Observer variation and discriminatory value of biopsy features in inflammatory bowel disease.

If skilled histopathologists disagree over the same biopsy specimen, at least one must have an incorrect interpretation. Thus, disagreement is associated with, although not the cause of, diagnostic error. The present study aimed to determine the magnitude of variation among 10 observers with a special interest in gastrointestinal histopathology. They independently interpreted the same biopsy specimens for morphological features which may discriminate between patients with Crohn's disease and ulcerative colitis and normal subjects. Thirty of 41 features had agreement measures significantly better than expected by chance (p < 0.05). The range of agreement in the 45 observer pairs over the final diagnosis was 65-76%. There was good agreement in discriminating between normal slides and those showing confirmed inflammatory bowel disease. For normal slides, however, the term nonspecific inflammation was often applied and without any consistency. In addition, true Crohn's disease slides were often and consistently thought to be ulcerative colitis. Having identified 11 important discriminatory morphological features, two multiple regression analyses were then carried out to produce a scoring system for inflammatory bowel disease. These results suggest there is considerable room for improvement in the reliability of colonic biopsy specimen interpretation and that this could probably be achieved using more exact definitions of morphological features and diseases.     

Emotional adjustment of early adolescents with cystic fibrosis

Eighty-five 12- to 15-year-old adolescents regularly attending the cystic fibrosis (CF) clinic of The Hospital for Sick Children were asked to complete the Children's Health Locus of Control and the Tennessee Self Concept Scale. Their parents were requested to complete the Child Behavior Checklist. Thirty-four males (72%) and 28 females (74%) participated in the study. This study found that adolescents with CF are able to maintain a good self concept, be socially competent, and perceive that they are in control of their health while showing an increase in behavior problems. Females rely heavily on denial and are more behaviorally compliant, whereas boys use less denial but show more behavior problems. Males appear to integrate having a physical disorder into their self concept, whereas females do not. The findings demonstrate a difference in mechanisms of coping with cystic fibrosis between male and female adolescents with CF, which may contribute to the decline in physical status in females and better survival of males.     

The immotile cilia syndrome: radiological manifestations

The immotile cilia syndrome (ICS) is an uncommon disorder characterized by specific and genetically determined defects of cilia that cause upper and lower respiratory disease. We reviewed the radiographic patterns in 30 patients who had ICS (15 females, 15 males) and ranged in age from newborn to 26 years. Except for two neonates, sinusitis and otitis were present in all patients. Chest radiographic abnormalities, universally present, included bronchial wall thickening, hyper-inflation, segmental atelectasis or consolidation, and segmental bronchiectasis. Situs inversus, present in 50% (7 females, 8 males), was not an essential part of this disorder. Radiologically, the disease progresses from bronchial wall thickening with or without hyperinflation, to increasing hyperinflation plus parenchymal changes including segmental atelectasis, consolidation, and bronchiectasis. There is also a predilection for anatomic middle lobe abnormalities. The radiological appearance and clinical state have similarities to cystic fibrosis, although they are less severe and less progressive. ICS should be considered in the differential diagnosis of slowly progressive chronic lung disease, sinusitis, and otitis.