Fanconi Syndrome and CKD in a Patient With Paroxysmal Nocturnal Hemoglobinuria and Hemosiderosis

Hsiao, Po-Jen; Wang, Shou‐Chieh; Wen, Mei‐Chin; Diang, Liang-Kuang; Lin, Shih‐Hua

doi:10.1053/j.ajkd.2009.07.022

Cited by 14 publications

(12 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Laboratory studies showed proximal renal tubular acidosis, hypophosphotemic hyperphosphaturia, normoglycemic glucosuria, and aminoaciduria. [1821] In three of our patients, there was systemic metabolic acidosis, but the median urine pH was 6.0. The FEHCO 3 was more than 15%, suggesting the diagnosis of proximal renal tubular acidosis.…”

Section: Discussionmentioning

confidence: 91%

“…Iron and hemosiderin deposits occur mainly in proximal tubules and to a lesser degree in the loop of Henle and interstitium. [192021] In animal models of experimental hemosiderosis, a decreased glomerular filtration rate (GFR) associated with tubular dysfunction is almost always present. [22] Not many patients of Fanconi syndrome due to PNH have been reported in the past.…”

Section: Discussionmentioning

confidence: 99%

“…[22] Not many patients of Fanconi syndrome due to PNH have been reported in the past. [21] In a previous report, a 51-year-old female had PNH diagnosed 17 years earlier. She had received multiple blood transfusions because of hemolytic episodes.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Renal manifestations in paroxysmal nocturnal hemoglobinuria

et al. 2017

View full text Add to dashboard Cite

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired chronic disorder characterized by a triad of clinical features - hemolytic anemia, pancytopenia, and thrombosis. Not many reports of renal involvement in PNH are available in literature. We present a case series of PNH with renal involvement. We present the data of PNH patients who attended to Departments of General Medicine and Nephrology at a government-run tertiary care institute in South India. The diagnosis of PNH in these patients during initial phase, between 1998 and 2004 was based on sucrose lysis and Ham's test. After 2004, the diagnosis was based on flow cytometry to detect CD59 (membrane inhibitor of reactive lysis), a glycoprotein, and CD55 (decay accelerating factor) in regulation of complement action. The patient data were collected from 1998 to 2014. There were 14 patients of PNH in this period. The mean age was 37 years and the range was 16–68 years. There were eight females. Acute kidney injury (AKI) was noted in six patients. Dialysis was performed in four of them. The mean serum creatinine and urea at the initiation of dialysis were 5.4 ± 0.6 and 64.1 ± 6.1 mg/dl, respectively. The median number of hemodialysis sessions done was four. Renal biopsy was done in four patients. In three patients, the urinalysis and serum chemistry were suggestive of Fanconi syndrome. In our patients, three renal manifestations of PNH were identified. They were AKI, renal vessel thrombosis, and Fanconi syndrome. Chronic renal failure was not identified.

show abstract

Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Renal manifestations in paroxysmal nocturnal hemoglobinuria

et al. 2017

View full text Add to dashboard Cite

show abstract

“…Iron deposition can result in proximal tubular dysfunction [8,9,10]. In addition, chronic renal failure due to hemosiderosis and subsequent interstitial scarring can occur in patients with long-standing PNH [10,11,12,13,14]. Electron microscopy of renal parenchyma confirms the loss of proximal tubular brush borders and demonstrates decreased infoldings at the basolateral membrane of proximal tubular epithelial cells.…”

Section: Case Discussionmentioning

confidence: 99%

Renal Involvement in Paroxysmal Nocturnal Hemoglobinuria

2013

View full text Add to dashboard Cite

Renal involvement in paroxysmal nocturnal hemoglobinuria (PNH) is not usually apparent but in cases with clinical involvement varies from reversible acute dysfunction to chronic irreversible damage. Early diagnosis and treatment is crucial to prevent disease progression and irreversible chronic kidney disease (CKD). The ultimate outcome of CKD in many patients is the need for renal replacement therapy, which necessitates ever-growing dialysis and transplantation programs, thereby imposing a significant economic burden on the healthcare system. In a third-world country like Pakistan, increased burden due to CKD can be very hard on families. Doctor visits, hospitalization, and dialysis are all out-of-pocket expenses, therefore prevention, early detection, and timely intervention are the only cost-effective strategies. We report a case of acute kidney injury (AKI) due to PNH. This case shows AKI as one of the complications of PNH which may have a clinical course like acute tubular necrosis (ATN). This could be due to ATN or AKI superimposed on CKD due to hemosiderin deposits in the renal tubular epithelial cells. Our patient was dialyzed initially and discharged with a permanent catheter in place with advice to continue dialysis three times a week. He required dialysis for 1 week then started producing urine. His subsequent outpatient visits showed improved renal function. The permanent catheter was removed and maintenance dialysis was stopped. Here, we briefly review the literature on renal involvement in PNH, treatment options for PNH and pigment-induced nephropathy followed by a question-and-answer session at the clinicopathological conference held on March 4, 2011, at Sindh Institute of Urology and Transplantation in Karachi, Pakistan.

show abstract

“…This excess iron can be detected by magnetic resonance imaging [9, 10]. Excess iron deposition can result in proximal tubular dysfunction [2, 11, 12]. Chronic renal failure due to hemosiderosis and subsequent interstitial scarring may occur in patients with long-standing PNH [2, 13].…”

Section: Discussionmentioning

confidence: 99%

Improvement of Renal Function by Long-Term Sustained Eculizumab Treatment in a Patient with Paroxysmal Nocturnal Hemoglobinuria

Ninomiya

Obara

Niiori-Onishi

et al. 2015

Case Reports in Hematology

View full text Add to dashboard Cite

Chronic kidney disease (CKD) is one of the major manifestations of paroxysmal nocturnal hemoglobinuria (PNH). CKD in PNH is induced mainly by intravascular hemolysis of PNH-affected red blood cells (RBC) missing the glycosylphosphatidylinositol-anchored proteins with complement-regulatory activities, CD55 and CD59. CKD develops by heme absorption in the proximal tubules resulting in the interstitial deposition of iron in the kidneys. We administered eculizumab to a patient with PNH, who was one of 29 patients enrolled in the AEGIS clinical trial, an open-label study of eculizumab in Japan. The patient was complicated by stage 3 CKD with impaired estimated glomerular filtration rate (eGFR), at grade G3b, and had obvious proteinuria (2-3+, 1-2 g/day). In a two-year extension to the 12-week AEGIS study, eGFR improved significantly, and the eGFR has since been maintained at grade G2 without proteinuria by sustained eculizumab treatment (>6 years). Renal function improved and maintained by long-term sustained eculizumab treatment, presumably by clearance of iron from the kidney as well as inhibition of the production of anaphylatoxin C5a, even in advanced stages of CKD, is one of the benefits of eculizumab treatment in PNH.

show abstract

Fanconi Syndrome and CKD in a Patient With Paroxysmal Nocturnal Hemoglobinuria and Hemosiderosis

Cited by 14 publications

References 21 publications

Renal manifestations in paroxysmal nocturnal hemoglobinuria

Renal manifestations in paroxysmal nocturnal hemoglobinuria

Renal Involvement in Paroxysmal Nocturnal Hemoglobinuria

Improvement of Renal Function by Long-Term Sustained Eculizumab Treatment in a Patient with Paroxysmal Nocturnal Hemoglobinuria

Contact Info

Product

Resources

About