Biomedical subjects
Publications and source records attributed to R L Sanchez.
Reviewed herein are the major types of vasculitis affecting children, including Henoch-Schönlein purpura, necrotizing vasculitis of small vessels, Kawasaki's disease, and, less commonly, classic polyarteritis nodosa, Wegener's granulomatosis, Churg Strauss syndrome, and lymphomatoid granulomatosis. The clinical features, diagnosis, treatment, and suspected etiologies and pathogenic mechanisms for each entity are discussed.
BACKGROUND: Amplification of HER-2/neu, a protooncogene related to the epidermal growth factor receptor, has prognostic significance in patients with breast cancer. Alterations in protooncogenes have not been determined for endocrine tumors in which prognosis is difficult to predict. We have addressed the question of whether amplification of HER-2/neu or epidermal growth factor receptor occurs in DNA from human endocrine tumor lines and have sought to characterize the HER-2/neu gene and its products in carcinoid tumors of the gut. METHODS: The differential polymerase chain reaction procedure was used to detect genomic amplification in DNA samples from human endocrine tumor cell lines (BON, SIM, STAN) and from paraffin-embedded samples of carcinoid tumors. Sequencing techniques were used to determine whether mutations of the transmembrane domain of HER-2/neu existed. We then further characterized the gene products (RNA and protein) in carcinoid tumors. RESULTS: Amplification of HER-2/neu was identified in all three endocrine tumor cell lines. HER-2/neu amplification was found in four of 10 carcinoid tumors of the gut; three of these four tumors were invasive or metastatic. In addition, HER-2/neu mRNA and protein were expressed in carcinoid tumors. CONCLUSIONS: Amplification of the HER-2/neu protooncogene occurs in endocrine tumors of the gut; quantitation of the actual copy number may be an important prognostic determinant. The unique human endocrine cell lines, established in our laboratory, will be useful models to further examine the significance of alterations of the HER-2/neu gene in endocrine tumors.
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Cryofibrinogenemia refers to the presence of cold-precipitable plasma proteins associated with a variety of disorders, including malignancies, inflammatory processes, and thrombohemorrhagic phenomena. Few cases of essential or primary cryofibrinogenemia are reported. We report a case in a 48-year-old man of essential cryofibrinogenemia and hemorrhagic necrosis of the ears and of the upper and lower extremities. Histopathologic studies demonstrated extensive eosinophilic thrombi in the dermal vasculature, with minimal inflammation and no vasculitis. Cryofibrinogenemia should be considered in the investigation of patients with otherwise unexplained cold intolerance and dermal thrombosis associated with minimal or no inflammation.
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An unusual case of isolated exophytic elastic tissue nevi in the scrotal region of a 64-year-old man is described. The histological and ultrastructural findings were those of abundant abnormal elastic fibers and increased reticulin in the dermis. This hamartomatous lesion possesses clinical and histological features previously undescribed in connective tissue nevi.
BACKGROUND: We describe common surgical and medical hospital admission rates for Maryland residents, exploring systematic effects of race and income. METHODS: The data comprise Maryland hospital discharges and population estimates for 1985 to 1987. Patient income is the race-specific median family income of residence zip code. Logistic regression is used to measure incidence by race, income, and residence for surgical and medical reasons for admission. RESULTS: Population rates for discretionary orthopedic, vascular, and laryngologic surgery tend to increase with community income levels. Coronary and carotid artery surgery rates are two to three times higher among Whites. The more discretionary the procedure, the lower is the relative incidence among Blacks. By contrast, admission rates for most medical reasons decline with increasing income levels and are elevated among Blacks. The affluent receive coronary artery procedures whereas the poor are hospitalized for coronary artery disease. CONCLUSIONS: Blacks and the poor appear to have higher illness burdens requiring hospital care. Discretionary surgeries have a White predominance and increase with income; medical admissions have a Black predominance and decline with income. Race and community income level are important factors in differential hospital utilization rates.
A 36-year-old Latin American man with the basal cell nevus syndrome had multiple, soft, hairless, depressed scars of the face, scalp, neck, and back, ranging in size from 0.3 x 0.5 cm to 3.5 x 3.9 cm. These areas had never been treated and previously were sites of basal cell carcinomas that underwent complete spontaneous regression 4-5 years from the date of onset. Pathology of these spontaneously regressed sites was consistent with the criteria established by Curson and Weedon (1979). Type III transepithelial elimination was demonstrated for a basal cell carcinoma. Type III transepithelial elimination may play a role in the spontaneous regression of basal cell carcinomas.
From October 1985 to November 1987, a sample of 294 Colombian homosexual men volunteered to answer a questionnaire on sexual practices and consented to HIV-1 testing. Testing for HIV-1 was performed using an ELISA and those positive were confirmed with envelope- and core-specific ELISAs. Statistical methods for data analysis included Mantel-Haenszel methods on contingency tables. The overall seropositivity rate was 21.1%. Subjects who reported a receptive role (either as predominantly receptive or as mixed receptive-insertive intercourse) had a seropositivity rate of 23.7%, which was significantly higher than the 10.3% found in those reporting predominantly insertive intercourse (RR = 2.30, 95% C.I. = 1.16-4.57). For subjects reporting receptive intercourse, sexual contact with foreign visitors was a significant risk factor for HIV-1 infection (RR = 1.84, 95% C.I. = 1.13-3.00). Factors of borderline significance included having had more than ten homosexual partners in the preceding year (RR = 1.53) and a history of international travel (RR = 1.43). These associations did not hold for those reporting predominantly insertive intercourse. The data indicate the need to monitor the spread of HIV-1 at the international level and provide information on subgroups of high transmission rates.
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A patient had skin and subcutaneous manifestations of angiolymphoid hyperplasia with eosinophilia. The diagnosis and treatment of this angioproliferative disease with a predilection for the head and neck were determined, and recommendations were made for avoiding surgical misadventure.
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Spreading pigmented actinic keratosis is a rarely reported premalignant, pigmented, epidermal tumor that can be difficult to distinguish clinically from other benign and malignant pigmented lesions. In an attempt to determine the cause of clinical hyperpigmentation, seven lesions from five patients were examined by light microscopy. Three of these lesions were also examined using electron microscopy. Findings from these studies indicate that hyperpigmentation is due to enhanced melanosome formation and distribution and not to a melanocyte-keratinocyte melanosome transfer block.
We describe a child who began developing subepidermal blisters in the recipient sites of split-thickness skin grafts; this process has continued for almost a year and continues to spare nongrafted skin. Routine histologic and immunofluorescence mapping studies demonstrated this disorder to be a relatively noninflammatory one characterized by sub-lamina densa blister formation. Results of direct immunofluorescence were negative. By electron microscopy, anchoring fibrils were sparse in number and in some areas appeared malformed; otherwise, the basement membrane zone was morphologically unremarkable. Bullous pemphigoid antigen, laminin, type IV collagen, epidermolysis bullosa acquisita antigen, and LDA-1 were all normally expressed along the dermoepidermal junction. In contrast, KF-1 antigen was absent. These findings suggest a disease process confined to skin graft recipient sites with features identical to those previously described with recessive dystrophic or immunofluorescence-negative acquired epidermolysis bullosa.
Lafora's disease is a neurometabolic disease characterized by progressive myoclonus epilepsy. It is thought to be transmitted by autosomal recessive inheritance. The diagnosis of Lafora's disease is based on both clinical symptomatology and histologic detection of intracytoplasmic periodic acid-Schiff-positive inclusions, termed Lafora bodies. Despite the absence of cutaneous clinical findings, typical inclusions are present in eccrine duct cells and peripheral nerve of skin biopsies. By electron microscopy, the inclusions are electron-lucent and contain glycogenlike granules and filamentous material. Skin biopsy is a convenient and the least invasive method of establishing the diagnosis of Lafora's disease.
This article describes a case of hematuria and urethritis related to androgen stimulation in a female-to-male transsexual receiving testosterone cypionate. Biopsy of the affected urethral tissue revealed periurethral glands which demonstrated a strong positive reaction with immunoperoxidase staining for prostatic specific antigen. When the androgen stimulus was reduced, the patient's symptoms resolved. This report gives evidence for possible androgen-induced pathology in the female urethra.
A circulating immune complex-mediated pathogenesis for lesions of secondary syphilis has been postulated. Textbook descriptions of a lymphoplasmacytic histopathologic picture have contradicted a role for circulating immune complexes in lesion pathogenesis. Four patients with early cutaneous lesions of secondary syphilis were studied. All four patients had serum Raji cell and/or Clq binding assay evidence for circulating immune complexes. Three patients showed a neutrophilic vascular reaction on histologic study of early lesions. The patients studied had immunofluorescence microscopic evidence of immunoreactant deposition in dermal blood vessels (4 hours) and/or a neutrophilic vascular reaction (24 hours) after intradermal histamine injection. Dieterle staining of lesional tissue from all patients showed the presence of treponemal organisms in dermal blood vessels. This new preliminary evidence adds some support to a circulating immune complex-mediated pathogenesis of cutaneous lesions in human secondary syphilis.