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.
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.
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).
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