ABSTRACT
Justification: Febrile seizures are quite common in children but there are controversies in many aspects of their diagnosis and management. Methods: An expert group consisting of pediatric neurologists and pediatricians was constituted. The modified Delphi method was used to develop consensus on the issues of definitions and investigations. The writing group members reviewed the literature and identified the contentious issues under these subheadings. The questions were framed, pruned, and discussed among the writing group members. The final questions were circulated to all experts during the first round of Delphi consensus. The results of the first round were considered to have arrived at a consensus if more than 75% experts agreed. Contentious issues that reached a 50- 75% agreement was discussed further in online meetings and subsequently voting was done over an online platform to arrive at a consensus. Three rounds of Delphi were conducted to arrive at final statements. Results: The expert group arrived at a consensus on 52 statements. These statements pertain to definitions of febrile seizures, role of blood investigations, urine investigations, neuroimaging, electroencephalography (EEG), cerebrospinal fluid analysis and screening for micronutrient deficiency. In addition, role of rescue medications, intermittent anti-seizure medication and continuous prophylaxis, antipyretic medication and micronutrient supplementation have been covered. Conclusion: This consensus statement addresses various contentious issues pertaining to the diagnosis and management of febrile seizures. Adoption of these statements in office practice will improve and standardize the care of children with this disorder.
ABSTRACT
Fanconi anemia is known to have a predisposition to cancer, mostly associated with acute myeloid leukemia. We report an eight-year-old girl with treatment and naïve FA who developed acute-lymphoblastic-leukemia (ALL). She was initiated on chemotherapy but she failed to respond to treatment and died during induction phase of chemotherapy. While this association may be coincidental but possibility of transition of Fanconi anemia to ALL should be considered in view of high predisposition to cancer in this disorder.
ABSTRACT
Objective: To determine the survival of children ≤18y, treated with immunosuppresive therapy (IST) using equine antithymocyte globulin (e-ATG) and cyclosporine (CsA). Design: Prospective data entry as per a specified format. Setting: Tertiary care hospital. Patients: From January 1998 to December 2009, 40 children were diagnosed with acquired aplastic anemia; 33 patients, who received IST, were analyzed. 31 children (94%) received one course of e-ATG and CsA. 2 patients (6%) received two courses of ATG. Intervention: Immunosuppressive therapy using equine ATG and cyclosporine. Main Outcome Measures: Overall response and overall survival. Results: The overall response (complete response + partial response) to IST at 6 months was 87.9%. 8 (24.2%) patients achieved CR, 21 (63.6%) patients had PR and 4 (12.1%) patients did not respond to IST. Median follow-up was 24 (6-102) months. Overall survival at 24 months was 90%, with an acturial survival of 85.4% at 5 years. Seventeen patients (51.5%) received G-CSF for a median duration of 32 (23-64) days. The patients who received G-CSF had fewer infectious complications (P=0.002), but G-CSF administration did not influence survival/ outcome. No patient developed myelodysplastic syndrome or acute leukemia. Conclusions: The survival of patients who respond to IST is excellent. Also, G-CSF reduces the infectious complications without conferring any survival advantage.