive and thermography negative patients. Six of 18 linear scleroderma lesions were thermography positive. All 3 new or expanding lesions were thermography positive. All 3 lesions that were resolving clinically were thermography negative. Three of 12 lesions that were clinically unchanged over a 6-month period were also thermography positive. In summary, thermography is a noninvasive test that appears to demonstrate active lesions in linear scleroderma. It is not influenced by previous soft tissue damage induced by linear scleroderma and may enable better monitoring of the effectiveness of proposed therapies.
Adolescent↗
Hepatitis C infection in children with hemophilia A and B.
Antibodies to hepatitis C virus (anti-HCV) were quantitated in stored sera from selected groups of hemophilic children (less than or equal to 18 years of age). During the period 1987 to 1989, seropositivity rates were as follows: untransfused hemophiliacs 0% (0 of 11 cases), hemophiliacs treated exclusively with vapor-heated factor VIII or IX concentrates 0% (0 of 9 cases), hemophiliacs treated only with cryoprecipitate or single donor blood products 0% (0 of 9 cases), and hemophiliacs regularly treated with unheated or dry heat-treated factor VIII or IX concentrates 95% (21 of 22 cases). Corresponding alanine aminotransferase (ALT) results were similar: values were always below the upper limit of laboratory normal (40 U/L) in untransfused hemophiliacs, hemophiliacs treated with vapor-heated factor concentrates, or those who received only cryoprecipitate or single donor blood products. By contrast ALT values were greater than 40 U/L in 82% (18 of 22 cases) of hemophilic children regularly infused with unheated or dry heat-treated factor concentrates. Three conclusions are drawn from this data: (1) HCV is a major cause of chronic hepatitis in multitransfused hemophilic children, (2) unheated and dry heat-treated clotting factor concentrates carry a very high risk of transmitting HCV infection, and (3) clotting factor concentrates inactivated by vapor heating carry a very low and perhaps zero risk of transmitting HCV infection. These findings are of therapeutic significance for previously untransfused hemophiliacs susceptible to HCV infection.
Alanine Transaminase↗
Short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency in muscle: a new cause for recurrent myoglobinuria and encephalopathy.
We report on a 16-year-old girl with short-chain L-3-hydroxyacyl-coenzyme A (CoA) dehydrogenase deficiency resulting in juvenile-onset recurrent myoglobinuria, hypoketotic hypoglycemic encephalopathy, and hypertrophic/dilatative cardiomyopathy. Urinary organic acids showed traces of 3-hydroxy-dodecanedioic acids and small amounts of suberic, sebacic, and adipic acids. There was a marked decrease in L-3-hydroxyacyl-CoA dehydrogenase activity in muscle with acetoacetyl-CoA as substrate (2.48 mumol/min/gm; normal = 6.90 +/- 1.80 mumol/min/gm of tissue; n = 11), contrasting with normal L-3-hydroxyacyl-CoA dehydrogenase activity with 3-ketooctanoyl-CoA and 3-ketopalmitoyl-CoA as substrates. Short-chain L-3-hydroxyacyl-CoA dehydrogenase activity was normal in fibroblasts, suggesting a tissue-specific defect.
3-Hydroxyacyl CoA Dehydrogenases↗
Wegener's granulomatosis in childhood.
The authors reviewed the medical histories and radiological examinations of five pediatric patients with a histologic diagnosis of Wegener's granulomatosis (WG) seen over a six year period in whom a total of 22 thoracic CT scans were performed. Involvement of both the upper and lower respiratory tracts was seen in all patients at presentation. One patient had subglottic stenosis necessitating tracheotomy. Pulmonary hemorrhage occurred in three patients at initial diagnosis. Classic cavitary lung nodules were seen in two patients--one at initial presentation, the other at relapse. The plain radiographic lower respiratory tract manifestations of pediatric WG were protean both at initial presentation and during follow up. Similarly, disease expression was highly variable on thoracic CT examinations but, overall, multifocal parenchymal infiltrates with or without small peripheral nodules were the commonest thoracic CT manifestations. As a consequence of cytotoxic and corticosteroid therapy the long-term prognosis of WG has improved considerably in recent years. Knowledge of the varied patterns of the primary disease and potential for iatrogenic complications are necessary for successful radiologic assessment of pediatric patients with WG.
Adolescent↗
Splenic necrosis in Wegener's granulomatosis.
The CT appearance of asymptomatic splenic necrosis in a 11-year-old girl with Wegener's granulomatosis is presented. Sonography showed splenic inhomogeneity with patency of the splenic artery and vein. Follow-up CT showed lobulation and shrinkage of the spleen with return to a normal attenuation.
Adolescent↗
Absence of an association between HLA typing in cystic fibrosis arthritis and hypertrophic osteoarthropathy.
Fourteen patients with cystic fibrosis arthritis and eight patients with cystic fibrosis and hypertrophic osteoarthropathy were typed for HLA-A, B, C, DR, and DQ antigens and were compared with age and sex matched controls with cystic fibrosis. The diagnosis of cystic fibrosis arthritis and hypertrophic osteoarthropathy was confirmed by radiography and bone scanning. The prevalence of HLA-A, B, C, D, antigens in the cystic fibrosis group (44 patients) did not differ from that in the control group. A comparison between patients with cystic fibrosis arthritis or hypertrophic osteoarthropathy and their respective controls did not show any significant differences in HLA prevalence. It is concluded that HLA antigens may not be a factor in the susceptibility of patients with cystic fibrosis to cystic fibrosis arthritis or hypertrophic osteoarthropathy.
Adult↗
Reconstitution of impaired autologous mixed lymphocyte reactivity in rheumatoid arthritis.
We examined the ability of recombinant IL-2 to reconstitute the autologous mixed lymphocyte reaction (AMLR) defect in peripheral blood mononuclear cells (PBM) from patients with rheumatoid arthritis (RA). Our results revealed an ability to fully reconstitute RA AMLRs with pharmacologic concentrations (100 units/ml), but not physiologic concentrations (10 units/ml) of IL-2. Full reconstitution of RA AMLRs was achieved whether IL-2 was added as the initiation of culture or at 48 or 72 hours prior to termination of the cultures. Impaired IL-2 production was noted throughout the time course of the RA AMLRs. Neither an inhibitor of IL-1 nor IL-2 was detected in AMLR culture supernatants. Moreover, IL-1 in pharmacologic concentrations up to 50 units/ml failed to reconstitute impaired AMLR reactivity. In 2 patients whose AMLRs failed to reconstitute fully with 100 units/ml IL-2, addition of 10 units/ml IL-1 in combination with IL-2 fully reconstituted the AMLR defect. The results may suggest that defective IL-2 generation alone cannot fully account for impaired AMLR reactivity in RA patients.
Arthritis, Rheumatoid↗
[Pressure measurement in lymph capillaries of the human skin].
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Adult↗
Bowel perforation and interstitial cystitis in childhood systemic lupus erythematosus.
A 13-year-old girl presented with abdominal pain, fever, dysuria, incontinence and pyuria and was subsequently diagnosed as having systemic lupus erythematosus (SLE) with extensive gastrointestinal involvement and an associated interstitial cystitis. Despite aggressive therapy with high dose prednisone and cyclophosphamide she developed a small bowel perforation and subsequently died. The combination of bowel symptoms and interstitial cystitis seems unique to the population with SLE, while the separate complication of bowel perforation carries an extremely poor prognosis in this group of patients.
Adolescent↗
Acquired Brown's syndrome associated with hypogammaglobulinemia.
We describe a 10-year-old girl with hypogammaglobulinemia who presented initially with painless progressive diplopia on right upward gaze and associated tenderness in the area of the superior oblique tendon (Brown's syndrome). She was given prednisone (40 mg/day) with gradual improvement of her symptoms. Prednisone was tapered and her symptoms remained unchanged for 10 months. She then experienced rapid deterioration. Prednisone was reinstituted with similar improvement and then therapy was switched to naproxen. She remains with only slight diplopia on extreme upward gaze.
Agammaglobulinemia↗
Intravenous gamma globulin therapy in systemic juvenile rheumatoid arthritis.
Intravenous (IV) gamma globulin has been successfully used as replacement therapy for antibody-deficient patients and, more recently, in the treatment of autoimmune diseases such as idiopathic thrombocytopenic purpura, myasthenia gravis, and Kawasaki disease. In view of the successful treatment of these diseases, we initiated a pilot study of the effect of IV gamma globulin in systemic juvenile rheumatoid arthritis (JRA). Eight patients with active systemic JRA that was unresponsive to first-line agents, second-line agents, and/or corticosteroids received this therapy monthly for 6 months. Outcome measures included changes in articular and extraarticular features, steroid dosage, and laboratory parameters. Following IV gamma globulin therapy, there was significant improvement in arthritis and/or morning stiffness in 5 of 8 patients, while extraarticular features significantly improved in 7 of 8 patients. At study entry, 6 of 8 patients were receiving prednisone; at study end, prednisone was discontinued in 3 patients and decreased by more than 50% in the other 3. Overall, there was an 80% reduction in the prednisone dosage. Initially, all patients had anemia, low levels of serum albumin, and an elevated erythrocyte sedimentation rate, while a thrombocytosis was seen in 7 of 8 patients. Serum IgG was initially elevated in 6 patients. IV gamma globulin therapy resulted in a significant increase in hemoglobin and albumin levels and a significant decrease in the mean serum IgG level, platelet count, and erythrocyte sedimentation rate. In only 1 patient did IV gamma globulin fail to significantly improve the clinical or laboratory features of the disease. We suggest that this therapy may be beneficial in the treatment of systemic JRA.
Arthritis, Rheumatoid↗
Juvenile psoriatic arthritis and HLA antigens.
The clinical, laboratory, and radiological features, including histocompatibility typing, of 28 patients with juvenile psoriatic arthritis are reported. The most common presentation was that of psoriasis preceding or occurring simultaneously with arthritis. The most common course of juvenile psoriatic arthritis was to start as an oligoarthritis and progress, usually to polyarthritis. No patients with juvenile psoriatic arthritis had uveitis. Overall, most patients had a good outcome (93% in functional class I and II), though 8/28 (29%) did require disease modifying drugs over a mean period of 8.8 years of follow up. The clinical features of these patients were very similar to those of a group of 158 adult patients with psoriatic arthritis with the same disease duration followed up in the clinic. Although there was an increased prevalence of B17 in both juvenile and adult psoriatic arthritis, juvenile psoriatic arthritis showed increased prevalence of A2, whereas adult psoriatic arthritis showed increased prevalence of B27, Bw39, and Cw6. This HLA association differed from that reported in other forms of juvenile arthritis.
Adolescent↗
Endothelial cells modulate both T-cell-dependent and T-cell-independent plaque-forming cell generation in vitro.
The effects of live endothelial cells (EC), paraformaldehyde fixed EC, and EC supernatant were measured on pokeweed mitogen (PWM)-induced T-cell-dependent plaque-forming cell (PFC) generation by peripheral blood mononuclear cell (PBM). At low doses (less than or equal to 2 x 10(4) cells/culture) live EC helped PFC generation. At higher doses (greater than or equal to 10 x 10(4) cells/culture) the effect of live EC was always marked suppression (less than 10% of baseline PFC). In contrast both fixed EC and EC supernatant provided help exclusively over a wide dose range. The EC-helper effect enhanced the sensitivity of PBM to suboptimal PWM doses and also accelerated the rate of PFC generation during culture. EC influences on PFC could not be modified by gamma-interferon induction of surface DR which is known to modify EC accessory cell ability. There was also only minimal helper activity of live EC and fixed EC on the PFC generation by Epstein-Barr virus-induced cultures of purified B cells (which had been depleted of both T cells and monocytes). In contrast, suppression (greater than 97%) of PFC in isolated B-cell cultures was found even when EC constituted less than 1% of cultured cells. These results imply EC have the potential of providing multiple regulatory signals which modulate in vitro antibody production. EC-derived mechanisms are independent of their accessory cell function and require interaction with non-B cells for help, but suppression may occur directly at the B-cell level.(ABSTRACT TRUNCATED AT 250 WORDS)
B-Lymphocytes↗
A review of current concepts on the pathogenesis of juvenile rheumatoid arthritis.
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Arthritis, Rheumatoid↗
Methotrexate-nonsteroidal antiinflammatory drug interaction in children with arthritis.
In order to assess the interaction between methotrexate (MTX) and nonsteroidal antiinflammatory drugs (NSAID), we studied the pharmacokinetics of oral MTX alone and in the presence of the usually prescribed NSAID in 7 children with chronic arthritis. The NSAID studied included tolmetin, indomethacin, naproxen, and aspirin. Six patients were treated with multiple NSAID. The mean MTX elimination half-life was prolonged when NSAID were coadministered (1.7 +/- 0.5 vs 1.2 +/- 0.1 h; p = 0.03). However, neither the apparent MTX clearance (CI) (10.6 +/- 5.5 vs 13.1 +/- 3.5 l/h; p = 0.19), the area under the serum MTX concentration-time curve (Auc) (2.1 +/- 1.0 vs 1.5 +/- 0.6 mumol/l/h; p = 0.08) or the apparent volume of distribution (Vd) (23.0 +/- 6.2 vs 21.9 +/- 6.4 l; p = 0.53) was significantly altered by the administration of NSAID. Although the differences between the mean Cl and Auc were not statistically significant, a wide variation in the impact of NSAID on MTX Cl was observed. In 6 of 7 patients, the Auc increased during NSAID administration from 19 to 140%. This degree of increase may be clinically significant in some individuals. It is consequently recommended to closely monitor patients who are receiving MTX and NSAID for MTX toxicity until these results can be verified in a larger population.
Anti-Inflammatory Agents, Non-Steroidal↗
Impaired late suppression of Epstein-Barr virus (EBV)-induced immunoglobulin synthesis: a common feature of autoimmune disease.
We examined regulation of Epstein-Barr virus-induced plaque-forming cell generation in peripheral blood mononuclear cells from several autoimmune and seronegative diseases and correlated these results with Epstein-Barr virus-induced proliferation. We confirmed the defective regulation of Epstein-Barr virus-induced plaque-forming cells in peripheral blood mononuclear cells of patients with rheumatoid arthritis and scleroderma. Peripheral blood mononuclear cells from patients with seronegative arthropathies and chronic infective inflammation (cystic fibrosis) had normal regulation of Epstein-Barr virus-induced plaque-forming cells. Peripheral blood mononuclear cells from rheumatoid arthritis had excessive plaque-forming cell generation in the face of a normally regulated decrease in Epstein-Barr virus-induced proliferation. In contrast, peripheral blood mononuclear cells from scleroderma had defective suppression of both Epstein-Barr virus-induced proliferation and plaque-forming cell generation. Thus, impaired regulation of Epstein-Barr virus-induced plaque-forming cell generation is a common feature of autoimmune disease and demonstrates some specificity for these disorders.
Adult↗