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Rahul Naithani

Publications and source records attributed to Rahul Naithani.

14 recordsLinked to original sources
PubMed2006

Peroxidative stress and antioxidant enzymes in children with beta-thalassemia major.

OBJECTIVE: Regular blood transfusions and secondary iron overload make thalassemic erythrocytes prone to peroxidative injury. Although some reports suggest endogenous free radical damage in thalassemia, there remains discrepancy in the status of antioxidant enzymes. The aim of this study was to evaluate the extent of lipid peroxidation and status of antioxidant enzyme in children with beta-thalassemia. METHODS: Fifty transfusion-dependent beta-thalassemics were subjected to analysis of lipoperoxides as malondialdehyde (MDA), nitric oxide (NOx), superoxide dismutase (SOD), glutathione (GSH), and glutathione peroxidase (GPx) along with serum iron and ferritin, liver functions and uric acid. Plasma MDA was analyzed to indicate the oxidative parameters, whereas the erythrocyte SOD, GPx, and plasma NOx were measured to show the antioxidant status of the children. All these parameters in 30 non-anemic healthy controls attending the child health promotion clinic of hospital were also studied. RESULTS: All the patients were iron overloaded. Markers of free radical injury such as MDA and antioxidant enzyme SOD and NOx levels were significantly elevated in thalassemic children while mean GPx levels were decreased in patients compared to controls (P < 0.001). All these markers significantly correlated with serum ferritin levels. There was no significant difference in levels of GSH measured but it correlated with serum iron levels. CONCLUSION: Our study results suggest that iron overload causes peroxidative damage in beta-thalassemia and antioxidant systems try to compensate for reducing lipid peroxidation to lower tissue damage.

Adolescent↗
PubMed2006

Autoimmune hemolytic anemia in India: clinico-hematological spectrum of 79 cases.

The clinico-hematological profile and treatment outcome of patients with autoimmune hemolytic anemia (AIHA) were assessed using a retrospective case record analysis. There were 79 (52 primary; 27 secondary) consecutive patients identified with a median age of 30.5 years. The main presenting complaints were pallor (94%), fever (46%), jaundice (51%), bleeding manifestations (10%) and splenomegaly (68%). Jaundice was much more common in primary (63%) as opposed to secondary (26%) disease. The direct antiglobulin test was negative in six patients. Oral prednisolone produced remission in 87.5% patients. Six patients (three children, three adults) relapsed after a median period of 2 months after response. All of these responded to a second course of steroids, in a median period of 14 days. No correlation was found between response and the parameters of age, sex, jaundice, low pretreatment hemoglobin, reticulocyte count, total leucocyte count (TLC), platelet count, subtype of AIHA and hepatosplenomegaly. Relapse correlated with increased duration between the onset of symptoms and treatment (r = 0.996; p = 0.0001).

Administration, Oral↗
PubMed2006

Thalassemia major-- on the verge of bleeding or thrombosis?

UNLABELLED: Thrombotic events have been reported in adult thalassemic patients. To investigate this further, we measured hemostatic parameters in thalassemic children to identify possible predisposing factors in early childhood. OBJECTIVE: To assess hemostatic derangements in polytransfused children with beta-thalassemia major (beta-TM). METHODS: Complete blood count, prothrombin time, activated partial thromboplastin time, protein C, protein S, Antithrombin III (AT III), fibrinogen, d-dimer assay, serum iron, serum ferritin and liver function tests were measured in 54 patients and 30 controls using standard lab methods. RESULTS: Sixteen patients exhibited bleeding manifestations. None of the cases had thromboembolic phenomena. The average pretransfusion haemoglobin in the cases studied was 8.45 +/- 1.6 g/dl, thrombocytopenia was seen in 33.3%, prolongation of prothrombin time was seen in 40.7% and prolongation of aPTT was seen in 46.3%. None of our patients had laboratory features of DIC. Protein C was low in 26.2%, protein S in 28.6% and AT III levels in 46.8% of cases. Mean fibrinogen levels and d-dimers were similar in cases and controls. Serum ferritin levels in the patients were high with a mean of 3,709 +/- 1,625 ng/ml. Serum ferritin had a significant positive correlation with PT (r = 0.382) and ALT (r = 0.315) and a significant negative correlation with protein S (r = - 0.376). Prolonged PT correlated with prolonged aPTT, low protein C, low protein S and serum ferritin levels. Protein C had a significant positive correlation with AT III. Low AT III activity correlated positively with age, aspartate transaminase and alanine transaminase. Average hemoglobin maintained correlated negatively with serum ferritin levels (r = - 0.540), and AST (r = - 0.417). Bleeding episodes correlated with age, liver size and number of transfusions. CONCLUSION: Significant alterations in the hemostatic system already exist in polytransfused children with beta-thalassemia that make it a high-risk condition for both hemorrhagic manifestations and future development of thromboembolic events.

Adolescent↗
PubMed2006

Disseminated Langerhans cell histiocytosis-an interesting case report with concise review of literature.

A 2-year-old boy presented with fever, recurrent infections and multiple skin lesions. He had anemia, eczematous skin lesions, cervical lymph node enlargement, hepatomegaly and lytic lesions on skull x-ray. The skin infiltrates were CD 68, CD 1a positive and S100 positive. He was diagnosed as disseminated langerhans cell histiocytosis. The occurrence of histiocytosis is reviewed and possible treatment is discussed.

Antigens, CD↗
PubMed2005

Hearing loss in chronic myeloid leukemia.

A 12-year-old girl presented with abdominal pain, fever, and hearing impairment of 6 months duration. She had massive hepatosplenomegaly and anemia. On the basis of her peripheral blood and bone marrow findings, she was diagnosed as chronic myeloid leukemia (CML) in chronic phase. Her hearing was assessed by brainstem evoked responses (BERA), which showed objective improvement in hearing with hydroxyurea. The rare occurrence of deafness in CML is reviewed and possible pathogenesis is discussed.

Child↗
PubMed2005

Primary myelodysplastic syndrome in children--clinical, hematological and histomorphological profile from a tertiary care centre in India.

We describe the clinical, hematological and histomorphological features in children of primary myelodysplastic syndrome (MDS) seen at the All India Institute of Medical Sciences over three years (Jan 2001-Jan 2004). Twenty-one patients of primary MDS aged 17 year or less were classified using the latest proposed WHO classification for Pediatric MDS. The median age was 9 years with male predominance (80%). Pallor was present in all the cases while fever and bleeding diathesis was present in more than 50% of the cases. Morphological assessment of the peripheral blood showed macrocytosis in 50%, pancytopenia in 15% and blast cells in 45% of cases. A complete analysis of clinical features in conjunction with the bone marrow profile revealed 8 cases of refractory cytopenia (RC), 3 cases of refractory anemia with excess blasts (RAEB), 5 cases of refractory anemia with excess blasts in transformation (RAEB-T), 4 cases of Juvenile myelomonocytic leukemia (JMML) and a solitary cases of acute myeloid leukemia (AML) in Downs syndrome. These children were followed up from 1-36 months (mean 15 months). Three patients of RAEB-T progressed to AML within 3-4 months. RC had the best prognosis and all are alive and under regular follow up. The solitary case of AML of Downs syndrome died 1.5 months after initial diagnosis. All 3 cases of RAEB are under regular follow-up and doing well. Three cases of RAEB-T died (all had progressed to AML); the remaining 2 cases were lost to follow up. Of the 4 cases of JMML 1 died within 6 months of diagnosis; the other 3 cases are under regular follow up of whom 1 has a progressively increasing blast count. We conclude that the latest proposed WHO classification for Pediatric MDS can be successfully applied to all cases of primary MDS.

Adolescent↗
PubMed2005

Safety of oral iron chelator deferiprone in young thalassaemics.

UNLABELLED: Deferiprone is now widely used for iron chelation in patients with thalassaemia. Studies on its efficacy and safety have largely included older children and adults. OBJECTIVE: To assess the safety of deferiprone in children <6 yr of age. METHODS: The study is based on scrutiny of follow-up data of 44 patients of age <6 yr receiving deferiprone for a variable period of time. Occurrence of various side effects including gastrointestinal, osteoarticular were noticed and complete blood counts were performed every 2-4 wk. RESULTS: Nausea and vomiting were noticed in 12 (27.2%), joint symptoms were reported by four (9.1%) and neutropenia was observed in only two patients (4.5%). None of the patient had agranulocytosis. Thrombocytopenia was observed in 20 patients (45.45%), which occurred 3 months to 1 yr after deferiprone therapy. Interruption of deferiprone for 2-4 wk led to reversal of symptoms in all but two patients. CONCLUSION: Thrombocytopenia is one of the major side effects in young thalassaemics and necessitates frequent close monitoring of blood counts but its resolution after discontinuation and absence of clinical evidence of bleeding does not preclude its use.

Blood Cell Count↗
PubMed2005

Acute myeloid leukemia after intensive immunosuppressive therapy in aplastic anemia.

A 10-year-old boy was admitted with complaints of fever, pallor, fatigue and skin bleeds of 10 days duration and diagnosed as very severe aplastic anemia. He was given intensive immunosuppressive therapy but showed no response to therapy. He later evolved into acute myeloid leukemia. The occurrence of AML is reviewed and possible pathogenesis is discussed.

Anemia, Aplastic↗
PubMed2005

Voriconazole.

Voriconazole (VRZ) is a second-generation triazole antifungal agent active against many species of Aspergillus and Candida and acts by inhibiting ergosterol synthesis. VRZ has less nephrotoxicity and less infusion-related toxicity than that of Amphotericin B. Oral and parental formulation have similar pharmacokinetics and thus oral formulation shortens the duration of hospital stay. It is overall well tolerated but has significant drug interactions.

Antifungal Agents↗
PubMedDate not supplied

Life threatening exacerbation in idiopathic pulmonary hemosiderosis salvaged by cyclophosphamide infusion.

A seven-year-old girl presented with frequent fever, cough and shortness of breath of three months duration. On the basis of her clinical features, peripheral blood and sputum findings, she was diagnosed as a case of idiopathic pulmonary hemosiderosis. After initial stabilisation with steroids and chloroquine, she presented four years later with massive pulmonary hemorrhage and respiratory failure, which responded dramatically to cyclophosphamide infusion. The rare occurrence of pulmonary hemosiderosis and different treatment regimens is discussed.

Child↗