inadequate response to the Pneumococcal vaccine; Literature Report.  This literature marketed report has been received from the authors of a published literature article and was presented for the long-term safety and efficacy of short-course immune tolerance induction (ITI) in a relatively large cohort of cross-reactive immunologic material (CRIM)-negative and CRIM-positive infantile Pompe disease (IPD) patients by evaluating anti-rhGAA IgG antibody titers, absolute neutrophil count (ANC), aspartate aminotransferase (AST), alanine aminotransferase (ALT), B cell and T cell quantitation, vaccination history and titers against vaccines, left ventricular mass index (LVMI), and overall and ventilator-free survival. Vaccine titers were available and adequate humoral response was observed in all except an inadequate response to the Pneumococcal vaccine in two CRIM-positive IPD patients which was as following.  This literature report refers to a male patient, who was currently 5.9-years-old (reported as 70.9 months). The patient's relevant medical history was not reported. Concurrent condition included CRIM-positive infantile Pompe disease. Concomittant therapies were not reported. On and unknown date, the patient had received a short-course of ITI with rituximab, methotrexate (dose, frequency, route, batch/lot# was not reported) in enzyme replacement therapy (ERT) for infantile Pompe disease and was subsequently vaccinated with pneumococcal vaccine, polyvalent (23-valent) (manufacturer unknown) (dose, frequency, anatomical location, batch/lot# were unknown) for prophylaxis.  The patient received 1 round of immune tolerance induction. Cross-reactive immunologic material (CRIM) status on an unknown date was positive.  On an unknown date, the CRIM-positive IPD patient demonstrated an inadequate response to certain serotypes of the pneumococcal vaccine, polyvalent (23-valent) (manufacturer unknown) (reported as "pneumococcal vaccine") (therapeutic response decreased).  It was reported that the patient was vaccinated prior to enzyme replacement therapy. The patients B cell reconstitution, vaccination status at baseline and after ITI, and titers against routine vaccines were collected. On an unknown date, gene encoding acid alpha-glucosidase (GAA) disease-associated variants for allele 1 was c.2457_246delCTG and for allele 2 was c.2560C greater than T. Enzyme replacement therapy (ERT) dose at the time of initiation 20 milligram per kilogram (mg/kg) every other week (EOW). Patient was initiated on or subsequently received ERT at a higher dose than the recommended dose of 20 mg/kg EOW. B Cell recovery was found 13 weeks post rituximab (RTX). Weeks post-RTX at last CD19 percentage was 248 weeks. Anti-rhGAA IgG antibody titers at peak titers was 0, at last titers was 0 (171 weeks on ERT) and was immune tolerant. The patient's humoral response to routine vaccinations was reviewed and response to tetanus, measles, mumps, rubella was found adequate and response to Pneumoccocal strain was confirmed to be inadequate.   Efficacy of ERT and ITI: Left ventricular mass index (LVMI) baseline was at 186 and final assessment was 61 (81 weeks on ERT).  Motor Status was reviewed: Baseline revealed good muscle strength, tone. Minimal head lag, lifts head up and Final assessment revealed to be not ambulatory, does not move legs in supine, requires assistance for head control in supported sitting (274 weeks on ERT). Ventilation Status: Baseline revealed no support and final assessment was reported as invasively ventilated (27weeks on ERT).  Feeding Status: At Baseline via nasogastric tube (NG) and at final assessment with gastrostomy tube (G Tube) (274 weeks on ERT)  Action taken with the co-suspect therapies rituximab and methotrexate was unknown. The outcome of the event was unknown. The Author considered the event therapeutic response decreased to be related to the suspect therapy. It was also reported that it is not clear if the lack of response to polysaccharide vaccines in this patient was due to immunosuppression or normal variability in response to pneumococcal vaccines.  A copy of the published article is attached as further documentation of the patient's experience.  This is one of two reports received via same literature article.; Sender's Comments:  US-009507513-2009USA003961: