Biomedical subjects
Publications and source records attributed to L Bielory.
Managed care organizations and academic medical centers initially have been at opposite ends of the cost containment spectrum. UMDNJ, the largest free-standing health care university in the United States, responds to the competitive managed health care market.
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There are several reasons for considering the hypothesis that food hypersensitivity might relate to rheumatic diseases. Food can evoke immune responses. Food can cause immunologically mediated symptoms. Immunological mechanisms are important in the pathogenesis of rheumatic diseases. Antigens triggering rheumatic responses are unknown. Rheumatic diseases have been associated with foods in many reports.
We present an unusual case of adult-onset systemic mastocytosis with biopsy-proven skin and bone manifestations with the rare presentation of lumbar fracture and osteopenia. The normal 24-hour urine histamine is surprising in this patient, but it does not rule out mastocytosis. It is a sensitive assay that can give false negative results if stored before the assay is performed. Also histamine and PGD2 metabolites are more sensitive and specific. It is surprising that our patient did not have gastrointestinal symptoms. In one prospective study of 16 patients with systemic mastocytosis in which 75% had bone marrow involvement as the most common extracutaneous site, 80% were found to have gastrointestinal symptomatology, indicating such symptoms to be more common than previously thought. The lack of pruritus is also surprising, as most cutaneous lesions of urticaria pigmentosa are pruritic. Symptoms of systemic mastocytosis, particularly pruritus may respond to antihistamines and the bone disease may not respond to calcium and hormone manipulation. The main role of the latter is to prevent further bone loss. We may consider the use of cromolyn or ketotifen if she does not respond. Close follow-up will be needed to check for progression, however she appears to have a good prognosis with skin involvement and no hematologic abnormalities.
BACKGROUND: Home health care has been touted as a cost-saving option for many immune disorders, especially those requiring intravenous immunoglobulin (IVIG). OBJECTIVE: To determine the costs for home infusions of IVIG, a comparative study of Home Health Agency prices was undertaken. METHOD: A survey of 25 Home Health Agencies that provide IVIG therapy in northern and central New Jersey was conducted by telephone and office interviews. RESULTS: Thirteen companies provided price quotations for services and IVIG. The highest-priced companies charge approximately four times more for IVIG infusions than the lowest-priced companies, and many agencies offer the therapy at moderate costs. CONCLUSION: IVIG can be obtained and administered in the home in more cost-effective ways if comparative pricing is done.
OBJECTIVE: To define what role vitamin C may or may not play in the treatment of asthma. DATA SOURCES: A comprehensive literature search of relevant English-language papers identified through a Medline search and from bibliographies of the identified papers. STUDY SELECTION: We identified papers and studies pertaining to vitamin C in asthma and allergy and analyzed these studies according to their design, inclusion and exclusion criteria, population studied, variables or factors tested, method of intervention or treatment with vitamin C, and results and conclusions. We reviewed our data and divided it based on significant or insignificant roles of vitamin C in asthma and allergy. RESULTS: From our review, we found a number of studies that support the use of vitamin C in asthma and allergy. Significant results include positive effects on pulmonary function tests, bronchoprovocation challenges with methacholine or histamine or allergens, improvement in white blood cell function and motility, and a decrease in respiratory infections. Our review also revealed several studies that did not support a beneficial role in vitamin C in asthma and allergy. These studies did not report improvements in pulmonary function tests or bronchoprovocation challenges. No benefit was noted in these studies when testing cutaneous reactivity or specific immunologic factors and levels. CONCLUSIONS: Clearly from our review, the role of vitamin C in asthma and allergy is not well defined. The majority of the studies were short term and assessed immediate effects of vitamin C supplementation. Long term supplementation with vitamin C or delayed effects need to be studied. Although, the current literature does not support a definite indication for the use of vitamin C in asthma and allergy, the promising and positive studies revive curiosity and interest. With a large portion of health care dollars being spent on alternative medicine and vitamin C in particular, further studies are needed to define its role.
Classically, Reiter's syndrome was defined as a triad of arthritis, urethritis, and conjunctivitis. In 1981, the American Rheumatism Association revised its defining criteria for Reiter's syndrome as an episode of peripheral arthritis of more than 1 month's duration occurring in association with urethritis and/or cervicitis. Reiter's syndrome is also associated with mucocutaneous lesions. Reiter's syndrome was first described in association with the human immuno-deficiency virus (HIV) in 1987. The course of Reiter's syndrome in HIV is more severe, progressive, and refractory to treatment than in non-HIV-positive patients. The immunopathogenesis of Reiter's syndrome is linked to HLA-B27, which has been described as the disease susceptibility factor. The association of Reiter's syndrome and HLA-B27 positivity is 80%. Infectious agents may play a critical role in the initiation or perpetuation of Reiter's syndrome. It has been suggested that an amino acid sequence within the HLA-B27 molecule allows microbial peptides to bind and be presented to T cytotoxic cells (CD8), which results in a primary T cytotoxic cell response against various tissues. The role of the HIV in the pathogenesis of Reiter's syndrome is still being investigated. Human immunodeficiency virus may directly cause arthritis; it may increase the host's susceptibility to infection with arthritogenic organisms. Additionally, HIV infection increases the relative number of CD8 cells, which may play a key role in the pathogenesis of Reiter's syndrome. In this article, we present a case of Reiter's syndrome in an HLA-B27 and HIV-positive patient, and a review of the literature on the infectious, immunologic, and dermatologic aspects of Reiter's syndrome in HIV disease.
Increasing utilization of intravenous immunoglobulin home therapy has stimulated a scramble by various home health agencies for this high-priced business. A comparison of costs illustrates wide disparity among companies serving patients in New Jersey.
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In an attempt to prevent disease exacerbations, intravenous gamma globulin (500 mg to 2 g/kg) plus methylprednisolone was administered monthly to 14 patients with progressive multiple sclerosis, 11 of whom were steroid dependent. Seventeen exacerbations of disease activity were seen in 11 patients over a mean follow-up period of 7.8 months. Four exacerbations occurred in 3 patients within one month of receiving 1.6 to 2.0 g/kg of intravenous gamma globulin (IVGG). Most exacerbations occurred within 2 weeks of steroids being tapered; thus a steroid sparing effect of IVGG could not be demonstrated. We conclude that IVGG plus methylprednisolone can be given safely at monthly intervals for a prolonged period but in the dosage administered did not prevent exacerbations in 80% of patients with progressive multiple sclerosis.
The use of intravenous immunoglobulin (IVIG) has been expanding to include a variety of immunodeficiency, autoimmune, and inflammatory disorders. Several researchers have demonstrated home administration of IVIG is economically feasible and safe.
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Sera from 53 patients with acute Guillain-Barré syndrome (GBS), 15 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 13 patients with other neurological diseases (OND) and 31 healthy controls were tested for IgM and IgG antibodies to sulfoglucuronyl paragloboside (SGPG) and sulfatide by both an ELISA and a thin-layer chromatogram-overlay technique. Although the mean levels of anti-SGPG or anti-sulfatide antibodies in GBS patients were not elevated compared to controls, the occurrence of anti-SGPG antibodies was more frequent in GBS patients than in controls (P less than 0.02). Acute GBS patients with antibodies to SGPG or sulfatide were clinically indistinguishable from other GBS patients. Our data suggest that elevated levels of antibodies to SGPG could be important in the pathogenesis of neuropathy in some GBS patients.
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Four patients with granulomatous disease of the anterior visual pathway presented with optic neuropathy between July 1986 and February 1987, secondary to an orbital pseudotumor (n = 1) and sarcoidosis (n = 3). All but one became resistant to corticosteroid therapy, and all were subsequently treated for 1 to 2 years with low-dose cyclosporine alone or in combination with tapering doses of prednisone. Two patients experienced pronounced recovery of visual function, and visual function was stabilized in the other two; in one of these, magnetic resonance imaging showed substantial regression of intracranial lesions. Steroid-induced complications of uncontrolled hyperglycemia (n = 2) and obesity (n = 4) were controlled with cyclosporine. The authors conclude that low-dose cyclosporine is a safe and effective therapeutic alternative in ocular granulomatous diseases.
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At the age of 12, a prematurely born boy with an otherwise unremarkable past medical history developed bilateral optic neuritis associated with transverse myelopathy. Over the ensuing 3 years, recurrent bouts of optic neuritis OU, with dyschromatopsia, and acuity and field loss (arcuate, central, and paracentral scotomas) were controlled with increasing doses of corticosteroids. However, the patient became steroid-dependent and experienced recurrent optic neuritis during multiple attempts at tapering the steroids. He developed optic atrophy and steroid complications, including cushingnoid features and growth maturation delay. Immunoglobulin G subclass 2 and 3 deficiencies were the only serologically detectable abnormalities. Administration of intravenous gammaglobulin (25 g monthly) allowed discontinuation of steroids without further ophthalmic or neurologic disease. Following steroid withdrawal and institution of gammaglobulin, the patient grew 6 inches within 2 years, regaining his vision, retrieving his stature, and normalizing his psychosocial development.
The role of IgG-subclass antibodies in the spectrum of immunologic disorders has not yet been fully defined. In an attempt to understand its role in an immune complex-mediated disease, we studied patients who developed serum sickness (SSX) after treatment with an equine-derived immunoglobulin, antithymocyte globulin (ATG), for bone marrow failure. The predominant IgG subclass produced was IgG1, representing nearly 80% of all IgG anti-ATG activity present. The appearance of IgG anti-ATG antibodies and C1q-containing immune complexes was closely correlated with symptoms of SSX. Although other antibody isotypes were present and may have contributed to the patients' symptoms, it is evident that IgG1 is the predominant IgG subclass produced in human SSX caused by a heterologous protein.