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Biomedical subjects

A Kher

Publications and source records attributed to A Kher.

At least 91 records · Page 5Linked to original sources
PubMed1969

Diktyoma.

Explore the source record for details and available documents.

Child, Preschool↗
PubMedDate not supplied

Spondylocostal dysostosis.

Spondylocostal dysostosis is a rare condition characterized by short stature due to a short trunk, multiple morphological abnormalities of the vertebrae and ribs due to malsegmentation of the axial skeleton. Radiological features include reduced number of vertebrae and ribs, hemivertebrae, fused or sagitally cleft vertebrae or multiple rib fusions. Three distinct clinical entities are described. We report nine cases of this syndrome, seven were infants and presented with an abnormal shape of the thorax. Two of them had a meningocoele, and one succumbed to the CNS anomalies on the third day of life. The other two cases were two and eight years of age. Cardiac lesion was detected in one case and renal malformations in three cases. Reduction in the rib number was present in all cases, and rib fusion in seven cases. Thoracic vertebral dysegmentation was noted in all, lumbar in three and cervical in one case. This is the first large series from India. No clear single etiology was established.

Abnormalities, Multiple↗
PubMedDate not supplied

Storage disorders presenting like mucopolysaccharidosis.

The term dysostosis multiplex is specifically applied to the group of radiological features collectively found in a number of specific metabolic disorders including the mucopolysaccharidoses, mucolipidosis, mannosidosis, fucosidosis and several other rarer conditions. We report eight cases of mannosidosis, fucosidosis and mucolipidosis with special emphasis on the differentiation from the more common mucopolysaccharidoses.

Child↗
PubMedDate not supplied

Mucolipidoses--II: A report of three cases.

Mucolipidoses II is a rare lysosomal storage disorder with autosomal recessive inheritance. There cases with typical clinical features in early infancy like coarse facial features, severe psychomotor retardation and joint contractures are being reported. All the cases had no mucopolysacchariduria. These cases had normal values of lysosomal enzymes in leucocytes but markedly increased values in serum thus confirming mucolipidoses II. Despite the fact that there is no specific treatment, genetic counselling and prenatal diagnosis is indicated.

Chromosome Aberrations↗
PubMedDate not supplied

Laboratory assessment of antithrombotic therapy: what tests and if so why?

A critical review is given of the tests available for the assessment of the action of anticoagulants, such as heparins, oral anticoagulants and direct thrombin inhibitors, in patients under antithrombotic therapy. The principle of action and the performance of the thromboplastin time (PT), the activated partial thromboplastin time (aPTT), the whole blood clotting time, the thrombin time, the ecarin clotting time and the endogenous thrombin potential (ETP) is discussed, as well as the evidence behind the accepted therapeutic ranges. The two most common tests, PT and aPTT, respond in an essentially different way to clinically effective anticoagulation with heparin and with oral anticoagulants. This means that they covariate with, but do not themselves represent the essential parameter influenced by anticoagulation. The experimental basis for the widely accepted two times prolongation of the aPTT as an indicator for adequate anticoagulation is shown to be meagre in the case of unfractionated heparin and lacking for the other anticoagulants. Common sources for error in the interpretation of anti-factor Xa- and anti-thrombin activity of heparins are indicated. Extensive experience with new tests like the ecarin clotting time and the ETP is still lacking. On the basis of preliminary data and in view of the importance of the enzymatic action of thrombin in the pathogenesis of thrombosis, the ETP is considered a possible candidate for a common parameter to assess different types of anticoagulants.

Anticoagulants↗
PubMedDate not supplied

Efficacy of fine needle capillary sampling in the diagnosis of stage III and IV cervical carcinoma.

OBJECTIVE: To test the sensitivity of fine needle capillary sampling (FNCS) as compared to scrape cytology in cervical carcinoma, stage III and IV, and to study the quality of material obtained by FNCS. STUDY DESIGN: Prospective. In 48 cases of cervical carcinoma, clinically stage III and IV, FNCS was done along with scrape cytology. The results were compared, considering histopathology as the gold standard. The quality of material obtained by both methods was compared using the parameters background, cellularity and cellular preservation. RESULTS: FNCS had a sensitivity of 87.5% as against 62.5% for scrape cytology. Material obtained by FNCS had a cleaner background and better cellularity and morphologic preservation. CONCLUSION: FNCS is superior to scrape cytology for the diagnosis of stage III and IV cervical carcinoma.

Biopsy, Needle↗
PubMedDate not supplied

[Follow-up of the treatment by direct thrombin inhibitors: activated partial thromboplastin time or ecarin clotting time].

The clinical use of the direct inhibitors of thrombin requires a reliable test to monitor the treatment and to predict the hemorragic risk. The activated partial thromboplastin time (APTT) is the most common test used to monitor treatment with unfractionated heparin. Thus APTT has been first chosen to follow patients treated with direct thrombin inhibitors, but studies have shown that it was probably not the most appropriate test. Indeed, APTT values were not well correlated with the dose administered and were dependent on the type of the thrombin inhibitor used and on the APTT reagent. The ecarin clotting time (ECT), which converts prothrombin into meizothrombin has been then tested and seemed to be a better test. In vitro studies have shown a good correlation between ECT and the different concentrations of thrombin inhibitors. Furthermore, the ECT in contrast to APTT is not sensitive to heparin or oral anticoagulant and interindividual variations are low with ECT. ECT which is a reliable test and is easy to perform seems to be a more appropriate test to monitor treatment with direct thrombin inhibitors but further studies are needed to validate its use in a clinical setting.

Animals↗
PubMedDate not supplied

MURCS association--a review of 7 cases.

MURCS association (Mullerian hypoplasia/aplasia, renal agenesis and cervicothoracic somite dysplasia) is emerging as the second most frequent cause of primary amenorrhoea after Turner syndrome. Seven cases have been described and analysed. All cases had absence of uterus and tubes 85% had cervical spine abnormalities such as vertebral fusion, hypoplasia of vertebrae or butterfly vertebrae and short stature and 28% had renal agenesis or ectopy. The latter finding is in contrast to the reports in world literature where the frequency of renal agenesis is higher. There was no familial incidence in these seven cases lending credence to the belief that the association is essentially sporadic.

Abnormalities, Multiple↗