Chediak–Higashi syndrome in the intensive care unit

• Published in: Pediatric anesthesia Vol. 14; no. 8; pp. 685 - 688
• Main Authors: Demirkiran, Oktay, Utku, Tughan, Urkmez, Seval, Dikmen, Yalim
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.08.2004; Blackwell
• Subjects: Acinetobacter Infections - diagnosis; Acinetobacter Infections - drug therapy; anaesthesia; Anesthesia; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Anti-Bacterial Agents - therapeutic use; Biological and medical sciences; Blood Transfusion - methods; Chediak-Higashi Syndrome - diagnosis; Chediak–Higashi syndrome; Child; Critical Care - methods; Female; Hematocrit - methods; Humans; intensive care; Intensive Care Units; Intubation, Intratracheal - methods; Medical sciences; Monitoring, Physiologic - methods; Oral Surgical Procedures - adverse effects; Plasma; Postoperative Hemorrhage - therapy; Pseudomonas Infections - diagnosis; Pseudomonas Infections - drug therapy; Respiration, Artificial - methods; Immunopathology; Skin disease; intensive care; Hemopathy; Chediak syndrome; Immune deficiency; Intensive care unit; Genetic disease; Eye disease; Chediak-Higashi syndrome; Anesthesia; Leukocyte disease; anaesthesia
• ISSN: 1155-5645; 1460-9592
• DOI: 10.1111/j.1460-9592.2004.01257.x

Summary Chediak–Higashi Syndrome is a rare autosomal recessive disease characterized by recurrent infections, giant cytoplasmic granules and oculocutaneous albinism. We describe the clinical and laboratory findings of a patient with Chediak–Higashi syndrome who was diagnosed and treated in the intensive care unit because of bleeding tendency after surgery.