Children can be accurately diagnosed with celiac disease without biopsy analysis. Diagnosis based on level of TGA-IgA 10-fold or more the ULN, a positive result from the EMA tests in a second blood sample, and the presence of at least 1 symptom could avoid risks and costs of endoscopy for more than half the children with celiac disease worldwide. HLA analysis is not required for accurate diagnosis. Clinical Trial Registration no: DRKS00003555.
Background: Systemic aspergillosis is a serious disease of dogs for which the clinical characteristics are poorly described. Objective: To describe the clinical and diagnostic imaging characteristics of dogs with systemic aspergillosis. Animals: Thirty dogs with systemic aspergillosis. Methods: Retrospective case review. Medical records were reviewed for signalment, clinical features, and results of clinicopathologic testing and diagnostic imaging. Diagnosis was confirmed by culture of Aspergillus terreus (n 5 13), Aspergillus deflectus (n 5 11), or other Aspergillus spp. (n 5 6).Results: Compared with the background hospital population, German Shepherd dogs and female dogs were overrepresented (odds ratio [OR] 43, 95% confidence interval [CI] 20-91, P o .0001, and OR 2.9, 95% CI 1.2-6.7, P 5 .02), respectively, with 20 of the 30 dogs being German Shepherd dogs and 77% (23 of 30) of the dogs being female. The median age was 4.5 years (range 2-8 years). Anemia, leukocytosis, hyperglobulinemia, azotemia, hypercalcemia, and hypoalbuminemia were present in 8, 21, 12, 9, 8, and 6 dogs, respectively. Diskospondylitis, osteomyelitis and thoracic lymphadenomegaly were present in 16, 10, and 5 dogs, respectively. Sonographic findings were enlarged hypoechoic lymph nodes (n 5 12), mottled and irregular kidneys with or without masses (n 5 12), pyelectasia, and an aggregate of echogenic material in the renal pelvis (n 5 9). Thirteen dogs were treated with antifungal drugs, with survival times ranging from 0 to 25 months after diagnosis.Conclusions and Clinical Importance: Systemic aspergillosis typically involves young to middle-age female German Shepherd dogs, and there are characteristic abdominal ultrasound findings with the disease process. Infection with A. deflectus was as common as A. terreus, and in rare cases, long-term survival was associated with antifungal therapy.
BackgroundEvidence for the use of telephone consultation in childhood inflammatory bowel disease (IBD) is lacking. We aimed to assess the effectiveness and cost consequences of telephone consultation compared with the usual out-patient face-to-face consultation for young people with IBD.MethodsWe conducted a randomised-controlled trial in Manchester, UK, between July 12, 2010 and June 30, 2013. Young people (aged 8–16 years) with IBD were randomized to receive telephone consultation or face-to-face consultation for 24 months. The primary outcome measure was the paediatric IBD-specific IMPACT quality of life (QOL) score at 12 months. Secondary outcome measures included patient satisfaction with consultations, disease course, anthropometric measures, proportion of consultations attended, duration of consultations, and costs to the UK National Health Service (NHS). Analysis was by intention to treat. This trial is registered with ClinicalTrials.gov, number NCT02319798.FindingsEighty six patients were randomised to receive either telephone consultation (n = 44) or face-to-face consultation (n = 42). Baseline characteristics of the two groups were well balanced. At 12 months, there was no evidence of difference in QOL scores (estimated treatment effect in favour of the telephone consultation group was 5.7 points, 95% CI − 2.9 to 14.3; p = 0.19). Mean consultation times were 9.8 min (IQR 8 to 12.3) for telephone consultation, and 14.3 min (11.6 to 17.0) for face-to-face consultation with an estimated reduction (95% CI) of 4.3 (2.8 to 5.7) min in consultation times (p < 0.001). Telephone consultation had a mean cost of UK£35.41 per patient consultation compared with £51.12 for face-face consultation, difference £15.71 (95% CI 11.8–19.6; P < 0.001).InterpretationWe found no suggestion of inferiority of telephone consultation compared with face-to-face consultation with regard to improvements in QOL scores, and telephone consultation reduced consultation time and NHS costs. Telephone consultation is a cost-effective alternative to face-to-face consultation for the routine outpatient follow-up of children and adolescents with IBD.FundingResearch for Patient Benefit Programme, UK National Institute for Health Research.
The mucopolysaccharidoses (MPSs) are a group of inherited, metabolic disorders characterized by progressive multisystem accumulation of partially degraded glycosaminoglycans. This manifests with multilevel airway obstruction, presenting with obstructive sleep apnea (OSA). We systematically reviewed the literature to determine the severity and prevalence of OSA in MPS based on polysomnography analysis. Fifteen studies with 294 participants met the inclusion criteria for review. The pretreatment prevalence of OSA in MPS was 81% with a mean apnea-hypopnea index (AHI) of 10.4. Patients with MPS I are most significantly affected, with 75% suffering with moderate to severe OSA (mean AHI, 16.6). Enzyme replacement therapy (ERT) results in an almost significant reduction in OSA in MPS I (P ¼ .06), while adenotonsillar surgery significantly improves AHI (P ¼ .002). Obstructive sleep apnea least affects MPS III. There is a lack of long-term post-ERT and hematopoietic stem cell transplant data relating to OSA outcomes in this population, with further prospective studies required to determine the ongoing response to treatment.
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