• Vishal Jatana, Jonathan Gillis, Boyd H Webster, and Lesley C Adès.
• Paediatric Intensive Care Unit, The Children's Hospital at Westmead, New South Wales, Australia. vishaljatana@ausdoctors.net
• Pediatr Crit Care Me. 2007 Sep 1;8(5):459-63; quiz 464.

ObjectiveTo report on the experience of a pediatric intensive care unit (PICU) with patients with deletion 22q11.2 syndrome: 1) to delineate the clinical characteristics and management of these patients; 2) to assess whether these patients were managed appropriately, especially in terms of blood transfusion; and 3) to make recommendations for PICU management.DesignRetrospective assessment of medical records of patients with fluorescent in situ hybridization-proven 22q11 deletion admitted to the PICU at the Children's Hospital at Westmead, Sydney.SettingPICU in a tertiary university-affiliated children's hospital.PatientsSixty-five consecutive admissions in 40 patients with diagnosis of 22q11 deletion over a 4-yr period.InterventionsNone.Measurements And Main ResultsThirty-seven (57%) of 65 admissions were postoperative cardiac surgical and accounted for the most common reason for admission to the PICU. Thirteen (20%) admissions were for velopharyngeal/laryngeal problems. Four (6%) admissions were associated with hypocalcemia, with two being first presentations. Five (12.5%) of 40 patients had immune dysfunction, one of whom developed cytomegalovirus pneumonitis. Twenty-nine (72.5%) patients received blood products either immediately before PICU admission or in the PICU. Of these, 16 received nonirradiated cellular blood products. There were two deaths from complications of congenital heart disease.ConclusionsPICUs need to be familiar with deletion 22q11.2 syndrome, especially the recommended use of irradiated and cytomegalovirus-seronegative blood components in these immunocompromised patients. The guidelines were inconsistently followed in the cohort of patients reported here. The extent of this problem may be more widespread in PICUs, and we recommend that individual units review their practice in this regard. Hypocalcemia may manifest at any time, and a regular survey of the calcium status is required in the intensive care setting. Admission to PICU should afford the opportunity to invite subspecialty referral and optimize extended care.

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