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Biomedical subjects

Catherine R Weiler

Publications and source records attributed to Catherine R Weiler.

8 recordsLinked to original sources
PubMed2006

Use of intravenous immunoglobulin in human disease: a review of evidence by members of the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma and Immunology.

Human immunoglobulin prepared for intravenous administration (IGIV) has a number of important uses in the treatment of disease. Some of these are in diseases for which acceptable treatment alternatives do not exist. In this review we have evaluated the evidence underlying a wide variety of IGIV uses and make specific recommendations on the basis of these data. Given the potential risks and inherent scarcity of IGIV, careful consideration of the indications for and administration of IGIV is warranted.

Asthma↗
PubMed2006

Clinical and laboratory characteristics of 75 patients with specific polysaccharide antibody deficiency syndrome.

BACKGROUND: There are limited studies of large cohorts of patients with specific polysaccharide antibody deficiency (SPAD) syndrome. OBJECTIVE: To study the clinical and laboratory characteristics of patients with specific polysaccharide antibody deficiency syndrome. METHODS: We retrospectively studied 75 patients with total IgG levels of at least 500 mg/dL and fewer than 9 of 12 responses to vaccination with pneumococcal vaccine polyvalent. Exclusion criteria included an IgG level less than 500 mg/dL, established immunodeficiency syndrome, and secondary immunodeficiency. RESULTS: The most common clinical presentation was frequent infections (n = 69; 92%), including sinusitis (n = 53; 77%), pneumonia (n = 29; 42%), ear infections (n = 18; 26%), and bronchitis (n = 19; 28%). Other presentations were systemic infections (n = 5; 7%), autoimmune or rheumatic diseases (n = 6; 8%), and chronic diarrhea (n = 4; 5%). The median IgG2 level of patients with no response to pneumococcal vaccine polyvalent tended to be lower than that of patients with at least 1 response (150 vs 193 mg/dL, respectively; P = .06). There was no association between total IgG level (categorized as 500-600 or > or = 600 mg/dL) and frequency of infection (P = .43). Patients with fewer responses to pneumococcal vaccine polyvalent and a higher frequency of infections were more likely to receive intravenous immunoglobulin (IVIG) therapy (P = .01 and .003, respectively). Treatment with IVIG significantly reduced the number of infections (P < .001). CONCLUSION: Patients with no response to pneumococcal vaccine polyvalent tended to have lower IgG2 levels; those with fewer responses were more likely to receive IVIG therapy.

Adolescent↗
PubMed2006

Monoclonal gammopathies and malignancies in patients with chronic urticaria.

BACKGROUND: Monoclonal gammopathy of undetermined significance (MGUS) has been described in association with chronic urticaria (CU) in patients with Schnitzler syndrome. Chronic urticaria may be a manifestation of hematologic malignancies. METHODS: This study was conducted to evaluate the frequency and characteristics of MGUS or malignancy in patients with CU. The Mayo Clinic electronic database was reviewed to identify patients with the diagnosis of CU. RESULTS: Of the 1639 patients presenting with CU between 1994 and 2001, 797 (49%) underwent laboratory evaluation for the presence of a coexisting monoclonal protein. Forty-seven CU patients had MGUS, 142 had a malignancy, and 24 had both. Fifteen percent of CU patients with MGUS had a hematologic malignancy compared with 0.9% of CU patients without MGUS (P < 0.001). Patients presenting with a new diagnosis of CU at an older age (> 56 years) were more likely to have associated underlying MGUS. The occurrence of MGUS in this group was higher than the reported incidence of MGUS in the general population. CONCLUSIONS: Patients with CU younger than 43 years were unlikely to have associated MGUS or malignancy. A higher percentage of patients with CU and MGUS had an associated diagnosis of hematologic malignancy.

Adult↗
PubMed2006

Genetic test indications and interpretations in patients with hereditary angioedema.

Patients with hereditary angioedema (HAE) present with recurrent, circumscribed, and self-limiting episodes of tissue or mucous membrane swelling caused by C1-inhibitor (CI-INH) deficiency. The estimated frequency of HAE is 1:50,000 persons. Distinguishing HAE from acquired angioedema (AAE) facilitates therapeutic interventions and family planning or testing. Patients with HAE benefit from treatment with attenuated androgen, antifibrinolytic agents, and C1-INH concentrate replacement during acute attacks. HAE is currently recognized as a genetic disorder with autosomal dominant transmission. Other forms of inherited angioedema that are not associated with genetic mutations have also been identified. Readily available tests are complement studies, including C4 and C1-esterase inhibitor, both antigenic and functional C1-INH. These are the most commonly used tests in the diagnosis of HAE. Analysis of C1q can help differentiate between HAE and AAE caused by C1-INH deficiency. Genetic tests would be particularly helpful in patients with no family history of angioedema, which occurs in about half of affected patients, and in patients whose C1q level is borderline and does not differentiate between HAE and AAE. Measuring autoantibodies against C1-INH also would be helpful, but the test is available in research laboratories only. Simple complement determinations are appropriate for screening and diagnosis of the disorder.

Angioedema↗
PubMed2005

Common variable immunodeficiency: test indications and interpretations.

Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder that can present with multiple phenotypes, all of which are characterized by hypogammaglobulinemia, in a person at any age. A specific genetic defect that accounts for all CVID phenotypes has not been identified, and it is likely that several distinct genetic disorders with similar clinical presentations are responsible for the observed variation. In this review, we summarize the known genetic mutations that give rise to hypogammaglobulinemia and how these gene products affect normal or abnormal B-cell development and function, with particular emphasis on CVID. Additionally, we describe specific phenotypic and genetic laboratory tests that can be used to diagnose CVID and provide guidelines for test interpretation and subsequent therapeutic intervention.

B-Lymphocytes↗
PubMed2004

Angioedema related to the use of hyaluronidase in cataract surgery.

PURPOSE: To report a series of cases of angioedema related to the use of hyaluronidase in cataract surgery. DESIGN: Observational case series. METHODS: Three eyes of three patients who had periorbital angioedema after phacoemulsification at the Mayo Clinic, Rochester, Minnesota, between April 2001 and May 2002 were retrospectively skin tested for allergies using the prick technique. RESULTS: Skin testing was positive in all three patients for allergy to hyaluronidase (150 U/cc) and histamine control; and negative for prick control, 2% lidocaine, 1% cyclopentolate, 2.5% neosynephrine, 0.5% proparacaine, 0.3% ofloxacin, and thimerosal. CONCLUSIONS: The use of hyaluronidase in ophthalmic anesthesia injection may cause immediate type hypersensitivity reactions such as angioedema.

Angioedema↗
PubMed2003

Hypersensitivity to progesterone-in-oil after in vitro fertilization and embryo transfer.

OBJECTIVE: To report the occurrence and management of pulmonary compromise, marked leukocytosis, and eosinophilia in a patient receiving P-in-oil after IVF and embryo transfer. DESIGN: Case report.A tertiary referral reproductive medicine unit. PATIENT(S): A 29-year-old patient receiving P-in-oil supplementation after IVF embryo transfer. INTERVENTION(S): Extensive diagnostic testing and surveillance for hypersensitivity to P in sesame oil; development of an alternative oil-based P-in-oil suspension. MAIN OUTCOME MEASURE(S): Tolerance of alternative P vehicle; clinical pregnancy. RESULT(S): The patient tolerated an alternative P oil vehicle and successfully achieved a clinical pregnancy after frozen embryo transfer. CONCLUSION(S): Although rare, hypersensitivity reactions may occur in patients receiving P-in-oil supplementation after IVF embryo transfer. Testing for tolerance and subsequent use of alternative P vehicles may be an effective strategy in managing patients with sensitivity to P-in-oil.

Adult↗