Localized primary (AL) amyloid tumor of the breast. Cytologic, histologic, immunocytochemical and ultrastructural observations

Two examples of localized primary amyloid tumor of the breast are presented, including one patient with metachronous bilateral lesions. Our findings and review of the literature indicate that this rare lesion occurs predominantly in elderly females and can be mammographically and clinically confused with carcinoma. Fine-needle aspiration biopsy can be a useful procedure to make a preliminary diagnosis. Congo red staining with prior potassium permanganate incubation confirmed the AL type of amyloid in our two cases; this might be the predominant type in the localized form involving the breast. Immunofluorescence studies demonstrated IgA, with kappa and lambda light-chain deposition within the amyloid foci in one case, and intracytoplasmic IgG with both light chains within plasma cells and amyloid deposits of the second case. Ultrastructural examination of one of the cases showed characteristic findings of straight, nonbranching fibrils of 4-9 nm, diagnostic of amyloid. From our findings and a review of the literature, we conclude that amyloid tumors of the breast can occur in three separate settings: secondary amyloidosis, systemic or multiple myeloma associated amyloidosis, and as a localized primary type having a benign course.     

Sensitivity and specificity of clinical case definitions for pertussis

We evaluated the diagnostic performance of 15 clinical case definitions for pertussis in 233 patients who developed acute respiratory illness during community outbreaks in Wisconsin and Delaware. Using results from culture (Regan-Lowe media) and serology (Ig-class-specific ELISA) as diagnostic standards, cough for greater than or equal to 14 days was both sensitive (84 per cent-92 per cent) and specific (63 percent-90 per cent) in identifying patients with pertussis. This definition may be useful in monitoring pertussis outbreaks and for investigating contacts of culture-positive cases.     

Chronic lymphocytic leukemia presenting in the marrow of a phalanx

Systemic disease may present first in the foot. The authors present a case in which the marrow of resected bone tissue from a phalanx showed the changes of chronic lymphocytic leukemia (CLL). Subsequent blood examination confirmed this diagnosis.     

Head and neck malignancies associated with HIV infection.

Immunosuppressed persons are at greater risk of developing malignancies. In human immunodeficiency virus (HIV) immunosuppression the most common oral cancers are Kaposi's sarcoma and non-Hodgkin's lymphoma. Squamous cell carcinoma has also been reported to be associated with HIV disease. Kaposi's sarcoma is the most frequent neoplastic disease in acquired immunodeficiency syndrome and is by far the most common in the head and neck area. This article reviews the prevalence, clinical features, and management of these diseases in HIV infection.     

The epidemiology of Alzheimer disease in intellectual disability: results and recommendations from an international conference

Among adults with intellectual disability, virtually everyone with Down's syndrome (DS) over the age of 40 years has neuropathology currently viewed to be consistent with a diagnosis of Alzheimer disease (AD), while other adults with intellectual disability without DS display an increased prevalence of Alzheimer-type neuropathology after they reach the age of 65. This paper presents the results of discussions by an epidemiology workgroup, formed at an international conference convened to discuss AD among people with intellectual disability, concerning: (I) the incidence and prevalence of clinical dementia in adults with intellectual disability; (2) risk factors for the development of AD in adults with intellectual disability; and (3) a minimum data set that would be of great utility for future research on AD in adults with intellectual disability.     

Estrogen inhibition of a Mr 39,000 glycoprotein secreted by human breast cancer cells

We have identified a secreted glycoprotein with an apparent molecular weight of 39,000 that is inhibited by estrogens and stimulated by antiestrogens in the MCF-7 human breast cancer cell line. The protein was detected within 24 h of hormone treatment with inhibition occurring at concentrations as low as 10(-11) M estradiol and stimulation occurring with antiestrogens. When phenol red, which has been found to have estrogenic activity, is removed from the tissue culture medium, no inhibition of cell growth or induction of the Mr 39,000 protein is seen with antiestrogens. A 6-fold decrease in the amount secreted in the presence of estrogens is seen under these conditions. The induction was specific in that growth inhibition by several other means did not induce expression of the Mr 39,000 protein. The Mr 39,000 protein has been identified as a glycoprotein complex that can be resolved into four polypeptides with two major components that migrate in the pH range of 5.5 to 6.3 by two-dimensional gel electrophoresis. This protein is the first evidence that estrogens inhibit a specific protein and it may prove useful as a marker of antiestrogen-induced growth arrest in breast cancer.     

Alpha-methyltyrosine blocks the expression of rotation classically conditioned with apomorphine.

Rats with unilateral 6-hydroxydopamine lesions of substantia nigra rotate (circle) when placed, undrugged, in the environment in which they have previously been treated with apomorphine. This conditioned rotation, like the unconditioned rotation which acutely follows the administration of apomorphine, is directed away from the side with the lesion, i.e., the rotation is contralateral. Here, rats that had been administered apomorphine weeks earlier were tested, in a crossover design, for the expression of conditioned rotation following treatment with saline and with alpha-methyltyrosine. When administered four hours prior to testing, 100 mg/kg alpha-methyltyrosine significantly antagonized the expression of classically conditioned rotation. In a second group of animals, alpha-methyltyrosine had no effect on the unconditioned rotation induced by 0.05 mg/kg apomorphine.     

Parental education and guided self-management of asthma and wheezing in the pre-school child: a randomised controlled trial

BACKGROUND: The effects on morbidity were examined of providing an educational intervention and a written guided self-management plan to the parents of pre-school children following a recent attendance at hospital for asthma or wheeze. METHODS: A prospective, randomised, partially blinded, controlled trial was designed at two secondary care centres. Over a 13 month period 200 children aged 18 months to 5 years at the time of admission to a children's ward or attendance at an accident and emergency department or children's (emergency) assessment unit (A&E/CAU) with a primary diagnosis of acute severe asthma or wheezing were recruited. 101 children were randomised into the control group and received usual care and 99 were assigned to the intervention group and received: (1) a pre-school asthma booklet; (2) a written guided self-management plan; and (3) two 20 minute structured educational sessions between a specialist respiratory nurse and the parent(s) and child. Subjects were assessed at 3, 6, and 12 months. The main outcomes were GP consultation rates, hospital re-admissions, and attendances at A&E/CAU. Secondary outcomes included disability score, caregivers' quality of life, and parental knowledge of asthma. RESULTS: There were no statistically significant differences between the two groups during the 12 month follow up period for any of the main or secondary outcome measures. CONCLUSIONS: These results do not support the hypothesis that the introduction of an educational package and a written guided self-management plan to the parents of pre-school children with asthma who had recently attended hospital for troublesome asthma or wheeze reduces morbidity over the subsequent 12 months.