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Ureterolithotripsy under Spinal Anesthesia in Pompe Disease (Glycogen Storage Disease Type 2): A Case Report

Open Journal of Anesthesiology 2013年第2期3卷 61-63页

作者： arzu acar Mustafa Nuri Deniz Elvan Erhan Gulden Ugur Department of Anaesthesiology and Reanimation School of MedicineEge UniversityIzmirTurkey.

Pompe disease is an autosomal recessive inherited glycogen storage disease, usually respiratory muscles, cardiac muscle and skeletal muscles are affected in childhood and mortality is inevitable but has a slower progression in the adult. The patient was a 35-year-old male patient who was scheduled to undergo ureterorenoscopy due to right ureteral stone. He was diagnosed with pompe disease after detection of myopathy on EMG due to muscle weakness in the legs and hips. He was started on myozyme vials once every 15 days. Spinal anesthesia with 3 ml 0.5% hyperbaric bupivacaine was performed and adequate sensorial and motor block was achieves at T10 level. The surgery was completed with no complications and he was discharged two days later. The selection of appropriate anesthetic technique and a careful perioperative and postoperative monitoring are of utmost importance due to progressive muscle weakness, hypotonia, respiratory failure and cardiomyopathy in pompe disease.

关键词： Pompe Disease Spinal Anesthesia Ureterorenoscopie

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引用

World Journal of Gastroenterology 2010年第14期16卷 1765-1771页

作者： zcan eneli Zübeyde Nur zkurt Kadir acar Seyyal Rota Sahika Zeynep Aki Zeynep arzu Yegin Münci Yagci Seren zenirler Gülsan Türkz Sucak Department of Hematology Gazi University Faculty of Medicine Besevler 06500 Ankara Turkey Department of Microbiology Gazi University Faculty of Medicine Besevler 06500 Ankara Turkey Department of Gastroenterology Gazi University Faculty of Medicine Besevler 06500 Ankara Turkey

AIM: To investigate the frequency of occult hepatitis B, the clinical course of hepatitis B virus (HBV) reactivation and reverse seroconversion and associated risk factors in autologous hematopoietic stem cell transplantation (HSCT) recipients. METHODS: This study was conducted in 90 patients undergoing autologous HSCT. Occult HBV infection was investigated by HBV-DNA analysis prior to transplantation, while HBV serology and liver function tests were screened prior to and serially after transplantation. HBV-related events including reverse seroconversion and reactivation were recorded in all patients. RESULTS: None of the patients had occult HBV prior to transplantation. Six (6.7%) patients were positivefor HBV surface antigen (HBsAg) prior to transplantation and received lamivudine prophylaxis; they did not develop HBV reactivation after transplantation. Clinical HBV infection emerged in three patients after transplantation who had negative HBV-DNA prior to HSCT. Two of these three patients had HBV reactivation while one patient developed acute hepatitis B. Three patients had anti-HBc as the sole hepatitis B-related antibody prior to transplantation, two of whom developed hepatitis B reactivation while none of the patients with antibody to HBV surface antigen (anti-HBs) did so. The 14 anti-HBs-and/or anti-HBc-positive patients among the 90 HSCT recipients experienced either persistent (8 patients) or transient (6 patients) disappearance of anti-HBs and/or anti-HBc. HBsAg seroconversion and clinical hepatitis did not develop in these patients. Female gender and multiple myeloma emerged as risk factors for loss of antibody in regression analysis (P < 0.05). CONCLUSION: Anti-HBc as the sole HBV marker seems to be a risk factor for reactivation after autologous HSCT. Lamivudine prophylaxis in HbsAg-positive patients continues to be effective.

关键词： Autologous stem cell transplantation Hepatitis B reactivation Occult hepatitis Multiple mye- loma Lymphoma

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