Disseminated Mycobacterium scrofulaceum infection in a child with interferon-γ receptor 1 deficiency

Summary Disseminated disease caused by non-tuberculous, environmental mycobacteria (EM) reflects impaired host immunity. Disseminated disease caused by Mycobacterium scrofulaceum has primarily been reported in patients with AIDS. Moreover, observing M. scrofulaceum as the agent of localized disease in childhood has become increasingly rare. We report the first case of disseminated disease caused by M. scrofulaceum in a child with inherited interferon-γ receptor 1 (IFN-γR1) complete deficiency. As in this case, mycobacterial bone infections in IFN-γR1 deficiency can sometimes mimic the clinical picture of chronic recurrent multifocal osteomyelitis

Scanning electron microscopy study of small bowel biopsies in chronic diarrhoea in chlldhood

In thi, study wc have compared the results ot Scanning Electron Microscopy (SEM) with Light- and Stereomicroscopy in a series of small bowel biop\ies in children. In 9 cases displaying features of partial or wbtotal atrophy, Light and Dissecting-Microscopy yiclded similar results. The distinction between coeliac and noncoeliac chronic diari-hoen was only possible on cliiiical grounds, and by the iininunological detection of specific antibodies. On SEM however coeliac patients showed characteristic alterations consisting of: absence of villi: prominent crypt outlets resulting in a mosaic appearance: concentric furrows running al1 around the openings; and downy brush feature at higti power. The microvilli were loosely distributed and had an irregular pleomorphic outline; they often displayed a druinstick swelling of tlic tip and were bent. In contra5t. non-coeliac chronic diarrhoea cases were characterized bv a thick mucous layer on the mucosa1 surface, that made it impossible to visualize further chan~es. U Peculiar \iascular changes in Iymphangiectasia and in sickle beta thalasseinia could be detected only by Liglit Microscopy. In addition, in the lymphangiectasia ca\e SEM allowed the detection of enteroadherent bacteria: and in the lambliasi, case, of pseudomembi-rine\. Absence of glycocalyx was noted both in controls and in patients. The results of this study point to a diagnostic utility of SEM particularly in the differential diagnosis of chronic diarrhoea; moreover they suggest tliat enteroadherent bacteria may not be pathogenic and that the absence of glycocalyx is not specific for allergic enteropathy as previously claimed