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Biomedical subjects

William K Dolen

Publications and source records attributed to William K Dolen.

6 recordsLinked to original sources

A large kindred with familial cold autoinflammatory syndrome.

BACKGROUND: Familial cold autoinflammatory syndrome (FCAS), formerly known as familial cold urticaria, is a rare condition characterized by fever, rash, and arthralgias elicited by exposure to cold. Recently, mutations responsible for FCAS were identified in a novel gene (CIAS1), making it possible to confirm the diagnosis in most patients. OBJECTIVE: We present a summary of clinical data from a large family with FCAS to further define the characteristics of the disorder and to validate previously proposed clinical criteria. METHODS: A total of 73 participants were evaluated by interview and questionnaire, including 36 affected individuals. Responses from the questionnaire were analyzed and comparisons of proportions were made using the Z test. DNA was isolated and genotyping was performed on all subjects. Affected haplotypes (genotype patterns) were identified and used to confirm the diagnosis. Sequencing of the CIAS1 gene was performed in selected patients to confirm the mutation. RESULTS: The prevalence of rash, fever/chills, joint complaints, nausea, headache, and thirst were not significantly different from previously reported proportions. There was statistically significant differences in conjunctivitis, sweating, and drowsiness with alpha = 0.01. The mean temperature required to produce symptoms was 22 degrees C, and the average earliest onset of symptoms after exposure was 1.5 hours. CONCLUSIONS: Applying the proposed clinical criteria, 41% of affected subjects met all six criteria, 90% met five criteria, and 100% met four criteria for FCAS. None of the unaffected subjects met more than two criteria. Using a threshold of 4 of 6 clinical criteria, the data support the diagnostic validity of the proposed clinical criteria.

Adolescent↗

Sensitization to recombinant Aspergillus fumigatus allergens in allergic fungal sinusitis.

BACKGROUND: Allergic bronchopulmonary mycosis is primarily caused by Aspergillus fumigatus. Despite similarities, allergic fungal sinusitis (AFS) is thought to be caused by various fungi. OBJECTIVE: Identify fungal elements in AFS allergic mucin and determine the prevalence of specific immunoglobulin (Ig)E to recombinant A. fumigatus allergens (rAsp) in AFS patients. METHODS: Allergic mucin from 17 definitive and 10 probable AFS patients were histologically examined for fungal elements. Sera from 18 definitive AFS patients, 10 probable AFS patients, 6 chronic sinusitis patients, and 5 A. fumigatus-allergic patients were tested for specific IgE to A. fumigatus and five rAsps. RESULTS: Ten of the 17 definitive cases had hyphae morphologically resembling Aspergillus or Fusarium spp. One probable AFS patient had similar findings. Of definitive patients, 94% (17 of 18) showed A. fumigatus-specific IgE (> or = 0.35 kUa/L), and 67% were positive to one or more rAsp. Four of 10 probable patients demonstrated A. fumigatus-specific IgE, and 2 had IgE to one or more rAsp. The definitive group had greater mean A. fumigatus IgE (P < 0.05) versus the probable and chronic sinusitis groups. The definitive group's rate of IgE to the rAsps was statistically greater. All definitive patients with Aspergillus or Fusarium spp. in situ had A. fumigatus-specific IgE, and 7 of 10 had IgE to at least one rAsp. CONCLUSIONS: Most definitive AFS patients have A. fumigatus-specific IgE and many have specific IgE to rAsps. Many also demonstrate Aspergillus spp. or Fusarium spp. in situ. Findings suggests that A. fumigatus is an important causative agent in AFS in the southeast United States.

Allergens↗

Antihistamine onset of action: the importance to the patient.

Although some older antihistamines have a relatively slow onset of action, modern agents indicate an efficacy between 1 and 3 hours of administration. For patients who use antihistamines regularly, a short onset of action is relatively unimportant. When antihistamines are used as needed, either because of poor compliance or in accordance with emerging therapeutic guidelines, antihistamines with a short onset of action are preferable.

Histamine H1 Antagonists↗