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

HO/09(P) EVALUATION OF THYROID FUNCTION IN PATIENTS OF THALASSEMIA MAJOR

Rajwanti K Vaswani

Deptt of Pediatrics, Seth G. S. Medical College and K.E. M. Hospital, Mumbai-400012

Introduction: Aggressive transfusion regimen has played an important role in prolonging the lifespan of patients of thalasemia major resulting in awareness of late complications. Growth abnormality is a commom complication in thalassemics and is attributed to various factors; one of them being thyroid hormone dysfunction. Objectives: To evaluate thyroid function in a cohort of children with transfusion dependent beta thalasemia , study its role in growth failure and to assess the correlation between the degree of iron overload and thyroid function. Design: Prospective study Setting: Pediatric ward of a tertiary care center. Methods: Fifty thalassemic patients receiving regular transfusions for last 5 years were investigated together with ten age and sex matched controls. Physical growth was expressed by height and weight percentiles ( NCHS standards) and skeletal maturation was assessed by radiographs of wrist and elbow.Thyroid function was assayed estimated using radioimmunoaassay. Pearson’s chi-square test and the unpaired ‘t’ test were used to analyze the results. P value < 0.05 was considered significant. Results: Of 50 thalassemic patients, hypothyroidism was detected in 18% of the cases .Significant growth retardation was seen in cases than in the controls. Weight age was more retarted than the height age. Thyroid dysfunction correlated well with liver span (increased) and serum ferritin (high) while spleen size, total units of blood transfused were not associated. Conclusion: There is a high prevalence of subclinical hypothyroidism in patients of thalassemia major and it is also related to the degree of iron overload determined by large liver span and high serum ferrritin. Early chelation can reduce the incidence of hypothyroidism.
HO/10(O) GRANULOCYTE TRANSFUSION IN PEDIATRICS.

Shrishu.R.Kamath, Suresh Babu , V. Lakshmanan,.Revathy Raj

Apollo Hospitals , Greams Road , Greams Lane , Chennai-600 006
Introduction: Prolonged neutropenia is a major risk factor for development of severe bacterial and fungal infections in children undergoing stem cell transplantation and chemotherapy for malignant disorders. There has been renewed interest in granulocyte transfusion due to better donor preparation, newer facilities for separating blood components and aggressive chemotherapy schedules resulting in prolonged neutropenia. This study was done to analyse the benefits and outcome of this adjuvant modalityType of study: Retrospective chart review.Place of study: PICU (Paediatric Intensive Care Unit) and BMT (Bone marrow transplantation) unit of a tertiary hospital. Inclusion criteria: All children with severe neutropenia causing sepsis, who failed to show defervesence despite aggressive antibiotic and hematopoietic factors managementMaterials and methods: Eleven children were included in the study. The male to female ratio was 2.6:1. Three children had granulocyte function disorder, 1 child had severe aplastic anaemia where as remaining 7 children had malignant disorders. Among the children with malignant disorders, 4 children had acute myeloid leukaemia of which 2 children had it due to myelodysplastic disorder. Two children had acute lymphoblastic leukaemia and 1 child was post bone marrow transplantation for neuroblastoma. Granulocyte transfusion was initiated when ongoing optimal therapy failed to improve the sepsis and neutropenia. A total of 30 units of granulocyte transfusion was used and collected from dexamethasone(8mg) primed donors. Resolving neutropenia and improving sepsis were considered endpoints for stopping granulocyte transfusion. Six children survived with the above measures. Children with granulocyte dysfunction showed better outcome than children with malignancy. No complications related to the transfusion were seen. Conclusion:Granulocyte transfusion may have a role as adjuvant therapy in treatment of severe neutropenia causing sepsis.The outcome is better when granulocyte transfusion is used in children with granulocyte dysfunction and overwhelming sepsis.

HO/11(P) GLUCOSE-6 PHOSPHATE DEHYDROGENASE DEFICIENCY

Rajiv Kumar, Nomeeta Gupta

Department of Pediatrics, Batra Hospital & Medical Research Centre, New Delhi-110062.

G-6-PD (Glucose-6-phosphate dehydrogenase) is an enzyme present in the RBC. Deficiency of this enzyme leads to shortening of the red-cell life span and hereditary non-spherocytic hemolytic anemia. It is inherited as an X-linked disorder. Case Report: A six years old male child born of third degree consanguineous marriage presented with fever for 10 days and high colored urine for 5 days. On examination, he had pallor, icterus and hepatosplenomegaly. He had bradycardia and basal crepitations suggestive of congestive cardiac failure. He had been treated with chloroquine 5 days back by a private practitioner for his fever. A clinical diagnosis of pyrexia with acute hemolysis was considered. His hemoglobin was 4.4 gm% with a WBC count of 10,400/cumm and platelet count of 4, 72,000/cumm. His reticulocyte count was 5.1% with corrected reticulocyte count of 1.7%. His serum bilirubin was 1.5 mg/dl with indirect bilirubin of 0.9 mg/dl. His liver enzymes were normal with direct coombs test and indirect coombs test being negative. A urine routine examination showed 40-50 pus cells with absent bile salts and bile pigments. Urine culture did not show any organism. Ultrasound of the kidneys showed normal kidney sizes with bright echotexture. He was screened for G-6-PD deficiency, which was low. The parents were negative for G-6-PD deficiency. Thus a diagnosis of urinary tract infection with G-6-PD deficiency was considered with acute hemolysis due to chloroquine ingestion. He was treated with IV cefotaxime for his UTI and required blood transfusions for anemia. He was advised to avoid antimalarials, sulpha drugs, chloramphenicol, nitrofurantoin, nalidixic acid and Vitamin K due to his G-6-PD deficiency state. He was discharged with iron supplements. On follow up after 1 month, his hemoglobin was 12gm%.
HO/12(P) NEONATAL PURPURA FULMINANS IN THREE CONSECUTIVE BABIES OF A FAMILY—DIAGNOSIS AND MANAGEMENT

Sona Chowdhary,Uma Khanduri,Rajeev Arora

Department of Pediatrics,St. Stephen's Hospital,Tis Hazari,Delhi-54
Background Neonatal purpura fulminans is a manifestation of thromboembolic phenomenon. It may have a varied aetiology ranging from infections, NEC, congenital viral disease to the uncommon hereditary thrombotic disorders.Infants with this condition in clinical practice, are commonly assumed to be due to sepsis. Aim To present a series of babies born to the same parents with neonatal purpura fulminans and report their aetiological diagnosis and outcome. FIRST BABY: boy , born of a non consangiious marriage at term, was found to be normal at birth. In the early neonatal period he developed black coloured patches over the body. A diagnosis of sepsis and DIC was made though the blood cultures were sterile, but died at 22 days despite treatment. SECOND BABY: A boy born at term by LSCS. No abnormality was found at birth but developed black patches, similar to the first baby, on fourth day of life. He was extensively worked up and was found to have Protein C activity level of 0% which increased to 16% at about 1 year of age. He was managed with repeated FFP transfusions and warfarin,but succumbed at 16 months. THIRD BABY: Girl, born at our hospital at term by LSCS. Physical examination at birth showed no abnormality.On third day of life, she developed necrotic patches over skin over both the trochanters, despite prophylactic FFP transfusion.. Sepsis screen was negative and coagulation profile was within normal limits. Protein C levels were found to be only 39%. She came back at 15 days of life with necrotic skin patches over abdomen.She remains on follow up with FFP transfusions,heparin and warfarin. Conclusion Neonatal purpura fulminans should be extensively worked up so that the rare aetiology which may have a serious implication on the final outcome of the baby is well recognized.