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To evaluate the current role of liver transplantation (LT) for methylmalonic acidemia (MMA), we reviewed the literature on outcomes of this treatment, and describe three of our own cases of living-donor liver transplantation (LDLT). The total number of LT cases identified was 18. Transplantation mode was deceased donor LT in 12, including five combined liver-kidney transplantations (CLKT) from deceased donors, and LDLT in six. Three hospital mortalities were noted, because of metabolic decompensation, sepsis and aspergillosis. Although mean postoperative serum MMA level decreased to 13.8% +/- 9.2% (range 1.25-26.1%) of preoperative levels, four patients (22.2%) had renal insufficiency after isolated LT and three (16.7%) had postoperative neurological disability. Continuing metabolic damage to the kidney and brain may occur even after successful LT. Further evaluation is required to determine the long-term suitability of this treatment modality.

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Several studies have reported that postnatally acquired cytomegalovirus (CMV) infection can cause sepsis-like syndrome in premature infants. We here report a 622-gram birth weight male infant of 23 weeks’ gestation who had sepsis-like syndrome and pneumonia. Substantial CMV loads were detected in peripheral blood cells, plasma, and urine when the patient was in crisis, but was decreased in parallel to clinical improvement without using ganciclovir. CMV DNA was not detected from his umbilical cord or Guthrie card, even by highly sensitive real-time PCR. Molecular profiles were indistinguishable between the CMV strain isolated from his urine and that from maternal breast milk, indicating postnatal acquisition of CMV through breast milk. Although he had transient hearing impairment, his neurodevelopmental outcome of 30 months of corrected age was normal. Further accumulation of clinical and virological data in postnatal CMV infection is necessary for evaluating the severity and selecting patients requiring antiviral therapy.

No abstract