PubMed Health⌕ Search

Biomedical subjects

A Das Gupta

Publications and source records attributed to A Das Gupta.

At least 19 recordsLinked to original sources

Mitochondrial myopathy presenting as ataxia with dilated cardiomyopathy.

Mitochondrial disorders are multisystem diseases with very heterogeneous clinical manifestations. Common cardiac features include cardiomyopathy and conduction defects. We report a five-year-old boy who presented with signs of congestive cardiac failure and was diagnosed to have dilated cardiomyopathy. Six months later, he developed progressively worsening ataxia, hypotonia, other cerebellar signs, hearing loss, severe sensory peripheral neuropathy and lactic acidosis. Electronmicroscopy of skeletal muscle biopsy was consistent with mitochondrial myopathy.

Ataxia↗

Changes in parents' mental distress after the violent death of an adolescent or young adult child: a longitudinal prospective analysis.

This study examined changes in bereaved parents' mental distress following the violent deaths of their 12- to 28-year-old children. A community-based sample of 171 bereaved mothers and 90 fathers was recruited by a review of medical examiner records. Data were collected 4, 12, and 24 months post-death. Repeated measures analysis of variance showed significant reductions in 8 of 10 measures of mental distress among mothers and 4 of 10 for fathers, with the most change for both genders occurring between 4 and 12 months post-death. During the 2nd year of bereavement, mothers' symptoms continued to decline, whereas fathers, who started out with less distress than mothers, reported slight increases in 5 of 10 symptom domains. Nonetheless, 2 years after the deaths, mothers' mental distress scores were up to 5 times higher than those of "typical" U.S. women and fathers' scores were up to 4 times higher than "typical" U.S. men. Of the 7 intervening variables examined, higher scores on self-esteem and self-efficacy predicted lower distress for both mothers and fathers 4, 12, and 24 months post-death. Repressive coping was predictive of distress among fathers. It was concluded that violent death bereavement has sustained, distressing consequences on parents of children who die as a result of accidents, homicides, and suicide.

Adaptation, Psychological↗

Broad-spectrum group treatment for parents bereaved by the violent deaths of their 12- to 28-year-old children: a randomized controlled trial.

This study assessed the efficacy of a 10-week broad-spectrum intervention offered to bereaved parents about 4 months after the deaths of their 12--28-year-old children due to accidents, homicide, or suicide. For three outcomes of distress there was a significant interaction between treatment and baseline values for each outcome for mothers both immediately posttreatment and 6 months later. The intervention appeared to be the most beneficial for mothers most distressed at baseline. Fathers showed no immediate benefits of treatment. Further research is needed to investigate these unexpected results for fathers and to further characterize those who benefit from similar programs.

Adolescent↗

Transferrin receptor expression by blast cells in acute lymphoblastic leukemia correlates with white cell count & immunophenotype.

Transferrin receptor (TR) expression by blast cells in 127 cases of acute lymphoblastic leukemia (ALL) at presentation and 19 cases at relapse was examined using three anti-TR monoclonal antibodies to find its correlation with prognostic features such as the total leucocyte count (TLC), the morphology of blast cells and their cytochemical and immunophenotypic properties, as well as age and sex of the patients. Blasts in 62 per cent of thymic (T) ALL cases at presentation showed significant TR expression as compared to only 10.9 per cent in common ALL (CALL) (P < 0.001). This differential expression of TR was also observed among cases with > 50 x 10(9)/l TLC, while in cases with < 50 x 10(9)/ l TLC no such pattern was observed (30% TR positivity in T-ALL vs 20% TR positivity in non-T-ALL). Furthermore, the percentage of TR positive blasts was significantly higher (P < 0.005) in cases with > 50 x 10(9)/l TLC as compared to those with < 50 x 10(9)/l (48.3-54.4% vs 24.9-28.8%). In contrast to CALL cases at presentation, those at relapse showed a very high TR positivity (54-66%), similar to the T-ALL cases (53-84%). This suggests a high proliferative rate of blast cells in ALL at relapse, irrespective of its immunophenotype. There was no correlation of TR expression with blast cell morphology (FAB L1 vs L2), their cytochemical properties and sex of the patients. However, a significantly higher incidence of TR positivity was observed in patients above 10 yr of age compared to those below 10 yr (47% vs 15%; P < 0.001). The incidence of T-ALL was also significantly higher in the former group (56%) compared to the latter (33%) (P < 0.005). Our data suggest that by virtue of its association with features of poor prognosis, e.g., age above 10 yr, expression of thymic markers, high leucocyte count and disease relapse, TR expression by blast cells in ALL could serve as a biological marker of poor prognosis.

Humans↗

Red cell distribution width as a measure of severity of iron deficiency in iron deficiency anaemia.

Of the various red cell parameters used for distinguishing iron deficiency anaemia (IDA) from beta-thalassaemia trait BTT, red cell distribution width (RDW), which is an objective measure of the degree of anisocytosis, was examined by us for its discriminating value. RDW was measured in 55 patients of IDA and 56 patients of BTT at presentation with the help of an automated haematology analyser. The mean RDWs in IDA and BTT patients were 18.2 +/- 3.8 and 15.1 +/- 1.2 respectively (P < 0.001). In IDA, RDW showed an inverse relationship with the haemoglobin level (r = -0.543; P < 0.001), while no such correlation was observed in BTT patients. An inverse relation was also observed in IDA between RDW and transferrin saturation (TS). Patients with high RDW had low TS and vice versa. The latter finding, although statistically not significant, suggested that the degree of elevation of RDW in IDA could reflect the severity of iron deficiency. Our study revealed that red cell count, which was significantly higher in BTT patients (P < 0.001), the RDW, and the discriminant function (DF) calculated from these two parameters could be useful in distinguishing IDA from BTT. A RDW above 17.1 strongly suggests the presence of IDA. For RDW below 17.1 the DF can be applied for further discrimination. RDW has the advantage of being obtained directly from the analyser, while DF is a calculated value.

Adolescent↗

Effect of hydroxyurea on foetal haemoglobin in myeloproliferative & myelodysplastic syndromes.

The effect of hydroxyurea on foetal haemoglobin (HbF) levels was evaluated in 36 patients of myeloproliferative and myelodysplastic disorders. In 17 (47.2%) patients, HbF levels increased from 1.40 +/- 1.17 to 3.03 +/- 1.97 per cent after 4 wk therapy with hydroxyurea. In the responders this increase was highly significant (P less than 0.001). The rise in the HbF levels after hydroxyurea therapy was significant in patients with chronic myeloid leukaemia but not in the other groups.

Adolescent↗

Correlation of transferrin receptor expression with histologic grade and immunophenotype in chronic lymphocytic leukemia and non-Hodgkin's lymphoma.

The neoplastic lymphoid cells in 33 patients with chronic lymphocytic leukemia (CLL), 1 case of prolymphocytic leukemia (PLL), and 29 patients with various types of non-Hodgkin's lymphoma (NHL) were examined for the expression of transferrin receptors (TR) using a panel of three anti-TR monoclonal antibodies (MAb). Both immunofluorescence and immunoperoxidase techniques were applied in each case. All cases of B-CLL (31) were negative for TR expression, while both the cases of T-CLL, and the only case of T-PLL showed significant TR-positive cell populations. Similarly, all 13 cases of "low-grade" NHL were TR negative, while 6 of 7 cases of "high-grade" lymphomas (lymphoblastic and immunoblastic) had up to 28% TR-positive cells. A proportion (4/9) of "intermediate grade" lymphomas had 10-15% TR positivity. Interestingly, the majority of B-leukemia/lymphoma (49/55) cases were TR negative, while all 8 cases of T-cell leukemia/lymphoma were TR positive. This clearcut association of TR expression with high-risk morphologic and immunophenotypic subgroups of lymphoid leukemia/lymphoma points to the potential role of TR as a prognostic marker in lymphoproliferative disorders.

Antibodies, Monoclonal↗

Cytochemical and immunophenotypic heterogeneity in acute promyelocytic leukemia.

The leukemic promyelocytes in 37 cases of acute promyelocytic leukemia (APML; FAB, M3) were examined for their cytochemical property. Thirty-two cases (86%) showed strong myeloperoxidase (MPO), chloroacetate esterase (Es-chl) and Sudan black B (SBB) positivity, suggesting a pure neutrophilic differentiation of the leukemic cells. However, in 5 out of 37 cases, a strong, diffuse alpha-naphthyl acetate esterase (Es-a) positivity, which was sensitive to sodium fluoride treatment was observed in addition to strong MPO, Es-chl and SBB positivity. This suggested monocytic differentiation of a proportion of APML cases. In 31 cases, surface marker studies were carried out with the help of a panel of monoclonal antibodies consisting of two panmyeloid antibodies (GM 58/8 and 1G10), one anti-HLA-DR antibody (7.2) and one 'myeloid' antibody (5F1) with restricted reactivity with monocytes (CD14). The purpose of including the monoclonal antibodies 5F1 and 7.2 was to determine if a correlation could be established between strong Es-a positivity and reactivity of the leukemic promyelocytes with 5F1 and 7.2 in individual cases. All the 5 cases with 'monocytoid' cytochemistry were unreactive with 5F1, and only one case in this group showed 15% 7.2-positive cells. The lack of immunophenotypic support for the monocytic cytochemistry of the 5 cases of APML suggests that the monocytic phenotype of leukemic promyelocytes is both aberrant and incomplete. Since normal promyelocytes are purely neutrophilic, this could be a manifestation of an 'intralineage infidelity' in APML, similar to that observed in other types of acute leukemia. The clinical significance of 'monocytic' phenotype of APML is not clear; cases with monocytic differentiation did not show different clinical and hematological features when compared to the more common, pure neutrophilic variety.

Antibodies, Monoclonal↗

Limitations of the therapeutic regimens for myelodysplastic syndrome.

Thirty patients of myelodysplastic syndrome (MDS) were treated over a period of 2 yr using 3 different treatment regimens. Twelve patients received hydroxyurea, 4 were given low dose cytosine arabinoside and 14 others were treated with an aggressive acute myeloid leukaemia (AML) induction regimen. A low complete remission was obtained in the first 2 groups (17 and 25% respectively), whereas 9 (64%) patients attained complete remission with the AML induction regimen. Remission in the latter group was associated with prolonged and severe pancytopenia requiring intensive support. Patients in all the 3 groups had a short duration of remission culminating in death with progressive marrow failure or evolution to AML, indicating the limitations of the current treatment strategies for MDS and highlighting the need for exploring newer therapeutic approaches.

Adolescent↗

Sudan black B positivity in acute lymphoblastic leukemia.

The records of 458 patients with acute lymphoblastic leukemia (ALL) have been reviewed. The diagnosis of ALL was based on clinical examination and morphological, cytochemical and immunophenotypic characteristics (20% or more blasts reacting with lymphoid monoclonal antibodies) of blood and/or bone marrow. Blast cells of 6 (1.3%) patients showed positive reaction with Sudan black B (SBB) in the absence of reactivity to any other myeloid markers. Positive reaction with SBB stain cannot be considered specific for myeloid series.

Adolescent↗

Myeloid lineage specificity and high sensitivity of monoclonal antibody GM 58/8 proves its usefulness as a diagnostic reagent in acute leukemia.

Monoclonal antibody (MoAb) GM 58/8 was earlier reported to be directed against an antigen expressed by myeloid progenitors (CFU-GM), myeloid precursors, granulocytes, and monocytes. Immunophenotyping of 216 cases of acute leukemia [acute myeloblastic leukemia (AML) = 147 and acute lymphoblastic leukemia (ALL) = 69] and 18 cases of chronic granulocytic leukemia in blast crisis (CGLBC) with this antibody showed that GM 58/8 reacted with 92% of AML cases (M1-M5) and 100% of myeloblastic crisis in CGL cases. All cases of ALL, lymphoblastic crisis in CGL, erythroleukemia, and erythroblastic crisis in CGL were unreactive with GM 58/8. The antibody revealed the myeloid phenotype in an additional 15 cases of otherwise unclassifiable acute leukemia and six cases of CGLBC. Eleven cases of acute "mixed lineage" leukemia were also diagnosed with the help of GM 58/8. The high specificity (100%) and sensitivity (92%) of MoAb GM 58/8 for myeloblastic leukemia is unmatched by almost all previously described myeloid MoAb and proves its usefulness as a single diagnostic reagent for AML and myeloblastic crisis in CGL.

Acute Disease↗