Anesthetic Management of Prader Willi Syndrome pediatric patient undergoing Tonsillectomy Surgery: A Case Report

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 →

Category: 2023, 2023b, Case Reports
Keywords: anesthesia, Prader-Willi syndrome, tonsillectomy
Authors (a/b order): Çekmen N., Gökdemir B.N.

Anesthetic management of renal transplantation in a patient with Alport syndrome: A Case Report

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 →

Category: 2023, 2023a, Case Reports
Keywords: Alport syndrome, anesthetic management, hemodynamic monitoring
Authors (a/b order): Bihorac E., Çekmen N.

Anaesthetic Management In A Pediatric Patient With Malignant Transformation Of Hepatic Adenoma In Glycogen Storage Disease Type 1 (Von-Gierke) For Liver Transplantation: A Case Report

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 →

Category: 2022, 2022d, Case Reports
Keywords: anaesthetic management, Glycogen storage disease, hypoglycemia, Lactic acidosis, Liver transplantation
Authors (a/b order): Çekmen N., Ersoy Z., Sivrioğlu D., Torgay A., Tuç Ö.

Non-Operating Room Anesthesia (NORA) Management And VACTERL Syndrome: A Case Report

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 →

Category: 2022, 2022d, Case Reports
Keywords: Difficult Airway, multiple anomalies, non-operating room anesthesia, preoperative management, VACTERL syndrome
Authors (a/b order): Çekmen N., Uslu A.

Anesthetic Μanagement Of A Glycogen Storage Disease Type 1a With Air Embolism During Liver Transplantation

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 →

Category: 2022, 2022d, Case Reports
Keywords: air embolism, Glycogen storage disease type 1a, Liver transplantation
Authors (a/b order): Çekmen N., Haberal M., Kandemir E., Torgay A.

Anesthesia management of a patient with hereditary angioedema under Dexmedetomidine: A Case Report.

Hereditary angioedema (HAE) is a life-threatening rare autosomal dominant (AD) disorder caused by C1-esterase inhibitor (C1-INH) deficiency or dysfunction. Continue reading →

Category: 2022, 2022d, Case Reports
Keywords: anesthetic management, dexmedetomidine, fresh frozen plasma, Hereditary angioedema, icatibant
Authors (a/b order): Bihorac E., Çekmen N., Ersoy Z., Tuç Ö.

Anesthetic Management In A Patient With Crigler-Najjar Syndrome Undergoing A Dental Procedure: A Case Report

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 →

Category: 2022, 2022d, Case Reports
Keywords: anesthetic management, Crigler Najjar Syndrome, dental procedure, hyperbilirubinemia
Authors (a/b order): Çekmen N., Haka D., Kaya D.