A case of focal xanthogranulomatous pyelonephritis (XPN) in a child, successfully cured with antibiotics only, is presented, and this report is followed by a review of the literature on XPN in both children and adults. XPN is a rare inflammatory disease of the kidney. Factors strongly implicated in the development of XPN are genitourinary obstruction and urinary tract infection, although other contributing factors have been suggested. The clinical presentation of the disease is remarkably nonspecific and variable; it therefore is frequently misdiagnosed as other, more common diseases that cause focal or diffuse renal masses, including renal neoplasms, abscesses, and tuberculosis. Diagnosis requires acquisition of renal tissue for special pathological staining. The long-standing mainstay of therapy for XPN has been nephrectomy or partial nephrectomy. However, this report and other recent reports of the successful medical management of focal XPN suggest that a trial of antibiotics prior to surgery is warranted in cases of focal XPN.

Use of this in-line digestive cartridge was safe and well tolerated, and resulted in significantly increased levels of plasma omega-3 FA used with enteral formula, suggesting an overall increased fat absorption.

Azithromycin was associated with a significant reduction in the risk of pulmonary exacerbation and a sustained improvement in weight, but had no impact on microbiological outcomes in children with early Pa. Clinical trial registered with clinicaltrials.gov (NCT02054156).

Objectives:Pancreatic insufficiency (PI) and malabsorption of fats lead to reduced caloric intake, inability to maintain weight, and increased gastrointestinal symptoms. Thus, enteral nutrition (EN) is used in patients with cystic fibrosis (CF) and poor nutritional status. The current study evaluated safety, tolerability, and improvement of fatty acid (FA) status in red blood cell (RBC) membranes, a marker of long-term FA absorption, with an in-line digestive cartridge (RELiZORB) that hydrolyzes fat in enteral formula.Methods:Patients with CF receiving EN participated in a multicenter, 90-day open-label study during which RELiZORB was used with overnight EN. The primary endpoint was change over time in RBC uptake of docosahexaenoic acid (DHA)+ eicosapentaenoic acid (EPA). Gastrointestinal symptoms were collected to evaluate safety and tolerability. Several clinical and anthropometric parameters were also assessed throughout the study.Results:A total of 36 subjects completed the study with a mean age of 13.8 years, body mass index of 17.7 and 6.2 years mean use of overnight EN. Fat absorption significantly improved as shown by increased RBC levels of DHA+EPA, improved ω-6/ω-3 ratio, and increased plasma levels of DHA+EPA. RELiZORB use was not associated with any unanticipated adverse events.Conclusions:RELiZORB use was found to be safe, well tolerated, and resulted in increased levels of FAs in RBCs and plasma. This is the first prospective study to show EN can improve FA abnormalities in CF. Because improvement in omega-3 levels has been shown to help pulmonary and inflammatory status as well as anthropometric parameters in CF, RELiZORB may have important long-term therapeutic benefits in patients with CF.

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Background Biomarkers of inflammation predictive of cystic fibrosis (CF) disease outcomes would increase the power of clinical trials and contribute to better personalization of clinical assessments. A representative patient cohort would improve searching for believable, generalizable, reproducible and accurate biomarkers. Methods We recruited patients from Mountain West CF Consortium (MWCFC) care centers for prospective observational study of sputum biomarkers of inflammation. After informed consent, centers enrolled randomly selected patients with CF who were clinically stable sputum producers, 12 years of age and older, without previous organ transplantation. Results From December 8, 2014 through January 16, 2016, we enrolled 114 patients (53 male) with CF with continuing data collection. Baseline characteristics included mean age 27 years (SD = 12), 80% predicted forced expiratory volume in 1 s (SD = 23%), 1.0 prior year pulmonary exacerbations (SD = 1.2), home elevation 328 m (SD = 112) above sea level. Compared with other patients in the US CF Foundation Patient Registry (CFFPR) in 2014, MWCFC patients had similar distribution of sex, age, lung function, weight and rates of exacerbations, diabetes , pancreatic insufficiency, CF-related arthropathy and airway infections including methicillin-sensitive or -resistant Staphylococcus aureus , Pseudomonas aeruginosa, Burkholderia cepacia complex, fungal and non-tuberculous Mycobacteria infections. They received CF-specific treatments at similar frequencies. Conclusions Randomly-selected, sputum-producing patients within the MWCFC represent sputum-producing patients in the CFFPR. They have similar characteristics, lung function and frequencies of pulmonary exacerbations, microbial infections and use of CF-specific treatments. These findings will plausibly make future interpretations of quantitative measurements of inflammatory biomarkers generalizable to sputum-producing patients in the CFFPR. Electronic supplementary material The online version of this article (10.1186/s12874-019-0705-0) contains supplementary material, which is available to authorized users.

Advances in the care and treatment of cystic fibrosis (CF) have led to improved mortality rates; therefore, considerably more individuals with CF are living into adulthood. With an increased number of CF patients advancing into adulthood, there is the need for more research that surrounds the aging adult CF patient. It is important to conduct research and collect results on the aging CF population to help better prepare the CF patient, who is dealing with the heavy treatment and financial burden of their disease, build autonomy and increase their quality of life. Of note, research has found that social, behavioral, and physical factors influence the ability of those with CF to follow dietary recommendations. A primary treatment goal in CF is a high calorie, high protein, and high fat diet. A socio-economic factor that has not been adequately investigated with regards to dietary compliance of individuals with CF is food insecurity. The aim of this community case study was to document the experiences and estimate the prevalence of food insecurity among CF patients residing in Idaho. The correlation between food insecurity and health outcomes (lung function and body mass index) was also examined. Participants included adult patients and parents of pediatric patients with CF. Food insecurity rates among CF patients of all ages were found to be significantly higher than that seen in the overall community; however, no specific correlation between food insecurity and body mass index (BMI) or lung function emerged. This case study highlights the need for continued research around food access issues in this patient population. The data resulting from this study shows the value of CF advocacy organizations promoting efforts to build resources and provide education around food insecurity issues.

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