Akademik Veri Yönetim Sistemi
Anestezi Dergisi, cilt.24, sa.1, ss.42-44, 2016 (Scopus)
Prolidase is an enzyme in the cell cytoplasm which has a crucial role particularly in the catabolism, and partially in the biosynthesis of collagen. Prolidase is responsible for the hydrolysis of dipeptides which contain proline and hydroxy-proline in the C-terminal. Prolidase deficiency is a rarely seen autosomal recessive disorder. However, since collagen constitutes about 1/3 of the total protein in the body and the foundation of tissue structure, it can affect patients within a wide spectrum. Clinical symptoms such as dysmorphic facial features, mental retardation, repetitive infections especially in the respiratory system, skin ulcerations, skeletal deformities, hematological anomalies and splenomegaly can be seen. These clinical cases may be serious and can sometimes be life threatening. Although this disorder is very rarely seen and not much is known about it, the accompanying clinical conditions may affect the management of anesthesia. In this case, a patient who was successful in the intelligence test in the borderline, with dysmorphic facial features, splenomegaly, diagnosis of ulcer in both bilaterally feet and osteomyelitis and prolidase deficiency diagnosis is presented. We would like to underline that mask ventilation may be difficult in the anesthesia management of these patients due to dysmorphic facial features, micrognathia, mandibular protrusion and the importance of careful evaluation of the accompanying clinical conditions in the pre-operative period.