The fourth issue of the Greek e-journal of Perioperative Medicine for 2022 presents various case reports concerning perioperative medicine.
The case of Sivrioğlu D et al. presents the perioperative management of an 8-year-old patient with Glycogen storage disease type Ia (GSD type-Ia), with malignant transformation of hepatic adenoma, that underwent liver transplantation. The authors note that the anesthetic management of these patients has important implications for anesthesiologists due to different system involvements.
The case report of Uslu A et al. presents the preparation and management of non-operating room anesthesia (NORA) in an 8-year-old patient diagnosed with VACTERL syndrome (multiple congenital malformations: vertebral, cardiac, tracheo-esophageal, renal and extremity anomalies, cardiac anomaly-large secundum ASD-, right renal agenesis, operated tracheoesophageal fistula and esophageal atresia).
The article of Kandemir E et al. evaluates the anesthetic management and considerations of a glycogen storage disease type 1a pediatric patient with air embolism during liver transplantation. The authors conclude a multidisciplinary approach and care should be taken in terms of limiting the presence of perioperative complications.
Next, the article of Papagiannopoulou O et al. shows that the intraoperative use of transesophageal echocardiogram (TEE) establishes the diagnosis (aneurysm dissection of the ascending aorta, a large thrombus… Continue reading
Glycogen storage disease type Ia (GSD-Ia; Von Gierke disease) is an inherited disease caused by glucose-6-phosphatase deficiency. Perioperative management of patients with GSD has important implications for anaesthesiologists due to different system involvements. Continue reading
VACTERL syndrome presents many challenges related to anesthesia management due to multiple congenital malformations that may include vertebral, cardiac, tracheo-esophageal, renal and extremity anomalies. Continue reading
Glycogen storage disease type 1a is a rare inborn error of metabolism. It causes severe fasting intolerance and lactic acidosis due to the deficiency of glucose-6-phosphatase enzyme. Continue reading
Although Computed Tomographic Angiography (CTA) is the established gold standard test for the detection of an aortic dissection, many researches and studies have proofed and established that transesophageal echocardiogram (TEE) can rule in or rule out the diagnosis of this catastrophic disorder, with greater accuracy. Continue reading
Hereditary angioedema (HAE) is a life-threatening rare autosomal dominant (AD) disorder caused by C1-esterase inhibitor (C1-INH) deficiency or dysfunction. Continue reading
Crigler Najjar Syndrome (CNS) is an ultra-rare autosomal recessive inherited disorder caused by a mutation in the uridine glucronyl 5’-diphosphate-glucronyl transferase (UGT) gene. Continue reading
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