Cystic fibrosis and renal disease

Clinical and Laboratory Findings of Infants with Cystic Fibrosis

“…Akciğer transplantasyonu sonrasında renal histopatolojik lezyonlarda artış bildirilmiştir. 44 İdrar kültürüne hastaların tamamında ilk tanı esnasında bakıldı ve %30,8 hastada pozitif bulundu. Böbrek fonksiyonlarında bozukluk %19,2 ve ultrasonografide renal parankim ekosunda grade-1 artış %14,1 oranında tespit edildi.…”

Glomerulonephritis and the Cystic Fibrosis Patient

“…One autopsy study of thirty-eight CF patients found that thirty-five had evidence of nephrocalcinosis, including one still-born and two neonatal infants (Katz et al, 1988). This predilection seems to be multifactorial and results from the interplay of 1) impaired vitamin D absorption leading to impaired calcium absorption, 2) chronic disease characterized by increased metabolic tempo, 3) immobility, 4) increased osteoclast activity, 5) inadequate caloric intake due to increase work-of-breathing, 6) an imbalance between protein anabolism and catabolism, and 7) loss of oxalate-degrading bacteria due to frequent, and often chronic antibiotic use (Stephens & Rigden, 2002). Furthermore, Andrieux et al, in their 2010 study, found that 75% of children in their study population had hypocitraturia and 70% had hyperoxaluria, both of which are risk factors for nephrolithiasis (Andrieux et al, 2010).…”

“…In literature reviewed for their 2002 article on renal disease in Cystic Fibrosis, Stephens and Rigden report that IgA Nephropathy appeared to be the most common form of glomerulonephritis described in CF patients, though the occurrence of this condition appeared rare (Stephens & Rigden, 2002). With respect to all renal disease in CF patients, the most common renal pathology found has been nephrocalcinosis.…”

“…In Stephens and Rigden's review of renal disease in Cystic Fibrosis, they report that among the glomerulonephritides, IgA Nephropathy is the most frequently reported (Stephens & Rigden, 2002). In a 1999 case series reported by Stirati and colleagues, four out of the five CF patients who underwent a renal biopsy for proteinuria had findings consistent with IgA Nephropathy.…”

“…Thus, this autopsy study would suggest that MPGN is a major cause of renal disease in the CF patient. However, more contemporary reviews of the subject do not find nor discuss MPGN in the CF patient (Stephens & Rigden, 2002;Yahiaoui et al, 2009). Indeed, the only recent report of this association was published by Soriano andcolleagues in 2008 (Soriano et al, 2008).…”