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Biomedical subjects

U Khanduri

Publications and source records attributed to U Khanduri.

17 recordsLinked to original sources
PubMed2001

Abnormal secretion and function of recombinant human factor VII as the result of modification to a calcium binding site caused by a 15-base pair insertion in the F7 gene.

A case of a novel mutation in the F7 gene that results in factor VII coagulant activity (VII:c) of less than 1% and VII antigen (VII:Ag) levels of 10% is presented. DNA analysis revealed a homozygous 15-base pair (bp) in-frame insertion-type mutation at nucleotide 10554. This insertion consisted of a duplication of residues leucine (L)213 to aspartic acid (D)217 (leucine, serine, glutamic acid, histidine, and aspartic acid), probably arising by slipped mispairing between 2 copies of a direct repeat (GCGAGCACGAC) separated by 4 bp. Molecular graphic analyses showed that the insertion is located at the surface of the catalytic domain in an exposed loop stabilized by extensive salt-bridge and hydrogen bond formation at which the calcium binding site is located. The mutation probably interferes with protein folding during VII biosynthesis and/or diminishes functional activity through the loss of calcium binding. In vitro expression studies demonstrated that the levels of VII:Ag in lysates of cells transfected with wild type VII (VIIWT) were equivalent to those with mutant type VII (VIIMT), but the level of secreted VIIMT was 5% to 10% that of VIIWT. Pulse chase studies demonstrated that VIIMT did not accumulate intracellularly, and studies with inhibitors of protein degradation showed that recombinant VIIMT was partially degraded in the pre-Golgi compartment. Accordingly, only small amounts of VIIMT with undetectable procoagulant activity were secreted into conditioned media. These results demonstrate that a combination of secretion and functional defects is the mechanism whereby this insertion causes VII deficiency.

Base Pair Mismatch↗
PubMed2000

Acute myelomonocytic leukaemia with pseudo-Chediak-Higashi inclusions in leukaemic blasts report of an unusual case.

Giant inclusions in the blast cells resembling that of Chediak-Higashi syndrome have been reported only in a handful of cases with acute leukaemia. There is a paucity of data on the ultrastructural appearance of such unique light microscopic findings. As many of such patients were reported in late seventies and early eighties [1-4] the outcome of treatment using present day multiple induction/consolidation regimens in such patients has also been rarely reported due to the rarity of such patients. We report here on a patient with acute myeloid leukaemia showing pseudo-Chediak-Higashi inclusions in leukaemic blasts with unusual ultrastructural morphology.

Adolescent↗
PubMed1993

Developing hemophilia services in India.

With a population of 853 million there should be 51,204 patients with hemophilia A in India assuming a prevalence of 6/100,000 population. With the current birth rate of 32/1000, 1,300 new patients with hemophilia A will be born each year. Hospital based data suggests that hemophiliacs in India suffer from preventable morbidity because doctors do not know enough about the disease and its management, because laboratory diagnostic facilities are inadequate and because there is not enough therapeutic material or even if it is available the patients do not have the resources to purchase it. This article reviews the current status of hemophilia in India and suggests measures to improve hemophilia services within the health care infrastructure available in the country.

Adolescent↗
PubMed1993

Cerebro-spinal fluid cytomorphology in tuberculous meningitis--a preliminary report.

The morphology of cells in centrifuged cerebrospinal fluid (C.S.F.) of 25 patients with tuberculous meningitis (T.B.M.) in different stages of the illness is presented. Blood brain barrier studies were also done in 6 patients. Maximum variability of cells in the initial stages of the illness and persistence of polymorphs in the later stages of the illness is noted. No correlation was established between cerebrospinal fluid cytomorphology and complications of the illness.

Humans↗
PubMed1992

Intraobserver and interobserver reproducibility of the FAB classification in acute leukaemia.

Intraobserver and interobserver reproducibility of FAB classification for acute leukaemia was assessed using the modified criteria of the FAB classification. Leishman stained peripheral smear and May Grunwald Giemsa stained bone marrow smears from 72 cases of acute leukaemia were used for this purpose. Cytochemical stains used were peroxidase, PAS and Sudan black B. Intraobserver and interobserver concordance/discordance was calculated. Kappa statistic was used to correct the chance expected agreement. Intraobserver and interobserver concordance was 76% which improved to 91% when cytochemistry was included. Lymphocytic/Nonlymphocytic concordance was 87.5% and 90% respectively for intraobserver and interobserver groups.

Blood Cells↗
PubMed1991

Anti-streptokinase levels in Indian patients.

Levels of anti-streptokinase antibodies were measured in 75 Indian patients who were divided into three groups. The first group consisted of 25 healthy blood donors; the second group of 25 patients with ischaemic heart disease with stable angina; and the third group of 25 patients with acute myocardial infarction. The mean level of anti-streptokinase for the entire group was 0.37 SD 0.20 million international units (IU) (range 0.09 to 1.15 million IU). There was no significant difference in the anti-streptokinase levels among the three groups. In order to neutralise the anti-streptokinase levels in 90% of the study population, 0.57 million IU of streptokinase was necessary. These levels are more than twice the current Western levels. In the light of this study, it may be necessary to reconsider the adequacy of the conventional dosage of streptokinase while treating acute infarctions in India and, possibly, other tropical countries where prevalence of prior streptococcal infection is high.

Adult↗
PubMed1991

The indirect platelet suspension immunofluorescence test in the detection of platelet antibodies in idiopathic thrombocytopenic purpura.

Among the various techniques developed for the detection of platelet antibodies, the platelet suspension immunofluorescence test has been reported to be simple, sensitive and reproducible, and therefore more clinically useful than other techniques available. An initial evaluation of the test was carried out for the detection of platelet autoantibodies in ten cases of idiopathic thrombocytopenic purpura. The indirect PSIFT was found to be positive in 60%. The technical aspects of the test and the problems encountered are discussed.

Adult↗
PubMed1989

Slow thaw siphon technique for cryoprecipitate production.

The efficacy of the slow thaw siphon technique for making cryoprecipitate was evaluated. A hundred consecutive bags of cryoprecipitate were subjected to analysis. The average Factor VIII:C level in donors plasma was 1.02 IU/ml with slightly lower levels of Factor VIII:C in O group persons and in the older age group. The average Factor VIII:C content per bag of cryoprecipitate was 132 units with the average Factor VIII:C yield of 57.8 per cent. Recovery of the cryoprecipitates made by this method was tested in vivo in ten haemophiliacs with less than 1 per cent activity. An average increment of 1.91 per cent in Factor VIII:C levels was obtained by infusion of 1 IU/kg body weight.

Adolescent↗
PubMed1986

Triton X-114 phase separation of platelet membrane glycoproteins from normal subjects and a patient with type I thrombasthenia.

Surface-labelled normal and thrombasthenic platelets have been subjected to phase separation in Triton X-114. Triton-rich and Triton-poor fractions have been analysed by SDS-PAGE and IEF-SDS-PAGE. Partitioning characteristics of the major glycoproteins have been defined. The Triton-rich fraction contained GPIIb, III, IV, VI, VII, VIII, GP38 and the IIb beta subunit. In contrast, the Triton-poor fraction contained the HMWGP, GPIa, Ib, IIb, III, V and GPIX. Analysis of the platelet membrane glycoproteins of a patient with Type 1 thrombasthenia has been carried out using Triton X-114. The value of the method in diagnosis of this condition and differences between our findings and those published previously are discussed.

Blood Platelet Disorders↗
PubMed1981

Glanzmann's thrombasthenia. A review and report of 42 cases from South India.

In the 14 year period from June, 1966 to June, 1980, 42 cases of Glanzmann's thrombasthenia have been diagnosed, all fulfilling the criteria of prolonged bleeding time, with normal venous platelet count, defective clot retraction and decreased platelet aggregation, associated with a lifelong bleeding tendency. Few cases have been reported from India though it is the fourth most common congenital bleeding disorder among the patients seen at the Christian Medical College Hospital, Vellore. The large number of such cases found in South India as compared with reports from other parts of the world may be due to the high degree of consanguinity which is part of the accepted culture in this area. Reliable diagnosis of this condition is possible with fairly simple laboratory procedures.

Adolescent↗
PubMed1981

Essential athrombia. A report on 4 cases from South India.

Four cases of essential athrombia have been diagnosed in the 14 year period from June, 1966 to June, 1980 at the Coagulation Laboratory of the Christian Medical College Hospital, Vellore. All cases fit Inceman's description of the original case and fulfill the diagnostic criteria of prolonged bleeding time, decreased platelet aggregation, normal platelet count, normal morphology and normal clot retraction. No cases have been reported from India in the past 15 years.

Adolescent↗
PubMedDate not supplied

Hereditary factor XIII deficiency.

Twelve cases of hereditary factor XIII (FX III) deficiency diagnosed over five years (1986-1990) at Christian Medical College and Hospital, Vellore are presented here. Although all the cases had a history of umbilical cord bleeding and subsequent frequent bleeding episodes, diagnosis was considerably delayed. All but two patients required transfusions for bleeding episodes. Ten patients had a history of consanguinity in parents. Clinical features and family history are described in detail here. The ease of performing the Urea solubility test and problems in it's interpretation are highlighted. The role of prophylactic transfusion is also discussed.

Adolescent↗
PubMedDate not supplied

Surgery in patients with congenital coagulation disorders.

BACKGROUND: Surgery is occasionally necessary in patients with congenital coagulation disorders. Major surgery for patients with haemophilia was not being done in India until recently. This paper reports the experience of a single referral centre. METHODS: The data of 52 patients who were operated upon were collected from the hospital records retrospectively between 1984 and 1986 and prospectively thereafter. They included the surgical procedure performed, replacement therapy used and complications encountered. RESULTS: Fifty-nine procedures were performed of which 26 were major, 30 minor and 3 were diagnostic angiograms. Blood components produced in the hospital blood bank were commonly used for replacement and primary haemostasis was achieved in all patients. Delayed bleeding due to inadequate factor levels occurred in 12 procedures and was controlled by increasing the factor replacement. One patient died of suspected acute myocardial ischaemia. CONCLUSION: In India surgical procedures can be safely performed in patients with congenital coagulation disorders.

Adolescent↗