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Biomedical subjects

C L Christian

Publications and source records attributed to C L Christian.

At least 19 recordsLinked to original sources

Patients with systemic lupus erythematosus at risk for Pneumocystis carinii pneumonia.

We describe 6 cases of patients with systemic lupus erythematosus (SLE) who developed Pneumocystis carinii pneumonia. All were treated with high dose corticosteroids, and all developed the infection within 4 months of beginning new or revised cytotoxic therapy. All patients tested (5 of 6) were negative for human immunodeficiency virus (HIV). Those patients who developed Pneumocystis carinii pneumonia had more severe lymphocytopenia (median 595 vs 833/mm3) and received higher doses of corticosteroids (median prednisone dose = 43 vs 20 mg/day) than other patients with active SLE. A threshold lymphocyte count of 350/mm3 identified 4 of 6 cases but only 1 of 20 controls. Patients with SLE treated with high dose corticosteroids and cytotoxic drugs and with severe lymphocytopenia may be at increased risk for this opportunistic infection.

Adult

Radiological findings in Hallermann-Streiff syndrome: report of five cases and a review of the literature.

Hallermann-Streiff syndrome (HSS) is a rare disorder with an associated constellation of radiological findings that may aid in the diagnosis of affected individuals. We reviewed the skeletal surveys of 5 affected individuals and noted some characteristic and constant findings. Radiological findings can include a large, poorly ossified skull with decreased ossification in the sutural areas. There was an increase in the number of Wormian bones. Severe mid-facial hypoplasia was present along with a prominent nasal bone. The skull films also showed an abnormally obtuse or nearly straight gonial angle. The teeth appeared small. The long bones were thin and gracile in appearance and often showed poor demarcation of the cortex from the medullary portion. Abnormal bowing of the radius and ulna was seen neonatally in 2 cases. There was widening at the metaphyseal ends of the long bones. The ribs were thin, but normal in length. The vertebral bodies were noted to be small and 3 cases had platyspondyly. There was a decreased number of sternal ossification enters. The metacarpals were also thin and gracile in appearance with metaphyseal widening. We conclude that these characteristic radiological findings in the newborn with HSS can aid in the diagnosis, and a skeletal survey in suspected individuals may be valuable in confirming the diagnosis.

Adolescent

Epidemiology of childhood IDDM in U.S. Virgin Islands from 1979 to 1988. Evidence of an epidemic in early 1980s and variation by degree of racial admixture.

OBJECTIVE: The epidemiology of insulin-dependent diabetes mellitus (IDDM) was evaluated in a predominantly black population in the U.S. Virgin Islands. RESEARCH DESIGN AND METHODS: Primary ascertainment of diabetic subjects was by retrospective review of hospital and clinic records, and IDDM was defined by Diabetes Epidemiology Research International Group criteria. RESULTS: For the period 1979-1988, 28 children less than 15 yr of age were diagnosed with IDDM resulting in an average annual IDDM incidence rate (IR) of 7.5/100,000 (95% confidence interval 4.7-10.3). A significant increase in IDDM incidence (P less than 0.01) was observed when the IR rose to 28.4/100,000 in 1984. White children had the highest IR (28.9/100,000). The IR for Hispanics (7.2/100,000) was slightly higher than that for blacks (5.9/100,000). Among black children, a slight but nonsignificant male excess in incidence was observed (male-female ratio 1.5). When black or Hispanic patients were compared with age-matched control subjects with respect to grandparental race, the diabetic subjects had a greater percentage of white ancestry (P less than 0.02 and P less than 0.05, respectively). The incidence of IDDM in Caribbean blacks (West Indians) in the U.S. Virgin Islands was similar to blacks in the U.S. CONCLUSIONS: The epidemic of IDDM in 1984 provides support for a possible pandemic in the early 1980s.

Adolescent

Development of a registry for diabetes in persons 0-19 years of age in the U.S. Virgin Islands.

A juvenile-onset diabetes registry was recently established in the United States Virgin Islands. Hospital and clinic records were used as the primary sources of case data, while data procured through a physician survey and mass media campaign were used to assess the completeness and validity of the registry listings. The data indicated that 39 people 0-19 years old developed diabetes during the study period (1 January 1979-31 December 1988), of whom 36 probably had Type 1 diabetes. Completeness of the primary source data was estimated at 92.3% and validity seemed very good (around 100%). Evaluation of these registry data has provided new insights into the epidemiology of Type 1 diabetes in the U.S. Virgin Islands.

Adolescent

Prenatal diagnosis of cystic fibrosis.

Since 1983, prenatal diagnosis for CF has been available. The indirect method of microvillar intestinal enzyme analysis was first used, followed by DNA linkage studies by RFLPs, and then aided by disequilibrium data. The past year has brought new promise with the identification of the CF gene and of a single amino acid deletion that is present in 70% of the gene mutations. In the next several years, it is hoped that the remaining mutations will be found, thus increasing the accuracy of prenatal diagnosis by direct detection of the mutations and allowing for carrier testing of the general population.

Chromosomes, Human, Pair 7

Uncommon illness from common infections.

Individual common infectious agents such as EBV, CMV and HBV have well characterized relationships to a variety of acute and chronic disease states; the host and/or environment variables underlying this clinical heterogeneity are not known. Given the high frequency of these viral infections in the general population, there should be a cautious interpretation of antiviral serological data.

Antigens, Viral

Differences among immune complexes: association of C1q in SLE immune complexes with renal disease.

Studies that made use of multiple assay systems demonstrated increased levels of immune complexes (IC) in patients with systemic lupus erythematosus (SLE), but no consistent correlations of IC concentration to patterns or activity of disease have been observed. Furthermore, consistent associations between qualitative differences in IC and disease manifestations have been elusive. IC interaction with erythrocytes and mononuclear phagocytic cells is another variable in SLE that may also mediate some of the biological effects of IC. The present report concerns studies of the composition of purified IC obtained from individuals with SLE and other rheumatic diseases; a 64,000 dalton component identified as the A-B subunit of C1q was detected in purified IC from 27 of 51 SLE patients (53%). The presence of this 64,000 dalton component was not related to either IC concentration or to the serum C1q level. However, the presence of the C1q component in isolated SLE IC did correlate with the presence of renal disease (p less than 0.02). These observations are interpreted relative to a recently described kinetic model of IC clearance.

Antigen-Antibody Complex

Clinical and microbial features of prosthetic joint infection.

A one-year experience with prosthetic joint infection, in which 63 cases were identified, is reviewed. Thirty cases (48 percent) were early infections, in the first postoperative year, and 33 cases (52 percent) were late, occurring more than one year after implantation. Pain was the predominant symptom, but clinical clues suggesting infection were frequently absent, with fever in 43 percent and leukocytosis in only 10 percent. The radiographic appearance was more frequently abnormal in late infections (67 versus 37 percent, p less than 0.02). Staphylococci were predominant organisms, constituting 59 percent of prosthetic joint infections, and S. epidermidis was the predominant species in both early and later infections. Of the hematogenous infections, 11 of 13 occurred in the group with late infections; these were mostly nonstaphylococcal . Antigenic proteins of S. epidermidis were characterized by gel electrophoresis, but no infection-specific antigens could be identified when patient serum was compared with normal samples. Precipitating antibodies to the extracellular proteins of S. epidermidis were present in 50 percent of patients with S. epidermidis prosthetic joint infections, 27 percent of patients with nonstaphylococcal infections, 20 percent of patients with S. aureus infections, and 11 percent of normal subjects. In view of the increasing importance of prosthetic joint infection, further study of the pathogenesis of the infection and the host immune response is warranted.

Adult

Characterization of sequential immune complexes in infective endocarditis by Western blot analysis.

A patient with cutaneous vasculitis during infective endocarditis due to Lactobacillus casei was studied. Immune complexes (IC) were isolated from serum at the time of diagnosis and after 4 wk of therapy. Purification of IC used differential polyethylene glycol precipitation and competitive binding to staphylococcal protein A. In situ radioiodination of IC was performed, followed by SDS-polyacrylamide gel electrophoresis (PAGE). Anti-IC antisera were raised in rabbits by immunization with purified IC. IC were characterized by SDS-PAGE followed by electrophoretic transfer to nitrocellulose, incubation with antiserum and then with 125I protein A, and autoradiography. Although early and late IC differed quantitatively, there were no differentiating immunochemical features. Both IC contained a 60,000 dalton component that did not react with preimmune serum nor with anti-normal human serum. This component reacted with antiserum rendered specific for L. casei by affinity chromatography. The restricted antigen-antibody representation in IC contrasted with a wider panel of antibody activity in patient serum. The Western blot analysis proves to be an ideal method for the characterization of IC because of its sensitivity, dissociative capability, and preservation of immunoreactivity. IC isolated at a time removed from the original antigenic challenge may provide insight into the nature of the inciting antigen.

Animals

Induction of polymeric IgA rheumatoid factors in infective endocarditis.

IgA and IgM rheumatoid factor activity, circulating immune complex levels, and antinuclear antibodies were measured in 17 patients with subacute or chronic infective endocarditis. Approximately three fourths of these patients had both IgA and IgM rheumatoid factors detectable by radioimmunoassay, and IgA and IgM rheumatoid factor activity was strongly correlated (p less than 0.01). In three patients studied, IgA rheumatoid factor activity was predominantly polymeric as assessed by sucrose density ultracentrifugation (55 to 92 percent of total rheumatoid factor activity) and could bind radiolabeled secretory component. No correlations between rheumatoid factor activity and circulating immune complex levels or antibodies to nuclear antigens were observed. These observations indicate that circulating polymeric IgA antibodies do not necessarily signify a mucosal stimulus for IgA production and also demonstrate differences in the intensity and spectrum of autoantibody production when compared with autoimmune diseases.

Adult

Prognostic and therapeutic implications of immunologic test results in rheumatic disease.

In systemic lupus erythematosus, some immunologic tests quantify disease activity, enable prediction of higher risk patterns of disease, and serve as therapeutic guides. In rheumatoid arthritis, serologic phenomena are significantly associated with more severe and sustained joint disease and with visceral manifestations (vasculitides and granulomas), but they have not been useful in monitoring the course of disease or response to therapy. In other rheumatic syndromes, a variety of antinuclear specificities show promise as marker antibodies, but their prognostic and therapeutic implications remain largely unexplored.

Arthritis, Rheumatoid

Serial circulating immune complexes and mononuclear phagocyte system function in infective endocarditis.

Twenty patients with infective endocarditis were followed prospectively and all had elevated levels of circulating immune complexes (CICs) detected by staphylococcal binding assay. Mean CIC levels declined for the group as a whole (193 micrograms/ml +/- 24 to 100 +/- 17, p less than 0.05) and became undetectable in eight patients (47%) who were cured. Patients who died or had complicated courses had higher mean CIC levels at the start and finish (254 micrograms/ml +/- 24 and 145 +/- 37) of antibiotic therapy than patients with uncomplicated courses (178 micrograms/ml +/- 19 and 38 +/- 24), p less than 0.05. CIC levels did not decline significantly in patients with glomerulonephritis or arthritis, in contrast to patients without these features. Despite elevated CIC levels, 10 patients had enhanced mononuclear phagocyte system (MPS) function as assessed by Fc-dependent IgG-coated red blood cell clearance. These data suggest that CICs probably are pathogenic in endocarditis and may contribute to the development of arthritis and glomerulonephritis. Elevated CICs in infective endocarditis do not appear to be directly related to defective MPS function.

Adult

Variable response to oral angiotensin-converting-enzyme blockade in hypertensive scleroderma patients.

Twelve scleroderma patients with hypertension, seven of whom had malignant hypertension and renal failure of scleroderma renal crisis, were treated with captopril. The first dose lowered mean pressure in all patients by 21.3 mmHg; in 6 patients it relieved encephalopathy. Blood pressure was controlled in all patients. Two of 7 patients with scleroderma renal crisis had improvement in renal function; the 5 patients who did not have malignant hypertension improved or stabilized. Despite good pressure control, however, renal failure developed in 5 patients with scleroderma renal crisis. The data indicated that captopril is effective antihypertensive therapy in scleroderma and, when given early, may prevent renal failure and death.

Administration, Oral

Identification of bacterial antigens in circulating immune complexes of infective endocarditis.

The presence of circulating immune complexes (IC) in patients with infective endocarditis has been well documented but the contributions of host and bacterial components to these IC have not been defined. To study this question, IC were isolated from serum of a patient with Streptococcus faecalis endocarditis by differential polyethylene glycol precipitation and competitive binding to staphylococcal protein A. A rabbit antiserum raised against the purified IC had reactivity by crossed immunoelectrophoresis primarily with an antigen derived from the cytoplasm of the infective organism. The antigen was a protein with a 12,000-dalton molecular mass. In situ radiolabeling of the IC bound to the protein A demonstrated a component of the same molecular mass as the bacterial antigen recognized by the antiserum. The patient serum had multiple antibody specificities reactive with bacterial antigens, including the antigen recognized by the rabbit anti-IC antiserum. These techniques for isolation and characterization of circulating IC may have value in the study of IC diseases in which the inciting antigens are not known.

Animals