Virgil Hanson

The Varni/Thompson Pediatrie Pain Questionnaire. I. Chronic musculoskeletal pain in juvenile rheumatoid arthritis

&NA; The Varm/Thompson pediatrie pain questionnaire (PPQ) represents an attempt to empirically assess the complexities of pediatrie chronic, recurrent pain. This initial investigation targeted chronic musculoskeletal pain in children with juvenile rheumatoid arthritis. The PPQ provides a developmental step toward the comprehensive assessment of the pain experience in children with chronic pain. Further reliability and validity studies are needed to determine the generalizability of the PPQ with larger numbers of children with a variety of acute and chronic pain experiences across a diversity of settings.

Reflex neurovascular dystrophy in childhood

Reflex neurovascular dystrophy has rarely been recognized in children. During the past eight years we have observed 24 instances of RND in 23 children. Lower extremity involvement was manifested in 20 of them and upper extremity in four. The major complaint was pain; swelling and vasomotor instability were prominent, and exquisite tenderness was characteristic. Chronic trophic changes were not observed. Antecedent illness or trauma could be related to the RND in less than half of the children, but personality factors appeared contributory to the development of RND in most children. Physical therapy was the principal form of treatment; therapy with a corticosteroid or by sympathetic blockade was not employed. Reduction in the evidences of disease, including improvement in function, were present in all children at the termination of therapy; improvement was maintained in all but one child after a mean period of 2.4 years. The excellent response to conservative therapy suggests that RND may be a more benign condition in children than in adults.

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.

Course of treated juvenile dermatomyositis

Sixty-six patients with possible juvenile dermatomyositis (JDMS) were observed at the Childrens Hospital of Los Angeles from 1960 to 1982. In patients initially given high doses of corticosteroids followed by low-dose therapy, three different clinical courses had previously been observed: monocyclic, polycyclic, and chronic continuous. We reviewed the records of 32 patients who met study criteria. The course of JDMS was monocyclic in eight children, chronic polycyclic in 10, and chronic continuous in 14. Of these children, 25 are well and not receiving medication; one has mild JDMS, without corticosteroid therapy; four have active JDMS despite corticosteroid therapy (one is severely handicapped); and two have died. Our results support the improved prognosis of JDMS after corticosteroid therapy, but also the great clinical variability of the disease. Understanding of this variability, as reflected in the three disease courses, facilitates physician choice of the optimal treatment with the least drug toxicity for the individual patient, continuing efforts to clarify the disease pathogenesis, and research efforts to improve current treatment programs for the patient with severe JDMS.

Cardiac involvement in juvenile rheumatoid arthritis

Fifty-five patients with JRA were studied by echocardiography, electrocardiography, and chest radiography. Systolic time intervals were determined in 40 of these children. Twenty (36%) had echocardiographic evidence of pericarditis. This was most common in patients with the acute systemic form of the disease and was associated with anemia, leukocytosis, and elevated ESR.

A histologic evaluation of mixed connective tissue disease in childhood

Abstract Mixed connective tissue disease (MCTD) includes features of scleroderma, dermatomyositis and systemic lupus erythematosus (SLE), and has speckled antinuclear antibodies (ANA) and high titers of anti-RNP antibodies. There are no comprehensive investigations of its histopathology. We have followed 15 children with MCTD, of whom four have died (mean disease duration prior to death 5.4 years). The immediate causes of death were pneumococcal sepsis (two patients), meningococcal sepsis (one patient) and uncontrollable thrombocytopenia (one patient). Material from the three available autopsies and five renal biopsies was reviewed. The most prominent histopathologic feature was widespread proliferative vascular lesions including intimai vascular change in 31 of 58 organs (53 per cent) and medial vessel wall thickening in nine organs (16 per cent). Systemic hypertension was absent; the normal vascular responses to aging could be excluded. Inflammatory infiltrates, often with prominent plasmacytosis, were present in 26 of 58 organs (45 per cent), but fibrinoid vascular change (9 per cent) and fibrosis (14 per cent) were rare. Eight renal specimens all showed some degree of glomerulonephritis; membranous change was present in three, and six showed significant vascular sclerosis. The histopathology of MCTD is superficially similar to systemic sclerosis, but it may be distinguished by less frequent fibrosis, the frequency of organs with intimal vascular change, and a predilection for intimai thickening of large arteries including coronary, pulmonary, renal and aortic. A distinctive replacement of muscle layers by hyaline in the gastrointestinal tract, and an unusual nodular hyperplasia of the thymic medulla were also observed and may be unique features of MCTD. The findings from this study suggest an immunologic basis for MCTD different from those postulated for other rheumatic diseases, and strongly suggest that adjustment of morbidity and mortality expectations for MCTD are necessary.

Original articleMixed 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.

Chronic musculoskeletal pain and functional status in juvenile rheumatoid arthritis: an empirical model☆

&NA; An empirical model is proposed and tested on variables hypothesized to influence functional status in 23 children with juvenile rheumatoid arthritis experiencing chronic musculoskeletal pain. Child psychological adjustment, family psychosocial environment, chronic musculoskeletal pain, and disease activity were entered into multiple regression analyses to statistically predict 4 functional status criterion variables: activities of daily living (ADL), activities involvement, school functioning, and social functioning. Predictor variable relationships were found for all 4 functional status criterion variables, suggesting initial support for this empirical model of functional status in children with juvenile rheumatoid arthritis experiencing chronic musculoskeletal pain.

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.

Nailfold capillary abnormalities in childhood rheumatic diseases

The nailfold capillary patterns of 84 patients with a variety of childhood rheumatic diseases and 34 normal control subjects were observed. Distinctive morphologic abnormalities with capillary dilation and dropout of surrounding structures were noted in two groups: patients with childhood dermatomyositis and with scleroderma (P less than 0.001). Among those with scleroderma, capillary abnormalities were found in all nine patients with systemic disease and in none of 10 patients with cutaneous disease only (Fishers exact P less than 0.001). Of 25 patients with dermatomyositis for whom muscle biopsies were available for analysis, abnormal nailfold capillary pattern was found with highest prevalence in patients with two or more specific vascular lesions noted on biopsy (Fishers exact P = 0.041). Nailfold capillary abnormalities are present in distinct populations of childhood rheumatic diseases, reflect the underlying vasculopathy of childhood dermatomyositis, and may be of diagnostic value in distinguishing localized from systemic scleroderma.