Carnitine palmitoyltransferase (CPT) II deficiency, a very rare inherited disorder of mitochondrial fatty acid oxidation, is characterized by myalgia attacks leading to rhabdomyolysis, hyperkalemia, and acute renal failure. Here, we report an anesthetic management in an infant patient with CPT II deficiency. A 16-month-old child underwent implantation of a totally implantable central venous access device. General anesthesia was performed with sevoflurane, fentanyl, and rocuronium. No complications occurred during the perioperative period. In patients with mitochondrial fatty acid oxidation disorders such as CPT II deficiency, it is important to ensure continuous glucose infusion with blood sugar monitoring. Avoiding drugs associated with rhabdomyolysis and maintaining normothermia are keys for safe anesthetic management.
Yuhei MATSUDA1), Masaaki KARINO1), Katsumi HIDESHIMA1), Ichiro KANEKO1), Satoe OKUMA1), Rie OSAKO1), Hiroto TATSUMI2), Takashi KOIKE1), Yoshiki NARIAI3), Takahiro KANNO1) 1)Department of Oral and Maxillofacial Surgery, Shimane University Faculty of Medicine, Izumo, 693-8501, Japan 2)Division of Oral and Maxillofacial Surgery, Oki Hospital, Okinoshima-cho, 685-0016, Japan 3)Department of Oral and Maxillofacial Surgery, Matsue City Hospital, Matsue, 690-8509, Japan (Received August 30, 2019; Accepted October 1, 2019)
Mesenchymal stem cells (MSCs) perform multiple functions, such as immunomodulation and tissue repair, and they are also capable of differentiation into bone, cartilage, and fat cells. Furthermore, an MSC culture method has been established, and clinical safety is guaranteed; therefore, MSCs can be clinically applied for the treatment of many diseases. MSC treatment for hematological diseases is expected to be effective against refractory acute graft-versus-host disease (GVHD). It is presently used for treating chronic GVHD, preventing GVHD, promoting the engraftment of hematopoietic stem cells, and treating refractory aplastic anemia. However, owing to the cellular properties of MSCs, there are some concerns including increases in relapse, the deterioration of infectious diseases, and tumor formation or malignant transformation of MSCs. In the present review, I describe the present situation, problems, and prospects of the clinical application of MSCs for treating hematological diseases, including recent topics such as placental-derived decidual stromal cells and highly purified MSCs.
