Kawasaki disease with atypical presentation.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to R M Laxer.
Explore the source record for details and available documents.
The safety of naproxen suspension was assessed in an open study in children with juvenile rheumatoid arthritis. Fifty-eight patients aged 1 to 13 (mean, 4.5 years) were studied. Based on the patient's condition, naproxen was prescribed at dosages ranging from 9 to 20 mg/kg/day. Follow-up assessments were made during regular clinic visits, as often as deemed necessary by the physician. Forty-four patients completed a minimum of six months' treatment. One patient was lost to follow up and 13 were withdrawn early: three because of unsatisfactory therapeutic response, one because of disease remission, five because of taste complaints, and four because of other side effects. The side effects were mostly gastrointestinal and were mild to moderate in severity. Investigators' subjective evaluations indicated that 84% of the patients who completed six months' treatment had good or excellent therapeutic responses at termination. The study results demonstrated that naproxen suspension is a well-tolerated anti-inflammatory agent for young children with juvenile rheumatoid arthritis.
A seroconversion to Borrelia burgdorferi, the etiological agent of Lyme disease, was documented in a 5 year old girl from central Ontario. Our patient developed juvenile arthritis in January, 1984 when she was 14 months old. It was relatively well controlled with aspirin. Her arthritis flared in April, 1986 at which time seroconversion to B. burgdorferi was demonstrated by indirect immunofluorescence and by enzyme linked immunosorbent assay. She was treated with phenoxymethyl penicillin and has had no further flare of her arthritis. The possible relationship between her rheumatic disease and her seroconversion to B. burgdorferi is discussed.
Explore the source record for details and available documents.
A 5-year-old girl who developed systemic lupus erythematosus 3 1/2 years after having had Kawasaki disease was found to be homozygous at both class I and class II MHC loci. This homozygosity suggests that she may also be homozygous for an MHC or non-MHC linked gene that could allow for the appearance of 2 diseases with similar immunoregulatory abnormalities, as occurs in the MRL/1pr/1pr mouse model. Although this is the first report of the occurrence of a second immunologic disorder in a patient with a history of Kawasaki disease, North American children who developed Kawasaki disease early in life are only now reaching the age when other inflammatory disorders become relatively common. Therefore, this group of children will have to be closely observed for the development of other inflammatory disorders.
A case of Acremonium species arthritis in a previously healthy child is reported. This fungus has frequently been implicated in cases of mycetoma and keratomycosis in the tropics, and invasive disease has occurred almost exclusively in immunocompromised patients. The acute presentation of this illness and its successful treatment with IV amphotericin-B are highlighted to alert physicians to this pathogen.
Recent observations that juvenile dermatomyositis (JDMS) may be a heterogenous disorder, in both its clinical course and prognosis, suggest that subgroups of patients with this disease may respond to various modes of therapy. Therefore, current treatment recommendations of daily, long-term oral corticosteroid treatment, with its attendant side effects, may not be necessary for all children with JDMS. We report the results of treatment with high-dose, pulse intravenous methylprednisolone in 7 children with JDMS, 3 of whom had an excellent response with complete remission and no need for daily steroid therapy. We suggest that patients with early, mild JDMS may be candidates for therapy with intravenous methylprednisolone.
Idiopathic subperiosteal new bone formation is uncommon in childhood. We report a case of diffuse periostitis in association with hypergammaglobulinemia and hypoalbuminemia in a 12-year-old boy. In 1966, 2 cases of idiopathic periosteal hyperostosis with accompanying dysproteinemia were documented. This report serves as a review of this unique and perplexing problem in clinical diagnosis.
We report seven patients with chronic recurrent multifocal osteomyelitis, an uncommon childhood disease of unknown etiology. These patients presented with insidious onset of bone pain at one or more sites associated with erythema, swelling and tenderness. Scintigraphy and radiography were consistent with osteomyelitis at multiple sites. Bone biopsies confirmed osteomyelitis but no organisms were consistently isolated. During a 1- to 3-year follow-up, most patients developed new symptomatic lesions. The disease was unaffected by antimicrobial therapy. Two of our patients had psoriasis and all were rheumatoid factor-, antinuclear factor- and HLA-B27-negative. We speculate that chronic recurrent osteomyelitis is a noninfectious inflammatory condition, a seronegative spondyloarthropathy. Chronic recurrent osteomyelitis is a clinical entity that should be recognized so that invasive diagnostic procedures and antimicrobial therapy are appropriately used. The patient may be reassured that this is not a malignant condition although there may be exacerbations over many years.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Antibodies from 5 patients with systemic sclerosis reacted with an antigen localized to the metaphase chromatin, the cleavage furrow and the midbody of anaphase and telophase HEp-2 cells. The titer of antimidbody antibodies ranged from 1:160 to 1:1280. Four patients had systemic sclerosis and one had idiopathic Raynaud's phenomenon. In situ biochemical characterization of the antigen revealed that it was resistant to DNase I, micrococcal nuclease and RNase A, but was sensitive to trypsin treatment. The antigen remained insoluble in 400 mM acetic acid but was extracted from the cells with 400 mM hydrochloric acid. The antibody was not seen in sera from 2500 normal female blood donors, 120 patients with systemic lupus, 60 patients with rheumatoid arthritis, 15 patients with linear scleroderma or 25 patients with Raynaud's disease.
Renal failure occurred in a 14-year-old girl with peripheral arthritis associated with inflammatory bowel disease while she was being treated with naproxen. She had previously received aspirin and tolmetin sodium and had no complications. A renal biopsy showed a severe tubulointerstitial nephritis. Although her renal function improved somewhat with corticosteroid treatment, it worsened when the steroids were discontinued. This case emphasizes that renal failure can develop insidiously in children on nonsteroidal anti-inflammatory drug therapy and that such children must be monitored closely for signs of nephrotoxicity.
Five ambulatory children with various types of chronic arthritis developed renal papillary necrosis (RPN), as documented by intravenous pyelography. Each child was being treated with nonsteroidal anti-inflammatory drugs (NSAIDs) at the time of diagnosis of RPN and had had episodes of gross or microscopic hematuria for several months prior to diagnosis. In each child hematuria was associated with more than one NSAID, and three of the five children also had ingested acetaminophen intermittently. Glomerular function has remained normal and hypertension has not developed, but management of these children has necessitated reduction or elimination of NSAIDs. As evidenced by these five cases, microscopic or gross hematuria in a child with chronic arthritis treated with anti-inflammatory drugs should raise the possibility of RPN.
Explore the source record for details and available documents.
The use of intraarticular triamcinolone hexacetonide in the management of persistent arthritis of the knee joint that is unresponsive to nonsteroidal anti-inflammatory drugs was prospectively evaluated in 40 children with chronic arthritis. Of 49 knees that were injected, 63.3% maintained complete resolution of effusion and other signs of inflammation at the 6-month followup. This favorable outcome correlated with a young age, a short disease duration, and a higher dose of triamcinolone hexacetonide. At the 12-month followup, 45% of the injected knees remained in remission.
Explore the source record for details and available documents.
Eleven children with reflex neurovascular dystrophy were investigated by technetium-labeled methylene diphosphonate bone scanning. Eight of 12 scans demonstrated abnormal findings, four showing diffusely decreased uptake and four diffusely increased uptake of the radionuclide in the affected site. Three scans showed normal findings initially, as did one previously abnormal scan when repeated in the asymptomatic patient 6 months later. Diffusely abnormal findings can be helpful in the diagnosis of childhood reflex neurovascular dystrophy, but a normal scan does not exclude the diagnosis.