Renal dysfunction in cystic fibrosis: Is there cause for concern?

Most people associate cystic fibrosis (CF) with lung disease. Although this is the major cause of morbidity and mortality, CF is in fact a multi‐organ disease. Patients with CF are living longer. Accompanying their increased life expectancy are complications not previously encountered. One of the less obvious concerns is that of renal dysfunction associated with long‐term exposure to aminoglycosides as well as renally toxic immunosuppressants in lung transplant recipients. This article reviews what is known about the extent of the problem, summarizes what the current practices of measuring and monitoring renal function in patients with CF, and makes suggestions for alternative approaches. In particular, the potential role of cystatin C will be discussed. Pediatr Pulmonol. 2009; 44:947–953. ©2009 Wiley‐Liss, Inc.     

A silent march-Post covid fibrosis in asymptomatics – A cause for concern?

We report a case series of patients presenting with undiagnosed pulmonary fibrosis as a primary manifestation. On evaluation, after excluding other causes, the fibrosis was attributed to asymptomatic or mild COVID illness in the past. This case series serves to highlight the difficulties posed to clinicians while evaluating pulmonary fibrosis in the post-COVID era, more so in mild to asymptomatic COVID-19. The intriguing possibility of fibrosis setting even in mild to asymptomatic COVID is discussed.     

Diabetes and osteoporosis – Treating two entities: A challenge or cause for concern?

People with T1D and T2D have an increased risk of fractures than the general population, posing several significant pathophysiologic, diagnostic, and therapeutic challenges. The pathophysiology is still not fully elucidated, but it is considered a combination of increased skeletal fragility and falls. Diagnostics issues exist, as regular and even newer scan methods underestimate the true incidence of osteoporosis and thus the fracture risk. Therefore, co-managing diabetes and osteoporosis by using top-line strategies is essential to preserve bone health and minimize the risk of falls. The therapeutic focus should start with lifestyle implementation and physical exercise interventions to reduce diabetic complications, strengthen bones, and improve postural control strategies. In addition, osteoporosis should be treated according to current guidelines by including bisphosphonates and antidiabetic drugs that support bone health. Finally, potentially modifiable risk factors for falls should be managed.     

Aerosolized antibiotic therapy for chronic cystic fibrosis airway infections: continuous or intermittent?

The use of inhaled therapies for chronic respiratory infections in cystic fibrosis represents a substantive treatment burden to patients. In this paper, we review the evidence supporting two commonly used inhaled antibiotic regimens for chronic respiratory infections - continuous vs. intermittent (28 days on followed by 28 days off) therapy. We included trials of good methodological quality and excluded those in which the primary intent was eradication. In total, we included 13 trials (5 of intermittent therapy and 8 of continuous therapy) and summarized their main findings, placing particular emphasis on change in FEV(1), emergence of resistance and patient adherence. What is evident from our review is that both continuous and intermittent inhaled therapies work. Although an intermittent regimen would be intuitively "better" in terms of cost savings and patient tolerability, there is currently a lack of head-to-head trials that compare the same drugs (and dosages) using the two different regimens to make such a recommendation based on robust clinical evidence.     

Small bowel lymphoma in unrecognized coeliac disease: a cause for concern?

BACKGROUND AND AIM: It is not clear whether the increased risk of small bowel lymphoma seen in typical coeliac disease also applies to unrecognized or screening-detected coeliac patients. The aim of the present study was to determine the features of small bowel lymphoma and whether it is associated with unrecognized coeliac disease at the time of presentation. DESIGN: A retrospective search by the five pathology laboratories serving Northern Ireland, UK. METHODS: Snomed searches were used to identify cases of adenocarcinoma (SBA) and lymphoma (SBL) affecting the small intestine between 1987 and 1996. Pathology reports were obtained and analysed with respect to the clinical features, site of pathology, type of tumour, grading and the presence or absence of distant villous atrophy. RESULTS: One hundred and thirty-eight cases were identified of whom 69 (44 men; mean age 60.2 years) were SBL and 69 (41 men; mean age 68.0 years) were SBA. Comparing the SBL to the SBA group the clinical presentation was perforation (10 vs 1, P= 0.009), small intestinal obstruction (20 vs 20, P= 1.0) and small intestinal mass (13 vs 15, P= 0.67). B-cell lymphomas occurred in 20 cases, T-cell lymphomas in 24 cases and 25 were unclassified. There was one known coeliac patient in the SBL group and none in the SBA group (P = 1.0). Villous atrophy at a distant site was recorded in 13 patients in the SBL group compared to none in the SBA group, all of these patients having T-cell lymphomas. CONCLUSIONS: Similar numbers of SBL and SBA occurred in the study period. Their clinical presentation was similar although perforation was more common in the SBL group. One-third of all SBL patients (over half of T-cell lymphomas) had distant villous atrophy, indicating that small bowel lymphoma appears to be significantly associated with unrecognized coeliac disease.