Williams-Beuren syndrome: a rare presentation of aortic hypoplasia

William-Beuren syndrome (WBS) is a rare genetic disorder resulting from a micro-deletion of the elastin gene located on the long arm of chromosome 7 (7q11.23). Characterized by typical elfin facies, mental deficiency, overfriendly personality and occasionally infantile hypercalcemia together with various eye, teeth, cardiovascular, renal and skeletal abnormalities. Herein, we describe two cases of diffuse narrowing of the entire aorta, with associated pulmonary artery narrowing. To the best of our knowledge, these two cases of aortic hypoplasia associated with WBS are the first to be reported in the region

Incidence of Ventilator-associated pneumonia in the critical care unit at Kenyatta National Hospital, a public tertiary care hospital

Background: Ventilator-associated pneumonia (VAP), a severe type of hospitalacquired pneumonia develops 48-72 hours after initiation of mechanical ventilation. Objectives: This study aimed to determine incidence of VAP using the Clinical Pulmonary Infection Score (CPIS) which combines clinical, radiographic, physiologic and microbiological data into a numerical result, ranging from 0 to 12, and to identify risk factors associated with its development. A secondary objective was to assess the diagnostic utility of a positive culture of pathogenic bacteria on tracheal aspirate in predicting a positive culture on a mini-Broncho Alveolar Lavage (Mini-BAL). Design: A hospital-based, prospective cross-sectional study carried between 01st January 2015 to 31st March 2015. Setting: Kenyatta National Hospital, a tertiary care hospitalSubjects: Ninety-two subjects who met the inclusion criteria were included. Results: Of the 92 patients studied, 50 had a CPIS of ≥6, an incidence of 54.4% (C.I. 44.0-64.7%). Factors that appeared to show an association with VAP included documented aspiration (OR 2.0), a high nurse to patient ratio (OR 4.0), postsurgical patients (OR 2.5) and those who were nasally intubated (OR 4.0) and those with oral candidiasis (OR 3.5). Of the 50 patients that showed a CPIS of ≥6, 46 (92%) patients had a positive culture on tracheal aspirate and 31 (62%) patients demonstrated a positive mini-BAL culture. The sensitivity and specificity of a positive tracheal aspirate in predicting a positive min-BAL culture were 100% (C.I 88.7-100.0%) and 21.1% (C.I 6.2-45.6%) respectively. Negative predictive value of 100.0% (C.I 40.2-100.0%) and a positive predictive value of 67.4% (C.I 52.0-80.5%). Conclusion: Our study, the first documented in East Africa, found a high incidence of VAP. Further studies are needed to compare the diagnostic utility of various invasive and non-invasive tests for diagnosis of VAP

Successful retrieval of an embolised intracardiac umbilical venous catheter fragment in a 1190 gm preterm

Umbilical venous catheters (UVC) can be lifesaving in preterms and sick neonates. Although safe and effective, they are prone to various rare complications, most importantly, embolization of catheter fragments. We report a case of accidental transection of an UVC that was lodged in the left atrium appendage (LAA), with its inferior end within the inferior vena cava (IVC) and a challenging, however, successful retrieval using an EN Snare Catheter technique with improvised use of a Fogarty Balloon. To our knowledge, our case is the first reported in East Africa

Acquired subglottic stenosis, a rare case following short-duration endotracheal intubation post cardiac surgery

Subglottic stenosis is a known complication following intubation, both prolonged or of short duration. Patients who present with even mild symptoms of airway obstruction following a period of mechanical ventilation should be evaluated for possible post intubation subglottic or tracheal stenosis. Herein, we describe a rare case of subglottic stenosis following a two-day intubation period. To the best of our knowledge, this is the first to be reported in the region