ABSTRACT
Metabolism is a tightly regulated sequence of events, supported by key reactions between enzymes and enzyme-specific substrates. These reactions have the potential to produce metabolic side products that can have deleterious effects to further key metabolic reactions. The nicotinamide repair system consists of two partner enzymes, NAD(P)HX epimerase (NAXE) and NAD(P)HX dehydratase (NAXD). These enzymes regulate the levels of metabolic side products. Here we present a case of an 11-month old child who presented to our paediatric department with pyrexia, lethargy and multiple cutaneous lesions on the background of NAXD deficiency, a lethal neurometabolic disorder of early childhood. Despite early intervention with intravenous antibiotics, the patient failed to improve and subsequently passed away. The skin lesions were thought to be a consequence of systemic disease rather than a propagator of infection. Clinicians should be aware of this incredibly rare metabolic disease, its potential to cause widespread systemic dysfunction and the developing avenues for management.
ABSTRACT
Capnocytophagia canimorsus (C. canimorsus) is a Gram-negative bacilli present in the gingival flora of canine and feline species. It is the second most common cause of infection following dog bites and contact with canine saliva, leading to severe sepsis in immunocompromised patients with no evidence of a breach to the skin. We present the case of a previously healthy 51-year-old male who presented with disseminated intravascular coagulopathy, acute renal failure with widespread haemorrhagic bullae and skin necrosis. He was treated empirically with broad-spectrum antibiotics for sepsis of unknown origin for several days before C. canimorsus infection was identified on blood cultures. Following this, a more detailed social history identified the vital historical detail that the patient owned 7 dogs. His purpura fulminans secondary to sepsis was managed conservatively with regular dressings by the Burns Department. Our experience demonstrates that C. canimorsus should be considered as a causative organism in patients presenting with sepsis of unknown origin after contact with domestic animals and treated with early antibiotic therapy.
ABSTRACT
OBJECTIVES/HYPOTHESIS: To this date the effect of the time taken to complete an evaluation on the psychometric properties of the instrument has not been reported. The goal of our study was to assess the effect of time taken to complete an evaluation on its validity. STUDY DESIGN: Cross-sectional validation study. METHODS: The global and checklist parts of tonsillectomy, mastoidectomy, rigid bronchoscopy, and endoscopic sinus surgery were used in the operating room by the otolaryngology faculty to evaluate the surgical skills of the residents. We categorized evaluations into two groups depending on the time taken to complete an evaluation (group A ≤ 6 days, group B >6 days). Construct validity was calculated for both groups by comparing the mean global and checklist scores of the residents across advancing postgraduate year levels. RESULTS: A total of 468 evaluations, consisting of global and checklist parts, were completed for 29 residents by 32 evaluators. Mean number of days taken to complete an evaluation was 7.7 days. For all the evaluations completed within a 6-day time period, the construct validity was significant for both global and checklist parts of the four instruments. In cases of the evaluations completed after 6 days, the construct validity was significant for the tonsillectomy instrument only. CONCLUSIONS: Our results indicate that the time taken to complete an evaluation has a significant effect on the construct validity of the objective instrument. In the future, efforts should be focused on faculty development to ensure timely completion of the evaluation for a more valid assessment process.
