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Biomedical subjects

L M Pachman

Publications and source records attributed to L M Pachman.

At least 55 records · Page 3Linked to original sources
PubMed1990

Juvenile dermatomyositis: a clinical overview.

The child who develops the symptoms of the specific rash, proximal muscle weakness, and fatigue should seek medical care promptly. With the advances in physical and medical therapy, many of the consequences of the disease can now be ameliorated. There are suggestive data that JDMS and PM may each have a different pathophysiology, but more evidence is needed. The next few years should be exciting as there is increased effort to determine if there is, in fact, a causal relationship between Coxsackievirus B or other enterovirus and genetic factors that alter the susceptibility to or severity of the course of the disease.

Child↗
PubMed1989

Validity of reported pain as a measure of clinical state in juvenile rheumatoid arthritis.

Reported pain is one valid indicator of clinical state which should be used in the assessment and management of children with juvenile rheumatoid arthritis. Parents' reports of children's pain for 101 children with juvenile rheumatoid arthritis differed significantly by type of juvenile rheumatoid arthritis. When multiple regression was used separately for pauciarticular and polyarticular classifications of juvenile rheumatoid arthritis a measure of clinical state, which reflected joint activity, morning stiffness, and overall disease activity as rated by the doctor, had a significant and independent effect upon pain reported by parents. The child's age was not significantly related to the pain reported by parents. Children's pain reports did not differ significantly between older and younger children.

Adolescent↗
PubMed1988

Cryoprecipitates in Kawasaki syndrome: association with coronary artery aneurysms.

Cryoprecipitates are postulated to play a role in the pathogenesis of several vasculitis illnesses and infectious diseases. To investigate the presence of cryoprecipitates in Kawasaki syndrome, we studied sera from 25 children with acute Kawasaki syndrome. None of the subjects was treated with intravenous gamma-globulin. Cryoprecipitates were detectable in sera of 11 of 25 (44%) children studied. The mean (+/- SE protein concentration of the cryoprecipitates was 88.0 (+/- 20.2) micrograms/ml serum. Cryoprecipitates consisted primarily of IgG and IgM; no complement components were detected but highly sensitive methods were not used. The presence of cryoprecipitates in the serum of children with acute Kawasaki syndrome was associated with the subsequent development of coronary artery aneurysms detected by echocardiogram (P less than 0.05). There was no association between detectable cryoprecipitates and either peak platelet count or erythrocyte sedimentation rate. In one patient, measurement of cryoprecipitates in serial samples showed a reduction in concentrations that paralleled subsidence of disease activity. We speculate that cryoprecipitates may be a marker for increased risk of coronary aneurysm formation and may play a role in the pathogenesis of the cardiac disease in Kawasaki syndrome.

Child↗
PubMed1988

Age related changes in the concentration of serum keratan sulphate in children.

Serum was obtained from 155 children at the time of admission to hospital for elective surgery. The concentration of serum keratan sulphate was determined by an ELISA which uses an antibody specific for keratan sulphate, a molecule found predominantly in cartilage. Concentrations of keratan sulphate rise progressively during the first four years of life (0-2: mean = 357 micrograms/l; 2-4: mean = 422 micrograms/l) and then remain high until 12 years of age (mean = approx. 500 micrograms/l). At this time, concentrations drop markedly (13-year olds: mean = 377 micrograms/l; 14-year olds: mean = 318 micrograms/l). After age 15, concentrations continue to fall toward the concentrations found in normal adults. Serum concentrations did not show significant differences with respect to disease category, sex or race but were found to vary, sometimes markedly, from child to child at any one age. The results suggest human cartilage undergoes significant changes in metabolic activities during maturation. Measurements of keratan sulphate concentration in serum may prove useful in studying the biochemical and physiological bases of these changes and in monitoring growth or endochondral ossification during maturation.

Adolescent↗
PubMed1988

Pediatric rheumatology: a survey of physician practices and fellowship programs.

We report the results of a 1982 pilot survey of pediatric rheumatology physicians and fellowship training programs in the United States. All physicians indicating in the ARA directory that they could care for rheumatic diseases of children were surveyed, with a response rate of 57.6%. There were 35 directors of United States pediatric rheumatology programs among the respondents. The survey provided basic information concerning training and practice characteristics of respondent physicians and the sources of funding, size, and emphasis of the fellowship programs. Specific attention is given to indicators of demand for pediatric rheumatology services.

Education, Medical, Graduate↗
PubMed1987

Risk of coronary abnormalities due to Kawasaki disease in urban area with small Asian population.

The epidemiology of Kawasaki disease in the six-county Chicago metropolitan area (total population, greater than 7,100,000 inhabitants) was characterized by identifying cases, with onset occurring from 1979 to 1983, inclusively, that had been studied by echocardiography. A retrospective survey of the records from pediatric echocardiographic laboratories and pediatric cardiologists at teaching hospitals, as well as a random sample survey of nonteaching hospitals with pediatric beds in the metropolitan area, was carried out. A total of 190 cases were identified, yielding an annual mean minimum incidence of 5.95 per 100,000 children less than 5 years old. Cases occurred endemically with superimposed spring clusters in 1980 and 1983. As seen in other studies, the male-female ratio was 1.58:1, and the peak incidence occurred in children between 1 and 2 years old, with 85% of cases occurring in children under 5 years of age. The racial distribution of cases was as follows: whites, including Hispanics, 62%; blacks, 32%; Asians, 5%; and half-white/half-Asian, 1%. Asians were slightly overrepresented in that they made up only 1.7% of the study area population. The annual minimum incidence for Asian Americans was 24.4 per 100,000 children less than 5 years old; this rate was significantly greater than those for the other racial groups. Although few cases were observed in Japanese-American children, the calculated annual minimum incidence in this small group was approximately 44 per 100,000 children less than 5 years old. The highest incidence was observed in several suburban Chicago zip code areas, where annual rates as high as 84.7 per 100,000 children less than 5 years old were documented. Coronary artery abnormalities were diagnosed by echocardiography in 30 (16%) of 190 cases; the male-female ratio of patients with such abnormalities was 2.75:1. Whites and children under 1 year of age demonstrated the highest incidence of coronary artery abnormalities. White children under 1 year of age appeared to be at particularly high risk for development of coronary abnormalities, with 11 (41%) of 27 white infants manifesting such findings by echocardiography. These infants may represent a subgroup of patients who would benefit particularly from therapy with intravenous gamma globulin for prevention of coronary abnormalities and who require particularly close follow-up care.

Adolescent↗
PubMed1987

Radiological approaches in the evaluation of joint disease in children.

In summary, the newer technologies in radiology have allowed us to visualize more clearly the manifestation of joint disease in children. The presence of small erosions and cartilage damage can be seen much better with magnetic resonance imaging than with any other modality short of arthrography, a much more invasive examination. Joint effusion, although sometimes visualized with conventional radiography, is probably best recognized with ultrasound or magnetic resonance imaging, although it can be detected with computed tomography as well. For the evaluation of avascular necrosis that can be associated with steroid use in joint disease, bone scintigraphy is a simple, sensitive method. Magnetic resonance may be as or more sensitive and gives additional information as well. In the detection of change with time, conventional radiography probably will remain the standard as it is still the simplest, least expensive examination; however, it has many limitations in specific cases. Bone scintigraphy may be of value in selected cases. Although we have still not had enough experience with magnetic resonance imaging to use it as a way of evaluating progress of joint disease, it promises to be the most sensitive radiologic measure of evaluating progress as small anatomical changes can be detected within the cartilage, which cannot be done easily with other means.

Arthritis, Juvenile↗
PubMed1986

Prevalence of Coxsackie B virus antibodies in patients with juvenile dermatomyositis.

A number of viruses have been implicated as being the cause of various forms of myositis, including acute transient myositis, chronic polymyositis, and dermatomyositis. However, the cause of juvenile dermatomyositis (JDM) has remained elusive. Our study of serum samples taken within 4 months of the onset of disease in 12 children with JDM showed that 83% had detectable titers of complement-fixing (CF) antibody to 1 or more coxsackie B viral antigens. Detectable titers were found in only 25% of age-, sex-, and date-matched control sera taken from 24 patients with juvenile rheumatoid arthritis (JRA), and in 25% of serum samples taken from 2,192 "normal" children who had been hospitalized because of viral syndromes. Titers of CF antibody to coxsackie B1, B2, and B4 were positive in 58%, 50%, and 58%, respectively, of the JDM patients. In matched JRA controls, the respective values were 8%, 13%, and 8%. There were no significant antiviral titers and no significant differences in the results of tests for 13 other viral CF antigens, hepatitis B surface antigen, and Mycoplasma pneumoniae CF antigen in the JDM patient sera compared with the JRA patient sera. When titers of neutralizing antibody were determined, 58%, 58%, and 67% of the JDM patients were positive for coxsackie B2, B4, and B5, respectively, whereas 16%, 26%, and 21%, respectively, of the JRA controls were positive for the 3 antigens. These data suggest that the host response to coxsackie B virus might be related to the pathophysiology of JDM.

Antibodies, Viral↗
PubMed1986

The early involvement of pulmonary prostaglandins in hyperoxic lung injury.

To further study the role of arachidonic acid metabolites in the development of hyperoxic lung injury and the function of PMNs and/or alveolar macrophages in facilitating this role, we exposed adult rabbits to greater than 95% O2 or air for 24, 40, 48, or 65 hours. At the end of each study, bronchoalveolar lavage [BAL] of the left lung was performed, and the right lung was inflated and fixed for light and electron microscopy. PGE2, 6-keto-PGF1 alpha and thromboxane B2 were measured by RIA in arterial and venous plasma at the beginning and end of each study and in BAL fluid obtained at sacrifice. Production of these three PGs by BAL cells placed in cell culture was also measured. Significant hyperoxic lung injury did not develop until 65 hours, as evidenced by significant increase in BAL total protein and percent PMNs, and by morphologic findings. At 40 hours, however, BAL fluid PGE2 and 6-keto-PGF1 alpha increased and BAL cell production of all 3 PGs was significantly increased (p less than .05). In summary, the early PG increases observed in these studies may directly contribute to the development of hyperoxic lung injury or, rather, may be representative of a generalized increase in all arachidonic acid metabolites, including the lipoxygenase pathway. The increase in BAL cell PG production and increased PG concentrations in BAL fluid prior to any increase in BAL PMNs suggest that the AM may be the source of the early arachidonic acid metabolite increase in response to hyperoxia.

6-Ketoprostaglandin F1 alpha↗
PubMed1986

Juvenile dermatomyositis.

Myositis in childhood is characterized by elevated serum levels of muscle-derived enzymes, proximal symmetrical muscle weakness, abnormal EMG findings, and a muscle biopsy, which frequently documents an inflammatory process. In the pediatric age group, JDMS, which has characteristic cutaneous involvement in addition to myositis, is much more common than PM and is more common among female patients. With the use of steroids, mortality has been reduced from 33 per cent to 7 per cent. The development of calcifications can be the most debilitating consequence of JDMS. It is our premise that JDMS is a distinct disease entity and that the increase in HLA-B8 and DR3 in JDMS suggests that genetic background may predispose to disease development. There are conflicting data concerning immunologic abnormalities in JDMS, but there appears to be impairment of natural killing and evidence of complement activation. Results of tests for ANA frequently are positive in JDMS, but Jo-1 antibody, found in some adults with PM, has not been found in JDMS. Most newly diagnosed JDMS patients have antibodies to coxsackie B that may be related to the pathogenesis of this disease. Specific pathologic findings of endothelial cells containing reticulotubular inclusions are associated with small vessel occlusion, subsequent obliteration, and increased factor VIII levels in clinically active disease. In addition to physical therapy, steroids are used most frequently, but other immunosuppressive agents and plasmapheresis have been tried in severely ill children. Rigorous evaluation of the efficacy of these modalities is needed.

Child↗
PubMed1985

Immunogenetic studies of juvenile dermatomyositis. III. Study of antibody to organ-specific and nuclear antigens.

Ninety children with definite juvenile dermatomyositis (JDMS), who had been HLA typed, were tested for the presence of tissue or organ-specific antibodies. Sixty had active disease at the time of study. The mean disease duration was 4 years, and 30 had soft tissue calcifications. The following autoantibodies were sought: thyroid, gastric parietal cells, smooth muscle, striated muscle, microsomes, mitochondria, DNA, extractable nuclear antigen, Sm, PM-1, antinuclear antibody (ANA), and rheumatoid factor. Only the ANA and PM-1 were more frequent in patients than in controls (P less than 0.0002 and P less than 0.001, respectively). Higher levels of immune complexes (P less than 0.01) were found in sera from patients with JDMS than in sera from controls and were correlated with the presence of ANA in patients (P less than 0.01). Soft tissue calcification was not associated with any autoantibody or HLA antigen, but with disease duration and activity (P less than 0.001 and P less than 0.05, respectively). There was no association between the occurrence of any autoantibody and the presence of HLA-B8 or DR3 among the white patients with JDMS. The frequency of autoantibodies in 43 full siblings of children with JDMS was not increased. We conclude that children with JDMS, with or without HLA-B8/DR3, do not show evidence of a generalized nonspecific antibody response to tissue antigens. The significance of the increased antibody to nuclear antigens ANA and PM-1 remains to be determined.

Adolescent↗
PubMed1985

Evidence for intravascular coagulation in systemic onset, but not polyarticular, juvenile rheumatoid arthritis.

After observing a child with systemic onset juvenile rheumatoid arthritis (S-JRA) who developed purpura fulminans in association with disseminated intravascular coagulation, with subsequent gangrene and autoamputation, we undertook a prospective study of coagulation parameters in children with JRA. Ten consecutive children with S-JRA, 10 children with rheumatoid factor-negative, polyarticular juvenile rheumatoid arthritis (P-JRA), and 10 age- and sex-matched controls were studied. Routine coagulation screening tests were performed, as were tests for plasma fibrinopeptide A (a sensitive measure of intravascular thrombin generation), factor VIII-related antigen (an endothelial cell protein), and platelet factor 4 (a platelet-secreted protein). Our studies suggest that activation of intravascular coagulation is common in systemic onset JRA, but not in rheumatoid factor-negative, polyarticular disease. The coagulopathy may cause severe morbidity. In addition, marked elevations of plasma factor VIII-related antigen suggest perturbation of endothelial cells and vascular involvement in S-JRA, but not in P-JRA. Normal ranges of platelet factor 4 indicate that intravascular platelet consumption does not occur in either type of JRA, despite the thrombocytosis common in both.

Adolescent↗