Ankylosing spondylitis (AS) is a chronic inflammatory arthritis characterized by progressive ossification of the spinal column with resultant stiffness. Patients, with AS, have a suppressed immune function due to proinflammatory cytokines and chemokines which are responsible for the inflammation of the joint. Continue reading
Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by various abnormalities, such as hypotonia, high body mass index (BMI), craniofacial anomalies, and obstructive sleep apnea. Anesthetic management of these patients requires special perioperative planning and care due to multisystem involvement. Continue reading
Alport syndrome (AS) is a rare hereditary disease, in which there is a defect in type IV collagen, an essential constituent of basal membrane in kidney, ear and eye. This can cause progressive renal damage and eventually renal failure, as well as hearing loss and visual impairment. Definitive treatment, when end stage renal disease (ESRD) has ensued, is renal transplantation. 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
Four patients (♂/♀: 1/3), aged 51, 52, 50 and 58 years old, who underwent general surgery procedures, suffered acute major blood loss intraoperatively. For the management of acute hemorrhage, 4ml/kg NaCl 7.5% were administered in each patient at the fastest possible rate through the existing intravenous line. Both standard monitoring and Oesophageal Doppler Monitoring (ODM) were applied and heart rate (HR), mean arterial pressure (BPmean), stroke volume (SV), peak velocity (PV), mean acceleration (MA) and corrected flow time (FTc) were recorded at six different phases, before blood loss (Phase 1), before and after completion of NaCl 7.5% administration (Phases 2 & 3), 10min and 20min after NaCl 7.5% administration (Phases 4 and 5) and at the end of the surgical procedure (Phase 6). Continue reading
