Autoimmune Hemolytic Anemia in Children: Clinical Profile and Outcome.

OBJECTIVE: To discover the common triggers for AIHA in children, their clinical profile, treatment response, and outcome. METHODS: This was an ambispective descriptive study conducted between 2013 and 2020. Children aged 1 mo to 14 y with hemolytic anemia and a positive direct antiglobulin test (DAT) were included. Children with a positive DAT but without any clinicolaboratory evidence of hemolysis were excluded. Data were collected from a structured pro forma with particulars comprising clinicolaboratory profile, treatment administered, and disease outcome. RESULTS: A total of 46 children (aged between 1 mo and 14 y) were enrolled in the study. The mean age of onset was 8.7 (± 4.34) y, and 24 (52.8%) were males. Secondary causes were observed in 29 (63%) cases, while the primary cause was found in 17 (37%). Systemic lupus erythematosus (SLE) was the common trigger in 13 (45%) cases, followed by malignancy in 4 (14%) cases. Pallor (98%), hepatomegaly (72%), and splenomegaly (48%) were the most commonly observed clinical signs. The mixed immunophenotype was observed in 27 (59%) cases, followed by warm type in 12 (26%) and cold agglutinin type in 7 (15%) cases. All children received glucocorticoid therapy, and mycophenolate mofetil was commonly used as second-line therapy in 15 (33%) cases. 13 cases (71%) of primary AIHA and only 4 (14%) cases of secondary anemia achieved complete remission. Overall, 7 children (15%) died, all belonging to secondary AIHA. CONCLUSION: Secondary AIHA was more common than primary in the present study, and SLE was the standard trigger. Primary AIHA carries a better prognosis than secondary.

Effect of Vitamin D and Calcium Supplementation on Bone Mineral Content in Children with Thalassemia.

OBJECTIVE: To evaluate the effect of vitamin D and calcium supplementation for osteoprotection in thalassemia. METHODS: 29 children (age 2-12 y) were supplemented with oral vitamin D (1000 IU/d) and calcium (500 mg/d) for 1 year. The dual energy X-ray absorptiometry (DXA) was done to assess bone mineral content at baseline and 12 months. Serum 25-hydroxy vitamin D, intact parathyroid hormone, osteocalcin, calcium, phosphate, alkaline phosphatase, and spot urine deoxypyridinoline (DPD)/creatinine were done at baseline, 6 months and 12 months. RESULTS: The mean (SD) bone mineral content increased from baseline value of 8.4 (2.8) g to 10.8 (3.5) g (P<0.001). The mean (SD) vitamin D level increased from baseline value of 16.0 (5.8) ng/mL to 23.4 (6.6) ng/mL (P<0.001). The change in serum osteocalcin and spot urine DPD/creatinine ratio were not significant (P=0.062). CONCLUSION: Oral vitamin D and calcium supplementation increases bone mineral content in children with thalassemia.