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Biomedical subjects

Amita Trehan

Publications and source records attributed to Amita Trehan.

15 recordsLinked to original sources
PubMed2006

Attenuated form of Evans syndrome among pediatric ITP patients.

Long term follow up of adult patients with immune thrombocytopenic purpura (ITP) have shown evolvement of secondary autoimmune diseases such as SLE, Evans syndrome, autoimmune neutropenia, Graves disease etc. We studied 30 cases of pediatric ITP patients for evidence of hemolysis to assess the possibility of Evans like syndrome. Measurement of free serum haptoglobin, a sensitive indicator of red cell destruction was used after careful exclusion of micro angiopathic hemolysis, SLE or overt Evans Syndrome. Results showed abnormally low level of free serum haptoglobin in 11 of the 30 (36.7%) patients compared to that in 20 age matched controls (P < 0.001) as an evidence of hemolysis. Our data in pediatric patients is similar to that reported in adult ITP cases and support the observation of Evans made 50 years ago that there is a spectrum like relationship between primary thrombocytopenia and hemolytic anemia. Thus the concept of attenuated form of Evans syndrome could be considered, in group of patients with ITP in pediatric age group.

Adolescent↗
PubMed2005

Interferon therapy in congenital dyserythropoietic anemia type I/II.

Congenital dyserythropoietic anemia (CDA) is a rare disorder, characterized by the association of ineffective erythropoiesis, variable degree of anemia, and erythroblastic morphological abnormalities. alpha-Interferon has been reported to be effective in type I CDA, but efficacy in other types of CDA is uncertain. Encouraged by the reports, we evaluated the efficacy of alpha-interferon in 6 children with CDA. Diagnosis of CDA was established on the basis of clinical profile, distinct morphological findings on light microscopy, and the Ham's test, following the exclusion of the more common causes of hemolysis. Erythrocyte agglutinability and lysis to anti-i and anti-l sera, electron microscopy, and SDS-polyacrylamide gel electrophoresis were not performed, due to nonavailability. There were 3 cases, each, with type I and type II CDA. The mean age was 5.5 years (range: 6 months to 11.5 years). Five of the 6 patients were transfusion dependent. alpha-Interferon was administered subcutaneously for a mean duration of 19 weeks (range: 12-30). The dose ranged from 2.6 to 6.5 million IU/m2/dose. The frequency of injections varied from thrice weekly to alternate days. No favorable effect on hemoglobin, reticulocyte count, or transfusion frequency was observed. alpha-Interferon therapy was found to be ineffective in all the patients. These observations question the use of interferon in CDA until further studies in a larger number of patients establish its efficacy.

Anemia, Dyserythropoietic, Congenital↗
PubMed2005

Assessment of adrenal endocrine function in Asian thalassemics.

Adrenal endocrine function was assessed in a cohort of 20 patients, between 10 and 20 years of age, with transfusion dependent beta thalassemia. Cortisol levels were assayed before and after ACTH stimulation with 1 micrograms and 250 micrograms. Adrenal dysfunction was defined as a basal cortisol of greater than 400 nmol/L and/or peak cortisol levels of greater than 500 nmol/L. Overall, 9 patients (45 %) had in vitro evidence of adrenal dysfunction. A statistical significant correlation (r=0.4308; P < 0.05), between wasting and the basal cortisol level, was observed. Similarly, there was correlation between the number of transfusions received and growth failure (r=0.4774;P < 0.05). In comparison to the involvement of other endocrine axes in polytransfused thalassemics, the adrenal endocrine function abnormalities are minor and clinically of little consequence. The observations, albeit, in a small cohort of thalassemics, stress the need for an annual estimation of basal cortisol level, especially in patients with wasting.

Adolescent↗
PubMed2004

Griscelli syndrome.

An eight month old male infant presented with recurrent infections and partial albinism. Initially a possibility of Chediak Higashi syndrome (CHS) was considered, but a negative investigative work up prompted us to look for an alternate diagnosis. A literature search revealed that Griscelli syndrome (GS) has overlapping symptoms and signs. The findings in skin and hair biopsies in Griscelli syndrome are distinctive.

Chediak-Higashi Syndrome↗
PubMed2004

Androgens in childhood acquired aplastic anaemia in Chandigarh, India.

This study assessed the efficacy of stanozolol in acquired aplastic anaemia (AA) in children, who were unable to opt for alternative therapies due to financial constraints. It uses a retrospective case-record analysis. Medical records of children with AA who received stanozolol were analysed. Their clinical and haematological profile was studied and the outcome assessed. Forty-nine children with acquired AA received stanozolol (1 mg/kg/day) in the period between January 1991 and December 2000. The median age at diagnosis was 9 years (range: 1.5-13). Thirteen (26.5%) defaulted therapy after periods of 1-4 weeks and 11 (22.4%) died within 2 months of starting treatment. Ten (20.4%) responded, whilst 15 (30.6%) did not. None of the patients with very severe AA responded, two (28.6%) with severe AA responded and eight (38%) with non-severe AA had a response. All responses were partial except one. Median time taken to respond was 11 weeks (range: 1-20). Responders were treated for a median duration of 25 weeks (range: 13-155), and they were followed-up for a median duration of 25 months (range: 3-144). Eight (80%) responders were off drugs for a median duration of 18 months (range: 6-110). Stanazolol is ineffective for severe and very severe AA. As it induced remission in 38% of our patients with non-severe AA, it can be tried in this group if other methods of treatment are unaffordable or have failed. A short history of presentation (< 3 months) and > or = 70% lymphoid cells in marrow correlated with poor prognosis.

Adolescent↗
PubMed2004

Wheat grass juice reduces transfusion requirement in patients with thalassemia major: a pilot study.

Wheat grass juice is the juice extracted from the pulp of wheat grass and has been used as a general-purpose health tonic for several years. Several of our patients in the thalassemia unit began consuming wheat grass juice after anecdotal accounts of beneficial effects on transfusion requirements. These encouraging experiences prompted us to evaluate the effect of wheat grass juice on transfusion requirements in patients with transfusion dependent beta thalassemia. Families of patients raised the wheat grass at home in kitchen garden/pots. The patients consumed about 100 mL of wheat grass juice daily. Each patient acted as his own control. Observations recorded during the period of intake of wheat grass juice were compared with one-year period preceding it. Variables recorded were the interval between transfusions, pre-transfusion hemoglobin, amount of blood transfused and the body weight. A beneficial effect of wheat grass juice was defined as decrease in the requirement of packed red cells (measured as grams/Kg body weight/year) by 25% or more. 16 cases were analyzed. Blood transfusion requirement fell by >25% in 8 (50%) patients with a decrease of >40% documented in 3 of these. No perceptible adverse effects were recognized.

Adolescent↗
PubMed2004

An outbreak of hospital acquired diarrhea due to Aeromonas sobria.

Six children admitted in a 18 bedded hematology-oncology unit, developed acute diarrhea in a four week period between March and April 2001. Salmonella senftenberg was the additional pathogen in the stool sample of one patient who developed cola colored urine and pneumonia in the course of his illness. All the Aeromonas strains had a similar biotype and antibiogram. The diarrhea subsided spontaneously in two children whilst three responded to antimicrobial therapy. One patient sought discharge and did not return for a follow up. Aeromonas sobria with a similar profile as the isolates from the patients could be isolated from only one of several environmental sites. The outbreak could be contained by appropriate interventional measures.

Aeromonas↗
PubMed2003

Congenital dyserythropoietic anemia: clinical and hematological profile.

Congenital dyserythropoietic anemia (CDA) is a rare disorder, which manifests clinically with varying degrees of anemia and hepatosplenomegaly. These features are not pathognomic and a diagnosis of CDA is generally considered after other causes of chronic hemolytic anemia have been ruled out. The clinico-hematological profile of 10 patients with CDA is presented in this communication. Six patients had CDA type II and four had CDA type I. Age at onset of pallor ranged from birth to 9 years. Blood transfusion requirements varied from nil to monthly. This is the first report of CDA type I from India.

Anemia, Dyserythropoietic, Congenital↗
PubMed2002

Melanotic neuroectodermal tumour in an infant.

Most jaw swellings in infancy are benign odontogenic cysts or tumours. The encounter with an unusual melanotic neuroectodermal tumour of the jaw in a young infant is described in this report. There has been no recurrence, after complete surgical excision, in a follow-up period of six months.

Female↗
PubMed2002

Chemotherapy related fatal neurotoxicity during induction in acute lymphoblastic leukemia.

Neurotoxicity is a common complication during cancer chemotherapy. It is estimated that 3-10% of children with acute lymphoblastic leukemia (ALL) experience acute, transient neurotoxicity during induction chemotherapy. Fatal acute neurotoxicity is rarely encountered. Neurological evaluation of children with ALL at diagnosis and during treatment is of value in order to diagnose neurological complications early so that appropriate intervention can be adopted. This communication describes the profile of two children with unexpected, acute fatal neurologic toxicity during induction chemotherapy for ALL.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2002

Sjogren-Larsson syndrome.

Sjogren-Larsson Syndrome is a rare autosomal disorder which occurs with 100% penetrance and is classically characterized by ichthyosis, spasticity and mental handicap. This has rarely been described in people of Indian origin, case reports mainly being restricted to Caucasians. We have described a 6-year-old girl with classical features of this syndrome, the symptoms having started in infancy. The spasticity is mainly diplegic in nature. Skin changes of ichthyosis are generalized and more in the flexural areas. Mental retardation is severe. Management is supportive.

Child, Preschool↗
PubMed2002

Clinico-haematological profile of pure red cell aplasia in children.

Pure red cell aplasia (PRCA) is a rare disorder, characterized by isolated failure of erythropoiesis. The clinico-haematological profile of 16 patients with PRCA is presented in this communication. Fourteen patients had Diamond-Blackfan anaemia (DBA), one had transient erythroblastopenia of childhood, and one patient had PRCA secondary to carbamazepine. Physical abnormalities were observed in 50 per cent of patients with DBA. Of the nine patients with DBA who were administered prednisolone and had a regular follow-up, four (44.4 per cent) had no response, three (33.3 per cent) responded fully, and two (22.2 per cent) were steroid dependent.

Anemia, Diamond-Blackfan↗
PubMedDate not supplied

Hepatitis-associated aplastic anemia: successful outcome following immunosuppressive therapy.

Hepatitis-associated aplastic anemia is an uncommon variant seen in young, previously healthy individuals. The pancytopenia follows hepatitis by a few weeks and is usually severe and prolonged. Bone marrow transplantation remains the cornerstone of therapy. However, immunosuppressive therapy has been found to be effective. We report an 8-year-old girl who had non-A, B, C and E hepatitis-associated severe aplastic anemia. She became transfusion-independent and had consistent, albeit incomplete recovery after immunosuppressive therapy with antithymocyte globulin and cyclosporine.

Anemia, Aplastic↗