Comment on the article by Sheerin et al.
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Adolescent↗
Biomedical subjects
J Baum
Publications and source records attributed to J Baum.
Joint pain. It isn't always arthritis.
When a history of trauma or overuse is absent in a patient presenting with pain and stiffness in a joint, bursitis and tendinitis should be considered, especially if the patient is elderly. Appropriate questioning can narrow the differential diagnosis, and physical examination confirms the impression gathered by history taking. Treatment may include one or more injections of a local anesthetic (with or without a long-acting corticosteroid), use of an oral corticosteroid or nonsteroidal antiinflammatory agent, and in some cases, surgery.
Arthritis↗
Lectin receptors of normal and dystrophic corneas.
In recent years, our laboratory has used lectins to detect carbohydrate-related abnormalities in dystrophic corneas and species-specific differences in the carbohydrates of normal corneas. These studies have: 1) demonstrated that abnormal glycoconjugates are present in corneas with macular and lattice dystrophy, 2) provided additional means of differentiating corneas with macular, lattice and granular dystrophy, 3) demonstrated that distinct species-specific differences are present in the glycoconjugates of normal corneas and 4) provided guidelines for the selection of an animal model for the study of corneal glycoconjugates.
Animals↗
Altered keratan sulfate epitopes in keratoconus.
Extracts of normal and keratoconus human corneas were assayed for keratan sulfate (KS) and for the protein core of corneal keratan sulfate proteoglycan (KSPG), using a solid-phase immunoassay. Aliquots from guanidine-HCl extracts of four to six pooled corneas were adsorbed to nitrocellulose membrane and assayed for binding of a monoclonal antibody against KS or of an affinity-purified rabbit polyclonal antibody against the KSPG protein core using 125I-labeled secondary antibodies. The amount of antigen in the extracts was estimated from standard curves with purified bovine KSPG. Extracts from keratoconus corneas contained only an average of 48% as much (P less than 0.001) KS-antigen as normal corneal extracts when assayed with anti-KS monoclonal antibody. There was no significant difference in the amount of KSPG core protein antigens in keratoconus and normal corneal extracts. These results suggest that corneas with keratoconus contain a form of KSPG that contains fewer keratan sulfate chains or in which the keratan sulfate has a modified structure.
Antibodies, Monoclonal↗
Protein-related abnormalities in keratoconus.
Two-dimensional electrophoretic maps of extracts from eleven normal and eleven keratoconus corneas were compared. Of the eleven corneas analyzed, eight were pooled and the remaining three were analyzed individually. Several differences were demonstrated between electrophoretic patterns of normal and keratoconus corneas. In keratoconus corneas, 1) two abnormal components (MW 54kD and 26kD) were observed; 2) three normal corneal components (MW 12kD, 14kD, and 39kD) were present in significantly higher amounts; and 3) three normal corneal proteins (MW 66kD, 55kD, and 13kD) were present in reduced amounts. The molecular weight and isoelectric point of one of the normal corneal proteins that we found to be reduced in keratoconus corneas were close to that of a subunit of prolyl-4-hydroxylase, an enzyme required for hydroxylation of proline residues of collagen. The possibility the abnormal proteins detected in the keratoconus corneas were derived from those normal corneal proteins which were absent or were present in reduced amounts in the keratoconus corneas remains to be established. This study may provide protein markers for elucidation of the biochemical abnormality in keratoconus.
Adult↗
The frequency of juvenile arthritis.
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Arthritis, Juvenile↗
Dark skin discoloration of finger joints in juvenile arthritis.
We examined the frequency of a known but poorly described finding, darkened skin over the proximal interphalangeal joints in patients with polyarticular juvenile arthritis. Examining a consecutive group of patients we found that 77% showed this sign. It reflects the presence of or previous episodes of inflammation of these joints. The reason for this finding is unknown.
Adolescent↗
Erythrocyte sedimentation rate and C-reactive protein compared in the elderly.
The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) concentrations were studied in 101 elderly individuals (mean age 72 y) to determine their utility as diagnostic aids in subjects with underlying infection/inflammation. Whereas ESR and CRP were both significantly increased in patients with infection or inflammation, or both, analysis of variance indicated that those subjects still alive six months later had significantly lower ESR values. Analysis of sensitivity, specificity, and positive predictive values indicated that neither test satisfactorily discriminated between patients with and those without ongoing active or chronic disease. Receiver-operating characteristic curve analysis confirmed the low true-positive/false-positive ratios of both ESR and CRP. In the elderly, neither CRP nor ESR has distinct advantages over the other, and both tests evidently have limited utility.
Aged↗
Bilateral keratitis as a manifestation of Lyme disease.
An 11-year-old girl developed bilateral keratitis, which we believe was a manifestation of Lyme disease. She had had several attacks of Lyme arthritis and was twice treated with parenteral penicillin. The keratitis developed five years after the initial episode of Lyme arthritis at a time when there were no other manifestations of Lyme disease. It cleared completely in both eyes after topical corticosteroid therapy.
Child↗
Glycoconjugate abnormalities in cultured keratoconus stromal cells.
We examined the plasma membrane glycoconjugates of cultured corneal stromal cells derived from patients with keratoconus and healthy subjects. Using radiolabeled lectins as probes, we found that a majority of our keratoconus strains contained more binding sites for concanavalin A, Ricinus communis agglutinin I, and soybean agglutinin than did the controls. The number of binding sites in cultured keratoconus cells for radioactive peanut agglutinin, wheat germ agglutinin, and Griffonia simplicifolia agglutinin I, however, was similar to that found in normal controls. Using a second method with biotin-labeled lectins as probes, we confirmed that increased amounts of glycoconjugates would bind to concanavalin A and R communis agglutinin I in stromal cells of patients with keratoconus compared with those of the control subjects. This study suggests that plasma membranes of cells derived from some patients with keratoconus may contain elevated amounts of glycoconjugates with glucose and/or mannose, terminal galactose, and N-acetylgalactosamine residues.
Adolescent↗
A histochemical comparison of human corneal stromal glycoconjugates with eight other species. Distinct species-dictated differences in binding sites of Griffonia simplicifolia I.
Frozen sections of human, calf, rabbit, rat, cat, dog, goat, lamb, and hog corneas were stained with various lectins using an avidin-biotin-peroxidase complex to study glycoconjugates of stromal matrix. Staining of the stromal matrix and keratocytes with an alpha-galactose-specific lectin, Griffonia simplicifolia I (GSA-I) was species-dependent. The stromal matrices of cat, dog, and hog corneas invariably reacted intensely with this lectin, whereas those of the human, calf, rabbit, rat, and lamb did not react. A positive reaction with GSA-I could be abolished in each instance by preincubation of the sections with alpha-galactosidase. The stromal matrices and keratocytes of all nine species reacted positively with wheat germ agglutinin, concanavalin A, and Ricinus communis agglutinin but did not react with soybean agglutinin. Results of this study may help select an appropriate animal model for further investigate human corneal stromal glycoconjugates.
Animals↗
Plasmapheresis treatment in patients with severe atopic keratoconjunctivitis.
A 19-year-old woman with hyperimmunoglobulinemia E (the hyper-IgE syndrome [HIE]) and a 62-year-old man with atopic dermatitis experienced dramatic improvement in their chronic ocular symptoms and signs of atopic keratoconjunctivitis after plasmapheresis was instituted. Both patients had previously received topical and oral corticosteroids as well as topical cromolyn sodium without significant beneficial effect. The authors recommend a trial of plasmapheresis in selected patients with recalcitrant and debilitating atopic keratoconjunctivitis when standard therapy proves unsuccessful.
Adult↗
Clinically atypical granular corneal dystrophy with pathologic features of lattice-like amyloid deposits. A study of these families.
Four patients from families in Pennsylvania, Massachusetts, and Argentina were diagnosed clinically as having granular dystrophy. Results of pathologic examination of the corneal buttons from each patient after penetrating keratoplasty confirmed granular deposits in the anterior third of the stroma. Amyloid was demonstrated within some of these granular deposits by Congo red staining with birefringence and dichroism and by electron microscopy. In addition to the morphologically granular deposits, numerous fusiform deposits identified as amyloid by histochemistry and electron microscopy and morphologically identical to those seen in lattice corneal dystrophy were detected deep to the granular deposits. It was further shown that the histochemical pattern of staining of the granular material by a series of lectins was similar to that present in corneas with lattice dystrophy. Although a relationship between these patients cannot be definitively proven, each family traces its origins to the Italian province of Avellino.
Adult↗
HLA-B27 associated dactylitis in children.
A number of conditions have been found to be associated with the HLA-B27 locus including ankylosing spondylitis, psoriatic spondylitis, Reiter's syndrome, juvenile arthritis, and others. An unusual clinical syndrome of "sausage-like" toe, or dactylitis, associated with an oligoarthritis involving the lower extremities has been reported in adults, as has HLA-B27 associated spondyloarthritis and enthesopathy in children. We describe 3 children with dactylitis of the toe who carry HLA-B27. Their course and treatment are presented, and the importance of establishing this diagnostic entity is discussed.
Adolescent↗
Tarsorrhaphy for corneal disease in patients with rheumatoid arthritis.
We report two patients with keratoconjunctivitis sicca and rheumatoid arthritis whose corneal surfaces regained their integrity following tarsorrhaphy when conservative therapeutic measures were unsuccessful. We suggest that tarsorrhaphy be considered early in the course of the disease in such patients when other measures have failed to arrest progressive thinning of the cornea.
Aged↗
Involvement of the hip in juvenile rheumatoid arthritis. A longitudinal study.
Thirty-five children who had rheumatoid arthritis were followed for three to twenty-two years. Four categories of involvement of the hip were seen. In the first group, thirteen patients had mild disability and slight radiographic changes. In six of those patients the disease was in remission at the time of writing, and the patients were asymptomatic. In the second group, two patients had episodic disability that correlated with the activity of the disease. In the third group, fourteen patients had progressive disability and radiographic changes. Fifty-five of the fifty-nine procedures that were performed on the hip and knee in this series were done on those fourteen patients. In the fourth group, six patients had dramatic clinical and radiographic findings but, at the time of the latest follow-up, had little functional disability. In all six of these patients, the disease was in remission. In the children in this study, protrusio acetabuli was more cephalad than has been found in adults who have rheumatoid arthritis. All of the children had psychosocial problems, but they responded well to counseling. These problems influenced the timing of the surgical treatment.
Adolescent↗
The advantages of partial patching or tarsorrhaphy over complete eyelid closure.
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Cornea↗
Lectin receptors of amyloid in corneas with lattice dystrophy.
We analyzed lectin binding patterns of amyloid glycoconjugates in patients with lattice dystrophy of the cornea. Results of paraffin and frozen sections differed in some instances. With paraffin sections, three lectins--wheat germ agglutinin (WGA), Ricinus communis agglutinin I (RCA-I), and concanavalin A (Con A)--stained the abnormal deposits. In frozen sections, the abnormal deposits were stained by five lectins--WGA, RCA-I, Con A, peanut agglutinin (PNA), and soybean agglutinin (SBA). In paraffin sections, PNA and SBA did not stain amyloid deposits. In both paraffin and frozen sections, some lectin-positive deposits corresponded to the Congo red-positive material, whereas others were present surrounding and encroaching on the Congo red-reactive material. This study demonstrates that WGA-, RCA-I-, Con A-, PNA-, and SBA-positive abnormal deposits are present in corneas with lattice dystrophy. Since lectins bind to specific sugar residues, we conclude that the abnormal deposits consist, at least in part, of glycoconjugates and that these glycoconjugates contain oligosaccharides with N-acetylglucosamine/sialic acid, mannose/glucose and terminal beta-galactose residues and chains with terminal beta-galactose-N-acetylgalactosamine disaccharides.
Adult↗