Anesthetic management of a child with phosphomannomutase-2 congenital disorder of glycosylation (PMM2-CDG)

Abstract Background Glycosylation is one of the major posttranslational modifications of proteins and it is essential for proteins to obtain normal biological functions. Congenital disorders of glycosylation (CDGs) are very rare genetic disorders that lack enzymes needed for glycosylation. Phosphomannomutase-2 (PMM2)-CDG is the most common type of CDG. CDGs can cause a wide variety of clinical symptoms in almost every organ system. Muscular hypotonia is often present in patients with CDGs and is one of the most notable problems for anesthetic management because the susceptibility to nondepolarizing neuromuscular blocking agents (NMBAs) in patients with CDGs is unknown. Case presentation The patient was a 17-month-old boy who weighed 6.5 kg and was 71 cm tall. He presented for strabismus surgery. He had muscular hypotonia, mental retardation, hepatic dysfunction, mild cerebellar hypoplasia, and some dysmorphic features including inverted nipples and abnormal subcutaneous fat distribution of the hips. Gene analysis revealed a compound heterozygous mutation in the gene encoding PMM2 and the patient was diagnosed as having PMM2-CDG. General anesthesia was performed with sevoflurane, nitrous oxide, and rocuronium. Neuromuscular monitoring was performed during anesthesia using train-of-four (TOF)-Watch® (MSD, Japan). As postoperative analgesia, the surgeon performed sub-Tenon’s anesthesia. We did not use any intravenous analgesic. After completion of the operation, residual rocuronium was competed by administration of sugammadex. The patient gained consciousness and spontaneous breathing was established shortly thereafter, and the trachea was smoothly extubated. He was transported to an inpatient ward and was discharged to his home the next day without any complication. Conclusions We performed safe anesthetic management in a 17-month-old boy with PMM2-CDG using rocuronium under neuromuscular monitoring. A patient with PMM2-CDG may show nearly normal susceptibility to nondepolarizing NMBAs

Epidural anesthesia combined with sedation with dexmedetomidine for appendectomy in a patient with amyotrophic lateral sclerosis: a case report

Abstract Background Patients with amyotrophic lateral sclerosis (ALS) present increased risks for anesthesia-related complications. We present a case of epidural anesthesia combined with sedation with dexmedetomidine for open appendectomy in a patient with ALS who refused invasive mechanical ventilation. Case presentation A 50-year-old man with a 3-year history of ALS was scheduled to undergo open appendectomy due to repeated appendicitis. He refused to undergo invasive mechanical ventilation using an endotracheal tube. Hence, we decided to administer epidural anesthesia combined with sedation with dexmedetomidine for anesthesia during the surgical procedure. The patient underwent open appendectomy without complications and with no pain or discomfort during surgery. There were no neurological complications at the 3-month follow-up after surgery. Conclusions Epidural anesthesia combined with sedation with dexmedetomidine may be effective for the anesthetic management of patients who would benefit from regional anesthesia

Remote ischemic preconditioning reduces myocardial ischemia-reperfusion injury through unacylated ghrelin induced-activation of the JAK/STAT pathway

遠隔虚血プレコンディショニングの心臓虚血再灌流傷害に対する心保護戦略とされているが，その機序には液性保護因子や神経系の関与が指摘されているが全容解明に至っていない．本研究では遠隔虚血プレコンディショニングの液性保護因子の1つとして，非アシル化グレリンの分泌がJAK/STAT経路の活性化に関与し心臓虚血再灌流傷害の減弱に寄与している可能性がある

Case Report Tracheal Intubation through the I-gel for Emergency Cesarean Section in a Patient with Multidrug Hypersensitivity: A New Technique

31-year-old female with hypersensitivity to local anesthetics and neuromuscular blocking agents presented for emergency Cesarean section. We successfully performed I-gel-assisted tracheal intubation without using neuromuscular blockers. We believe this method would be helpful in selected situations

Evaluation of the Effects of the Japanese Herbal Medicine “Goreisan” on Traumatic Cerebral Edema in Rats and Identification of Active Ingredients in Cultured Astrocytes

Purpose: Traumatic brain injury is associated with a high mortality rate, and the ensuing cerebral edema further raises the fatality rate. Goreisan（Oryeongsan）, a traditional Japanese herbal Kampo medicine, has been recently shown to be effective against chronic subdural hematoma and cerebral edema associated with tumors or ischemia. However, no study has reported these effects of Goreisan. Thus, we investigated the effect of Goreisan on cerebral edema occurring as a sequela of traumatic brain injury and explored the possible mechanism underlying its activity. Methods: Goreisan was administered to rats after the induction of traumatic brain injury via the open-skull controlled cortical impact method, and brain water content was measured. In astrocyte cultured cells, cellular edema was measured after exposure to Goreisan, Sojutsu（a crude constituent of Goreisan）, and β-eudesmol（an ingredient of Sojutsu）. Results: Seven days after traumatic brain injury surgery, the brain water content of rats treated with Goreisan was significantly lower than that of rats treated with saline（P = 0.0035）. Swelling was significantly suppressed in the Goreisan-, Sojutsu-, and β-eudesmol-exposed groups（P = 0.013, 0.0383, and 0.0010, respectively）. Conclusion: Goreisan appears to be effective in suppressing traumatic cerebral edema, and the effect may be mediated by its components, Sojutsu and β-eudesmol.departmental bulletin pape