Publications by authors named "T J Gribble"

Background: Timely diagnosis and continued monitoring of patients with type I Gaucher disease is critical because skeletal involvement can permanently disable patients and visceral organ involvement can lead to abdominal pain and secondary hematologic and biochemical complications.

Objective: To seek clinical consensus for minimum recommendations for effective diagnosis and monitoring of patients with type I Gaucher disease. PARTICIPANTS, EVIDENCE, AND CONSENSUS PROCESS: Contributing authors collaborated in quarterly meetings over a 2-year period to synthesize recommendations from peer-reviewed publications and their own medical experiences.

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Objectives: The incidence and severity of growth retardation in children with type 1 Gaucher disease and the response to enzyme replacement therapy with alglucerase were studied.

Study Design: A retrospective analysis of growth in 99 children and adolescents with type 1 Gaucher disease before treatment, and in 54 of those subjects during treatment, was done. Growth was compared with gender, age, and dosage of replacement enzyme.

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Hereditary factor VII deficiency is a rare autosomal recessive condition, usually associated with normal or reduced levels of a functionally defective molecule. The available means of treating this condition in North America presents serious health risks to the patient. Transfusion with fresh frozen plasma carries a risk of volume overload and a significant risk for viral transmission.

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HealthDesk for Hemophilia is an interactive computer software application designed on the premise that successful chronic illness self-management requires information, self-care skills, on-going communication with health care providers, and user-friendly record keeping. The software was pilot tested for six months in the homes of eight hemophilia patients. The purpose of the pilot was to assess the impact of HealthDesk for Hemophilia on patient satisfaction, patient-provider communication, and user confidence in chronic illness self-management.

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Persons with hemophilia who have received therapy since 1978 are at risk for the acquired immunodeficiency syndrome. Plasma and serum specimens collected from 1980 to 1985 from 73 New Mexico residents with hemophilia were tested by enzyme immunoassay for antibody to human T-cell lymphotropic virus type III (HTLV-III), and positive results were confirmed by Western blot. Antibody to HTLV-III was first detected in New Mexico residents with hemophilia in 1981.

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