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Background: Adequate nutrition is an important concern in children with leukemia. Malnutrition and weight lost are common and are due to verity of mechanism involving the tumor, the host response to the tumor such as infection and pharmacokinetics of chemotherapeutic drugs. Objective: To evaluate and compare the nutritional status of children with ALL at diagnosis and after completion of induction therapy. Methodology: This prospective observational study included 60 children newly diagnosed as ALL, aged 2-15 years, over a period from April 2012 to September 2012 in the Department of Pediatric Hematology and Oncology, BSMMU. The anthropometric measurements and serum albumin level were taken. Anthropometric indices are calculated by NCHS (WHO-2000) and classified as Z score. Children

not availableNorthern International Medical College Journal Vol.9(1) July 2017: 248-251

Epistaxis in children is one of the important presenting symptoms for attending emergency department in paediatric patients. Recurrent epistaxis is common in children. Although epistaxis in children usually occurred due to different benign conditions, it may be one of the important presenting symptoms of some inherited bleeding disorder. Whereas most bleeding disorders can be diagnosed through different standard hematologic assessments, diagnosing rare platelet function disorders may be challenging. In this article we describe one case report of platelet function disorders on Glanzmann’s thrombasthenia (GT). Our patient was a 10-year old girl who presented to us with history of recurrent severe epistaxis. She had a bruise on her abdomen and many scattered petechiae in different parts of the body. Her previous investigations revealed no demonstrable haemostatic anomalies. After performing platelet aggregation test, she was diagnosed as GT.

Background: Leukemia is characterized by persistent and enormous proliferation of immature white blood cell. Without CNS-directed therapy relapses originating from CNS are up to 75%. The presence of overt CNS disease at initial presentation (cranial nerve palsies) negatively affects the EFS of children with ALL. Early intensive intrathecal and systemic therapy is now more successful in treating and preventing CNS leukemia and reducing the cumulative risk for all relapses with CNS involvement of 4.4%, even in patients whose leukemic blast cells is iatrogenically introduced into the cerebrospinal fluid by traumatic lumbar puncture. Methods: 546 patients were enrolled based on initial central nervous system (CNS) status: CNS1 (CNS negative, n=445), CNS2 (≤5 WBC/HPF CSF with blasts, n=35), CNS3 (CNS positive, n=18), TLP+ (TLP with blasts, n=38) and TLP- (TLP without blasts, n=10). Lumbar punctures (LP) were performed at initial presentation and again at initial intrathecal (IT) therapy. Effect of traumatic lumbar puncture (TLP) were evaluated and followed-up for three years. Two extra doses of intrathecal methotrexate (IT MTX) were given with CNS2 and TLP+ status of patients. Additional IT MTX and cranial irradiation (CI) 24 Gy units were given to children of CNS3 status. Results: Children with CNS2, CNS3 and TLP+ status found with unfavorable characteristics (high WBC count, raised serum LDH, hepatosplenomegaly, lymphadenopathy, immunological status, response to therapy and risk group etc.). Event free survival (EFS) with one contaminated TLP++ with blasts in CSF was worse than that of without any contamination (p=0.001). EFS with two consecutive contaminated TLP++ in CSF sample found to have significantly poor outcome (5 years EFS=50 ±7%). Cox regression analysis showed hazards of adverse effects 2.39 times more common with TLP++ status than those with CNS1 (95% confidence interval (CI), 1.36-4.20; p=0.003, Cumulative incidence (CI) with TLP++ status was higher than those with CNS1 (5 years: 32± 10 and 11±2 respectively). TLP+ and CNS3 status had significant prognostic value: risk ratio (RR) = 2.39, 95% CI, 1.4 to 3.6; p=0.0005; TLP+: RR=1.5, 95% CI, 1.02 to 2.20; p= 0.04. Overall 5-year EFS were found 75%. EFS with CNS1 and CNS2 status were 82% and for the TLP-status was 85%. EFS for CNS3 status was 52% and suffers from more systemic involvement. CI of relapses with CNS involvement was higher compared with CNS1 status (0.13 versus 0.06). Children with TLP+ status had significant decreased EFS (75%, p=0.003) due to increased incidence of CNS relapses. Children with CNS2 had same prognosis as that with CNS1 status, whereas EFS of TLP+ status was inferior to CNS1 status but was superior to CNS3 status (p=0.001). EFS with two consecutive TLP++ status contaminated CSF sample was particularly poor. Conclusion: Contamination of CSF samples with circulating leukemic blasts during diagnostic lumbar puncture, an additional IT MTX is indicated which prevent adverse effect and outcome of newly diagnosed ALL. For improved treatment outcome every attempt should be made to prevent TLP. Occurrence of this adversely affects the quality of life, making the need of additional IT MTX therapy. Methods: 546 patients were enrolled based on initial central nervous system (CNS) status: CNS1 (CNS negative, n=445), CNS2 (≤5 WBC/HPF CSF with blasts, n=35), CNS3 (CNS positive, n=18), TLP+ (TLP with blasts, n=38) and TLP- (TLP without blasts, n=10). Lumbar punctures (LP) were performed at initial presentation and again at initial intrathecal (IT) therapy. Effect of traumatic lumbar puncture (TLP) were evaluated and followed-up for three years. Two extra doses of intrathecal methotrexate (IT MTX) were given with CNS2 and TLP+ status of patients. Additional IT MTX and cranial irradiation (CI) 24 Gy units were given to children of CNS3 status. Results: Children with CNS2, CNS3 and TLP+ status found with unfavorable characteristics (high WBC count, raised serum LDH, hepatosplenomegaly, lymphadenopathy, immunological status, response to therapy and risk group etc.). Event free survival (EFS) with one contaminated TLP++ with blasts in CSF was worse than that of without any contamination (p=0.001). EFS with two consecutive contaminated TLP++ in CSF sample found to have significantly poor outcome (5 years EFS=50 ±7%). Cox regression analysis showed hazards of adverse effects 2.39 times more common with TLP++ status than those with CNS1 (95% confidence interval (CI), 1.36-4.20; p=0.003, Cumulative incidence (CI) with TLP++ status was higher than those with CNS1 (5 years: 32± 10 and 11±2 respectively). TLP+ and CNS3 status had significant prognostic value: risk ratio (RR) = 2.39, 95% CI, 1.4 to 3.6; p=0.0005; TLP+: RR=1.5, 95% CI, 1.02 to 2.20; p= 0.04. Overall 5-year EFS were found 75%. EFS with CNS1 and CNS2 status were 82% and for the TLP-status was 85%. EFS for CNS3 status was 52% and suffers from more systemic involvement. CI of relapses with CNS involvement was higher compared with CNS1 status (0.13 versus 0.06). Children with TLP+ status had significant decreased EFS (75%, p=0.003) due to increased incidence of CNS relapses. Children with CNS2 had same prognosis as that with CNS1 status, whereas EFS of TLP+ status was inferior to CNS1 status but was superior to CNS3 status (p=0.001). EFS with two consecutive TLP++ status contaminated CSF sample was particularly poor. Conclusion: Contamination of CSF samples with circulating leukemic blasts during diagnostic lumbar puncture, an additional IT MTX is indicated which prevent adverse effect and outcome of newly diagnosed ALL. For improved treatment outcome every attempt should be made to prevent TLP. Occurrence of this adversely affects the quality of life, making the need of additional IT MTX therapy. Objectives: The study was designed to determine the prognostic significance of leukemic blasts cell infiltration or traumatic lumbar puncture (TLP) in cerebrospinal fluid (CSF) of newly diagnosed children with acute lymphoblastic leukemia (ALL).Background: Leukemia is characterized by persistent and enormous proliferation of immature white blood cell. Without CNS-directed therapy relapses originating from CNS are up to 75%. The presence of overt CNS disease at initial presentation (cranial nerve palsies) negatively affects the EFS of children with ALL. Early intensive intrathecal and systemic therapy is now more successful in treating and preventing CNS leukemia and reducing the cumulative risk for all relapses with CNS involvement of 4.4%, even in patients whose leukemic blast cells is iatrogenically introduced into the cerebrospinal fluid by traumatic lumbar puncture. Objectives: The study was designed to determine the prognostic significance of leukemic blasts cell infiltration or traumatic lumbar puncture (TLP) in cerebrospinal fluid (CSF) of newly diagnosed children with acute lymphoblastic leukemia (ALL).

Introduction : Haemophilias are the most common inherited coagulation disorders transmitted by X- linked recessive fashion affecting the males and females are the carriers of the disease. Haemophilias are distributed worldwide and have heterogeneous presentation depending upon its severity starting from neonatal period. Knowledge of spectrum of presentation of haemophilia helps in early diagnosis and planning of management. Objectives : To observe the clinical presentation of haemophilia in children Methodology : This observational study was carried out in the Department of Pediatric Hematology and Oncology, Bangabandhu Sheikh Mujib Medical University for a period of one year from 1 st July 2007 to 30 th June 2008. Clinical profile of 50 diagnosed cases of haemophilia

DOI: http://dx.doi.org/10.3329/bjch.v37i1.15349 BANGLADESH J CHILD HEALTH 2013; VOL 37 (1) : 27-40

DOI: http://dx.doi.org/10.3329/bjch.v36i3.14273 BANGLADESH J CHILD HEALTH 2012; VOL 36 (3) : 111-114