Ho/01(P) incidence of iron deficiency in non anaemic children

HO/13(O) UTILITY OF IMMUNOPHENOTYPING IN ACUTE LEUKEMIAS – AIIMS EXPERIENCE     

T.Seth, J Wadhwa, Bk Mohanti, A Ranjan,

Depts Medical Oncology, Radiotherapy, AIIMS, New Delhi

Objectives: Medulloblastoma in children over 3 years, is treated with a combination of surgery, radiotherapy and chemotherapy. Long-term sequelae include growth retardation, reduction in IQ and hypothyroidism. The appropriate dose of radiation to the craniospinal axis in combination with chemotherapy for low-risk medulloblastoma remains to be defined. Methods: We studied low risk medulloblastoma patients (greater than 3 years, residua <1.5cm2 and no neuraxis involvement) who were given 23.4 Gy neuraxis irradiation followed by posterior fossa (PF) boost to 50.4-55.8 Gy and chemotherapy consisting of vincristine (VCR) weekly during RT. This was followed by VCR, cis-platinum (CDDP), and lomustine (CCNU) for eight, 6-week cycles. The patients were staged by post-operative contrast CT, MRI and CSF cytology. Baseline eye, auditory examination, thyroid function and (a) The Malin’s Intelligence scale for Indian Children (MISIC) (b) Schonell Attainment Test for reading, spelling (c) Aston Index- auditory sequential memory, visual perceptual skills (c) Brigance (IBS) to test listening and reading comprehension were performed. 4 patients, ages 7-11 years, were treated on this protocol and evaluated for relapse free survival by repeated MRI every 3 months. Results: Median follow up of 14 months, chemotherapy course complicated by myelosuppression, thrombocytopenia requiring chemotherapy delay in all patients. No relapses to date, two patients have cerebral atrophy (due to long standing hydrocephalous). IQ scores showed a mean reduction of 6 points, (not significant), other reading and comprehension parameters were age appropriate. Hypothyroidism was found in the 7 year old child, however this complication has been reported to occur late. Conclusion: reduced dose chemotherapy to the neuraxis can be tried, to decrease long term sequelae in children, however meticulous planning is required. Preliminary data suggests that this may be feasible, however long term follow up is required to see relapse rates and survival.

HO/14(P) SEVERE COMBINED IMMUNODEFICIENCY SYNDROME

Vasundhara Chennuri, Pankaj Parikh,Mukesh Desai, Rakesh Shah

Sir H N Hospital & Research Centre, Mumbai 400004

Introduction: Severe combined immunodeficiency is the most severe of all recognised immunodeficiencies caused by diverse genetic mutations which lead to absence of all adaptive immune finction . The phenotypic variations are 1] ( T - B- NK +)both T and B lymphocytes are deficient but natural killer cells (NK) are normal( RAG1 and RAG2 gene defects ). 2] (T-B+NK+) T lymphocytes are deficient however B lymphocytes and NK cells are normal (IL7R receptor or CD 45 deficiency 3](T-B-NK-) all three cells are deficient ( adenosine deaminase deficiency. Case :A 5 month old female child born of third degrre consanguinous marriage presented with repeated episodes of upper respiratory tract infection, diarrhea, oral thrush and failure to thrive since 2 months of age.During the diarrhea episodes stools showed evidence of pus cells ( 40 - 50 cells per low power field). There was evidence of rectovaginal fistula with perianal tags . Papular lesions over the face , trunk, and groin suggested disseminated candidiasis. She had persistent leucopenia ( absolute neutrophil count < 1500/cmm and absolute lymphocyte count less than 2000/cmm)Mother tested negative for HIV ELISA. The X- ray chest showed an absence of normal thymus shadow. The stool in additon to showing pus cells ( 40-50 per low power field) grew E. coli.The serum levels of IgA, IgE and IgM were normal. The CD3, CD4, CD8 ( T cell markers) , CD19 ( B cell marker) line age lymphocytes were depleted { CD3 = 25/cmm ( N = 1375 - 3769), CD 4 =25/cmm ( N= 174 - 1388), CD8 = 4/cmm ( N = 231- 2394), CD 19 = 2/cmm ( N = 739 - 2353). The CD 56 ( NK cell marker) levels were normal.The above cliical picture and investigations ( persistent lymphopenia ) clinched the diagnosis of SCID (T - B + NK + ) type.Parents were counselled for bone marrow replacement for the child but they refused. Conclusion : Infants with SCID have lymphopenia ( < 2000) at birth . If routine white blood count and differential count were done on cord blood at birth they could be easily detected. This is a true pediatric emergency and unless immunologic reconstitution in the form of bone marrow transplant is done at the earliest ,death is inevitable, usually by the end of one year.

HO/15(P) PSYCHOSOCIAL STATUS AND PAIN SCORE INTHAL

Bhavan, Nilesh Patel, K. M. Mehariya, Deepak Panday

Deptt. of Pediatrics, M. P. Shah Mediacal College, Jamnagar –361008

Introduction : Many frequently transfused thalassemic children remain under chronic duress due to repeated painful events and other factors like loss of schooling limitation of physical activity, distorted body features. Quantification and management of pain as also pyschosocial support in those significantly affected may substantially reduce the misry of these patients the study is conceived to quantify these problems and need for intervention. AIMS & OBJECTIVES : To measurs magnitude of pain, to identify the need for psychosocial councelling and pain management in thalassemia major patients. MATERIALS AND METHODS : 71 childrens 5 to 18 years old, thalassemics receving blood transfusions in thalassemia were recruited for the study EXCLUSION : CRITERION: single parent chronic illness in family members chronic comorbidity, Child abuse. After rapport building data was collected by a structured interview CHILDHOOD PSYCHOPATHOLOGY MEASUREMENT SCHEDULE :(SAVITA MALHOTRA, 1984) was use for screening pain was measured using VISUAL ANALOG SCORE. RESULTS: 34% subjects tested positive for psychopethology with 18 of 47 in 5 to 10 years of age group incidence of positive CPMS was greater in school drop outs and those who received no schoolling 72% subjects had pain score more than 50 incidence of cpms was signigicantly higher in those with pain score more than 10 majority of them have on set of psychopathology atleast as eaely as 2 nd half of 1st decade of life as against most other comrlications of thal major.pain managemeant should be considered in all such patients and adequate psychosocial support should be provided to the affecated
HO/16(P) PARAPARESIS WITH CHEST WALL MASS: UNUSUAL ETIOLOGY.

Keya R Lahiri, Mihir Bapat, Chaitali D. Warang, Rajwanti K. Vaswani, Milind S.Tullu, Rachna Kalra

Department of Pediatrics, Seth G.S.Medical College and King Edward Memorial Hospital, Parel, Mumbai, 400012
Introduction: Primitive neuroectodermal tumors of childhood are a heterogeneous group of rare tumors of neural crest origin (8% childhood cancers). Case 1: An 11-year-old girl presented with acute weakness in lower limbs & urinary retention. On examination, she had paraplegia & hypotonia in lower limbs. There was no local tenderness over spine. There was reduced air entry in left interscapular area. Chest radiograph revealed well-defined rounded homogenous opacity in left hemithorax, no calcifications, or rib erosions noted. MRI revealed tumor mass with intense post contrast enhancement, extending D4 to D10 levels & displacing the thecal sac. In view of rapid progression, she underwent surgery for tumor resection. Histopathology revealed nests of small round cells with necrosis & immunohistochemistry showed presence of MIC 2 & NSE (neuron specific enolase), suggestive of Askin’s tumor. Case 2: A 5-month-old female presented with respiratory distress since day 7 of life, paucity of movements of lower limbs & distension of abdomen for 2 months. She had generalized lymphadenopathy and a soft, fusiform swelling in right thoracolumbar region, hepatoslenomegaly and paraparesis. Chest radiograph revealed paravertebral soft tissue mass with punctate calcification. CT Chest revealed mass arising from the adrenal fossa with calcification, extending to the chest wall & spine, suggestive of Neuroblastoma. Urinary VMA was elevated. Bone marrow examination was normal. She was started on chemotherapy. Discussion: PNETs are characterized by nests of less differentiated small round cells with scanty cytoplasm and darkly staining nuclei on histopathology. Immunohistochemical & cytogenetic markers confirm that these tumors have common histogenesis & genetic aberration. Age of presentation varies (infants to adolescence). They have insidious onset with paravertebral or chest wall mass,& may also have respiratory or neurological manifestations. These tumors metastasize to the other skeletal sites, marrow, and lung. Therapy is multimodality based including surgery, chemotherapy and radiotherapy. Conclusion: A high index of suspicion is required to detect these rare tumors which otherwise have a guarded outcome. Early diagnosis & therapy are essential to improve survival.