LOWE SENDROMLU OLGUDA ANESTEZİK YAKLAŞIM

Berrin IŞIK; Mustafa ARSLAN; Mehmet AKÇABAY

Article Details

LOWE SENDROMLU OLGUDA ANESTEZİK YAKLAŞIM

Authors:

Berrin IŞIK | Mustafa ARSLAN | Mehmet AKÇABAY

Publisher:

Erciyes University

Journal:

Journal of Health Sciences 2005 Vol.14 Issue 3, pp.205-208

Document Type:

Article

Publication Date:

11/2005

Document Language:

Turkish

Document ID (Org No.):

2000615111

Frequency:

Tri-annual

pISSN:

1018-3655

Abstract:

 

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 electrolyt levels and being avoided from applications which can influence acidose and affects of acidose, the case mentioned hereby, 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 post operative period could be maintened.

 

Okülo-Serebro-Renal Lowe (OCRL) Sendromu ya da Lowe Sendromu (LS); konjenital katarakt, mental retardasyon ve Renal Fanconi Sendromuyla seyreden, X kromozomuna bağlı, resesif geçişli, nadir görülen bir sendromdur. Renal Fanconi Sendromu proksimal tubulus fonksiyon yetersizliği nedeniyle yenidoğan döneminde asidoz, aminoasitüri, fosfatüri ve proteinüriye neden olur. OCRL Sendromu seyrinde izlenen katarakt ve glokom nedeniyle bu olgular genel anestezi uygulanmasına adaydırlar. Ancak kas iskelet sistemine ait anomaliler, dental kistler ve metabolik problemler nedeniyle anestezi uygulaması öncesi veya anestezi sırasında dikkat edilmelidir. Sunulan; OCRL Sendromu tanılı, entübasyon güçlüğü yaşanan ve operasyon sırasında sodyum bikarbonat verilmesi gereken olguda peroperatif kan gazları ve plazma elektrolit düzeyleri izlenerek asidoz ve asidozun etkilerini artıracak uygulamalardan kaçınıldığında anesteziden kaynaklanabilecek komplikasyonların oluşmadığı postoperatif dönemin sağlanabildiği gözlenmiştir.

Article Details

LOWE SENDROMLU OLGUDA ANESTEZİK YAKLAŞIM

Authors: Berrin IŞIK | Mustafa ARSLAN | Mehmet AKÇABAY

Publisher: Erciyes University

Journal: Journal of Health Sciences 2005 Vol.14 Issue 3, pp.205-208

Document Type: Article

Publication Date: 11/2005

Document Language: Turkish

Document ID (Org No.): 2000615111

Frequency: Tri-annual

pISSN: 1018-3655

Issues

Authors:

Berrin IŞIK | Mustafa ARSLAN | Mehmet AKÇABAY

Publisher:

Erciyes University

Journal:

Journal of Health Sciences 2005 Vol.14 Issue 3, pp.205-208

Document Type:

Article

Publication Date:

11/2005

Document Language:

Turkish

Document ID (Org No.):

2000615111

Frequency:

Tri-annual

pISSN:

1018-3655