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Biomedical subjects

J A Carney

Publications and source records attributed to J A Carney.

At least 19 recordsLinked to original sources
PubMed1992

Multiple endocrine neoplasia type 2B: eighteen-year follow-up of a four-generation family.

Seven members with multiple endocrine neoplasia type 2B from a 15-member family have been followed for 18 years. All affected had the neuroma phenotype in a distribution compatible with autosomal dominant inheritance. The phenotype features have allowed 100% initial and continuing prediction of affected versus nonaffected status in as early as 1.5 years. Among the affected: immunoreactive plasma calcitonin (iCT) concentration was high in 100%; thyroid palpation was false-negative in 71%; and thyroid scintiscan was false-negative in 83%. All had total thyroidectomy, plus lymphadenectomy in three, for bilateral medullary thyroid carcinoma (MTC) or C-cell hyperplasia (in the youngest). None has died directly from MTC. The index case died at age 68 and his son at age 32 years from complications of the syndrome. All but the youngest have continuing high iCT concentrations. No patient has had parathyroid disease. During preoperative calcium infusion, immunoreactive serum parathyroid hormone concentration declined by 35% to 84% of basal. At operation, macroscopically and microscopically normal parathyroid glands were found in all. No patient has had chemical suggestion of pheochromocytomas: at postmortem the index case had no adrenal medullary disease; his son had bilateral nodular adrenal hyperplasia; his daughter has had adrenal medullary hyperplasia and a renin-secreting juxtaglomerular tumor. Initially nonaffected members remain so.

Adolescent↗
PubMed1992

Parathyroid carcinoma: clinical and pathologic features in 43 patients.

Parathyroid carcinoma accounts for 0.5 to 5% of all cases of hyperparathyroidism. We reviewed the clinical, surgical, and pathologic features observed in all patients with parathyroid carcinoma evaluated at the Mayo Clinic from 1920 through 1991. Forty-three patients (22 women, 21 men; mean age, 54 yrs, range 29-72) were identified, including 2 with familial hyperparathyroidism. Information on initial presentation was available in 40 patients: 15 (38%) presented with polydipsia or polyuria, 11 (27%) with myalgias or arthralgias, 7 (17%) with weight loss, and 4 (10%) with nephrolithiasis; 3 patients (7%) were asymptomatic at presentation. Of 31 patients in whom the initial neck examination was recorded, 14 (45%) had a palpable neck mass. The mean serum calcium and serum phosphorus levels were 14.6 mg/dl and 2.3 mg/dl, respectively. Parathyroid hormone levels were elevated in 21 of 21 patients (mean elevation, 10.2 times upper limit of normal). Complications included nephrolithiasis in 14 of 25 patients (56%), bone disease in 20 of 22 patients (91%) and both in 8 of 15 patients (53%). All patients underwent primary surgical resection of parathyroid carcinoma. Twenty-six of 43 patients (60%) required a second operation with 18 patients requiring multiple re-explorations. At the second operation, residual tumor was found in the neck (68%), mediastinum (16%), or both (12%). Six patients received radiation therapy to the neck (5 patients) or bones (1 patient) for recurrent or metastatic disease. Of these, 1 patient appeared cured of parathyroid carcinoma by radiation therapy 11 years after documented tumor invasion of his trachea. Repeated excision of tumor recurrences was an effective means of controlling hypercalcemia in these patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Academic Medical Centers↗
PubMed1991

Segmental distribution of colonic neuropeptides in Hirschsprung's disease.

Despite continued research, the pathophysiologic mechanism responsible for functional obstruction in the aganglionic segment of bowel in Hirschsprung's disease remains controversial. Narrowing of the affected segment is thought by many investigators to be the result of loss of intrinsic inhibitory innervation. For this hypothesis to be consistent, inhibitory neuropeptides should be present in the dilating, transitional segment of bowel. In order to quantitate reported changes in peptidergic nerve staining in Hirschsprung's disease, we measured concentrations of five neuropeptides (vasoactive intestinal peptide, peptide histidine-methionine, met5-enkephalin, substance P and bombesin-like immunoreactivity) by radioimmunoassay in the affected segments of bowel from six patients with Hirschsprung's disease. Tissue extracts were prepared using gut obtained at surgery from the: (1) constricted, aganglionic segment, (2) dilating, aganglionic transitional segment and (3) dilated, proximal ganglionic segment. Concentrations of vasoactive intestinal peptide, peptide histidine-methionine, substance P and met5-enkephalin were significantly reduced in both the muscularis externa and the mucosal-submucosal layers from the constricted aganglionic segment. By contrast, concentrations of the candidate inhibitory neuropeptides, vasoactive intestinal peptide and peptide histidine-methionine, were minimally reduced in the dilating, aganglionic transitional segment. These results are consistent with the hypothesis that constriction of the aganglionic segment is due to loss of intrinsic inhibitory innervation. Concentrations of bombesin-like immunoreactivity were similar in the three segments of human gut, suggesting the presence of this immunoreactive neuropeptide in extrinsic nerve fibers.

Bombesin↗
PubMed1991

The Carney complex with ocular signs suggestive of cardiac myxoma.

We treated a patient who had ophthalmic findings of the Carney complex that led to a search for and the discovery of asymptomatic cardiac myxoma. Substantial morbidity and mortality are associated with the complex because of the occurrence of cardiac myxoma. Facial and eyelid lentigines, conjunctival and caruncle pigmentation and eyelid pigmentation may precede signs or symptoms of cardiac myxoma. A study of the patient's primary relatives disclosed manifestations of the complex transmitted in a manner consistent with mendelian autosomal dominant inheritance.

Adult↗
PubMed1991

Inhibitory neuropeptides and intrinsic inhibitory innervation of descending human colon.

The effects of aging on inhibitory neuropeptide concentrations and intrinsic inhibitory innervation of circular muscle were investigated using normal descending colon obtained at surgery. Immunoreactive vasoactive intestinal peptide, peptide histidine-methionine, met5-enkephalin, neuropeptide Y, and somatostatin were extracted from specimens of muscularis externa (patient ages: 19-84 years) and measured by radioimmunoassay. Intracellular electrical activity was recorded from strips of circular muscle (patients ages: 49-84 years) using glass microelectrodes; inhibitory junction potentials were evoked by electrical field stimulation. There were no significant differences (t tests: P greater than 0.05) between neuropeptide concentrations in patients less than 70 years old (N = 28) compared to patients greater than or equal to 70 years old (N = 12). However, the amplitude of inhibitory junction potentials declined with increasing patient age (r = -0.58, P = 0.02, N = 16), with no change in resting membrane potentials (r = 0.22; P greater than 0.05). The decline in amplitude in women (r = -0.68, P = 0.03, N = 9) preceded the decline in men (r = -0.62, P = 0.10, N = 7). Age-related decline in inhibitory junction potentials could be related to decreased: density of inhibitory nerves, release of inhibitory neurotransmitter, density of binding sites for inhibitory neurotransmitter on smooth muscle, or a combination thereof. Alternatively, this decline might represent a change in interaction of inhibitory neurotransmitter with the smooth muscle membrane, such as a change in coupling of binding site with the potassium channel, decreased number of potassium channels, or altered permeability of the potassium channel.

Adult↗
PubMed1991

Mannan estimation as a measure of the growth of Candida albicans.

Determination of Candida albicans mannan levels by an enzyme-linked immunosorbent assay was compared with dry weight measurement and viable counting as a means to estimate growth of the fungus in broth cultures. Levels of extracted mannan showed good correlation with fungal biomass (dry weight) (r = 0.984) and, although biomass and mannan levels correlated with viable counts, the correlation was not as strong (r = 0.954-0.959). The technique offered an accurate alternative to viable counts in the brain and kidney when mannan estimation was used to follow the distribution of C. albicans in an experimental model of systemic candidosis in mice. Mannan levels were less useful in following the course of the infection in the liver and spleen where accumulation of free and serum-bound mannan was a problem. Mannan estimation would therefore seem to offer an accurate alternative to viable counts for determining the quantity of Candida both in vitro and in certain infected organs of experimental animals, where measurement of fungal biomass is not feasible and viable counts can be grossly inaccurate.

Animals↗
PubMed1991

Myxoid fibroadenoma and allied conditions (myxomatosis) of the breast. A heritable disorder with special associations including cardiac and cutaneous myxomas.

Among 145 patients with the complex of myxomas, spotty pigmentation, endocrine overactivity, and psammomatous melanotic schwannomas, 31 (21%) had mammary lesions. The ages of these 26 females and five males ranged from 6 to 64 years (mean, 30 years). Five patients had breast symptoms. In 21 (81%) of the females, benign mesenchymal lesion(s) were detected pathologically. These were characterized by accumulations of large amounts of ground substance in the lobules that alterated the stroma to a very loose and myxoid tissue. The change involved single lobules (lobular myxoid change), small groups of lobules (nodular myxoid change), and large aggregates of lobules (myxoid fibroadenoma); the interlobular stroma was affected to a lesser degree. The lesions were multicentric and bilateral in eight patients (38%). Because the myxoid breast lesions were familial, were frequent findings in the complex, and were similar histologically to the cardiac and cutaneous myxomas in the complex, they undoubtedly are a component and a pathologic marker of the complex. They were the presenting feature of the complex in six patients (19%). Therefore, discovery of the myxoid breast lesions on pathologic examination should raise suspicion of the complex, and affected patients (and their primary relatives) should be evaluated accordingly.

Adenofibroma↗
PubMed1991

Ductal adenoma of the breast with tubular features. A probable component of the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas.

The features of ductal adenoma of the breast, a solid intraductal tumor, include the following: arrays of long, straight, narrow, roughly parallel tubules composed of distinct epithelial and myoepithelial cells; a modest amount of fibrous tissue that separates the ducts from one another; and a fibrous capsule. We found this neoplasm in four women (ages 27 through 61 years) who had the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas, an autosomal dominant familial syndrome. The lesion was bilateral in two of the women. Each of the patients had mammary myxoid mesenchymal lesions typical of the complex. Two tumors were symptomatic (bloody nipple discharge); the four others were not. Five of the six tumors formed palpable masses that were located close to the areola. The mammograms suggested carcinoma. On microscopic examination, four of the six adenomas were mistaken for carcinoma; none recurred or metastasized. Circumstantial evidence suggests that the ductal adenoma of the breast is a component of the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas.

Adenoma↗
PubMed1990

Prevalence of columnar-lined (Barrett's) esophagus. Comparison of population-based clinical and autopsy findings.

In this study, two different methods were used to investigate the prevalence of columnar-lined (Barrett's) esophagus. First, a population-based study of clinically diagnosed cases was performed in Olmsted County, Minnesota. Twenty-five residents of this county, who had undergone endoscopy and biopsy between 1969 and 1986, were diagnosed as having Barrett's esophagus. On January 1, 1987, 17 of these patients were still living in the county, representing an age- and sex-adjusted prevalence rate of 22.6 cases per 100,000 population (95% confidence interval, 11.7-33.6 cases). A prospective search of Mayo Clinic autopsy material for Barrett's esophagus was conducted using the same diagnostic criteria as in the clinical study. Over an 18-month period ending in September 1987, 7 cases of Barrett's esophagus were found in 733 unselected autopsies. In 5 of the 7 cases, Barrett's esophagus was first detected at the time of autopsy. Using the age- and sex-specific prevalence from the clinically diagnosed study, researchers expected to find 0.19 cases of Barrett's esophagus at the 226 autopsies performed on Olmsted County residents, although 4 were actually observed (P less than 0.001). This approximately 21-fold increase (95% confidence interval, 5-54 cases) corresponds to an autopsy estimated prevalence of 376 cases per 100,000 population (95% confidence interval, 95-967 cases). In conclusion, a majority of cases of Barrett's esophagus, a condition that predisposes to esophageal malignancy, remains unrecognized in the general population.

Aged↗
PubMed1990

Altered inhibitory innervation of circular smooth muscle in Crohn's colitis. Association with decreased vasoactive intestinal polypeptide levels.

To determine whether decreased tissue vasoactive intestinal polypeptide levels might affect inhibitory neural input, fresh colonic specimens were obtained from patients with Crohn's colitis (n = 7) and normal subjects (n = 13). Immunoreactive vasoactive intestinal polypeptide levels were measured in the muscularis externa by radioimmunoassay and localized in tissue sections by immunostaining. Circular muscle strips were maintained in an organ bath; inhibitory junction potentials evoked by short- and long-duration field stimulation and resting membrane potentials were recorded using intracellular impalements. In Crohn's colitis, vasoactive intestinal polypeptide levels displayed a bimodal distribution in which 3 specimens had vasoactive intestinal polypeptide levels greater than or equal to 4 SE lower than the mean in normal specimens. In 3 specimens from Crohn's colitis with decreased vasoactive intestinal polypeptide levels, immunoreactive material was absent from the circular muscle layer and the myenteric plexus. Mean resting membrane potentials, mean amplitude of inhibitory junction potentials evoked by short-duration stimulation, and mean amplitude of initial inhibitory junction potentials evoked by long-duration stimulation were not different between the two groups. However, the mean amplitude of the 60th inhibitory junction potential during prolonged stimulation was decreased (p less than 0.01) in Crohn's colitis (6 mV) compared with normal specimens (11 mV). These results show that diminished neural input to circular muscle in Crohn's colitis was associated with decreased extractable vasoactive intestinal polypeptide levels and decreased staining of nerve fibers containing vasoactive intestinal polypeptide.

Adolescent↗
PubMed1990

Metastatic ossified gastric carcinoid with hypergastrinemia associated with gastric and thyroid autoantibodies.

A 42-year-old white woman was seen by her physician because of somatic complaints of the neck and right arm discomfort, difficulty in swallowing, and "heartburn." Findings of the workup led to the diagnosis of metastatic ossified gastric carcinoid. Review of the literature suggests that this is the third report of ossified gastric carcinoid. However, this is the only case in which such a tumor was associated with hypergastrinemia, gastric (antiparietal cell), and thyroid (antimicrosomal) autoantibodies.

Adult↗
PubMed1990

Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome.

Schwannoma, a benign tumor of peripheral nerve sheath, infrequently contains melanin and even less frequently features psammoma bodies. Forty schwannomas that displayed both findings were obtained from 31 patients, aged 10 to 63 years. Seventeen patients (55%) had the complex of myxomas, spotty pigmentation, and endocrine overactivity, a familial syndrome. Six (19%) had multiple tumors. Tumor sites included posterior spinal nerve roots (11 tumors), alimentary tract (11 tumors), and bone (three tumors). Two-thirds of the neoplasms caused symptoms. Grossly, the tumors were black, brown, or blue, encapsulated, and solid or spongy. Microscopically, they were circumscribed but incompletely encapsulated; they contained spindle and epithelioid cells, melanin, psammoma bodies, and fat. The spindle cells were arranged in interlacing fascicles, and exhibited whorling and occasional nuclear palisading. Immunostaining revealed that the tumors were positive for S-100 protein and vimentin and negative for glial fibrillary acidic protein, actin, and keratin. Ultrastructurally, the cells features elongated processes, continuous basal lamina, melanosomes, and intercellular long-spacing collagen. Twenty-one patients (68%) were alive without evidence of the neoplasm; two of these each had two local recurrences. Seven patients died, three (10%) as a result of metastasis. Three patients were lost to follow-up.

Acromegaly↗
PubMed1989

Cytologic features of fine-needle aspirates of hyalinizing trabecular adenoma of the thyroid.

The cytologic features of five cases of hyalinizing trabecular adenoma of the thyroid are reviewed. All of these cases had been initially interpreted as either suspicious or positive for malignancy. On retrospective review, many of the cytologic features did mimic those of other lesions, particularly papillary carcinoma. The smears tended to hypercellularity, and nuclei were often slightly enlarged with pale chromatin, intranuclear holes, and longitudinal grooving of the nuclear membrane. On the other hand, some cytologic features were more suggestive of medullary carcinoma--poor cohesion of cells with elongated and spindle cell forms and hyaline acellular areas. Awareness of the histologic and cytologic features of hyalinizing trabecular adenoma may enable the cytopathologist to avoid a false positive diagnosis of papillary carcinoma or medullary carcinoma, although it is unrealistic to expect a definitive diagnosis of hyalinizing trabecular adenoma.

Adenoma↗
PubMed1989

Thyroid cysts.

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Cysts↗
PubMed1989

Long-term follow-up of a kindred with multiple endocrine neoplasia type IIA.

A kindred with multiple endocrine neoplasia type IIA was studied. In four of five fully documented cases, patients presented with adrenal medullary disease during the second or third decade; in three cases, medullary thyroid carcinoma was not diagnosed until the fourth decade. This kindred seems to have an aggressive variant of adrenal medullary disease in which clinical presentation is early, metastasis occurs, and oncological cure is uncommon. Early detection and operation were important, and symptoms were controlled with operations and alpha- and beta-adrenergic blockade in uncured members.

Adolescent↗
PubMed1989

Deoxyribonucleic acid ploidy pattern of cardiac myxomas. Another predictor of biologically unusual myxomas.

A group of patients with cardiac myxoma who have a heritable syndrome involving skin myxomas, endocrine tumors, and lentiginosis--the complex of myxomas, spotty pigmentation, and endocrine overactivity--has been described previously. Patients with the complex had cardiac myxomas at an early age (average, 26 years) with frequent multiple myxomas (53%) and recurrent cardiac myxomas (22%); however, no histologic differences were noted when these tumors were compared with sporadic cardiac myxomas. In the present study, deoxyribonucleic acid flow cytometric analyses of 35 cardiac myxoma specimens were correlated with clinical findings (mean duration of follow-up, 13 years). Among 30 patients with sporadic (nonfamilial) cardiac myxoma, 24 (80%) had a normal (deoxyribonucleic acid diploid) ploidy pattern, and six (20%) had an abnormal (deoxyribonucleic acid tetraploid) pattern. Specimens from each of the five patients with the complex had abnormal deoxyribonucleic acid tetraploid patterns (p = 0.002 compared with the sporadic myxoma group). Further, all four patients who had recurrent cardiac myxoma had an abnormal deoxyribonucleic acid ploidy pattern (p = 0.007 compared with patients with nonrecurrent myxomas). Unlike conventional histologic examination, the ploidy pattern of cardiac myxomas seems to be sensitive for detecting biologically unusual tumors, and a deoxyribonucleic acid tetraploid pattern suggests a high risk of recurrence.

Biomarkers, Tumor↗