Naveen Sankhyan, Neelam Grover , J P Bist
Deptt. of Pediatrics, Indira Gandhi. Medical College,Shimla,HP,171001
Objectives; To study the presentation and course of Henoch Schönlein purpura [HSP] Methods; In a retrospective analysis of 5 years, 30 children with HSP were studied with special reference to clinical presentation. Results; Data of 19 boys and 11girls with a mean age of 10.45 years was reviewed. Two thirds of cases presented during fall, winter & spring.One third of children lacked purpura at onset. Palpable purpura involved both upper and lower limbs in 60% cases. Subcutaneous edema was seen in 4(13.3%) patients and scrotal edema in 4 boys. The mean duration of appearance of skin lesions after preceding joint and gastrointestinal symptoms was 8.6 days and 6.6 days respectively. Abdominal pain was the most common GI symptom, observed in 87.5% of those with GI involvement. Joint involvement manifested as arthritis in 13 and equal number had arthralgias. Knee & ankle involvement occurred in more than 3/4th of the patients with arthritis. Seizures occurred in 2 patients, focal CNS signs, intussception & fundal bleeds in one each. Renal involvement manifested as hematuria in 5 and proteinuria in 7 children. Anti Streptococcal O (ASO) titer of 200 IU or more were detected in 10 patients. Atypical manifestations included vesciculobullous lesions in 2 patients and one patient with Rheumatic fever. Eventually purpura appeared in 100% of patients, joints were involved in 26 (86.7%), GIT in 24(80%), Kidney in 9(30%) & CNS in 3(10%). Conclusions; Most children with HSP will have classical manifestation of the disease but diagnostic confusion can occur in those with atypical or absent cutaneous features at the onset.
HO/23(O) A STUDY OF THE CLINICAL AND HISTOPATHOLOGICAL PROFILE OF PEDIATRIC CENTRAL NERVOUS SYSTEM TUMORS.
Sharma P, Mahajan A, Gupta V B, Mehra S.
Apollo Centre for Advanced Pediatrics, Mathura road, New Delhi
CNS tumors are the 2nd commonest malignancy in childhood. Improvements in neuroimaging, surgical techniques, radiotherapy and induction of newer chemotherapeutic agents have significantly improved survival in these children. There is however limited information on the profile of CNS tumors in children in India. Objective: To study the clinical and histopathological profile of CNS tumors in children presenting to a tertiary level hospital. Methods: Data was collected from all patients aged 0-18 years managed at our center from April 2002 to August 2005. Specific information was collected regarding age, sex, radiological and histopathological findings. Modalities of management and median duration from onset of symptoms to diagnosis were also analyzed. Results: The data of 60 patients who were managed during this period. at our center was obtained The most common clinical presentation was headache. The median age of presentation was 10 years with a male: female ratio of 2:1. Infratentorial tumors constituted the majority with 36(60%) patients. The distribution of tumors was as follows: Brain stem glioma 16(26%), Medulloblastoma 9(15%), Astrocytoma 11(18%) and Others 24(40%). The median duration from onset of symptoms to diagnosis was 7.5 months. At a median follow up of 2 years, 2/3(40%) of the patients were alive with minimal sequelae. Among the patients who died 80% were diagnosed as Brain stem glioma. Out of 60 patients 22(36%) received radiotherapy and 18(30%) received both chemotherapy and radiotherapy. Conclusions: Despite classical and frank symptoms, there was a definite delay in the diagnosis of the tumors. Infratentorial tumors were commoner than supratentorial tumors. The most common tumor and the most common cause of mortality in our study was Brain stem glioma.
HO/24(P) ACQUIRED APLASTIC ANAEMIA IN CHILDHOOD:CLINICOHAEMATOLOGICAL PROFILE AND THERAPEUTIC LIMITATIONS
V.Gupta, V.Tilak, B.D.Bhatia
Department of Paediatrics & *Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi-221005
Objectives: To study clinicohaematological profile, therapeutic options and survival outcomes in children with acquired aplastic anaemia. Methods: Prospective study. 30 children of acquired aplastic anaemia who fulfilled the following inclusion criteria: (a) Haemoglobin (Hb) < 10gm/dl & reticulocyte count < 50000/cmm (b) neutrophil count < 1500/cmm and (c) platelet count < 100000 /cmm were included in the study. The treatment options were grouped into (a) only supportive treatment (b) oral cyclosporine (Cy) and (c) Cy and anti thymocyte globulin (ATG). Children were followed to a maximum period of 15 months. Results: The mean age of the children was 7.8 years (Range 2-15 years) with a M: F ratio of 5:1. The mean Hb was 3.3 gm/dl (Range 2-6 gm/dl). The mean total leucocyte count, absolute neutrophil count and absolute platelet count were 2800/cmm , 600/cmm and 25300/cmm respectively. Four cases were lost to followup after initial diagnosis. 14 patients received only supportive treatment and expired within 6 months due to either infection or intracranial haemmorhage. 11 patients received only Cy monotherapy of which 3 patients showed partial recovery while remaining 8 patients did not show any improvement. One patient received both Cy and ATG and showed partial response but relapsed after 6 months. Conclusion: The incidence of acquired aplastic anaemia in this region is high. The therapeutic options are limited due to financial constraints and therefore the prognosis is poor.
Anila Chacko, Leni G Mathew, Indira Agarwal, Prabhakar D Moses
Dept of Child Health, Christian Medical College, Vellore – 632004
Objectives: To assess the clinical features, morbidity and mortality of Late Haemorrhagic Disease of the Newborn (LHDNB), and to make recommendations to reduce its incidence at a national level. Materials & Methods: Clinical case series of children presenting with LHDNB, over a period of 5 years to a tertiary care center. Inpatient and outpatient charts of 50 children (33 boys and 17 girls) admitted with LHDNB were reviewed and analyzed, with respect to history of Vitamin K administration, clinical manifestations, outcome, and status at follow up. Results: Of the 36 babies whose gestational age was documented or known, 32 (89%) were born at term. The median age at presentation was 36 days. The mean duration of symptoms was 3.5 days. 7 children had received one dose of oral Vitamin K and one child an intramuscular dose. 39 parents (78%) were either not aware about administration of Vitamin K or the data was not recorded. 3 parents were sure that they had not received Vitamin K. The common sites of bleed were intracranial (46%), injection sites (40%), umbilicus (32%) and gastrointestinal (24%). All children were treated with Vitamin K and blood products. Antibiotics were started in 33 (66%) till sepsis was ruled out. 8 (16%) children were either discharged in a moribund condition or died. One-third of the survivors were followed up for less than a month. Of the remaining two-thirds, 7 children were documented to have developmental delay. Conclusions: 14% of babies in our series developed LHDNB in spite of oral and parenteral Vitamin K administration. There is a need for prospective studies in India to assess protective efficacy of single-dose IM Vitamin K versus multi-dose oral Vitamin K in preventing LHDNB.
HO/26(O) OUT COME OF MEDULLOBLASTOMA TREATED WITH A COMBINATION OF CHEMOTHERAPY AND REDUCED RADIOTHERAPY-PILOT STUDY
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.
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