Articles: ninos.
-
Over the last decade, the medical treatment of inflammatory bowel disease (IBD) has been revolutionized, with increasing use of both immunomodulatory and biologic medications. Corticosteroids have increasingly been shown to increase the risk of serious and opportunistic infections, both independently and in combination with immunomodulator and biologic agents. ⋯ It is unclear if anti-tumor necrosis factor-alpha agents increase overall infectious risk in patients with IBD, but the available literature has demonstrated an increased risk of opportunistic infections, particularly in terms of tuberculosis and histoplasmosis. Combination therapy likely increases the risk of opportunistic infections in patients with IBD but this has not yet been conclusively proved.
-
Fistulas manifest frequently in Crohn disease and can result in significant morbidity and often lead to the need for surgical intervention. Historically, it has been more difficult to obtain complete fistula closure in patients with perianal Crohn disease. Anti-tumor necrosis factor-alpha agents and the use of more accurate imaging modalities such as magnetic resonance imaging and rectal endoscopic ultrasound have enhanced the ability to manage fistulizing Crohn disease. A combined medical and surgical approach usually presents the best option for most patients.
-
Monitoring bone density within the first 3 years of therapy does not provide useful information—and it is costly besides.
-
Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD) and limb girdle muscular dystrophies (LGMD) represent a significant proportion of paediatric and adult neuromuscular neurology practice. The proactive symptom-based multidisciplinary team (MDT) management and access to non-invasive ventilation have enabled improved survival into adulthood. Nevertheless the severe disability imposed by conditions such as DMD poses a challenge for successful transition of care and management for paediatric and adult neurology teams. ⋯ LGMDs are much rarer than DMD and BMD, and in addition there is a significant genetic and clinical heterogeneity, which leads to diagnostic difficulties. The clinical and laboratory diagnostic features of seven LGMD subtypes are summarised, and their allelic "non-limb girdle" phenotypes are tabulated to illustrate the theme of one gene causing multiple clinical phenotypes, with the aim of refining the clinician's diagnostic approach. The lessons learnt from DMD MDT management to improve survival are broadly applicable to LGMDs with severe motor disability/multisystem complications.