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

HO/01(P) INCIDENCE OF IRON DEFICIENCY IN NON ANAEMIC CHILDREN

Mir M.S,Qadri M.I, Sethi A.S, Farooq A., Buch N. A,

Govt. Hospital for Children Srinagar and SKIMS Soura Srinagar

Introduction: Iron deficiency ismost widespread nutritional deficiency in developing countries it manifests as latent iron deficiency and then iron deficiency anaemia.Among various parameters used to diagnose latent iron deficiency, serium ferritin essay stands the most dependable one. Aims and Objective: This study was an effort to know the incidence of latent iron deficiency in Kashmiri Paediatric publition present studies also shows that seruim ferritin estimation ismost dependable criterion for diagnosis of latent iron deficiency. Magnitude of latent iron deficiency in a population would guide in dietry modification and planing of iron suprementation to prevent iron deficiency anaemia which forms the main bulk of haemotological problems. Material & Method: Eighty non anaemic healthy children and 20 anemic children (as control) were subjected to blood sampling for complete haemogram, HCT bywintrops method, serium iron estimation by adoptation of peters method and serium ferritin by enzyme immunoessay test. Bone Marrow aspiration was done in aneamic children. results were compared and were tested for statistical significants. Results: Sd value of HB (gm/dl) HCT%, serium iron (mcg/dl) serum ferritin (ng/ml)in aneamic and non aneamicchildren were 6.2 +/- 1.52 VS12.4+/-0.87, 21.65 +/- 3.99 VS37.5 +/- 2.2, 49.9 +/- 50.7 VS 113.9+/- 64.4 and 16.6 +/- 14.6 VS 42 +/- 39.5 respectively. Differences were statistically significant.There was good correlation between bone marrow store and serium ferritin Conclusion: Serium Ferritin <12ngm/ml is dignostic of latent iron difeciency in non aneamic patients and iron dificiency aneamia in aneamic children. Incidence of latent iron difeciency in non aneamic children is 26.8%. Few studies are done to asses iron stores in indian children. Serum ferritin essay would be of imence help to diagnose latent iron difeciency and to asses iron status in population survey studies.

HO/02(P) IRON STATUS IN CHILDREN WITH SICKLE CELL DISEASE AND TRAIT

Sudhir Mishra, Dilip Kumar, DP Patra

Department of Pediatrics, Tata Main Hospital, Jamshedpur- 831001

Introduction: Sickle Cell Disease (SCD) and iron deficiency anemia are widely prevalent in tribal children of Eastern India. Increased iron absorption due to persistent anemia is considered to prevent iron deficiency in these children. Therefore iron is not given even if the hemoglobin is low but above levels requiring blood transfusion. Frequent blood transfusion increases risk of iron overload. Aims and Objectives: To study the iron status and its therapeutic implication in children with SCD and Sickle Cell Trait (SCT). Material and Methods: A prospective study, including children with hemoglobin AS and SS pattern between 3-18 years. Children with sickle cell beta thalassemia were excluded. Clinical details were recorded on pre-designed and pre-tested proforma. Investigations included complete blood count, reticulocyte count, MCV, MCH, MCHC, S. ferritin and S.iron. Other investigations were as indicated. Results: Thirty- six children (SCT-26, SCD-10) were studied. Low S. iron and ferritin were found in nine (25%) children (SCT-8, SCD-1). All eight children with high S. iron and S. ferritin (SCD-4, SCT-4) had received more than 10 units of blood. The difference in iron deficiency between children with SCD and SCT was statistically significant (p < 0.05). All children with iron deficiency showed clinical improvement within 15 days and haematologic parameters within one month of iron therapy. Chelation therapy reduced iron and ferritin levels. Conclusion: Data from this study suggests that iron status should be monitored in children with SCD and SCT as it has therapeutic implications.
HO/03(O) INTENSIVE IMMUNOTHERAPY FOR SEVERE/ VERY SEVERE APLASTIC ANEMIA.

Jagdish Chandra, Rahul Naithani, Shashi Narayan, Varinder Singh, Harish Pemde, Sunita Sharma, A K Dutta

Division of Pediatric Hematology, Deptt. of Pediatrics and 1Pathology, Lady Hardinge Medical College, Kalawati Saran Children’s Hospital, New Delhi.
Background and Objective: Acquired aplastic anemia (AA) is an uncommon but serious disorder characterized by pancytopenia resulting from nonfunction of the bone marrow. Although bone marrow transplantation (BMT) from a HLA identical sibling donor is the treatment of choice for severe / very severe aplastic anemia (SAA / VSAA), in developing countries this approach is limited due to various factors including cost of treatment, non-availability of expertise and matched donors. Several recent studies have shown encouraging results with intensive immunotherapy (IIST) including a combination of antithymocyte globulin (ATG) and cyclosporine (CSA) but the patients treated in these studies were primarily adults. There are several differences in disease characteristics, treatment methods, and therapeutic response between children and adults that have received little attention. The objective of this article is to report the results of IIST in SAA/ VSAA in children. Methods: Patients with AA were eligible if they met the following criteria: age < 12 years, recently diagnosed disease (within 120 days) without specific prior treatment, and severe to very severe aplastic anemia. The disease was considered SAA if at least two of the following were noted: a neutrophil count of less than 0.5 × 10⁹/L, a platelet count of less than 20 × 10⁹/L, and a reticulocyte count of less than 1% with hypocellular bone marrow . VSAA was defined if the criteria for severe disease were fulfilled and the neutrophil count were below 0.2 × 10⁹/L. Patients were treated with horse ATG (Lymphoglobuline; Merieux, Lyon, France) or Atgam (Upjohn, USA) or Thymogam (Bharat Serum, India)] with CSA . ATG was administered at a dose of 15-mg/ kg/day for 5 days 6 hours after a test dose. Cyclosporine (10 mg/kg/ day orally) was started on day 1 and continued at least until day 180.  Eight patients also received high dose methyl-prednisolone. Supportive therapy with packed red cell and platelet transfusion and appropriate antibiotics was administered as per protocol. Complete response (CR) was defined as transfusion independence with a neutrophil count >1.5 × 10⁹/L, a platelet count >100 × 10⁹/L, and a hemoglobin level of >10.0 g/dL.Partial remission (PR) was defined by transfusion independence and by an unsupported increase of the counts of at least one cell line over baseline values (Hb by at least 2 g/dL and actual value> 8g/dl; granulocytes by at least 0.5 × 10⁹/L, if previously lower than 0.5 × 10⁹/L and platelets > 30 × 10⁹/L, if previously lower than 20 × 10⁹/L) in patients with SAA or VSAA. Results : Seventeen (14 M: 3 F) aplastic anemia patients (6 VSAA and 11 SAA) were with median age 8 years (range 6-12 years) were included . Two patients died within 1 month of therapy. At 6 months, 8/15 (53 %) patients achieved at least PR. At the end of 1 year; 2 patients had achieved CR and 5 patients continued in partial remission while one died of septicemia after 8 month of therapy. The response was greater in SAA (54.5 %) with 2 CR and 4 PR; than in VSAA (33%) with 2 PR. One patient developed AML 13 months later. Seven (41%) patients were alive without response. Serum sickness was observed in three patients. Elevation of serum creatinine and liver enzymes due to CSA were observed in 8 patients but the changes were reversible on reducing the dose or temporarily stopping the drug. Conclusion: We conclude that IIST with antithymocyte globulin and cyclosporin with or without high dose methyl prednisolone is an effective treatment for severe and very severe aplastic anemia patients who are ineligible for bone marrow transplantation. IIST is well tolerated.
HO/04(O) DIVERSE CLINICAL PRESENTATION OF LANGERHANS CELL HISTIOCYTOSIS (LCH) WITH LIVER INVOLVEMENT

Sharma S, Mahajan A, Sibal A

Indraprastha Apollo Hospital, Mathura Road, Sarita Vihar, New Delhi
Childhood histiocytosis is rare and its diverse presentation makes it difficult to diagnose when encountered in pediatric practice. Liver lesion with presence of dysfunction at time of diagnosis is a poor prognostic sign and is seen in 20% of cases with LCH. In the past 18 months we saw 4 children aged 12,13,14 and 36 months, presenting with varied clinical features. The diagnoses before presenting to us were fever of unknown origin, galactosemia, tuberculosis and chronic otitis media with seborrhoeic dermatitis. They presented to us after no improvement was achieved. The three younger children had persisting fever for more than 2 months with skin rash and additionally presented with a)increasing irritability with reduced oral intake b)jaundice, hepatosplenomegaly and respiratory distress and c)weight loss, progressive icterus, pale stools and hepatomegaly. The 3-year old had recurrent ear discharge with skin rash and later developed localized swelling on the forehead. Skull X-ray showed a lytic bone defect and on direct questioning he was found to have polydipsia and polyuria. Ultrasound abdomen showed a solitary solid mass in the liver. LCH with liver involvement was considered and confirmed by histopathology in all 4 children. Treatment was initiated according to LCH III protocol and while 2 children are on follow-up, two children expired few months after presentation. While mild disease has an excellent prognosis and is self-resolving, multi-organ involvement (especially liver, lung and bone marrow) in children less than 2 years of age and lack of response during the first six weeks of treatment are poor prognostic factors with up to 66% mortality rate. Therapy for multi-organ involvement includes vinblastine, etoposide and prednisolone. Studies adding 6-mercaptopurine and methotrexate to the treatment plan have shown low incidence of sequelae of LCH, while overall mortality remains unchanged. New treatment approaches include the use of cyclosporine, antimetabolite 2-chlorodeoxyaenosine (2-Cda) and monoclonal antibody therapy with CD1a as the target epitope.