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Biomedical subjects

J G Guccion

Publications and source records attributed to J G Guccion.

At least 19 recordsLinked to original sources

Adult polyglucosan body disease.

We describe a case of adult polyglucosan body disease with characteristic clinical symptoms of peripheral neuropathy, upper motor neuron signs, and bowel and bladder dysfunction. Sural nerve biopsy revealed diagnostic intra-axonal polyglucosan bodies. On electron microscopic examination, the inclusions were located mainly within myelinated nerve fibers and consisted of branched filaments that were 6 to 8 nm wide. The diagnosis of adult polyglucosan body disease was confirmed by a skin biopsy from the axilla showing similar inclusions in myoepithelial cells of apocrine glands. This report provides additional evidence that skin biopsy, to date advocated by a single case report only, may be a less invasive and simpler diagnostic alternative to sural nerve or brain biopsies.

Apocrine Glands↗

Renal sarcoidosis with superimposed postinfectious glomerulonephritis presenting as acute renal failure.

We describe two patients with sarcoidosis with lesions of granulomatous interstitial nephritis (GIN) and postinfectious glomerulonephritis (GN). Both patients presented with heavy proteinuria, hematuria, and renal failure. Renal histology in both showed GIN and glomerular changes of proliferative GN with hump-like subepithelial deposits by electron microscopy of postinfectious GN. Antecedent history of pneumonia was present in one, and ASO titer was elevated in the other. The proteinuria and azotemia improved in both with steroid therapy. Reports of "postinfectious" or diffuse proliferative GN in patients with sarcoidosis are rare. The authors are unaware of reports of concomitant sarcoid GIN and postinfectious GN. Although acute renal insufficiency or failure can occur with GIN or other more common renal lesions primary glomerular disease should be considered in patients with sarcoidosis who present with renal dysfunction. This is a US government work. There are no restrictions on its use.

Acute Kidney Injury↗

PCR amplification from fixed tissue indicates frequent involvement of Brachyspira aalborgi in human intestinal spirochetosis.

PCR procedures amplifying portions of the 16S rRNA and NADH oxidase genes of Brachyspira aalborgi and Serpulina pilosicoli were applied to DNA extracted from paraffin-embedded human colonic or rectal tissues from 30 Norwegian, Australian, and U.S. patients, 16 of whom had histologic evidence of intestinal spirochetosis (IS). B. aalborgi-specific sequences were identified by PCR in 10 of the IS patients (62.5%) but none of the others, while S. pilosicoli sequences were not detected in tissues from any patient. Direct sequencing of products from three of the positive samples provided further confirmation of the presence of B. aalborgi. B. aalborgi may be a more common cause of intestinal spirochetosis than has been previously thought.

Adolescent↗

Tumor of the broad ligament in von Hippel-Lindau disease of probable mullerian origin.

A rare neoplasm seen in women with von Hippel-Lindau disease is the papillary cystadenoma of the broad ligament, only three cases of which have been reported. All three exhibited characteristic histologic features identical to those of epididymal tumors that occur in affected patients, and were presumed to be of wolffian origin. This is the case of a broad ligament tumor in a woman with von Hippel-Lindau disease that had features more consistent with a mullerian rather than wolffian origin. This is the first report of a broad ligament tumor of probable mullerian origin in von Hippel-Lindau disease.

Adult↗

Porocarcinoma of the heel. A case report with unusual histologic features.

BACKGROUND: Eccrine porocarcinoma is an uncommon neoplasm of the intraepidermal sweat gland duct. METHODS: A case of porocarcinoma of the right heel in a male age 51 years is described with a review of pertinent literature. The surgically excised neoplasm was evaluated by routine histology and transmission electron microscopy. RESULTS: The porocarcinoma showed extensive nuclear pleomorphisms with frequent, multinucleated tumor giant cells, focal epidermotrophic spread within the epidermis, a peripheral, eccrine syringofibroadenoma-like growth pattern, and an origin in a contiguous eccrine poroma. Ultrastructurally, the squamous tumor cells contained rare intracytoplasmic lumens. CONCLUSIONS: The extensive nuclear pleomorphism with frequent tumor giant cells was an unusual feature of the porocarcinoma. Its epidermotrophic spread within the epidermis and its origin in a contiguous eccrine poroma supported the diagnosis of porocarcinoma. The eccrine syringofibroadenoma-like growth pattern in the periphery of the tumor was a unique and previously undescribed feature of the porocarcinoma. The presence of intracytoplasmic lumens in squamous tumor cells mimicked embryonic development of the intraepidermal sweat gland duct.

Acrospiroma↗

Tripartite differentiation in a carcinoma of the duodenum.

BACKGROUND: Carcinomas containing three distinctly different cell lines have been encountered in the colon and rectum, but a tripartite malignancy in the small intestine has not been reported previously. METHODS: A duodenal carcinoma was studied by light and electron microscopic examination and immunohistochemistry. RESULTS: The duodenal carcinoma was found to have tripartite glandular, squamous, and neuroendocrine differentiation. Histologically, an adenocarcinoma, which originated in a villous adenoma, was continuous with squamous cell carcinoma and small cell carcinoma components. Tumor cells of the squamous cell carcinoma component had conspicuous intercellular bridges but did not form keratin pearls. Immunohistochemical analysis showed strong expression of carcinoembryonic antigen (CEA) by the adenocarcinomatous component. The squamous cell carcinoma component demonstrated focal weak CEA and neuron specific enolase (NSE) reactivity. Ultrastructurally, tumor cells of this component had frequent desmosomes and free tonofilaments. The small cell carcinoma had clusters of dense core granules in tumor cell cytoplasmic processes, which are indicative of neuroendocrine differentiation. This neuroendocrine component was immunoreactive for somatostatin and NSE. CONCLUSIONS: This case of tripartite duodenal carcinoma supports the theory of an origin from an intestinal pluripotential stem cell capable of differentiating into multiple cell types.

Adenocarcinoma↗

Hemodialysis-associated amyloidosis presenting as lingual nodules.

Hemodialysis has permitted long-term survival of patients with kidney failure, but not without many complications, such as mineral loss from bones, secondary hyperparathyroidism, and increased risk of infection. Recently a new form of amyloidosis, AB2M, has been characterized that is specifically associated with long-term dialysis. The tissues and organs affected by deposits of AB2M appear to differ from those affected by the other major types of amyloidosis; AB2M affects mainly the carpal ligament, synovia of large joints, juxtaarticular bone, and intervetebral disks, while involvement of the oral tissues has been rare. We present here a case in which the biopsy of superficial lingual deposits of AB2M led to the recognition that the patient had developed hemodialysis-associated amyloidosis. It is suggested that periodic inspection of the oral soft tissues for signs or symptoms of amyloidosis should become a part of the dental care of patients undergoing dialysis.

Amyloidosis↗

Eosinophilic products lead to myocardial damage.

Eosinophils have been associated with endomyocardial fibrosis. However, their effect on the myocardium and the reactions they elicit have not been emphasized. We describe four patients with extensive myocardial eosinophilic infiltration with and without endomyocardial fibrosis. Patients with a short duration of disease had eosinophilic infiltrates, myocardial eosinophilic abscesses containing granular eosinophilic material, and/or Charcot-Leyden crystals with and without profuse histiocytic and giant cell reaction with granuloma formation. In the early phase, the granular eosinophilic material can be visualized by the Luna stain and/or by electron microscopic examination. In patients with a long duration of symptoms, well-developed granulomas and profuse fibrosis are seen and eosinophilic material may not be visualized. Toxic eosinophil granular protein may be involved in the development of these lesions.

Adult↗

Diagnosis of progressive multifocal leukoencephalopathy by brain biopsy with biotin labeled DNA:DNA in situ hybridization.

DNA:DNA in situ hybridization using a cloned JC virus (JCV) DNA probe labeled with biotin confirmed the presence of JCV DNA in formalin-fixed, paraffin-embedded brain biopsies from four cases of progressive multifocal leukoencephalopathy (PML). Only small pieces of tissue were available in each case. Detection of the JC DNA:DNA hybrids was carried out by affinity cytochemistry. JCV DNA was identified predominantly in the nuclei of interfascicular oligodendrocytes in demyelinated areas of the biopsies. JC virus was isolated from one case, and the diagnosis of PML was substantiated in all cases by electron microscopic identification or immunocytochemical labeling of JC viral antigen. In situ hybridization using a biotin labeled JCV DNA probe is a specific, sensitive and convenient method for confirming the diagnosis of PML in suspected cases evaluated by brain biopsy.

Adult↗

Canalicular adenoma of the buccal mucosa. An ultrastructural and histochemical study.

A canalicular adenoma that occurred in the minor salivary glands of the buccal mucosa in a 62-year-old black man was examined by electron microscopy and histochemistry. The ulcerated bowl-shaped tumor consisted mostly of ductlike tubules of columnar and cuboidal epithelial tumor cells which resembled intercalated duct cells ultrastructurally. Tumor cells resembling myoepithelial cells were not associated with these tubules. Less frequently, there were narrow tubules and cords adjacent to the capsule. Occasional cells in these cords contained variable amounts of microfilaments, often associated with focal densities, and thus resembled myoepithelial cells in varying stages of differentiation. Other noteworthy ultrastructural findings in this neoplasm included marked replication of the basal lamina of capillaries and the presence of a single, luminally oriented cilium in a tumor cell lining a tubule.

Adenoma↗

Establishment and identification of small cell lung cancer cell lines having classic and variant features.

Using a chemically defined medium containing hydrocortisone, insulin, transferrin, 17 beta-estradiol and selenium, with or without serum supplementation (2.5% v/v), continuous cell lines can be established from 72% of all fresh biopsy specimens of small cell lung cancer (SCLC) containing tumor cells. No differences were observed in the rate of establishing cell lines from newly diagnosed untreated patients, or from patients who have relapsed from prior therapy, or from a variety of different organ sites. Biochemical characterization of 50 SCLC cell lines for the expression of L-dopa decarboxylase; bombesin-like immunoreactivity; neuron-specific enolase, and the brain isozyme of creatine kinase, revealed that SCLC cell lines can be subdivided into two distinct classes: classic SCLC cell lines (35 lines), which express elevated levels of all four biomarkers; and variant SCLC cell lines (15 lines) which have undetectable levels of L-dopa-decarboxylase and bombesin-like immunoreactivity, but continue to express neuron-specific enolase and the brain isozyme of creatine kinase. The presence of the latter two markers distinguishes variant lines fron non-SCLC cell lines. In addition, four distinct classes were identified morphologically. The biomedical differences among established SCLC cell lines may account for the differences in response rates to cytotoxic therapy observed in newly diagnosed SCLC patients. A prospective study of biomarker characterization of SCLC tumors will determine if clinical differences exist between classic and variant SCLC tumors.

Animals↗

Pulmonary hyalinizing granuloma. Electron microscopic and immunologic studies.

We present a case of pulmonary hyalinizing granuloma (PHG). On light microscopy, the pulmonary nodular lesions consisted of extracellular, eosinophilic hyaline lamellae. Histochemical stains of the hyaline lamellae for amyloid were focally positive and the diagnosis of amyloidosis was seriously considered; however, on electron microscopic examination, the hyaline lamellae consisted of electrondense, compact, amorphous material quite unlike fibrillar amyloid. Although circulating immune complexes containing IgA were detected in our patient, immunoperoxidase stains did not reveal immunoglobulins in the hyaline lamellae. This case illustrates the value of electron microscopy in differentiating PHG from amyloidosis and supports the hypothesis that PHG represents an exaggerated immune response.

Antigen-Antibody Complex↗

Demonstration of the stem cell nature of clonogenic tumor cells from lung cancer patients.

The tumor cell colony-forming efficiency in soft agarose was evaluated in 190 specimens obtained from patients with either small cell carcinoma of the lung (SCCL) or adenocarcinoma of the lung (ACL). Tumor cell colony formation was observed in 45 out of 53 (84%) specimens containing cytopathologically identifiable SCCL tumor cells obtained from a variety of metastatic sites. Tumor cell colony formation was also observed in 6 out of 10 specimens containing ACL tumor cells. The colony-forming efficiency for these lung cancer specimens ranged from 0.003 to 0.72% per nucleated cell plated and from 0.05 to 1.5% per tumor cell plated. There were no differences in plating efficiency for SCCL and ACL. The tumor cell origin of the cells in the agarose colonies was confirmed by cytologic examination, flow cytometric DNA content analysis, and electron microscopy examination. The 'stem cell' nature was demonstrated by the ability of colonies to form typical SCCL or ACL tumors when inoculated into athymic nude mice. In addition, continuous tumor cell lines were established from several specimens forming tumors in nude mice. These data confirm the tumor cell origin and 'stem cell' nature of lung cancer cell colonies growing in soft agarose. Whether the low colony-forming efficiency represents a property inherent in the tumor cells, or a defect in the culture system, remains to be determined.

Adenocarcinoma↗