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Biomedical subjects

V Hanson

Publications and source records attributed to V Hanson.

At least 55 records · Page 3Linked to original sources

Mixed connective tissue disease in childhood. A clinical and serologic survey.

Mixed connective tissue disease is a syndrome with overlapping clinical features of SLE, scleroderma, and polymyositis. Only one other child with MCTD has been described in detail. In this study 14 children with MCTD are described. Each had overlapping clinical findings that evolved over an extended period of observation, and all 14 had high serum titers of speckled ANA and antibodies to RNP. A serologic survey of 127 children with various rheumatic diseases confirmed the specificity of high titer of speckled ANA and antibodies to RNP for MCTD in children. Significant cardiac and renal involvement, and thrombocytopenia, may be more common in affected children than in adults with MCTD, may lead to longer therapy with higher doses of a corticosteroid, and may contribute to a more serious prognosis than in adults.

Adolescent↗

Comparison of tolmetin sodium and aspirin in the treatment of juvenile rheumatoid arthritis.

The Pediatric Rheumatology Collaborative Study Group was established in 1973 to undertake systematic trials of new drugs in the treatment of juvenile rheumatoid arthritis. The first drug evaluated was tolmetin (1-methyl-5-p-toluoylpyrrole-2 acetic acid), a new nonsteroid anti-inflammatory agent. A four-week open trial with 30 patients and a subsequent 12-week double-blind trial against aspirin with 107 patients were conducted. Tolmetin and aspirin had equal anti-inflammatory and analgesic effects in the treatment of JRA. Elevations of transaminase values attributed to aspirin were not found with tolmetin. Adverse effects accompanying administration of tolmetin did not appear to be of major clinical significance.

Adolescent↗

Minoxidil therapy in children with severe hypertension.

Six children, from 1.3 to 18 years of age, with severe hypertension associated with the hemolytic uremic syndrome, periarteritis, and renal transplant rejection received minoxidil, an antihypertensive agent, for three to 36 weeks. All had severe hypertension resistant to oral antihypertensive medications; five required frequent intravenous diazoxide therapy prior to minoxidil therapy. The mean pretreatment systolic and diastolic blood pressures were 176 and 117 mm Hg, respectively. Following treatment, the mean systolic and diastolic blood pressures were 133 and 82 mm Hg, respectively. Concomitant antihypertensive medications were decreased in all six patients once optimal blood pressure control was obtained. The initial dosage of minoxidil was 0.1 to 0.2 mg/kg/day; maximal dosage for blood pressure was 0.3 to 1.4 mg/kh/day. Major complications of therapy were fluid retention and hirsutism. Transient asymptomatic pericardial effusions occurred in two patients. Three patients on prolonged minoxidil therapy had persistent increases in right ventricular end diastolic diameters. Minoxidil is an effective oral antihypertensive agent for treatment of severe hypertension in pediatric patients. Avoidance of fluid retention is mandatory to prevent congestive heart failure.

Adolescent↗

Prognosis of juvenile rheumatoid arthritis.

The true prognosis of JRA is unknown. The best interpretation of reports to this date may be that at any given time of examination between 5 and 15 years after onset, 30-50% of children will have grossly active disease and that 70-90% of patients will be in class I-II functional status. Published studies, however, are not comparable because of differing criteria and selection of support data to be reported. Close analysis of four cases of JRA illustrate some of the difficulties in utilizing loosely defined criteria. A preliminary plan for improving the precision of reporting course and prognosis of JRA has been outlined.

Arthritis, Juvenile↗

The clinical spectrum of systemic lupus erythematosus in childhood.

The onset and course of 108 children with systemic lupus erythematosus have been analysed. There were more black patients than one would expect from hospital population statistics. There was a greater preponderance of boys with onset of the disease at less than 12 years of age and there is a large number of familial cases. Major signs and symptoms differed from those observed in adults only in the greater degree of reticuloendothelial involvement and in a possibly greater propensity for children to change renal biopsy category. Diffuse proliferative renal lesions remain a major contributor to death both in children and in adults, but the importance of the extrarenal mortality factors plus the greater proportion of male deaths is emphasized.

Adolescent↗

Mixed connective tissue disease in children.

Seven girls and 3 boys with MCTD have been described. As a group their clinical characteristics and serological findings are similar to those reported in adults, with several important differences. Children with MCTD may have marked thrombocytopenia and more frequently they have RF. Significant cardiac and renal involvement are more common in children, may lead to longer and higher dose corticosteroid therapy, and may contribute to a less optimistic prognosis than that described in adults.

Adolescent↗

Reiter's syndrome in childhood.

Seven boys with Reiter's syndrome are described. Three had diarrhea and 2 had venereal contact as antecedent events. All developed the complete triad of symptoms in a 5- to 24-day period. Joint involvement of the lower extremities was seen in each boy. HLA-B27 typing was positive in 6 of 7 (86%). Serum and synovial fluid levels of CH50 and C3 were elevated in 5 boys and confirm similar findings in adults. Two boys recovered spontaneously without therapy and 3 boys received aspirin with a rapid and complete resolution of symptoms. The two oldest boys had the most severe joint involvement and were receiving phenylbutazone with continuing active arthritis when they were lost to followup. Reiter's syndrome in children may be infrequently reported because its antecedents and the triad symptoms are common occurrences in pediatric practice.

Adolescent↗

Childhood polymyositis with cardiac conduction defects.

Pediatric polymyositis may be an entity distinct from dermatomyositis. Chronic polymyositis can occur in childhood and, as in adults, may be associated with arrhythmias. Microscopical involvement of the myocardium and pericardium in dermatomyositis is probably far more common than suspected on clinical grounds. Cardiac evaluation is suggested for all children with dermatomyositis or polymyositis, particularly prior to surgical procedures. Intracardiac electrographic recording techniques offer improved accuracy in determining the site of cardiac conduction defects, and may aid in planning for the use of antiarrhythmia medications or a pacemaker. In children with dermatomyositis or polymyositis known cardiac stimulants should be administered with care.

Arrhythmias, Cardiac↗

Systemic lupus erythematosus in childhood correlations between changes in disease activity and serum complement levels.

Serial complement component (C3 and C4) determinations were performed in 26 children with systemic lupus erythematosus. Twenty-one children with SLE had 52 episodes of C3 depression (mean duration 25 weeks); only 11 of these children had active nephritis when serum concentrations of complement were depressed. Fourteen children had active rash associated with low C3; in seven of these children rash was the only clinical evidence of disease activity. Ten children had active CNS disease; in seven children the CNS involvement correlated with low C3. In general, variations in serum concentrations of C4 did not reflect changes in SLE activity which were not reflected by changes in serum concentrations of C3. Serum C4 occasionally remained depressed longer than C3, perhaps reflecting continuing subclinical disease activity. Increased C3 occurred in 18 of 26 children as doses of corticosteroid were increased, in six of 14 when cyclophosphamide was added, and in two children when hydroxychloroquine was added. Our findings suggest that a wide variety of manifestations of childhood SLE may produce hypocomplementemia. In addition to renal disease, variations in serum concentrations of C3 and C4 can reflect, or occasionally predict, changes in rash and CNS disease.

Adolescent↗

Antinuclear antibodies and lupus-like syndromes in children receiving anticonvulsants.

Drug-induced systemic lupus erythematosus (SLE)-like syndromes in children are most commonly associated with the administration of ethosuximide, diphenylhydantoin, and trimethadione. Five children receiving ethosuximide who presented with syndromes suggestive of SLE were studied. Each and fever, malar rash, arthritis, and lymphadenopathy. Two children had pleural effusions and another developed myocarditis and pericarditis. Three patients had anti-DNA antibodies associated with low serum C3. In four of five children symptoms disappeared with the discontinuation of ethosuximide; two of these continue to have antinuclear antibodies (ANA). One child continues to have active SLE with nephritis. A group of 101 children from a seizure clinic were tested for the presence of ANA. ANA were found in 14 of 70 children receiving ethosuximide and/or diphenylhydantoin; 2 of 14 had anti-DNA antibodies. Serum ANA titers in the drug-induced SLE group did not differ significantly from those of the asymptomatic seizure patients. ANA were also present in 5 of 23 children receiving phenobarbital only. The induction of ANA by phenobarbital is a possible hypothesis. Quantitative immunoglobulins and C3 were not significantly altered in the asymptomatic children with ANA. Follow-up studies at ten months showed no asymptomatic child with ANA to have developed clinical with ANA to have developed clinical evidence of SLE. This study suggests that asymptomatic children who develop ANA should have careful observation, but need not have their anticonvulsants discontinued.

Adolescent↗