Education of rheumatic disorders at Dutch medical schools.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to J C Jacobs.
Explore the source record for details and available documents.
The 5th International Kawasaki Disease Symposium was held in May 1995 in Fukuoka, Japan. No one infectious agent has been found to initiate the syndrome, although the occurrence of Kawasaki disease in epidemics and its resemblance to toxic-shock syndrome make an infectious etiology seem most likely.
OBJECTIVE: To determine the safety and therapeutic potential of sulfasalazine (SSZ) in the treatment of a large cohort of patients with juvenile rheumatoid arthritis (JRA). METHODS: All patients who required the addition of a second line agent were offered SSZ and assessed at regular intervals. Thirty patients took SSZ as their sole drug therapy. One hundred thirty-nine patients with an average age of 11.5 yrs (range 1.5-21.8 yrs) took the medication. The duration of symptoms at the start of treatment was 30 mo (1-130 mo). All subtypes of JRA were included in the study group. Patients were treated for a mean of 13 mo (1-42 mo) with 31 mg/kg/day of SSZ. Significant improvement was defined as 50% decrease in the number of joints with active arthritis; or 50% decrease in the number of joints with effusion; or 50% decrease in total degrees of joint contractures; or normalization of an elevated erythrocyte sedimentation rate within 12 months of starting treatment. Data on adverse reactions, remissions, and treatment failure were also collected. RESULTS: One hundred two patients (73%) had significant improvement after starting SSZ. Fifty-six patients (40%) were able to stop all other medication at an average of 9.5 mo. Thirty-nine patients (28%) remitted and discontinued all medication. Twenty-three patients (17%) discontinued the drug for adverse reactions. All reactions resolved completely when the drug was discontinued. CONCLUSION: SSZ is safe and appears to be an effective primary or second line therapy for JRA, and should be studied further in a multi-institutional, placebo controlled study.
To examine the role of circulating fat in the regulation of carbohydrate metabolism, dogs were studied during rest and 90 min of moderate treadmill exercise with nicotinic acid infused to suppress lipolysis with (+Fat; n = 5) or without (-Fat; n = 5) Intralipid. Isotopic and hindlimb arteriovenous methods were used to assess metabolism. Plasma glucose was similar in both protocols during rest and exercise. Differences in insulin, catecholamines, and cortisol between groups were insignificant. Glucagon was approximately 50% greater during rest and exercise in -Fat. The following values represent those at 30 or 40 min of muscular work because peak responses were seen at these times. Arterial free fatty acid levels were 1,129 +/- 253 and 272 +/- 17 mu eq/l at rest and 756 +/- 145 and 269 +/- 51 mu eq/l with exercise in +Fat and -Fat, respectively. Glucose production was 4.2 +/- 0.3 and 5.0 +/- 0.4 mg.kg-1.min-1 at rest and 8.5 +/- 1.3 and 11.4 +/- 0.6 mg.kg-1.min-1 with exercise in +Fat and -Fat, respectively. Glucose utilization was 4.3 +/- 0.3 and 5.3 +/- 0.2 mg.kg-1.min-1 at rest and 9.2 +/- 1.2 and 12.7 +/- 0.8 mg.kg-1.min-1 with exercise in +Fat and -Fat, respectively. Significant glucose flux differences were present during rest and exercise. Limb glucose uptake rose similarly with exercise in +Fat (29 +/- 7 to 82 +/- 22 mumol/min) and -Fat (28 +/- 7 to 88 +/- 16 mumol/min). Arterial blood lactate was 50-100% greater in -Fat compared with that in +Fat.(ABSTRACT TRUNCATED AT 250 WORDS)
The pulmonary complications in 110 consecutive renal transplant recipients on cyclosporin and low-dose steroid immunosuppression were studied retrospectively. The pulmonary complications were: acute pulmonary oedema in 19 patients, pneumonia in 18, tuberculosis in 9, acute pulmonary embolism in 5, and lung abscess in 1. Sixty-nine patients (62.7%) had no pulmonary complications; 69% of the complications occurred in the first 4 months after the transplant. Pulmonary tuberculosis became evident later. The mean age, period of follow-up, human leucocyte antigen (HLA) B/DR mismatches, mean serum urea and serum creatinine concentrations, systolic and diastolic blood pressures, and cyclosporin dosage did not differ between the groups with no complications, infectious complications and non-infectious complications. The number of rejection episodes treated with bolus steroids was significantly higher in the infectious and non-infectious complications groups compared with the group with no complications. The incidence of pulmonary complications after renal transplantation, especially pneumonia and tuberculosis, was still high despite the use of low-dose steroids and cyclosporin. Pulmonary complications were the commonest cause of death in the first 3 years after the transplant. A high index of suspicion for pulmonary tuberculosis and pulmonary embolism in these patients is necessary.
Explore the source record for details and available documents.
In two children ages 8 months and 4 years, tuberculous synovitis of the knee was suggested by pathologic examination of synovial tissue obtained through needle biopsy. Culture of the biopsy material was positive for Mycobacterium tuberculosis in one case. In this child, the tuberculin test was negative, in the absence of anergy, at the time the child was first evaluated, and the primary lung lesion was not identified by the radiologist. Tuberculous synovitis has not previously been recognized as part of primary tuberculosis during the early weeks when the tuberculin skin test may be negative. Magnetic resonance imaging (MRI) is a sensitive modality for demonstrating joint fluid, synovial hypertrophy, and associated osteomyelitis, if present. With the increasing frequency of cases of tuberculosis in the population, greater awareness of the risk of tuberculous arthritis in childhood is important in order to recognize this unusual presentation. If negative early on, the tuberculin skin test should be repeated after 6 weeks of arthritis. A needle biopsy of the synovium is helpful and appropriate in all children with monoarticular arthritis who have a positive tuberculin skin test.
The lesions of HIV-associated nephropathy occur in patients with AIDS, AIDS-related complex and in individuals clinically asymptomatic for HIV infection. We report on a 35-year-old black South African woman who presented with nephrotic syndrome and renal failure. The renal biopsy appearance suggested HIV infection and this was subsequently verified. This finding emphasises the possibility that otherwise asymptomatic patients presenting with renal disease may be HIV-positive.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Eighty-eight children with juvenile rheumatoid arthritis (JRA) who completed a double blind, randomized placebo controlled trial of oral gold were entered into an open label extension phase during which they received auranofin (AF) at a dosage of 0.15-0.2 mg/kg/day (9 mg/day maximum). Eleven (12.5%) patients completed 5 years of AF therapy; 77 (87.5%) did not. Fifteen (17%) of the 88 were in disease remission at the final visit. Mean duration of therapy for those who discontinued was 646 days. Parental/patient decision and insufficient therapeutic effect were the 2 most frequent reasons for early termination, followed by adverse effects. Though relatively well tolerated, AF provides adequate longterm management for only a small percentage of patients with JRA.
After the parenteral administration of iron-dextran (imferon), the increased total iron concentrations in serum can be determined by atomic absorption spectroscopy and by colorimetric methods involving sodium dithionite, which reductively dissociates iron from the dextran complex. We report that constant-potential coulometry detects only about 55-70% of dextran-bound iron before dithionite reduction and variable amounts after reaction with the reducing agent. In addition, we have developed a procedure for determining transferrin-bound iron, total iron-binding capacity (TIBC), total iron, and dextran-bound iron with the Kodak Ektachem colorimetric system. In determining total serum iron, the sample is first mixed with sodium dithionite, which rapidly dissociates all dextran-bound iron, but does not remove iron from either transferrin or hemoglobin. After the mixture is applied to an Ektachem slide, transferrin-bound iron is released at pH 4 and is detected together with the iron previously bound to dextran. TIBC is determined by mixing serum with ferric citrate in moderate excess and filtering through a small alumina (Al2O3) column, which binds excess free iron and iron-dextran; the iron in the column eluate represents the TIBC. Transferrin-bound iron is determined by applying diluted serum without added ferric citrate to an alumina column and measuring the iron in the column eluate. Dextran-bound iron is equivalent to the difference between total and transferrin-bound iron. Using this method, we found that transferrin iron-binding sites are saturated in vitro by excess iron-dextran less efficiently than by ferric citrate.
In this report, we describe a case of acute leukemia possessing a unique immunophenotype. Leukemic cells isolated from peripheral blood were analyzed by automated fluorescence-activated flow cytometry. They demonstrated the following antigen pattern: CD7+, CD38+, transferrin receptor+, HLA-DR+, common ALL antigen (CALLA)-, and terminal deoxynucleotidyl transferase (TdT)-. No expression of B cell or myeloid antigens was observed. The observed antigen pattern suggests a pre-T cell origin. In addition, the tumor cells contained germline T cell receptor beta (T beta) and gamma (T gamma) chain genes, and a germline immunoglobulin heavy chain (JH) gene. These findings support the concept that pre-T cell leukemias may demonstrate germline T beta and T gamma genes as well as lack TdT expression. In addition, our results suggest that CD7 expression may be one of the earliest events in T cell differentiation, occurring prior to both T beta an T gamma gene rearrangements, and TdT expression.
Explore the source record for details and available documents.
We report a small kindred in which the father and daughter with positive antinuclear antibodies (ANA) (proband) had diffuse cutaneous and visceral scleroderma (systemic sclerosis, SSc), antitopoisomerase autoantibodies and shared the HLA-A23 C- B- DR5 DRw52 DQw3 haplotype. The ANA- granddaughter (daughter of the proband) was noted to have severe isolated pulmonary hypertension within the first 6 months of life, and had the other maternal HLA-A1 Cw8 B14 DR1 DQw1 haplotype, which included a B1, B2 duplication of the C4B allele. All 3 members shared DRw52. The possibility that neonatal pulmonary hypertension represents an isolated autoimmune disease or a hitherto undescribed neonatal syndrome is proposed and the immunogenetic autoantibody implications are discussed.