Clinical and immunohematological characterization of autoimmune hemolytic anemia in children.

Autoimmune Hemolytic Anemia (AIHA) in childhood is uncommon and estimated to be three per million annually under 18 years of age. Detailed immunohematological and clinical characterizations are essential for correct diagnosis of the disease and its management. In this study we described AIHA in children with regards to patient demography, underlying etiology, disease classification, antibody characterization, clinical features, degree of in vivo hemolysis and transfusion management. The prospective observational study was conducted over a period of 6 years and included 29 children with newly diagnosed AIHA. Patient details were obtained from the hospital information system and patient treatment file. The median age of the children was 12 years with a female preponderance. Secondary AIHA was observed in 62.1% patients. The mean hemoglobin and reticulocyte were 7.1 gm/dL and 8.8 percentages respectively. The median polyspecific direct antiglobulin test (DAT) grading was 3+. Red cell bound multiple autoantibodies were found in 27.6% children. Free serum autoantibodies were present in 62.1% patients. Twenty six of the 42 units transfused were "best match" or "least incompatible". Follow-up of 21 children showed clinical and laboratory improvement with DAT still positive at the end of 9 months. AIHA in childhood requires advanced and efficient clinical, immunohematological and transfusion support. Detailed characterization of AIHA is important, as they determine degree of in vivo hemolysis, disease severity, serological incompatibility and necessity of blood transfusion. Although blood transfusion in AIHA is a challenge but it should not be withheld in critically ill patients.

Polymorphism in the promoter of the CCL5 gene (CCL5G-403A) in a cohort of North Indian children with Kawasaki disease. A preliminary study.

OBJECTIVES: Kawasaki disease (KD) is a common vasculitic disorder and a leading cause of acquired heart disease in children. However, there is a paucity of information on KD from developing countries. The clinical phenotype of KD in India is different from that in the West. In this study, we investigated the association of a promotor gene variant of chemokines like chemokine ligand 5 (CCL5) and a deletion in chemokine receptor CCR5 (which is a common receptor for CCL5, macrophage inhibitory protein 1α and 1ß), in a cohort of North Indian children with KD. METHODS: CCL5 G-403A and CCR5Del 32 gene variants were genotyped in the KD cohort (n=40) and in healthy controls (n=100) using the PCR-RFLP assay. Logistic regression analysis was performed in order to examine the association of these variants with KD, with special reference to those with direct (on echocardiography) or indirect (on myocardial scintigraphy) evidence of coronary involvement. RESULTS: No significant difference in genotype or allele frequency of CCL5 G-403A variant was observed between patients and controls. However, patients with evidence of coronary involvement had a higher frequency of the minor allele CCL5 -403A (p<0.004; OR- 2.25, 95%CI: 1.13-4.46). CCR5 Del 32 variant was found to be monomorphic (minor allele frequency <0.05) in our cohort. CONCLUSIONS: CCL5 -403A variant may be associated with coronary involvement in North Indian children with KD. Our results, however, have to be replicated on a larger sample before any definitive conclusions can be drawn.

Malabsorption syndrome and leukotriene inhibitor.

Previously described treatments used for eosinophilic diseases of the gastrointestinal tract have included dietary restrictions primarily of cow milk protein, anti-inflammatory therapy utilizing suplatast, budesonide and corticosteroids, cromolyn sodium, anti-histamines and oral inhalable steroids. We describe a 12-year-old girl with diarrhea and malabsorption, who was later diagnosed to have eosinophilic gastroenteritis, was unresponsive to standard therapies, but exhibited marked improvement with use of montelukast.