Özet:

Oculocerebrorenal Syndrome of Lowe (OCRL) or Lowe Syndrome (LS), is a rare X chromosome-linked disorder that is characterized by severe mental retardation, congenital cataracts, and Renal Fanconi Syndrome. The Renal Fanconi Syndrome develops in the neonatal period with impaired renal proximal tubular function including acidosis, aminoaciduria, phosphaturia, and proteinuria. Patients with OCRL may also candidate to general anaesthesia by reason of cataract and glaucoma. However musculo-skeletal abnormalities, dental cysts and metabolic acidosis and hypokalemia are the most important aspects and should be noticed preoperative or intraoperatively. In this case report; By monitoring peroperative blood gases and plasma electrolyte levels and being avoided from applications that can influence acidose and affects of acidose, the case mentioned here by, who has OCRL Syndrome and has entubation difficulties with the need of sodium bicarbonate that has to be given during operation, didn't show any complication caused by anesthetics and the postoperative period could beined

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