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Biomedical subjects

D A Person

Publications and source records attributed to D A Person.

At least 37 records · Page 2Linked to original sources
PubMed1992

A unique case of reducing body myopathy.

The clinical, electromyographic, and histologic characteristics of a 17-year-old girl with reducing body myopathy are described. She is, to our knowledge, the oldest reported case and the only patient described with severe mitral valve prolapse and scoliosis. Electromyography demonstrated spontaneous positive sharp waves and fibrillation potentials with many low-amplitude, short, polyphasic motor unit potentials. The right deltoid muscle was characterized by focal areas with large fibers associated with increased endomysial connective tissue and "split" fibers. Purple-gray sarcoplasmic masses stained with trichrome were PAS-negative, appeared as "empty" spaces with both ATPase and NADH-TR, and stained darkly with menadione NBT. The features described expand the clinical presentation of this myopathy, and may lead to a better understanding of its etiology.

Adolescent↗
PubMed1991

Auranofin therapy for juvenile rheumatoid arthritis: results of the five-year open label extension trial.

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.

Administration, Oral↗
PubMed1990

Ibuprofen suspension in the treatment of juvenile rheumatoid arthritis. Pediatric Rheumatology Collaborative Study Group.

Ninety-two children with juvenile rheumatoid arthritis were randomly assigned to treatment in a multicenter, double-blind, 12-week trial designed to compare the efficacy and safety of a liquid formulation of ibuprofen at a dosage of 30 to 40 mg/kg/day versus those of aspirin at a dosage of 60 to 80 mg/kg/day. No significant intergroup differences in response rates or in the amount of improvement in articular indexes of disease activity were observed. More children treated with aspirin discontinued treatment early because of adverse reactions. After this trial, 84 additional patients with juvenile rheumatoid arthritis entered a 24-week, multidose (30, 40, and 50 mg/kg/day), open trial of ibuprofen suspension. Favorable response rates for the three groups were similar, and continued improvement was observed throughout the 24-week period. A dose-response relationship was observed with respect to adverse reactions of the upper gastrointestinal tract. We conclude that ibuprofen suspension is an effective nonsteroidal antiinflammatory drug and that its tolerability in children is acceptable.

Adolescent↗
PubMed1988

Prognosis of children with poststreptococcal reactive arthritis.

Patients with Group A beta-hemolytic streptococcal infection and articular disease who do not fulfill the modified Jones criteria for a diagnosis of acute rheumatic fever (ARF) have been classified as poststreptococcal reactive arthritis/arthralgia. We reviewed the initial clinical characteristics and outcome of 12 poststreptococcal reactive arthritis/arthralgia patients. During the initial episode all had arthritis or arthralgia and a documented streptococcal infection. None had carditis and none received prophylactic antibiotic therapy during an average follow-up of 17 months (range, 6 to 42 months). One patient developed classic ARF with valvulitis 18 months after the initial episode. Two children had later episodes of arthritis and two had at least one additional episode of arthralgia. Poststreptococcal reactive arthritis/arthralgias seems to be part of the disease spectrum of ARF and therefore the use of prophylactic antibiotic therapy to prevent subsequent development of ARF and carditis in these patients should, perhaps, be reconsidered.

Adolescent↗
PubMed1986

Recurrent Kawasaki disease.

This case report describes a boy who had Kawasaki disease (KD) at age 12 months and had a recurrence one year later. The coronary arteries were normal following the initial episode; however, during the second episode he developed coronary aneurysms. Gallium-67 radionuclide imaging, echocardiography, and angiography were used to diagnose the coronary abnormalities.

Aneurysm↗
PubMed1986

Sjögren's syndrome in children.

Sjögren's syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration of the lacrimal and salivary glands; it eventually leads to keratoconjunctivitis sicca and xerostomia. The disease usually affects middle-aged women and is second to rheumatoid arthritis as the most commonly diagnosed connective tissue disorder. Surprisingly, only 31 cases have been reported in the pediatric population. The diagnosis should be considered, however, in children with a foreign body sensation in the eyes, chronic dryness of the eyes or mouth, or recurrent salivary gland enlargement. This has prompted us to describe our experience in treating five children with SS at Texas Children's Hospital, Houston. One 5-year-old child with no other evidence of connective tissue disease was diagnosed as having primary SS. The remaining four children either had juvenile rheumatoid arthritis or systemic lupus erythematosus in addition to SS, and the diagnosis of secondary SS was made. Other autoimmune disorders associated with SS include polyarteritis nodosa, scleroderma, and polymyositis. Interestingly, one child with secondary SS and chronic lymphocytic thyroiditis developed papillary carcinoma of the thyroid. Both forms of SS are probably more prevalent than the literature would suggest. Recognition of this fact is important if the immediate complications of corneal damage and severe dental decay are to be prevented. Symptomatic treatment with steroids, eyedrops, artificial saliva, and prophylactic fluoride treatments is available. With an adequate follow-up program the late morbidity associated with SS, such as malignant disease, can be minimized.

Adolescent↗
PubMed1986

Longterm auranofin therapy in patients with juvenile rheumatoid arthritis.

Auranofin (AF, Ridaura) was administered to 23 children with juvenile rheumatoid arthritis during a prospective, open labelled, noncontrolled trial designed to establish longterm safety and preliminary efficacy. Dosages of AF were up to 0.2 mg/kg/day, with either aspirin (60-80 mg/kg/day), naproxen (400-600 mg/m2/day), or tolmetin sodium (20-40 mg/kg/day) serving as the concurrent nonsteroidal antiinflammatory drug. Nearly all patients showed an initial favorable response, however tachyphylaxis occurred in one-third (mean duration of therapy prior to the development of inefficacy = 22.6 mo). Clinical remission was observed in 6 patients an average of 15 months after enrollment. The drug appears to be safe for extended periods; 7 children are continuing AF at the present time with a mean duration of therapy of 4.25 years (maximum followup = 4.6 years).

Adolescent↗
PubMed1985

Hypermobility of the joints in juvenile episodic arthritis/arthralgia.

It has been suggested that hypermobility of the joints may predispose children to the development of arthritis or arthralgia. To determine the normal frequency of hypermobility, 260 normal schoolchildren (5 to 17 years of age) were examined. In addition, 34 patients with juvenile rheumatoid arthritis (JRA) and 32 children with juvenile episodic arthritis/arthralgia (JEA) were tested. Any child who met at least three of the following criteria was considered to have joint hypermobility: (1) passive apposition of the thumbs to the flexor aspect of the forearms; (2) passive hyperextension of the fingers so that they lie parallel with the extensor aspect of the forearms; (3) hyperextension of the elbows greater than 10 degrees; (4) hyperextension of the knees greater than 10 degrees; (5) flexion of the trunk with knees extended so the palms rest on the floor. Thirty-two (12%) of 260 normal schoolchildren and 21 (66%) of 32 with JEA had hypermobility. Further, a significantly higher proportion (23 of 126) of normal girls than normal boys (nine of 134) had hypermobility (chi 2 = 8.0, P less than 0.005). Hypermobility was not common in children with JRA. These findings support the hypothesis that hypermobility may be an important factor in the cause of JEA.

Adolescent↗
PubMed1984

Blood gold concentrations in children with juvenile rheumatoid arthritis undergoing long-term oral gold therapy.

During an uncontrolled, open-labelled, open-ended clinical trial of auranofin in children with juvenile rheumatoid arthritis (JRA) we obtained serial blood samples for the purpose of assessing gold content. Our objectives were (1) to observe the pattern of blood gold concentrations over a period of time in children undergoing long-term oral gold therapy, and (2) to observe the effect of changing dosage levels on blood gold concentrations. The initial dosage of auranofin was 0.1 mg/kg/day with allowable increases to 0.2 mg/kg/day. A concurrent nonsteroidal anti-inflammatory drug was allowed. Twenty-one patients were enrolled in the study, and we obtained 2 or more serial samples on 13 of the children. At a constant dosage of 0.1 mg/kg/day, steady state blood gold concentrations were attained in 11 to 13 weeks of therapy and, in the absence of a dosage change, remained remarkably constant through extended periods. The blood gold concentration was related to total daily dosage rather than to the cumulative amount of gold received. Increasing or decreasing the dose resulted in a direct effect on concentration. The clinical value of blood gold levels resulting from auranofin therapy in JRA will have to be established through double-blind controlled trials.

Adolescent↗
PubMed1983

Early experiences with auranofin in juvenile rheumatoid arthritis.

Pharmacologic management of juvenile rheumatoid arthritis is only one of several modalities necessary for effective control. The stepping stones to proper management include a planned long-range program, physical therapy with swimming, good health habits, and consultation with other health professionals who are part of the management team. Pharmacologic therapy includes nonsteroidal anti-inflammatory drugs initially, occasionally corticosteroids, and slow-acting antirheumatic drugs, including injectable gold when therapeutic response is inadequate. Early experiences with oral gold are reported here. Auranofin (triethylphosphine gold) was administered to 21 patients with juvenile rheumatoid arthritis during a segment I, open ended, open-label, noncontrolled trial designed to establish safety and preliminary efficacy. Initial dosage was 0.1 mg/kg per day; incremental increases to 0.2 mg/kg per day were allowed (with usual increase to 0.15 mg/kg per day). Aspirin (80 mg/kg per day) or tolmetin (20 to 40 mg/kg per day), or naproxen (400 to 600 mg/m2 per day) were allowed as rapidly acting antiinflammatory agents. Stable measurable plasma concentrations of gold were attained in all patients during the study. More than half the patients sustained clinically significant improvement (greater than 25 percent) with regard to the number and severity of joints with swelling, pain on motion, and tenderness. In nine of the 19 patients, the total number of joints with active arthritis decreased by at least 25 percent. All articular disease indices measured indicated improvement of group mean changes between the initial and final visit. Eleven of 16 patients with an elevated erythrocyte sedimentation rate showed decreases of at least 25 percent. The group given higher dosages had a greater proportion of responders in regard to decreases in erythrocyte sedimentation rate (nine of 11 patients). Four of six patients whose serums contained rheumatoid factor showed decreases in the titers. Discontinuation of auranofin was necessary in two patients: one because of headache and one because of hematuria and anemia associated with a severe flare-up of polyarticular disease. The results from this trial reveal sufficient patient improvement to plan a double-blind trial of auranofin in children with juvenile rheumatoid arthritis.

Administration, Oral↗
PubMed1983

Auranofin in the treatment of juvenile rheumatoid arthritis.

Auranofin (triethylphosphine gold) was administered to 21 patients with juvenile rheumatoid arthritis during an open-ended, open-label, noncontrolled trial designed to establish safety and preliminary efficacy. Initial dosage was 0.1 mg/kg/day; incremental increases to 0.2 mg/kg/day were allowed. Aspirin (80 mg/kg/day), tolmetin (20 to 40 mg/kg/day), and naproxen (400 to 600 mg/m2/day) were allowed as rapidly acting anti-inflammatory agents. All patients attained measurable plasma concentrations of gold during the study. Clinically significant improvement (greater than 25%) occurred in more than half the patients with regard to the number and severity of joints with swelling, pain on motion, and tenderness. The number of joints with active arthritis decreased by at least 25% in nine of the 19 patients. Group mean changes between the initial and final visit indicated improvement in all articular disease indices measured. Eleven of 16 patients with an elevated erythrocyte sedimentation rate showed decreases of at least 25%. The group given the higher dosage had a greater proportion of responders with decreases in erythrocyte sedimentation rate. Four of six patients whose sera contained rheumatoid factor showed decreases in its titer. Discontinuation of auranofin was necessary in two patients because of headaches and because of hematuria and anemia associated with a severe flare of polyarticular disease, respectively. The results from this trial are sufficiently encouraging to merit a double-blind trial of auranofin in children with juvenile rheumatoid arthritis.

Adolescent↗
PubMed1983

Blood and serum concentrations of gold after a single dose of auranofin in children with juvenile rheumatoid arthritis.

Three children with juvenile rheumatoid arthritis were given a single oral dose of approximately 0.1 mg/kg of auranofin. Blood concentrations up to 49 ng/ml were detectable at 24 h (earliest sample) and remained detectable for at least 2 weeks (final sample). Disappearance of measurable gold from both the blood and serum appeared to be linear through time. None of the patients experienced significant laboratory abnormalities nor adverse reactions. Gold levels attained in these children were somewhat lower than those seen in adults administered an equivalent dose.

Administration, Oral↗