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Biomedical subjects

A Gordon

Publications and source records attributed to A Gordon.

At least 181 records · Page 10Linked to original sources

X linked mental retardation: a family with a separate syndrome?

Four males with X linked mental retardation are described. Manifestations similar to those seen in the FG syndrome include severe constipation, tall, broad foreheads, hypotonia, and cowlicks of the hair line, but no individual patient had all the features of the syndrome and none had macrocephaly. The facial appearance was distinctive but different from that seen in the FG syndrome. The cases are presented in order to discuss the phenotypic limits of the FG syndrome and to consider the need to separate other distinct but similar entities.

Female↗

Primary cerebral lymphoma presenting with cranial diabetes insipidus.

A 30 year old woman with an 8-year history of thirst and polyuria was found to have cranial diabetes insipidus. There were no neurological abnormalities at presentation but she subsequently developed diverse signs and died 26 months later. Autopsy revealed a diagnosis of diffuse primary cerebral lymphoma. Cranial diabetes insipidus with otherwise minimal abnormality of hypothalamic/pituitary function has not previously been reported as a presentation of this neoplasm.

Adult↗

Differentiation in medulloblastomas and other primitive neuroectodermal tumours.

Fifty-four cases of primitive neuroectodermal tumour (PNET) including 47 medulloblastomas, were examined for evidence of neuronal and glial differentiation, using antibodies to neuron-specific enolase (NSE), neurofilament protein (NF), and glial fibrillary acidic protein (GFAP). In 30 of the cases, antibodies to vimentin, alphafetoprotein, cytokeratin, epithelial membrane antigen and lymphoid markers were also used. Most of the 47 medulloblastomas in the group were NSE positive but NF negative; about half were GFAP positive and three of them were positive for both neuronal markers and for GFAP. Vimentin was demonstrated in four cases and was not always co-expressed with GFAP. Medulloblastomas were negative for all the other markers. Supratentorial PNETs were sometimes positive with neuronal markers but were GFAP negative. The cell specificity of these markers and the interpretation of immunocytochemical findings are discussed in relation to differentiation potential in primitive neuroectodermal tumours.

Adolescent↗

Pleomorphic xanthoastrocytoma. Report of four cases.

Four cases of pleomorphic xanthoastrocytoma (PXA), a low-grade leptomeningeal glioma with a reported favorable prognosis affecting young patients, are reported together with a discussion and review of management and prognosis. A literature review has confirmed a favorable prognosis in at least 50% of patients with this disorder. Seventeen of 35 reported patients are still alive and often seizure-free for a mean period of 7.4 years (range 2 to 18 years) after diagnosis. Five patients have died within 2 years and four between 9 and 25 years after diagnosis of PXA. In some cases in which death followed shortly after diagnosis, there may have been histological confusion between PXA and a malignant glioma with heavily lipidized tumor cells. Nonetheless, transformation of PXA into a malignant astrocytoma or glioblastoma with eventual death may occur many years after initial diagnosis. From the currently reported cases it does not appear possible on clinicopathological grounds to predict which patients will have a favorable prognosis. Optimal management of PXA seems to be primary surgical resection with later surgery for residual or recurrent tumor. The role of radiotherapy in the management of PXA is at this time uncertain.

Adolescent↗

Malaria treatment practices among mothers in Guinea.

A survey of mothers of young children in Guinea, West Africa (June-July 1988) suggests that individual, sociocultural, and structural factors influence the use of antimalarials for episodes of fever presumed to be malaria. A survey of 243 mothers was carried out in eight different sites (four urban and four rural) in two regions of the country. Among mothers interviewed, use of Western antimalarials for infants and children with fever was acceptable, but access to treatment was problematic. Mothers interviewed were less likely to use antimalarials during pregnancy, partly because of certain misconceptions about their effects and lack of access to the medication. Despite programmatic efforts to promote practices of presumptive treatment of fever among young children and chemoprophylaxis during pregnancy among mothers in towns and villages surveyed, variables associated with low use of antimalarials by mothers and their young children reflect important barriers to the implementation of child survival initiatives.

Adult↗

Solubility of neurofibrillary tangles and ultrastructure of paired helical filaments in sodium dodecylsulphate.

Temporal cortex from 14 cases of Alzheimer-type dementia and 6 cases of Down's syndrome, all selected for severe Alzheimer pathology, was homogenised in distilled water, NaOH, or sodium dodecylsulphate (SDS) containing 0.1% beta-mercaptoethanol. The homogenates were stained with Congo red, and the neurofibrillary tangles and plaque cores were counted under crossed-polarisation microscopy. The number of tangles and plaque cores in the water-treated extracts was not related to age, sex, post-mortem interval or duration of dementia. The number of tangles after extraction in SDS or NaOH, as a percentage of tangles in water-treated extracts, was 57 +/- 25 (mean +/- SD) for 1% SDS, 43 +/- 17 for 5% SDS and 37 +/- 22 for 0.2 M NaOH. Plaque cores were essentially insoluble in all three agents. The percentage of tangles insoluble in 1% SDS did not correlate with age or post-mortem interval but decreased with increasing duration of dementia. Enhanced tangle solubility with increasing duration of dementia suggests that the nature of tangles changes with time; one possibility is that this reflects transformation of intracellular to extracellular tangles. Paired helical filament (PHF) length and the number of repeats per PHF were measured in electron micrographs of PHF prepared with and without treatment by 1% SDS. There was no significant multimodality of PHF length to suggest that PHF broke at regular intervals. The mean repeat length (PHF length/number of repeats) was greater for PHF isolated in the presence of 1% SDS than in its absence, showing that SDS affects ultrastructure by untwisting PHF.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

"Pseudocatheter" due to inadvertent ventricular catheter placement.

Ultrasound is frequently used in the routine follow-up of patients who have been shunted for obstructive hydrocephalus. We report a case with "pseudocatheter" appearance due to the initial incorrect placement of the ventricular catheter. To our knowledge, such an appearance has not been previously described.

Catheters, Indwelling↗

Multicentric glioma presenting with hemorrhage.

We report a case of multicentric malignant astrocytoma occurring simultaneously in both cerebrum and cerebellum, presenting with hemorrhage into the cerebellar tumor. Hemorrhage into a cerebellar glioma in an adult is very rare. None of the cases of multicentric glioma described in the literature presented with intratumoral hemorrhage. This combination in our patient has prompted us to report this case and to reaffirm the need to consider the diagnosis of malignant glioma in patients with multiple intracranial tumors.

Adult↗

Cultured lymphocytes from alcoholic subjects have altered cAMP signal transduction.

Previous work has shown that freshly isolated lymphocytes from alcoholic subjects show significantly reduced basal and adenosine receptor-stimulated cAMP levels. This decrease could be due to ethanol-induced cellular adaptation or to a genetic difference in the regulation of cAMP signal transduction. Therefore, we cultured human lymphocytes in defined medium without ethanol for 7-8 days and then examined differences in receptor-dependent cAMP accumulation between lymphocytes from alcoholic and nonalcoholic subjects. After four to six generations in culture without ethanol, lymphocytes from alcoholic subjects have significantly higher cAMP levels than do cells from nonalcoholic subjects. Thus, a difference in cAMP signal transduction is demonstrable in cells from alcoholic subjects grown without ethanol. We also found that cultured lymphocytes from both alcoholic and nonalcoholic subjects show a decrease in receptor-stimulated cAMP levels after exposure to 200 mM ethanol for 48 hr. To determine whether alcoholic subjects have increased sensitivity to ethanol, lymphocytes were exposed to only 100 mM ethanol for 24 hr. Under these conditions, receptor-dependent cAMP levels did not change in cells from nonalcoholic subjects. However, lymphocytes from alcoholic subjects showed a 39% decrease (P less than 0.003) in adenosine receptor-stimulated cAMP levels. Taken together, the results show that (i) chronic ethanol treatment in culture reproduces the suppression of cAMP levels found in circulating lymphocytes from alcoholic subjects and (ii) despite four to six cell divisions in culture without ethanol, lymphocytes from alcoholic subjects exhibit significantly increased adenosine receptor-dependent cAMP levels and increased sensitivity to chronic exposure to ethanol. These findings suggest that the suppression of cAMP levels observed in freshly isolated lymphocytes from alcoholic subjects results from both a direct effect of chronic exposure to ethanol and a genetic difference leading to altered cAMP signal transduction.

Adenosine Deaminase↗

Prospective screening for thimerosal hypersensitivity: a pilot study.

This pilot study was conducted to determine if prospective patch-test screening could predict ocular tolerance to a thimerosal-preserved chemical disinfection system. Thirty patients were patch tested for thimerosal hypersensitivity before beginning soft contact lens wear. Only one patient exhibited a positive prospective patch test and subsequently developed an ocular thimerosal hypersensitivity. Two other patients with negative patch tests developed thimerosal hypersensitivity reactions over the 1-year study duration. Prospective patch testing for thimerosal hypersensitivity was neither clinically valuable nor sensitive in this particular sample.

Adolescent↗

Use of immunohistochemical methods in the differential diagnosis between primary cerebellar haemangioblastoma and metastatic renal carcinoma.

The potential role of immunohistochemistry in making the distinction between primary cerebellar haemangioblastoma and metastatic renal carcinoma was investigated by examining the reaction pattern of 10 cerebellar haemangioblastomas (seven women, three men, aged 20-40 years) and 10 primary renal carcinomas (six men, four women, aged 49-82 years) to a panel of epithelial, glial, and neural/neuroendocrine antisera. The tumour cell membranes of the renal carcinomas stained strongly with epithelial membrane antigen (EMA); membrane staining was totally absent in the haemangioblastomas. Strong neurone specific enolase (NSE) and S100 staining were also seen in haemangioblastomas but were more variable than EMA staining in renal carcinomas. It is concluded that a panel of antisera is required to distinguish between histologically similar areas in primary haemangioblastomas and metastatic renal carcinomas, and that while complementing conventional histological techniques, new problems of interpretation result which must be taken into account.

Adult↗

Striated muscle fibers differentiate in primary cultures of adult anterior pituitary cells.

Anterior pituitary cells from adult male rats were cultured on a natural extracellular matrix (ECM). From the 5th day, spindle-shaped cells, fusing to form elongated fibers, were observed among the epithelial cells. These fibers later increased in size and number and were identified as striated muscle fibers, based on their multi-nucleation, cross-striation and rhythmic contraction. Striated muscle development was further validated by the appearance of cholinergic-nicotinic receptors, as demonstrated by the binding of 125I-alpha bungarotoxin (a-Btx). The effect of various supplements added to the cultures on a-Btx binding was used as a measure of muscle cell differentiation. ECM and serum were essential for myotube formation. Insulin (or IGF-I) and cortisol synergistically caused a 20-fold increase in myogenesis. FGF and EGF were effective only in the presence of insulin and cortisol. The data suggest that: (a) cells, derived from adult pituitary, differentiate, in primary culture, into striated muscle fibers; (b) the induction of this differentiation is multi-factorial; ECM, serum, insulin and glucocorticoids facilitate this myogenesis.

Animals↗

A randomised prospective comparison of cefotaxime versus netilmicin/penicillin for treatment of suspected neonatal sepsis.

In an open prospective study performed in 2 neonatal units, infants with suspected neonatal sepsis (SNS) of unknown microbial cause were randomly allocated to receive treatment with either cefotaxime (CTX) or netilmicin plus penicillin (N + P). 236 patients were entered into the trial, of whom 222 were evaluable. The number of 'definitely' and 'probably' infected babies was similar in both groups. There was no difference in clinical outcome between patients in the 2 treatment groups and no side effects were recorded for either of the antibiotic regimens. Antibiotic sensitivity testing of bacterial isolates from peripheral sites showed almost universal sensitivity of potential pathogens to both antibiotic regimens at the start of treatment in all infants. Thereafter, organisms resistant to CTX were isolated from patients in both treatment groups, possibly reflecting the antibiotic sensitivity profile of the colonising bacteria in both neonatal units. The results of this study indicate that either CTX or N + P are suitable, in our units, for the 'blind' treatment of early SNS. In units where listerial infections are prevalent, specific cover should be added to CTX. For SNS developing after admission, the choice of antibiotics will depend upon the background antibiotic sensitivity profile of the colonising bacteria.

Bacterial Infections↗

Optometric services and private health insurance.

This article provides an overview of the private health insurance system and its relevance to optometric practice. The extent of private health coverage in the U.S. and the projected decline of fee-for-service income mandates a working knowledge of the insurance system by optometrists and their staff members. The coverage of eye examinations and related services is dependent upon the diagnosis. Using a well-designed fee slip will expedite claims processing for patients and reduce insurance paperwork for the practitioner. Fee slip design and implementation are discussed in detail and illustrated with clinical examples.

Adult↗

Mood-related obsessive-compulsive symptoms in a patient with bipolar affective disorder.

The authors present a case of coexisting obsessive-compulsive disorder (OCD) and bipolar affective disorder in which the obsessive-compulsive symptoms disappeared during episodes of mania and reappeared during periods of depression. Although patients with coexisting bipolar disorder and OCD are relatively rare, careful study of those patients may increase our understanding of the complex relationship between obsessive-compulsive symptoms and mood. Abnormalities in serotonergic neurotransmission have been postulated in both affective disorders and OCD and may provide important clues to the pathophysiology of OCD.

Bipolar Disorder↗

Management of monocular polyopia using an artificial iris contact lens.

Monocular polyopia is a relatively uncommon symptom reported to the ophthalmic practitioner. The specific etiology can be difficult to ascertain and management options may be unsatisfactory to both patient and clinician. A case is presented of a low vision patient with monocular polyopia secondary to lenticular changes. The patient was successfully managed with an opaque, artificial iris soft contact lens.

Adult↗

Heterotopic brain cyst from middle cranial fossa to submandibular region.

The case of a newborn boy with a congenital cystic neck mass causing respiratory distress is reported. This lesion extended from the submandibular region through a bone defect to the middle cranial fossa but remained totally extradural. There was no underlying brain abnormality. The cyst was composed mainly of mature neuroglial tissue, with some ependymal and choroid plexus elements, and was diagnosed as heterotopic brain. After 8 years' follow-up, the child still has no deficits.

Brain↗

Malignant fibrous histiocytoma of the broad ligament: a case report and literature review.

A case of malignant fibrous histiocytoma arising primarily in the broad ligament is described. This entity has not been previously reported. The patient's presentation and clinical course, including her partial response to Adriamycin and DTIC chemotherapy, are outlined. Clinical and pathologic features of tumors arising in the broad ligament are discussed.

Adnexa Uteri↗