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    Anesthesia management of a patient with Wilson's disease
    (Anestezi Dergisi, 2015) Do?u, Tu?ba; Şahin, Hasan; Erbaş, Mesut; Şimşek, Tuncer; Toman, Hüseyin; Altinişik, Hatice Betül; Kiraz, Hasan Ali
    Wilson's disease (WD) is an autosomal recessive disorder characterized by mutations in the ATP7B gene, which causes the disorder of copper excretion into bile. In this study we aimed to discuss our anesthesia experience in a Wilson's disease patient who underwent emergency surgical procedures for appendicitis. A 42-year-old male WD patient suffering the disease for 6 years with a diagnosis of cardiomyopathy and asthma, was planned an emergency surgery for appendicitis. The patient's neurological examination was normal. General anesthesia was preffered and for induction 2 mg kg-1 propofol, 1 ?g kg-1 fentanyl, and 0.5 mg kg-1 rocuronium was intravenously administered. Anesthesia was maintained with 50% oxygen and 50% air and 2% sevoflurane. The patient was extubated uneventfully, subsequently postoperative choreiform movements occurred. During the postoperative follow-up, the patient's choreiform movements were observed to disappear and declined by between 3-6 hours. We did not observe a delay in the patient's anesthesia recovery. Consequently, we think general anesthesia can be uneventually applied in Wilson's disease patients by using least toxic agents to the liver, protective measures and with an attentive follow-up in the perioperative and postoperative periods.
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    Anesthetic management in Conn Seyndrome: Case report
    (Anestezi Dergisi, 2015) Altinişik, U?ur; Altinişik, Hatice Betül; Do?u, Tugba; Aydin, Halide; Erbaş, Mesut
    Objective: Primary hyperaldesteronism (Conn Syndrome) is a disease with characteristics that affect aspects of anesthesia. We present this Conn Syndrome case, with attacks of hypertension, late diagnosis and surrenalectomy planned, to draw attention to anesthetic management. Case Report: A 41-year old male monitored by the endocrinology service for Conn Syndrome applied to the anesthetic clinic with planned laparoscopic surrenalectomy. The case had fatigue and muscle weakness. He first had a hypertensive attack in 2002, however diagnosis wasnt made. The case was using aldactazide, olmesartan and lercanidipine. Examination showed no extra pathology. Preoperative KCl infusion was recommended. On operation; blood pressure (BP): 185/115 mmHg, K: 4.2 mmol L-1 and glucose:133 mg dL-1. Midazolam used for premedication. For induction 1 mg kg-1 fentanyl, 2 mg kg-1 propofol, 05 mg kg-1 lidocaine, and 0.6 mg kg-1 rocuronium were administered while 50%O2 %50 air mixture with additional 6%desflurane was used. Arterial catheterization was done. When BP was above 160/100 mmHg, 0.15 mg kg-1 h-1 remifentanil was started. Slight metabolic alkalosis was identified on blood gas check. The frequance and tidal volume were reduced using the mechanical ventilator. During the rest of the operation hemodynamics, blood gas, potassium and glucose values remained stable. The case woke without incident and was sent to the ward. Conclusion: Anesthesia in Conn Syndrome; hypertension, hypervolemia, hypokalemia and metabolic alkalosis may be observed; appropriate preoperative anti-hypertensive treatment and potassium replacement is extremely important. Additionally blood pressure, potassium and blood gas values require frequent monitoring. Also we believe that patients with advanced age require careful evaluation for increased insulin resistance and heart and renal failure linked to fibrosis.